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ROCKEFELLER MEDICALLIBRARY
NATIONAL HOSPITAL LIBRARY Not to be taken away.
NERVOUS DISEASES, DANA,
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FIG. 1. GIANT CELL, ANTERIOR CENTRAL.
FIG 2 PYRAMIDAL CELL, 3D LAYER INFERIOR PARIETAL.
FIGS. 3, 4, 5. SMALL PYRAMIDAL CELLS, 2D LAYER.
FIG. 6.' SPINDLE CELL, 4TH LAYER.
FIG. 7. NEUROGLIA CELL.
TEXT-BOOK
OF
NERVOUS DISEASES
« AND
PSYCHIATRY
For the use of Students and Practitioners of Medicine
Professor of Nervous Diseases in Cornell University Medical College; Visiting Physician to Bellevue Hospital; Neurologist to the Montenore Hospital; Neurologist to the Woman's Hospital; Consulting Physician to the Manhattan State Hospital; ex-President of the American Neurological Association; ex-President of the New York Academy of Medicine; Corresponding Member of the Societe de Neurologie, etc.
ILLUSTRATED BY TWO HUNDRED AND SIXTY-ONE ENGRAVINGS AND THREE PLATES IN BLACK AND COLORS
BY
CHARLES L. DANA, A.M., M.D., LL. D„
SEVENTH EDITION
LONDON J. & A. CHURCHILL
7 GREAT MARLBOROUGH STREET
1909
Printed in New York By WILLIAM WOOD AND COMPANY
see
PREFACE TO THE SEVENTH EDITION.
The seventh edition of this work has been very largely revised, and I have tried to make it a correct presentation of Neurology of the present day. When the first edition was written, there were no very adequate descriptions of the finer anatomy of the nervous sys- tem in English text-books. Hence, I introduced that feature into the work. While there is less necessity of such an addition at the present time, it has seemed to me wise to allow it to stand. I have made some additions to the sections on the histology of the neuron and oil the anatomy and physiology of the brain. The work of Sherrington, Grunbaum, Campbell, and Horsley, in particular, has made this necessary. Something has been written regarding the methods of psychotherapy, and the subjects of neurasthenia, hysteria, and psychasthenia, which latter condition I described four years ago under the term phrenasthenia, have been re-written and re-arranged. Muchhas been added also to the subjects of neuritis, tabes, multiple sclerosis, brain tumors, the tics, and paralysis agitans. A good many illustrations have been added. Enough of what is now old or not im- portant, has been cut out, so that the book has not been materially enlarged.
No one can possibly appreciate so well as myself, the defects and limitations of the present work. As it has, however, maintained its popularity for a long time, I must feel convinced that it has some merits, and I have tried in the present volume, to make it more worthy of the reception which has been given to it in the past.
I am under obligation to many of my confreres for allowing me to use illustrations from their articles. Dr. G. H. Kirby, of the Manhattan State Hospital, has prepared a series of histories illustra- tive of the different psychoses and these have been appended in their proper place in the psychiatrical portion of the work. I am much indebted to Dr. Kirby for the careful and efficient work he has done. I am also under obligation to my friend, Dr. J. Ramsey Hunt, for his assistance in the proof-reading of the book. C. L. D.
New York, April, 7, 1908.
iii
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PREFACE.
IT is the object of the author in this treatise to present the sci- ence of neurology in a concise yet as far as possible complete form. Each subject has been taken, all the available facts regarding it have been ascertained, the writer's own experience has been collated, and with the data thus gathered the chapters have been written. The labor involved in such a task has been very great, but I am encouraged to believe that the result will be a useful one; for the work does not compare or compete with the large treatises which are already in the field nor with the smaller introductory text-books. I have tried to furnish a book which will be suitable for the student and practitioner and not valueless to the specialist.
The extreme importance of a knowledge of anatomy has led me to pay especial attention to furnishing in a condensed form the most recent accessions to our knowledge of this subject. Starting with the facts that can be gained in ordinary anatomical works, the student can, I believe, acquire a good idea of modern neuro-anatomy with the help of the anatomical chapters given here.
In the classification of nervous diseases and the description of their pathology, I have tried to apply the modern knowledge of , general pathology as modified by bacteriology. This 1 have done conservatively, yet not less than in my opinion is absolutely de- manded. A good deal of havoc will be wrought eventually in our conception of the nature of nervous diseases by the newer patho- logical doctrines ; I have made as little change as was consonant with undeniable facts.
The limits placed upon me have made it impossible to furnish a bibliography or to give due credit to every original investigator. Full references to literature are to be found in the works of Hirt, Erb, Seeligmuller, Ross, and Gowers.
In many topics I have been much helped by valuable monographs of my American colleagues. While a part of these are credited to their proper source in the text, I feel that I ought to refer here to some of the articles that have been of special service to me. They include monographs on Spinal and Brain Tumors by Mills and Lloyd ; on Cerebral Palsies of Children by B. Sachs; on Muscular Dystrophies
v
PREFACE.
and Writer's Cramp, by G. W. Jacoby and by M. Lewis; on Aphasia, Cerebral and Spinal Localizations by M. Allen Starr; on Cranial Temperatures and on Neurasthenia by L- C. Gray ; on Degenerative Neuritis, by W. H. Leszynsky ; on Poliomyelitis, by Wharton Sinkler; on Craniometry and Cranial Deformities, by F. Peterson and by E. D. Fisher; on Angioneurotic CEdema, by Jos. Collins; on Brain Tumors, by P. C. Knapp, and on Sclerosis of the Cord, by J. J. Putnam. I am indebted to Tourette's recent treatise on hysteria, to that of Fere on epilepsy, and to the annual volumes of Bourneville on these subjects. The masterly lectures of Charcot and the treatises of Ross, Gowers, Hammond, Hamilton, and Putzel have necessarily been freely used. In the anatomical part I have used the works of Edinger, to whose courtesy I am particularly indebted, the treatise of Ober- steiner, and many monographs by Golgi, Marchi, Cajal, His, Waldeyer, and others. My own work in teaching anatomy and pathology has enabled me to do more than present a compilation.
I must finally express my thanks to my publisher, Mr. W. H. S. Wood, for his patience and helpful generosity in my efforts to make my work a production that would be creditable to American neurology.
To the Student.
As a special text-book the present work will be used by two classes of readers, one consisting of those who simply consult it for reference in connection with their cases, the other composed of students who desire to ground themselves systematically in a knowl- edge of neurology. To this latter class I venture some advice as to the method they should pursue. Neurology is a difficult branch of medicine to master, nor is there any royal road to it. Still, it can be made comparatively easy if its study is undertaken in a proper and systematic way.
In using the present work, the student should first refresh his general knowledge of nervous anatomy as furnished in ordinary text- books. He should then go carefully over the anatomical descriptions here given of t'ie general structure of the nervous system and of that of the nerves, spinal cord, and brain. A thorough knowledge of anatomy and physiology makes clinical neurology comparatively easy, and in fact reduces much of it simply to a matter of logical deduction.
The student should next master the general facts of nervous pathology, symptomatology, and etiology, for he will find common laws underlying apparently the most varying phenomena. Finally,
PREFACE.
Vll
he must begin to study the special diseases. The number of these is very great; in the present work I have described 176. Many of these are rare, and it would be wrong for the student to burden his memory with the details about them. He need know only of their existence and general physiognomy. There are, however, according to my enumeiation, about 65 nervous diseases which are either very common or extremely important, and it is these that the student should master and make part of his working knowledge. Since the distribution and names of the common and rare diseases may be a useful guide, I append here a table and a list:
|
Peripheral. |
Spinal Cord. |
Brain. |
Functional. |
Totals. |
|
|
Common and important |
|||||
|
nervous diseases |
31 |
12 |
IO |
65 |
|
|
Rare |
56 |
27 |
16 |
I I |
I I I |
|
87 |
40 |
28 |
21 |
176 |
The common or important nervous diseases are : General. — Neuritis, multiple neuritis, degeneration, neuralgia, paresthesia (5).
Cranial Nerves. — Anosmia, optic neuritis, optic atrophy, ptosis, ophthalmoplegia, abducens palsy, headache, migraine, trigeminal neuralgia, facial spasm, facial palsy, tinnitus, vertigo, ageusia, wryneck (16).
Spinal Nerves. — Cervical neuralgia, hiccough, brachial palsies, single and combined, bracial neuralgia, intercostal neuralgia, herpes zoster, lumbar neuralgia, sciatica, leg palsies (10).
Spinal Cord. — Spina bifida, hemorrhage, pachymeningitis, lep- tomeningitis, poliomyelitis, transverse myelitis, acute and chronic, secondary degenerations, locomotor ataxia, the progressive muscular atrophies, bulbar palsy, muscular dystrophies, spinal irritation (13).
Brain. — Malformations, hyperemia, pachymeningitis, leptomen- ingitis, simple, tuberculous, and epidemic, abscess, hemorrhage, embolism, thrombosis, children's palsies, syphilis (12).
Functional. — Epilepsy, hysteria, the tics, chorea, tetanus, neu- rasthenia, spermatorrhoea, exophthalmic goitre, occupation neuro- ses, paralysis agitans (10).
TABLE OF CONTENTS.
PART I.
CHAPTER I.
General Anatomy, Physiology, and Chemistry. The General Histol- ogy of the Nervous System — The Neuronic Architecture of the Nerv- ous System, i
CHAPTER II.
The Causes of Nervous Diseases, 24
CHAPTER III.
General Pathology, 30
CHAPTER IV.
General Symptoms, 34
CHAPTER V.
Diagnosis and Methods of Examination. Spinal Cord Levels of the Superficial and Deep Reflexes — Disorders of Sensation — Cerebrospinal Fluid, ' 42
CHAPTER VI.
Hygiene, Prophylaxis, Treatment. Diet — Exercise — Hydrotherapy — ■ Massage — Climate — Electricity — Electro-Therapeutics — Radiant En- ergy— Psychotherapy, . 67
CHAPTER VII.
General Diseases of the Peripheral Nerves. Anatomical — General Pathology — Agina — Neuritis — Degeneration — General Symptoms — Mul- tiple Neuritis — Sensory-Motor Type — Endemic and Epidemic Types — Acute Pernicious — Complicating Forms of Neuritis and Neuritic Degen- eration— Tumors, 91
CHAPTER VIII.
Motor Disorders of Special Nerves. The Ocular Muscles — Gen- eral Symptoms — The Ophthalmoplegias — Third Nerve — Iris — Fourth Nerve — Sixth Nerve — Muscular Asthenopia and Muscular Insufficien- cies— Spasmodic Diseases of the Ocular Muscles — The Motor Branch of the Fifth Cranial Nerve — The Facial Nerve — Facial Spasm — Facial Palsies — The Glosso-Pharyngeal Nerve — Spinal Accessory — The Hypo-glossus, 114
ix
X
TABLE OF CONTENTS.
CHAPTER IX.
Neuroses of the Motor Spinal Nerves. The Upper Cervical — The
Lower and Brachial — The Thoracic — The Lumbar — The Sacral, . . 148
CHAPTER X.
Sensory Neuroses of the Cerebrospinal Nerves. Paresthesia — Neu- ralgia— Neuroses of the Nerves of Special Sense — The Optic Nerve— Trigeminal Nerve — Headache — Seventh Nerve — Acoustic Nerve — Nerv- ous Deafness — Tinnitus Aurium — Vertigo— Sensory Neuroses of the Glosso-Pharyngeal Nerve — Upper Cervical Nerves — Lower Cervical and Brachial Nerves — Intercostal Nerves — Lumbar Nerves — Mechani-
cal Origin — Peripheral Vasomotor and Trophic Neuroses, .... 169 CHAPTER XI.
Diseases of the Spinal Cord. Anatomy and Physiology 234
CHAPTER XII.
Diseases of the Spinal Cord. Malformations — Spina Bifida — Spinal Hemorrhage — The Caisson Disease — Hyperemia — Anaemia — Meningi- tis, 260
CHAPTER XIII.
Myelitis. Acute and Chronic Senile Paraplegia — Acute Ascending Paraly- sis, 274
CHAPTER XIV.
The Degenerative Diseases of the Spinal Cord. Locomotor Ataxia — Spastic Spinal Paralysis — The Combined Scleroses — Hereditary Spinal Ataxia — Hereditary Cerebellar Ataxia, 297
CHAPTER XV.
The Progressive Muscular Atrophies and Muscular Dystrophies. Hereditary Muscular Atrophy of the Peroneal Type — Chronic Anterior
Poliomyelitis — Glosso-Labio-Laryngeal Paralysis — Myasthenia Gravis — Amyotrophic Lateral Sclerosis — The Progressive Muscular Dystrophies, 339
CHAPTER XVI.
Tumors and Cavities of the Spinal Cord. The Recognition of Dis- eases of the Cauda Equina, 366
CHAPTER XVII.
Anatomy and Physiology of the Brain. The Connecting Tracts. . 380
CHAPTER XVIII.
Diseases of the Brain and its Membranes. General Symptons —
Malformations — Alcoholic Meningitis, 423
CHAPTER XIX.
Diseases of the Brain. Inflammation — Multiple Scleroses — The Apo- plexies— Infantile Cerebral Palsies, 448
TABLE OF CONTENTS. xi CHAPTER XX.
Tumors of the Brain. Syphilis, 495
CHAPTER XXI.
Functional Nervous Diseases. Epilepsy, 523
CHAPTER XXII.
Hysteria, 538
CHAPTER XXIII.
The Myoclonias or Twitching and Tonic Spasms. Chorea of Syd- enham—Myotonia— Rabies and hydrophobia, 555
CHAPTER XXIV.
Neurasthenia. The Sexual Neuroses and Psychoses — Exophthalmic
Goitre, 574
CHAPTER XXV.
Professional Neuroses, 609
CHAPTER XXVI.
Paralysis Agitans, 618
CHAPTER XXVII.
Trophic and Vasomotor Disorders. Hemiatrophy — Acromegaly —
Myxcedema — Cretinism — Angio-Neurotic (Edema, 629
CHAPTER XXVIII.
The Disorders of Sleep. Insomnia — Hypnotism — Morbid Somnolence
— Catalepsy — Trance — Lethargy — The Sleeping Sickness, .... 639
CHAPTER XXIX.
Cranio-Cerebral Topography, 656
CHAPTER XXX.
Neurological Therapeutics. Hydrotherapy — The Rest Treatment, . 662
PART II. CHAPTER I.
Definitions, Classification, General Etiology, General Path- ology, 671
CHAPTER II.
General Psychology, 677
xii
TABLE OF CONTENTS.
CHAPTER III.
General Symptoms and Symptom Groups. The Physical Symptoms of
Insanity, . 683
CHAPTER IV.
Methods of Examination and Diagnosis, 692
CHAPTER V.
General Prognosis and Treatment, 695
CHAPTER VI.
The Minor Psychoses and Psycho-Neurosis. Phrenasthenia — Psy- chasthenia— Hypochondriasis — Hysteria — Heredity or Constitutional
Inferiority, 697
CHAPTER VII.
Dementia Precox, 708
CHAPTER VIII.
Melancholia and Mania, 717
CHAPTER IX.
Paranoia, 732
CHAPTER X.
CONFUSIONAL INSANITY, 738
CHAPTER XI.
Organic Psychoses. General Paresis, 750
CHAPTER XII.
The Psychoses of Senility. Psychoses of Epilepsy, of Gross Organic
Brain Disease, and of Trauma, 775
PART I.
DISEASES OF THE NERVOUS SYSTEM.
CHAPTER I.
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY.
In studying the phenomena of life in the human body, we as physicians first learn about its normal structure and functions. We then note the new phenomena which develop when disease comes on, the causes which produce them, and the anatomical changes lying ) back of them ; we group our facts and give the disease a name. Lastly we apply the methods by which the disorder can be expelled and future attacks prevented. In fine, we investigate our subject just as we do that of any branch of natural history. Our study divides itself, therefore, into
Normal anatomy and physiology.
Etiology, a study of the causes.
Symptomatology, a study of the morbid phenomena. Pathology, under which we include a study of the morbid anatomy and physiology.
Diagnosis, or the method of recognizing and separating out the different groups of diseases.
Prognosis, a forecast of the future course of the malady. Treatment and prophylaxis.
Again, although nervous diseases show many phases and have many different morbid changes behind them, there are certain features common to all. It simplifies their study, therefore, to learn first what these general features are, just as a person can better survey and plot out country in detail if he knows certain general facts about its boundaries and topography. Hence I shall first present in a general way an outline of the fundamental facts that touch more or less on all forms of nervous disease. My first chapters will be de-
2
DISEASES OF THE NERVOUS SYSTEM.
voted to a general description of the anatomy and physiology, and then of the etiology, symptomatology, pathology, diagnosis, prog- nosis, and treatment.
GENERAL ANATOMY.
The nervous system is derived from the epiblastic layer of the developing ovum, and its constituents are modifications of epithelial cells. These cells in the embryo are of two kinds: I l2\J l\ neuroD^as^s' wmcn develop into nerve-cells and fibers; I I and spongioblasts, which develop into a supporting
I I / structure called neuroglia (His). y~~ ( The nervous system is composed of :
U J (a) Neurons, which form the nervous tissue proper,
—I and are made up of nerve-cells, of their processes, one of Hlj which becomes a nerve-fiber; and neuroglia. }---( (b) Non-nervous tissue, consisting of connective
/]£ j tissue, blood-vessels, lymphatics, and epithelium. W-f These tissues are united together to form a central
W nervous system, consisting of the brain, spinal cord, and
the peripheral nervous system. This latter is composed FlG T of nerve-fibers, and structures attached to the termina- tions of the nerves, called end-organs, and finally the ganglionic or sympathetic nervous system.
The Arrangement of the Nervous System. — -The subdivisions of these parts, and their descriptions in detail, belong to general anatomy. But there have been so many special subdivisions, and particular
Fig. 2.
names given to them in recent years, that I deem it necessary, in order to prevent confusion, to describe briefly the subdivision accepted by modern anatomists. The names here used are those adopted by the
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY.
3
committee on anatomical nomenclature of the German Anatomical Society, and they have also been adopted by a large number of writers on neuro-anatomy.
Beginning with the brain, we find that its particular subdivisions are based upon the embryological development of this organ. As will be shown in more detail later, the brain is developed out of three vesicles, known as the anterior, middle, and posterior vesicles (Fig. i). The most anterior of these vesicles is the prosencephalon or anterior brain; the middle vesicle becomes the mesencephalon or mid-brain, and the posterior vesicle develops into the rhombencephalon or posterior brain.
The anterior vesicle develops two secondary vesicles : the anterior portion of these, including the corpora striata, olfactory lobes and the cerebral hemispheres, forms the telencephalon (I2), while the hinder portion of this vesicle, which includes the thalamus and mammary bodies, forms the diencephalon (I1). The middle vesicle is the mesen- cephalon, and it includes the corpora quadrigemina and cerebral pedun- cles (II). The posterior vesicle is divided, from before backward, into three different parts: (i) the isthmus, which includes the superior cerebellar peduncles and valve of Vieussens, and part of the cerebral peduncles; (2) the metencephalon or hind-brain, which includes the cerebrum and pons Varolii; and (3) the myelencephalon or after-brain, which includes the medulla oblongata.
f Prosencephalon / 1. Telencephalon.
I (anterior brain). \ 2. Diencephalon.
I I1 and I2
■p . 1 Mesencephalon \ 3. Mesencephalon.
Brain. ; (middle brain). II
I Rhombencephalon \ ^^mUS" 1 i
z , • r , • N 1 5. Metencephalon. (posterior brain). a tv/t i i. 1
I III and IV L ' MyelencePhalon-
These different parts can be understood better by means of the accompanying figure (Fig. 2), which represents in a schematic way the brain of a mammal (Edinger).
They are intimately connected by strands of nerve-fibres, and are connected closely also with the next portion of the nervous system, the spinal cord. The brain and spinal cord are spoken of as the cerebrospinal axis, and this is in close relation with the peripheral nervous system.
This peripheral nervous system is composed of two portions — first, the cerebrospinal mixed nerves, whose origin, distribution, and rela- tions are comparatively easy to follow; and second, the ganglionic or
4
DISEASES OF THE NERVOUS SYSTEM.
sympathetic nervous system. This has relations which are not so easily described, and which are as yet not wholly understood. This portion of the nervous system is composed of two sets of ganglia — one, the vertebral ganglia, i.e., the chain of ganglionic masses on each side of the vertebral column, and of certain ganglia connected with the cranial nerves; secondly, a very large number of ganglionic masses
Fig. 3. — Multipolar cell of first type. (Deiter's.)
distributed in the viscera, and known as the peripheral ganglia. The sympathetic nervous system is made up very largely of nerves from the cerebrospinal centers. Motor fibres pass out through the anterior roots of the spinal cord, sensory fibres arise from the posterior spinal ganglia, and both pass in part to the peripheral ganglia and the viscera direct, and part to cells in the vertebral ganglia, with which they are connected. The ganglionic system contains also nerve-cells of its
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY.
5
own, which are, however, in dependence in the main upon influences from the cerebrospinal centres. Some of these cells send fibres into the spinal cord, and according to Collins and Onuf the sympathetic ganglia have representations in the spinal gray matter.
The General Histology of the Nervous System.
The NERVE-CELLS form the central body of the neuron and are minute objects varying much in size. The largest are .1 mm. (¥H
Fig. 4. — Multipolar of second type. (Golgi's.)
in.) in diameter and are almost visible to the naked eye. The smallest are. 7^ inch) in diameter; so that the average diameter
is rather greater than that of a white blood-cell. In shape nerve- cells are for the most part irregularly spheroidal, but some are pyramidal, others spindle- or flask-shaped, and others globular. They all give off one or more fine processes or poles, and hence, in accordance with the number of these, the nerve-cells are often spoken of as multipolar, bipolar, or unipolar.
6
DISEASES OF THE NERVOUS SYSTEM.
In most cells one of the processes is continued on a long way and finally becomes a nerve-fibre. This process is called the axis-cylinder, or neuraxon or axon (Figs. 3 and 4 and plate). The other processes are relatively shor t and are called protoplasmic processes, or dendrites. The nerve-cell, then, is a protoplasmic body giving off several dendrites and usually a single neuraxon, the whole forming the neuron. The dendrites branch off irregularly and subdivide, but never anastomose. In some parts of the nervous system they have upon them little nodules or
Fig. 5. — Pyramidal cell of cerebral cortex, a, Neuraxon. The fibroid masses are shown. (Cajal.)
buds, and in the cerebral and cerebellar cortex these are so numerous as to give them the appearance of budded stalks. The dendrites are usually not very long, but in some cells they extend a very great way, reaching many times the diameter of the cell. The axis- cylinder process, or neuraxon, is given off directly from the body of the cell, as a rule. It very soon becomes clothed with a thin sheath (myelin sheath), and as it passes along gives off branches at right angles, which form what are known as the collaterals. The
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY.
7
neuraxon and collaterals finally end by splitting up into a number of fine branches, which lose their myelin sheath and form the end-brush or terminal arborization. In some neurons instead of an end-brush, there are several minute ovoid bodies which lie upon the cell or its den- drites. They are called " end-buds " or terminal "buttons" (Fig. 5). They are seen especially in the medulla and pons, but not in the cerebral or cerebellar cortex. The axis-cylinder process or neuraxon does not anastomose with other cells either through its own end-brush or through the end-brushes of its collaterals. The end-brushes, how- ever, pass in among the dendrites of other cells, and sometimes closely surround the cell-body. In this way one neuron comes into very intimate relation with others, but there is never any true union. Each neuron of the nervous system is an independent unit.*
There still remain some important facts to be stated regarding the intimate structure of the cell-body proper. This is composed of a
semifluid albuminous substance, somewhat like the white of an egg, and spoken of in a general way as protoplasm or, more specifically, as the cytoplasm. Within this lies the nucleus and within the nucleus a nucleolus. The body proper is not homogeneous, but is made up of a network of fine fibers or librillae which pass in bundles from dendrite to dendrite and from dendrite to the neuraxon, called the endocellular fibrillar network (Fig. 5). Within its meshes and arranged in a rather definite manner are certain stainable bodies called Nissl or chromophilic granules (Fig. 6). These form the chromatic substance of the cell. While most nerve-cells have these granules, some, such as the granule-cells of the cerebellum, do not. The nerve-cells which
* This view is now denied by certain authorities, and there is no doubt that in some of the lower animals fibrils from the end-brushes of one neuron pass into the dendrites of another and thence through the body of the cell into its neuraxon. But though there may be this anatomical unity, the genetic and physiological independence of the neurons still continues to be a fact. Anatomists also describe a net-work of fibrils sur- rounding and lying upon the body of the cell. It is called the pericellular net-work and probably is in relation with the end-brushes, or terminal buttons, but it is believed to be glia tissue and not to have any anatomical connection with the fibrillary net-work in the cell-body.
Fig. 6. — Various forms of nervc-cell nuclei. (Cajal.)
8
DISEASES OF THE NERVOUS SYSTEM.
do stain and have the chromophilic granules are called somato- chromes, the others are called karyochromes (Nissl). The chromo- philic granules are arranged differently in cells of different function. They are believed to represent the nutrient substance of the cell, while the fibrillae form the conducting and functioning part. The cell-body usually contains a little pigment (Fig. 6).
The nucleus of the cell is a small spherical body, which is also made up of a reticulated structure known as the chromatin network, be- cause of its taking up dyes and staining very intensely (Fig. 6). Besides this network, however, there is a finer network, which is similar to that in the body of the cell, and which is known as the linin network. The chromatin is practically identical with nuclein. Within the nucleus is a smaller body, known as the nucleolus, which stains still more intensely.
Nerve-cells are surrounded by a pericellular space, but are not inclosed in capsules, excepting those of the posterior spinal and ver- tebral ganglia.
Central Nerve-cells.— -There are three kinds of central nerve-cells, that is, cells lying in the brain and cord, and they are classified in accordance with the peculiarities of the axis-cylinders (neuraxons) into :
1. Cells of the first type, or cells of Deiter. These are the kind just described above, and they form the great mass of nerve-cells. The neuraxon is continued as a nerve-fibre (Fig. 3).
2. Cells of the second type, or cells of Golgi. In these the axis- cylinder soon gives off numerous collaterals and quickly splits into a number of fine branches (Fig. 4). None of these branches ends in becoming a nerve-fibre and none travels far from the cell-body. These are probably association cells while type 1 are conducting cells.
3. Cells of the third type, cells of Cajal. In these there are two or more neuraxons. They are found in the superficial layer of the cerebral cortex.
Peripheral Nerve-cells. — The cells of the peripheral nervous sys- tem resemble fundamentally the central nerve-cells, but undergo some changes in order to adapt themselves to their peculiar func- tions. Thus the cells of the posterior spinal ganglia have quite peculiar anatomical characters. They are rather large in size, being from 29 to 60 M (4^ to inch). They are spheroidal in shape and have one process, which speedily divides in two, in a T-shaped fashion. They are surrounded by an endothelial sheath, which is analogous to the myelin sheath of the nerve-fibre. The cells have one large nucleus with a nucleolus. The body is composed of short granular fibres, which are arranged in a somewhat concentric layer.
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY.
9
The nerve-cells of the sympathetic or vertebral ganglia are very like the central nerve-cells in the anterior horns. They are multipolar in shape and have dendrites and a neuraxon. The neuraxon goes to other neighboring cells, or it passes on and becomes a fibre of Remak. The cell is surrounded by a connective tissue capsule lined with endothelium, like those of the posterior spinal ganglia. The peripheral or terminal sympathetic nerve-cells lying in the viscera resemble those of the vertebral ganglia. The cells of the special sense-organs have many peculiarities of shape and structure, but they are all developments of the same model. In the retina and olfactory bulb there are cells without axis-cylinders (spongioblasts of Cajal and granules of olfactory bulb).
P^ig. 7. — From a transverse section through the sciatic nerve, ep, Epineurium, p, perineurium; n, nerve fibres constituting a nerve bundle of fasciculus in cross-section; , at tissue surrounding the nerve. (Klein.)
Nerve-cells are classified in accordance with their shape and num- ber of processes. The multipolar cell is the common type and is found throughout the brain, cord, and sympathetic ganglia. Bipolar cells are found chiefly in the column of Clark of the spinal cord; and unipolar cells in the posterior spinal ganglia. Small nuclear cells and flask-shaped or Purkinje's cells are found in the cerebellum. Besides these are described in the brain cortex angular, granular, pyram- idal, globose, and spindle-cells.
The nkrvK-fibres of the nerve centres are found chiefly in the white tissue or white matter. In the periphery they form the nerve proper of gross anatomy. The peripheral nerve is composed of bundles of nerve-fibres called nerve fasciculi (Fig. 7) It is surrounded by a con- nective-tissue sheath called the sheath of Henle, or epineurium. From
n
IO DISEASES OF THE NERVOUS SYSTEM.
this sheath, connective-tissue fibres pass in and surround the fasciculi. The sheath of the fasciculus is called the perineurium. From the perineurium, strands of connective tissue run in among the ultimate nerve-fibres, forming the endoneurium (Fig. 8). Lymphatic spaces lined with endothelium exist in the layers of the peri- and endo- sheaths. In the nerve-centres, the nerve-fibres have no such sheaths, but are supported by a connective tissue and neuroglia frame-work.
The nerve-fibre is a long fine strand of tissue varying in diameter. It may be white or gray, according to its structure. It is composed from within out of (i) an axis-cylinder, (2) a myelin sheath, and (3) a neurilemma. (1) The axis-cylinder is the essential part of the
nerve. It is the prolongation of the neu- raxon of a nerve-cell and consists of proto- plasm. It is itself made up of fine fibrillae (primitive fibrillae) which run longitudinally. By means of reagents, a transverse striation can be seen also. (2) The myelin sheath, medullary sheath, or sheath of Schwann, sur- rounds the axis-cylinder. It is composed of a semifluid, fatty substance, which chemically consists of lecithin, neurin, and some choles- terin. It varies much in thickness, and this is the principal cause of the different sizes of nerve-fibres. The myelin sheath is inter- rupted at regular intervals by constrictions called the "nodes of Ranvier." These con- strictions involve the myelin sheath alone. The axis-cylinder passes through and the outer sheath (neurilemma) passes over it. There is a little granular matter at the point, called intercellular cement. The nerve-fibres, if they divide, always do so at a node (Fig. 11). The part between two nodes is called a nerve segment. In each segment there is an oval nucleus embedded in the myelin sheath. The nodes are about 1 mm. apart.
The myelin sheath is probably developed, like the axis-cylinder, from the epiblast, and is closely related nutritionally to the axis- cylinder, which it protects and isolates. (3) The neurilemma or primitive sheath is a delicate homogeneous covering forming the outer- most sheath of the nerve. It is of connective tissue origin. The sheath is absent in the fibres of the central nervous system and in some fibres of the periphery.
Variations in the Types of Fibres. — In accordance with the arrange -
Fig. 8. — A simple funiculus more highly magnified. The apparent small nucleated cells are sections of the nerve fibres and their axis cylinders. a, Axis cylinder; w, white substance of Schwann or medullary substance; ^neu- rilemma; e, endoneurium; p, perineurium; x, connec- tive-tissue cells of the same. (Piersol.)
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY.
II
ment [of the sheaths of the nerve-fibres, several kinds are described. The principal types are the medullated and non-medullated.
Medullated nerve-fibres make up the bulk of the white matter of the brain and cord and cerebrospinal nerves. They consist of a myelin sheath and axis-cylinder, and may or may not have a neurilemma. Fibres with myelin sheath, but without a neurilemma, make up the white matter of the central nervous system.
Non-medullated fibres, or fibres of Remak, occur principally in the sympathetic system, but they are also found in the cerebrospinal
Fig. 9. Fig. 10. Fig. ii.
Fig. 9. — Medullated nerve fibre, a, Axis cylinder; n, nucleus; m, medullary sheath; c, node of Ranvier.
Fig. 10. — Medullated nerve fibre, showing axis cylinder, nuclei of medullary sheath nodes, and oblique incisures of Schmidt.
Fig. 11. — Medullated nerve fibre, showing mode of division.
nerves. They are grayish and faintly striated, and consist of axis- cylinders, wkh a thin, homogeneous, nucleated sheath lying directly upon them. This sheath, however, cannot often be demonstrated (Shaefer).
Naked axis-cylinders are found in the peripheral terminations of nerves as well as in the brain and cord.
Size. — The nerve-fibres are of two kinds as regards size. The small fibres are about 2/x or TiriiTro" inch in diameter, the large 20^ or ^ 2*00 inch. The small fibres are connected with smaller cells, and either run a shorter course or are distributed to the involuntary muscular fibres of the blood-vessels and viscera. The motor fibres are larger than the sensory.
I 2
DISEASES OF THE NERVOUS SYSTEM.
The peripheral nerve-fibres, except the optic, have no neuroglia; they terminate in fine fibrillae among epithelial cells, or in special end- organs.
The central nervous fibres make up the white matter of the brain and cord. They are, like the peripheral nerves, the prolongations of the neuraxons. They are composed of an axis-cylinder process and myelin
Fig. 12. Fig. 13.
Fig. 12. — Non-medu Hated nerve fibre, n, Nucleus; b, striations.
Fig. 13. — Diagram showing the neuron and mode of connection between nerve units throughout the nervous system. 1, nerve cell; 2, nerve process; 3, collateral; 4, end brush; 5, nerve cell.
sheath, but have no neurilemma, and probably no nodes. At fre- quent intervals each fibre gives off branches at right angles form- ing the "collaterals."
Connections of Nerve-cells and Nerve-fibres. — One nerve-cell is never connected directly with another, so far as anatomical investigation can show. One nerve process becomes an axis-cylinder, receives a
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY.
13
myelin sheath, gives off collaterals, and finally breaks up into a fibrillary "end-brush" surrounding a cell, but not passing into it. There is physiological, but no apparent anatomical continuity (Fig. 13).
The Neuroglia. — The supporting tissue of the peripheral nerves is connective tissue only; that of the central nervous system is connective tissue and, in addition, a peculiar substance called neurog- lia. The neuroglia or supporting tissue of the nervous centres is derived from the epiblast. It is composed of cells with very num- erous and finely ramified processes, which make a supporting net- work about the nerve-cells and fibres (Fig. 14). The neuroglia
cells are sometimes known as "spider-cells." They differ somewhat in size and shape, but not in general characteristics. The cell- body is composed of granular protoplasm, lying in which is a large nucleus, within which is the nucleolus. The body of the cell is small in amount in proportion to the nucleus. The fibrillary processes form a felt-like network, and in regions where there is much neuroglia tissue this looks like a homogeneous matrix. It is, however, made up of the fine fibrils. These connect with the walls of the blood-vessels. Weigert has shown that the processes become changed in their chemical and physical character, so that they take a different stain
Fig. 14. — Glia celis as impregnated in siJver-chromate preparations. (After Ramon Cajal.)
\
14
DISEASES OF THE NERVOUS SYSTEM.
from that of the cell-body itself, and thus form a really separate structure (Plate I., Fig. 7). In inflammatory conditions the cells multiply, swell up, and assist in carrying off irritating products (scavenger cells of Lewis). Neuroglia tissue is richly deposited about the central canal of the spinal cord, beneath the ependyma of the ventricles, and beneath the pia mater of the brain and cord. The
epithelial cells of the central canal and ependyma of the ventricles send down fine processes which form a minor part of the supporting frame-work.
The Non-nervous Tissue — The Blood-vessels. — The peripheral nerves are richly supplied with blood. Each nerve receives arterial supply from many different branches, but always from the same general source. The artery passes to the nerve-sheath obliquely, then divides dichotomously and sends branches a long distance up
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY. 1 5
and down on the sheath. It may pierce the sheath, however, first, and then divide, as above described. The dichotomous branches send off arterioles and capillaries, which form plexuses about the nerve fascicles. These are "the interfascicular arcades." The arter- ies subdivide in such a way as to prevent sudden impact of a large blood-stream into the tissue of the nerve. In this respect the nerve circulation resembles that of the brain and cord. The veins subdivide dichotomously, like the arteries. They freely anastomose with the muscular veins, so that muscular action helps nerve circulation. The veins of the superficial nerves connect with those of the deep nerves (see Figs. 15, 16, 17).
The blood-vessels of the spinal cord and brain will be described later.
Lymphatic vessels and spaces are found in the epineurium and perineurium. There are no distinct lymphatics in the fasciculi, but lymph-spaces probably exist.
The Neuronic Architecture of the Nervous System.
Having described the component parts, I shall now show the way in which these parts are arranged to form the nervous system.
The nervous system, as already shown, is composed of single nerve units that are called neurons, and the neuron is made up of a cell-body and numerous processes, one of which is the neuraxon, the others the dendrites.
The neuraxon has always the function of carrying impulses away from the cell; it is a cellufugal fibre; the dendrites, however, bring impulses to the cell, and are cellupetal in function. The dendrites are in contact with end-brushes or end-buds of the neuraxons of other cells, and in this way receive the nerve impulse and transmit it to the cell-body. The nervous system is thus a mass of neurons which are packed closely together, and form with each other most intricate relations, but never connect directly one with another. No nerve-fibre or dendrite of one cell anastomoses with that of another, as blood-vessels do. Each neuron is anatomically independent.
It is the purpose of this neuronic mass to receive impulses from within or without the body, to transfer and modify them, and to send out impulses in such way as properly to control the vital func- tions and keep the individual in proper harmony with his environ- ment. The nervous system is a great receiving, regulating, control- ling, and discharging machine, the machinery being the neurons, the force that works in it being called nervous energy. The nerve-cells are the reservoirs and direct generators of this force, while the den-
i6
DISEASES OF THE NERVOUS SYSTEM.
drites and neuraxons receive and distribute it. The nerve-cells are massed together for the most part in the brain and spinal cord, form- ing the gray matter, while the neuraxons as distributors make up the white matter and the cranial, spinal, and sympathetic nerves. It is convenient to make a division, therefore, into the central nervous system, or cerebrospinal axis, and the peripheral nervous system, which includes the cranial and spinal nerves and the sympathetic nervous system. Since the nerve-fibres of the brain and cord are white in texture, while the cells in mass are of gray color, it is very easy to distinguish the deposits of cells from the fibres and thus make subdivisions of the central nervous tissue. One portion of this gray matter is found deposited in the centre of the spinal cord, extending up to the floor of the medulla, thence underneath and around the aqueduct of Sylvius to the floor of the third ventricle. This is called the central gray matter. Another deposit, much larger in amount, covers the whole of the cerebrum and cerebellum, and forms the cerebral and cerebellar cortex. Smaller deposits make up the great basal ganglia, corpus striatum, optic thalamus, and cor- pora quadrigemina, besides several small deposits (the small basal ganglia), such as Luys' body and the red nucleus.
The peripheral nervous system contains nerve-cells, as well as fibres. Their anatomical arrangement is easily understood, but their relation to the central nervous system is less simple.
Most of the nerve-cells of the periphery, aside from those in the special sense-organs, like the eye and nose, have been considered to belong to a kind of special or "sympathetic" nervous system. There is no harm in using this nomenclature, provided it is understood that this system is really a part of the rest of the nervous system and not in any sense an independent mechanism. The peripheral nerve-cells of this system are collected in two distinct groups. One is made up of the vertebral and cranial ganglia of the sympathetic, and the second of the peripheral ganglia of the sympathetic, such as the cells of the plexus of Auerbach, Meissner, and the various interstitial ganglia of the glands and muscles.
I come now to a description of the general arrangement of these various nerve units; and here I must suppose that my reader has a knowledge of the ordinary anatomy of the subject.
The nerve-cells of the ganglia on the posterior spinal roots fur- nish the best starting-point in an attempt to trace out the connec- tions. These cells give off a single process, which quickly divides in a T shape. One branch of the T passes peripherally through a mixed spinal nerve to the skin, forming a sensory nerve. The other passes
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY. 1 7
centrally, enters the posterior spinal roots, and breaks up into little filaments, which surround a nerve-cell in the posterior horn or analo- gous nuclei. This forms the first or outer sensory neuron. The outer branch of the spinal ganglion cell which went to the periphery as a sensory nerve was its dendrite, or protoplasmic process, which has evolved into a sensory nerve and is cellupetal in function. The other process is the neuraxon proper and it is cellufugal, carrying impulses away from the ganglion cell into the cord.
The next neuron begins as a cell in the posterior horn, or in like parts. It sends a neuraxon up the spinal cord, a collateral branch passes to the cerebellar cortex, while the direct fibre surrounds a cell in the optic thalamus. This forms the second sensory neuron. The cell in the thalamus gives off a neuraxon which passes to the gray matter of the cerebral cortex, and here it either directly affects the cells in this region or does it through the medium of another shorter neuron, which is called "associative."
Thus each sensory impulse from the periphery reaches the con- scious centres of the brain by passing along three or four neurons. The primary neuron in all cases lies mainly outside the central ner- vous system and forms a sensory nerve. The sensory nerves do not therefore arise in the cord or medulla, but have their terminal nuclei there. Afferent nerves serve the purpose of conducting impulses which arouse sensation, also reflex action.
The sensory nerves of the surface of the body are called extero-ceptive when they excite reflex action. They are more closely associated with the cerebrum and the activity of the skeletal muscles. The viscera nerves which excite reflexes are called proprio-ceptive. They are associated with the cerebellum, and the visceral muscles. They give rise to the tonic reflex which keeps the voluntary muscles at a certain degree of tension (Sherrington).
The neurons of the brain cortex cannot yet be distinctly classi- fied, and I shall not attempt it here at all. The matter will be brought out more fully in connection with the anatomy of the brain. It is sufficient to say that nerve units connect together the cerebellum and cerebrum with the basal ganglia, the frontal lobes and the cerebellum, the two hemispheres of the cerebrum, and different areas of the cerebral cortex. Leaving out of consideration these neurons, which are largely psychic in function, we start with the large motor cells in the central convolutions of the brain. These send down neu- raxons, which pass into the spinal cord and surround the cells of the anterior horns. They form the primary or central motor neurons. The anterior horn-cells send off neuraxons, which pass out through the
2
1 8
DISEASES OF THE NERVOUS SYSTEM.
anterior roots and thence to the voluntary muscles. These are the secondary or peripheral motor neurons. These motor neurons have a double function — that of exciting muscles to contract, and that of inhibiting muscular activity and causing them to relax. The efferent nerves are almost equally inhibitory and motor. With each voluntary muscular act impulses to contract, excite for example the flexor groups, and impulses to relax, cause the opposing extensors to relax. The motor mechanism is a motor-inhibitory mechanism. This has been
Fig. 17. — Diagram showing the arrangement of the neurons or nerve units in the architecture of the nervous system. M. Neurons I. and II., Motor neurons; S. Neu- rons I., II., III., sensory neurons; A. Neuron, associative or commissural neuron.
especially established by Professor Sherrington. Besides this there are groups of cells in the lateral horns and central parts of the spinal cord which send off neuraxons that also pass through the anterior root, but they leave the cerebrospinal nerves and enter the vertebral sympathetic ganglia. Here they in part surround the cells of these ganglia and have their terminals there. These sympathetic ganglion cells in turn send neuraxons, which pass in the sympathetic nerves to the peripheral ganglia, where they meet a third group of neurons. They also con- nect with the other ganglia of their own class and send neuraxons
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY. 1 9
through the posterior spinal roots to the cord. It is not known with what neurons higher up in the nerve-centres the lateral horn-cells are connected, but probably with cells in the thalamus.
Such in outline is the neuronic architecture of the nervous system. I do not attempt here to work out the neurons of the special senses, nor to introduce the spinal cerebellar neuron. This will be done later. It is sufficient to say that the studies in this direction show a marvellous harmony as well as beauty in nature's scheme.
The neuronic architecture is shown in the accompanying diagram (Fig. 17).
GENERAL PHYSIOLOGY.
The Peripheral Neurons. — The nerves which run between nerve- centres and end-organs carry impulses each way. They are, therefore, divided into the afferent, centripetal or ingoing, and efferent, centri- fugal or outgoing. The old division into motor and sensory nerves will not answer, for there are many outgoing nerves which are not motor. The afferent nerves are : 1. The sensory, including:
Nerves of general / Pain nerves or pathic nerves, heat and cold or thermic sensation. \ nerves.
f Contact,
f Tactile, including < Pressure, ( Locality.
Nerves of special !
sensation. | Nerves of muscular and articular sense.
I Nerves of special sense of smell, sight, taste, hearing and ( space
w n f Kxtero-ceptive.
2. Lxcito-reliex nerves. <[ • ,
y Jrropero-ceptive (see note page 18).
Protopathic and Epicritic Sensibility. — According to Dr. Henry Head, the afferent nervous system is composed of two different sets of sensory fibres, called the protopathic and the epicritic.
The protopathic set or system which is distributed to the skin and viscera, and to the muscles.
(a) The fibres to the skin run in the peripheral nerves and give to it a low degree of sensibility. Fibres to the viscera which run in the sympathetic and give to it also a low degree of sensibility.
(b) A set of deep fibres to the muscles which supply the sense of deep pressure. These fibres run in the muscular nerves and are con- nected with the Paccinian bodies.
The "protopathic system," then, as a whole, consists of fibres of
20
DISEASES OF THE NERVOUS SYSTEM.
sensibility which supply the skin, the viscera, and all parts of the body, inside and out. It furnishes a low degree of sensibility to the viscera, muscles and to the skin. This system enables one to appre- ciate a sense of pain and temperature, though not to a very delicate extent.
There is another system of afferent fibres which supply the skin alone, a purely cutaneous system, and this is called epicritic. This epicritic sensibility enables us to appreciate light touch, the points of the compass, localization, and minor degrees of temperature, ranging between 220 and 400 C.
Epicritic sensation gives us the sense of the point (a variant of appreciation of relative size).
Protopathic sensation gives sense of pain apart from the knowl- edge of the pain producing object.
The epicritic fibres furnish a delicate and localized appreciation of pain. Protopathic fibres carry sensations which are badly local- ized, widely diffused and sometimes referred to other parts than that of the stimulation. The protopathic fibres are incapable of appre- ciating light touch, and minor degrees of heat and cold, and pain appreciation is a diffuse tingling and thrilling sensation. This distribution of sensory fibres exists only in the peripheral nerves. In the nerve-roots and central nervous system the systems are fused. [The above description of the views of Dr. Head and his associates has been seen and corrected by Dr. Head, of whose courtesy I make acknowledgment. — Ed.]
The efferent nerves are :
1. Motor nerves, going to voluntary or striped muscles, heart muscle, smooth muscle, including the vaso-constrictor and dilator nerves.
2. The secretory. These act upon glands. Impulses to the blood-vessels (vasomotor) generally accompany the secretory impulses.
3. The trophic.
4. The inhibitory. These nerves control muscular movements, secretion, perhaps also nutrition.
The Central Neurons. — There are intercentral or associative neurons, which connect different parts of the cerebrospinal system together. Some of these connect symmetrical parts on each side together and have a coordinating function. Others connect higher with lower centres and carry ascending and descending impulses.
In the peripheral nervous system we have end-organs. These are delicate and in some cases complex arrangements of the nervous
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY. 21
and other tissue at the periphery of the nerves. Their object is to allow the nerves to be irritated by special stimuli which would not otherwise affect them, e.g., light or sound. Their object is also the proper utilization of efferent impulses upon other tissues. There are end-organs, therefore, for both sensory or afferent and for efferent nerves. The end-organs of the afferent nerves are — eye, ear, taste-buds, corpuscles in the Schneiderian membrane, various tactile cells and bodies the space sense-organ, the muscle-spindles.
For the efferent nerves — neuro-muscular corpuscles in the vol- untary muscles, local ganglia about the arteries, local ganglia in the glands. Trophic end-organs are not known. In many cases the end-organ is nothing but the terminal fibre of the nerve. This loses both medullary sheath and neurilemma, leaving only the axillary cylinder. It then splits up into a terminal plexus, or else without splitting passes between and around the cells which it is to affect.
The nerves, centres, and end-organs thus described may be ar- ranged in mechanisms, each mechanism subserving a special func- tion. These form the mechanisms of the nervous system proper, and those of the other organs of the body.
They may be classified somewhat as follows:
f I. The psychical mechanism.
II. The automatic mechanisms or the mechanism of inherited and Cerebro- J acquired aptitudes,
spinal. j III. The sensory mechanism.
I IV. The voluntary motor mechanism. [ V. The reflex mechanism.
C The secretory mechanism. Visceral J The trophic mechanism, mechanisms. 1 The thermic mechanism.
[ The vasomotor mechanism.
Various of the simpler mechanisms are combined to form those more complex. Thus the automatic and psychical mechanisms embrace in their activity other mechanisms of lower grade.
In the same way mechanisms are combined for the regulation of visceral functions. Thus we have the cardiac, respiratory, and other visceral mechanisms. These mechanisms are liable to be func- tionally or organically disordered and give rise to corresponding neuroses.
The work of the Cell-body of the Neuron. — In the working of these mechanisms the nerve-cell body is the agent which generates or directs; the energy of all nerve-force, by which impulses are started, controlled, and distributed. The larger the nucleus of the cell in
22
DISEASES OF THE NERVOUS SYSTEM.
proportion to its protoplasmic body, the more stable or less sensitive the cell. The larger the amount of protoplasm relative to the nucleus, the more active the discharging power of the cell. The nucleus is the part of the cell-body which is essential to constructive meta- bolism. By means of it the cell builds up its protoplasmic sub- stance. When ihe nucleus dies, the cell may live or function for a time, but it lives only on what has been stored up; it can build no more and soon dies. Nerve-cells with few exceptions (spinal ganglia) have no cencrosomes ; they cannot divide and multiply. Once dead they cannot be restored.
The nerve-fibres conduct impulses generated by nerve-cells. These impulses travel at the rate of about 100 to 120 feet per second. It is less in visceral nerves (25 to 30 feet per second). Nerve-cells are exhausted by successive stimulations, but nerve -fibres can be con- tinually excited and are practically non-fatiguable. There are no electrical currents in normal living nerves (Landois) except when an impulse travels along them. Then an electrical current travels along with the impulse. It is called the current of negative variation. The irritability or excitability of a nerve is the power it has of responding to a stimulus. When a constant. electrical current is passed along a nerve its irritability is modified. This modified condition is called electrotonus. When a nerve-impulse passes up an afferent nerve and is then reflected along an afferent nerve, it is called a reflex action. The time required for this process is called the reaction time. This averages from 0.125 to 0.2 of a second.
The specific gravity of nervous tissue is about 1.036; that of the brain is 1.038; of the spinal cord and nerves, 1.034 (Bischoff, Krause). The reaction is alkaline, but this is lessened by activity, owing to the development chiefly of lactic acid. The gray matter is less alkaline than the white.
The nervous system has the following composition (Baumstark, quoted by Hammarsten) :
CHEMISTRY.
White Matter. Gray Matter.
Water in 1,000 parts Solids
695-35 769-97 304.65 230.03
25.11 10.08
Insoluble albumin and connective tissue
Cholesterin
Nuclein
Neurokeratin
Inorganic salts
50.02 60.79 45.12 23.81 2.94 1.99
18.93 10.43 5-23 5-62
GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY. 23
Water makes up nearly three-fourths of nervous tissue, there being more in the gray than in the white matter and least in the sympathetic nerves. The inorganic salts amount to about .5 per cent. The largest single constituent is phosphorus (Breed) com- bined with potassium, sodium, magnesium, calcium, and iron, forming phosphate salts. Of the other constituents chlorid of potassium is the most important.
Protagon is a very complex substance of a fatty character, con- taining nitrogen and united with glycerin-phosphoric acid instead of glycerin. It is said by some to be made up of two bodies, cerebrin and lecethin, the latter containing an ammonia compound called neu- rin. Protagon is especially found in the white matter. The gray matter and axis-cylinders contain globulins, nucleo-proteid and nuclein, a very important substance in cell metabolism. Nuclein (C29H49N9P3022, Miescher) is composed of nucleic acid, a substance rich in phosphorus and a variable amount of albumin. The gray matter, i.e., the nerve-cells, contains also various albuminous substances. Nucleo-keratin is found in the neuroglia and medullary sheaths. The nuclein and the allied substance nucleo-albumin are called albuminoids (Halliburton). They both contain phosphorus and are found chiefly in the nucleus. The albuminous substances, called also proteids by Halliburton, have little or no phosphorus, and make up the most of the cell-body or cytoplasm.
CHAPTER II.
THE CAUSES OF NERVOUS DISEASES.
Nkrvous diseases are produced in part by predisposing influences which may be likened to a fecund soil ; in part they are due to exciting causes, which are like the seeds dropped upon the soil in the accidents of life.
Heredity is the most serious and important of these predisposing causes, in particular of those neuroses that are constitutional and are not the results of bodily accidents. A nervous disease, however, is rarely directly inherited. Parents do not pass down special mala- dies, but only a general tendency to nerve disease, which is not devel- oped into any distinct trouble unless some disturbing cause arises. Nervous parents may have children who have unstable, over- irritable, and inadequate nervous systems. Such persons have what is called a neuropathic constitution or diathesis. This dia- thesis may be transmitted when the parents, though not especially neurotic, suffer from syphilis, alcoholism, and diseases of malnutri- tion, like tuberculosis. So far as the office of parentage goes, persons of great talent in affairs, or great artistic genius in any direction, may be counted as neurotic and are very likely to have children of neuro- pathic constitution. This is less apt to be the case when one parent is of stable and lymphatic type. If two persons having not simply a nervous constitution but distinct nervous or mental disease marry, their children are liable to serious nervous or mental disease. The intermarriage of blood relations, such as first cousins, does not lead to neurotic children if the parents are not neurotic themselves, and are of robust health and dissimilar temperaments. Injuries or even severe shock to the mother during the early months of pregnancy sometimes leads to nervousness in the offspring. The mother transmits neuroses more often than the father. There are certain rare nervous diseases which appear in different branches and members of a family, such as an uncle, cousin, nephew, and son. These diseases may pass also by direct inheritance from parent to child, or may skip a generation. They are called "family diseases," and are of the nature of congenital defects, like webbed fingers or club-foot.
Morbid traits that have become fixed in a family reappear at about
24
THE CAUSES OF NERVOUS DISEASES.
25
the same age in the descendants. If these traits or tendencies are disappearing from the family, however, they appear later in life with each successive generation in the descendants. If they are becoming more intensified, they develop at an earlier age in the successive descendants. Thus migrainous attacks which have existed in a family usually appear between twelve and fifteen. If now they do not de- velop till the age of thirty it shows that the migrainous taint is dying out.
When a nervous disease develops in a grandchild, having skipped a generation, it is called a manifestation of atavism. Atavism is a very slight factor in nervous diseases, and rarely goes back more than two generations.
Degeneration is the name given to a condition in which there is a morbid deviation from the normal average in mental traits, physio- logical powers, or physical structure. It is a term most often used in connection with mental characteristics. It is almost always an inherited state, and the word degenerate is often used to indicate a person who has a hereditary neuropathic constitution. Degeneracy in a moderate degree often accompanies great mental powers, especially of the artistic kind, and it is almost invariably associated with genius. It is quite compatible with mental soundness and a fair degree of physical health. It is then well to use the term, suggested by Walton, of deviation. Those who have unusual mental gifts and degenerate characteristics are called superior degenerates. The criminal and the insane and erratic and eccentric persons of weak judgment have also the neurotic constitution, and are called inferior degenerates. The weak-minded, imbecile, and idiots form the lowest class of degen- erates, and are called the debiles. The degenerate tends to sterility, and if two degenerates marry, and have children, their children are likely to be more abnormal than the parents. Degenerate families tend to die out. But this tendency can be avoided by the infusion of sound blood.
Age. — In infancy and early childhood, nervous diseases are rather frequent on account of the accidents at birth, the liability to infectious fevers, and malnutrition, and the high degree of sensitiveness of the yet immature nervous system. Still, a carefully watched infant is relatively safe. Motor disorders, such as paralyses, convulsions, and chorea, are much the more common troubles. At the time of puberty sensory disorders, such as headache and migraine appear, and often epilepsy, hysteria, and disorders of sleep. Hereditary tendencies to nervous disease also begin to develop at this time or a little later. At the period of adolescence, the maladies already mentioned also may
20
DISEASES OF THE NERVOUS SYSTEM.
be brought out; but in addition neurasthenic, morbid sexual, hypo- chondriacal, and insane tendencies are seen. From maturity to the time when degenerative changes begin, forty to forty-five, the indi- vidual suffers from those nervous disorders brought on by accidents, injuries, prostrating attacks of sickness, over-strain, infections, in- dulgence in alcohol and narcotics, and the abuse of the bodily func- tions. At and after the climacteric, one sees oftenest such maladies as result from vascular disease, apoplexies, brain softening, also severe forms of neuralgia, and spasm.
Sex. — Sensory and functional disorders are more frequent in women; motor and organic disorders more frequent in men.
Condition and Occupation. — No general facts will be laid down here. Celibates, however, it may be said, suffer more from nervous disorders than married people. It will be shown later that certain occupations entail special nervous disorders and that indoor life promotes functional nervous diseases. The influence of education in the development of nervous diseases is very great, but it can be best considered in connection with special diseases.
Work. — Hard, constant, excessive muscular work, leads to arterial sclerosis and the neuroses that result from a defective circulation.
Mental work produces no direct effect upon metabolism and is a healthful form of activity even intense and long-continued mental work being comparatively harmless. It is the worry and strain and emotional excitement associated with hard work that does harm and leads to neurasthenic disorders.
Mental A ttitude. — The expectant and untrained mind is much more liable to nervous disease as the result of injury, shock, etc. Little evil can befall a prepared mind.
Habits. — Excessive indulgence in alcohol is a most prolific cause of nervous disease, chiefly by the action of this substance on the blood-vessels and the stomach. Excesses in eating, in tea-drinking, irregularity in sleeping, and bad habits of working predispose to nervous disease. Sexual excesses are usually the result rather than the cause of nervous disorders. They are the evidence of mental more than of nervous weakness. Bad mental habits acquired usually in early life as the result of wrongful education or a poor environ- ment and example lead to many functional nervous disorders. By bad mental habits is meant especially the tendency to lay too much stress on trivial things, to worry and "fuss" over details, to have "precisions," to get "notions" and assume as guides in life foolish types of religion, philosophy or sanitation. The obsessive neu-
THE CAUSES OF NERVOUS DISEASES.
-7
rasthenic is one of the characteristic features of our American civilization.
Climate and Civilization. — Nervous diseases are most frequent in temperate climates, and in those which are dry and elevated. They increase with the progress of civilization and the greater strain, complexity, and luxury of modern social life. Those organic nervous diseases which are largely dependent on vascular disease are fre- quent in the poorer classes, among whom syphilis, alcoholism and bad feeding prevail. Functional and degenerative disorders are frequent in the higher classes. Nervous diseases, if we except those of the degenerative type, prevail more in urban populations.
Diathesis. — -The rheumatic and gouty diatheses predispose to ner- vous troubles, more especially those which are of a peripheral and functional nature. Lithsemia, and allied conditions in which the prod- ucts of tissue waste are not properly oxidized and eliminated, have a similar influence.
Trauma and Shock. — Exhausting hemorrhages and trauma may be the direct cause of or may predispose to nervous disease. Trauma and mental shock may cause functional diseases, such as neurasthenia, or may lead to the development of insanity or indirectly to degenera- tive organic disease. Mental shock, and especially a fright, oftener than severe bodily injury, leads to the development of functional neuroses. However, it is only the weak, the neurotic or unprepared who can be thus affected.
Infections. — In comparison with their frequency, the infective fevers are not great factors in producing nervous disease, but prac- tically they often play an important part. Scarlet fever is the most dangerous disorder in this respect. Measles perhaps ranks next; then follow influenza, diphtheria, typhoid fever, and pertussis. Among chronic infections syphilis ranks first; malaria, the pellagra, and beriberi are also to be mentioned.
Poisons. — -Tea, coffee, cocoa, tobacco and lead, mercury, copper, and arsenic, are to be placed among the causes of nervous disease. But alcohol is justly credited with exerting the most sinister influence on the nervous system of all poisons.
Ethyl alcohol is generally classed among the narcotic agents. It has, however, a primary stimulating effect upon the organs of the circulation, respiration and digestion, and also upon the brain. Its effects vary enormously with the dose and with the constitution of the patient. It is a food in the sense that about 90 per cent, is used up in the body and transformed into heat and energy, like sugar. It is not, however, a very good food under ordinary condi-
28
DISEASES OF THE NERVOUS SYSTEM.
tions, but it can be used as such, temperately, by the majority of people, after adolescence, and common observation shows that it can be thus used for many years without appreciable damage to the constitution.
Alcohol is also, correctly, said to be a poison, though this may be said of almost any substance that is taken into the system, if used to excess. Alcohol, however, is a dangerous poison to a minority of people, either because they cannot use it temperately, a small dose leading them at once to excesses, or because it excites and disagrees with the functional activities of the system. Alcohol is always a poison when taken in excess and in some people a small amount may be an excess. The degenerate types of nervous systems are very sensitive to alcohol, and usually made worse by it, so that the use of it by degenerates tends to make them worse and extinguish this group of the race.
On the other hand, alcohol by intemperate use, can lead to an acquired degeneracy in healthy constitutions. History shows that both those races which indulge excessively in alcohol and those which do not use it at all, either degenerate or do not progress. Al- cohol causes a small percentage of epilepsy through inheritance, and a still smaller percentage of epilepsy among its users. It is a large factor in the production of acute mental disturbances, such as delirium tremens, but not a large factor in causing degenerative insanities or imbecility or idiocy. In my own experience, the per- centage of alcoholism in the asscendants of the insane, imbecile and idiotic, is about 5. The ratio of insanity to the population is about the same in prohibition as in other States in this country. In per- sons who inherit degenerate constitutions, that is to say, in persons with a weak and unstable nervous system, alcoholism is often only an expression of this instability, not a cause of it. For it is a sign of degeneracy to drink alcohol to excess or to have a morbid suscepti- bility to its effects.
Alcoholism and habits of alcoholic excess in this country almost always develop before the age of thirty, and if drinking were prohib- ited to those below that age, there would be but little trouble. Wine drinking, if we except champagne drinking, is almost never a cause of alcoholism in this country, and is a much less important factor than beer. This is largely, no doubt, due to the fact that wine is relatively little drunk in America. Beer and ale, if used in excess, are the most injurious forms of alcoholic beverages, except to out-door workers, because they lead to digestive and metabolic disturbances. It would be wise to forbid the use of alcohol to those under thirty,
THE CAUSES OF NERVOUS DISEASES.
20
except in the form of light wines or beer, and those only to workmen and people who live out of doors. Even after this age it should only be given to the certified immunes, that is, to those able to drink moderately.
It follows that, theoretically, at least, the sale of alcoholic liquors should be controlled by the Boards of Health.
It would not be wise to abolish the use of alcohol altogether from civilization until we can be sure it has no good function, or until we can be sure that its abolition would not be followed by the use of more injurious substitutes. It is impossible to deny that civilization has satisfactorily advanced, despite alcohol, and has shown its finest types among those nations who have used it most. Owing to the fact that sanitation and civilization have developed a rather more delicate type of nervous organization, and to the fact that sanitary care has kept more of the unstable and weakly alive, we cannot use alcohol as freely as formerly. Therefore, alcohol has got to be used more and more carefully, and under closer supervision. Persons of a neurotic constitution usually bear alcohol badly, and it is a very good and wise rule to prohibit it in most forms of nervous, and all forms of mental disease.
Arterial sclerosis is a condition brought about by many of the factors that have been here enumerated as causing nervous disease. Its special and direct importance will be shown in the special chapters.
Reflex Causes. — Among other causes are local disease of viscera, such as renal, uterine, and ovarian diseases, dyspeptic and liver .dis- orders, visual and auditory troubles. Reflex irritations are distinc- tively exciting causes, but with few exceptions they cannot cause a nervous disease unless there is a predisposition to it. They may, however, cause many distressing nervous symptoms, such as pain, spasm, and even convulsion.
CHAPTER III.
GENERAL PATHOLOGY.
The nervous system is composed of nerve-cells and nerve-fibres forming neurons, connective tissue, the neuroglia, blood-vessels, and lymphatics. Its disorders involve one or more of the above tissues.
The following is a list of the forms of disease which affect the ner- vous system:
1. Malformations; incomplete development, or agenesis; defective development, or dysgenesis.
2. Hyperemia, anaemia, hemorrhage, oedema, and arterial and venous diseases.
3. Degeneration and atrophy, softening, sclerosis, classed as regres- sive processes.
4. Inflammations.
5. Tuberculosis and syphilis.
6. Tumors and parasites; 3, 4, 5, and 6 being classed as progres- sive processes.
7. Nutritive and functional disorders, including disorders asso- ciated with metabolic and glandular defect, such as acromegaly and exophthalmic goiter.
Inflammation. — The pathology of most of the above types of diseases will be given elsewhere, and does not call for discussion here. It is, however, of the utmost importance that the student have a clear understanding of the nature of inflammation and degeneration as they affect the nervous tissue. Inflammation is a morbid process which has to deal primarily with blood-vessels, lymphatics and con- nective tissue. Inflammation, teleologically, is the reaction of the organism to an irritant. Wherever there is inflammation, there is irritation. The irritant in inflammation is practically usually the product of microbic action or some irritating product of tissue change. Without some microbic or tissue irritant there can be no inflam- mation. We make this exception only: that certain chemical sub- stances, such as alcohol, arsenic and lead, may at times excite a form of inflammation, which is, however, probably, primarily a degenerative or destructive process. Inflammation, when the irritant is removed, tends to subside. Bearing these facts in mind, it will be found that
3°
GENERAL PATHOLOGY.
31
inflammations of the nervous tissues never or very rarely occur with- out the presence of some microbe or some destructive process whose irritant products excite inflammatory reaction.
Inflammations may be divided into the (i) exudative and the (2) productive forms (Delafield). The exudative inflammations may be simple ; without necrosis, with necrosis ; purulent ; purulent and necrotic. (3) Degenerative or parenchymatous.
1. Simple exudative inflammation is accompanied with congestion, stasis, emigration of white corpuscles, and perhaps diapedesis of red cells, transudation of blood-serum, and formation of fibrin, the total result being an exudate containing white blood-cells, now called pus-cells, and fibrin in varying proportions. When there is little exudate or proliferation of cells and the issue cells are chiefly affected, some authors call it a parenchymatous inflammation.
In purulent inflammation there is a great accumulation of pus- cells and less relatively of fibrin.
If the tissue is destroyed, it is a purulent and necrotic inflammation.
In some exudative inflammations there is increase of connective tissue from the start, and the process continues till the inflammation subsides. Most exudative inflammations are acute or subacute. In- flammatory oedema is a form of exudative inflammation.
2. Productive or proliferative inflammation is a process in which there are little congestion and exudation, while new connective tissue is slowly formed. Productive inflammation is usually chronic; tuberculous and syphilitic processes are varieties of productive inflam- mation.
The principal poisons which may cause chronic productive in- flammations are alcohol, lead and arsenic. Certain irritating auto- toxgemic agents, such as occur in gout, rheumatism, diabetes, and states of inanition, appear able at times to cause productive inflam- mations.
Classification of Inflammations.
Form.
Cause.
Example.
Simple exudative with or with- out necrosis.
Purulent, with or without necrosis.
Microbic or toxic
Microbic
Meningitis. Poliomyelitis.
Meningitis and encephalitis.
Productive or proliferative
Microbic
Microbic or toxic
Acute purulent myelitis. Chronic meningitis.
Leprous neuritis.
Degeneration and Sclerosis. — By degeneration is meant in pathology a gradual death of the nerve-cells and fibres or, in other
32
DISEASES OF THE NERVOUS SYSTEM.
words, of the parenchyma of the organ. The cells swell up, become granular and fatty, and then either break up and become absorbed or enter into a condition of a dead coagulum (coagulation necrosis). Degenerations may be acute or chronic, primary or secondary.
Acute degeneration causes a condition known as softening or ne- crosis. It is due to cutting off of vascular supply, direct injury, and to necrotic and inflammatory poisons. Acute degeneration may be followed by a reparative process, which is called a reparative or reactive inflammation, and which ends, perhaps, in producing a cica- trix or sclerosis.
Chronic degeneration is accompanied and followed by a pro- liferative process which results in the production of connective tissue and sclerosis. Degeneration is by some writers classed as a degen- erative or parenchymatous inflammation.
Sclerosis is a process of connective-tissue proliferation, as a result of which the normal or injured parenchyma is supplanted by neuroglia and fibrous tissue. The word sclerosis is usually employed in describ- ing degenerative diseases, though it indicates the result rather than the primary nature of the process. In the nervous system there is often an increase or proliferation of neuroglia tissue in the processes of degeneration. Exactly how large a factor this is cannot yet be said. In one form of sclerosis — multiple sclerosis — the process of neuroglia proliferation seems to be the primary one, nerve-cell destruc- tion following. Hence this type of sclerosis is classed with the pro- liferative inflammatory processes.
A primary degeneration is one in which the process is due to inherent defect in nutrition or to some poison acting directly on the cell or fibre.
A secondary degeneration is one that is due to a cutting off of nerve-fibre or cell from its trophic centre, or to an injury or shutting off of its vascular supply. Ordinarily, in speaking of secondary de- generations, one refers to those due to the first-mentioned class. Practically, primary and secondary degenerations often occur in the same disease.
Degenerations. Forms. Examples.
f Primary / Myelomalacia.
\ Progressive muscular atrophy.
Acute and Chronic \
f Chronic myelitis. [ Secondary and mixed . . . \ Secondary lateral sclerosis.
Degenerations are caused by certain poisons, such as arsenic, phos- phorous, lead and the poisons of infectious disease. Degenerations also result from obliterating arteritis, such as occurs in old age or
GENERAL PATHOLOGY.
33
from humoral poisons. Degenerations sometimes are due apparently to an inherent defect in the cell nutrition — -a premature death of it; also to causes yet unknown. The question as to whether certain scleroses are forms of productive inflammation or of chronic degen- eration is one that has been much debated in the past. It is quite certain now that most of the so-called chronic inflammations of the nervous centres are really degenerative processes, and that the pri- mary trouble is in the parenchyma, and not in the connective tissue.
Gliosis. — When the degenerative process is the result of a pro- liferation of neuroglia, not of connective tissue, it is called gliosis.
Nutritive and Functional Disorders. — Under this head are included defects due to disorders of the blood and blood-glands, to defects in metabolism, to poisons, extrinsic and autochthonous, and to local diseases.
In conclusion some fundamental peculiarities of the nervous tissue may be noted here.
Nerve-cells once destroyed never develop again.
The same is true, though not so absolutely, for the nerve-fibres run- ning in the central nervous system. Peripheral nerves may grow again when cut or destroyed. They grow usually from the trophic centre, but may develop out of the cells of the neurilemma.* Nerve tissue in brain, cord or periphery can never be sutured so that it will functionally unite by direct union. There are a few apparent exceptions.
A further peculiarity of nervous tissue is that it is dependent for its integrity upon two things, blood-supply and trophic influences. The nerve-cell is solely dependent on a proper supply of blood, and dies when this is withdrawn. But the neuraxon is more de- pendent on the trophic influence of the neurilemma and of the cell of which it is a prolongation. It dies when cut off from its cell, but it can get along for a time with but little direct blood-supply. On the other hand, if the neuraxon is injured it reacts on the cell, leading to a partial but curable degeneration of the cell-body.
* This point seems to be again put in doubt by the experiments of Dr. R. G. Harrison, of Baltimore.
3
CHAPTER IV.
GENERAL SYMPTOMS.
When the nervous system is disordered it produces various symp toms, which are classified and receive names according to the parts affected and ihe kind of change present. The general name given to any kind of morbid nervous state is neurosis and the general name for any morbid mental state is psychosis. When the neurosis affects the motor sphere, whether in the brain or cord or nerves, it is a motor neurosis, or, more technically, a kinesioneurosis ; when the sensory par.s are disordered we have a sensory neurosis, or sesthesioneurosis. In the same way we have trophic, thermic, vasomotor and secretory neuroses.
The symptoms of nervous disease are further divided in accord- ance with the kind of disturbances present. Now a function can only be disordered in three ways. It may be exaggerated, lessened, to the point perhaps of entire loss of function, or it may be per- verted. In order to indicate this, certain Greek prefixes are used. They are "hyper," which means excess; "hypo," meaning diminu- tion; "a" or "an," indicating entire loss; and "para," meaning perverted. Thus we have, for example, hyperesthesia, or excessive sensibility; anaesthesia, or loss of sensibility; and paresthesia, which means perverted sensibility.
Finally, nervous symptoms are often spoken of as objective or subjective. The former are those symptoms which can be seen or directly noted by the physician without depending on the patient's statements. The subjective symptoms are those which are felt by the patient, but give no outward sign. Thus headache is a subjec- tive symptom, paralysis is an objective one.
So far we have been grouping together only like kinds of symp- toms; but it happens that many nervous diseases may have quite different kinds, some being motor, some trophic or sensory- Thus nervous diseases practically are to a considerable extent classified simply on the basis of the part of the nervous system diseased ; and we have spinal cord and brain diseases, gastric and sexual neuroses, and so on.
34
GENERAL SYMPTOMS.
35
Nervous symptoms, however, are always grouped together in accordance with the physiological function disturbed. So that we have the following tabulation (see also Fig. 1 8) :
1. Mental and cerebral, forming psychoses.
2. Motor and reflex, forming kinesioneuroses.
3. Sensory, forming aesthesioneuroses.
4. Trophic, forming trophoneuroses.
5. Vasomotor and thermic, forming angioneuroses and thermo- neuroses.
6. Secretory, forming secretory neuroses.
Combinations of these groups of symptoms may affect various organs. They are called mixed neuroses. Combinations of mental and nervous symptoms form psychoneuroses.
Fig. 18. — Diagram illustrating the principle of the classification of nervous symptoms.
The particular symptoms which nervous diseases cause will be de- scribed and recorded under the several heads given above.
1. The mental symptoms include all those found in insanity, idiocy, and imbecility, and will not be given in detail here. The common symptoms met with by the neurologist are mental irritability, depression, emotional excitement, morbid fears, volitional weak- ness and lack of self-control, persistent or fixed ideas, weakness of memory and of power of concentration, and a tendency to hypnotic and somnambulistic states.
Certain symptoms due to disturbance of brain function are often called cerebral, as distinguished from mental. They are: vertigo, disorders of equilibrium, insomnia, somnolence, stupor, coma. Head- ache, head pressure and similar feelings are also often described under the head of cerebral symptoms.
2. Motor Symptoms. — The symptoms of disordered motility are as follows:
36
DISEASES OF THE NERVOUS SYSTEM.
A. Symptoms of exaggerated motility.
Tremor: a, fibrillary and wave-like movements of muscles (myokymia). b, tremor proper. Rhythmical spasm athetosis. Convulsion :
Myoclonia, a general name for muscular twitchings and including the choreic and tic spasms. Hypertonia and contracture. Forced and associated movements. Exaggerated reflex conditienss.
B. Symptoms of lessened motility. Paralysis and paresis, myasthenia. Loss of reflexes, superficial and deep. Hypotonia or lessened muscular tonus.
The particular characteristics of these different symptoms will be best shown in the description of the special diseases, but a brief account will be given here.
Tremor is the result of a disorder in the tonic innervation of muscles. Muscles are kept normally in a state of slight tension by rhythmical impulses passing down at the rate of about twelve per second. When the rhythm and force of these normal impulses are interfered with we have tremor. The simplest form of tremor is one in which the normal tonic impulses have an apparently exag- gerated force. This causes a fine tremor of eight to twelve vibra- tions per second. When there is an interruption to some of the impulses wre have a coarse tremor. Here the vibrations are five to eight per second. It is caused by a partial or complete dropping out of the alternate impulse. Various technical names are used in describing the tremors. We have the fine and coarse, as described.
Intention tremor is one that occurs on voluntary movement, and is opposite in kind to the passive tremor or tremor of rest, which decreases or ceases on voluntary effort. Tremor is sometimes of a coarse, jerky, and inco-ordinate character, and these words are then used to indicate it.
Fibrillary tremor or myokymia is a fine twitching of the indi- vidual strands or parts of muscles, and occurs usually when they are wasting from lack of neurotrophic influence. Closely related to it is the wave-like contractions of the muscle-body without motor effect.
Convulsions consist of abnormal and exaggerated muscular con- tractions occurring in rapid succession. Convulsions may be clonic,
GENERAL SYMPTOMS.
37
i.e., the muscles rapidly and alternately contract and relax in an ex- aggerated and irregular way; or they may be tonic, i.e., contracted steadily and continuously. When a tonic muscular contraction is painful it is called cramp. Convulsions may be co-ordinate. In this case the patient moves the limbs and body in a more or less purposeful way. He throws himself about the bed, jumps, kicks, strikes, tears the clothes, etc. Convulsions are usually accom- panied with loss of consciousness.
Myoclonia is a term used to indicate muscular twitchings of various types, such as those of chorea and the "tics.'
Choreic movements are sudden jerking, twitching movements of different groups of muscles. The movements are purposeless and are not under control of the will. Convulsive tic is a form of spasmodic movement confined to certain groups of muscles which work together for a common purpose, like those of the face, or eyes, or larynx. The move- ments in the "tics" are definite in character and are limited to muscles physiologically grouped for a special function. Thus we have tics of the muscles of expression, or of respiration, or speech, or locomotion.
Athetosis is a name given by Hammond to a peculiar form of movement characterized by slow, successive flexion, extension, prona- tion, and supination of the fingers and hand and arm, or of analogous movement of the toes and feet. The motion rarely ceases in waking hours except for a short time. The
contractions are forcible, steady, and even, and sometimes painful. The hand assumes characteristic positions (Fig. 19).
A contracture is a tonic muscular contraction of long duration, i.e., days or months. A contracture may be functional or organic ; and in order to test this, one must find whether it ceases during sleep or under an anaesthetic; if so, it is functional (see Hysteria). It may be para- lytic; i.e., due to paralysis of opposing muscles, or it may be a con- tinuous spasm.
In forced movements the patient suddenly and involuntarily is thrown forward, sideways or whirled about in various ways.
Associated movements are those which occur involuntarily in a limb or muscle at rest when the corresponding limb or muscle is moved on the opposite side. Thus in hemiplegia the movement
3«
DISEASES OF THE NERVOUS SYSTEM.
of the normal arm may excite a movement in the one paralyzed. The patient is given a piece of chalk in each hand, and each hand is placed upon a blackboard lying on the table; attempts at draw- ing lines with the sound arm cause movements of a similar kind, but less perfect, on the paralyzed side.
Paralysis or akinesis is a loss of motor power. Monoplegia [is a condition in which one limb is paralyzed ; hemiplegia one in which one-half the body is paralyzed ; and paraplegia one in which the two lower limbs are affected. Sometimes a double hemiplegia or diplegia occurs. The term paralysis is sometimes used to indicate
Fig. 20. — Showing the course of an impulse in simple reflex action.
loss of any kind of function, as paralysis of sensation or secretion.
Paresis is a term used to indicate a partial paralysis. It is not to be confounded with the term general paresis, which is a form of insanity.
The Reflexes. — When an impulse started in an afferent nerve reaches the spinal cord or medulla and is thence reflected upon an efferent nerve, the result is called a simple reflex action (Fig. 20). The process is an involuntary one. It ordinarily occupies one-tenth to one-twelfth of a second. The afferent nerve may be an ordi- nary cutaneous sensory nerve, or it may be a special nerve whose
GENERAL SYMPTOMS.
39
function is to exeke reflex action. These latter nerves are called excito-reflex. This kind is principally supplied to the viscera.
In neurology we have to do with three kinds of simple reflexes:
The skin or superficial reflexes.
The tendonous or deep reflexes.
The visceral reflexes.
The idiopathic or direct muscle response is not, strictly speaking, a reflex.
All these may be exaggerated, lessened, or absent. Further de- scription of the reflexes will be given under the head of diagnosis.
The simple reflexes are combined to form compound or higher and more complex reflexes.
3. Sensory Symptoms. — The sensory functions include all those belonging to the nerves of general and special sensation. Sensory nerves have a part in reflex action and in the inhibition of motor and other functions. The nerves of special sense when deranged show various phenomena, which will be described in more detail later. In general there may be depression or loss, increase or perversion of their function. In accordance with this we have:
Anaesthesia, which is a loss of tactile sensibility.
Analgesia, a loss of sensibility to pain.
Thermo-ancEsthesia, a loss of sensibility to temperature. There may be loss of cold-sense or of heat-sense, or, as is usually the case, of both senses.
The term anaesthesia is often used with a general meaning to indi- cate loss of all forms of sensibility. Anaesthesia in this sense is a symptom referred to the skin, bones, mucous membranes, special senses or viscera. The muscles have two kinds of sensibility: a sen- sibility to pain and pressure and a special muscle-sense. Anaesthesia of the pain-pressure-sense of muscle is called loss of muscular sensi- bility or muscular analgesia. Anaesthesia of the special muscle- sense is one of the factors in causing a symptom known as ataxia.
Ataxia is a symptom due to loss of the special sensibility of the mus- cle, articular surfaces and tendons. This special sense is sometimes called the "deep sensibility." It informs the individual of the degree and strength of muscular movements, and by it definite and co-ordi- nated movements are made possible. The weight of objects and position of the limbs are also determined by it. In static ataxia there is loss of the power to preserve perfectly the equilibrium when standing. It is due to the form of anaesthesia just referred to. In locomotor or motor ataxia there is loss of power to co-ordinate the limbs properly in motion. In these conditions there is also usually
40 DISEASES OF THE NERVOUS SYSTEM.
a loss of power to appreciate weights or the position of the limbs. The term muscular anesthesia, however, is often used to indicate these latter symptoms. Cerebellar ataxia is a form of inco-ordination due to disease of the central organ of equilibration, viz., the cerebellum.
A stereo gnosis. — The ability to recognize the form of objects held in the hand or on other parts of the body is called stereognosis, and its loss astereognosis. It is a perceptive process, due to the fusing and elaboration of cutaneous and deep sensations, especially the latter. Astereognosis is present in peripheral or spinal lesions where there is complete tactile anaesthesia, and where, even with good tactile sense, there is loss of deep sensibility. As complete tactile anaes- thesia is rare in cerebral lesions, the cause of astereognosis here, may be due to loss of deep sensibility. As stereognosis is a percep- tive process, based mainly on the elaboration of sensations, it may be lost just as other functions of the brain may be lost. The lesion in such case is localized in the parietal lobe.
The ability to recognize not only the form, but the nature of an object, is called symbolia, and its loss asymbolia. The recognition of the nature of an object is only a higher elaboration of the precep- tive work than that in which simply form is recognized. But the nature and location of the process are the same practically, as for stereognosis.*
Hyperesthesia is an excessive sensibility to touch, contact, and other sensory stimuli.
Hyperalgesia is excessive sensibility to pain, and is nearly identical with tenderness.
Dysesthesia is an abnormal sensation, such as a "thrill" or feel- ing of discomfort produced by ordinary tactile or painful impressions.
Paresthesia is a term applied to all the morbid general sensations except pain. The paraesthesias include such feelings as numbness, prickling, formication, flushing, burning, itching, coldness, tickling, feelings of weariness, exhaustion, various peculiar visceral sensa- tions. Ordinarily, in speaking of paraesthesiae, however, we refer to such feelings as numbness, prickling and creeping.
Delayed sensation is a symptom in which an appreciable time exists, usually one or more seconds, between the time of applying a stimulus and its appreciation in consciousness. Normally a tactile sensation can be felt and responded to in less than one-tenth of a second.
Transferred or referred or reflex sensations are those in which the
*The terms tactile amnesia (Burr) and gnosia and agnosia are used for these or simi- lar perceptive processes.
GENERAL SYMPTOMS.
41
irritation is made at one point and felt at another. Thus an irritation in the stomach causes a pain felt in the forehead. The whole class of so-called reflex pains are really transferred sensations, since in reality there is no reflex action in the process, as will be seen later. Allo- chiria is a peculiar form of transferred sensation, in which an irrita- tion applied on one side of the body is referred to a corresponding point on the opposite side.
4. Trophic Disorders. — These are called trophoneuroses. They consist, so far as relates to neurology, chiefly of hypertrophy and atrophy of nerves, muscle, cutaneous and mucous tissues, joint de- generations, and various skin eruptions. The trophoneuroses, if they affect joints, are called arthropathies; if muscles, atrophies, hyper- trophies, and dystrophies; or if with atrophy there is a great sub- stitution of fat, the condition is known as lipomatosis. When nerves are affected there results degeneration. Trophoneuroses of the skin produce various symptoms, such as herpes, pemphigus and other eruptions, pigmentation, leucoderma, alopecia and bed-sores.
5. Vasomotor and Secretory Symptoms. — The nerves supplying the blood-vessels and secreting glands work together and are usually disordered together. Separate disturbances of the vessels and glands, however, occur. Angioneurosis is the term given to disorders of the vasomotor centre and nerves. Angiospasm is a condition in which there is increase of vasomotor tone and spasmodic contraction of the muscular coats of the arteries. Angio paralysis represents the opposite condition. Such disorders affecting the skin are shown by pallor and coolness or by flushing and heat. Angio-ataxia is a con- dition of variability and irregularity in the tonus of the blood-vessels.
6. The secretory neuroses affect the functions of the skin, mucous membranes and special glands. Hyperidrosis is an excessive sweat- ing. Anidrosis is excessive dryness. Paridrosis is a perversion of secretion in which peculiar odors or colors are noted. Hcsmidrosis is the term applied to bloody sweating.
The secretions of the internal organs are controlled by nervous influences, and their special disturbances often form part of the symptoms of nervous diseases. Thus we have watery diarrhoea in Basedow's disease, and a peculiar membranous discharge from the bowel in asthenic states.
The blood-glands, and particularly the thyroid and pituitary gland, have perversions of function which apparently lead to serious nervous symptoms, which will be described under the head of ex- ophthalmic goitre and acromegaly.
CHAPTER V.
DIAGNOSIS AND METHODS OF EXAMINATION.
The diagnosis of a nervous disease may be simply a clinical one ; that is to say, one may recognize it as belonging to a certain known and definite group of symptoms. Thus, in recognizing the phenomena of epilepsy, one makes a clinical diagnosis. In other cases, and especially in all organic nervous diseases, the physician must make in addition a local, and then a pathological diagnosis. That is, we must determine the seat and nature of the disease.
A diagnosis is made by first getting all the obtainable facts in the patient's past history, then by learning from him all his subjective symptoms, and finally by making an examination according to the technical methods to be here described. In examining a patient, it is imperative that a careful search for diseases outside the nervous system first be undertaken. Then the morbid nervous phenomena should be investigated. The physician should make it an invariable rule to make this examination in a certain fixed and systematic man- ner. The best method is first to get the family and personal history, and then to go over the mental, cerebral and special nervous functions serially in the way indicated under the description of general symp- toms, thus: Examine —
1. Physiognomy, general condition of nutrition, complexion, physi- cal defects (stigmata of degeneration), gait, station, posture, speech.
2. Mental and cerebral symptoms.
3. Motor and muscular symptoms, including muscular and joint atrophies, electrical reactions and the reflexes.
4. Sensory symptoms, general and special.
5. Vasomotor, trophic and secretory.
6. Visceral centres.
In addition to these routine methods we have often to make special tests to determine the existence of foreign bodies, fractures, tumors and disturbances of the blood, urine and cerebrospinal fluids.
In investigating the family history, it is often necessary to make very direct and probing inquiries, for patients are, as a rule, inclined to forget or ignore the existence of nervous and mental disease among relatives. The existence of consumption and inebriety,
42
DIAGNOSIS AND METHODS OF EXAMINATION.
43
epilepsy and syphilis in the direct line are very imporLan: facts; so also are those concerning birth. The patient should be questioned closely as to his previous diseases, especially syphilis; also as to his habits in relation to sexual indulgence, indulgence in alcohol, and smoking. In women, the tea habit should be inquired into. The patient may be allowed to tell his own story first. Proper queries should be put to supplement this, and finally the patient should be asked to state those symptoms which to his mind are main and dominant.
We will now go over the above points in detail.
i. The physiognomy, complexion, and general nutrition are first noted. Many nervous disorders are compatible with a very healthy appearance, and patients often make the introductory apology, "I don't look like a sick person." An anxious look, restless man- ner, and excited or diffident speech, however, often show something wrong. The nervous trouble is usually serious in reverse proportion to the voluble anxiety of the patient to make his condition exactly understood. The character of the gait may reveal at once the nature of the malady. The dropped foot and flaccid swing of the leg in poliomyelitis and neuritis, the stiff shuffling march of paraplegia from myelitis, the waddling movements of juvenile muscular dystrophy, and the bent head and careful stamp of locomotor ataxia are almost of themselves diagnostic:
Et verus incessu patuit morbus.
The speech also often betrays the malady. The physician soon gets to recognize not only the striking symptoms of aphasia, but also the weak piping of paralysis agitans, the stumbling enunciation of paresis, and the peculiar dysarthrias of multiple sclerosis and bulbar palsy. As a rule, the occurrence of speech difficulties in adults is significant of organic and often serious disease.
I regard it of much importance that in the chronic and constitu- tional nervous maladies careful note be made of the marks of degen- eration. The nature of this condition has already been described under the head of hereditary causes of nervous disease. As already stated, degeneration means a marked and morbid deviation from the normal standard of the race. The existence of degeneration im- plies an imperfect or an unbalanced development of the body. The condition is usually shown in some nervous or mental defect in the individual, and degeneracy, as ordinarily understood, implies a neuropathic or psychopathic state. But degeneracy may also mean only a lessened vital resistance to certain forms of infection or injury, as, for example, in persons of a tuberculous tendency, who
44
DISEASES OF THE NERVOUS SYSTEM.
often have marks of degeneracy. However, in the ordinary use of the term it applies to those who have inherited nervous and mental weak- nesses. The degenerate shows certain marks which are called the stigmata of degeneration. They are of three kinds : anatomical, physio- logical and mental. I have space to give only the more important.
Fig. 21. — Photograph of roof of the mouth of a drunkard showing torus palatinus
Anatomical stigmata:
Cranial anomalies, e.g. Asymmetry of cranium. Microcephalus.
Peculiar shape of skull, trigonal, scaphocephalic, plagiocepha- lic.
Facial asymmetry and excessive prognathism. Large jaws.
Deformities of the thorax, such as the pigeon-chest, the funnel- chest, the narrow barrel-shaped chest with rib set too obliquely on the spine.
DIAGNOSIS AND METHODS OF EXAMINATION.
45
Deformities of the palate and uvula, including high narrow
arch and the torus palatinus. Anomalies of the teeth, tongue, and lips.
Anomalies of the eyes: narrow palpebral fissure, muscular insufficiency, excessive astigmatism, nystagmus.
Anomalies of the ears: badly placed, ulgy shapes, asym- metry, adherent or lobeless ears, markedly conchoidal ears.
Anomalies of the limbs, genital organs and body generally.
Anomalies of the skin, excessive hairiness or absence of hair.
Physiological Stigmata. — These include tremor, tics, nystagmus and hereditary defects in the muscular system leading to atrophies. Excessive or defective sensiblity of the cutaneous and special senses, defects in speech, perversions of the sexual and Other instincts are to be classed here. A diminished resistance to nervous and emotional strain is a most frequent physiological mark of degeneracy.
Mental Stigmata. — -These include all those factors that make up the erratic, unbalanced and morbidly emotional individual. The specially morbid note in these persons, as Peterson says, is an excessive egotism, an intense self -consciousness, often with peculiar disturbances of the sense of personality. Mental degeneracy is often associated with great special aptitudes, even genius, and is quite compatible with sanity and a fair degree of health.
Of the foregoing the most important of the anatomical stigmata are deviations in the symmetry and shape of the skull, defects in the palate and under-jaws, badly shaped ears, badly set teeth, badly set ribs and a generally weak and badly developed body. Stress is laid upon the skull because its development corresponds with that of the brain. The palatal stigmata are in general those which make the cavity of the mouth smaller, it being the fact that the mouth cavity increases in size as we ascend the vertebrate series (Peterson). Abnormal palates are found in about 10 per cent, of normal people (Charon) and in from 46 to 80 per cent, of degenerates. The high, narrow palate is one oftenest seen by myself. The torus palatinus or longi- tudinal ridge on the hard palate is significant if it is well marked. The importance of defective ears is based upon comparative observa- tions. They are found in from 20 to 64 per cent, of degenerative per- sons.
While many of the stigmata have no significance in themselves, yet a combination such as impresses the observer with its prepon- derance is of great importance, for neuroses or psychoses developed among this class have a much more unfavorable prognosis. It is especially among neurasthenics, epileptics, severe forms of hysteria and in the insanities that these signs are to be looked for and studied. Among normal men about two or three anatomical stigmata are often found: among lunatics, criminals, abortive types of paranoia and primary forms of neurasthenia, the number is much greater.
The accompanying table will be of help in making the investiga- tions relating to the cranium (page 47).
To understand it, it is necessary to describe the skull landmarks,
46
DISEASES OF THE NERVOUS SYSTEM.
and to give briefly the classification and terms used by anthropologists and alienists in describing the dimensions and shape of the skull.
Dimensions and Shape of Skull — General Classification. — Anthro- pologists make a general classification of skulls into :
The dolichocephalic, in which the antero-posterior diameter or length is to the transverse diameter or breadth as 100 is to 75 or less; the brachycephalic, in which the length is to the breadth as 100 is to 80 or more ; the mesocephalic, in which the length is to the breadth as 100 is 75 to 80. The physiological limits of variation in the ratio of length to breadth range from 100 by 70 to 100 by 90. The dimensions and shape of the skull vary with age, sex, individual, race and with certain pathological conditions and artificial deformities. In general, however, the variations in the shape and size of the skulls of healthy adults of European and American races are fairly uniform.
Fig. 22. — M, /3, T, The triangle for ascertaining the empirical greatest height. (Benedict.)
Variations Dependent upon Age. — The proportions of the skull change most considerably in the first year, and continue to change up to the fourth year. After that, modifications are slight in amount and appear more slowly. By the end of the seventh year the skull has nearly reached its full size (see table) more nearly in girls than in boys. The chief measurements during childhood are given in the table. The protuberances and ridges are less marked in children.
The female skull is larger posteriorly, is broader, lower, with higher orbital diameter; often it has no glabella, no superglabellar depression, and is less well marked as to its ridges, prominences and sutures.
Variations as Regards Race. — The length-breadth index and other cranial indices and the volume are the only racial characteristics so far extensively studied. Even these are too indefinite factors to ht of any practical value. In general, we may say that the dolicho- cephalic or long-headed are: the English, Irish, Scandinavians, ne- groes, 73 ; Arabs, 74 ; Chinese, 76. The brachycephalic or broad-headed are: the Germans, 81; Russians, Turks 81. The mesocephalic or
DIAGNOSIS AND METHODS OF
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DISEASES OF THE NERVOUS SYSTEM.
medium-shaped heads are: the American Indians, 79: Hollanders, Parisians, 79.
The Variations Dependent upon Artificial Deformities, Accidents , Perversions of Growth and Development and upon Disease. — There are certain more or less pathological variations in the shape of the skull, due to a premature ossification of a suture, or arrest of development in a centre of ossification, or to a hyperplasia or aplasia of a part of the skull or of its contents. When one part is shut off from its natural expansion, other parts, as a rule, undergo compensatory development. This principle underlies the pathology of cranial deformities. Those deformities which it would be well to look for are :
The triangular or trigonocephalic skull; the keel-shaped or scapho- cephaly skull; the acrocephalic or pointed skull; the oxycephalic or steeple-shaped skull; the flat-headed skull; the plagiocephalic or obliquely deformed skull.
Variations in the Neuropathic and Psychopathic Classes. — Variation from the regular type is oftener found in these classes than in the nor- mal, but definite variations corresponding with a special type of disease are not yet made out.
For all ordinary purposes the only instruments needed in examin- ing the cranium are a tape, a strip of lead to use as conforamator and a pelvimeter.
2. Investigation of Symptoms of Disordered Motility.— In studying the attitude, expression, gait and speech, some notion of the condition of the motor functions has been obtained. Special disturbances of the various parts must then be investigated. The patient is made to extend the arms and move them in all possible ways; the face, trunk and lower limbs are put through all their changes. The degree of paralysis in some groups of muscles can be measured by dynamometers. The ordinary hand dynamometer measures the degree of paralysis in the flexors. It should be gradu- ated accurately in pounds or kilograms.
The average power of pressure on the dynamometer is, for an adult, forty to fifty kilograms for the right hand, and three to five kilograms less for the left. A woman has about two-thirds of the power of a man. I have had constructed an apparatus by which the strength of the leg push, i.e., of the extensors of the leg and foot, and the extensors of the thigh is tested. Dr. W. Kraus has devised a simpler instrument. The anterior tibial and calf muscles can also be tested by means of an instrument called the pedodyna- mometer devised by the late Dr. William R. Birdsall. A good idea of the degree of paralysis can be got by making the patient take the physician's two hands with his own and squeeze each at the same time. A malingerer or hysteric will often in this way unconsciously press much harder than he is aware. The physician's own ingenuity
DIAGNOSIS AND METHODS OF EXAMINATION.
49
will suggest various ways of testing the strength of the leg and thigh muscles, such as making the patient rise on one toe, climb upon a chair, push against an object with his foot, etc.
Tremor is tested by making the patient hold out the hands and arms at full length, spreading out the fingers at the same time. To determine whether the tremor increases on volitional movement, give the patient a full glass of water, let him hold it out for a moment, then bring it to his mouth slowly. If the tremor increases with this movement it is called "intention." As a general rule, the tremor of organic disease is increased by volitional movement, and ceases during rest of the extremity. Functional tremors are usually con-
paralysis agitans. As I have already said, tremor may be fine or coarse, i.e., four to six or eight to twelve per second. To determine this accurately a special apparatus is needed; but one can with a little experience determine this fairly well by observation alone. Or we can use a sphygmograph, as shown by Dr. F. Peterson. This intrument is fixed firmly on the table, and the tremulous forefinger held lightly against the lever. Coarse tremor is usually a sign of organic disease or of paralysis agitans, but it occurs also in hysteria and grave conditions of alcoholism. Tremor that is hardly observable by the eye can be felt by placing one's hand against the extended fingers of the patient. Tremor of the tongue and lips and facial muscles must be carefully looked for. It is tested by making the patient close the eyes tightly and show the
Fig. 22. — Leg dynamometer.
Fig. 23. — Foot dynamometer.
4
5°
DISEASES OF THE NERVOUS SYSTEM.
teeth or protrude the tongue. Facial tremor if very marked usuallv indicates a serious condition of nervous exhaustion, alcoholic poison, or perhaps oftenest of paresis. Tremor of the whole head due to the neck muscles must be distinguished from secondary shaking of the head due to a tremor of the trunk.
Fibrillary tremor, (Myokimia) which involves only certain fibres of the muscle, is seen oftenest in the tongue and face and muscles of the extremities. It indicates wasting or exhausted muscles or some toxic condition.
Choreic movements, tics, associated and forced movements, and the other forms of motor disturbance can be recognized by simple observation.
Myoidema is a tonic spasm of a part of a muscle usually near, etc. It is produced by a sharp blow upon the muscle. This causes
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Fig. 24. — Diagram of a fine vibratory tremor. Ten of the divisions on the lower
line equal a second.
the muscular fibres to bunch up into a small tumor for several sec- onds. Its presence may indicate rapid muscular wasting from ex- hausting disease.
Idiopathic muscular spasm is a phenomenon of a similar nature. When the belly of a muscle is struck with a dull instrument, a welt of contracted muscle appears and lasts several seconds. It indicates an exaggerated muscular irritability.
The Examination of the Reflexes. — These, as already stated, are of three kinds: (1) the superficial or skin, (2) the deep or tendonous, (3) the visceral.
1. A skin or superficial reflex is produced by scratching, tickling, pinching or irritating the skin with hot, cold or chemical irritants. The result is a contraction of the muscles supplying the parts near the irritation. The skin reflexes which can be ordinarily brought out are the plantar, cremasteric, epigastric, abdominal, erector spinal, interscapular, palmar, scapulo-humeral and cer:ain cephalic reflexes.
The plantar reflex is produced by tickling or scratching the soles of the feet. This causes usually, when carefully done, a slight flexion of the toes. In many cases there is, however, no response. In
DIAGNOSIS AND METHODS OF EXAMINATION.
51
irritable persons and children there is a sudden dorsal flexion of the foot, and often a contraction of the inner hamstring muscles. In pathological conditions involving the pyramidal tracts of the cord or even the motor centres and tracts in the brain there is a dorsal extension of the great toe. This is called the sign of Babinski. A similar phenomenon, i.e., extensor response of the big toe; is pro- duced by firmly compressing the muscles of the calf (Gordon reflex), and by causing the patient to draw his leg up sharply flexing the thigh on the abdomen and the leg on the thigh (Oppenheimer flex). With this dorsal flexion of the toe there is usually a slight plantar flexion of the other toes.
The cremasteric reflex is brought out by scratching the inner side of the thigh or the skin over Scarpa's triangle. It causes a drawing up of the testicle, not of the scrotum alone, on the same side.
The abdominal reflex consists of a contraction of the abdominal recti muscles, caused by irritating the skin over the outer border of the rectus.
The epigastric reflex consists of a contraction of the upper fibres of the rectus, caused by irritating the skin of the same region higher up.
The erector-spinal reflex consists of a contraction of some of the fibres of the erector spinse, caused by irritating the skin along its outer edge.
The scapular reflex consists of a contraction of some of the scapu- lar muscles, caused by irritating the skin over them.
The palmar reflex is produced by irritating the palms of the hands.
The cranial reflexes are the corneal; the reflex contraction of the lids caused by lightly touching the cornea with a camel's-hair brush ; the pupillary-skin reflex, which consists of a dilatation of the pupil caused by scratching the skin of the cheek or chin.
The supra-orbital reflex caused by striking a slight blow over the supra-orbital foramen. The naso-mental reflex, in which a contrac- tion of the levator mentis is caused by a tap on the side of the nose.
The palmar reflex is rarely present in healthy people except dur- ing sleep, and in children. The superficial reflexes depend upon the integrity of the reflex spinal arc, and to a less extent upon the degree of cerebral inhibition. When present, they show that the spinal cord at the level through which the impulses travel is healthy. When absent, they do not necessarily indicate much of anything, for they vary in amount in different persons and at different ages. In cerebral hemiplegia during and for a time af ter the acute attack, they are generally lessened or absent on the affected side. Later they may be exaggerated.
52
DISEASES OF THE NERVOUS SYSTEM.
The levels of the spinal cord through which the impulse travels are indicated in the accompanying table :
Spinal Nerve.
6|
I
2
3
4]
5\
r\
Deep and Super- ficial Reflex.
Elbow jerk
Scapular
Epigasti
Spinal Nerve.
Deep and Super- ficial Reflex.
Abdominal
Cremasteric
Knee jerk
Gluteal
} Ankle jerk and clonus
Plantar
Spinal Cord Levels of the Superficial and Deep Reflexes.
The deep reflexes are sometimes called tendon reflexes, though this is not a strictly correct name, since they can be called out by striking periosteum or muscle as well as tendons. The deep reflex in all these cases is not a true spinal reflex, but is due to the direct effect of the concussion or sudden stretching upon the muscle itself (Gowers), which is in a condition of slight tonus. Those who accept this view speak of the deep reflex as indicating the myotatic irritability or muscular tonus. The deep reflex implies the integrity of a reflex arc.
The deep reflexes are very numerous. The important are :
The patella-tendon reflex or knee-jerk. The ankle reflex or ankle-jerk and clonus. The wrist reflex. The triceps-tendon reflex or elbow-jerk. The jaw reflex or chin-jerk. The light (or pupil- lary) and accommodation (or ciliary) reflexes.
The patella reflex or knee-jerk consists of a sudden contraction of the quadriceps femoris, vastus internus, and subcrureus caused by striking the patella tendon when the leg hangs loosely at right angles
DIAGNOSIS AND METHODS OF EXAMINATION.
53
increased if, at the same time that the blow is struck, a voluntary contraction of some other muscles is made by the patient. Usually the patient is told to pull on his clasped fingers or tightly shut the
54
DISEASES OF THE NERVOUS SYSTEM.
hands. This process is called the re-enforcement of the knee-jerk (see Fig. 25). Such re-enforcement can be caused by irritating the skin and by various sensory or psychic stimuli. The nerve-roots involved are those, in man, of the second and third lumbar seg- ments. The peripheral nerve is the anterior crural. The most essential muscles are the vastus internus (Sherrington) and the quadriceps. The wrist reflex is brought out by striking the wrist tendons while the forearm is supinated and held limply on the
Fig. 26. — Getting the elbow-jerk.
hand of the physician. The triceps reflex or elbow-jerk is brought out by striking the triceps tendon while the arm is supported and the forearm allowed to hang down loosely at right angles to the arm (Fig. 26). These reflexes occur in normal individuals. The jaw reflex or jaw-jerk is brought out by having the patient open the mouth and leave the jaw relaxed. A flat instrument like a paper-cutter is then laid on the teeth of the lower jaw, and if this is struck smartly the elevators of the jaw contract. The light reflex is caused by throwing a bright light into the eye, and the ciliary or
DIAGNOSIS AND METHODS OF EXAMINATION.
55
accommodation reflex by making the patient look at a distant and then at a near object. The pupil normally dilates in the former case and contracts in the latter. When the light reflex is lost while the accommodation reflex remains, the condition is called the Argyll- Robertson pupil.
Ankle clonus is caused by having the seated patient extend the limb and hold it rather firmly in a semiflexed condition. The physician takes the foot by the toe and heel and quickly flexes the foot on the leg. He thus suddenly stretches the calf muscles, and they undergo rhythmical contraction. This phenomenon does not occur in healthy people. It is found in transverse and compression
myelitis and in degeneration of the lateral columns of the cord, and it usually indicates organic disease of the cord. A pseudo-clonus sometimes occurs in which there are a few rhythmical contractions on sudden dorsal flexion of the foot, but the contractions soon sub- side. This is seen in neurasthenia and hysteria.
The deep reflexes, and in particular the knee-jerk, for that is the one most easily and often tested, are practically always present in health. They may be decreased, delayed, absent, or exaggerated. Their exaggeration is common and not of special clinical signifi- cance. The absence of the knee-jerk is of great significance, indi- cating in persons who have no paralysis of the crural muscles, loco- motor ataxia, neuritis or some toxaemia, such as follows diphtheria or exists in diabetes.
Fig. 27. — Getting the ankle -jerk. (Bramwell.)
DISEASES OF THE NERVOUS SYSTEM.
The "paradoxical contraction" is a name given to the tonic con- traction of the anterior tibial muscles caused by the physician's suddenly flexing the foot on the leg, thus shortening these muscles. This is a rare phenomenon, never found in health, and usually associated with excessive spasticity of the legs.
The Electrical Conditions in Disturbances of Motility. — These can- not be understood without some description of the methods of using electricity, and hence the technic of electrical examinations for pur- poses of diagnosis will be described under the head of treatment.
Examination of the Disorders of Sensation. — The object of examining the sensory functions is to Jfjpfe see if they are exaggerated, perverted or
the patient's eyes should be closed and he should be carefully told to answer promptly whenever he feels the stimulus. It is best to insist that he always reply in the same way, e.g., using the word "now" the moment the sensation is felt. Many ingenious instru- ments have been devised, and I have described some of them, but for ordinary purposes a camel 's-hair pencil and a pin answer very well.
Sensations are of two kinds — general or common and special. A common sensation is one which is referred to the body, and it is sub- jective in character. A special sensation is one which is referred to the external world, and in particular to the object which causes the stimulus. The pain from a knife cut is referred to the body, and is a
Fig. 28. — Getting ankle-clonus.
DIAGNOSIS AND METHODS OF EXAMINATION.
57
common sensation. The coldness felt when a knife blade is laid on the skin is referred to the knife, and is a special sensation. Special senses give us very often objective symptoms; i.e., such as can be noted directly by the physician.
The sensory functions to be examined are :
The cutaneous sensations of touch, localizing touch, pain and temperature. These sensations belong in a degree also to mucous and serous membranes and to muscle.
The muscular, articular and tendonous sensations, which tell us of the movement and position of the limbs.
The periosteum and bones have a certain sensibility to vibration.
The visual, auditory, olfactory, gustatory and space senses.
Fig. 29. — Testing the pupil reflex, the patient sitting before a window and looking at a
distant object. (Bramwell.)
Visceral and general bodily sensations.
The sense of ihe form and nature of an object.
-The cutaneous sensations are: (1) The tactile sense, which includes pressure and contact; (2) the temperature sense, which includes the heat sense and cold sense; (3) the pain sense. The first two are special senses, the last is a general sense.*
To test the tactile sense, blindfold the patient and use the aesthesiometer. This is an instrument with two rather blunt points, which can be separated or approximated. A hairpin or two ordinary pins can be used in its stead. Its use depends upon the fact that the power to appreciate the contact of two points on the skin gradually approximated varies with the tactile sensibility of the
* Psychologists deny the independence of the pain sense, and assert that it is only a quality or modification of other senses.
58
DISEASES OF THE NERVOUS SYSTEM.
patient. The tongue, finger tips and lips are the most sensitive points. The back, arms and thighs the least sensitive.
The following table shows the average distance at which two points are appreciated as such by an intelligent adult :
Tip of Tongue i mm. (V- in.). Tip of toes, cheeks, eyelids. . 12 mm.
Tip of fingers 2 " Temple 13 "
Lips 3 " Back of hands 30 "
Dorsal surface of Neck 35 "
fingers 6 " Forearm, leg, back of foot. . . 40 "
Tip of nose 8 " Back 60-80 "
Forearm 9 " Arm and thigh 80 "
The figures vary somewhat with the thickness or softness of the skin and with the dullness or keenness of the nervous organization. If the distances are double those given above, it may be considered in most cases abnormal.*
The sense of contact, which is a form of tactile sense, istested by touching the skin very lightly with a hair or hair brush or bit of cotton. The sense of locality or power to localize a point on the skin that has been touched varies with the tactile sense and with the muscular sense. It is tested by placing the finger lightly on a given spot and telling the patient with closed eyes to place his finger on the part touched. He should come within five centimeters of it. In slight degrees of anaesthesia dependent upon disease of the sensori- motor areas of the cortex of the brain this is an important test. Further tests may be made by moving points along the skin and aking the patient to indicate the direction of the motion, or by laying variously shaped objects on the skin and asking the patient to tell their shape and position.
To test the pressure sense, one may use the bargesthesiometer, an instrument made with a spring scale measuring the amount of press- ure made, A simpler way is to have the patient rest the hands on a table and then try and determine the weight of different objects. The ligh.es: weight that can be appreciated on the hands or face is
* The tactile sense may also be tested by the writing-method (Rumpf). Figures or letters are written upon the skin with a hard-pointed instrument, and the patient is asked to tell them. The figures drawn are made larger or smaller in accordance with the de- crease or increase of sensibility. The following table shows the different sizes as appre- ciated on the normal skin :
Finger tips 0.5 cm. (i in.) high
Palm 1
Neck 1
Cheeks 1
Forehead 1
Arm, forearm, and back of hand 1 . 5 to 2 . 5
Scapula 1.5 to 2 . 5
Calf and sole 3
(f in.)
(f to I in) (i t in.)
DIAGNOSIS AND METHODS OF EXAMINATION.
59
one of about 0.02 gram (gr. J). Differences of light weights of 1 and 5 grams and of 25 and 30 grams are about all that can be ordinarily appreciated by the skin. Much smaller differences, of 0.5 to 2 grams, can be detected if great care is used. Weighted rubber balls may be used in the foregoing test. I prefer to use differently weighted metal bodies held by a wire. Pressure sense is acute on the forearm and abdomen, where locality sense is feeble; also on the brow, tem- ples and back of the hand. By the use of these tests held freely in the hand we test also the musculo-articular or deep sensibility.
Most of the above tests are not ordinarily needed. With two pins, using the heads, the presence and degree of anaesthesia can be detected and approximately measured.
The temperature sense is tested by test-tubes filled with hot and cold water, or by using hot and cold spoons, or roughly by breath- ing and then blowing on the part. A thermo-aesthesiometer may be used. This has a round, flat surface one centimeter in diameter, and contains in its terminals thermometers by which the degree and differences in temperature may be noted (Fig. 30). A small heated or chilled surface is appreciated much less easily than a large one.
The indifferent range where objects are felt to be neither warm nor cold is from 270 to 300 C. (80.60 to 86° F.). Fine differences (0.20 to i. 50 C.) are appreciated above the indifferent range. Lower down in the scale, differences from i° to 1.30 C. (20 to 30 F.) are appreciable. It may be considered a morbid symptom if tempera- tures of 6o° to 650 F. are not felt as cold, or temperatures of 86° to 950 F. are not felt as warm; also if between the ranges of i° C. (320 F.) and 400 C. (1040 F.) differences of 20 C. are not appreciated. A painful degree of sensitiveness to heat or cold sometimes exists. These conditions are called hyperthermalgesia and hypercyalgesia (Skinner, Starr). When the heat or cold is in ense, a sensation of pain is felt. Cold pain is produced more easily in some places, such as the elbow, than others, as, for example, the finger tips. Cold pain is produced by temperatures of from + 2. 8° C. to — 11.40 C. Heat pain is produced by temperatures of from 36. 30 C. to 52. 6° C.
The pain sense is tested by pricking the skin with needles or the sharp points of an aesthesiometer. The faradic battery with metal points or a wire brush may also be used. Instruments for pinching the skin and measuring the sensibility by the strength of the pinch have been devised. The power of localizing pain is lessened in propor- tion to the analgesia.
The muscular sensibility, i.e., the general or pain sensation of mus- cles, is tested by passing the faradic current through the part. Some
Fig. 30. — Showing the segmental or root distribution of cutaneous sensory nerves on the right side, and the peripheral distribution on the left side. (Drawn by H. T. Shannon.)
Fig. 31. — Showing the segmental or root distribution of cutaneous sensory nerves on the left side, and the peripheral distribution on the right side. (Drawn by H. T. Shannon.)
62
DISEASES OF THE NERVOUS SYSTEM.
writers speak of a "deep muscular sensibility" and test it by pressing directly upon the muscular mass.
Pain and temperature sense are usually affected together.
Delayed Sensation. — The time taken for a sensation to be felt and produce a voluntary response is, for —
A touch on the hand, about 0.12 second
The tactile sense, as well as the other special and the general sen- sations, may show a delay in conduction. The tactile sense especially should be tested on this point. The delay may amount to several seconds.
The stereognostic sense is one dependent on the use of the tactile and muscular senses. It is really a part of perception or judgment. It is tested by placing variously shaped objects in the patient's hands
and asking him to name them. It depends upon the integrity of the tactile or of the deep, i.e., musculo-articular sensibility or upon that of the stereognostic centre in the parietal lobe.
Double Sensations or Polycesthesia. — When the touch of one point is felt as two or more, the symptom is known as polysesthesia. Re- ferred sensations and allochiria are described under Symptomatology. The distribution of the ansethesia must be determined. The normal nerve-supply of the skin is shown in Figs. 31 and 32.
Tests for the Condition of the Special Sense of Muscles, Joints and Tendons, i.e., for Ataxia or Deep Muscular Sensibility. — Anaesthesia of the special sensory nerves of the muscles, joints and tendons causes a axia and inco-ordinadon. Muscle anaesthesia causes chiefly a loss of weight sense or loss of power to determine weights. It is tested by the use of weights suspended by a string so as to exclude pressure sense ; also by causing the patient to squeeze a dynamometer up to a certain fixed number.
In articular and tendonous anaesthesia there is loss of posture sense.
foot,
0.17 0.13 0.16 0.15
Hearing Sight . . . Taste . .
DIAGNOSIS AND METHODS OF EXAMINATION.
63
It is tested by the physician's moving the patient's limbs and shaving the blindfolded patient tell in what direction the movement is made. Or he is told to follow with one limb the movements which the ex- aminer makes with the other.
Muscular, articular and tendonous anaesthesia usually exist to- gether; there is then ordinary ataxia. Such ataxia shows itself in standing and in locomotion and other voluntary movements. Thus we have a static ataxia and locomotor or motor ataxia. Static ataxia, or inability to stand (or sit) without swaying or irregular movements, is tested by making the patient stand with the eyes closed and the heels and toes close together. Normally, the head moves not over an inch in this position, and the patient holds the head and body more rigid with the eyes closed than with them opened. In ataxic states the reverse is true, and decided swaying or even complete loss of equilibrium occurs with the eyes closed, or even with the eyes open, and the base narrowed by putting the feet together. This phenomenon is called the " Brauch-Romberg symptom." In static ataxia, muscular and articular sensations are both involved. The degree of this can be accurately measured by the ataxiagraph (Figs. 33, 34 and 35).
Fig. 33. — Unat axia- graph.
Fig. 34. — Ataxiagram with eyes open.
Fig. 35.— With eyes closed
With the eyes open a healthy person standing erect with feet together tends to sway forward. The antero-post rior excursion of the head averages 3.7 cm. (1+ in.), the maximum being 9 cm. (3+ in.). The lateral excursion averages 1.9 cm. (| in.), maximum being 5.4 cm. With the eyes closed the antero-posterior excursion averages 3.4 cm. (ij in.), maximum 3^ in.; the lateral excursion 1.9 cm. (if in.), maximum 6.8 cm. (2§ in.). In other words, the person normally stands a little steadier with the eyes closed, the average excursion
64
DISEASES OF THE NERVOUS SYSTEM.
being X } in. with eyes open, X f in. with eyes closed (Bull- ard and Brackett).*
Ataxia of motion is tested by the gait. The patient cannot walk a straight line and cannot walk without watching the floor with the eyes. The rms cannot be moved in a co-ordinate way. With the eyes closed, the patient cannot place the finger on the tip of the nose or lobe of the ear or any indicated spot. Ataxia of motion involves especially the articular and tendonous sensations, but not these exclusively. It may be measured by noting how close in walking the patient keeps upon a given line ten feet long; how near he can place the finger upon a centre of a board marked like a target. The patient is placed ten feet away and made to walk directly at it and place the finger in the centre.
To sum up the foregoing, we have :
|
Due to |
Tested by |
|
|
Ataxia |
Muscular anaesthesia |
Weights, etc. Position of limbs. Co-ordinate movements. Station and gate |
|
Articular and tendonous anaesthesia Combined forms, e. g., static ataxia Locomotor ataxia |
||
Hypotonia or loss of muscular tonus is tested by having the patient lie on a couch. The physician takes the foot and lifts the leg by it, the knee being kept stiff. Ordinarily the leg cannot be carried quite to a right angle without the knee bending. If it does go to a right angle or beyond, there is hypotonia of the limb, the fingers, wrists, toes, ankles, knees show also abnormal flexibility. This condition is natural to some extent in children and it is sometimes a physio- logical characteristic of the adult.
The vibration sense is tested by placing a tuning-fork upon bony prominences. The feeling of vibration thus communicated varies about as does the tactile sense, and the test has no great importance.
Vision. — The special modes of examination are given under the head of Diseases of the Optic Nerve and Ocular Muscles. The special points which the neurologist must investigate are visual acuity, astigmatism, errors of refraction, limitation of the visual field, exophthalmos, retraction of the bulb, color blindness, the state of the pupil and its reflexes.
Hearing. — -The special methods of examination are given else- where. The points chiefly to be investigated are acuity, range, bone conduction, aerial conduction, electrical reactions.
*This is not always the case.
DIAGNOSIS AND METHODS OF EXAMINATION.
65
Sense of Smell and Sense of Taste. — See Cranial Nerves.
The X-ray tests are useful in determining the presence of frac- tures, bony growths, atrophies and deformities. Occasionally the X-ray photograph helps in the recognition of brain tumors.
Blood examinations are necessary in acute inflammatory conditions pernicious anaemia, etc. It is hardly necessary to speak of the need of tests of the urine or of those which inform us regarding conditions in the gastro-intestinal tract. In many forms of nervous diseases every organ and fluid of the body must be examined so that the laboratory is an especially important aid to neurological diagnosis.
The Calmetfe test for tuberculosis has not yet an assured position, but seems to be the most valuable one we possess at the present time.
Examination of the Cerebrospinal Feuid is one of the help- ful methods of diagnosis, and its technic is as follows : The cerebro- spinal fluid is normally a clear liquid and shows practically noth- ing under the microscope. In certain nervous diseases, such as tabes, spinal syphilis, paresis and in inflammatory diseases various lymph- oid cells are found. The special characteristics are referred to under the different diseases. The method of obtaining the fluid is simple and harmless. The skin of the patient's back is sterilized and the second and third lumbar spines are found by counting down from the twelfth dorsal vertebra to which the twelfth rib is attached.
The patient lies in bed upon one side. The instruments usually used are what are known as the "Quincke set," consisting of trocar, cannula and manometer. I have, however, been accustomed to use an ordinary aspirating needle, and it answers perfectly well. It also possesses the advantage that if no fluid comes away, a hypo- dermic can be attached and gentle suction made. The part should be cocainized by a deep injection of 30 minims of a \ per cent, so- lution. After waiting at least five minutes, the needle or trochar is introduced one inch to one side of the median line. It should be quickly thrust through the skin down to the lamina with one motion; then having felt the end of the needle for a space between the lamina, it should be thrust quickly between the laminae of the spine with a second movement. In this way, and with cocaine, hardly any pain is produced. The fluid, under ordinary conditions, comes away in drops, about one or two a second ; sometimes, however, it flows very slowly, only six or seven drops in a minute. In conditions of pressure it spurts out. It is necessary to obtain from 4 to 6 c.c. I believe that 4 c.c. is enough and the patient will be less likely to have headache later. There should never be taken out more than half an ounce. 5
66
DISEASES OF THE NERVOUS SYSTEM.
The fluid should be dropped into the centrifuge tube, which should be previously sterilized. It should not be caught in a test-tube and then poured into the centrifuge. It must be centrifuged for ten minutes within one or two hours after the fluid is withdrawn. It cannot be kept till the next day. After this process, if there is any lymphocytosis, there is a slight cloud at the bottom of the tube. A drop of this cloudy precipitate is collected in a capillary pipette and placed on a glass for purposes of study.
The prccedure should never be performed in tumor of the posterior fossa or cerebellum.
CHAPTER VI.
HYGIENE, PROPHYLAXIS, TREATMENT.
In the treatment of nervous disease, the physician attempts to relieve distressing symptoms, to secure radical cure, and to prevent return. This calls for various measures which may be classed under the heads of general hygiene, diet, exercise, climate hydrotherapy, mas- sage in various forms, electricity, drugs, external applications and surgical intervention.
General Hygiene. — To secure and keep steady nerves and to prevent the supervention of organic nervous disease, would require a considerable reconstruction of the present social system. I can only give some hints as to the kind of advice physicians should give to help along the desired end, this being meant more especially for the neuropath. Thus two people of very nervous temperament should not marry. Blood relations of the same temperament should not marry, and families with a psychopathic taint should not intermarry. Children should be brought up to eat slowly a mixed diet, to sleep early and long, to play in the open air, to learn self-control and obedience. Their parents should keep from them all infective fevers. System- atic study and work are good for all children. It is the strain due to defective vision, poor light and ventilation and unsuitable tasks that hurts the neurotic. Education and occupation are the best kind of builders up of healthy nerves. There are children, however, who cannot follow the ordinary educational lines and who must be specially trained in consequence. The queer and eccentric children with some twist, or precocious talent, need especial care. They usually must be brought up to follow lives on a low mental plane. Too many good farmers and artisans are spoiled by being made poor professional men, or being set up in responsible business positions. Adults need to keep in mind especially : moderation, exercise and the avoidance of a luetic infection. With these they need not fear the use of alcohol, tobacco, tea, coffee or even occasional irregularities in sleeping and eating. Physical and mental shocks, infective fevers and poisons are prolific promoters of nervous disease. Syphilis stands out as the most im- portant single factor in producing organic nervous diseases. If it
67
68
DISEASES OF THE NERVOUS SYSTEM.
could be removed we would have little if any locomotor ataxia, paresis, or myelitis, and far fewer cases of apoplexy.
Alcohol is a less important factor, but does much to produce mental disease, vascular disease, and hereditary degeneration.
Diet. — -There is no definite formula for diet in nervous diseases. The kinds of foods which are suitable for infants and children, and growing youths, are not equally suitable for the mature or the senile. There are some general facts, however, which may be laid down, re- garding the diet for the nervous; that is to say, for persons who have a more or less neurotic and unstable constitution. This class of per- sons seem to be, as a rule, unable to tolerate sweets or large amounts of flesh-food, and they do best upon a mixed diet which contains a moderate amount of proteid together with vegetables and fruit. The question, whether neurotic patients do better upon the so-called "high" proteid of flesh or the "low" proteid of vegetables, does not seem yet to be settled, though the practice is, on the whole, rather more in favor of using the flesh proteid than the vegetable proteid, especially in the early half of life.
There are four kinds of what may be called general diets pre- scribed for patients. I do not mean to include in these four, how- ever, the special diets that are used in the acute and organic diseases, such as diabetes and fevers, serious gastric disturbances, and so on.
These four diets are : first, vegetarianism ; second, the modified veg- etarian or purin-free diet; third, the Salisbury high-proteid diet, and, fourth, the Chittenden or "low diet."
The pure vegetarian diet is one which has no scientific basis, and which seems to me, on the whole, as unwise, though it apparently suits certain constitutions, and is, if anywhere, especially suitable for those of rather advanced years.
The purin-free diet is vegetarianism plus the use of milk and cheese. In this, there is a reduction of the proteids, but an increase some- what, of the fats and carbohydrates.
The Salisbury diet consists of lean beef and hot water, and its use involves a large increase of the proteids and of the purin-bodies, while there is a decrease of the fats and carbohydrates. This diet is usually somewhat modified by the addition of bread or toast, and sometimes of fruits, so that the diminution in the carbohydrates is not so marked.
The Chittenden diet is one in which the food, as a whole, is cut down in all its forms, the proteid being reduced nearly one-half. Experience has shown, that all these different diets may lead to the same therapeutic results — that is to say, that a purin-free diet will
HYGIENE, PROPHYLAXIS, TREATMENT.
69
cure a migraine or lessen the attacks, and that a Salisbury or meat diet will do the same.
The injury caused by any form of diet is largely due to the fact that there gets into the blood an excess of food or fuel, and if this diet is modified so that such excess is lessened, without weakening the system, we can secure good results. In fine, the main factor in pre- scribing any diet, is to see that it is a digestive and assimilable one, and that there does not get into th system an excess of fuel, pro- ducing what is termed a " hyperpyrsemia." This is really the key to the situation in prescribing a diet — that it should be simple, digestible, thoroughly masticated — and that the food be not taken in excess. Those who practice excessive mastication or " Fletcher- ization" of the food, will sometimes find that this process itself induces indigestion.
The average diet is represented as follows (Voit) :
Proteids. Carbohydrates. Fats. Fuel Value.
Grams. Grams. Grams. Calories.
118 500 56 3,°oo
In hard muscular work these figures all rise so as to furnish full value of 4000 calories or over, or drop in rest to 1500 calories. In a fruitarian diet and in vegetarian diet the proteids drop to 40 or 50 grams or less, and the fuel value to about 2000 calories. Atwater has shown that the proteid can be reduced even in athletes to 60 grams and that food of a fuel value below the ordinary figure of 3000 will support a person in health. There is no evidence that very low proteid diets, including the reduction, fruitarian and vege- tarian diets are especially useful to the nervous constitution, but the tendency should be toward these ; and more especially toward a diet of not excessive fuel value. The proteids do not supply energy to the body and, therefore, we would expect in persons with lack of nervous energy more direct help from the fats and carbo- hydrates. Yet sugar dyspepsia is the commonest phenomenon of the neurotic. A low diet in proteid and especially in carbohydrates is thus most generally indicated.
When a rigid diet is to be laid down, there is no better list for ner- vous invalids than the following: fowl; beef; mutton and lamb; fish, boiled or broiled; oysters; milk; butter; eggs, raw or soft-boiled; cocoa; graham bread and gluten bread; spinach; Brussels sprouts; string beans ; stewed fruits.
In elderly persons the amount of nitrogenous food needed in the form of meat is usually less ; and they often do well on a vegetarian diet.
70
DISEASES OF THE NERVOUS SYSTEM.
As a rule, however, vegetarians after a few years find that a return to some carnivorous food is indicated.
Some neurotic persons seem to need a great deal of food but, as a rule, harm comes from full diets and one cannot get strong by stuffing.
The frankly nervous and especially the hysterical patients should not use alcohol at all. Tea and coffee can be taken in very small amount and best without sugar. The various alkaline mineral waters may be used temperately with impunity, but none of them have much specific effect in relieving nervousness or curing the nervous temperament.
Water should be drunk between or before meals and a moderate amount at meals. At least three pints of liquid should be taken daily. American neurotics do not drink water enough. They have half-desiccated nerves, and desiccation increases nervous irritability. An exclusive milk diet is indicated in some forms of hysteria, hypochondriasis, and neurasthenia accompanied with dyspepsia. Karell's method is to give four to eight ounces of warm skim milk at 8 a.m., 12 m., 4 p.m., and 8 p.m. The amount is gradually in- creased. Such diets are, however, only to be kept up for a limited time.
Exercise. — As a prophylactic against nervous disorders, the value of exercise, if taken out of doors, can hardly be overestimated. Brain workers are better for moderate exercise, but they do not need much; and after twenty-five, severe intellectual work can rarely be done by persons in athletic training. Before the age of twenty-five, when the system is exuberant with vitality, hard study and hard physi- cal exercise can be pursued successfully together by some. Persons of a neuropathic constitution are most benefited by regular exercise when it interests the mind. In-door gymnasium exercise with the ordinary apparatus do little good except through the bath that follows it. In many forms of chronic organic nervous disease, exer- cise is to be prohibited. These will be discussed later.
The best forms of exercise are those which take one out of doors, interest the mind, and call into play the muscles of the chest and arm. Walking fulfills but two and often only one of these condi- tions. Calisthenics are useful when they interest and are vigorously done. Horseback riding, golfing, and bicycling fulfill best the con- ditions required for a good form of exercise. Lawn tennis, bad- minton, golf, are all exercises which can be taken up by both sexes and at nearly all ages. Active exercise is not so necessary and should be taken in moderation after forty or forty-five.
The most important line of effort in prophylaxis is the warding off
HYGIENE, PROPHYLAXIS, TREATMENT.
7*
of excessive or premature arteriosclerosis. The surest preventive of this is a good heredity. But given an average constitution, one can delay arterial degeneration only by leading a perfect life, that is to say, th n individual must avoid all mental and physical strain and shock, alcohol, excesses in eating and tobacco. He must not get syphilis. He must keep the emunctories open and get as much as possible of fresh air. All of which is the council of per- fection, except for children. Here wise parents can do much to prevent the development of neuroses. The neurotic child should live and play out-doors until he is nine. He must live and play, eat and sleep in a regular way; he should learn to have regular habits and to acquire self-control and thoughtfulness of others. A little disciplinary study cannot be begun too early. After puberty most children do best working en masse in schools rather than with tutors.* Hydrotherapy. — Hydrotherapy is the science of applying water in the treatment of disease. The modes by which it is used in neurological therapeutics are:
I. General hydrotherapy:
1. Tonic hydrotherapy.
2. Sedative hydrotherapy.
3. Indifferent baths for mechanical purposes.
II. Local hydrotherapy.
1. Tonic Hydrotherapy. — For purposes of stimulating nutrition and increasing vasomotor tone we employ cold plunges, the rain-bath or shower, the jet, cold sponging, cold sitzbaths, cold sheets, local applications of ice or cold compresses or cold rubbing, ice bags, brine baths, brief cold packs and sea-bathing.
The cold plunge. The patient fills the bath-tub with water at from
* Arterial sclerosis is an important factor in producing morbid nervous conditions; though this importance has been overemphasized of late. It is a condition that is usually rather general throughout the vascular system, but it may be especially developed in the cerebral arteries, in the heart, the viscera and the extremities. When a general condition, it is not very important unless associated with some albuminuria or other evidence of cirrhotic kidneys. In its severer generalized forms it may cause insomina, vertigo, mental apathy and fatigability, sometimes headache and cerebral or peripheral paresthesia, such as sensations of constriction or pressure in the head, sensations of coldness or heat of the body, cold areas, hot areas, etc., and pains in the extremities or trunk. Marked per- ipheral sclerosis may cause attacks of cramp-like pains in the extremities or back or neck (anginal pains). Severe local visceral arterial sclerosis will cause colicky or anginal pains in the abdominal organs and heart. Severe grades of cerebral arterial sclerosis lead to symptoms resembling tumor of the brain. Arterial sclerosis is also associated with the apoplexies and terminal senile symptoms. Cerebral arterial sclerosis causes vertigo, headache, twitching, syncopal attacks, convulsions, weakness of memory, tem- porary aphasia, excitement, confusion, disorientation. In the young arterial sclerosis is sometimes seen as a result of hereditary influence, excessive work, acute in fections and syphilis.
DISEASES OF THE NERVOUS SYSTEM.
6o° to 700 F. He then gets in, and at once jumps out and rubs him- self vigorously until reaction occurs.
The rain--bath and Charcot douche. The patient stands in a tub with the feet preferably in warm water, and allows the cold water to fall on the back and rest of the body for one or two minutes. Or a solid jet of cool water is thrown with force upon the back of the patient by an attendant who stands ten or twelve feet away (Char- cot douche). The cold jet may be alternated with a warm one (Scottish douche).
The cold sheet or drip sheet is used by wringing a cotton sheet out in cold water and wrapping it suddenly about the patient who sits or stands with the feet in a tub of warm water. The patient is then dried, put to bed, and vigorously rubbed.
Ice-bags are worn upon the spine for one or two hours once or twice or oftener, daily; or they may be applied for one or two hours at night.
Most of the above measures have a general stimulating and tonic effect.
The simplest form of tonic bath is a moderately cool or even warm tub-bath at 700 to 900 F. in which the patient lies for a few minutes. This is followed by a cool spray and rub down.
Cold baths in their various forms, but especially the jet-baths like the Charcot douche, are best preceded by a short stay in a hot-box until the patient begins to perspire.
The half-bath and wash-off consists of a tub partly filled with water at a temperature of 650 to 700 F. The water only half covers the reclining body. While lying in it, the patient is vigorously rubbed.
A cold cloth may be laid on the head. After five to twenty minutes, affusions of colder water are poured over the shoulders. The bath may be made as warm as 8o° at first.
Brine-baths contain about 2 per cent, of salt, this being about the amount in sea-water — twenty-five pounds to thirty gallons of water. They are given at a temperature of ioo° F. for twenty to thirty minutes daily, or cool baths at a temperature of 700 F. may be given for five to ten minutes, the patients exercising meanwhile. The Nauheim or carbonic acid bath is of especial use in neuroses asso- ciated with cardiovascular weakness.
Physiology. — Cold applications produce a local contraction of blood- vessels, followed by dilatation. There are usually increased tissue metamorphosis, increased secretion of urine, increased absorption of oxygen, and increased excretion of carbonic acid. In non-febrile per- sons, cold applications abstract some heat, but they also stimulate the heat-producing centres so that the total effect is to increase the
HYGIENE, PROPHYLAXIS, TREATMENT.
73
heat of the body. Only very cold or prolonged baths lessen heat- production as well as excretion of C02. Cold baths at first accelerate and then tend to retard pulse and respiration. Cutaneous sensibility is at first increased. After a cold bath there is a sense of exhilaration and increased muscular power, provided the bath be not too cold or too long continued. The duration necessary to produce a reaction varies with different people, and some weak and sensitive patients never can be made to react. Cold baths systematically taken furnish a kind of vasomotor gymnastics. The neuro-mechanism controlling the blood-vessels becomes more supple, and the tendency to local congestion of the viscera and mucous membranes is prevented. The shower and jet furnishes the most valuable means of securing tonic effects in nervous disorders. These are not used with cold water alone. It is often better to apply at first a warm stream at 950 or 1050 F. and then gradually lower it, or to apply the hot and cold jets alternately. In this way tonic effects can be obtained even with very feeble persons.
Tonic water treatment has to be modified greatly according to the age and strength of the patient.
2. Sedative Hydrotherapy. — The sedative baths are the lukewarm bath, the wet pack, Turkish and Russian' baths, the hot sitzbaths, pedal-baths, compresses and fomentations, and hot-water bags.
The luke-warm bath is given at a temperament of 950 to 980 F. for ten minutes to half an hour daily. If a slight tonic effect is desired also, the patient should receive an effusion afterward, i.e., basins of cool water at 6o° to 700 F. should be poured over the shoulders. The addition of salt or of pine-needle extract is often useful.
The wet pack. A large, thick blanket is spread upon the bed, and upon this is laid a linen or cotton sheet wrung out in cold water, 400 to 6o° F. The nude patient lies on this, and the sheet is then smoothly wrapped about him, the head and feet not being included. The sheet is carried between the legs and made to lie evenly in contact with the body. Then the blankets are folded over him, and other blankets may be applied upon this. Sometimes it is well to place hot-water bottles at the feet and a cool compress on the head. The patient lies in this pack for thirty to forty-five minutes and is then rubbed off. A cool affusion may be given first. With delicate patients it is well at first simply to wrap the patient in warm flan- nels until free perspiration results, then give a cold affusion or wash-off and rubbing.
In Turkish baths the patient is exposed to a temperature at first of 1300 or 1400 F. for fifteen to thirty minutes, and then to one of
74
DISEASES OF THE NERVOUS SYSTEM.
1600 or 200° F. for a shorter time. This is followed by massage and a cold affusion or plunge or shower. The effects of these baths are somewhat tonic if not too prolonged. The patient should never go into the hotter rooms until he perspires and he should select bath- rooms that are well ventilated. Russian baths have similar effects, but the bodily temperature is raised to a higher degree in them than in Turkish, owing to the lessened amount of perspiration due to the presence of steam.
Hot sitzbaths. The patient sits in water at a temperature of ioo° to 1250 F. for twenty or thirty minutes. Sometimes mustard is added. Cold sitzbaths are given in the same way, and are often useful in sexual neurasthenia.
Hot compresses consist of layers of flannel wrung out in hot water and covered with dry flannels and rubber cloth. They are used to re- lieve local pains and inflammations. They may be applied over the abdomen for insomnia. Hot sprays and douches are used for similar purposes as fomentations. The hot spinal bag is applied at a tem- perature not above 1200 F.
Physiology. — Warm baths, if applied in the form of the moist pack, followed by sponging with tepid water, lessen temperature by increas- ing heat radiation and conduction. If applied so as to prevent radia- tion, the bodily heat is raised. Warm baths increase the circulation of the skin, lessen cutaneous sensibility, withdraw blood from the central organs, increase the exhalation of C02, but lessen the respira- tory activity on the whole. Nitrogenous metabolism is increased by from 2 to 3 per cent, and more urea is excreted. Pulse and res- piration are increased. Nervous excitement is lessened, and the gen- eral effect is to cause sedation and a feeling of languor. The wet pack is a most useful sedative in neurasthenia and insomnia, and may take the place of medicinal sedatives, like the bromides. It should be given three or four times weekly, or for a short time daily. The luke- warm bath ranks next in its sedative efficacy. It is believed that appli- cations of water to the feet and abdomen especially affect the intra- cranial circulation, given to the thigh and wrists the pulmonary circulation; cold causing congestion, and heat anaemia of the distant parts. Cold to the spine is believed to cause at first constriction, and later dilatation, of the thoracic, abdominal, and pelvic vessels ; heat has the opposit effect. Hence cold applications are used to relieve cold feet and anaemic conditions of the viscera.
The fact must be borne in mind that cold baths and frequent bath- ing of any kind debilitate some few persons. Special details of hydro- therapy are given in the appendix.
HYGIENE, PROPHYLAXIS, TREATMENT.
75
Massage. — -The term massage may be made to include all the ma- nipulations of the body for the purpose of curing disease. The different methods of applying it as classified by Jacoby are:
Effleurage or gentle stroking. The maximum force to be applied here should not exceed the weight of the hand. Massage d friction or rubbing. Energetic strokes with one hand and strong circular or to-and-fro friction with the other. Petrissage or kneading. Tapote- ment or percussion with the fingers, hands, or instruments. Frictional movements , passive, active and combined with movements are made by the operator. The physician may be reminded that a male opera- tor is a masseur, a female a masseuse, and that the patient is massed.
Massage accelerates the lymph and venous currents, and thus promotes absorption. It increases at least temporarily the number of red blood-cells (Mitchell). It increases the rapidity and force of the heart-beat (except abdominal massage which slows the heart) and helps to relieve local congestions and inflammatory deposits. It presses and stretches the terminal nerve filaments, increases the irri- tability of motor nerves and the contractility of muscles. It may either increase or lessen the irritability of sensory nerves according as it is applied. Of the various forms of massage, tapotement is frequently useful and is the kind often used in neuralgias. It is applied not only with the fingers and hand, but also by the aid of rubber tubes known as muscle beaters, rubber balls with rattan or whalebone handles, percussion hammers and various percuteurs.
Regular muscular movements according to a certain fixed schedule are used in the treatment of locomotor ataxia and paralysis agitans. The details of these exercises, known as the method of Fraenkel, will be given later.
Massage is of considerable value in certain forms of atonic neu- rasthenia and hysteria associated with anaemia, dyspepsia and feeble circulation, in hemiplegia, in the paralyses of peripheral origin, in func- tional spasm, especially in some forms of writer's cramp and allied neuroses, in cerebral hyperemia, insomnia, constipation and in headache and some neuralgias, especially those about the head, neck, and arm. It is contraindicated in heart disease, arteritis or when there is danger of dislodging a thrombus.
Climate in Nervous Diseases. — The factors which make up a special kind of climate are : Purity of air; temperature; humidity; sun- light; rarefaction of air; ozone; wind; electricity; soil; trees; social conditions.
Regarding these points, some facts are very well settled. The air in the country is purer than in cities. The air on the sea and
70
DISEASES OF THE NERVOUS SYSTEM.
at high levels is purer than in other localities. The temperature above the sea level diminishes about i° F. for every 300 to 350 feet, and is less the dryer in the air. Alterations in temperature are less near the sea and less in the southern hemisphere. The higher the elevation and the colder the air, the less moisture does it contain. About the factors of ozone and electricity in the air little definite is known. As to sunlight, an excessive amount of it seems eventually to enervate patients.
Climates are classified by Weber into marine, low-level inland and high-level inland. These all have great variations in quality, depend- ing upon their temperature, moisture, etc. As a general rule, warm marine climates and sea voyages are best for neurasthenic invalids of the irritable type. On the other hand, in atonic and anaemic con- ditions high inland climates are better, at least for a time. Such climates should not be too dry or windy.
In organic degenerative diseases of the nervous system, marine climates and low levels are better. As a whole, it seems to be the con- clusion that plenty of fresh air is the essential in all climates and is better than any special climates. Relatively short changes from low to high levels or from temperate to tropic climates often leave a distinctly tonic effect.
Germany, the Riviera, the Bermudas, the West Indies, southern Colorado, Arizona and Southern California are favorite places for sending neurasthenic Americans. Camp life in the Adirondacks or other forests is also found most useful.
Electricity in Nervous Diseases.
Physical. — Electricity is a form of energy. Physicists assume that the electrical force exists in two conditions, positive and nega- tive, and we speak of positive electricity and negative electricity accordingly. Under ordinary conditions these fluids are united and in equilibrium; but by certain agencies, such as friction, heat, chem- ism, etc., they can be separated. We assume that the condition of electrical equilibrium is that of zero, and that the earth's electricity is at zero. Positive electricity is raised above, negative electricity pulled below, this zero point. Electrical phenomena result from attempts of the fluids to become equalized or stable again at the zero point, just as the phenomena of heat result from differences of temper- ature and those of gravity from difference of pressure. The distance to which the electrical fluids are separated from the zero point is spoken of as the difference in "potential." This potential corre-
HYGIENE, PROPHYLAXIS, TREATMENT.
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sponds to the term "degrees" in measuring heat. Now, the greater the difference in potential, the greater the effort of the fluids to re- turn to zero. High and low potentials correspond to high or low intensity of heat. Tension is the result of the widely separated fluids striving to return to the zero point. It is the same thing as dif- ference of potential, and the term may as well be dropped. When the two fluids, at different potentials, attempt to become equalized, they pass along certain paths and form electrical currents. Elec- tricity will pass along any substance, but some substances conduct it more easily than others, and these are known as conductors. The relative value of different conductors is shown in the following table:
i. The metals; 2, charcoal; 3, plumbago; 4, dilute acids; 5, saline solutions; 6, pure water; 7, living animals; 8, flame. Of the metals, silver and copper are the best conductors. The human body would have about the same conductibility as the saline solutions, if it were not for the skin, which is a very poor conductor, especially when it is dry. Those substances along which electricity passes with great difficulty are known as non-conductors or insulators. The following is a list of some of these, the substances arranged in accordance with their relative value:
1. Caoutchouc; 2, silk; 3, glass; 4, wax; 5, sulphur; 6, resins; 7, shellac ; 8, dry air.
The electrical fluids may be kept by insulators at different poten- tials, the insulators preventing them from becoming equalized or re- duced to the same potential. Electricity in this condition is called static. Its study is called electrostatics.
The electric fluid in motion is called dynamical electricity, and its study is electrodynamics.
Technical Terms. — -There are certain technical terms which it is necessary to understand. Electromotive force (symbol, EMF) is the force which tends to set electricity in motion. An electric current results. The current strength (symbol, C) is the t^rm used to ex- press the capacity of the separated fluids to overcome resistance in their attempts to reach equilibrium or equalization again. This cur- rent strength, or simply the current, naturally is in proportion to the strength of the electromotive force, which is constantly dissociating the electrical fluids and generating the current. If, however, as is always the case, the electrical fluid meets resistance in seeking equi- librium, the resistance diminishes its current. Hence we have the formula known as Ohm's law:
Current strength =
Electromotive force Resistance
; or C =
EMF R '
7«
DISEASES OF THE NERVOUS SYSTEM.
All bodies offer some resistance to electric 1 currents, and it is important to have some standard unit of resistance for the sake of com- parison. Such standard unit has been adopted and is called an ohm. It is the resistance offered to a current by a certain piece of wire of definite size and length.
A volt is the unit of electromotive force, i.e., it represents the force which will generate a certain amount of electricity in a second of time. A Daniell cell is of not quite one volt strength.
An ampere is the unit of working power or current strength. It is the current strength produced by one volt of electromotive force working against one ohm of resistance. A milliampere is one-thou- sandth of an ampere. A watt is the unit of work.
When a given current flows along from a large into a small con- ductor, the quantity in this latter conductor in a given section is greater and the current is said to be denser. The instrument by which the strength of a current is measured is known as the amperemeter; in medical practice, only fractions of the ampere are used, and the instrument is called the milliamperemeter. A rheostat is an instrument for interposing resistance in a current.
Electrical Appliances. — The bacteries used in neurological prac- tice ar of three kinds: the static, the faradic, and the galvanic.
The static electrical batteries are mostly modifications of the Holtz influence machine. They are inclosed in glass to prevent the effects of moisture. The instruments made in this country for medical pur- poses will usually furnish electricity all the year round. The battery accessories consist of an insulated stool and brass-ball electrodes with glass handles. The patient is placed on the stool, which is connected by a rod with one of the prime conductors. The battery being started, the patient becomes enveloped, as it were, with a layer of electricity which is at a very high potential and constantly flying off, being retained only by the dry, non-conducting air. The electrode held by the operator and connected directly or indirectly to the other prime conductor is now brought near the patient. The electrical fluid bounds to the zero point with such force that some of the metallic parts of the electrode are carried off and ignited, causing the disruptive spark. The patient thus is discharged of the fluid; but it contin- ually reaccumulates upon him, and thus one can keep on drawing sparks from all parts of the body. The electric spark causes a mus- cular contraction and, a little later, a small punctate red spot. There is some pain connected with it, but as the electricity penetrates the body for only an infinitesimal period of time, it directly affects the viscera but little and is not dangerous. Various ingenious electrodes
HYGIENE, PROPHYLAXIS, TREATMENT.
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have been devised for modifying the character of the static discharge, but they all have much the same effect as the spark.
By approximating the prime conductors so that they almost touch, and then connecting the outer surface of the two Leyden jars which hang from the prime conductors with sponge electrodes, one gets the static induced current described by Dr. William J. Morton. The special peculiarity of this current, as well as of the spark discharge, is that it is made up of electricity at a very high potential, and that, being composed of oscillating or alternating currents of extremely short duration, the quantity of electricity is very small. The result is that when a muscle degenerates, it loses its irritability to the static current very early. The static current will thus reveal a beginning degeneration of nerve sooner than the other currents ; it also promotes tissue changes more than other forms. It sometimes stimulates a greatly degenerated muscle, so that later the other forms will pro- duce a contraction. It has a powerful psychical effect, and lends itself readily to quackery.
In faradic medical batteries the electricity is produced by induction. The chief elements are a cell and two coils of insulated copper wire. One coil is placed around the other, the outer coil being longer and of finer wire. The cell generates a current which in turn "induces" the electricity which is received by the patient. The original current is so arranged that it is being constantly broken and closed or "made" again. At both break and make, a current is induced in the inner coil of coiled wire; this forms what is called the primary induced current and is made up of a succession of short currents. This current, though theoretically alternating, is really a current of one direction. For the "make" current is opposed and nullified by the original or battery current. The same is true of the secondary induced current which is excited in the outer coil by the currents of the inner coil. Both currents are really composed of a rapid series of single currents going the same way, and they each have a positive and a negative pole. The primary current is one of lower tension, and is rather less strong. It can be used when the resistance of the parts is not great and a very powerful current is not needed. The secondary current is one of higher tension, it overcomes resistance better, and can be employed in connection with the wire brush and in anaesthetic conditions. It is also the current used in measuring the strength of the faradic application, as will be shown later. The current of the second- ary coil resembles that of the static induced current. It has, how- ever, a lower potential, vastly slower alternations and more quantity. It can contract muscle which the static current cannot affect. The
8o
DISEASES OF THE NERVOUS SYSTEM.
secondary coil should be made of wire of a standard size, length and layers of coil. A standard coil of wire 800 metres long, .225 mm. in diameter, wound on a spool 100 mm. long, has been adopted.
Faradic batteries are made with the zinc-carbon or Grenet cell, the Leclanche, the silver-chloride cell, or some modification of these. A dry cell made with muriate of ammonia has now come into use. Faradic and galvanic currents are now often obtained from the electric- light wire now so generally supplied to houses.
Electro-Therapeutics.
High and Low Potential Currents — Currents of "Tension" and "Quantity." — The current of the faradic battery varies in quality in accordance, (1) with the length and number of turns in the coil; (2) the form of the electrical wave; (3) the number of vibrations; and, (4) the strength of the battery.
The currents from short coils of coarse wire have a lower potential and slightly more voltage. They are more efficient in producing muscular contractions and are more irritating to the sensory nerves. These qualties are increased with slow interruptions of three or four per second. When muscular contractions and mechanical exercise with stimulation are desired, the short coils (primary or secondary) with slow interruptions are indicated. The current from long coils has a higher potential and less voltage ; it has less power in contracting muscles and a different effect on the sensory nerves. When the in- terruptions are very rapid and the coil is very long, the effect on the sensory nerves seems more sedative; perhaps, in part, because of a change in the form of the electrical waves.
Instrument-makers have devised instruments with long coils (1,500 yards) which are tapped at three places, so that with one coil it is pos- sible to get a current of low potential (or "quantity," as it has been called) or of high potential (or " current of tension"). The vibrator is made also so that currents of extremely rapid interruption can be obtained. These instruments are expensive, but are of special use to the neurologist in those cases in which he desires to apply faradism for spasm or neuralgia. They are said to be very useful in gynaeco- logical work.
The physiological effect of a current depends, as I have stated, in part upon the character of the wave of electrical force. If this is high and sharp, the stimulation is different from that produced by a wave which gradually rises to its height. D'Arsonval has de- vised an instrument for producing these blunt-topped waves, and
/
HYGIENE, PROPHYLAXIS, TREATMENT.
8 1
at the same time reversing the current. The current is called sinus- oidal and is produced by revolving magnets, and the machine is expensive and complicated. I am not aware of any special thera- peutic results in neurology from this kind of current. The Leduc current is a sharply interrupted-galvanic current. It is claimed that when sufficiently powerful, 20 to 60 volts or more, it produces local anaesthesia and when passed through the head to produce general anaesthesia and sleep.
Very rapidly interrupted and alternated currents of extremely high voltage are passed through the body and cause increase in metabolism and lowering of blood-pressure.
Galvanic Batteries. — There are two kinds of these in practical use. The one includes the cells which act as soon as the circuit is closed ; the
Fig. 36. — Author's electrode set.
other includes those which act only when the elements composing the battery (e.g., zinc, carbon, platinum, copper, etc.) are dropped in the exciting fluid. The former class (known as two-fluid cells) are not touched except to renew water or add some chemical. In the latter (the single-fluid cells), one of the elements is always taken from the fluid when the current is not in use. The electric-light current has been utilized to supply continuous currents and for purposes of the cautery, and rendered portable or individual batteries less necessary.
The ordinary accessories to the faradic and galvanic batteries are electrodes, rheostat and milliamperemeter. 6
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DISEASES OF THE NERVOUS SYSTEM.
The electrodes needed for ordinary purposes are: An indifferent electrode measuring 5 cm. by 15 cm. A normal electrode, 10 sq. cm. A unit electrode, 1 sq. cm. A soft wire brush. Three handles: one 10 cm. and one 40 cm. long, one short handle with an interrupter. A milliamperemeter. ' A rheostat. (See Fig. 36.)
Methods of Application. — Static electricity is applied for fifteen or twenty minutes daily or tri-weekly. For general tonic or sedative effects, sparks are drawn from all parts of the body except the face. In paralysis or spasm or pain, sparks are applied to the affected area. For headaches and cerebral parsesthesiae, the electrical breeze is very useful, but it must be strong.
The faradic and galvanic currents are used for about the same time and intervals as the static. In some cases, however, the galvanic current should be given daily or even two or three times a day. As a rule, a course of electrical treatment should be continued for six to eight weeks, and then discontinued for a time.
The special methods used in applying these currents are:
1. General galvanizatkn and faradization cr general electrization.
2. Local electrization by galvanization of the brain, of the neck, of the spine, of the special senses, limbs, and viscera. Or by faradi- zation of the neck, spine, limbs, and \dscera.
3. The combined faradic and galvanic currents. These ase given by means of the De Watteville switch. General and local electriza- tion can be given in this way.
4. The polar method. This is employed chiefly in using the gal- vanic current. The indifferent electrode is placed on the sternum or back, and the other electrode applied wherever indicated.
5. Cataphoric electrization by means of Peterson's electrode.
6. Electrolytic applications are used in enlarging strictures and affecting inflammatory deposits and neoplasms.
In general electrization, whether galvanic or faradic, the indifferent electrode is placed on the sternum, feet or back, and the other pole is carried over the limbs, trunk, neck and, if indicated, the head. In some cases, however, the two electrodes are applied together upon the different muscles of the body. In local electrization, the large elec- trode may be applied on an indifferent spot, and the other applied to the affected limb or limbs, or the two electrodes may be used together on the same segment of muscles. There are special points at which the muscular contraction is most easily brought out. These are called the'motor points. (See Figs. 36 to 41.) In the ordinary practice of apptying electricity for spinal-cord disease with galvanic currents, a very minute amount of electricity reaches the cord. With large
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electrodes, however, and currents of 50 to 140 milliamperes the cord is reached with a current of \ to 1 \ ma. strength. The details for galvanizing the brain, special senses and viscera must be obtained from special text-books.
When an electrode is held steadily upon a part, it is called stabile; when it is removed over it, it is called labile. The positive pole is called the anode (An), the negative the cathode (Ca). The size of an electrode is indicated in square centimeters.
A formula for applying electricity may be written thus :
Anod. galvanization, 10 ma. 5 X 15 cm. daily, 5' stabile.
This means that the positive pole of the galvanic current is to be applied steadily at a given place daily for five minutes with an elec- trode of 75 square centimeters.
The terms "ascending" and "descending" currents are rarely used, the name of the pole being employed instead. Thus, anodal galvanization of the brain or arm means that the positive pole is applied at these localities. With the faradic and static currents, neither the pole nor the direction of the current makes much difference.
Electro-Diagnosis . — When a motor nerve is cut off from its centre in the spinal cord, or when this centre itself is diseased, the nerve and later the muscle undergo a degeneration. As a result of this, their reaction to electrical currents is changed, and we get what is termed "partial degeneration reactions" and "complete degenera- tion reactions," according to the degree of disturbance. These reac- tions are due mainly, if not wholly, to the degeneration in the ter- minal nerve-fibres and motor end-plates in the muscle. When the muscle alone is diseased, the reaction is not changed until very late. The change in irritability is due to the fact that as the nerve-fibre wastes it takes an electric current of comparatively long duration and considerable strength to stimulate it.
The first effect is to cause it to lose its contractility or reaction to weak currents, then to extremely rapid, short currents like the static, then to the faradic, and last to the galvanic. Such change is known as the quantitative alteration in electric irritability.
But besides this, the nerve and muscle are affected in a different way by the different poles of the galvanic battery. In normal nerve and muscle, a contraction is caused more readily by the negative pole than by the positive. But muscles with degenerated nerve supply sometimes respond as well or better to the positive pole. This forms what is called the qualitative or serial change in the irrita- bility of the muscle.
84
DISEASES OF THE NERVOUS SYSTEM.
Finally, degenerated muscles respond more sluggishly than normal to the gahanic and faradie currents. The contraction, instead of being sharp and jerky, is sluggish and almost tetanic. This is called the modal change in irritability, and it is far the most important sign of muscular degeneration.
The qualitative change is gotten only by placing the active elec- trode over the muscle, but the quantitative and modal changes may be gotten by placing the electrode over the nerve as well as over the muscle. In describing these changes, the following abbreviations are used:
DeR = degeneration reaction.
AnCC = anode or positive-pole closure contraction. CaCC = cathode or negative-pole closure contraction. AnOC = anode opening contraction. CaOC = cathode opening contraction. Te = tetanus.
D = circuit is closed and current flowing.
AnDTe = tetanic contraction while the positive pole is applied and the circuit closed.
The sign > means greater than; <, less than. Thus AnCC < CaCC means anode closure contraction is greater than cathode closure contraction.
Degenerations in nerve do not occur except in lesions of the nerve or spinal cord and in very late stages of primary atrophy of muscles. Hence, when one finds degenerative reactions, he can almost abso- lutely exclude disease of the brain, functional disease and primary disease of the muscle. The following rules may be formulated for testing for degeneration reactions:
Use the faradie current first.
The Faradie Current. — Use a secondary induction coil of wire .225 mm. in diameter and 800 metres long. The distance over which the coil moves is divided into a hundred parts. The strength of cur- rent is indicated by percentage or millimetres.
In many scales it takes 30 to 40 mm. of CD, or coil distance, to cause a muscular contraction. Record the minimum necessary for muscular contraction, using the same electrodes and in the same way as in testing with galvanism.
The Galvanic Current. — 1. Place the indifferent pole over the sternum and a 10 sq. cm. electrode over the muscle. 2. Pass the
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85
current for one minute. 3. Then find the minimum strength needed for a cathode closure contraction. 4. Then for an anode closure con- tration. Repeat this test three times. 5. With a given current, note whether the cathode closure contraction is stronger than AnCC or otherwise. Test this three times. 6. Note the character of the contraction, if sharp or sluggish. 7. Test nerve in same way.
The qualitative changes may be expressed by a formula like AnCC = or > CaCC; i.e., the positive-pole closure contraction is equal to or greater than the negative-pole closure contraction. Or, better, th° minimum strength of current required to cause a contrac- tion in the muscle is recorded for the positive pole and for the nega- tive. Thus :
The following table (modified from De Watteville) and diagram (Fig. 37) show the diseases in which degeneration reactions may be expected :
Eig. 37.
AnCC 5 ma. or 8 cells. CaCC 4 ma. or 6 cells.
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DISEASES OF THE NERVOUS SYSTEM.
Table Showing the Lesion, its Results, the Names of the Diseases the Electrical Reactions.
Lesion of-
Disease.
Electrical Reaction as to Qualitative.
i to 2\. Cortex to cord.
3, 4 and 5. Cornua.
2 to 2% 3 to s. Lat. cols, and ant. corn. 5 to 6.
Nerve . Muscle
[Paralysis, contractures: Hemiplegia from hemorrhage.
I Embolism, tumors, lateral sclerosis.
Paralysis, degenerative! Acute and chronic anterior atrophy of nerve andi poliomyelitis, muscle.
Paralysis, contractures Amyotrophic lateral sclerosis.
Nerve and mus- cle.
Degenerative atrophy
of muscle. Degenerative atrophy
of muscle.
Later, degeneration of nerve.
Progressive muscular atrophy (spinal form) , bulbar paraly- sis.
Paralysis; degenerative Neuritis; from wounds, tox- atrophy of nerve and emia, or pressure, muscle.
Wasting, paresis.
Nerve: normal. Muscle: normal.
Nerve: DeR. Muscle: DeR.
Nerve: normal I Muscle: partial j
Nerve: normal. Muscle: or partial DeR. When disease is advanced.
Muscle: DeR. Nerve: DeR.
DeR.
or I Nerve and muscle normal until late in the disease.
Simple atrophy ; _ primary
idiopathic myositis. Juvenile form of progressive muscular atrophy; pseudo- muscular hypertrophy; other types of primary myopathies Paresis and atrophy. [Rheumatic atrophy and pare- No'.DeR. unless severe phy. sis.
It should be said, finally, that it is the sluggish contraction which is the most important element in showing degeneration; also that it is the muscle which should be tested most carefully, as only over it does one get the qualitative changes.
Therapeutics.- — Electricity is used as a counter-irritant and as a general mechanical tonic in states of muscular and nervous weakness. It is used in paralysis, spasm and pain, and for its supposed specific action in certain functional and organic diseases.
The faradic and static currents have a counter-irritating, stimu- lating and excito-reflex effect. The galvanic current has a sedative and antispasmodic effect.
Electrolytic, cauterizing, and cataphoric effects are also produced, but are rarely needed by the neurologist. A considerable portion of the effects of electricity are psychical, but they are not the less real or valuable.
Radiant energy in the form of X-rays, ultra-violet rays, radium emanations have been of more help to surgery than medicine in therapeutics. The X-ray and violet rays and radium are said to have some effect in neuritis and neuralgia, but the results are not uniform or as yet conclusive.
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Psychotherapy.
Psychotherapy is a term applied to that form of healing in which mental influences are used to correct morbid processes.
Psychotherapeutics has always been used in an irregular and casual way to cure disease. Modern therapeutics endeavors to formu- late definite rules for its use and to indicate systematically the condi- tions for which it should be applied.
Hypnotism is the oldest and best known method. In hypnotic therapeutics the patient is put into a partial or complete hypnosis, in which the psychical activities are in abeyance, the volition is suspended, and the body reduced to an automatic state. Sugges- tions and directions as to cure are then made, which the patient on coming out of his hypnosis follows. This method of treatment is uncertain and difficult and is of use only in a narrow field. It has been practically abandoned, though occasionally its use gives bril- liant results, mainly in hysterical conditions, morbid habit of not long standing and the minor psychoses. It is more effective in the younger subjects. It is of no use in the serious psychoses.
Suggestion with minor hypnosis is only a modification of the preceding method. Here the patient is simply persuaded into a quiet, receptive mood or into a slight degree of hypnotic trance, which is oftener really only a slightly somnolent state. Then suggestions are patiently and insistently repeated. This method is more simple, and on the whole more useful than the first. It has to be repeated perhaps as often as a dozen times before the desired impression is secured. But it is fairly efficient in the phrenasthenic, more particu- larly those with fixed ideas, morbid fears, attention pains and morbid melancholic moods. The treatment is most useful here also in cases that are not of very long duration. It deserves a permanent place in neurological therapeutics, but it can only be applied by those who have a thorough knowledge of the patient, of the disease and, I may add, of themselves. It requires a steady and forceful and confident personality. Formally and strictly applied, it has not a wide field. In a simpler and common-sense way, however, the method can be used by any physician, and is used, no doubt, all the time, for it really consists in giving the patient the vital sense of proportion in his judgment of things. In many patients the harmful thoughts are not entirely subconscious. The patient knows them, but has not quite recognized their importance and the need of treating them sanely and definitely and putting them in their right relation.
The method of psychic analysis and reintegration. This is also
88
DISEASES OF THE NERVOUS SYSTEM.
called the method of Freud. It cannot be described in detail here. We assume that many of the morbid mental states and bodily per- versions are dependent on the activity of a subconscious activity of the mind. Below the field of ordinary conscious processes, there is memory of some earlier event acting, unknown to the patient, upon his conscious mental states and leading him to certain morbid ideas or emotional moods which cause his sickness. The operator, by study- ing and questioning, by learning of his dreams, by telling him to " empty himself " of his troubles by a kind of "mental catharsis, " finds certain subconscious states which relate to events of his past; or, finally, by hypnotizing, finds out what this lurking idea or emotional state is which is doing mischief. He then confronts the patient with the facts, shows him what is the real cause of his obsession, persuades him of its unreasonableness or accustoms him to look upon it in a normal way. In other words, he brings the true cause of the trouble to light and enables the patient to fight it.
The Method of Re-education. — This may be called also the method of "therapeutic talk." It is known as the method of Du Bois. It is really an application of the principles of suggestion and of readjust- ment, or re-education, used in the preceding methods. The patient has his condition explained to him, the aims of his physician are placed before him, the reasonableness of his cure, the unreasonable- ness of his mental state and of his point of view, are persistently argued over. The patient is sometimes persuaded, sometimes bullied into health. All this is done, however, after first isolating the patient from everyone and clearing the ground entirely of exogenous irritation. The doctor with the nurse, a necessary adjunct, has the field entirely to himself. Obedience is enjoined. It is, in fact, a rest and isolation cure, combined with forceful presentations of the reasonableness and possibilities of cure. No medicines are given, as a rule. This is a method now having much vogue, but it is only a modification of the Weir Mitchell cure, in which the psyche receives rather more dominantly the attention of the physician.
In estimating the value of these cures it must be remembered that they are useful practically only in the phrenasthenic and neurasthenic groups of patients, which includes neurasthenia proper, psychas- thenia, hysteria and the impulsive psychoses.
The mild melancholias so often mistaken for neurasthenia get well anyway. The melancholia in anxiety psychosis of involution is little affected by these methods, nor are the major psychoses, if we except perhaps paranoia and early types of dementia praecox, of largely psychogenesis origin. The early and abortive types of the
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89
anxiety psychosis, usually called hypochondriasis, may also be affected.
Irregular psychotherapy. — Therapeutic results are obtained by mandatory methods, by the method of extravagant promise or ab- solute assurance. These are the methods of the parent and guar- dian and of the charlatan and patent medicine label. Their suc- cess is more than counter-balanced by the evil done by inconsiderate assurance, in improper cases and by misleading persons; to use measures not in the least indicated by their condition.
The employment of psychotherapy in conjunction with religious work has recently been advocated and in some cities successfully carried out. A general indorsement of this union of medical and ecclesiastical work cannot be given, and its usefulness must have very definite limitations. Still, under very rigid control, it may prove an effective measure and be especially useful in counteracting the, on the whole, bad influence of various types and schools of "healers." One measure cannot cure all diseases or even all of one kind of disease, and the exploitation of infallible cures works an injury to society in the end.
Psychotherapy by Emotional Shock. — This is seen typically in the exhortations of religious healers and the dramatic methods of charlatans.
Philosophic Psychotherapy. — Most successful results are obtained among the always large number of suggestable and credulous minds by instillating a certain formula, squared by which their symptoms must cease, and the disease disappear. This is the method of the Doctrinaire, or Philosopher, or Apostle. For example, it is asserted that God is good, God is all, therefore there can be no evil in you. Sickness is an evil, therefore it cannot exist. Therefore you can- not be sick. You are well. This doctrine ingeniously and eloquently poured into simple ears gradually gains conviction, the patient re- ceives assurance, the attention is distracted from morbid symptoms and the patient gets well, sometimes. The doctrine applied to many thousands always reaches some who only need this distraction of attention from themselves. They ignore symptoms, and gradually their consciousness gets less sensitive to them. They harden their sensorium, and though they have conditions which would make some sensitive souls feel distressed, these conditions do not arouse their attention which is complacently and wisely directed elsewhere.
The only objection to this method of therapeutics is that its fundamental propositions are unproven, and its general application to surgery, infectious and organic disease is disastrous. But the
go
DISEASES OF THE NERVOUS SYSTEM.
philosophy of attention-diversion to the healthy and holy side of life is wise and is indeed used by all physicians and in every form of scientific psychotherapeutics.
The essential thing in all forms of systematic psychotherapy is to have the patient on the proper stage. He or she must be removed from all influences but those of the doctor and his attendants. The atmosphere must be such that only his personality and views and counsels affect the patient. Then the work must be kept up for at least a few weeks and often longer. With this environment, the results are reasonably certain in proper cases. But without it, there is only a chance that the wisest counsel prevail.
Then the physician himself must be suited to the kind of work. He need not have a commanding personality, but he must be simple and impressive and sincere, and he must have no unpleasant personal habits.
The nurse, if there is one, must equally be a person of tact and experience, good personality and able to co-operate.
It can be seen that the systematic application of psychothera- peutics, at its best, is not possible for all, and is not suitable for most. Its unsystematic or rather informal use will always be a part of the equipment of the wise physician. It makes it all the more imperative that physicians be good as well as wise.
CHAPTER VII.
GENERAL DISEASES OF THE PERIPHERAL NERVES.
Introduction — Anatomical. — The peripheral nervous system consists of twelve pairs of cranial and thirty-one pairs of spinal nerves, with their root ganglia and terminal sense organs, and the sympathetic nervous system. The sympathetic system is composed of the inter- vertebral and the cranial ganglia and the peripheral ganglia. The latter arise during embryonal life from ganglionic cells of the same class as those of the spinal root ganglia, and migrate to their adult position in the sympathetic (Minot). The peripheral nervous system, therefore, to use the language of modern anatomy, is composed of peripheral motor neurons, peripheral sensory neurons, and peripheral ganglionic neurons (Minot).
The Origin of Nerves. — The discoveries of the true nature of the nerve-cell and its relation to the nerve-fibre have made a great difference in our conception of the nervous centres. We assume that the peripheral nerves are essentially the prolongations of the axis-cylinder processes of the nerve-cells, with certain anatomical additions which nature has made in order to isolate and support them. The peripheral nerve-fibres are only a part of certain neurons. The motor nerve-fibres come from the motor nerve-cells, and from the peripheral part of the peripheral motor neurons. The sensory fibres are derived from the sensory nerve-cells, and are only a part of the sensory neurons. All peripheral nerve-fibres of motor nerves have, in the spinal cord or brain, certain cells of origin, which are known as the nuclei of origin of these nerves. These motor nuclei lie in the anterior and lateral horns of the spinal cord, and in the corresponding parts of the medulla and pons. It has been customary to refer the sensory nerve-fibres also to nuclei of origin in these same parts. This, however, according to modern views, is not correct. All sensory nerve-fibres take their origin from nerve-cells in the posterior nerve ganglia, or in corresponding cranial ganglia, such as the petrous and the jugular, lying upon the roots of the cranial nerves. The nerve-cells in these ganglia send off a single process which divides in a T-shaped fashion, the peripheral branch going out to form the sensory fibre, the central branch passing into the cord or brain, to end in a terminal
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arborization which surrounds groups of sensory nerve-cells. These sensory nerve-cells in the cord are, therefore, not really nuclei of origin, but are terminal nuclei. There are no nuclei of origin for sensory nerves in the central nervous system. This changed point of view is of especial importance in our consideration of the anatomy of the cranial nerves (Fig. 38).
Hyperemia and Anemia. — Under the head of hyperemia and anaemia there occur types of nerve irritation, leading to different forms of neuralgia, paresthesia and motor weakness or irritation.
Fig. 38.— Showing the cells of origin of the motor nerves in the anterior horns of the spinal cord, and the cells of origin of the sensory nerves in the posterior spinal ganglia. (Van Gehuchten.)
Hyperaemia and anaemia are, however, secondary conditions and are rarely recognized clinically. It cannot always be determined whether an irritated nerve is congested or anaemic, or whether the central part of the nervous system is not mainly at fault.
Angina of the Nerves. — In arterio-sclerotic conditions of the blood-vessels of the posterior ganglia and nerves there occur with spasms closure of the vessels or, in other words, anginal attacks, which cause severe neuralgic pains. The pathology of this condition will be referred to later.
Inflammation of Nerves — Neuritis. — There are two forms of neuritis: 1. Interstitial and perineuritis. 2. Diffuse neuritis with parenchymatous degeneration. The first form may be acute or chronic.
General Pathology.
GENERAL DISEASES OF THE PERIPHERAL NERVES. 93
In the first type there is hyperemia, with sometimes extravasation of blood. An exudation occurs into the fibrous frame-work of the nerve, with migration of leukocytes. The inflammation may be- come suppurative or gangrenous. If severe, it destroys the nerve- fibres; but oftenest the axis-cylinders are not destroyed, and re covery takes place.
In mild grades of perineuritis such as occurs in sciatica there is perineuritic exudation, a kind of serous inflammation with not much
Fig. 39. — Acute infectious neuritis, showing hemorrhage, connective-tissue proliferation, diseased nerve fibres, and obliterated vessel. (Rosenheim.)
cellular proliferation. This serous neuritis is probably the main factor in the typical neuralgias like sciatica and brachial neuralgia.
Chronic interstitial neuritis and perineuritis are accompanied with hyperplasia of the connective tissue, compression and more or less destruction of the nerve (Fig. 39). It may ascend or descend, and it is called, accordingly, ascending, descending, or migrating neuritis. It may affect only certain segments of the nerve, when it is called segmental neuritis or disseminated neuritis. Tuberculous and syphi- litic neuritis are of the chronic interstitial or diffuse type. These latter forms rarely involve peripheral nerves, but rather the intra-
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DISEASES OF THE NERVOUS SYSTEM.
cranial parts of the cranial nerves and the spinal nerve-roots in men- ingeal tuberculosis or syphilis. A syphilitic peripheral multiple neu- ritis is, however, thought to occur sometimes. Leprous neuritis is a very typical form of proliferating chronic perineuritis. Cancerous neuritis sometimes occurs, and it is of the diffuse type, though some- times an actual cancerous process invades the nerve.
The second type is called degenerative neuritis and this process of degeneration is the dominant one, so that the changes can be best de- scribed under the head of degeneration of nerves:
Degeneration of Nerves. — This is a process in which the nerve- fibres gradually die; the myelin sheath and axis-cylinder disappear, leaving only a strand of connective tissue.
Fig. 40. — Simple atrophy of a nerve in manrasmus.
There are three forms of nerve degeneration: (1) primary; (2) secondary; (3) neuritic or toxic.
1. The primary form is rare, slight in extent, and of little clini- cal significance. In it there is simply a gradual wasting and disap- pearance of the axis-cylinder and myelin sheath. It occurs in old age, wasting diseases and as part of locomotor ataxia (Fig. 40).
2. Secondary degeneration or Wallerian degeneration. This form occurs when the nerve is cut across, or compressed, or destroyed by inflammation, neoplasms or injuries.
The essential part of the nerve-fibre, the axis-cylinder, is simply a prolongation of the process of a nerve-cell. Its next essential part is the myelin sheath. This is of epiblastic origin and consists of a hollow cylinder inclosed in a thin membrane and containing a fatty substance. In degenerative processes of peripheral nerves the medullary sheath is first affected, then the axis-cylinder, least
GENERAL DISEASES OF THE PERIPHERAL NERVES.
95
and last the neurilemma. The myelin becomes turbid, splits up into fragments and droplets. The axis-cylinder also breaks up into fragments or swells up and becomes liquefied. Bxtravasated leuko- cytes pick up the products of disintegration and form fat-granule cells. The neurilemma and its nuclei usually remain intact; The nerve dur- ing this time shrinks in volume and looks grayish and translucent, or grayish-red. The nerve finally become only a fibrous cord. Changes can be seen in the nerve within forty-eight hours, and by this time its irritability, which was first slightly increased, is lost. In about two weeks the disintegration of the myelin sheath and axis is practically complete (Fig. 41). The peripheral end of the cut nerve shows a loss of nearly but not quite all the fibres as far as
Ftg. 41. — Showing different stages in the process of nerve degeneration. Second, third, sixth and ninetieth days after section. (Ranvier.)
its termination. In the central end, the degeneration ascends at first only to the first or second node of Ranvier. Very soon, however, a change occurs in the cell from which the fibre springs. This change is called the reaction at a distance, or degeneration of Nissl. Thus when the neuraxon is injured the whole neuron suffers, but the peripheral end far the most.
When a section is made between the spinal ganglia and the cord, the fibres all degenerate toward the cord, and even within it, but the peripheral fibres do not degenerate. Hence the spinal ganglia are the trophic centres of the sensory nerves (see Fig. 42).
Degeneration occurs in the motor nerves, also, when the cells of the anterior horns are destroyed. Hence these cells are the trophic centres for all motor nerves. The process of degeneration takes place at about the same time throughout the whole length of the
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DISEASES OF THE NERVOUS SYSTEM.
nerve. The motor end-plates in the muscles are affected a little the earliest. About the cut end, little bulbous tumors may develop, which contain numerous nerve fibrils and connective tissue. The general law is that nerves degenerate in the direction in which they carry impulses, but this is not the whole case, as has been just de- scribed. If the injury to the nerve is permanent, a slow decay affects the whole neuron. Supposing for example a motor nerve is injured or inflamed at the point D. Immediately a degeneration takes place
Fig. 42. — Showing effects of section of mixed nerves and sensory root. PMN., Section through mixed nerve; MR., motor root; S.R., sensory root; S.G., spinal ganglion.
along the parts below to T, and in a few days a slight degeneration takes place in the cell-body C (see Fig. 43).
Within the central nervous system degeneration also occurs mainly in the direction of the nerve impulse. The axis-cylinder is first af- fected. There is sometimes a preliminary swelling or hypertrophy of this axis-cylinder. Degeneration with calcification of the nerve-fibres sometimes occurs. In associative or commissural fibres the degenera- tion extends only part of the length of the nerve.
Fig. 43.
3. Neuritic or toxic nerve degeneration. This form occurs in connection with neuritis, and will be described under that head. Its chief characteristics are that the degeneration attacks the nerve in segments, that the axis-cylinders are not so much affected, and the myelin breaks up into small fatty droplets instead of into large masses. The same general laws apply to it as to Wallerian degeneration.
Degenerative processes in the non-medullated nervous fibres have
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97
been observed in the fine fibres of the cornea and in the submucous and mesenteric plexuses of the alimentary tract.
Regeneration of nerves is a process that usually follows degenera- tion. It occurs only in peripheral nerves — very little, if at all, in the nerves of the central nervous system of mammals. It is an unique process, in that the nerve is the only specialized tissue that can grow again after being destroyed. Regeneration occurs whenever the trophic centres are healthy, when the mechanical obstacles to a union of the divided fibre are not too great, and when the peripheral nerve is not too completely atrophied. It occurs most quickly, there- fore, when the cut ends are carefully apposed and when the separa- tion has not lasted for a long time, i.e., for years. It progresses always from the central end toward the periphery. The fibres of the central stump grow out into the degenerated peripheral fibre. Union by first intention or second intention never occurs. Human nerves cannot be made to unite physiologically, but only anatomically. Several high authorities have recently asserted that there is an auto- regeneration of nerves (Bethe, Ballance, Stewart et al.) ; i.e. that a severed nerve, after degenerating, regenerates of itself in man. Others explain this apparent regeneration by the powerful attractive force of degenerating nerve tissue on living nerves (neurotropism) (Fross- mann). The matter is not settled except in this respect that prac- tically no complete and efficient regeneration occurs without a re- establishment of the anatomical connection between the. cut nerve and its cell of origin.
Regeneration may be complete in a few months in short nerves. In the sciatic it may take one or two years. When regeneration takes place, the axis-cylinders of the central stump swell and divide into a number of new cylinders which pierce or creep around the in- tervening tissue, enter in bundles the peripheral nerve, and become inclosed in new myelin sheaths and neurilemma.
General Symptoms.
The dominant symptoms occurring in the distribution of the peripheral nerves are paralysis and atrophy of muscles in a greater or less degree. But since the motor nerve carries with it some vasomotor and secretory fibres, there may also be congestion, oedema, sweating, etc. When the motor neuron is damaged only to a moderate extent or is irritated by any agent, twitchings and spas- modic movements of the muscle may occur. Spasmodic symptoms in the distribution of peripheral nerves may also be due to disturb- 7
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DISEASES OF THE NERVOUS SYSTEM.
ances acting upon the central motor cell. The seat of the trouble is either in the sensory neuron, as in reflex spasmodic troubles, or in higher motor neurons. Thus, in a spasmodic disorder affecting the motor nerve A, the irritation may be in the cell body C, but is more often reflex, starting in the sensory neuron S or in a higher motor neuron whose neuraxon is represented at P (Fig. 49).
A symptom that is also very common in disease of the peripheral nerves is neuralgia, or the milder degree of nerve irritation called paresthesia. We have besides this various forms of anaesthesia, due to cutting off the sensory fibres. Neuralgia in its simple and commoner pathological forms is due to some irritation of the peripheral sensory neuron, especially its ganglionic centre. The particular nerve affected tells the location of the irritation. But the peripheral sensory neuron is in close relation with the central neurons that carry the painful impressions to the brain. Hence the whole sensory path may become disordered, and this is particularly the case in young and neurotic patients. As a consequence we find that neuralgias and pains are more diffuse and less sharply localized at this period of life. Besides this, it sometimes happens that the central sensory neurons in the brain are themselves the seat of injury or irritation, and we have at times, therefore, central neuralgias. How- ever, there can be no doubt that the central sensory neurons are not easily irritated to the point of exciting pain referred to a peripheral nerve, and this is because the ascending sensory tract is a widely dif- fused one and not massed in a single compact fasciculus.
It will be seen from the foregoing that the nervous diseases affect- ing the functions of the peripheral nerves will fall into three classes: first, those affecting a large part of the spinal nerves, at the same time causing general symptoms of paralysis, pain, etc.; next, diseases in- volving especially certain nerves of motion and, finally, those affecting especially nerves of sensation. So I shall describe general neuroses involving the functions of many and mixed nerves, then neuroses in the limits of the motor, and those in the distribution of sensory nerves.
MULTIPLE NEURITIS— POLYNEURITIS.
Multiple neuritis has been divided into many types in accordance with the cause, the location of the process, and the complications. 1. The common type is a motor-sensory one involving all four extrem- ities and due oftenest to alcohol. This type may be caused also by arsenic and other poisons and also by the infection of beri- beri.
GENERAL DISEASES OF THE PERIPHERAL NERVES. 99
2. The sensory and ataxic is much less common, is due more often
to infections and other poisons than alcohol, but it may be caused by alcohol also.
3. A pernicious and hemorrhagic type, due to overwhelming toxins
and infections.
There is also a pure motor neuronitis, multiple in character, due oftenest to lead. This is not, strictly speaking, a multiple neuritis, but a degenerative disease or overwhelming poisoning of the motor neurons. There is also a degenerative neuritis of the sensory neurons, especially noted in diabetes.
It seems best not to have too many kinds of multiple neuritis, and in fact the motor-sensory type is the common and real disease ; the others are terminal conditions, complications or sequelae.
The Sensory-Motor Type of Multiple Neuritis {Alcoholic [Paralysis, Arsenical Paralysis, Diphtheritic Paralysis). — This is the common form of the disease, making up fully ninety per cent, of all cases seen in the country. While sensory and other symptoms always accompany this type, it is the paralysis and atrophy of muscles which are the leading and chronic conditions. It is a malady coming on acutely, running a subacute or chronic course, rarely fatal, and char- acterized by weakness or paralysis of all four extremities associated with atrophy, pain, tenderness and various vasomotor, secretory, and trophic disturbances. It is a disease pre-eminently due to poisons and infections, alcohol being far the commonest factor.
It occurs oftener in the female, owing to the fact that alcohol poisons the nerves of women more than of men, and perhaps also be- cause of the predisposing influence of tea-drinking.
Multiple neuritis is essentially a disease of early adult life. Almost all cases occur between adolescence and the period of degen- erative changes, twenty to forty-five. Young children are very rarely subject to it, but cases have been reported occurring in children at the age of seven, nine, ten and fourteen, and G. M. Hammond has reported several cases occurring in infants. A few cases have oc- curred in persons over sixty ; but they are only a little less susceptible |than children. It occasionally occurs as an epidemic and is then due to some infection. Beri-beri is a particular type of infectious or toxic ultiple neuritis.
The sporadic forms of polyneuritis from alcohol and various jpoisons and infections occur without much reference to a seasonal in- fluence. Probably more cases occur in spring and fall, owing to sud- den changes in temperature. Epidemic influence like that causing cerebrospinal meningitis may increase the number of cases of mul-
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DISEASES OF THE NERVOUS SYSTEM.
tiple neuritis. Practically, in this country, the question of drink settles the question of the distribution of polyneuritis. It is rare in the temperate rural districts and smaller towns, and much rarer in native Americans than in foreigners.
Sexual excesses, exposure to cold and wet, insufficient diet, ex- cessive tea-drinking, the presence of tuberculosis, predispose to the disease. The same neuropathic tendency leading persons to excesses in alcohol, tea and to suicidal indulgence in arsenic is of some moment in leading to the development of neuritis.
The list of the special and exciting causes is long and includes nearly all infectious fevers, many chemical and autochthonous poisons. The common infections are diphtheria, puerperal and other septic fevers, and endemic infections of unknown origin. Nearly every infectious fever and possibly malaria may be added to the list.
Of chemical poisons alcohol heads the list, causing over two-thirds of the adult cases. Next come arsenic, lead and phosphorus, mer- cury, copper, tricnal, chloral, etc.
Wood alcohol is extremely poisonous, and may promptly cause a multiple neuritis. It has, however, a special tendency to cause optic neuritis and blindness. Even so small an amount as 3 oz. has caused blindness and 5 oz. blindness and death. Among the autochthonous poisons, rheumatism, diabetes and the metabolic products resulting from starvation and cachexia lead to multiple neuritis.
Among the foregoing causes, arsenic and diabetes produce more often decided sensory symptoms.
Prodromata. — The disease often begins with prodromata lasting several weeks. The patient suffers from numbness, slight pains, and weakness affecting especially the lower extremities. Sometimes a peculiar condition of mental confusion and weakness precedes the attack. Usually the symptoms come on rather suddenly. The patient suffers from pains and tenderness in the legs and feet, and is obliged to go to bed. There may be a fever for a day or two, the temperature rising to 1020 or even 1040 F., but this is not the rule. The pains and weakness increase. The muscles and nerves are very tender. The fingers, hands and arms are often similarly but less affected. At the same time the skin becomes reddened or slightly cedematous. The muscles of the legs grow weak, and in a day or two the patient is unable to stand. In a week or two there may be a complete loss of power in the anterior tibial muscles and a lesser degree of paralysis in the extensors of the hand (Fig. 44). Nearly all of the leg and forearm muscles become eventually involved. Atrophy sets in at the
GENERAL DISEASES OF THE PERIPHERAL NERVES. IOI
same time and very severe pains are present. The motor cranial nerves are in rare eases affected, and paralysis of the facial or of the third, fourth or sixth nerve has been seen. When the disease is fully developed, which is within a fortnight, there is paraplegia with foot- drop, some degree of wrist-drop, muscular atrophy, and slight oedema, especially of the feet. The skin reflexes are ofLen, the knee-jerk and elbow- jerk usually lost. There is some tactile anaesthesia, often with hyperalgesia. Temperature and pain sense are later lessened and slowed in transmission. The anaes- thesia occurs in patches or diffusely. Muscle and articular sense are lost in the sensory or pseudotabetic form, and are usually somewhat involved in the ordinary paralytic form. Pain and sensitiveness continue.
The nerves lose their irritability and the muscles show degeneration reaction, partial or complete, the characteristic being a great varia- bility of reaction over different groups of nerves and at different stages of the disease, and an early loss of faradic and lessening of galvanic irritability. There is sometimes retinal hyperemia and even optic neuritis. Of the vis- ceral nerves, the vagus seems oftenest to show signs of involve- ment, in rapid pulse and disturb- ances of respiration. The sphinc- ters are rarely involved and then only for a few days. In such cases there is, perhaps, involvement of the cord or of the abdominal and pelvic splanchnics. In alcoholic and occasionally in other forms of neuritis, mental symptoms, such as a low, muttering delirium, are very often present, and occasionally a well-marked confusional insanity develops. (Korsakoff's psychosis.)
The disease usually reaches itsjieight in a week or two and then starts on a chronic course; but it sometimes happens that exacerba- tions occur, or that a paralysis and atrophy progress for several weeks
Fig. 44. — Alcoholic multiple neuritis with foot-drop and wrist-drop.
102 DISEASES OF THE NERVOUS SYSTEM.
before regression begins. In alcoholic cases there is often great general prostration ; the patients lie for several weeks in a delirious condition, and finally develop pneumonia and die.
In diphtheritic neuritis some of the eye and throat muscles are in- volved, while the extremities are usually but slightly or temporarily affected and the sensory symptoms are few.
Some further details should be added.
Motor Symptoms. — The characteristic paralysis of multiple neuritis is a quadruplegia, all four extremities being involved. The special characteristic is the foot-drop, which is indicative of alcoholic neuritis, just as wrivSt-drop is of lead palsy. The paralysis is typically a per- ipheral one. It affects the feet and legs, hands and forearms. It usually involves the anterior tibial muscles more than the calf muscles, but sometimes the reverse occurs. The muscles become wasted and flabby. They soon lose in bad cases all reaction to fara- dism, and they require a strong galvanic current to produce a contraction. In anterior poliomyelitis, on the other hand, the dim- inution in galvanic irritability comes on only after weeks or months. Hence an early loss of galvanic as well as faradic reaction is an im- portant sign of neuritis. As the nerve and muscle recuperate, the galvanic irritability increases. After a time, if the paralysis is great, contractures occur. The feet are extended, the legs are flexed on the thighs, and are almost immovable, and the patient's condition is most pitiable.
Sensory Symptoms. — Numbness, hyperesthesia, severe pains (dull and sharp), burning sensations, great tenderness, all occur and are very marked symptoms. They are felt mostly in the feet, legs and hands. Hyperesthesia is usually followed by anaesthesia to touch and somewhat to pain and temperature. The transmission of these latter two sensations is delayed. The anaesthesia sometimes occurs in patches, at other times diffusely over foot, leg and hand. Muscular and articular anaesthesia are common, and in the sensory form are the dominant symptom, causing an ataxia of gait and station. The other special senses are not affected except in rare cases in which there is optic neuritis.
Vasomotor and Trophic Symptoms. — There is often oedema, some- times redness of the skin; occasionally the epidermis of the soles and palms peels off. Glossy skin and profuse perspiration are rare. Eruptions and ulcers do not occur.
Mental Symptoms. — The most common mental disturbance is that so often seen in acute alcoholism, viz., a muttering delirium. This is associated with great general vital depression. If a true insanity
GENERAL DISEASES OF THE PERIPHERAL NERVES. 103
develops, it also resembles, as a rule, alcoholic insanity or acute confusional insanity. The characteristic symptoms are a curious de- gree of forgetfulness together with many and varying hallucinations and delusions rapidly succeeding each other. These often relate to the pains and paresthesia from which the subjects suffer. They think that there are gloves on their hands or that something is on their feet. They often think that they have been out walking or riding. They are talkative, incoherent and sleepless.
Organic Centres. — The bladder is occasionally affected for a short time, the other centres not at all. This freedom from involvement of the sphincters is an important characteristic of the disease in dis- tinguishing it from myelitis. The blood shows a moderate degree of leukocytosis in uncomplicated cases.
From the foregoing it will be seen that the dominant symptoms are paresthesia, pains (burning, lancinating and dull), muscular tenderness, some anaesthesia, paralysis affecting especially the lower extremities and causing foot-drop, muscular wasting, with degenera- tion reactions; with no involvement of the sphincters; sometimes peculiar mental disturbances.
The sensory or pseudo-tabetic type of multiple neuritis is caused less often by alcohol and more often relatively by diabetes and the metallic and infectious poisons. Arsenic given medicinally in doses of one-sixth of a grain or more may cause such a neuritis. Multiple neuritis from lead is not often seen in painters, but usually when the poison is taken in large doses, as in snuff takers. The general course of sensory neuritis is much like that of the paralytic form, but there is less paralysis, and on the other hand there are more of the burning, tearing pains, a greater degree of anaesthesia, with a very decided muscular anaesthesia causing symptoms of a subacute locomotor ataxia. The paresis, muscular wasting, trophic changes, such as shedding of the epidermis and electrical reactions, serve to distinguish the disease. A facial paralysis sometimes com- plicates this type.
Endemic and Epidemic Types {Beri-beri or Kakke, Ignipedites, Acrodynia, Malarial Multiple Neuritis). — Beri-beri or endemic multiple neuritis is seen in this country rarely, and only by accident. Beri-beri is the Indian name; kakke, meaning "the leg disease," is its Japanese name. Ignipedites is a name given by Indian physicians to probably the same disease. French physicians gave the name of "acrodynia" to an epidemic disease which prevailed in France and the Crimea in the early part of the last century. It was probably multiple neuritis. Epidemic multiple neuritis has been observed in two State hospitals in
104 DISEASES OF THE NERVOUS SYSTEM.
this country, in Alabama and Arkansas. It has also been observed in epidemics in New York, Connecticut and Vermont. There are various types of this disease, in some of which the neuritic symptoms seem subordinate to those of other organs. The forms described by Scheube and Taylor are :
The acute pernicious, the acute or subacute benign, the atrophic or dry, and the dropsical or wet.
The symptoms generally resemble those of multiple neuritis, as already described, plus oedema, extensive serous effusions, serious heart weakness and gastro-intestinal disorders. I have observed a case of undoubted endemic neuritis (beri-beri) which ran precisely like an ordinary multiple neuritis. The paralysis affects especially the lower extremities, but in beri-beri there seems to be an especial tendency also to involvement of vasomotor and visceral nerves. Beri-beri occurs especially in persons who work bare-footed. It is thought to be an infection through trauma of the feet and due to a microbe. This specific microbe has not been definitely isolated. The view that the disease is due to poor diet, especially a fish and rice diet, seems disproved.
Malarial Multiple Neuritis. — Jamaica seems to be the only place in which the malarial poison produces an endemic neuritic paralysis (Strachan), and the causation in these cases is not yet demonstrated. Dr. Strachan's description of the symptoms of what he terms malarial peripheral neuritis shows it to be quite extensive, often involving trunk and cranial nerves, and accompanied by much pain and wast- ing. Cramps and skin eruptions are often noted, complications that do not occur in the ordinary types. There are sporadic forms of multiple neuritis occasionally seen in this country, but it is yet to be proved that the malarial Plasmodium can alone cause neuritis. It is more likely that "malarial" multiple neuritis is a form of beri-beri which is not rare in some of the islands of the West Indies.
Acute; Pernicious Mui/tipi^e Neuritis. — There is a form of mul- tiple neuritis which comes on suddenly, progresses rapidly, and causes death in a few days or weeks. These cases usually show the ordinary symptoms of neuritic paralysis, with final involvement of the cardiac and respiratory nerves, causing death. The agent in these cases is apparently of the nature of sepsis. The neuritis is interstitial and hemorrhagic. The disease is due to an infectious poison which over- whelms the system before it has time to set up any inflammation or organic change. In these cases the anterior-horn cells of the spinal cord are also involved and the disease is one that attacks the whole
GENERAL DISEASES OF THE PERIPHERAL NERVES. 105
peripheral motor neuron. It is thought that Landry's paralysis and severe forms of poliomyelitis are due to this same cause.
Pathology. — In multiple neuritis the disease affects the periphery of the nerves most, and extends up, very rarely reaching the roots. The anterior tibial and musculo-spiral nerves on the two sides are oftenest and most diseased. The process when mild in grade re- sembles a secondary degeneration following section of the nerve. In severer cases there is evidence of interstitial inflammation as well as degeneration (Fig. 45). This process, however, varies in degree at different points of the nerve's course. Hence it has been called
Fig. 45. — Diphtheritic neuritis, chiefly interstitial (Siemerling); with secondary degeneration.
segmental or disseminated neuritis. In some of these cases and in all acute pernicious cases there is still more interstitial inflammatory change; small hemorrhages occur, exudation takes place, and col- lections of leukocytes about the vessel walls and among the nerve- fibres are seen (Fig. 46). The muscles supplied by the diseased nerves undergo atrophy. This is usually simple and non-inflammatory. But sometimes there is an interstitial myositis with exudation compress- ing the fibres (Senator).
If the disease progresses, the nerve-fibres degenerate and their place is taken by connective tissue, and the same process occurs in the muscles.
DISEASES OF THE NERVOUS SYSTEM.
The spinal cord when examined by the help of Nissl and Marchi stains shows some involvemen;. The anterior-horn cells undergo the same degeneration as that which occurs when the nerve is cut across, and slight areas of degeneration are found in the posterior and lateral columns. The changes are very slight compared with those in the nerves, and in the writer's experience are secondary.
It will be seen, therefore, .ha. in multiple neuritis there may be: (i) Simple degeneration; (2) degeneration with some evidences of
Fig. 46. — Longitudinal section of a nerve in multiple neuritis, showing rich proliferation of nuclei (Leyden). The process here is inflammatory as well as degenerative.
interstitial neuritis (degenerative neuritis) ; (3) decided interstitial neuritis with degeneration of nerve-fibres.
It seems probable that multiple neuritis of the common motor type is due to some infection or toxin. As stated there is especial evidence for this in beri-beri, but no organism has been isolated.
Diagnosis. — Multiple neuritis must be diagnosticated from diffuse or transverse myelitis anterior poliomyelitis, locomotor ataxia, spinal meningitis, and hemorrhage and Landry's paralysis. Practically, diffuse myelitis is the disorder from which it has oftenest to be
GENERAL DISEASES OF THE PERIPHERAL NERVES. 107
distinguished. From this it is recognized, first, by investigating the cause and onset. Neuritis begins more slowly and with sensory prodromata; it affects the legs and feet, especially the extensors, and if it ascends it skips the hips and trunk and attacks the forearms. There is more muscular atrophy than in myelitis; the knee-jerks are absent. It progresses more slowly, and after four or eight weeks gradually regresses. Electrical degeneration reactions are more varied and decided. There are tenderness over the muscles and nerves and peculiar burning, darting pains. The cutaneous anaesthesia, if present, is not so extensive and complete, as a rule, while muscular anaesthesia is more decidedly marked. There is very rarely involvement of the sphincters nor are there bedsores. There may be belt-like constric- tions felt round the extremities, but not around the waist. The gradual improvement of the paralysis and atrophy and eventual re- covery confirm the diagnosis of neuritis. The presence of neuritis of the cranial nerves would strengthen the theory of a general neuritis.
From poliomyelitis it is distinguished by the presence of pain and other sensory symptoms, the early fall in galvanic irritability, the age of the patient and the etiology.
From locomotor ataxia neuritis is distinguished by its more rapid onset, the presence of paralysis and atrophy of muscles, paresis, with degeneration reactions, and the absence of involvement of the organic centres and pupils.
Spinal hemorrhage usually leads soon to a secondary diffuse mye- litis easily distinguishable from neurkis by the characters above given. Here there is also usually pain in the back. Spinal meningitis is associated with characteristic pain, tenderness and stiffness along the back. Acute ascending paralysis in its typical form shows but very slight sensory disorders, and no wasting or change in electrical irritability.
The complication of multiple neuritis and myelitis or posterior sclerosis is possible, but is very rare. In the former case the ordinary symptoms of myelitis are added to those of neuritis. In locomotor ataxia there is often some nerve degeneration and occasionally neu- ritis. The nerve degeneration probably causes only slow atrophic changes and paresis; the neuritis causes pains, anaesthesia, skin erup- tions and local trophic disorders.
Prognosis. — Alcoholic multiple neuritis is a serious disease, be- cause of its associated conditions. Nearly one-half of my hospital patients have died, mainly because they continued the use of alcohol after paralysis appeared. They do not die of neuritis, but of alco- holism, pneumonia or of phthisis. Other forms of neuritis rarely
108 DISEASES OF THE NERVOUS SYSTEM.
cause death. The great majority recover almost completely. It may be from six months to two years before all symptoms disappear. The average time is about a year.
Treatment. — The patient needs, first of all, rest in bed. The limbs are often extremely tender and the patient's pains excruciating. To relieve these the legs may be painted with menthol and enveloped in cotton batting. In other cases flannels wrung out in hot water and renewed every two hours give relief. Internally phenacetin, antipyrin or other coal-tar products may be given for the pains. Fluid extract of ergot in doses of 3i- to 5ii- repeated in three hours sometimes relieves pain. In the early stages, salicylate of soda in doses of gr. xx. every two or three hours is recommended. If there is a great deal of depression from alcoholic poisoning, strychnine, gr. q. 3 h., and aromatic spirits of ammonia, 3SS- °r 3 h«> should be used.
There is no drug which really cuts short the process. The best measures for this purpose are rest, thorough cleansing of the alimen- tary trace, abstinence from alcohol and a nourishing diet.
After the acute stage is passed the labile galvanic current occasion- ally interrupted may be applied, 5 to 10 ma. for ten minutes three times daily. Later, by the sixth week, the faradic current, massage and careful exercise can be given. At this time or earlier (third week), strychnine, iodide of potassium, arsenic in small doses and tonics may be given. In old cases in which a great deal of paralysis and contracture have occurred, forcible extension of the limbs, the use of splints and rubber muscles are needed. With patience and perseverance the worst cases can eventually be brought to a complete recovery.
Complicating Forms of Neuritis and Neuritic Degenera- tion.— Neuritis and neuritic degeneration complicate many diseases, but they especially mark and modify subacute and chronic rheu- matism, locomotor ataxia, diabetes, paralysis agitans, wasting dis- eases and old age.
A neuritic degeneration almost always affects the nerves in the neighborhood of an old rheumatic joint. The chief result of this is to produce wasting and some paresis of the muscles moving the joint (Pitres and Vaillard). The process is a reflex atrophy (see Arthritic Muscular Atrophy).
In locomotor ataxia parenchymatous nerve degeneration is very often present. It does not produce the cardinal symptoms of this disease. It does, however, cause some of the anaesthesia, pares- thesia, muscular atrophy, skin dystrophies, and visceral crises.
GENERAL DISEASES OF THE PERIPHERAL NERVES. 109
In diabetes the neuritis takes the form of the sensory type of mul- tiple neuritis, and causes symptoms like those of locomotor ataxia. The patient has "sciatic pains, burning or numb feet, loss of tendon reflex, ataxia. The upper extremities are rarely affected.
In Wasting Diseases and Old Age. — In various wasting diseases, such as phthisis, cancerous cachexia, long-continued fevers, marasmus, and in senility, a simple parenchymatous degeneration of nerves, with atrophy, occurs (Arthaud, Koster, Jappa). The symptoms caused by these changes are very slight. They contribute to the weakness and wasting. In old age, the atrophy of the nerves is one cause of the lessened sensibility and activity of the skin and its underlying muscles.
TUMORS OF NERVE.
These consist of :
1. Nerve hyperplasia.
2. True neuromata.
3. Fibro-neuromata.
4. False neuromata.
1. Hyperplasia or hypertrophy of nerve trunks is very rare. Generally the increase in size, is due to increase of the interstitial con- nective tissue. Sometimes, however, there is an increase in the number of fibres and thickening of the myelin sheath.
2. True neuromata are also very rare, and occur almost ex- clusively on spinal nerves. In some there is an increase in medul- lary fibres; in others only an increase of non-medullated fibres, i.e., only the axis-cylinders and neurilemma increase. They occur either singly or multiply. Multiple neuromata are generally of a neuro- fibromatous character. Neuro-fibromata may form growths known as fibroma molluscum.
True neuromata are usually small, ranging from i cm. (two-fifths of an inch) to 6 cm. in diameter. They may be much smaller or larger. They are usually few in number, but may be very numerous. Gowers estimates in one case that as many as one thousand were present. Even larger numbers have been observed. They rarely cause serious symptoms, but may produce local pains and parasthesiae.
3. Multiple false neuromata when numerous constitute a condi- tion known as Neurofibromatosis.
This is a rare condition, but one of importance because sometimes these tumors develop on cranial nerves, gradually increase in size and become practically brain tumors. The following description is from an article upon this subject by Fraenkel and Hunt.
no
DISEASES OF THE NERVOUS SYSTEM.
The tumors occur in varying size from that of a bead to that of a child's head. In some cases the manifestation of the disease is expressed by a solitary growth upon one nerve, and in other cases almost innumerable growths have been present. They occur on all cerebrospinal and sympathetic nerves except on the optic and the olfactory. The acoustic nerve, has been shown to be particularly liable to the formation of fibromata and fibrosarcomata. The origin of these tumors is usually from the endoneurium, rarely from the perineurium and exceptionally from the epineurium. They are situ- ated either centrally within the nerve or eccentrically on the periph- ery of the nerve. The general conformation of the tumors is spherical or elliptical and in generalized cases the nodular out- growths give the nerve a convoluted appearance, sometimes resembling a rosary.
Histologically, the fibroma nervorum is made up of connective tissue elements, which present all the variations in structure between the fibroma molle and the fibroma durum.
The neurofibromata are usually slowly growing tumors of a benign nature. Sometimes, however, they undergo a retrograde metamor- phosis and show cystic, fatty or myxomatous degeneration or they undergo sarcomatous transformation, assuming a malignant character.
Neurofibromatosis depends often on a hereditary or congenital dis- position. It is frequently associated with more or less mental impair- ment, imbecility, and somatic stigmata of degeneracy. Scrofulosis and tuberculosis are often found in the histories of the cases.
Neurofibromatosis may present itself clinically in one of the fol- lowing ways: (i) Tubercula dolorosa (painful tubercles, Wood); (2) multiple neurofibromata of the skin (molluscum fibrosum, Reckling- hausen's disease) ; (3) neuromata or neurofibromata of a single peripheral nerve; (4) neuroma plexiforme, pachydermatocele, and elephantiasis neuromatodes ; (5) generalized neurofibromatosis; neu- rofibromata of the skin, cerebrospinal and sympathetic nerves.
1. The tubercula dolorosa are characterized by the appearance of small, very painful cutaneous nodules. They rise apparently from the sensory skin filaments and may occasion paresthesias, con- tractures, reflex spasms, and, according to good authority, even epileptiform seizures.
2. The multiple neurofibroma of the skin is a dermatologically well- known condition. The disease is apparently congenital, occurring mostly in dwarfish, undersized people of somewhat impaired intel- lectual capacity. It is characterized clinically by numerous (several hundred) cutaneous nodules of all sizes (from a pin head to a goose
GENERAL DISEASES OF THE PERIPHERAL NERVES. Ill
Fig. 47. — Neurofibromatosis. (Fraenkel and Hunt."
112
DISEASES OF THE NERVOUS SYSTEM.
egg). These tumors are sessile and usually not painful. They give rise to no symptoms when existing on the skin only.
3. Neurofibromata of individual peripheral nerves (isolated neu- romata) are surgically well known. They express themselves clinically by paresthesias and progressive motor weakness of the peripheral nerve type, and are of good prognosis when recognized. They occur most frequently on the median and sciatic nerves; they are rarely without symptoms and are prone to give rise to considerable hyperesthesias and little paralysis.
4. Neuroma plexiforme, pachydermatocele and neuromatous ele- phantiasis are clinical curiosities, and are more of dermatological and surgical than of neurological interest.
5. The generalized neurofibromatosis of the skin and cerebrospinal and sympathetic nerves is apparently the rarest of all enumerated types and the basis of exceptional and unique clinical pictures.
All of the above-mentioned types are expressions of the same patho- logical tendency. Atypical and unusual syndromes are occasionally found explained by multiple neurofibromatosis : attacks of neuralgia paresthesias, reflex spasms, contractures, mono-, para-, quadruplegias.
The diagnosis is obviously a very easy one, and has to be dif- ferentiated only from multiple lipomatosis of the skin, the so-called molluscum pendulum.
4. False Neuromata. — This term is applied to the various nerve tumors in which a fibroma, myxoma, glioma, sarcoma, carcinoma or syphiloma grows upon or in the nerve. The fibroneuroma as above described is really a false neuroma and is the common form; glioneuroma has been observed on the auditory nerve. Syphiloma occurs only on the intracranial or intraspinal nerves. Carcinoma of nerves may be primary, but is generally secondary, and is of scirrhous or medullary type, rarely the colloid. Leprous neuritis sometimes forms neurofibromatous swellings. A few cases, have been observed of multiple malignant neuromata. Trauma and hereditary influence are the etiological factors. The great nerve trunks are oftenest affected, the median; sciatic and crural ranking first. The tumors start from the perineurium; they are at first spindle-shaped, and may grow very large. Sarcomatous cells are oftenest found in them; but they may be myxomatous, fibromatous or mixed.
Etiology. — Three general causes exist for the production of neu- romata, viz. : 1 . A hereditary or a neuropathic predisposition, this tends to cause the true, the multiple, and the plexiform neuromata. 2. Injuries, surgical operations; these cause especially the fibroneuromata of which the amputation neuroma is an example. 3. Diathetic, e.g.,
GENERAL DISEASES OF THE PERIPHERAL NERVES.
■tuberculous influences, and whatever produces the various tumor formations, sarcoma, carcinoma, form the third etiological factor. Neuromata of the plexiform type are often congenital. Multiple neuromata may develop early in life. Men are far more subject to multiple neuromata than women.
Symptoms. — Neuromata often cause no symptoms. Perhaps the most frequent evidence of their presence, however, is pain and some :enderness. The pain is exacerbating, and may be stopped scme- zimes by pressure on the nerve above the tumor. Paresthesia, anaesthesia, paralysis, and reflex spasm may be present. Some forms }f intractable headache are possibly due to multiple neuromata. K neuroma upon one of the cranial nerves, usually the acoustic, may jiievelop into and cause the symptoms of a brain tumor. Hence the Dresence of neurofibromata of the skin with brain symptoms should ilways suggest the possible presence of an intracranial growth. [Multiple and plexiform neuromata cause symptoms less often than a jingle larger neuroma. A neuroma on the pneumogastric or other splanchnic nerve may cause severe symptoms. Multiple (true) neu- romata may last for years and cause no serious inconvenience. Malignant neuromata cause always such symptoms as would naturally follow irritation and compression of a nerve.
The treatment of neurofibromata and of nerve tumors generally is lsually purely surgical when any treatment is indicated.
8
CHAPTER VIII.
MOTOR DISORDERS OF SPECIAL NERVES.
The general distribution of the paralyses from injury or disease of the different motor nerves is shown in the accompanying table of cases. I am indebted to my friends Dr. Wm. P. Wilkin and Dr. Alex- ander S. Leverty, for the larger part of the work of compilation:
Cranial-nerve paralyses (including 19 occuring in locomotor ataxia. . 206
Spinal -nerve paralyses 377
Multiple neuritis 114
697
Of the cranial nerves:
Ocular (third, fourth, sixth) 52
Fifth 3
Seventh 136
Tenth and eleventh 4
Of the spinal nerves:
Arm 194
Thigh and leg 24
Of the arm palsies:
Total arm palsies 10
Musculo-spiral 112
Ulnar 31
Circumflex 24
Median 15
Cranial nerve palsies are twice as frequent in men as in women and arm palsies, four times more frequent in men.
The brachial plexus and its branches are oftenest affected by paralysis, next the seventh cranial nerve, then the ocular nerves, and last the lumbar and sacral plexus. This of course represents the frequency in a neurologist's experience. In general practice and especially in surgical practice there would be fewer cases of cranial- nerve disease and more of lumbar and sacral palsies.
The Ocular Muscles.
Anatomy. — It will help the student in learning the diseases of the cranial nerves if their points of origin and general relation are shown, as in the accompanying illustration (Fig. 48).
The motor nerves of the eye are :
(a) The third or oculo-motorius, supplying the internal, superior and inferior recti, inferior obliquus, the levator palpebral, the ciliary muscle, and constrictor of the iris.
(b) The fourth or trochlearis supplying the superior oblique.
(c) The sixth or abducens, supplying the external rectus.
114
MOTOR DISORDERS OF SPECIAL NERVES.
(d) The sympathetic, consisting of fibres from the cervical cord to the dilators of the iris, to its blood-vessels, and to Miiller's muscle.
Motor fibres from the nucleus belonging to the third nerve run out with the fibres of the seventh (Mendel), supplying the orbicularis palpebrarum.
Fig. 48. — Showing the apparent origin of the cranial nerves.
The third and fourth nerves arise from a series of nuclei in the floor of the equeduct of Sylvius. They leave the brain at the anterior edge of the pons. They run in the cavernous sinus and enter the orbit through the sphenoidal fissure.
The sixth nerves arises from a nucleus in the floor of the fourth ventricle. It emerges at the posterior edge of the pons, runs in the cavernous sinus, and enters the orbit through the anterior lacerated foramen.
The nuclear gray matter from which these nerves arise is made up of a series of nests of cells and each pair supplies a different set of muc- sles of the eye, as shown in the diagrams (Figs. 51 and 52).
n6
DISEASES OF THE NERVOUS SYSTEM.
The nucleus of the sixth lies farther back in the floor of the medulla, but it belongs to the same serial deposit of gray matter and represents the continuation of the anterior horn of the spinal cord (Fig. 51).
The motor nerves of the eye, third, fourth and sixth, are closely connected with each other and other nerves by a long commissure, the posterior longitudinal bundle.
The fibres of the third and sixth nerves pass to their nuclei on the same side, then decussate and pass up in the inner part of the crusta to the frontal part of the central convolutions of the cortex. A few
Fig. 49. — Showing the course of the third and sixth cranial nerves.
fibres decussate and enter the nuclei of the opposite side. They are connected with the internal rectus nucleus.
The fibres of the fourth nerve almost entirely decussate, running forward a long distance before they finally reach their nuclei. Thus it appears that the fourth is the only cranial nerve except the optic which largely decussates before reaching its nucleus. However, those fibres of the third which supply the internal rectus also decussate, as already stated.
The arrangement of the nuclei is believed to be as follows, the upper on the list being anterior:
Median line, f Sphincter iridis, Ciliarius. Ill N I levator P&lP-> Rect- int- ' j Rectus superior, Rect. inf. [ Obliquus inferior.
IV. N. j Obliquus superior. VI. N. < Rectus externus.
Near the sphincter of the iris there is perhaps a dilator center also.
MOTOR DISORDERS OF SPECIAL NERVES.
117
In order to understand the peculiarities of eye palsies, to be de- scribed later, the relations of the sixth to that nucleus of the third nerve which innervates the internal rectus must be understood. In turning the eyes to one side, these two nuclei and their nerves act together, causing the external rectus of one eye and the internal rectus of the other to contract at the same time. The impulse from the brain which does this decussates and acts first upon the sixth, and through this upon the external-rectus nucleus of the same side. The impulse from this nucleus then goes to the third-nerve fibres of the same side and thence to the internal rectus. This can be better undersood by the diagram, Fig. 52.
Thus lesions in the brain at (a) cause paralysis of the sixth nerve of the opposite side and internal rectus of the same side. The eyes turn toward the side of the lesion.
Lesions in the pons at (b) cause paralysis of the sixth on the same side and internal-rectus nucleus of the opposite side. The eyes turn away from the side of the lesion.
The movements of the eyeball are made by the simultaneous action of several muscles. Most of them act as their names indi- cate. But the oblique muscles help to depress or elevate, and then help to rotate in or out according as the internal or external rectus is acting.
The cortical centres for the eye muscles are not positively known. Lesions in the inferior parietal lobule sometimes cause disorder of the
n8
DISEASES OF THE NERVOUS SYSTEM.
third nerve, but the dominant centres are in the prefrontal lobe at the base of the upper and middle frontal convolutions.
As the optic nerve is the special sensory nerve of the eye, so the third, fourth, sixth and part of the seventh nerves are the motor nerves. By means of the optic nerve and its receptive and refrac- tive apparatus, the form, color, movement and, to some extent, relations and distance of objects are determined. The motor nerves adjust the eye to near and distant objects, inform us as to size and distance, and enable us to follow moving objects and to shift the gaze readily. They also assist in protecting the eye against injury.
Frc 51 — Diagram showing the arrangement of the nuclei of the motor nerves of the eye, and the decussations of the fourth and internal rectus branch of the third nerve.
Fig. 52. — Diagram showing the probable relations of the nuclei of the sixth and of the internal rectus branch of the third to the brain. P. L. B., Posterior longitudinal bundle.
Genkrae Symptoms. — It is impossible always to disassociate dis- eases of the oculo-motor nerves from those involving their nuclei. Hence we must study here really the affections of the whole neu- rons. These are:
(1) Paralyses or ophthalmoplegias, which may be acute, chronic, or progressive; (2) pareses or myasthenic states, called ordinarily muscular asthenopias; (3) spasms, such as strabismus, nystagmus, and blepharospasm ; (4) reflex disorders.
There are many special terms which are used to indicate the peculiar effects of various paralyses and spasms of the ocular mus- cles and nerves, and some of these I will define here:
E.R.
Fig. 51.
Fig. 52.
MOTOR DISORDERS OF SPECIAL NERVES.
II9
Erroneous projection is a condition in which the patient is unable to judge exactly of the relation of external objects to the body ; for this relation is determined by the movements of the ocular muscles, and, these being weak, wrong sensations are conveyed to the brain. Vertigo may result from this disturbance of muscular sensation.
Diplopia or double vision is a condition due to the erroneous sen- sation resulting from eye-muscle palsy, and to the fact that the images of the object fall upon non-corresponding retinal fields. Diplopia is simple or homonymous when the false image is seen on the same side as the affected eye. When a red glass is placed over this eye two images are seen, the red one being on the side of the eye involved. Diplopia is heteronymous or crossed when the false image is on the side opposite to the sound eye.
Conjugate deviation of the eyes is a condition in which both eyes turn strongly to one or the other side. It may be paralytic or spas- modic. The mechanism is a complicated one and not perfectly understood. In general, destructive lesions of the brain cause a paralytic deviation toward the side of the lesion, and irritative or compressing lesions the opposite effect. Destructive lesions in the pons cause a deviation away from the side of the lesions. The palsy then involves the sixth cranial nerve and the branch to the internal rectus from the third. The sixth-nerve nucleus is the dominant one, and impulses from the brain go to it first (see Fig. 52).
In diseases of the motor nerves of the eye it is found that the paralyses occur in various ways, which may be best grouped as follows:
Ophthalmoplegias.
I. Paralysis of the Oculo-motorius or Third Nerve — Etiol- ogy.— The commonest causes are exposure to cold which means some simple infection, and syphilis. Other causes are basal menin- gitis, intracranial tumors, injuries, compression from orbital tumors, the diphtheritic poison; excessive use of tobacco and alcohol, mor- phine, or other poisons may be predisposing causes. Temporary palsy sometimes occurs in migraine or it may take the place of an attack of migraine. Partial palsies also occur in locomotor ataxia and in certain primary muscular atrophies and in myasthenia gravis.
The Ophthalmoplegias.
120 DISEASES OF THE NERVOUS SYSTEM.
The common causes, however, are, as stated, rheumatic influences and syphilis.
There occurs, in rare cases, an acute inflammatory degeneration of the nuclei of the ocular-muscle nerves similar to acute anterior poliomyelitis. This condition has been called "polioencephalitis superior" or upper bulbar palsy.*
Symptoms. — When all the muscles supplied by the third nerve are paralyzed, there is dropping of the lid (ptosis, Fig. 53) ; the eye can be moved only outward and downward and inward ; there is, therefore, divergent strabismus and double vision (diplopia). The pupil is somewhat dilated and does not contract to light, owing to paralysis of the constrictors of the iris; and there is loss of power of accommo- dation, so that the patient cannot read print close to him.
The patient suffers much annoyance from the lid-drop and the double vision, and there are sometimes vertigo and photophobia. Only one nerve is involved at a time, as a rule. The various eye muscles supplied by the third are FIG> 53— Double ptosis, rarely all attacked. The levator may escape almost entirely; the ciliary muscle and iris may also be but slightly involved; but these latter muscles are never involved alone in ordinary types of the disease.
The affection usually runs a subacute course, lasting but a few weeks. Functional palsies last but a few days; syphilitic palsies are usually temporary (one to three weeks), but may relapse or become extremely obstinate. Periodical palsies occur every year or six months or even oftener; they last a few days or weeks and are accom- panied at first by some pain. They continue to recur for years. They may be associated with attacks of migraine and are migrainous phenomena.
In diphtheritic eye palsies the first three or four nuclei of the series making up the origin of the third nerve are oftenest affected, causing paralysis of accommodation, paralysis of the iris and of the internal rectus, the three muscles concerned in accommodating the eye to near objects.
Pathology. — In the rheumatic palsies there is a low grade of per- ipheral neuritis, and the same is true of most diphtheritic and other
* The whole group of acute epidemic and endemic inflammations including anterior poliomyelitis, acute bulbar palsy, acute ophthalmoplegia, acute encephalitis, Landry's acute ascending paralysis have been classed under the name "The Heine-Medin Disease."
MOTOR DISORDERS OF SPECIAL NERVES.
121
palsies of infectious origin. In syphilitic and tabetic palsies there is usually a specific basilar meningitis involving the nerve-roots. The meningitis may be slight or may amount to gummatous deposit. In functional and some periodical palsies there is a vasomotor dis- turbance causing congestion or anaemia or perhaps simply inhibition of the nuclear centres. Some periodical palsies have been found to be due to small tumors involving the nerve-root. In rare cases there is primary muscular atrophy of the eyeball nerves or primary degeneration of the nuclear centres or a cerebral lesion. The nuclear inflammation forming " polio-encephalitis superior " is a disease prob- ably infectious and quite similar to anterior poliomyelitis.
Diagnosis. — One must first determine how extensively the muscles supplied by the third are involved.
If only the levator palpebrae, there is simply falling of the lid. If the eyeball muscles are involved, we get the following symptoms. They are :
Limitation of movement of the globe.
Strabismus and secondary deviation.
Erroneous projection.
Double vision or diplopia, which is either simple or crossed. Paralysis of the iris or iridoplegia and of the ciliary muscle or cycloplegia.
Concentric limitation of the visual field.
All these points must be tested, but the detailed knowledge of them is best gained by consulting ophthalmological works.
The extent of involvement of eye muscles can generally be suf- ficiently tested by making the patient move the affected eye in various directions, and by testing for accommodation and for the pupillary reaction to light.
Paralysis of thk iris, or iridoplegia, and of the ciliary muscle — cycloplegia.
The motor fibres of the third nerve to the iris supply the sphincter and when paralyzed there is immobility of the pupil.
Iridoplegia, or paralysis of the iris may occur leaving the pupil small and contracted (myosis) or large and dilated (mydriasis). There is a loss of light reflex. If the light reflex is gone but accommo- dation reflex persists, we have the so-called Argyll-Robertson pupil.
There are probably centres in the third-nerve nucleus both for sphincter dilatation, and sphincter contraction. There is a spinal centre for pupil dilation in the eighth cervical and first dorsal segments. The fibres from this pass up through the first dorsal nerve, then through the cervical sympathatic and the carotid plexus to the eye.
122
DISEASES OF THE NERVOUS SYSTEM.
This spinal centre is not, however, the one commonly involved in the "stiff pupil;" i. e., the pupil with loss of light reflex. The lesion is probably one involving the third-nerve nucleus and the fibres passing from it to the anterior quadrigeminus (Meynert's bundle). In other words, Argyll-Robertson pupil is probably usually due to a nuclear lesion of the 3d nerve or fibres going to it.*
Iridoplegia without cycloplegia is most significantly present in parasyphilitic conditions. Iridoplegia may also occur in infections like diphtheria and from toxic states and local disease.
Cycloplegia, or paralysis of accommodation, occurs as part of paralysis of the third nerve. It also may occur in syphilis, tabes, paresis and in diphtheria. It sometimes occurs, as a temporary symptom in migraine.
Paralysis of Convergence. — The internal recti muscles may be quite normal except that they cannot converge the eye. When this is the case there is also loss of the associated pupil contraction. This form of paralysis sometimes occurs. It is inferred that there are two separate centres for contracting the pupil. One associated with the light reflex and one with convergent movement (Turner).
Paralysis of the levator palpebrcE, causing ptosis, is sometimes seen alone, but usually other branches of the third nerve are involved. A functional palsy of the lids sometimes occurs in anaemic and nervous people at the time of waking. It is a temporary morning or waking ptosis.
Paralysis of the sympathetic fibres of the eye causes con- traction of the pupil (myosis) from the unopposed action of the third nerve. There is also a slight retraction of the eye-ball, narrowing of the palpebral fissure, sweating of the face on the affected side and lessened intraocular tension. The pupil does not dilate when the skin of the cheek or neck is irritated, a condition known as loss of oculo-skin reflex. The spinal centre as stated is in the first dorsal and eighth cervical segment.
II. Paralysis of the Fourth Nerve. — This is a rare affection and not always easily detected. The causes are much the same as those of palsy of the third nerve. The symptoms are slight conver- gent strabismus when the eye is moved downward and diplopia on looking down. There is defect in the movements of the eye down- ward and outward.
Paralysis of the sixth nerve (abducens) is the most frequent
^However, Reichert claims to find degeneration in the endogenous fibres of the posterior column of the upper cervical cord.
MOTOR DISORDERS OF SPECIAL NERVES.
I23
of eye palsies, and occurs especially often in syphilis and in locomotor ataxia. It causes convergent strabismus and double vision.
III. Progressive Ophthalmoplegia {Progressive Upper Bulbar Palsy). — Besides the palsies already described, there occur certain forms which have a peculiar origin and course. They begin slowly, as a rule, and steadily progress. In some cases only do they reach a certain stage and then remain chronic. The term " progressive " applies fairly well to them. They often affect the third, fourth and sixth nerves together. In accordance with the muscles invaded, these palsies are called external, internal, partial and total. Thus if those branches of the third nerve supplying the iris and ciliary muscle are involved alone, it is ophthalmoplegia interna ; if the other branches are involved, it is called ophthalmoplegia externa.
Definition. — Progressive ophthalmoplegia is a degenerative dis- ease of the nuclei of the motor nerves of the eye. It is in most cases the same disorder as of bulbar paralysis and progressive muscular atrophy.
Etiology. — It develops between the ages of fifteen and forty, but may occur later. The sexes are equally affected. Lead, diphtheria, traumatism, syphilis, appear sometimes to be the cause. It may complicate locomotor ataxia ; more often it forms part of progressive muscular atrophy.
The symptoms are often not noticed until the disease is well advanced. The vision is not disordered, and there is only a gradual limitation of mobility of the eyeball. A slight drooping of the lids, causing a sleepy look, or a slight squint, usually divergent, is noticed. Then upon examination it is found that the eyes are immovable and cannot follow the finger, except to a slight extent. The peculiar physiognomy which results is known as the "Hutchinson face" (see Fig. 54). The iris reacts to accommodation and light usually. Double vision may be present. Usually the patient accustoms himself to monocular vision. The disease lasts a longtime, and it may become stationary. If complicated with progressive muscular atrophy, however, the course is relatively rapid, death occurring from the latter disease in two or three years.
Pathological Anatomy. — In all progressive cases there is a degen- erative atrophy of the nuclear cells. In a few stationary cases the anatomical change is that of neuritis.
The treatment is that for the disease which it complicates or the condition which causes it. That is to say, it is the treatment for loco- motor ataxia, progressive muscular atrophy, syphilis or lead poisoning. Iodide of potassium, strychnine, arsenic, nitrate of silver, and phos-
124
DISEASES OF THE NERVOUS SYSTEM.
phorus may be given. Electricity is of very doubtful value, and only the galvanic current would be indicated. General tonic measures and rest to the eyes should be employed.
Muscular Asthenopia and Muscular Insufficiencies. — This is a term employed to indicate a lack of equilibrium Gf the muscles of the eye, as a result of which the visual axes cannot be kept parallel without an effort. This effort is often unconscious, and shows itself only by a ready tiring of the eye on attempting to read or by the production of headaches and cerebral paresthesias. Examination of
F]G. 54. — Drooping lids; immobile eye. "The Hutchinson face."
the eye by means of prisms reveals the special character of the trouble. The term is usually applied to functional, congenital and neuras- thenic weaknesses, not to those due to organic disease or severe toxaemias.
When the eye muscles act normally, the condition is called one of orthophoria. When some of the muscles are weak it is called hetero- phoria. There are various forms of heterophoria, viz. : esophoria, a tending of the visual lines inward, from weakness of the externi; exophoria, a tending of visual lines outward ; hyperphobia, a tending of the visual line of one eye above its fellow.
The condition is tested in various ways. The simplest is this: Refractive errors having been corrected, a series of prisms is placed
MOTOR DISORDERS OF SPECIAL NERVES. 1 25
over the eye, at first with the base inward, while the patient looks at a candle twenty feet distant. The prisms are increased in strength until the patient can no longer coalesce the images. The degree of prism is noted, and this indicates the strength of abduction or of the externi. The same process is gene through with fcr the interni, the the base of the prism being out. The externi should overcome a prism of about 8°, the interni one of 230 to 250 or more. There are great individual variations, and there is also considerable variation in individuals.
The above tests measure the amount of abduction and adduction.
To test the presence of heterophoria, the writer uses the Maddox double prism held in a frame. The line where the bases of the two prisms unite is brought directly over one eye, and is held there in a perfectly horizontal position while the patient looks at a candle twenty feet away. A red glass is at the same time held over the other eye. With the eye covered by the double prism the patient sees double, one flame being above the other; with the other eye he sees a red flame lying just between the two white ones. If the red flame is directly in a vertical line, there is orthophoria; but if it lies to one side or the other there is exophoria or esophoria according as the red flame was on the side opposite to the eye covered with red glass or on the same side. If heterophoria is found, prisms are placed over the eye until the three lights are in a vertical line. The number of the prism required to correct the heterophoria indicates itsextent in degrees. By changing the double prism so that its common base line is vertical, the test for hyperphoria can be made.*
Muscular asthenopia is said to cause a disturbance of vision, vertigo migraine, cerebral paresthesia, and pains in the head, more parti- cularly in the occipital and cervical region. It is believed to be a possible factor in producing choreic twitchings in the face. In neurasthenic persons it may cause a wider range of nervous symptoms. It is said to be an essential factor in causing epilepsy, chorea, and hys- teria. The author cannot accept this latter view, and believes that the importance of muscular asthenopia in causing general nervous symptoms is not great. Much of it, if not all, may be relieved by correcting refractive errors and by helping the general health of the patient.
The treatment of it, after all myopia, or hypermetropia, or as- tigmatism, if present, is relieved, consists in building up the gen- eral health, the systematic use of prisms for training the muscles,
* More elaborate and exact methods have been devised by Dr. Stevens and other ophthalmologists.
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DISEASES OF THE NERVOUS SYSTEM.
the wearing of proper glasses. Some advise graduated or complete tenotomies.
Spasmodic Diseases of the Ocuear MusceES.
These are: (i) Conjugate deviation from spasm; (2) irregular and associated spasms from convulsive and irritative brain disorder; (3) nystagmus.
Spasmodic conjugate deviation occurs from an irritating lesion of the ocular nuclei or of the brain in its cortical motor areas and tracts. Irregular spasmodic movements occur in meningitis, hydrocephalus, and in lesions involving the semicircular canals. Peculiar associated spasms occur in hysterical attacks. Various spasmodic movements and contractions of individual eye muscles occur from ocular disease, errors of refraction, muscular weakness, and paralysis of certain eye muscles.
Rhythmical spasm or nystagmus occurs as the result of hereditary visual weaknesses and refractive errors of various kinds, in albinoes, and in chronic hydrocephalus. It is found usually in neurotic cases associated with ocular defects, in multiple sclerosis, and sometimes in epilepsy, chorea, hysteria, neurasthenia, and insanity. It occurs also in certain nervous disorders, such as hereditary ataxia, tumors, especially of the cerebellum, and other focal lesions, and in meningitis. It occurs in miners, and is called miner's nystagmus. It may be a reflex symptom from a remote irritation.
In nystagmus the oscillation of the eyeballs is usually lateral. It may be brought out when slight in degree by causing the patient to look steadily to one side. Vertical and a kind of rotating nystag- mus sometimes occur, and are due to much the same causes as those of lateral nystagmus.
Spasm of the levator palpebrce is sometimes seen and is usually tonic.
The above troubles are usually symptomatic, and their treatment depends upon correction of some local disease or cerebral neurosis.
The Motor Branch of the Fifth Craniae Nerve.
The anatomy of this nerve is described under the head of the neu- ralgias of the trigeminus.
The diseases of the motor branch of the trigeminus are rare, and generally symptomatic of some more general disorder.
Trismus (eockjaw) is the only important independent affection of this motor nerve. It is a tonic spasm of the muscles of mastication.
Etiology. — It occurs in infants, usually through infection from
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the umbilicus. It is then known as trismus nascentium. It forms part of the symptoms of tetanus and rabies. It may be sympto- matic of brain disease, and forms one of the manifestations of the epileptic fit. There may be a reflex trismus from irritations of the teeth and jaw and from gastro-intestinal trouble. Neuritis and hys- teria are causes of trismus and it may be associated with a trigeminal neuralgia.
Symptoms. — There is, as shown above, a symptomatic, an in- fectious, a reflex, a neuritic, and a hysterical trismus. In all, the symptoms are very manifest. The jaws are firmly locked and the
Fig. 55. — Showing position of cranial nuclei in the medulla.
masseters and temporals stand out. If the disease is unilateral, which is rarely the case, the lower jaw is pushed over toward the sound side.
The treatment of both tonic and clonic spasms depends upon the cause. Symptomatically, morphine is to be given, and later the anti- spasmodics, such as the* bromides and chloral. In rheumatic cases, hot applications and diaphoretics are indicated.
The: Facial Nerve.
Anatomy. — The facial nerve has its primary origin in a single nu- cleus deeply situated in the lower part of the pons (Fig. 55). It belongs to the same series of nuclei as the vagus, glosso-pharyngeal, and of of the spinal accessory; in other words, it is a prolongation of the lateral
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DISEASES OF THE NERVOUS SYSTEM.
horn of the spinal cord. It has not a nucleus common to it with the sixth, as is sometimes stated. Those fibres of the nerve which go to the orbicularis, however, appear to come from a nucleus in the third- nerve series and to reach the knee of the facial by the posterior longi- tudinal bundle (Mendel). The deep fibres of the facial take a tortuous course, passing inward, dorsally, then curving down and out around the nucleus of the sixth nerve (Fig. 56). The cortical origin of the seventh is in the lower part of the prsecentral convolu- tion. The fibres pass down through the knee of the internal capsule and enter the crusta at the inner side of the pyramidal or motor tract. They decussate and reach the nucleus. The nerve has its exit at the posterior edge of the pons, external to the sixth nerve. It then has to take a long course through a bony canal, during which it receives taste fibres from the second or third branch of the trigeminus (or the glosso-pharyngeal). These fibres leave the nerve in the chorda
Fig. 56. — Longitudinal section, showing position of the nuclei of medulla. (Edinger.)
tympani, and join the lingual branch of the fifth nerve to supply taste to the anterior two-thirds of the tongue. The facial nerve has also a sensory part, the fibres of which arise from the geniculate ganglion and pass in the nerve of Wrisberg; these fibres give sensation to a small area in the concha and the posterior wall of the external meatus. (J. Ramsay Hunt.)
The facial nerve supplies motion to all the muscles of the face; to the stapedius, stylo-hyoid, buccinator, and platysma myoid. It also contains trophic and secretory fibres. It does not supply, how- ever, the muscles of mastication.
The taste fibres of the facial nerve come in most cases from the second branch of the fifth via Meckel's ganglion, the large superior petrosal nerve, and geniculate ganglion. In other cases they come from the glosso-pharyngeal nerve via the ganglion petrosum, Jacob- son's nerve, tympanic plexus and geniculate ganglion (Fig. 57). Some think that the intermediary nerve of Wrisberg, which arises in the upper part of the glosso-pharyngeal nucleus and connects with the geniculate ganglion, carries taste fibres to the facial, but it has been shown by Hunt that these are mostly sensory (cutaneous) fibres.
MOTOR DISORDERS OF SPECIAL NERVES.
I29
The facial nerve being mainly motor, its disease are spasmodic and paralytic. The two common types are facial tic and facial palsy, but there are other minor forms.
The spasmodic disorders are: (1) diffuse facial spasm or mimic tic and (2) spasm of single branches, including (a) blephaospasm and (b) nictitating spasm.
Facial Spasm (Mimic Tic).' — -This is a disease characterized by intermittent, involuntary twitchings of the facial muscles. It is always chronic and generally unilateral.
Fig. 57. — Showing the usual course of the taste fibres in the seventh and fifth
nerves. (Krause.)
Etiology. — It is a disease of middle and later life, and occurs oftener in women; there is usually a neuropathic constitution; it is not hereditary. The exciting causes are anxiety, shock, injury, and ex- posures. It often has a reflex cause, usually from irritation of some branch of the trigeminus, the cervico-brachial nerves. It is sometimes associated with tic douloureux. It may have an origin in reflex irritation of the sensory part of the seventh nerve, viz., the geniculate ganglion and nerve of Wrisberg. Organic diseases, such as tumors and softening, affecting the nerve nucleus in the pons or the cerebral centres, cause a symptomatic tic, but not the true dis- ease. Thus we may have a post-hemiplegic tic or a tic due to cortical lesion and associated with epilepsy. A facial tic may also be caused
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DISEASES OF THE NERVOUS SYSTEM.
by irritation or disease of the nerve trunk or of its nucleus. It sometimes though rarely follows a facial palsy.
Symptoms.- — The disease usually begins slowly and the orbicularis muscle and zygomatici are earliest affected. It rarely goes above }he eyebrows; i.e., to the corrugator supercilii and frontalis. The lower branch of the facial is little affected. The spasm is at first a clonic one ; the muscles of the face are affected by a series of light- ning-like twitches, with intervals of rest. Sometimes, however, the contraction becomes tonic and lasts several seconds or more. There is no pain. The spasm is increased by emotions, nervous excite- ment, conversation, exposure to light and cold, and is at its worst when the patient himself is most depressed. It is a very good gauge of the general nervous stability of the patient. There is no paralysis or atrophy and there are no secretory cr trcphic symptoms The taste fibres are rarely involved, though occasional subjective sensations of taste have been felt. The electrical irritability is either unchanged or slightly increased.
Associated movements of the eyes, of the jaw muscles and cheek muscles are sometimes seen. Pressure over the motor points of the nerve will sometimes arrest the movements for a time. The disease is a unilateral one. It lasts for years and even for the lifetime.
Pathology. — There is no known anatomical change in idio-pathic cases. The disease is allied in character to wryneck and other chronic tics. In these cases it is probably the expression of some functional deterioration of the higher reflex centres. The disease is sometimes a pure reflex neurosis from ocular or dental or other irritations.
Diagnosis. — Idiopathic facial spasm is chronic, unilateral, un- accompanied by pain or paralysis. It is distinguished from facial spasms of organic origin by the fact that the latter always have some other symptoms. Thus facial habit chorea is bilateral; spasm from cortical disease is attended by disturbance of consciousness and comes on in paroxysms ; the spasm occurring after hemiplegia is usu- ally tonic, and so is hysterical facial spasm.
Prognosis. — The disease is in most cases incurable, especially after it has lasted some time. If a reflex cause exist, the prognosis is better. Life is, however, never endangered by it.
Treatment. — The most important thing is attention to the general health, removal of all depressing influences, rest, and freedom from excitement. Among specific remedies arsenic, the bromides, can- nabis indica, gelsemium, conium, hyoscyamus, strychnine, codeine and morphine are recommended. Morphine is useful, but must be tried
MOTOR DISORDERS OF SPECIAL NERVES. 131
carefully and in small doses. Conium lessens the spasm, but this drug has to be given in large doses and is not entirely free from danger. Hyoscine and gelsemium sometimes do good. Careful examination of the teeth, eyes, nose, stomach and uterus for reflex irritation is imperative.
Galvanism, if carefully and persistently applied, almost always helps. It should be given daily. Various methods are described. The best way is to place the negative pole on the sternum or back of t he neck, and the positive pole over each motor point of the nerve for one to two minutes, then over the occiput and over the facial cor. tical area for the same time. Currents of from two to five millia m- peres should be used. Neurectomy of the supra-orbital, continuous pressure on the motor points, stretching the nerve itself, are all measures which may be considered valueless. Freezing the skin over the nerve with chloride of methyl has been recommended by Mitchell. Blistering and cauterization are needless inflictions. The anestheti- zation of the conjunctiva with cocaine is often helpful, both in diagnosis and treatment. Systematic training exercises in which the patient sits before a mirror and tries to hold the spasm in control and systematic facial and head movements help and may even cure these cases. I have seen a case apparently relieved by extract of parathyroid three times a day. Injections of alcohol into the nerve have cured some cases (Patrick).
Blepharospasm is the name given to a spasm mainly tonic of the orbicularis palpebrarum. It is generally caused by diseases of the eye, and its nature and treatment are matters belonging to ophthalmology. It is a rare symptom of hysteria.
Nictitating or winking spasm is a clonic spasm of the or- bicularis, and usually forms part of habit chorea or is a symptom of hysteria.
Tonic facial spasm is sometimes seen in major hysteria. Facial Palsies.
The paralyses of the facial nerve may be due to lesions that are central, nuclear, meningeal or peripheral.
Facial palsy of central origin is almost invarably an accompaniment of hemiplegia and is due to hemorrhage, softening, inflammation, or tumor of the brain. The lower two branches of the facial are chiefly involved.
Facial palsy of nuclear origin is very rare and is an accompaniment of glosso-labial palsy, of diphtheritic palsy, or of gross lesions of the pons.
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DISEASES OF THE NERVOUS SYSTEM.
Facial palsy of meningeal origin is due to tumors, meningitis, or fracture of the base of the brain, and is accompanied by lesion of other cranial nerves. Syphilis is the most important factor here. All these forms are simply part of other diseases.
Peripheral faciae palsy (Beee's paesy) is the common type of facial paralysis.
Etiology. — The typical cases of this disease are due to exposure, in- fection and so-called rheumatic influences. Males are oftener affected, and the common age is between twenty and forty. It is more frequent in the winter and in temperate climates. It is not hereditary. A neuropathic tendency predisposes to it. Syphilis sometimes causes an isolated facial palsy; although, it is apt to leave this nerve alone. Facial palsy may occur in multiple neuritis, when it is often bilateral, and in locomotor ataxia. Non-typical and accidental cases of per- ipheral facial palsy are due to injuries, fracture of the petrous bone, or ear disease. Forceps pressure in difficult labor causes some cases, and a very few have been congenital. The ordinary cases are mostly due to microbic infections similar to those of "colds" and influenza.
Symptoms. — The disease comes on rather suddenly, and reaches its height within a few hours, or, at most, two or three days. Pre- ceding and accompanying the onset there may be some pain about the ears and a little swelling is sometimes seen.
The patient feels a subjective discomfort on the paralyzed side of the face. He finds that he cannot completely shut the eye; if he tries to chew on the affected side, food gets between the teeth and cheek. He cannot pucker the lips, and his speech is a little muffled. The appearance of the face is most characteristic.
On the affected side the wrinkles are smoothed out, the angle of the mouth is lower, the mouth is drawn at first to the sound side, and owing to this distortion the tongue appears not to be protruded straight. In laughing or other emotional movements of the face, the trouble is most clearly brought out. But the most characterized appearance is produced by telling the patient to shut the eyes tightly and draw out the angles of the mouth so as to show the teeth (Fig. 58). The eye on the palsied side is not closed, and the eyeball turns up, showing the white of the eye. This test of the palsy is better than any examination of the wrinkles and folds of the face, for in children and in the young and plump these differences in the two sides of the face are not very marked, especially in the slighter cases. The nostril on the affected side does not expand on forced inspiration; the eye is apt to be watery and the conjunctiva somewhat injected.
If the disease extend well up into the Fallopian canal, so as to
MOTOR DISORDERS OF SPECIAL NERVES.
!33
involve the nerve to the stapedius, that muscle is paralyzed, the tensor tympani acts unopposed, the drum is tightened, and unusual sensitiveness to sounds results. This is rare, however; most of the ear symptoms in facial palsy being due to a concomitant disorder of thes .tympanum or the acoustic nerve.
If the disease involve the nerve between the geniculate ganglion and the|point where the chorda tympani is given off (see Fig. 57), some loss of taste follows, and this is a frequent symptom, but not
Fig. 58. — Patient with left facial palsy trying to close both eyes and show the teeth.
one of long duration. If the disease is located more centrally than the geniculate ganglion or more peripherally than the chorda nerve, taste is not involved. Usually, when there is no taste involvement, it is because the lesion is peripheral. By an examination of the taste sense and of the hearing, the location of the trouble can be accurately made out. After a few weeks some wasting of the face may be noted, but this is never very great.
In some cases there is with the paralysis a great deal of pain in and about the ear and an herpetic eruption on the ear, which may be very slight in amount. A slight anaesthesia of the concha and posterior
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DISEASES OF THE NERVOUS SYSTEM.
wall of the external meatus is noted. In these eases, the trouble is due to an inflammation of the geniculate ganglion which has extended and affected the facial nerve and the primary trouble is an herpetic inflammation of the ganglion. (J. R. Hunt.)
The electrical reactions are characteristic and important, since typical or partial degeneration reactions can usually be observed. For a few days there is an increase in irritability of the muscles to both faradic and galvanic currents. This is followed by a gradual loss or diminution in faradic irritability, while the galvanic irritatibility remains sometimes increased for a while and then falls. At the same time a reversal of poles and a sluggish contraction, particularly the latter, may be noted. At the end of five or six weeks faradic irrita- bility ought to begin to return. Great variations are found in the electrical reactions dependent upon the severity of the case. For example, in very severe cases the electrical irritability may be almost or entirely absent for days and even weeks.
If the disease lasts for two months or more and the palsy is not a complete one, secondary contractures begin to appear. The mouth now becomes drawn to the affected side, and the nasolabial fold becomes deeper than that on the sound side. In smiling or speaking or other facial movements there is an excess of movement on the paralyzed side, the teeth and upper gums in particular showing ab- normally. This is particularly the case in old palsies beginning in childhood.
Pathology. — The disease in its typical form is due to a diffuse neuritis. This attacks the periphery of the nerve in the face and ex- tends rapidly up into the Fallopian canal as far as the geniculate ganglion. The inflammation sometimes attacks most the peripheral filaments; at other times it is more central. The old idea that it was always a perineuritis of the nerve in the Fallopian canal is incorrect (Minkowski).*
As already stated, Dr. Hunt has shown that some cases of facial palsy are due to the extension of an inflammation of the geniculate ganglion, which is the sensory ganglion of the seventh nerve.
Facial paralysis is not, as is usually taught, a rheumatic or or refrigeration disease. It is not caused by rheumatic poisoning, nor does it occur in persons who have a particular rheumatic constitution, and it is not in the majority of cases associated with a distinctly rheumatic etiology. It is rather an infectious disease, and should be
*Dr. Pierce Clark, however, has recently brought evidence to show that the old idea of a " Fallopian neuritis," pinching the nei~ve and cutting it off in the canal, is the correct one.
MOTOR DISORDERS OF SPECIAL NERVES.
135
classed as such or as a post-infectious disease. Many cases will be found to occur after influenza or some infection which is allied to it. Indeed, it has seemed to me more like a "cold" of the seventh nerve than a rheumatism. This view is borne out by my experience in the treatment which shows that antirheumatic drugs, like the salicylates and iodides, do not appreciably modify its course.
Diagnosis. — The recognition of the palsy is made easy by causing the patient to contort the face. In children it requires more care to detect the side affected.
It is important to determine whether the palsy is cerebral, nuclear, basilar, or peripheral. If the cause is cerebral, the upper branch of the nerve is little affected and the patient can close the eye. The nerve and muscle show no degenerative reactions.
Nuclear palsy is very rare and is accompanied with other sym- ptoms, especially those of involvement of other cranial nerves. A history of diphtheria, lead palsy, or bulbar paralysis is obtained.
In palsies due to lesions at the base of the brain, such as gummy meningitis, the auditory and other cranial nerves are involved and there are signs of brain syphilis. By testing the sense of taste and hearing, the location of the peripheral trouble can be made out. Thus, if there be loss of taste on the anterior two-thirds of the tongue, the lesion must be between the geniculate ganglion and the point at which the chorda tympani is given off. If the taste be not involved, the lesion must be central or peripheral of the part of the nerve which includes the chorda. Practically in most cases it is peripheral to it. If central, the disease is usually of syphilitic or tuberculous origin; the palsy is severe and the loss of ability to close the eye very great. In the geniculate ganglion cases there is usually herpes, great pain and a slight cutaneous anaesthesia, as described. (Hunt's Syndrome.)
Prognosis. — The prognosis of peripheral palsy (Bell's) is good, although an absolutely complete recovery often does not take place.
In syphilitic cases the prognosis is not so good, though patients may recover. In central palsies the prognosis is the worst, because the lesion usually does not disappear. However, the central cases are from the beginning of a mild type, and give annoyance mainly from the secondary contractures.
Bell's palsy usually lasts three to five months. Occasionally there are mild cases that get well within a month. The prognosis as to duration is much helped by a close study of the electrical reactions. In proportion as the degeneration reaction is complete and persistent, the outlook is bad.
Treatment. — In the acute peripheral cases the patient should be
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DISEASES OF THE NERVOUS SYSTEM.
treated promptly and thoroughly. He should be given a diuretic and puragtive and a blister should be placed over the exit of the nerve. This should be followed or accompanied by hot fomentations. Salicylate of soda in full doses of twenty grains should be given during the first week. After the paralysis is established, iodide of potassium is indicated in moderate doses. Electricity is to be em- ployed with care at first. After a week it may be given daily for five minutes, using the galvanic currents just strong enough to contract the muscles. After three or four weeks, if the faradic current causes contraction, it may be used, otherwise the galvanic current is to be
Fic'59. — Section of medulla, showing nuclei of origin of the IX,X,XI, and XII cranial nerves, rac. d., Ascending sensory root of IX, X, and XI nerves.
continued. At the end of a month an application every other day is sufficient. At the end of three weeks, if the paralysis is severe, the corner of the mouth should be drawn up by means of a bent hook, which is carried back and fastened behind the ear. The patient should wear this most of the daytime, but not at night. The object is to take cff the strain caused by the pulling of the muscles cn the sound side.
At the end of a couple of months, if secondary contractures set in, massage may be tried, and the patient should practice facial gymnastics daily. Acupuncture combined with weak faradic currents and local injections of strychnine may be tried in bstinate caseos. If the eye cannot be closed it should be protected by a shade. In
MOTOR DISORDERS OF SPECIAL NERVES.
137
obstinate cases good results have been obtained by suturing part of the hypoglossal nerve to the facial.
The Geosso-pharyngeae Nerve.
Anatomy. — The glosso-pharyngeal nerve has motor, sensory, and visceral fibres. The nucleus of origin of the motor fibres is the nucleus ambiguus common to it and the
vagus and accessory. The sen- ^ VII
sory fibres arise from two small ganglia lying on the root of the nerve, the petrous and jugular. The cells of these ganglia are unipolar. The neuraxon bifur- cates, and sends its central fila- ment along the nerve-root into the medulla, to the gray nucleus, known usually as the nucleus of origin of the glosso-pharyngeal and lying close to the vagus nucleus (Fig. 59). Fibres also go to a track of gray matter lying close to the solitary bundle and known as the ascending sensory root of the ninth, tenth and eleventh nerves. The nucleus is really, however, a terminal one, and is the origin of secondary sensory neurons which send up fibres to the brain. The peri- pheral filaments of the root ganglia pass along the nerve and supply the fibres of sensation.
The nerve supplies general sensation to the tympanum, tonsils and pharynx (in connec- tion with the vagus) and upper part of the larynx; special sen- sation of taste to the posterior third of the tongue, and motion to the pharyngeal muscles and oesophagus (Kriedl) in connec- tion with the vagus.
The terminal filaments of the sensory taste fibres supply- ing the posterior two-thirds of the tongue end in fine fibres that pass into the taste buds. There are
no special peripheral cells of taste, as asserted by Fusari and Panasci. Its cortical representation so far as taste is concerned is in the hip-
Fig. 60. — Vagus and spinal accessory nerves. (Young.)
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DISEASES OF THE NERVOUS SYSTEM.
pocampal gyrus. The nerve gives very sensitive reflex fibres to the pharynx and is important in the reflex act of deglutition; it also car- ries sensations of nausea from pharyngeal irritation.
Motor Neuroses of the Geosso-pharyngeae.
This nerve is rarely affected independently by motor troubles Spasm of the pharyngeal constrictors occurs in general disorders like rabies, and reflexly in severe neuralgia of the trigeminus. This con- dition, known as dysphagia, is seen also in hysteria, and there is prob- ably sometimes spasm in connection with the symptom known as globus hystericus.
Paralysis of the throat constrictors occurs as one of the symptoms of glosso-labio-laryngeal paralysis and sometimes in diphtheritic paralyses.
The Pneumogastric Nerve and the Accessory Part of The
Spinae Accessory.
Anatomy. — The pneumogastric or vagus nerve has two nuclei of origin, a motor and a sensory.
1. The motor nucleus or nucleus ambiguus, which is a prolongation of the lateral horn of the spinal cord, lies deep in the medulla and has a nucleus common to the vagus and glosso-pharyngeal (Fig. 59).
2. The sensory fibres arise chiefly from two ganglia that lie on the root of the nerve, the jugular and plexiform. These bodies resemble spinal ganglia. The cells are unipolar and send off a neuraxon which bifurcates. The peripheral fibre passes on along the nerve and sup- plies in with its sensory fibres. The central part passes up to the gray matter of the floor of the fourth ventricle and ends in the so-called sensory nucleus (Fig. 59). This nucleus, however, is not the real nu- cleus of origin, but, as in the case of the ninth, it contains cells which send their axis-cylinders or neuraxons brainward and form secondary sensory neurons. Fibres also go to the ascending sensory root common to this nerve and the ninth. It is probable that some of the cells of the terminal sensory nuclei of the ninth and tenth are efferent visceral cells, like those of the columns of Clark, and send out visceral fibres. Both motor and sensory fibres are chiefly visceral in distribution and function.
The spinae accessory nerve is purely motor in function (Van Gehuchten). The accessory part arises from the cells of the nucleus ambiguus and passes into the trunk of the vagus.
The spinal part of the spinal accessory arises from the lateral horn and outer part of the anterior horn of the spinal cord. Its fibres of origin reach from the first to the third or fourth cervical nerves as far as the fourth or fifth cervical roots. The fibres unite in the cranium and pass out through the posterior lacerated foramen in the same sheaths as the vagus. After their exit from the skull, they divide into an in- ternal and external part. It is the external branch which contains the fibres of spinal origin. The internal part contains the fibres from the medulla and unites with the vagus. The nerve receives some sen-
MOTOR DISORDERS OF SPECIAL NERVES.
139
sory fibres from the first and sometimes from the second cervical nerve. The terminal branches receive motor fibres from several cervical nerves. The spinal accessory contains large and small or visceral fibres. The spinal part contains only the large fibres.
The spinal accessory supplies the sterno-cleido-mastoid almost exclusively, but only the upper part of the trapezius; the rest of this muscle is supplied by the cervical and dorsal nerves. The sterno- cleido-mastoid, when innervated, draws the chin up and over toward the opposite side. The upper fibres of the trapezius draw the head back slightly and down toward the same side. Physiologically, the spinal part of the accessory nerve is one of the motor cervical nerves; the accessory or medullary portion is part of the vagus, and has visceral and sensory as well as motor functions.*
The vagus and accessory part of the eleventh together have an ex- traordinary wide distribution and diversity of function.
Fig. 61. — Longitudinal section, showing the nuclear origin of the motor cranial nerves.
1. First they contain motor, inhibitory, and vasomotor fibres. These fibres go to the pharynx, larynx, trachea and bronchi, to the oesophagus, stomach, small intestines and spleen.
2. Sensory fibres, which go to the occipital and transverse sinuses and dura mater of the posterior fossa, to the external auditory meatus in part, to the pharynx, larynx and trachea and to the oesophagus.
3. Exci to-reflex fibres, which go to the lungs and heart, stomach, and to other organs mentioned as supplied by the vagus with sensation.
These reflex fibres stimulate or inhibit the vasomotor centre, the respiratory rhythm and the cardiac rhythm. They also excite reflexly deglutition and respiratory movements.
The secretory fibres go to the respiratory tract, oesophagus, stomach and pancreas and small intestines.
Cardio-inhibitory fibres go to the heart, while reflex accelerating fibres and inhibiting fibres go to the lungs. The accessory nucleus sup- plies the laryngeal adductors and the cardio-inhibitory fibres.
*Dees thinks that the spinal origin of the eleventh is continuous above with the twelfth, not with the anterior nucleus of the tenth. He denies that the medullary nucleus sends fibres to the larynx.
The internal branch of the eleventh sends motor fibres to the rectus posticus (E. Remak).
DISEASES OF THE NERVOUS SYSTEM.
Diseases of the Pneumogastric Nerve and of the Bulbar Portion of the Spinal Accessory.
These nerves are essentially visceral in character. Their diseases call for a study of laryngeal, pulmonary, cardiac and abdominal neu- roses, which would bring us into the domain of laryngology and general medicine. Hence, despite their great importance, I have thought it best to describe only in a general way the anatomical supply and function of the laryngeal nerves. Some of the symptoms are described in connection with locomotor ataxia, progressive mus- cular atrophy, exophthalmic goitre, and angina pectoris.
The Laryngeal Nerve-supply. — Phonation is mostly a volun- tary act, and has a centre in the cortex of both hemispheres at the lowest level of the praecentral convolution. By its stimulation adduction of the vocal chords can be produced.
Respiratory movement abduction of the vocal chords has no cortical centre. Its centre is in the medulla near the vagal nuclei.
The motor nerve of the tarynx is the recurrent laryngeal which intervates all the muscles, adductor as well as abductor, except the cricothyroid which is innervated by the superior laryngeal.
The motor fibres for the abductors (respiratory fibres) run in a separate bundle inside the recurrent nerve while the adductor or phonatory fibres run on the outer part. (R. Russell.)
The abductors are paralyzed first and most easily and are especi- ally apt to be affected by progressive organic disease in the bulb.
But in functional diseases (psychic, hysterical) the adductors are exclusively affected. This is because the impulses to the adductor muscles have a wider range of fibres to work upon. When there is complete recurrent nerve palsy, the vocal chord is motionless and in a position of near the median line.
The superior laryngeal is the sensory nerve of the larynx, and when it is injuried there is anaesthesia of the larynx and paralysis of the cricothyroid which is a tensor muscle.
In vagus paralysis high up on one side we have anaesthesia of the larynx with loss of reflex phenomena and consequent passage of foreign bodies, food, etc., into the laryngx; also tachycardia and paralysis of the abductors, which may be bilateral.
In paralysis of the superior laryngeal we have only the anaes- thesia of the larynx with its resulting loss of reflex protective activity.
In paralysis of the recurrent laryngeal we may have at first and only abductor paralysis, later total paralysis of the vocal chord.
The causes are surgical, infectious, and degenerative diseases, such
MOTOR DISORDERS OF SPECIAL NERVES.
141
as tabes, progressive bulbar palsy, and any organic bulbar lesions, such as tumors, hemorrhage and softenings. The commonly observed cause is aneurism of the aorta and tumors of the mediastinum or neck, such as goitre.
Neuroses op the Spinal Part of the Accessorius. This is a purely motor nerve, and its disorders are therefore spasm and paralysis.
Torticollis (Wryneck, Caput Obstipum). — Toricollis is a dis- ease characterized by clonic or tonic spasm of the muscles supplied by the spinal accessory and often of other muscles of the neck. There are several forms of wryneck, which must be distinguished from each other. They are: (1) congenital wryneck; (2) symptomatic wryneck; (3) spurious wryneck, from spinal disease ; (4) true spasmodic wryneck.
1. Congenital wryneck is due to some intra-uterine atrophy or ob- stetrical injury of the sterno-cleido-mastoid. It occurs oftenest after breech or foot presentations. The right side is usually affected. There is no spasm at all, but the neck is fixed to one side by the short ness of the muscle, and also rotated to the opposite side. The deformity becomes more noticeable as the child grows older, because the parts atrophy. The atrophy affects not only the shortened muscles, but the face on the affected side (Fig. 62).
2. Symptomatic wryneck is usually due to a rheumaticmyositis, and occurs chiefly in children. It may be due also to tumors, adenitis, abscesses and local syphilitic disease. In these cases there are always pain and tenderness associated with the deformity.
3. Spurious wryneck is an apparent or real spasm of the neck muscles due to caries of the spine.
Fig. 62.— Congenital wryneck of the right side.
142
DISEASES OF THE NERVOUS SYSTEM.
Treatment. — Congenital wryneck, if taken early, can be cured by tenotomy of the sterno-mastoid and subsequent fixation of the neck for a time. When osseous changes have occurred perfect relief is impossible. Symptomatic rheumatic wryneck is a trivial and tem- porary affection, which needs only to be palliated by hot applications and saline purges until cure takes place.
Suprious wryneck requires suitable orthopaedic measures, such as the plaster jacket and jurymast.
4. Spasmodic Wryneck. — This is a purely nervous disease charac- terized by spasm of the muscles supplied by the spinal accessory and often of those supplied by the upper cervical nerves also.
Etiology. — -Women are oftener affected than men. It occurs in
Fig. 63. — Typical wryneck involving the spinal accessory on the right side. (Walton.)
early adult and middle life, never in children or old people. A neuropathic constitution and heredity often exist. Overwork, ex- hausting emotions and a hereditary tendency are the commoner causes. It occurs in people who have led a sober life.
The exciting causes are occupations which put the lateral muscles of the neck on a strain, depressing emotions, physical shocks and blows, rheumatic influences. Sometimes no cause can be detected. Reflex irritations, perhaps, exist in some cases, but it is difficult to find them.
Symptoms. — The disease begins with slightly painful sensations in the neck, which are soon accompanied by spasm. The spasm is' at first clonic and intermittent. The sterno-mastoid is oftenest involved of single muscles, but the rule is that the upper fibres of the trapez- ius are also affected. The patient's head is inclined toward the
MOTOR DISORDERS OF SPECIAL NERVES.
143
affected side by the trapezius, the chin is raised, and the head rotated to the opposite side by the sterno-mastoid and trapezius, and this is the typical position in the disease (Fig. 63). If both trapezii are af- fected the head is pulled back, but this is a rare form. It is called retro-collic spasm. The complexus and obliquus superior are the only other neck muscles which can rotate the head to the opposite side. They are supplied by the upper cervical nerves and are some-times involved in wryneck. In torticollis the muscles affected with spasm have a similar physiological function. Hence, while the sterno-mas- toid, trapezius, complexus and superior oblique on one side are at- tacked by the spasms, muscles on the other side may be at the same time implicated. The opposite muscle commonly affected is the splenius, which inclines the head laterally and rotates it to the same side. Probably the deep muscles, recti capitis postici, major and minor, and the inferior oblique, which draw the head back and rotate to the same side, are also at times affected. The list of muscles that may be involved and their nerve supply are as follows :
|
Turning Head to Opposite Side. |
Turning or Inclining Head to Same Side. |
Nerve Supply. |
|
|
Muscles usually involved. |
Sterno-cleido-mastoid |
Eleventh. Eleventh. |
|
|
Muscles rarely involved. |
Superior obliquus |
Recti capitis postici, maj., min.j infer. obliq., splenius. |
Cervical. Cervical. |
Extreme rotation without much retraction of the head would indicate involvement of the sterno-cleido-mastoid and opposite splenius. Retraction of the head indicates involvement of both trapezii or the deep recti muscles.*
The disease may start in one muscle and gradually extend to others, even involving the facial, masticatory and brachial nerves. As it progresses the spasm becomes more constant, and finally it may be tonic, never yielding except to artificial means or during sleep. The pain associated with the disease gradually decreases. The affected muscles hypertrophy the muscles thrown into disuse atrophy. There is some deformity, in time, of the neck and shoulders, but facial asymmetry does not occur in this form unless it begins, as is very rarely the case, before maturity.
The disease may be complicated with or alternate with other neu- roses. I have known epilepsy to be associated with it.
*A full account of the physiology of the neck muscles is given later under the motor neuroses of the upper cervical nerves — where torticollis more properly belon gs.
144
DISEASES OF THE NERVOUS SYSTEM.
Pathology. — -The disease is a neurosis involving the bulbar and subconscious psychic centres. The neuro-mechanism controlling the movements of the neck is unstable and out of good volitional control. It seems to be in a sense worn out, consequently it sends out intermittent and irregular discharges of nerve force. The disease is a degenerative or perhaps to some extent an autotoxic one, and indicates the prema- ture decay of centres never perhaps originally perfect. It is called in its typical form by French writers "mental torticollis." By this is meant that the ordinary voluntary control of the neck movements by the will has been lost, the psyche has lost its grip and a subordinate subconscious mechanism is in control. The thyroid or parathyroid may have some part in the trouble.
The diagnosis has to be made from the other forms of wryneck men- tioned. The age, history and fixed character of the spasm serve to distinguish congenital wryneck. The history, the pain and tender- ness and the temporary duration differentiate the rheumatic forms.
The increased rigidity on passive motion, the pain, deformity, and other signs of cervical caries are sufficient to diagnosticate verteralb disease.
Prognosis. — The disease is not fatal* It generally reaches a certain stage and then remains chronic. In rare cases it is cured ; in many others it can be much ameliorated. Cases occurring in young people, in the hysterical and in those without a decided neurotic history are the most favorable.
Treatment.- — The drugs which are efficient are opium, atropin or hyoscyamin, conium, gelseminum, valerianate of zinc, asafoetida, chloral, bromides, arsenic and cocaine. Of these, opium, atropin the bromides, and zinc are generally the most efficacious. Opium must be given with great caution. Atropine should be given hypodermically in increasing doses up to intoxication (gr.y1^.) (lyeszynsky). The galvanic and faradic currents are useful adjuvants in helping to relax the spasm and keep up the nutrition of the muscles, but alone they are not curative. Massage and stretching the neck in a Sayre apparatus, together with systematic exercise of the neck muscles, often help. The only surgical measures to be advised are nerve resection, and possibly the partial cutting of the sterno-mastoid muscle. A very few cures and many failures have followed surgical interference. The pedagogical and psycho-therapeutic method of dealing with torticollis, by massage, exercises and relaxation, is the most important of all measures. This form of treatment must be combined with rest, conies and symptomatic remedies, and it must be began if possible early and kept at persistently and repeatedly — if necessary
MOTOR DISORDERS OF SPECIAL NERVES.
*45
even for two or three years. Hypnotism does no good, except perhaps in young and distinctly hysterical patients.
Splims and mechanical-fixation apparatus do no good, as a rule. Alcoholic injections may be tried.
Resection of the posterior branches of the upper three or four cervical nerves, as suggested by Keen, has done great good in a few cases (Powers).
Paralysis of the Spinal Part of the: Accessory. — Etiology.— The causes are injuries, caries of vertebra, progressive muscular atrophy, and all forms of spinal disease reaching high up in the cervical cord.
Symptoms. — When one nerve is paralyzed the head may still be held straight, but there is inability to rotate it perfectly. The prominence of the sterno-mastoid is absent — atrophy takes place. No spasm of the other muscle occurs, and there is no such thing as paralytic torticollis (Gowers). The involvement of the trapezius causes a depression in the contour of the neck, especially noticeable on deep inspiration. There is some trouble in raising the arm, the scapula is drawn away from the spine, and the lower angle is rotated inward. When both nerves are paralyzed there is great difficulty in rota ling the head or raising the chin. Paralysis of both sterno- mastoids causes the chin to drop backward, while paralysis of both trapezii in their upper parts causes the head to drop forward. Atrophy of the muscles attends the paralysis of the nerve, and degenerative reactions may be noted. The cervical nerves appear sometimes to supply the sterno-mastoid and upper part of the trap- ezius so much that in disease of the accessories decided paralytic symptoms are absent. When bo h parts of the spinal accessory are involved, dropping of the palate, dysphonia, and rapid pulse are added symptoms.
The diagnosis depends upon a thorough examination of the motility of the parts.
The treatment is based on a knowledge of the cause of the disease.
Anatomy. — The hypoglossal nerve arises from a long and large nucleus lying on the lower part of the floor of the medulla near the median line and to the outer and ventral side of the central canal. The nucleus is a continuation upward of the anterior horns of the spinal cord and is homologous with the sixth, fourth and third nerve nuclei higher up (see Fig. 59). It reaches below as far as the decussation of the pyramids and above as far as the glosso-pharyngeal nucleus. A second small-celled' nucleus lies just beneath the nucleus proper.
The Hypoglossus — XII.
10
146
DISEASES OF THE NERVOUS SYSTEM.
Its cortical representation is in the lower end of the central convolu- tions, to which it is connected by fibres that pass into the raphe and thence to the anterior pyramids. Its fibres pass out between the oli- vary body and the anterior pyramid. At its origin it is a purely motor nerve; it receives a few sensory fibres from the cervical nerves and the vagus. It supplies the following muscles:
Intrinsic muscles of the tongue: superior and inferior longitudinal and transverse. The extrinsic muscles of the tongue; hyoglossus, genio-hyoglossus, and styloglossus. (The palato-glossus and Unguals are supplied by the fifth and seventh cranial nerves respectively.) The depressors of the hyoid: the thyrohyoid and, with the cervical nerves, the sterno-hyoid and sterno-thyroid. The elevator of the hyoid: genio-hyoid. It is also thought to send fibres to the oral muscles (Tooth).
The hypoglossal nerve is concerned in the movements of the tongue and in fixing or depressing the hyoid in mastication and deglutition. When diseased, therefore, speech and deglutition are affected. The small nucleus of the nerve is thought to control the finer lingual movements of articulation.
Motor Neuroses of the Hypoglossal Nerve.
The diseases of this nerve consist of lingual spasms, lingual palsy and lingual hemiatrophy.
Linguae spasms take part in the disorders of articulation, help- ing to cause stuttering and speech cramps. Such troubles are often developmental in origin and belong to the habit choreas or convulsive tics.
Stuttering is a spasmodic disorder in which the tongue muscles are involved, preventing the proper enunciation of words and sentences.
Stammering is an imperfect articulation due sometimes to disease or defect in the hypoglossal nerve and its muscles. It is not a spasm.
The letters that are oftenest badly pronounced are R, L and S. Lisping is a form of stuttering. Stammerers are sometimes called the " R L S people."
Aphthongia is the name given to a form of spasm occurring in speakers and similar in nature to writer's cramp.
Clonic lingual spasm occurs in chorea, hysteria and during the attacks of epilepsy. Unique cases of this spasm also occur from reflex irritation or central nervous disease.
Tonic lingual spasm occurs in hysteria, and sometimes as an inde- pendent affection due to unknown causes, generally those of a debilitating and nervously depressing character. Reflex irritation may be a cause.
Linguae paralysis (glossoplEgia) is usually one of the symp-
MOTOR DISORDERS OF SPECIAL NERVES.
147
toms of glosso-labio-laryngeal palsy. It may be caused by a bilateral or even a single lesion in the cerebral hemispheres. The condition is then known as pseudobulbar paralysis. Diseases of the medulla and of the nerve itself may cause the paralysis.
The paralysis may be either unilateral or bilateral. The symp- toms are an impairment of speech and of swallowing. Fuller details will be given under the head of Bulbar Palsy.
Fig. 64. — Left lingual hemiatrophy.
Progressive; Lingual Hemiatrophy.— A progressive hemiatrophy of the tongue sometimes occurs. It is analogous in all respects to facial hemiatrophy, with which it is sometimes associated. It is probably due to a low grade of degenerative neuritis of the nerve. It is very rare.
The Phrenic nerve arises from the third, fourth and fifth cer- vical, oftenest from the fourth and fifth (37%). Next from the fourth alone (25%) and then from the third, fourth and fifth (23%) and from the third and fourth (15%) (Schroeder-Green).
The diaphragm is supplied by a few filaments of the intercortal nerves, but these cannot take the place of the phrenic and their func- tion is slight (Cavalie).
CHAPTER IX.
NEUROSES OF THE MOTOR SPINAL NERVES.
Anatomy and Physiology. — The spinal nerves arise from the spinal cord by two roots, anterior and posterior. These roots unite outside the jspinal canal to form mixed nerves. The mixed nerves divide and go to their various destinations. There are thirty-three pairs of spinal nerves, viz. :
Cervical 8
Dorsal 12
Lumbar 5
Sacial 5
Coccygeal 3 (all rudimentary).
33
The last two coccygeal nerves are microscopic in size, and the first pair is very small, so that practically there are but thirty sets of spinal nerves.
The posterior roots are closely connected with ganglia lying in the intervertebral canal, and called intervertebral ganglia, or ganglia of the posterior roots. These ganglia are the real origin of the great majority of the fibres of these roots. The mixed nerve is connected by fibres that come chiefly from the anterior root, with the sympa- thetic or prevertebral ganglia. The distribution of the spinal nerves is also shown in Plate II.
For the purpose of conveniently studying the diseases of the spinal nerves, we divide them into six different groups, each having a somewhat definite work to do. These groups are shown in the accom- panying table.
|
Strands of Spinal Nerves. |
Distribution. |
Associated Ganglia of Sym- pathetic. |
|
|
Group I. Group II. Group III. Group IV. Group V. Group VI. |
Upper four cervical. : Occipital region, neck. Lower four cervical and Upper extremities. first dorsal. Upper six dorsal. Thoracic wall. Lower six dorsal except last. Abdominal wall, upper lum- 1 bar, upper lateral thigh surface. Twelfth dorsal, four lumbar. Lumbar region, upper glu- teal, anterior and inner thigh and knee. Fifth lumbar and five sacral. Lower gluteal, posterior thigh, leg, pelvic organs. |
First cervical. Second and third cervical, first dorsal. First to sixth dorsal. Fifth to twelfth dorsal. First to fourth lumbar. First to fifth sacral. |
Group I. The Upper Cervical includes the first four of the spinal nerves. These divide into anter- ior and posterior branches. The posterior branches supply the
148
NEUROSES OF THE MOTOR SPINAL NERVES.
149
muscles and skin of the back of the neck and the occiput. The principal nerves are the suboccipital and the grea: occipital. The anterior branches form the cervical plexus. Its principal branches are the auricularis magnus, occipitalis minor, and phrenic. The special distribution of the nerves is shown in the table p. 250.
The upper cervical nerves supply motion to the muscles which rotate the head and draw it back and sideways.
One branch, the phrenic, supplies the diaphragm ; other muscles assist in fixing the thorax in forced inspiration. They innervate some of the hyoid and thyroid muscles, but have no influence on phonation or deglutition. . This group of nerves is in close connection centrally with the trigeminal nerve, whose descending root reaches down into the cervical cord. The fibres to the scalp and face also anastomose with the trigeminus in their peripheral distribution to the scalp and chin.
Special Anatomy. A knowledge of the anatomy and physiology of the neck muscles and nerves is important in studying the more serious neuroses of this region.
The muscles of the neck are :
_, , . , ( Wrinkles the neck, depresses the jaw ) Facial.
The platysma myoid . . . . j and angle of the mouth. j
Recti capitis antici Flexion of head. • V and C2.
Recti capitis laterales, • • -Lateral movement and slight rotation. Antr. branches, L-v. • : Scaleni med. and post! Lateral movement and slight extension. Antr. branches, C6,?
'"'/Lateral movement; also flexion and )q„j„„1 nn„attc,n^„ „„a r-2 Sterno-mastoid \ rotation of head to opposite side. f SRmal accessory and O .
Trapezius { ^teral movement and extension. } SL^oYc^and"
Levator anguli scapuli - { ^ s^^ ***** ^ } Anterior branches, C3 and (X
Splenius capitis { Draws head backward. } E"±v d^r °[ P°,s% branches ot
( ) cerv. nerve, .
Trachelomastoid { Dr^e ^ead backward, turns face to J pQst branches cerv, nerve> Cs.
Complexus- f Draws head back or rotates to same jPost. branches cerv. nerve
( side. j C1, C2, C3.
Obliquus superior | Extends and slightly rotates to oppo- ) Post branches cerv. nerve
_ _ 1 site side and bends to same side. j C1.
Obliquus inferior Rotates to same side. Post, branches cerv. nerve, C2.
Rectus cap. post Extends and rotates to same side. Post, branches cerv. nerve. C1.
The movements of flexion of the head are thus seen to be due to the sterno-mastoid and anterior recti capitis, innervated by the spinal accessory and by the C1 and C2 (anterior branches). In forced flexion the hyoid muscles come into play, innervated by the branches from tfi£ descendens and communicans hypoglossi, C3, C4, anterior branches.
The movements of extension are caused by the complexus, trachelo- mastoid, splenius, obliquus inferior and recti capitis postici and somewhat by the trapezius. These are innervated practically entirely by the posterior branches of the cervical nerves — from C1 to C3.
The lateral movements of the head are caused by the sterno-
T5°
DISEASES OF THE NERVOUS SYSTEM.
mastoid, splenitis, scaleni, trapezius, complexus, obliquus superior, rectus capitis lateralis, supplied by the spinal accessory, posterior branches of the cervicals C1 to C3, except the recti laterales, supplied by the anterior branches C1.
Extension and lateral movements of the head are mainly done by the spinal accessory and upper three cervical posterior branches. The sterno-mastoid gets some branches from the anterior cervical division C2, as do the recti capitis laterales.
Rotation of the head is caused by the sterno-mastoid, splenius
Rectus post .maj. OT)ttg-sW& inf
Obliq-siip-2< Corrfplexu;
Sensory 6r<
ensory brqnchto _ 'occinui ■
Complexus Splenius Trachelo-Mast
Occipital major.
Cervical ascend- Trans- colli
exus
Rectus lateralis C1Ca RectianteriorC'C2
D iaphr ag™(Phren) C3C*
Slerno mastoid C2 Levator anqs cap C3 Trapezius C3C4
^ ^ (Trans-cervical C2C 3 g TAAuricvlaris rnag^C* w ^Occipitalismin-C2 ° t\Supraclavicul-&rcC3C
if) a,
Compl
Complexus MultifidSpin-
Interspinal es}
Semispinales Do- Do
Fig. 65. — Diagrammatic representation of the anterior and posterior divisions of the cervical nerves.
trachelo-mastoid, complexus, obliquus superior and inferior, recti capitis postici and levator anguli scapuli. These are supplied by the spinal accessory and posterior branches of the cervicals C1 to C3, except the sterno-mastoid and recti capitis laterales, which is supplied by the C2 and C1 anterior branches.
Hence after division of the posterior branches of the cervical nerves C1 to C4 there still remain innervations of the recti capitis laterales and levator anguli scapuli, sterno-mastoid and trapezius. When the spinal accessory is cut these other innervations remain and the head may yet be extended, bent laterally and rotated.
The muscles which rotate the head to the same side are the recti
NEUROSES OF THE MOTOR SPINAL NERVES.
capitis lateralis (slightly) levator anguli scapuli, trachelo-mastoid (posterior C3), complexus (posterior C1, C,2 C3) obliquus inferior (posterior C2). See diagram, Fig. 65.
Those rotating to opposite side are the sterno-mastoid, obliquus superior (post. C1).
The diseases of the upper cervical group are spasms, paralyses and neuralgias.
Spasmodic Diseases. — Torticollis may be limited to the cervical nerves, as has been shown, though it is usually primarily a spinal accessory neurosis.
Tonic spasm causing a rigid neck is a frequent symptom of men- ingitis, and forms part of epileptic and other convulsions.
In oscillatory and rotatory spasms the cervical nerves are involved.
Spasmus Nutans {eclampsia nutans, nodding spasm, salaam spasm, oscillating spasm). — This is a disorder occurring chiefly in children and characterized by rhythmical nodding or oscillating movements of the head. It occurs, however, in adults.
Etiology. — The disease occurs in young children who are anaemic and badly nourished. Dentition, digestive disorders, basilar menin- gitis, gross disease of the brain, are causes. Sometimes it is only a kind of habit chorea, and occasionally this habit continues during life. It may be associated with or a precursor of torticollis. It occurs sometimes in elderly people forming part of a family or senile tremor.
Symptoms. — The disease may come on suddenly; more often it develops slowly. The patient has paroxysms usually of a rather violent character, lasting for minutes or hours, or even continuing nearly all the time except during sleep. The head moves thirty to sixty times a minute usually, but the motion may be slower or faster. Movements of the eyes and facial muscles often complicate the affection. The paroxysm may end in an epileptic attack. The condition in young children is usually a constant mild rhythmical spasm. It is usually "outgrown." In adults it may assume the chronicity of a torticollis or it maybe plainly part of a general tremor.
The diagnosis is easily made by the symptoms. The prognosis and treatment depend upon the etiology. I have found bromide of potassium and hyoscine useful in elderly cases, and syrup of iodide of iron in children.
In some forms of stuttering the phrenic nerve is involved in a clonic or tonic spasm.
Hiccough is a clonic spasmodic disorder of the phrenic nerve. It is usually due to gastric disturbance, with flatulent distention of the stomach.
DISEASES OF THE NERVOUS SYSTEM.
When chronic, it is caused by hysteria, neuritis, diaphragmatic pleurisy, or some pressure upon the nerve in ics course. I have seen cases in which it was probably a pure spasmodic neurosis, a form of tic. Ordinarily, hiccough can be stopped by simple carminatives like spirits of chloroform or lavender or a hot milk punch. In obsti- nate cases in which no known cause can be found, pilocarpin, hyoscine and bromides are useful. Counter-irritation along the neck helps some cases. A most effective measure is to lay the patient supine over a thick bolster so that the head hangs back and the thorax arches up. An injection of morphine and atropine promptly stops some cases.
Paralysis and Atrophy. — The cervical muscles are paralyzed in progressive muscular atrophy, in pachymeningitis hypertrophica, and occasionally in vertebral and peripheral disease or injury. Some deformities and weakness in head movements result, but the most serious consequence is involvement of respiration through palsy of the phrenic.
Paralysis of the Phrenic Nerve — Etiology. — Such paralysis may be due to disease or injury of the cervical cord and also to peripheral disease, to which the nerve is somewhat liable owing to its long course through the anterior mediastinum.
Pleurisy, peritonitis, mediastinal tumors, and multiple neuritis are among the special causes of phrenic paralysis. Spinal-cord dis- ease, such as tabes, acute ascending paralysis, and surgical injuries are, however, the commonest etiological factors.
Symptoms. — In diaphragmatic paralysis, if bilateral, as is usually the case, there is either no movement of the abdomen or the epigas- trium, and hypochondrium are drawn in, in inspiration instead of rising. On slight exertion there are dyspnoea and increase of respiration.
Diagnosis. — If no other muscles than the diaphragm are involved, the cause is probably in the trunk of the nerve. Inflammatory disease of the diaphragm may cause a paralysis which is recognized by its painful character and the febrile reaction.
Treatment. — This is to be guided by the cause. It need only be said that there is a motor point in the neck where by careful electriza- tion one can get a contraction of the diaphragm. In paralysis of the phrenic this fact should be borne in mind. The treatment otherwise is to be directed to the cause.
Group II. The Tower Cervical Nerves and Brachial Plexus.
Anatomy and Physiology. — The anterior branches of the lower four cervical nerves and first dorsal nerve unite to form the brachial plexus.
NEUROSES OF THE MOTOR SPINAL NERVES.
153
This gives off short nerves to the shoulder and trunk and long nerves to the arm.
The mode of formation of the brachial plexus is shown in the dia- gram (Fig. 68). It is in accordance with the descriptions of Walsh and Allen. The short or upper branches supply the shoulder and inter- costal muscles. The long or lower branches supply the arm and hand. The neurologist needs to know: (1) the muscular distribution of each nerve and the function of the muscle; (2) the cutaneous sensory distribution; and (3) the level of origin of the nerves.
The previous figure, Fig. 70, and table give these points, and will
found useful for study and reference. They are based upon the investigations of Ferrier and Yeo, Thorburn, and also on Abbe's and my own experiments.
The arrangement of the Brachial Plexus. — It is made up of three nerve trunks, which in turn make up three cords, these cords giving off various branches, thus:
1. Trunk from sixth and seventh cervical roots
2. Trunk from eighth cervi- cal and first dorsal roots
3. Trunk from fifth, sixth, seventh, and eighth cer- vical and first dorsal
forms outer cord, gives off
which f ^xt' ant' tnoracic- ] Musculo-cutaneous.
[ Outer head median.
Inner head median.
forms inner cord, which gives off
forms posterior cord, which gives off
Ulnar.
Int. cutan.
Int. ant. thorac. ( Intercost.-hum. { Subscapular. \ Circumflex. ( Muscula-spiral.
The following table shows the origin, muscular distribution, and effect of paralysis on the motor but not on the sensory nerves. This latter is indicated in Figs. 30 and 31.
Nerves and Roots of Origin. Muscular Distribution. Function as Shown by Effect of Paralysis.
Posterior thoracic. Fifth and sixth cervical.
Circumflex.
Fifth and sixth cervical. Suprascapular.
Subscapular, short.
Fifth and sixth cervical.
Subscapular, long.
Fifth, sixth, seventh and eighth
cervical. Anterior thoracic.
Musculo-cutaneous. Fourth and fifth cervical. Musculo-spiral.
Fifth, sixth, seventh and eighth aurical.
Serratus magnus.
Deltoid. Teres minor. Supraspinatus.
and
Infraspinatus and
minor. Subscapulars. Teres major. Latiss. dorsi.
Posterior edge of scapula is rotated out when arm is raised and carried forward. Weakening of elevation of shoulder and of
inspiration. Loss of power to raise arm.
Weakened power to raise arm; head of
humerus tends to fall. ' Loss of abductors, motion forward and inward rotation of humerus, teres Loss of rotation of humerus outward.
Weakens inward rotation of arm. Weakens power of elevating shoulder. Weakens power to depress shoulder and to pull arm backward and to side.
Pectoralis major. Loss of power to pull arm down and for-
ward and to shrug the shoulder. Biceps and brachialis Loss of flexion of forearm and weakness
anticus. of inspiration.
Triceps. ' Loss of extension of forearm.
154
DISEASES OF THE NERVOUS SYSTEM.
Nerves and Roots of Origin.
Muscular Distribution. Function as Shown by Effect of Paralysis.
Fourth, fifth, sixth, Supinatores. seventh and eighth cervical. '
Extensor carp. rad. Extensor carp. uln.
Extensor comm. digit.
Extensor p. i. pollic.
Extensor s. i. pollic.
Extensor oss. met. pollic. Median. Pronatores. Fifth, sixth, seventh, Flexor carp. rad. and eighth cervical. Flexor sublim. dig.
Flexor profund. dig. radial half.
Two lumbricales.
Abductor pollic.
; Flexor pollic.
Flexor carp, ulnar.
Ulnar.
Eighth cervical, first Flexor profund. dig. ulnar dorsal. I half.
Interossei. Two lumbricales. Flexor minor digit. Abductor pollicis. Inner half of flexor brev. pollicis.
Loss of supination.
Extension of wrist lost except when fingers are flexed; lateral deviation.
Impaired extension of first phalanges and wrist.
Impairment of extension of thumb.
Loss of pronation. Weakened flexion of wrist.
Weakened flexion of second and third phalan- ges of first and second fingers.
Loss of abduction and flexion of thumb.
Weakened flexion of wrist; radial deviation.
Weakened flexion of second and third phalan- ges of third and fourth fingers.
| Loss of flexion of first phalanges and of exten- sion of second and third.
Diseases of the Lower Cervical Nerve Group and the Brachial Plexus. — The nerves of this group are subject to the path- ological disturbances common to all nerves. I shall describe them from the clinical side, which gives the following disorders:
[ Combined arm palsies.
Paralyses J Upper arm type °r Erb'S palsy' y J Lower-arm type.
( Paralyses of individual nerves.
Spasmodic Disorders. \ Occupation neuroses.
[ Brachial neuralgia. Sensory Disorders. \ Digital neuralgia.
[ Numb hands.
Secret ry, Trophic, f
and Vasomotor Dis- \ Neurotic ceden a. orders. {
Spasmodic Disorders oe the Arm. — The arms and hands are especially subject to tremors, choreic and other spasmodic move- mentsr The only spasmodic disorders, however, which may be said to be especially located there are writer's cramp and allied occupa- tion neuroses. These are described elsewhere.
Brachial Paralyses, Arm Palsies. — These occur as combined or total-arm palsies, upper-arm and lower-arm types, and paralyses of single nerves.
Combined paralysis of the brachial nerves is a condition in which
NEUROSES OF THE MOTOR SPINAL NERVES.
155
all or nearly all the portions of the plexus and its branches are involved. Total arm palsies make up about six per cent, of all peripheral para- lyses, and are about one-fifth as frequent as single-nerve paralyses.
Etiology. — They occur oftenest in men, but are not rare in infants, being then due to injuries during parturition. After infancy they are most frequent in early and middle life.
The exciting causes are obstetrical and other injuries; deepseated inflammations and tumors; shoulder dislocations; primary neuritis; crutch and other forms of mechanical compression; functional palsies, from overwork and hysteria; in rare cases, spinal cord and brain disease.
The symptoms vary with the severity and extent of the lesion.
With regard to severity, there are three degrees. In the first there is simply a transient palsy, due to lying too long on the arm. The arm feels heavy, numb and "asleep." In a few minutes or hours this palsy disappears. In the second degree the nerves are so much compressed as to be mechanically injured. If the patient has been drinking hard, even moderate pressure may set up an inflam- matory or destructive process that leads to quite a serious palsy. In the third degree the nerves are actually cut or torn across, or so compressed as to lose their anatomical integrity.
The resulting symptoms are those common to all nerve injuries, viz., paralysis, wasting, changes in electrical reaction of the muscles. Pain, tenderness, anaesthesia, trophic, secretory and vasomotor disturbances are also present in varying degree.
The distribution of the paralysis, atrophy, and sensory disturbance depends, of course, upon the arm nerves chiefly involved. The cuts and table will enable one to see in any case where the trouble is localized.
There are three common symptoms, however, of which it is often very important to analyze the cause. These are the loss of power for elevation of the arm and for flexion and extension of the forearm.
Flexion of the forearm is performed by the biceps and brachialis anticus, and is helped by the supinator longus. These muscles are supplied by the musculo-cutaneous nerve, except the supinator, which is supplied by the musculo-spiral. Hence when a person can- not flex the forearm, the musculo-cutaneous is chiefly affected.
Extension of the arm is done by the triceps, which is supplied by the musculo-spiral.
Elevation of the Arm Outward. — Inability to raise the arm is the common and striking symptom in combined brachial palsies. The arm is raised by a number of muscles. The deltoid acts first and most,
DISEASES OF THE NERVOUS SYSTEM.
but i: can raise the arm only to a right angle. It is supplied by the circumflex nerve from the posterior cord of the pelxus. After the arm is raised to a right angle, it is further elevated by rotating the scapula, and this is done chiefly by the middle part of the trape- zius (lower cervical and upper dorsal nerves) and by the serratus magnus, supplied by the posterior thoracic nerve. A number of other muscles combine to strengthen elevation of the shoulder, but this action can be abolished only by paralysis of the deltoid or trape- zius and serratus mangus.
Fig. 66. — Upper-arm palsy of left side.
The diagnosis of these cases involves, first, consideration of the seat of the lesion and the special nerves involved; next, that of the nature of the lesion. A recognition of the seat of the lesion and of the special nerves involved depends entirely upon the study of the distribution of the palsy and of the atrophy and sensory disturbance. It is important to determine whether one is dealing with a total-arm palsy, an upper-arm type (Erb's palsy) or a lower-arm type.
In Erb's palsy there is involvement of the deltoid, biceps, bra- chialis anticus and supinator longus, with at times paralysis of the
NEUROSES OF THE MOTOR SPINAL NERVES. 1 57
supinator brevis and even of all the muscles supplied by the median nerve. The lesion is either in the cord formed by the fifth and sixth cervical nerves or a little lower in the brachial plexus, where the fibres supplying the musculo-spiral, circumflex and musculocutaneous lie close together. At all events, the lesion involves the central parts and upper cords of the plexus. The arm hangs by the side and the forearm cannot be flexed (Fig. 66).
In the lower-arm type the triceps, the flexors of the wrist, the prona- tors, the flexors and extensors of the fingers and the hand muscles are
Fig. 67.— Lower-arm palsy and wrist drop.
involved. The arm can be raised and the forearm flexed and supin- ated, but the hand is useless and the extension of the forearm is impossible. The lesion here involves chiefly the nerves from the seventh and eighth cervical and first dorsal roots (Fig. 67).
If the lesion is in the nerve there will be atrophy, changes in elect- rical reaction, sensory disturbances, and often, if there is neuritis, pain over the nerves. The reflexes will be lessened or absent. If the lesion is in the spinal cord, symptoms in other parts of the body will be present, or, if not, there will be no sensory disturbance, as in an arm palsy from anterior poliomeylitis. In rare cases arm palsies may be caused by spinal tumors or a local meningitis, in which case diagnosis is difficult.
The upper-arm type and lower-arm type palsies are caused by much
DISEASES OF THE NERVOUS SYSTEM.
the same factors as the combined palsies; their symptoms have been indicated above. The upper-arm type is especially frequent in infants and constitutes one of the obstetrical paralyses. It may in some cases be due to injury or hemorrhage in the cord, but is usually due to a rupture of the nerves of the brachial plexus.
The prognosis in arm palsies as a whole is usually good. Nearly all these cases get well, the duration of the incapacity being from two or three months to a year. Even in the severest cases recovery is possible after one or two years. If, however, the nerves are torn across as in birth palsies and the ends widely separated, recovery is doubtful unless an operation is promptly done.
Fig. 68. — Illustrating the formation of the brachial plexus; also the involvement of the plexus in degenerative neuritis. (Leszynsky.)
The treatment consists in electrical applications, mechanical sup- port, with potassium iodide internally and abstinence from alcohol. Local injections of nitrate of strychnine are useful, and massage should be used if it can be applied carefully.
In brachial palsies due to severe injuries, dislocations, fractures, etc., in which there is evidence, from the extreme atrophy and absence of electrical reaction, that the nerve is entirely cut across and that the ends are not in apposition, a surgical operation is stringently needed. The nerves should be exposed and the ends brought as near together as possible. When this cannot be done, the nerves can be split and the ends sutured or anastomoses made, or the two ends are tied together as near as possible and placed in a tube. If the separa- tion is over an inch, not much can be expected. In all these cases, however, it must be remembered that the two ends do not unite ; but
NEUROSES OF THE MOTOR SPINAL NERVES.
159
the central end grows down in the tract of the old degenerated per- ipheral stem.
A peculiar form of combined nerve palsy sometimes occurs, due apparently to a primary brachial neuritis. It begins in the plexus and involves first the nerves of the upper cervical roots. It may extend down and involve the ulnar, median, or musculo-spiral.
Fig. 69. — Paralysis of right posterior and thoracic nerve and serratus magnus muscle.
It occurs in adult men generally and in those exposed to rheu- matic influences. Perhaps lead-poisoning may exist.
It begins gradually wich slight pains and weakness in the shoulder and arm muscles. Atrophy and anaesthesia follow, and degeneration reactions are present. There is no: a great deal of pain (Fig. 68). The disease is usually confined to one side. It lasts several months, and nearly complete recovery finally takes place. Relapses may occur.
It is differentiated from progressive muscular atrophy by the pain, anaesthesia and electrical reactions, and from arthritic atrophy by
i6o
DISEASES OF THE NERVOUS SYSTEM.
the absence of any history of arthritis and the presence of degeneration reactions and anaesthetic areas.
Paralyses of Single Nerves — Paralysis of the Posterior Thoracic Nerve — Etiology. — This fare trouble usually occurs in male adults and is due to injury or sudden strains. Its paralysis may be part of a progressive muscular atrophy. The nerve goes to the serratus magnus.
Symptoms. — When paralyzed, there is difficulty in raising the arm above the horizontal position and the movements of the
Fig. 70. — Double paralysis of circumflex nerve.
shoulder are weakened. When the arm hangs by the side the lower angle of the scapula is drawn a little nearer the vertebral column and protrudes slightly. When the arm is held ouc hori- zontally the inner edge of the scapula protrudes and is drawn toward the middle line. When the raised arm is brought forward there is a deep groove formed between the inner border of the scapula and the thoracic wall (Fig. 69).
The disease often runs a long course and is accompanied by pain.
NEUROSES OF THE MOTOR SPINAL NERVES. l6l
Paralysis of the Circumflex Nerve. — The nerve goes to the deltoid, teres minor, third head of the triceps and shoulder-joint. It gives sensation to the skin of the shoulder. It is very often paralyzed. The commonest causes are a fall or injury, dislocation and rheu- matic inflammation of the joint. The arm cannot be elevated or rotated outward (Fig. 70). There are atrophy, anaesthesia and some- times pain.
Paralysis ok the Suprascapular Nerve. — The nerve goesto the spinati muscles, teres minor and shoulder-joint. Disease of this nerve alone is rare.
The supraspinatus rotates the shoulder in, the infraspinatus and teres minor rotate it out. When paralyzed, there is an impairment of rotation and some impairment of elevation of the shoulder.
Paralysis of the Musculo-spiral Nerve (Wrist-drop, Lead Palsy, Compression Paralysis). — The distribution of this nerve is given in the table and cut. Its function is to extend and supinate the forearm, to extend the wrist and fingers, and to adduct and abduct the fingers slightly. It extends directly only the last or ungual phalanges, the first and second phalanges being extended by the ulnar nerve.
Etiology. — The musculo-spiral, owing partly to its course, is the most frequently affected by paralysis of all the arm nerves. Pressure on the nerve during sleep, especially when the patient is intoxicated, crutch pressure, fractures, wounds, tumors, lead poisoning, arsenical, alcoholic and other forms of multiple neuritis are the causes of its disordered function.
Symptoms. — The symptoms of this paralysis are "wrist-drop," due to an inability to extend the wrist or fingers. The first and second phalanges can be extended somewhat by the interossei and lum- bricales, but the last phalanges cannot be extended at all. The first finger is least affected. The fingers can be only slightly abducted, supination is generally lessened or lost; if the lesion is high up, the triceps is involved and the power of extend ing the forearm weakened. There may be atrophy of the muscles and degeneration reaction. A swelling over the tendons of the wrist-joint may occur. Some numb- j ness and tingling exist, and occasionally there is anaesthsia in the distribution of the radial nerve on the hand. The disease lasts but a few weeks if due to compression ; for months if due to neuritis, lead poisoning or severe injury of the nerve. Eventually recovery takes I place, as a rule.
When the disease is due to lead poisoning there are some peculiarities in its course. Thus the supinator longus usually escapes ; the palsy begins gradually and usually involves both arms ; it may extend to
162
DISEASES OF THE NERVOUS SYSTEM.
the upper arm. Partial degeneration reactions are present. There is rarely any anaesthesia and but little pain. Often there is a lead line on the gum and a history of constipation and colic.
| In alcoholic and other forms of multiple neurtis there are pain and paresthesia, both arms are involved, and the flexors and other forearm muscles are somewhat implicated. There are marked sensory disturbances. The legs are also affected.
In compression palsy the supinators and often the triceps are involved.
When the lesion of the nerve is high up, as in crutch paralysis, there is but little anaesthesia, and that is found on the anterior surface of }he forearm, in the distribution of the external and internal cutaneous nerves. Lesion of the nerve lower down may give rise to some anaesthesia along the radial border of the forearm and back of hand ; but the anaesthetic area varies a great deal.
Eig. 71. — Paralysis of musculo-spiral.
The diagnosis of the paralysis is easily made. The most important point is to find out the cause. The different characteristics of lead palsy, neuritic palsy and compression palsy have been indicated in the description of the symptoms. One must be sure to exclude also progressive muscular atrophy.
The treatment consists of mechanical measures, such as electricity, massage, the application of rubber muscles and in bad cases the fixation of the forearm and hand in hyperextension by means of a splint and plaster-of-Paris bandage (Gibney). Internally in the early stage it is best to administer iodid of potas sium and sulphate of magnesium (in lead palsy), the salicylates or nitrate of silver in neuritis; later, hypodermic injections of strychnin may be given. Static sparks, galvanism and other forms of electricity unquestionably do good in some cases.
Paralysis of the: median nerve is rare as an isolated trouble,
NEUROSES OF THE MOTOR SPINAL NERVES.
163
and is almost always due to injury or neighboring disease. Embolism of the axillary artery after labor or from sepsis due to any cause has produced it.
When paralysis occurs the grip is weakened. Flexion and ab- duction of the thumb and flexion of the first and second fingers
Fig. 72. — Paralysis of median.
are impaired. Atrophy of the thenar eminence may occur. The anaesthetic area varies, but is well shown in the accompanying cut (Fig. 72).
Paralysis of the Ulnar Nerve — Etiology. — The ulnar nerve is rather commonly affected by paralysis, the occurrence ranking next in frequency to musculo-spiral palsy. It is rarely affected in lead
Eig. 73. — Paralysis of ulnar.
poisoning, but is usually early involved in progressive muscular atrophy. It is sometimes attacked by a primary degenerative neuritis. Injuries are the common cause.
The symptoms are shown by the table (p. 154). The hand cannot be closed tightly, the little and ring fingers being especially weak. The
164
DISEASES OF THE NERVOUS SYSTEM.
first phalanges are drawn back and the second and third phalanges flexed ; when the interossei and lumbricales atrophy, the result is the "griffin claw" or main en griff e. The ringers cannot be adducted or abducted except feebly.
There is anaesthesia over the area of distribution of the ulnar (Fig. 73) ; there may be pain and tenderness.
Symmetrical Spontaneous Ulnar Neuritis.- — A form of neu- ritis of the ulnar nerve sometimes occurs which has certain peculiar
sequel to some wound of or operation upon a nerve. The local neuritis extends usually up the arm (ascending neuritis). It is accompanied by intense pain, anaesthesia, paralysis and atrophy. The disease is very chronic and intractable. It has been relieved in some cases by resecting the posterior spinal roots.
Morvan's Disease, Analgesic Paralysis with Whitlow. — (Neuritic type of syringo-myelia.)
This is a very rare disease, characterized by a slowly progressive paralysis and atrophy of the hands and forearms, with analgesia and painless whitlows.
It occurs usually in young or middle-aged males. Occupations, such as handling fish, which involve exposure and trauma, predispose to the disease.
characters. It develops slowly in persons with a neuropathic history, and without known exciting cause. There are first some pain and paraesthesia in the region of the ulnar nerve of one hand; this is followed by weakness and atrophy of the muscles supplied by the ulnar nerve, and characteristic de- formity appears. Anaesthesia de- velops coincidently. The other hand soon becomes affected. The disease is very chronic, and com- plete recovery is rare. It is prob- ably a form of degenerative neuritis, and is due to some infection. It simulates the Charcot-Tooth type of progressive muscular atrophy.
Fig. 74. — Morvan's disease.
Migrating neuritis is a serious but rare malady which deserves some mention. It occurs as a
NEUROSES OF THE MOTOR SPINAL NERVES. 1 65
The symptoms come on slowly, with, at first, severe pains in the arms and hands. The muscles of these parts gradually get weak and atrophy. Anaesthesia and analgesia are present. Usually there is loss of pain and temperature, but not of tactile sense. Whitlows appear which are painless, and one or more of the terminal phalanges may necrose. There is usually a swelling and hard oedema of the parts. Both upper extremities may be affected, and sometimes the feet are also slightly involved (Fig. 74). There is sometimes spinal curvature. Hysteria may complicate the affection. The course of the disease is very chronic, but there may be long periods in which the symptoms are quiescent.
Pathological examinations always show a neuritis of the parts in- volved in the disease. In some cases a syringomyelia has been found and probably a syringomyelia or leprosy are always present.
The prognosis, so far as cure is concerned, is bad, but the disease may remain stationary a long time or improve.
The diagnosis is based on the combination of paralysis, atrophy, loss of pain and temperature but not of tactile sense, and whitlows.
Strychnine seems to have stopped the progress of the malady in one case. The treatment in general is only symptomatic.
Group III. The Thoracic or Dorsal Nerves.
Anatomy and Physiology. — The dorsal nerves are twelve in number. The first is the largest and belongs functionally to the arm nerves. The dorsal nerves carry motor and sensory fibres to the voluntary muscles, skin and other tissues of the trunk wall. They also carry splanchnic fibres to the lungs and abdominal viscera. They divide into anterior and posterior branches. The anterior form intercostal nerves, of which the first six are distributed to the wall of the thorax and the last six to the wall of the abdomen. All these nerves give off lateral and anterior branches. The posterior branches of the dorsal nerves are small and supply the muscles and skin of the back.
Besides the above, there are recurrent branches which enter the spinal canal and supply the intraspinal veins and membranes. The first six dorsal nerves contain fibres which enter the sympathetic and supply the pleura and lungs. Fibres from the last six enter the sympathetic and supply the pelvic viscera. The intercostal nerves in their course lie part of the way beneath the pleura ; they also pass along in close company with the intercostal artery and vein, lying just above these in the groove on the internal surface and inferior border of the ribs.
i66
DISEASES OF THE NERVOUS SYSTEM.
The upper six dorsal nerves, including both branches, are mainly inspiratory in function. They also extend and rotate the dorsal and cervical vertebrae. The lower dorsal are expiratory nerves ; they also assist in compressing the abdominal viscera, and in flexing, extending, and rotating the spine.
Motor Neuroses. — The thoracic motor nerves are mainly in- volved in respiratory cramps and paralyses ; sneezing, coughing, laugh- ing and crying are symptoms in which they play a large part. In complete respiratory paralysis also these nerves are affected. But there are few motor neuroses that are limited to the thoracic nerves. The neuroses of these parts are mainly sensory and will be described later.
Group IV. The Lumbar Nerves.
Anatomy and Physiology. — The lumbar nerves are five in number. The posterior branches supply the erector spinae, interossei, multifidse spinae and interspinales, and also the skin of the back. The anterior branches of the upper four unite to form the lumbar plexus. The fifth or lumbo-sacral nerve sends most of its fibres to the sacral plexus. The branches of the lumbar plexus are: (i) the ilio-inguinal, (2) ilio- hypogastric (from first lumbar), (3) geni to-crural, and (4) external cutaneous (mainly from the second), (5) obturator (from third and fourth lumbar), (6) the anterior crural (from second, third and fourth— Plate II).
The first four branches of the plexus are comparatively short and supply sensation to the abdominal wall and external genitals. The last two are longer and supply the hip and knee-joints, the muscles of the anterior inner and outer part of the thigh, the skin over this region and the inner side of the leg and dorsum of the foot (Figs. 30 and 31).
The diseases of the lumbar nerves and plexus so far as they form independent disorders are mainly the neuralgias. In making a diag- nosis of lumbar-nerve disease, one should remember that of the six branches of the plexus the upper four are mainly sensory, the lower two mixed nerves.
Paralyses and spasmodic troubles of the lumbar nerves are not rare, but are usually symptomatic of some extrinsic and often serious disease.
Etiology. — Hence it is well to catalogue here those affections which may produce lumbar palsies or spasms. They are pelvic tumors or injuries, impacted faeces, caries of the spine, psoas abscess, obtu- rator hernia, hip disease and pressure of the foetal head.
Symptoms. — When the upper two lumbar nerves are involved, only sensory symptoms in the distribution of their branches occur If the next two are also involved, there may be trouble in extend- ing the leg and flexing the hip on the trunk. The patient cannot
NEUROSES OF THE MOTOR SPINAL NERVES.
167
raise himself to a sitting posture. If there are irritation and spasm, the thigh is drawn up and adducted.
In paralysis of the obturator nerve there are loss of power to adduct the thigh and cross the leg and weakness of outward rota- tion of the thigh. Anaesthesia over the inner side of the thigh may be present.
In paralysis of the anterior crural nerve there are weakness of the muscles of the anterior region of the thigh, loss of power of extend- ing the leg and anaesthesia or pain over the crural area.
Paralysis of the posterior branches of the lumbar nerves causes weak- ness or paralysis of the erectors of the spine. The lumbar curve is very greatly exaggerated, the shoulders being thrown far back and the belly protruding. This condition occurs in progressive muscular dystrophy, particularly in the pseudo-hypertrophic form.
Group V. The Sacrae Nerves.
Anatomy and Physiology. — The sacral nerves are five in number. The first four divide into anterior and posterior branches. The fifth has no anterior branch. The posterior branches escape through the posterior sacral foramina and supply the multifidus spinae and the skin over the sacrum and coccyx. \
The first three anterior branches, with the lumbo-sacral nerve and a branch from the fourth sacral, unite to form the sacral plexus. This lies upon the pyriformis muscle in the pelvis, and escapes at the lower part of the sacro-sciatic foramen. The great mass of the fibres go to make up the sciatic nerve.
The roots of origin of the sacral and coccygeal nerves form the cauda equina.
The branches of the sacral plexus are the superior gluteal, muscular, small sciatic, inferior gluteal, pudic, great sciatic, perforating, cutaneous and articular. These are distributed to the muscles, skin and joints of the buttocks, thighs, legs and feet. Their distribution is shown in Figs. 30 and 31.
The sacral nerves are the main agents in station and locomotion. They control the legs entirely; also the posterior muscles of the thigh and buttocks; they give sensation to these parts. They carry also fibres that regulate the sexual function, bladder and rectum. From the sacral portion of the cord there is an outflow of nerves to the sym- pathetic, thence to the pelvic organs.
The diseases of the sacral nerves may be classified in a similar way to those of the brachial plexus.
Spasmodic Disorders op the Sacrae Nerves. — Tremor, rigidity, clonic and tonic spasms, myoclonus, athetoid movements, all affect the lower extremities, but they are almost invariably part of some general\)r"central disorder, such as chorea, paralysis agitans, hysteria,
DISEASES OF THE NERVOUS SYSTEM.
etc. Under the head of occupation neuroses there occur certain rare spasmodic troubles special to the legs. Saltatory spasm in- volves the legs alone. These disorders are, however, general neuroses.
Peripheral Leg Palsies. — Paralyses of the lower limbs from disease of the nerves may be either combined or single, just as is the case with arm palsies.
A combined sacral palsy is one in which all or nearly all of the branches of the sacral plexus are involved.
Etiology. — Such palsies are due to much the same causes as those affecting the lumbar nerves, viz., injury, dislocation, hip disease, tumors and neuritis. Hysteria may cause a functional sacral palsy.
Symptoms. — The symptoms are indicated by a study of the dis- tribution of the nerves, varying, however, in degree. The foot cannot be moved; the leg can be slightly extended by the anterior crural, but not flexed; the thigh cannot be drawn back freely or rotated perfectly. There is anaesthesia over the distribution of the sacral nerves ; pain may be present ; wasting and vasomotor and sec- retory disturbances occur unless the paralysis is functional.
The course depends on the severity of the lesion. If the nerve is totally cut or torn across, it may require one or two years for perfect recovery, which, however, occurs if the severed ends are properly approximated.
The diagnosis of a sacral palsy is based on the history and on the distribution of the anaesthesia and of the muscular paralysis. The sacral nerves do everything for the lower limb except extend the leg, flex and adduct the thigh, and to some extent rotate it. They supply sensation equally extensively /(see Figs. 30 and 31).
The distinction from spinal-cord disease is chiefly based on the unilateral symptoms, the absence of disorder of the sphincters and the combination of paralysis, wasting and sensory troubles in the course of the sacral nerves.
Single-nerve Sacral Palsies. — The symptoms of paralysis of single nerves are indicated by their function. The nerves rarely affected are the superior gluteal, muscular and small sciatic. The nerve oftenest affected is the great sciatic, and especially its anterior tibial branch. In the latter case a condition called "drop-foot" is produced.
In the pathology and treatment of sacral palsies there is nothing especial that can be said.
CHAPTER X.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES.
As the most common disorders of the sensory nerves are neuralgias and paresthesias, I shall introduce the subject with a general de- scription of these symptoms.
PARESTHESIA.
Local Paresthesia Acroparesthesia, Waking Numbness.
The condition known as paresthesia is one which should be more familiar to physicians and be more commonly recognized and under- stood. Paresthesia is the name given to a number of subjective sen- sations, such as prickling, numbness, creeping sensations, tickling and burning. It includes, in fact, nearly all the subjective sensa- tions of the skin, except those of pain. It is a condition which is, therefore, extremely common, and in its mildest and most trivial character is much more often experienced than pain. When these sensations fix themselves in certain locality, following perhaps the tract of the nerve, or fastening themselves upon the hand or foot, they take on a certain clinical type, and deserve to have the name of a disease, to just the same extent that a neuralgia does. Pares- thesia, in almost all cases, implies simply a lower grade of irritation of the nerve-fibres that occurs in neuralgia, and is a kind of ghostly simulacrum of that disease. It very often precedes or accompanies attacks of pain. There is sometimes a tingling of the teeth or burn- ing in the face which has a shadowy likeness to a toothache or tri- geminal neuralgia. In the same way, one finds paresthesias affecting the head, causing sensations of pressure and constriction, of burning, and general undefinable discomfort, which are entirely comparable to headaches.
In conditions of neurasthenia, paresthesias of the head are more common even than the headaches. Paresthesia sometimes follows the course of a nerve, as when one feels numbnesss of the hand if the ulnar is pressed upon at the elbow, or numbness in the foot where the sciatic is pressed upon, as when the legs are crossed.
169
170
DISEASES OF THE NERVOUS SYSTEM.
There is also paresthesia affecting one of the intercostal nerves or one of the crural nerves. On the other hand, paresthesia may affect all four extremities, so that they feel entirely benumbed or prickling. There is, I repeat, a very close analogy between these groups of paresthesias and neuralgias.
Etiology. — Paresthesia, whether local or diffuse, occurs rather more often in women than in men, and rather more often in the mature and middle-aged than in young people. It is most frequent in women of middle age, especially in those who are accustomed to hard work with the hands in washing and the exposure incidental to this. It also occurs in those who are obliged to walk a great deal and to be upon their feet, and it especially affects tailors, seamstresses, bookbinders and those who have to use their hands constantly in some skilled mechanical work. It sometimes occurs in old age, being accompanied by evidence of gout or glycosuria in some cases. It is associated with rheumatism (twelve per cent, of cases) and with alcoholism (eight per cent, of cases). It follows infections like typhoid fever and the grippe, and means in these, as in many other cases, a low grade of neuritis. As a rule, however, paresthesia is a neurosis in which exposure, rheumatism, alcohol, arterial sclerosis, and lithemia are the causes.
Paresthesie which at first are due to some direct or reflex nerve irritation may become a habit, and form a part of a psychosis. Par- esthesie are very common in melancholia and hypochondria.
Paresthesia affects single cerebro-spinal nerves just as neuralgia does, or it may be more generally distributed. In the latter case it affects most the feet and hands, and it is called acroparesthesia.
We meet then with :
1. Cephalic paresthesias, comparable to diffuse headaches.
2. Local paresthesias, comparable to local neuralgias.
3. Acro-paresthesia, involving the feet or hands or both diffusely.
4. Psychic paresthesias.
The cephalic paresthesias are usually symptoms of neurasthenic or lithemic states and will not be considered here. Among eighty-five cases of local and acro-paresthesie, not symptomatic of other and organic nerve disease, I found that there were of the local forms thirty- five cases, of acro-paresthesia fifty cases. The local paresthesias affected the arms in eighteen cases, next the thigh and leg nerves in twenty cases, and, last, the trigeminal nerve in three cases.*
*The following analysis of eighty-five cases of paresthesia occurring in my practice shows something of the cause and local development of the malady. The most frequent causes I find to be those concerned with occupation. Paresthesia, in its general mani-
SENSORY NEUROSES OE THE CEREBROSPINAL NERVES. 171
Symptoms. — The patients have feelings of numbness, prickling, coldness, heaviness, usually in both hands, extending a little way up the forearm and often also in the feet. The sensations are, as a rule, diffuse, but may follow the distribution of a nerve, especially the ulnar. In other cases the numbness is sharply limited to the finger tips or the second and third phalanges. There is no actual pain or tender- ness, nor is there any decided anaesthesia, cutaneous or muscular. In- stead of or with the numbness there may be burning sensations, especially felt in the palms or soles. There is often a slight degree of paralysis, but rarely any decided vasomotor or trophic changes. The symptoms exacerbate, being worse at certain periods of the day, usually in the morning. They may increase at night and prevent
Males. Females. Total.
Hands alone
General sensations
|
6 |
11 |
17 |
|
6 |
12 |
18 |
|
10 |
10 |
20 |
|
4 |
4 |
|
|
14 |
12 |
26 |
|
36 |
49 |
85 |
The special nerves affected were:
Trigeminal
Brachial
Ulnar
Radial
Crural
Peroneal
Sacral 1
Sciatic 1
Plantar 11
35
sleep. The arms are oftener affected than the lower extremities; and the symptoms never except in hysteria take a hemiplegic form, though one arm may be alone involved. The scalp and ears may be involved, though always in a minor degree, the patient complaining of sensations of heat, prickling, flushing and vertex pressure. In the local forms, there is numbness along the ulnar nerve or in one arm or along some branch of the lumbar nerves (meralgia). The symptoms cause a condition of mental unrest and nervousness which add to the sufferings materially. Evidences of over-work, of dyspep- sia and constipation, of anaemia or of muscular rheumatism will gen- erally be found. The urine is usually rather light in color and specific
festations, may be considered almost an occupation neurosis put down as follows:
Occupation 15
Rheumatism 10
Alcoholism 6
Infection 6
Senility 6
Among 85 cases there were 36 males and 49 females.
Cases of organic, spinal and'cerebral disease are not included,
The list of causes may be
Reflex irritation 2
Hysteria 3
Climacteric change 2
Various causes, such as neurasthenic
state, hypochondria, etc 12
172
DISEASES OF THE NERVOUS SYSTEM.
gravity (1.010 to 1.018), slightly cloudy, with excess of phosphates and occasional excessive discharges of urates. Under the head of parsesthesic neurosis, the affection known as waking numbness or night palsy may be described.
This is a disorder characterized by a temporary paralysis of an extremity, with numbness, noticed on first waking or after lying down for a time.
It is a rare disease and little is known of its cause. It occurs in adults and usually in the neuropathic. Sometimes evidence of weak heart or poor innervation of the vessels is present.
The symptoms are much like those caused by temporary compres- sion of a nerve when the leg or arm goes to sleep. The paralysis is temporary and there is no anaesthesia. It is often a very obstinate condition but leads to no serious result.
Pathology. — The pathology of paresthesia is based upon the sim- ilarity of the symptoms to those in mild or incipient cases of neuritis and upon the effects of drugs in relieving symptoms. It is believed that the peripheral nerves are being irritated by some poison circu- lating in the blood. Cooperating with this are nerves naturally over-irritable, or made so by anaemia, hyperaemia or exposure to cold. In some cases there is undoubtedly a low grade of neuritis, and in other cases there is a congestion or slight degree of degeneration of the nerve. Underlying many of the paraesthesias of middle and late life is an arterial sclerosis. Many of the cold and heat sensations of this period have this vascular origin, to which is added an undue mental irritability and sensitiveness.
The disorder is rarely recognized by the symptoms described. The diagnosis is mainly an investigation into the cause. Waking numb- ness and night palsy are intermittent disorders, disappearing within a short time after waking or rising from a recumbent posture. The numbness that is felt with digiti mortui and Raynaud's disease is accompanied by spasm of the blood-vessels and whitening of the fingers. In multiple neuritis there is usually some distinct anaes- thesia, pain or tenderness and also motor weakness. In arterial sclerosis we find evidences of thickening of the vessels, with tendency to spasm and often abnormal degrees of arterial tension. Tabes, rheumatism, gout, diabetes and alcoholism must be looked for.
Prognosis and Course. — The disease, if not treated, runs a course of several months, with some intermissions and relapses. It may disappear, to return the next year. It never progresses to any serious condition, and is in almost all cases, except in the aged and in diabetes eventually cured.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 73
Treatment.— The patient should receive alkalies, with bromides, iodides, pepsin, iron and strychnine. A low proteid diet, with exclusion of alcohol, coffee and tea, must be prescribed. The faradic current, mas- sage and stimulating liniments are useful. Rest and removal from exciting causes, such as exposure and special work, and life out doors are also indicated.
NEURALGIA.
Neuralgia is a condition characterized by pain in the course of a nerve or of nerves. It is not a distinct disease, but only a symptom, and the tendency is to do away with the term except to indicate a nerve pain. A large proportion of what were formerly called neural- gias are due to neuritis, to some form of organic disease or to some reflex irritation. There is, however, a certain percentage of per- sistent nerve pains for which we can find no organic basis, and as a matter of both necessity and convenience we still retain the word. We qualify it according to its cause and its location. Hence we have hysterical, gouty and neuritic neuralgias, and we have trigeminal, cervical, brachial, etc., neuralgias, and we can speak of neuralgias as symptomatic or idiopathic according as we know or do not know the basis of the trouble. But there is little if any idiopathic neuralgia for the observant physician.
The most frequent form is the trigeminal ; next in order come the sciatic and brachial.
Etiology. — -Neuralgia never affects young children, and, eaving out migraine and headache, it is rarely met with before the fifteenth year. From that time until twenty-five the frequency very rapidly increases. About one-fourth of all cases occur between the ages of fifteen and twenty-five; the relative number then slowly decreases to the forty- fifth year, when there is a rapid fall. Neuralgia is very rare in old age.
Women are more affected than men, in the proportion of five to three (analysis of 887 cases). In New York most cases occur in winter, next in the autumn. More cases occur in temperate climates and in wet and cold regions.
Hereditary influence, neurotic constitution, anaemia and debility, gouty and rheumatic diatheses, all predispose somewhat to the disease. The exciting causes can be included under the head of toxic agents, infections, exposure, over-exertion, emotional shock, injuries and neuritis of low degree.
Local irritation of nerves may excite neuralgia, direct or reflex in kind. Some help in recognizing the reflex origin of neuralgias and other pains may be gained from the accompanying diagrams.
174 DISEASES OF THE NERVOUS SYSTEM.
Symptoms. — These will be described in detail in the chapters on the special forms.
The dominant symptom is, of course, pain. This pain is sharp, darting, boring, stabbing or burning in character. It comes on in
Fig. 75. — Diagrams showing the distribution of the cerebrospinal strands of nerves and the location of transferred pains and neuralgia.
|
Strands of Cerebrospinal Nerves. |
Distribution. |
|
|
Area I Area III Area IV Area V Area VI Area VII |
Trigeminus, facial, etc. Upper four cervical. Lower four cervical and first dor- sal. Upper six dorsal. Lower six dorsal except last. Twelfth dorsal, four lumbar. Fifth lumbar and five sacral. |
Face and its orifices, anterior scalp. Occipital region, neck. Upper extremities. Thoracic. Abdominal wall, upper lumbar, up- per lateral thigh surface. Lumbar region, upper gluteal, an- terior and inner thigh and knee. Lower gluteal, posterior thigh, leg. |
paroxysms of great intensity. In the intervals there may be no pain or it may be simply a dull ache. The pain runs along the course of certain nerves, though it is not confined necessarily to them, but may be somewhat diffuse. Pain is increased or brought on by cold or heat or pressure on the affected part.
f
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 75
The skin and nerves are sometimes tender or even exquisitely sensi- tive. Firm pressure, however, is usually not painful. In about half the cases of long standing, tender points may be found which correspond to the exit of nerves from a bony canal or the substance of a muscle or fascia. In rare cases there is tenderness over the spine correspon- ding to that point where the affected nerves arise. Besides feelings of pain, there is often a sense of numbness cold, tingling , or heaviness of the limb. Vasomotor, secretory and trophic disturbances may occur; but when these are pronounced one must suspect neuritis or an organic central disease. Muscular spasm is sometimes present more rarely there is some muscular weakness. The paroxysms of pain may intermit regularly; sometimes they come on every day at the same hour. They are apt to be worse at night. The attacks of the disease often run a course of several weeks or months, and in some forms they last for years.
The painful sensations of neuralgia usually originate in the peripheral sensory neuron. In some cases as in the neuralgia of tabes and perhaps sometimes in tic douloureaux and in the herpetic neuralgias, the posterior spinal ganglion is chiefly at fault; in others the irritation affects the entire sensory nerve. The central sensory neurons that carry impulses to the brain are rarely the cause of neuralgia, and local diseases of the cord and brain do not, as a rule, cause pain by irritating these sensory pathways. Still there may be neuralgic pain from this cause; and "central nervous pains" have been observed in brain tumors and after brain hemorrhage or softening, when these lesions have involved the posterior part of the optic thalamus.
Pathology. — Many cases that used to be called neuralgia are now known to be forms of neuritis or perineuritis; e.g., sciatica and brachial neuralgia. Other forms are sometimes due to impaired nutrition of the neuron from an obliterating arteritis (tic douloureux) ; still others are due to the irritation of nerves from the diathetic poison of gout, rheumatism and diabetes, or to extrinsic poisons, such as alcohol, lead and arsenic. In these cases the sensory nerves of the nerve-sheaths (nervi nervorum) are affected. There remain many cases in which the trouble shifts from one locality to another or in which no special local or general irritation can be discovered. In these cases we assume that the pathogenic focus is in the spinal or cerebral sensory neurons and in a toxaemia.
There are some forms of neuralgia which may be called "reminis- cent" or " hallucinatory." The patient, who is an impressionable and sensitive person, has had a genuine cause for neuralgic pains; but
176
DISEASES OF THE NERVOUS SYSTEM.
this, after lasting some time, has ceased, while the painful impres- sions continue to remain in the cerebral cortex. The neuralgia is a morbid habit of feeling pain. Such neuralgias are promoted often by the use of morphin. There are neuralgic pains occurring oftenest in the legs, but sometimes in the back, shoulder or arms, due to spasms of thickened arteries and causing pain just as the pain of angina pectoris is caused. These are called anginal neuralgias. They are often accompanied with cramping and weakness of the muscles.
Pathogenetically we can divide neuralgias into (1) toxic; (2) exhaustion; (3) neuritic and perineuritic ; (4) anginal; (5) psychic. Often there is a combination of these factors.
Diagnosis. — This is based on the fact that neuralgic pains are shifting, paroxysmal, follow the course of nerves, are accompanied often by tender points and not accompanied by signs of organic nerve disease, such as paralysis, anaesthesia and tenderness over the nerve-trunk. Thermic sensations of burning or cold are rarely neu- ralgic, but are due to neuritis.
Prognosis is good, except for the reminiscent neuralgias, the neuralgias of hysterical and neurasthenic persons who are thoroughly anaemic, dibilitated, aged and broken down morally and physically. The neuralgias of the degenerative period of life are also very obstinate.
The treatment will be discussed under special heads.
Neuroses of the Nerves of Special Sense. The Olfactory Nerve.
Anatomy. — The olfactory nerves consist of a number of peripheral fibres that arise from the olfactory bulb, pass through the cribriform plate of the ethmoid bone, and are distributed to the mucous mem- brane of the superior and middle turbinated bones and the upper part of the nasal septum. The olfactory bulb is with its associated parts really a subdivison of the brain (rhinencephalon) and not a true nerve. For many reasons the anatomy of the rhinencephalon has a peculiar interest. It is developed from a secondary division of the first cerebral vesicle. It includes in man, from before backward, the bulb and peripheral nerves, the olfactory tracts, and their lateral roots with the gray root or trigonum between them, the hip- pocampal convolution and cornua ammonis, part of the convolu- tion of the corpus callosum, the nerves of Lancisi, and the anterior commissure. Recent studies of the anatomy of the olfactory bulb and its nerves show that this apparatus is very analogous to that of the retina. (Fig. 77.) Beginning with the nerve-cells of the nasal mucous membrane, it is found that these are bipolar, and are analogous to the cells of the posterior spinal ganglia; they send peripheral processes between the epithelial cells, and these processes receive the olfactory
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 77
stimulus; the other and central processes pass up through the ethmoid bone to the bulb. These form the so-called nerves of smell. They end in fine expansions which unite with similar terminal expansions from a deeper layer of cells. The tangle of fibres form, what are known as the glomerular bodies. Next is a layer of peculiar cells called, from their shape, mitral. They have neuraxons which become part of the olfactory tract. Deeper still is a layer of granular and central fibres, the former being peculiar in having no axis-cylinders. These cells resemble the spongioblast cells of the retina and are associative in function. The central olfactory fibres, which are now neurons of the third order — that is to say, they are are the third from the peripheral olfactory cell — pass to the hippocampus, cornu ammonis, and convo- lution of the corpus callosum. Through cells in these areas they are put in connection with the optic thalamus and with the motor tracts. The olfactory nerves do not decussate.
The sense of smell is rudimentary in man, yet it is still the sense by which we can appreciate the most attenuated matter; for the trillionth of a grain of mercaptan is able to awaken a sensation in the mind, but has weight and dimensions so infinitely minute as to be quite beyond the power of the imagination to grasp. According to Valentin, we can perceive T2Wo o"o °f a grain of oil of roses. Accord- ing to Fischer and Penzoldt, one can perceive -otg o to o o o o °f a grain of mercaptan. I have found that one can perceive the odor from 4 cm. of a solution of oil of cloves, 1 to 100,000. Matter to be per- ceived as odor must be in a gaseous form. Odorous sensations co- operating with taste sensations form "flavor." Variety in odorous sensations depends probably upon the rapidity of molecular vibrations as in the case of light; and substances having similar relations in vibration have similarity in odor (Haycraft). Males have a more delicate sense of smell than females (Nicolls and Bailey). The keen- ness of the olfactory sense is lessened in the insane and criminal classes.
Fig. 76. — -The olfactory nerve.
i78
DISEASES OF THE NERVOUS SYSTEM.
Anosmia. — The olfactory nerve is affected clinically by loss of function, or anosmia, increased sensitiveness of function, or hyper- osmia, and perversions of function, or parosmia.
Anosmia is far the most common disorder of olfaction.
Etiology. — Its usual cause is disease of the mucous membrane of the nose. Injuries, inflammations and tumors affecting any part of the course of the nerve, its bulb or central fibres may also cause it. Unilateral cortical lesions in the uncinate gyrus may lead to partial
Fig. 77. — Principal constituent elements of the olfactory nerve of a mammal. (Van Gehuchten.)
loss of smell. It will be not entire, because each nerve receives fibres from both hemispheres. Paralysis of the fifth or seventh nerve may indirectly cause some anosmia. Primary degenerative changes due to syphilis of the nervous system, as in locomotor ataxia, and excessive olfactory stimulation cause anosmia. Anosmia occurs sometimes as a pure psychosis in hysteria, melancholia or in neurasthenic states. There may also be a congenital absence of the nerves.
Diagnosis. — This is made by test odors. To test the sense of smell, a bottle of oil of cloves or of some familiar non-irritating odor
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 79
may be used. To detect quantitative disturbance one may use six phials containing oil of cloves, in purity and in watery mixture of 1 to 10, 1 to 100, 1 to 1000, 1 to 10,000, and 1 to 100,000. Special olfactometers have been devised. The sense of smell for any single odor is lost in about three minutes, but returns after one minute's rest.
Treatment. — For functional anosmia, snuffs containing strychnin gr. -sVand gum acacia 5ij can be used. Weak galvanic and faradic currents are recommended. Usually there is in anosmia a local diseased condition of the nose which requires treatment.
HypKrosmia occurs only rarely and then in neurasthenic, hys- terical or insane persons. In the latter it is more often a psychical phenomenon than a peripheral disorder. Hyperosmia can be culti- vated, and this is done sometimes by the blind and by those engaged in certain pursuits, such as tea tasting and wine tasting.
Hallucinations of smell occur in the insane, as just mentioned, and a few cases of epilepsy are reported in which the aura was a stench. When all olfactory sensations are disagreeable the condition is called kakosmia.
Parosmia is a not infrequent condition. In it everything smells alike to the patient, and the odor smelled is perhaps a peculiar or offensive one. This condition may be due to local disease, but is often a symptom of hysteria, or other psychoses.
The Optic Nerve.
Anatomy. — The optic nerve is not a true peripheral nerve, but a tract of the brain, and it connects the retinal cells with the brain proper. Tike other brain tracts, its fibres have a myelin sheath, but no neurilemma. The real origin of the nerve is in the retina, just as the olfactory nerve arises in the peripheral cells of the olfactory mucous membrane and the spinal sensory nerves arise in the spinal ganglia.
The retina is a nervous tissue formed essentially of three layers of nerve-cells. From without inward they are: the layer of visual cells, the layer of bipolar cells and the layer of ganglionic cells. This subdivision is shown in the diagram (Fig. 78). These different layers may be subdivided so as to give the following layers from without in- ward:
1. The layer of rods and cones. 1 r ^ , . , „
2. The external granular layer. ) ForminS the layer of visual cells.
3. The external molecular layer. \ ^ . ^ , r , . , „
4. Internal granular layer. / ForminS the layer of bipolar cells.
5. Internal molecular layer. \ Forming the layer
6. Ganglionic layer, with the fibres of the optic nerve. J of ganglion cells. *
The layer of visual cells is subdivided, as seen in the figure, into that of the rods and cones externally and that of the external granular internally. This is, however, practically a layer made up simply of
i8o
DISEASES OF THE NERVOUS SYSTEM.
bipolar nerve-cells with prolongations more or less long which run to the external surface of the retina and there form a series of bodies known as the rods and cones.
In the layer of bipolar cells are layers of cells with processes running horizontally, and in the internal molecular layer are larger horizontal cells, called by Cajal spongioblasts. There are also in the retina termi- nal arborizations of cells whose origin is in the thalamus, corpora geniculata, or anterior tubercles. These carry impulses to the retinal cells (Fig. 78, s).
Fig. 78. — Transverse section of a mammalian retina. A, Layer of rods and cones; B, bodies of visual cells (external granular); C, external molecular layer; E, layer of bi- polar cells (internal granular); F, internal molecular layer; G, layer of ganglionic cells; H. layer of optic-nerve fibres; a, rod; b, cone; c, body of the cone cell; d, body of the rod cell; e, bipolar rod cells; /, bipolar cone cells; g, h, i, j, k, ganglionic cells ramifying in the various strata of the internal molecular zone; r, inferior arborization of the bipolar rod cells, connecting with the ganglionic cells; rx, inferior arborization of the bipolar cone cells; t, epithelial or Muller cells; x, point of contact between the rods and their bipolar cells; z, point of contact between the cones and their bipolar cells; s, centrifugal nerve fibre. (Cajal.)
The neuraxons of the ganglionic cells send fibres which unite to form the optic nerve.
The optic nerves each contain about 500,000 fibres which is about the same as the total of all the sensory fibres of the spinal nerves. They pass to the optic chiasm, where about one-third of the fibres cross, in man. In lower animals the decussation is greater. Those fibres which do not cross come from the outer or temporal third of the retina; those which do cross come from the internal or nasal two-thirds. A few fibres pass from one optic centre of the brain along the posterior border of the optic chiasm to the centre on the opposite side (commis- sure of Gudden). After leaving the chiasm, the fibres form the optic tract. The tract curves up and back around the crus cerebri, and divides into a lateral and mesial root.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. l8l
These roots connect with the external geniculate body, the an- terior tubercles of the corpora quadrigemina and the posterior ganglion of the thalamus, i. e., the pulvinar. These ganglia are called the primary optic centres. Through the anterior tubercles of the corpora quadrigemina and by other means, the optic nerve is connected with the oculomotor nerve, and thus reflex movements of the pupils, lids and eyeballs are brought about.
From these primary optic centres, fibres enter the posterior part of the internal capsule, curve up and back toward the occipital lobes, forming the optic radiations of Gratiolet. The inferior longitudinal bundle is part of these radiations and connects the external geniculate body with the calcarine cortex (A. Meyer).
They are finally distributed to the cortex of the occipital lobe, and in man chiefly to the cuneus and the parts about the calcarine fissure.
It will be seen that each retina is connected with the occipital lobe of both hemispheres; further, that the outer or temporal half of each retina is connected with the occipital lobe of the same hemis- phere, and the inner or nasal half of each retina with the occipital lobe of the opposite side. The upper part of each retina seems to be connected with the lower part of the cuneus, and vici versa (Fig. 79).
Other connections exist by which the optic centres on the two sides are connected with each other and with other cranial nerves in the medulla.
The optic nerve is a nerve of special sense of vision and has no other function except that of an excito-reflex character.
Diseases of the Optic Nerve.
The optic nerve may be affected by nearly all forms of pathological change. For the neurologist, however, the especially important con- ditions are inflammations, degenerations, injuries and functional dis- sorders. Inflammation of the nerve, or optic neuritis, may occur as a papillitis or inflammation of the head of the nerve, a neuro-retinitis or descending neuritis, or a retro-bulbar neuritis. Perineuritis is rare. Neuro-retinitis and papillitis are closely associated clinically and pathologically (Noyes), so that practically only two forms of neuritis need be discussed separately.
Papillitis and Neuro-retinitis — Etiology. — This condition is seen by neurologists in connection with brain tumors, brain abscess, meningitis and occasionally multiple neuritis. The other causes are nephritis, diabetes, infectious fevers, lead, and severe hemorrhages.
182
DISEASES OF THE NERVOUS SYSTEM.
The disease occurs at all ages and in both sexes. In brain tumors it occurs in two-thirds of the cases, and especially often in cerebellar tumors. It occurs in eighty per cent, of cases of tuberculous meningitis.
Symptoms. — Subjective symptoms are often not present. The vision may remain good for a long time. In other cases there are concentric limitation of the vision field, loss of color sense, and scotomata. For a description of opthalmoscopic changes the reader
Fig. 79. — The optic nerve and its central connections (modified from Obersteiner)' R, Retina, dark on the side connected with left hemisphere; No, optic nerve; Ch, chiasm; Tro, optic tract; CM, Meynert's commissure; CG, Gudden's commissure; Tho, thalamus; Cgl, corpus genie, ext.; Qa, corpor. quad.; Ss, optic radiations; Co, occipital cortex; Lm, mesial lemniscus. (The shading of the retina and visual field should just reach the vertical lines..)
is referred to a special text-book. It is in this condition that "choked disc," which is a papillitis with much serous infiltration, occurs. The changes are less striking in neuro-retinitis. The disease may affect one or both nerves. In brain disease both nerves are usually involved.
Pathology and Pathological Anatomy. — The process is usually sub- acute or chronic. Congestion, exudation, small hemorrhages and collections of leukocytes occur. The sheath of the nerve just back
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 83
of the globe is often distended with a serous exudate. After a time the nerve-fibres atrophy, connective tissue proliferates and takes their place, and we have a secondary optic atrophy.
The process is essentially peripheral, but it extends back with lessening intensity into the trunk of the nerve. The purely mechan- ical theory of neuritis, that is due to compression, can be accepted only in some cases. It is probable that the neuritis results from an irritating serous fluid which extends down the sheath of the nerve, this sheath being a prolongation of the arachnoid cavity. Mechanical causes lead to constriction, accumulation of the fluid, and compression
Fig. 80. — Neuro-retinitis. (Jaeger.)
of the nerve at its periphery, and hence to inflammation. Some- times, at least, the irritating fluid contains microbes or microbic poisons.
Retrobulbar NEURiTis.-=fn this^ disease the lesion lies chiefly behind the globe. Its causes are especially toxaemia from alcohol and tobacco. It is also due to rheumatic influence, syphilis, lead and diabetes.
In the acute cases there is usually rather rapid loss of sight, with some pain and tenderness. The ophthalmoscopic changes are rela- tively slight. In chronic cases, which are usually toxic in origin and due to alcohol or tobacco, or oftenest to both, there are color scotomata or absolute scotomata and amblyopia. There is no pain. The condi- tion is known as tobacco or alcoholic amblyopia.
1 84
DISEASES OF THE NERVOUS SYSTEM.
The prognosis of neuritis varies with the cause. If this is remov- able, as in the toxaemias, recovery is the rule. This is a proof that in neuritis the connective tissue is the part chiefly involved, for a destroyed or atrophied optic nerve does not recover. In many cases, however, atrophy follows the neuritis.
The treatment is based on the cause. In acute cases one may use cups, salicylates, the iodides and mercury; later, the iodides and strych- nin. Perfect rest to the eyes should be enjoined.
Degeneration op the Optic Nerve, or Optic Atrophy. — This condition may be primary or secondary. Secondary atrophy is
usually the results of a neuritis. I shall describe here primary optic atrophy.
Etiology. — It occurs oftener in men than in woman (three to one). It occurs as part of locomotor ataxia in less than half the cases. Next to the spinal cord, it occurs oftenest with degenerative dis- eases of the brain, such as multiple sclerosis and general paresis. Hemorrhages, alcoholism and lead may be causes. It may occur apparently as a simple primary affection, though these cases are generally optic forms of tabes dorsalis.
The symptoms are those of gradual decrease of acuity of vision, concentric limitation of the visual field, loss of color sense, dilatation and immobility of the pupil. The sense of sight may remain good for a long time. Ophthalmoscopically, the nerve disc is opaque,
Fig. 8 i.— " Choked disc."
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 85
grayish, or dirty-looking, and often has a cup-shaped or "cupped- disc" appearance. The vessels are smaller and few in number.
The pathology and pathological anatomy are that of a parenchy- matous degeneration with loss of nerve-fibres, which are replaced by connective tissue.
The prognosis is almost uniformly bad.
The treatment is that usually of the cerebral or spinal disease. Mercury, iodides, strychnine, phosphorus and iron are given. Warm baths and salicylate of soda sometimes have a temporarily good effect. Strychnine in physiological doses gives, however, the best sympto- matic results. Electricity is not of any use. Stretching the nerve does no good.
The optic nerves and their primary and cortical centres are sub- ject to various other diseases. So far as these are organic, they will be described in detail under the head of brain diseases. But there are certain symptoms often of functional origin which are best described here. These are: (i) Amblyopia and amaurosis; (2) retinal hyper- esthesia and dysesthesia ; (3) hemianopsia.
Amblyopia and Amaurosis. — Amblyopia is a partial loss or dim- ness of vision, there being no observable lesion of the eye, or its nerves. Amaurosis is a total loss of vision, also without observable lesion.
Etiology. — The causes are shocks, hysteria, migraine, concussion of the brain, and severe hemorrhages. There are also certain toxic causes, chiefly alcohol and tobacco, quinine, and salicylic acid. Other causes are arterial sclerosis and renal disease leading to spasm of the retinal vessels or of those of the occipital cortex. Night blindness and snow blindness are forms of functional amblyopia.
The symptoms are diminution or loss of vision, usually sudden, temporary and involving both eyes. Amblyopia in hysteria is usually greater in one eye and associated with concentric limitation of the visual field and disturbance of color sense.
Underlying amblyopia there may be minute hemorrhages in the brain, causing temporary pressure, or a vascular spasm, causing anaemia.
The prognosis is usually good.
The treatment is purely a causal one. In most cases one must ex- amine for drug poison, uraemia, diabetes, migraine, or a hemorrhage.
Retinae or ocular hyperesthesia is a condition in which the eye is abnormally sensitive to light. It may be due to exposure to extreme light or to seclusion in a dark room. The nuerologist sees it oftenest as a symptom of hysteria (vide Hysteria) and neurasthenia
DISEASES OF THE NERVOUS SYSTEM.
and perhaps in hypnotic states. It occurs in mydriasis and albinism. It is not to be confounded with photophobia due to irritation of the conjunctiva.
Nyctalopia, or the condition of seeing better in a dim light, is a form of the disease.
Hemianopsia, or half-sightedness, or hemianopia, a condition in which there is a blindness of one-half the visual field, may be due to a functional or organic disorder of the nerve or its centres. It is a symptom of many lesions and conditions, and can be described only generally here.
Etiology. — Its principal functional cause is migraine. Its organic causes are tumors, inflammations, softenings or hemorrhages in- volving part of the optic nerve or its central connections.
Fig. 82. — Showing lateral homonymous hemianopsia. This is not quite complete, there being a space on the blind side where the patient can see. (Noyes.)
Symptoms. — Various descriptive terms are used to indicate the character of the hemianopsia. In lateral hemianopsia a vertical half of the field is involved. In lateral homonymous hemianopsia there is half-blindness on the left or right side of each eye, as the case may be (Fig. 82). In temporal hemianopsia the outer halves of the eyes, and in nasal the inner halves, are involved. The upper or lower segments or irregular segments of the visual field may be involved.
These various forms of hemianopsia depend upon the location of the lesion which cuts into and destroys the optic fibres in their course from the eye to the visual centre in the occipital cortex. The mechanism will be understood when it is remembered that each occipital lobe is supplied by nerve-fibres from one-half of the retina of each eye. A cut shows this better than any description (Fig. 79). In binasal hemianopsia the lesion must be at Ch, in front of or prac- tically directly on the chiasm. In bilateral temporal hemianopsia
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 87
it must be double and at N and N'. In lateral hemianopsia the lesion must lie farther back than the chiasm, in the tract, the primary centres, the optic radiations or occipital lobes.
In hemianopsia from disease of the nerve as far back as and in- cluding the primary centres in the optic thalamus and corpora quad- rigemina there is a loss of light reflex when a ray of light is thrown upon the blind side of the retina, but the pupil still contracts when
Fig. 83. — Showing the mode of origin of the trigeminal nerve, and the relations of the motor and sensory roots, rac. cer. is the cerebral or motor root and its fibres go to the inferior maxillary branch; rac. spin, is the spinal or ascending root. The cut shows the Gasserian ganglion giving origin to the sensory fibres. (Van Gehuchten.) _ uMl
light is thrown on the sensitive side of the retina. This phenomenon is called "Wernicke's hemiopic pupillary reaction." If in hemianopsia the light reflex is preserved, the lesion is back of the primary centres and involves the optic radiations or cortex.
A test for the condition of hemianopsia in its early stage, and one that is useful in stupid or partially comatose patients, is the following : When the finger is suddenly brought in front of the eye on the sound side, there is a wink; if brought in front from the blind side, the orbicularis does not contract.
Hemianopsia is almost always the sign of organic disease. It
DISEASES OF THE NERVOUS SYSTEM.
is not found in hysteria, but does occur in migraine. It is best made out and recorded by means of the perimeter.
Its course and treatment depend upon the cause.
Sensory Neuroses of the Trigeminal Nerve.
Anatomy. — The trigeminus or fifth nerve is one of the most exten- sively distributed and most delicately sensitive nerves of the body. Its sensory branches represent the atrophied and lost sensory roots of the third, fourth, sixth, seventh and twelfth cranial nerves. The trigeminal nerve is a mixed nerve. It has two nuclei of origin: a central nucleus for the motor part and a peripheral nucleus for the sensory part. The motor nucleus has two parts: a chief nucleus lying deeply in the substance of the pons Varolii, and an accessory
Eig. 84. — Showing the origin of the cranial nerves and the extent of the origin of the trigeminus. (Edinger.)
nucleus, which consists of a long tract of gray matter, known as the descending root and lying in the upper part of the dorsal portion of the pons. It passes down along the side of the aqueduct of Sylvius. There has been much discussion as to the exact function of this root or nucleus, but the most recent studies have shown quite conclusively that it is motor in function (Kolliker, Cajal). The sensory root of the trigeminal has its origin in the Gasserian ganglion, which is com- posed of unipolar cells, like those of the spinal ganglia. The axis-cylinder processes of these cells bifurcate; the external branches pass outward and become part of the peripheral sensory route, the internal branches pass into the substance of the pons, and there give off ascending and descending branches. The ascending branches are short, and pass to a terminal nucleus, known heretofore as the sensory nucleus of the trigeminus. The descending branches pass down to the pons and me- dulla, as far as the cervical part of the spinal cord. In their course they give off terminal filaments, which come in relation with sensory nerve-cells, and the whole forms a column of matter, known as the ascending root of the trigeminus.
The nuclei of the trigeminus reach the whole length of the pons and
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medulla, and are co-extensive with the origin of all the other cranial nerves (Fig. 84). Hence the frequency with which its disorders are complicated with those of these nerves. Its cortical origin is prob- ably in the lower part of the post-central convolution.
The trigeminus supplies sensation to the face, conjunctivae, nose, the frontal and maxillary sinuses, the teeth, the palate, tongue and part of the upper pharynx; also to the scalp as far back as the vertex and to the external auditory meatus (Fig 85). The distribution is
not always the same and is helped by fibres from the cervical nerves. It gives sensation also to the anterior three-fourths of the dura mater, the falx and probably the tentorium. The pia and arachnoid are not sensitive. The posterior fossa and the occipital part of the dura mater are supplied by the vagus. The trigeminus also supplies the above-named parts with trophic, vasomotor and secretory fibres. The vasomotor fibres are brought to it, in part, from the medulla and cervical spinal cord via the sympathetic; the secretory fibres have the same origin. An exception is to be made of the lachrymal secretory fibres which are brought by the motor nerves of the eyeball. The opinion, based largely on physiological experiment, that the trigeminus sends trophic fibres to the conjunctivae and cornea is contradicted by the many successful cases of entire extirpation of the Gasserian ganglion without any inflammation of the eye following.
DISEASES OF THE NERVOUS SYSTEM.
The trigeminus supplies motion to the muscles of mastication, viz., the two pterygoids, the temporal, masseter, mylo-hyoid and anterior belly of the digastric. The sensory neuroses of this nerve are neu- ralgia, paresthesia and anaesthesia.
Neuralgias of the Trigeminus. — The trigeminal nerve is sub- ject to two types of neuralgia, viz. : (i) The symptomatic form; (2) Tic douloureux.
1. The symptomatic neuralgias are by far the most frequent.
They are called supra-orbital, infra-orbital or supramaxillary, inframaxillary or dental, and mixed forms. The most common type is the supra-orbital; next, the mixed form.
Etiology. — The female sex is oftenest affected ; most cases are seen in the first half of life ; most attacks occur in the winter and spring. The left side is oftener affected. The second and third branches of the fifth nerve are most susceptible to rheumatic influences, the first branch to malarial and septic poisons. Dental disorders naturally are frequent factors in neuralgia of the second and third branches. Anaemia, exposure, child-bearing and other depressing influences are factors in causing these neuralgias. Ocular and nasal disease may cause pain in the supra-orbital nerve. Syphilis, malaria, hysteria, epilepsy, trauma, rheumatism, all may be factors in causing trigeminal pains.
Symptoms. — The pains of trigeminal neuralgia are sharp and in- tense, with exacerbations and remissions. The pain often lasts for days without entirely ceasing. It then goes away and may not return for a long time. In supra-orbital neuralgia there is sometimes great oedema of the lids and the parts about, together with suffu- sion of the eyes. There are tender points over the course of the nerve. The tender points in supra-orbital neuralgia are supra- orbital, palpebral and nasal; in infra-orbital neuralgia, nasal, malar and gingival; in infra-maxillary neuralgia, inferior dental and tem- poral. In the mixed forms we find various combinations of the above. There is often also a tender point over the parietal eminence and vertex.
The pain may radiate to the ear or occiput or it may be located in the orbit. Dental irritation may also cause an otalgia or a neu- ralgia in the upper branches of the fifth.
There is sometimes a dilatation of the pupil, and in severe cases a reflex facial spasm occurs.
Tic Douloureux {Prosopalgia, F other gill's Neuralgia, Epileptiform Neuralgia). — Tic douloureux is a special form of trigeminal neuralgia occurring in middle or advanced life, unusually severe in its symptoms
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 191
and obstinate in its course. It ought to be distinguished sharply from the ordinary forms of trigeminal neuralgia. These latter are sympto- matic pains almost altogether ; while tic douloureux is a special dis- order, dependent upon changes in the nerve itself.
Etiology. — It occurs, as a rule, in persons who are over forty, and is seen in the very aged. It is, indeed, almost the only neuralgia which old people have. It occurs in men and women in about equal frequency. It is brought on by exposure, overwork and depress- ing influences; sometimes, perhaps, by local diseases of the teeth and jaws. There is almost always decided evidence of arterial sclerosis.
Symptoms. — It is characterized by intense darting pains, which usually start in the upper lip and by the side of the nose. From here they radiate through the teeth or into the eye and over the temple, brow and head. They are confined to one side of the head. During a paroxysm the face usually flushes, the eyes water, the nose runs and the patient assumes an expression of the greatest agony. The attack lasts for a few minutes, then becomes somewhat less, but the pain rarely ceases entirely. A breath of cold air, speaking, eating, putting out the tongue — all bring on paroxysms. The pains are worse in winter and often become less or cease during summer. Occasionally they come on for a few months every year, usually during the spring. The pains are always limited to one side of the face and are centred chiefly in one branch of the nerve oftenest in the supramaxillary and next in the inframaxillary. They may spread so as to involve the whole of one side of the face and tongue.
Spasmodic movements of the face, tongue or jaws may be asso- ciated with the pain.
Examination rarely reveals any objective trouble, but in a few cases some anaesthesia may be noted. The disease causes loss of sleep and impairment of nutrition through the pain caused by eating. Much depression of spirits naturally exists, for the pain is the worst agony that nature in its hellish mood could devise. There is a tendency to a cure, however, and many patients get well spontaneously in from seven to fifteen years.
Pathology. — The disease is usually a degenerative one, and prob- ably is due to irritative and atrophic processes occurring in the nerve and its ganglion. A low grade of neuritis, perhaps from alveolar disease, has been found sometimes, but as a rule the nerve does not appear much changed. The arteries supplying the nerve, how- ever, often undergo the changes of endarteritis, their calibre is much lessened, and the nerve cannot get its proper supply of blood. Thus an obliterative arteritis underlies some cases of the disease. The
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DISEASES OF THE NERVOUS SYSTEM.
ganglion shows sometimes evidences of degenerative changes, but these must usually be considered secondary, for division of the nerve almost invariably stops the pain for one or two years at least. On the whole, the most satisfactory view of the pathology as, I first showed, is that the neuralgia is due to an obliterating neuritis causing a defective nutrition of the nerve and accompanied with vascular spasm. Tic douloureux is a kind of trigeminal angina.
Some cases occurring in younger people are explained by the pres- ence of distinct local disease and a hysterical pain habit.
Treatment. — In cases which occur in old people, the use of nitro- glycerin given in doses of gr. ^io q- 2 h. sometimes has a happy effect. A very good remedy is crystalline aconitia given in doses of 21)0 gr. until its physiological effect is obtained. Besides these measures, galvanism daily, iodide of potassium in large doses, gelsem- ium, croton chloral, codeine, external applications of menthol, freezing with chlorid of methyl, and heat — all may be found useful. The common practice of pulling out all the teeth is almost always un- successful, and ought not to be undertaken without specially good reason. Tonics containing iron, phosphorus, quinine or arsenic are generally helpful, and should always be given after a course of specifics. In younger patients the remedies recommended under the head of Migraine and Headache may be given. Change to a warm, equable climate may be tried ; it is not a certain resource. I have found that in cases not of over four or five years' duration, rest in bed with massive doses of strychnin sometimes effects striking cures. The drug should be given hypodermatically in doses of gr. once or better three times daily, gradually and very slowly increased until gr. i or i three times a day is reached. This is repeated four days and then the amount gradually reduced. The whole treatment takes about six weeks. The patient must be kept rigidly quiet and the full course persisted in. After finishing the strychnia, iodide of potassium and iron are given. The treatment may have to be repeated with lessened rigor. The treatment by large doses of opium, gr. iii. to vi. daily, is uncertain and often dangerous. Sometimes massive doses of quinine break up an attack.
Finally, surgical interference may be necessary. The removal of the nerve at as deep a point as possible is the only operation to be seriously entertained. This sometimes causes cure, but, as a rule, the pain comes back in six months to twenty years. Even such a respite, however, is often gladly seized upon. Removal of the Gasserian ganglion has been attempted with success. Ligature of the common carotid has been tried also, but of late years the operation
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 93
has been generally abandond. Simple drilling oat of the infraorbital nerve with a dental probe of piano wire has given long relief.
There are numerous methods of operating upon the different branches of the trigeminus.
Hartley, of New York, has devised an operation by which he enters the middle fossa through an opening in the temporal bone, thus reaching the root of the nerve. Rose, Krause and others have reported many successes following this rather serious operation. Abbe has modified it very successfully. He trephines the skull, enters the middle fossa, cuts off the second and third nerve-roots and plugs the foramen oval. The operation in proper hands is safe and most efficient. Injection of the Gasserian ganglion with alcohol intro- duced by a needle passed through the cheek and upward behind the jaw through the foramen oval is a new operation which has been suc- cessful. Injections are also made into the different branches of the nerve. The treatment is a safe one and produces at least temporary good results. There are several methods of operation, and the best one d.es not seem to have been yet worked out.
Trigeminal Paresthesia. — Sometimes persons suffer from pecu- liar numbness, thrilling or formication in the course of the trigeminus. The sensation may be nearly constant and excessively annoying. It never amounts to actual pain.
It occurs in anaemic, nervous and hysterical persons. It is to be regarded as an abortive form of neuralgia and so treated.
Trigeminal Anesthesia. — This occurs from various pathological lesions in the course of the nerve or in its nuclei. The most com- mon organic cause is syphiltic disease of the membranes at the base of the brain. Trigeminal anaesthesia occurs together with anaesthesia of other areas in hysteria and in organic disease of the nerve- centres. It is sometimes noted in tic douloureux and facial hemi- atrophy.
Flushing, pallor, lacrymation, salivation, are all symptoms of dis- turbance of the trophic, vasomotor and secretory fibres running in the trigeminal nerve. They are usually, if pathological, only concomi- tant symptoms of other diseases.
Facial herpes may be due to an inflammation of the Gasserian ganglion and be accompanied with much pain and anaesthesia, but it is a malady generally belonging to internal medicine or dermatology.
Headache (Cephalalgia).
Headache is the name given to attacks of diffuse pain affecting different parts of the head and not confined to the tract of a particular 13
194
DISEASES OF THE NERVOUS SYSTEM.
nerve. It usually comes on in paroxysms at various intervals, but may be continuous.
Etiology. — Headache is the most common of nervous symptoms. Ten to fifteen per cent, of school-children, twenty-five per cent, of men, and over fifty per cent, of women are subject to it, more or less, though this proportion would be much reduced if migraine were excluded from statistics.
The headache ages are from ten to twenty-five and thirty-five to forty-five; most cases occur between the ages of eight and twenty- five, especially in females. The number of headaches increases grad-
Fig. 86 — Showing the motor and sensory nerves of the face. (Merkel.)
ually from the period five to ten years up to the period fifteen to twenty, then falls until the thirty-fifth year, and rises again until about the age of forty. Early childhood and declining age are practically exempt from chronic functional headaches. Women suffer from it more than men in the proportion of about three to one. It is more frequent in city populations and among the wealthier classes. Head- aches are more common in the spring and fall and in temperate climates. Headaches may be classed, in accordance with their causes, as follows:
i. Haemic or autotoxic causes, in which impoverished or disordered blood is brought to the brain, as in (a) anaemia and congestion; (b)
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 95
diathetic states: gout, rheumatism, uraemia; (c) infections: malaria, fevers.
2. Toxic causes: lead, alcohol, tobacco, etc.
3. Neuropathic states: epilepsy, neurasthenia, hysteria, neuritis.
4. Reflex causes: ocular, naso-pharyngeal, auditory, gastric, sexual.
5. Organic disease, including arteriosclerosis, syphilis, tumors, meningitis and diseases of the cranial bones. Very frequently
Fig. 87. — Location of headache nodules. (Edinger.)
several causes act together. The autotoxic, dyspeptic, ocular and neurasthenic are the common forms of chronic and recurrent headache.
Edinger thinks that a large percentage of chronic headaches is due to the presence of certain nodules situated near the origin of the muscles of the back of the head. The location of these is indicated in Fig. 87. Massage by removing these relieves the headache. I have not found these often, and doubt if they have so much importance, but their existence and possible influence should be borne in mind, especially in gouty subjects.
Symptomatology. — Headaches may be classed in accordance with their location and the character of the pain. We have accordingly:
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DISEASES OF THE NERVOUS SYSTEM.
(1) frontal headaches; (2) occipital headaches; (3) parietal and tem- poral headaches; (4) vertical headaches; (5) diffuse headaches and various combinations of the above.
The most common form of headache is the frontal, next the fronto- occipital or diffuse, next the vertical and then the occipital.
The kind of pain differs with different persons and with different causes. We have: (1) pulsating, throbbing headache; (2) dull, heavy- headache; (3) constrictive, squeezing, pressing headache; (4) hot, burning, sore sensations; (5) sharp, boring pains.
The first form characterizes headaches with vasomotor disturb- ances, and usually indicates migraine (throbbing headache). The second is usually of a toxic or dyspeptic type (the dopey head-
Ansemia. Endometritis. Bladder? Constipation. \
Fig. 88. — Reflex head pains.
ache). The third is found in the neurotic and neurasthenic (the pressure headache). The fourth in rheumatic and anaemic cases. The fifth in hysterical, neurotic and epileptic cases.
The accompanying diagram shows some of the relations of local- ized pain to the cause. Chronic occipitofrontal headaches are usually neurasthenic, or ocular or both. Chronic occipital headache is usually neurasthenic, ocular or uterine.
Headaches may continue for a day or may last for weeks or months. Some persons have headaches only when constipated or bilious or when they have an attack of indigestion. Others suffer from a little pain nearly all the time, exacerbations occurring at various periods. Neurasthenic and ocular headaches are generally of this type. When headaches are persistent, examination should be made of the eyes of the nose and sinuses; the patient should be questioned as to syphilis, the continuous use of tobacco and chronic dyspepsia. The possibility of brain tumor, of pachymeningitis from blows, or sun- stroke or chronic alcoholism should be considered.
The persistent headaches not relieved by ordinary treatment are
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 97
due to eye trouble, anaemia, neurasthenia or spinal irritation, rheu- matic nodules, syphilis or pachymeningitis. Eye strain may cause true migraine or ordinary headache. Bye-strain headache is usu- ally associated with some weakness of eyesight and pains and dis- comfort about the globe, besides severer pains at times in the brow or occiput. The cause of the eye-strain is usually astigmatism and hypermetropia. Occasionally it is due to weakness or lack of balance of the eye-muscles.
Headaches may occur regularly every morning on awaking. They are called morning headaches, and are a symptom of neurasthenia, lith- aemia and arterial sclerosis. These occur oftenest in middle life or later.
Symptoms Associated with Headache. — The symptoms oftenest associated with chronic and recurrent headaches are vertigo, somno- lence, sensations of heat and pressure (cerebral paramnesias), and nausea. Vertigo goes oftenest with headaches of dyspeptic origin; some of the so-called bilious headaches of early life develop later into attacks of vertigo; this symptom often occurs with frontal headaches. Somnolence occurs oftenest with anaemic and malarial headaches; it may develop also with syphilitic head pains. Nausea I have found oftenest with occipital headaches.
Pathology. — Headaches are to be distinguished from neuralgias and from a special and common form of head pain known as migraine.
Headaches are diffused pains caused, as a rule, by irritations lo- cated in or referred to the peripheral ends of the fifth nerve supplying the dura mater.
Neuralgias, on the other hand, are caused by irritations of the gang- lia or trunks of these nerves. The pains are local and confined to the single branches of the nerve.
Migraine is a periodical neurosis in which there is a discharge of nerve force, not only affecting the trigeminus, but often other cranial nerves as well as sympathetic fibres. It is a general disease of which the headache is only one symptom.
The nerves of the dura mater are those most involved in headache. Headaches, when occipital, involve the sensory fibres of the vagus and the upper four cervical nerves. There is no anatomical change in the nerves except in organic headaches. But in many cases the membranes of the brain and their sensory nerves are congested or anaemic.
Diagnosis. — No symptom requires more careful investigation as to its cause than that of headache. The diagnosis is always to be made,
I98 DISEASES OF THE NERVOUS SYSTEM.
not of this symptom, but of its cause. Most of the foregoing descrip- tion accordingly refers to etiology.
Headaches which persist for months are worse in the day and leave the patient able to sleep at night, to recur on waking, are exhaustion pains and are due to a neurasthenic state.
Chronic headaches, worse at night, are usually of specific or organic origin.
Migrainous or "sick-headache" comes on paroxysmally, lasts a short time, and then leaves the patient feeling perfectly well or even better than ever. The attacks are often accompanied with nausea, flashes of light, strong pulsations of the head, vertigo, pallor or, more rarely, congestion of the face.
In neuralgic headaches the pains are sharp and shooting; they run along the tract of the nerve, and often are associated with suffusion of the eye and oedema. Tender points are felt.
Most of the persistent, recurrent headaches are of migrainous origin.
Treatment. — The constitutional treatment is based upon the etiology. Regulation of diet, securing a regular movement of the bowels, attention to ocular troubles, abstention from tobacco and alcohol and overwork are the important measures.
The symptomatic treatment consists in giving sedative drugs and local anodynes.
Antipyrin can be given in doses of gr. v. every twenty minutes until three or four doses are taken. Phenacetin often needs to be given in large doses of ten or even twenty grains. Antifebrin must be given in small doses. Exalgin is not a very good or safe remedy. It maybe tried imdoses of gr. iij. to gr. v. Muriate of ammonia is an excellent remedy given in very large doses, 5ss. to 3i-, well diluted. Menthol in doses of gr. v. to gr. x. in hot water sometimes stops head- aches. Combinations of caffeine citrate and the various coal-tar prod- ucts furnish the basis for the ordinary headache cures. Local appli- cations of a twenty per cent, solution of menthol, the ice bag, cloths wrung out in hot water or a piece of sheet lint soaked in chloroform liniment two parts and tincture of aconite one part are efficacious measures. A cathartic, rest in a darkened room, light diet — all these are measures which many patients themselves learn to adopt.
Finally, in headaches from organic disease we have often to resort to iodide of potassium, mercury and the use of some preparation of opium.
As will be seen, each case of headache requires special treatment and a certain amount of experimentation in order to learn the idio- syncrasy of the patient.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 1 99
Migraine:, Sick-headachk, Hemicrania. — Migraine is a constitu- tional neurosis characterized by periodical attacks of pain chiefly in the course of the fifth nerve. The pain is often associated with nausea or vomiting, mental depression, vasomotor disturbances, such as flushing, or pallor of the face, by flashes of light, vertigo, tinnitus aurium, and in rare cases by partial paralysis of one oculomotor nerve.
It will thus be seen that migraine is more than ordinary headache and unlike an ordinary neuralgia.
Etiology. — The disease is very common in civilized countries and is frequent in America. It occurs oftenest in women in the propor- tion of about three to one, and it begins in most cases at or a little before the age of puberty. It may begin as early as the fifth or even the second year. It occurs in neurotic families, and there is very often a history of direct inheritance. Other neuralgic troubles, epilepsy and gout may be found in the family history. The attacks occur oftenest in the winter in our climate. The cases that begin in childhood and early life are sometimes started by overwork at school, but usually no especial cause can be found. When they begin after maturity, a history of excesses in work, injury, shock or exhausting disease is found. Migrainous patients often have some refractive disorder of the eye or a weakness of eye-muscles, and the conditions may be factors in bringing on or keeping up the headaches. Autotoxaemia from uric acid and poisons developed in the intestinal tract is considered an important factor in migraine.
Symptoms. — The patient for several days may feel a sense of malaise and depression; usually, however, the prodromal stage lasts only a few hours or a day. The attack often comes on in the morn- ing and gradually increases in intensity until the victim has to give up work and lie down. Sometimes the pain comes on with almost epi- leptic suddenness and violence, waking a person from sleep or com- pelling him at once to lie down. Fulgurating migraine is the term applied to this type. The pain starts in one side of the head, usually in the forehead, but often in the occiput. It increases and finally may involve the whole head. The pain is of a tense, throbbing, binding character, increased by jars, light and noises. It is accompanied by dim- ness of vision, often by flashes of light or dark or light spots, variously colored, floating before the eyes. (Fig. 89.) Contraction of the visual field , usually in the form of bilateral hemianopsia, may occur. Vertigo, tinnitus aurium, confusion of ideas, feeling of stupor, disturbances of memory, are not uncommon; nausea and even vomiting are the rule. The vomited matter is at first chiefly mucus, but it may later become
200
DISEA S OF THE NERVOUS SYSTEM.
yellow and bitter from + oresence of bile. Hence the term "bilious headache," which is au mp/oper one, because the bile is only the result of retrostalsic ad >n from the vomiting. Migraine is not the result of gastric or liver isorder.
The patient's face usually is pa e and gives the evidence of acute suffering. The flushed face is very rare; the distinction between angiospastic or pallid migra ne and angioparalytic or congestive
Fig. 89. — Scintillating and zig-zag lights seen in migraine. (Babinski.)
migraine is not of clinical value. The pulse is small and hard, and may be lessened in rapidity. The temperature in children often rises.
The attack lasts from six to twelve or twenty-four hours, occa- sionally even two or three days. As the intensity of the pain lessens, the patient sinks to sleep, and awakens next morning feeling re- freshed and better than before the attack.
The attacks occur at varying periods, fortnightly or monthly, and even weekly. In women they often occur during menstruation.
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Some women are entirely free from them during pregnancy. At about the time of the menopause in women, and at about the same time of life in men, the disease lessens in severity and, as a rule, dis- appears. Some form of neuralgia or some neurosis in rare eases takes its place.
Complicating Symptoms. — Partial oculomotor paralysis, temporary aphasia, slight hemiplegia, heminumbness, peculiar odors or tastes, convulsive movements of the body almost resembling epileptic attacks occur.
Cases presenting these symptoms are rare. When they occur in one case, however, they always occur in each attack unless it is modi- fied by treatment.
Vicarious Attacks. — Migraine is sometimes associated with epi- lepsy or insanity; that is to say, persons in early life have had mi- graine and later developed the diseases mentioned. The relation between these diseases, is not, however, a very close one, nor does one disease lead to the other. The attack of migraine is sometimes replaced by various other functional neuroses. Sometimes, instead of a fully developed attack, the patient has a sense of mental depression, with confusion of ideas. Cases have been reported in which acute mania took the place of the headache.
Types. — Practically, we find two classes of cases:
1. The typical, associated with visual disorders and having most of the symptoms described above.
2. The irregular or mixed type, in which, with many symptoms of ordinary migraine, there is a history of rheumatic influences and often of anaemia or dyspepsia. These are cases of a true migrainous affection complicated with some form of symptomatic headache, such as has been already described. The mixed or irregular migraines are important to recognize, for they call for special treatment. Many patients have their "sick-headaches" and their "neuralgic headaches," so-called, and they distinguish between them. Both are migrainous headaches, but with other elements entering.
Pathology. — The seat of the pain is chiefly in the intracranial branches of the fifth nerve and of the pneumogastric ; the upper cervi- cal nerves, however, are often involved. There are no morbid ana- tomical changes known. The most plausible theory of the disease is that it is a fulgurating neurosis, in which there are periodical dis- charges of nerve force, or nerve storms. The seat of the discharge is perhaps in the cerebral cortex, or possibly in the primary sensory centres, i.e., the root ganglia of the fifth and vagus nerves. The disease is certainly not in the sympathetic system, as was once
202
DISEASES OF THE NERVOUS SYSTEM.
taught. The presence of excess of uric acid or of some other autoch- thonous poison as a factor in the disease may be regarded as probable.
Diagnosis. — The diagnosis is based upon the hereditary history, the periodicity and seat of the attacks, the nausea, the complicating visual and other sensory symptoms. It should not be forgotten that the same patient may have migraine and other neuralgias or may have also an organic brain or renal disease.
Treatment — Prophylaxis. — Children of families in which this neu- rosis exists should be carefully watched during the ages between five and twenty. The eyes and nose should be examined. They should not be subjected to excessive mental or visual strain, and if attacks develop they should be promptly treated The application of glasses should be considered, but not hastily adopted.
As regards constitutional treatment, the best measures for cur- ing a case of migraine consists in correcting any visual or nasal defect, such diet and exercise as secure health to the body and tone to -the nerves. The diet should be very simple and non-fermentative. It should be mainly of meats and green vegetables and cooked fruits. The meats should be moderate in amount, and sometimes only fish and poultry should be allowed.
Of the drugs, alkaline tonics, given well diluted before meals, are the most trustworthy. Extract of cannabis indica may be given in large doses and for a long time (gr. \ to gr. i., ter in die). The salicy- lates are useful combined with an alkaline laxative like Rochelle salts, a dose being taken night and morning. Much stress is laid by some upon ocular muscular insufficiencies, and I believe that such con- ditions should be remedied, but place little confidence in them alone. On the other hand, the correction of small or large degrees of astig- matism and hypermetropia sometimes produces surprisingly good results. The reported cure of numerous cases of migraine by treat- ment of nasal hypertrophies and catarrh should not excite too much confidence in such measures. In fact, since migraine is a constitu- tional neurosis, one cannot expect permanent results from removing reflex irritants alone.
For the relief of the attack the measures recommended under Head- ache are to be used. All drugs lose their effect after a time, and finally patients give up treatment or resort to codein or morphin. Chloral and a hot foot-bath break up attacks sometimes. Locally galvanic currents are sometimes helpful, and so are static sparks. Hot appli- cations and pencilling with menthol give relief to some. Quiet and rest are spontaneously resorted to.
sensory neuroses of the cerebrospinal nerves. 203
Neuroses of the Sensory Branch of the Seventh Nerve.
As stated under the head of Facial Palsy, the seventh nerve has a sensory part, and its disorders cause otalgia of simple type, an "otalgic tic douloureux" and a herpetic otalgia, due do lesion of the genicu- late ganglion. When both motor and sensory fibres are involved, there results the "Hunt syndrome" of facial palsy, otalgia and herpes of the ear.
Neuroses of the Acoustic Nerve.
Anatomy. — The auditory or eighth cranial nerve has two different parts. One portion passes to the cochlea and utricle and saccules; it has to do with the sense of hearing; the other goes to the semicircular canals , and has to do with that sense by which we appreciate the posi- tion of our body and its relations to space. The eighth nerve is thus an auditory and a space-sense nerve.
The auditory fibres enter the medulla by two roots, a lateral or posterior and a median or anterior. The lateral root has mainly audi- tory fibres. The space-sense fibres enter chiefly by the median root. These roots are connected with three nuclei, viz.: (i) The chief nucleus (dorsal, central, inner nucleus); (2) the large-celled nucleus (Deiter's); and (3) the accessory nucleus (ventral, anterior, lateral). The chief nucleus (1) is a large mass of gray matter composed of small nerve- cells and lies superficially just beneath the floor of the fourth ventricle The large-celled nucleus (2) lies to the outer side of and below it. The accessory nucleus (3) lies in the substance of the lateral root, and be- tween it and the median root.
The lateral root is the one coming chiefly from the cochlea, and is, as stated, mainly a nerve of hearing. It is connected most extensively with the accessory nucleus, but also with the other nuclei. Through the accessory nucleus it connects by a few fibres with the superior olives, mostly of the opposite side; thence fibres pass up through the lateral lemniscus, to the posterior corpora quadrigemina; thence to the cortex of the first and second convolutions of the temporo-sphenoidal lobes. Other fibres pass up directly through the lemniscus and the tegmental or sensory tracts to the cortex of the temporal lobes. Con- nections are numerous, also, with other cranial nerve nuclei. The lateral root also sends fibres to the chief nucleus and (via the striae acusticae) into the raphe, and thence to the formatio recticularis and sensory tract. These fibres go also to the posterior tubercles of the corpora quadrigemina and thence to the cortex of the temporal lobe.
The median root is connected chiefly with Deiters' nucleus (2) and through this with the vestibulo-spinal tract, and longitudinal bundle; finally with the cortex of the vermus.
The diseases of the eighth or acoustic and space-sense nerve which are of special interest to the neurologist are auditory paralysis or nervous deafness, hyperacusis, tinnitus aurium, and auditory vertigo
204
DISEASES OF THE NERVOUS SYSTEM.
or Meniere's disease. These diseases correspond with loss, excess and perversion or irritation of function.
Nervous Deafness (Acoustic Paralysis).
Nervous deafness may be due to lesions (i) of the cortical centres of hearing, (2) of the acoustic nuclei, and (3) of the acoustic nerve itself or its end organ. Practically, it is almost always the nerve and the internal ear which are affected.
Etiology. — 1. Cortical nervous deafness has been known to occur in a few instances from lesions of both temporal lobes (Mills, Mott). The deafness of hysteria is of cortical origin.
2. Deafness from lesion of the acoustic nucleus or nerve-root. Cerebrospinal meningitis in the young and syphilitic meningitis in the adult are the more frequent causes of this form. Tumors and hemorrhages may also be causes. When the auditory nucleus and nerve-root are affected by these diseases, its peripheral terminations in the labyrinth are also often involved, so that sharp distinction can- not always be drawn between this form and that due to labyrinthine disease.
3. Deafness from Labyrinthine Disease. — The causes are drugs, such as quinine and the salicylates; inflammations, including syphi- litic exudates; injuries; hemorrhages; tumors; primary atrophy, which may occur in locomotor ataxia; mechanical causes, such as the constant noises and jarring to which locomotive engineers and boiler-makers are subject.
Symptoms of Nervous Deafness. — The dominant symptom is loss of hearing, but this may be accompanied by vertigo, tinnitus and even forced movements.
In hysterical deafness the loss of hearing is rarely complete, is usually unilateral, is more of a bone conduction deafness and especially involves high and low notes. Deafness from involvement of the nucleus and nerve-root is usually accompanied by other symptoms of a basilar meningitis or lesion of the pons and medulla. Labyrinthine deafness is often associated with vertigo, tinnitus and forced movements, when it may become a symptom-complex known as "Meniere's disease."
Sudden total deafness is characteristic of hysteria and of syphilitic disease of the internal ear. In genuine nervous deafness, unless the deafness is absolute, hearing by bone conduction is lost or lessened, while aerial conduction is preserved. A tuning-fork vibrating on mas- toid is not heard by the affected ear, though it is heard when held in the air close by this ear. (Rinne's negative test.) Changes of reaction
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES.- 205
to the electrical current occur, but the tests are difficult and the results unsatisfactory.
The treatment depends upon the seat and nature of the lesion. In labyrinthine deafness it is generally limited to the use of iodide of potas- sium, mercury, pilocarpin, leeches and the galvanic current. Local applications and surgical interference may be required. The treat- ment of nervous deafness in which vertigo, forced movements and tinnitus are the dominant symptoms will be discussed under these heads.
Tinnitus Aurium. Tinnitus Cerebri (Noises in the Ear
and Head).
Subjective sounds resembling hissing, buzzing, humming, beat- ing, musical notes, etc., are classed together under the general head of tinnitus aurium. It is a very common symptom.
Etiology. — -The disease attacks adults in middle and later life. Men and women are alike affected. Neuropathic constitutions and an unstable circulation favor it. The arteriosclerosis of old age, cere- bral anaemia and congestion, sunstroke, tobacco and alcoholism lead to it. It occurs often in melancholia and in neurasthenia. Some local disease or congestion of the middle or internal ear is usually present.
Disease of the auditory nuclei and auditory tracts rarely, if ever, causes tinnitus; but chronic pachymeningitis, such as follows blows on the head, sunstroke, alcoholism, etc., may be attended by most annoying tinnitus, which is often not so much in the ears as in the head — a tinnitus cerebri. In old people with thickened arteries and imperfect brain nutrition a similar condition may occur.
Tinnitus accompanies insanity sometimes, and may be the source of aural hallucinations.
A kind of tinnitus may accompany migraine and take the form of an aura in epilepsy.
Despite this long list of causes, the chief factors may be summed up as neurasthenic states, local ear disease, humoral poisons and irri- tants, reflex irritants, arteriosclerosis. f|3
The symptoms are indicated by the name of the malady. They may come on suddenly, but usually develop slowly. Some deafness and occasional vertigo are often present. The noise is located in one ear, as a rule. Sometimes it is said to be simply "in the head." The sounds are generally present all the time, giving the patient little rest and making life a burden. They vary greatly in character and intensity. These variations are indicated in the study of the diagnosis.
2o6
DISEASES OF THE NERVOUS SYSTEM.
The Diagnosis. — The recognition of the symptom is easy. The principal thing is to discover its seat and cause.
The ear should, of course, be examined for external or middle ear disease.
If the tinnitus is pulsating and synchronous with the heartbeats and stopped by carotid compression, it may be inferred that it is due to vasomotor paralysis or inflammatory congestion or aneurism.
If the sound is not in the ear, but in the head, and not associated with deafness or ear disease, the trouble is probably central, and most likely is of meningeal or arteriosclerotic origin.
Noises which are complex or take the form of musical sounds or words are probably central.
Constant rushing, knocking, pulsating noises are due to conges- tion, hemorrhage or inflammatory effusion in the labyrinth.
Moist sounds of a gurgling, bubbling, boiling, singing, whistling, shell-like roaring character indicate disease of the middle ear, with fluid exudation or catarrh of the Eustachian tube, or irritation of the external auditory canal, mastoid cells or postnasal spaces.
Dry roaring and ringing noises are due to non-suppurative catarrh of the middle ear, disease of the muscles or nervous supply of the tympanum, meningitis, tumors and syphilis.
The condition of the digestion should be inquired into and the ex- istence of renal, arterial or central nervous disease investigated.
Treatment. — Hydrobromic acid and the other bromides, given in ordinary doses, are the surest remedies for this trouble. They may be combined with digitalis. Iodide of potassium and iodide of ethyl often are useful. Nitroglycerin is sometimes of value in patients with hard arteries. A combination of digitalis, bromide, and nitroglycerin has given very good results. Occasionally tonics are indicated. I have seen but little good from electricity or counter-irritation. Of course middle-ear disease must be treated if it is present.
Vertigo (Dizziness, Giddiness).
Vertigo is a disturbance of consciousness characterized by apparent movements of external objects or of the person himself. If external objects whirl around, the vertigo is called objective ; if the person himself seems to move, it is called subjective. Vertigo is almost always a symptom. In rare cases it appears to be idiopathic.
Vertigo is connected more or less with our space sensations, and hence it will be described here under disorders of the eighth cranial nerve and its central representations.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 207
Etiology. — The causes of vertigo may be classed somewhat like those of headache, as follows:
(1) Haemic, etc., anaemia, hyperaemia, toxaemia from tobacco and alcohol; (2) arteriosclerosis; (3) acoustic-nerve irritation; (4) neu- roses; epilepsy, neurasthenia; (5) reflex: ocular, gastric; (6) organic brain disease; (7) mechanical causes, like electricity, swinging, etc.
Based mainly on the etiology, we have as a practical classification of ordinary cases of vertigo: toxic, auditory, gastric and bilious, ocular, neurasthenic, and epilepic forms. The various causes of vetigo act partly by irritating the space-sense nerve and thus dis- turbing our sense of relation to external objects, partly by irritating the cortical centres of the brain.
Symptoms. — Vertigo comes on suddenly, and lasts, as a rule, for but a moment. The floor rises and sinks, or objects whirl around (objective vertigo), or the patient seems whirling around or falling. The ideas are confused ; there are a sense of alarm and a feeling of faint- ness. The patient totters, sometimes falls; there may be nausea or vomiting. In some forms there is momentary loss of consciousness or syncope. Vertigo usually comes on in short attacks, but in toxaemic states, as in alcoholism or nicotinism, it is almost constant while the poison is in the system. Vertigo may become chronic or nearly so ; and if severe it forms what is called the status vertiginosus (Mitchell). Vertigo is increased by rising or sudden movements and lessened by lying down.
Vertigo may be due to organic lesions of the cerebellum and its peduncles or of the labyrinth ; it is then associated with forced move- ments of the body.
Symptoms of Special Forms — Auditory Vertigo (Meniere's Dis- ease).— A large proportion of severe periodical vertigoes are due to disease or irritation of the eighth nerve and its centres. The com- mon cause is local disease of the labyrinth. When this produces severe attacks of vertigo with nausea and perhaps syncope, it is called "Meniere's disease." The name is often applied to any form of auditory vertigo. Meniere's type is always due to organic disease of the labyrinth. It is accompanied by progressive deafness, and some- times by tinnitus and forced movements cr even utter inability to walk steadily. When the deafness is complete the vertigo ceases, because the nerve end-organ is destroyed. Mild forms of auditory vertigo present nothing unusual except those due to involvement of the nerve of hearing.
Many forms of vertigo, such as the gastric and toxic, occur through a reflex disturbance of the eighth nerve. The auditory
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DISEASES OF THE NERVOUS SYSTEM.
nuclei are connected with those of the vagi. The labyrinth is sup- plied with blood by the vertebral artery, whose calibre is controlled by sympathetic fibres which are in close connection with fibres to the stomach. Hence reflex effects may occur through contiguity of the central nuclei and by reflex spasm of the vessels of the internal ear. "Stomachal vertigo" is the name given to a very severe form of reflex vertigo. It occurs generally in persons whose stomachs are overloaded and whose digestion is paralyzed by its load. It is accompanied by loss of consciousness.
Bilious and Lithcemic Vertigo. — -In condition of dyspepsia, con- stipation and hepatic torpor, the disordered stomach and bowel suddenly discharge into the blocd irritant substances which pass to the brain and by direct action on the nervous centres cause vertigo. This is probably the explanation of the vertigo of biliousness and con- stipation. It is a paroxysmal vertigo, noted most in the morning, not very severe, and often accompanied by nausea.
Neurotic Vertigo. — The symptoms of epileptic vertigo will be de- scribed under that head.
Neurasthenic vertigo is a not uncommon symptom. The attacks are short, generally subjective, not severe or accompanied by 'nausea or syncope, but they often cause much alarm. Underlying them are ex- hausted and irritable nerve-centres, with ocular, gastric and humoral irritations.
A neurotic vertigo occurs sometimes in the form of attacks almost exactly resembling seasickness. There are intense vertigo, nausea, and faintness lasting for hours, coming on suddenly without known cause except overwork or excitement. The attacks occur in neurotic subjects and are analogous to the other nervous crises. It is a period- ical neurosis of the space-sense nerve and resembles migraine.
A form of vertigo which is psychical in character occurs in neurasthenics. It consists in a sudden sensation of insecurity, an. apprehension of falling, of an approaching loss of consciousness. There is no true vertigo, either subjective or objective, and the patients really never stagger or fall. It is a psychosis rather than a nervous condition.
In some nervous subjects there occurs a sudden giving way of the legs. There is no conscious vertigo, yet such probably exists. The symptom is noted in exophthalmic goitre. It is a "stumbling vertigo."
Ocular vertigo is a rare symptom,- but is, when present, chronic and annoying. It is caused by refractive errors and unequal action of the ocular muscles.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 20g
The mechanical vertigoes, such as seasickness, car sickness, etc., are produced by swinging or whirling, the movements of the ship, steam car and elevators. Railway mail clerks, elevator boys, often suffer from chronic disturbances of a vertiginous character. Ocular and auditory nerve sensations enter mainly into the causation of the troubles.
Arteriosclerotic Vertigo, Senile Vertigo. — This occurs in persons who have arteriosclerotic changes in the brain vessels, either from disease or senility. The symptom is caused by impaired brain nutri- tion with consequent anaemia. Senile vertigo may also be due to a weak and fatty heart.
Pathology. — The consciousness of the proper equilibrium of the body and of its relations to the external world depends upon the con- tinuous inflow of nervous impulses from the eye and its muscles, from the nerves of the muscles, joints and viscera and from the ear. Anything which suddenly disorders this even inflow may cause a dis- turbance of consciousness and sensations of vertigo.
The aural impulses come from the semicircular canals and am- pullae; they are the most important. These impulses are not felt in consciousness normally, but go to certain lower centres chiefly in Deiter's nucleus and the vermis of the cerebellum. From this point they influence the acts concerned in holding the body in equilibrium.
When impulses from the eye and its adjusting mechanism do not flow in normally, there may be disturbance of consciousness and a feel- ing of vertigo. Probably visceral impulses can produce a similar disturbance. Everything which suddenly interferes with the nutri- tion of the cortex of the brain, such as anaemia and poisons, may lead to giddiness by lowering the level of consciousness and confusing the sensory inflow.
Diagnosis.- — In investigating vertigo the physician should find (i) whether it is subjective or objective; (2) paroxysmal or chronic; (3) accompanied by ear symptom, nausea, tinnitus, and loss of con- sciousness. He should then direct himself to finding the special cause and seat, remembering that the auditory, gastric toxic, and neu- rasthenic are the common forms. In elderly persons the arteries should be carefully examined. In young persons the possibility of epilepsy must be remembered.
The prognosis depends upon the cause. Epileptic vertigo and ver- tigo from organic disease are most serious. Labyrinthine vertigo usually ceases when complete deafness occurs. The other forms of vertigo are usually susceptible of relief.
Treatment. — The attack is treated by rest in the horizontal posi- 14
2IO
DISEASES OF THE NERVOUS SYSTEM.
tion arid the administration of a volatile stimulant. The disorder must then be treated in accordance with the cause.
In Meniere's vertigo the use of quinine by Charcot's method is said to be useful. Quinine is given in doses which are gradually in- creased until slight cinchonism results; then the drug is stopped. Mitchell advises the addition of hydrobromic acid ; Gowers advises the use of salicylate of sodium in five-grain doses instead of quinine. Hirt recommends ten drops of a two per cent, solution of pilocarpin in- jected hypodermically every other day. A better method is to give a sweating dose of pilocarpin muriate, gr. J to tV, with a tumbler of hot water early in the morning before the patient rises.
Neurasthenic vertigo is cured by rest and attention to diet, laxa- tives and mineral acids being used. Hydrobromic acid with pepsin and glycerin are often very helpful here. Gastric vertigo is to be treated with saline laxatives and simple bitters before meals.
In the vertigo of "biliousness" and lithaemia there is often a neu- rasthenic element, and a similar attention to diet and to the digestive organs is indicated. In arteriosclerotic and senile vertigo small doses of nitroglycerin and iodide of potassium, with or without digitalis, should be given. Rest and warmth of the extremities are indicated. In all forms- of vertigo bromide of potassium is helpful and will re- lieve the symptoms for a time. It is the best symptomatic remedy. Counter-irritation to the neck or mastoid region by the cautery does good occasionally.
There are two peculiar forms of disease to which the name vertigo has been attached which may be described here.
Laryngeal Syncope (Laryngeal Vertigo, L. Epilepsy). — This is a rare form of disorder characterized by attacks of paresthesia of the throat, with coughing, followed by sudden syncope, and some- times by slight convulsive movements.
The disease is probably an epileptic or tabetic phenomenon, or due to local or pulmonary disease.
At the onset of the attacks a burning or tickling sensation is felt in the larynx or trachea; there is a spasmodic cough, perhaps some asthmatic or dyspnceic symptoms, when the patient suddenly falls un- conscious for a short time. The attacks may occur daily or only once in a few weeks. Most cases are curable, yet the disease is not without danger.
The treatment should be directed to relieving any local condition or pulmonary trouble. Bromide of potassium should also be given.
Paralyzing Vertigo (GerliEr's Disease). — This is a disease occurring only on the farms in southern France and Switzerland.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 211
The symptoms consist of sudden attacks of ptosis, vertigo, paresis of arms and legs and cervico-occipital pain. The disease is most pre- valent in the summer time. It attacks chiefly males. Single attacks last not over ten minutes, but may occur frequently.
The cause is supposed to be a special microbe developed in the stables during the heat of summer.
We are not aware of its occurrence in America, though Seguin calls attention to the close similarity of the symptoms to those of poisoning by conium maculatum.
Hyperacusis (Auditory Hyperesthesia). — 'When there is undue keenness of the sense of hearing, the condition is called hyperacusis. It occurs in hysteria and hypnotic states. Some persons have natur- ally an extraordinary keenness of hearing. In facial paralysis there is sometimes hyperacusis due to paralysis of the stapedius.
When ordinary sounds cause painful feelings, the condition is called dysacusis. This occurs in the neurasthenic and hysterical in persons of enfeebled vitality, in the brain congestion of fevers and in menin- gitis ; also in local ear troubles of an inflammatory character.
Sensory Neuroses of the Glosso-pharyngeal Nerve.
The anatomy of this mixed nerve is described under the head of motor neuroses. The sensory fibres may be affected in hysteria, causing the symptom called globus, and also the pharyngeal anaes- thesia found in the same disease.
The special fibres of taste may be affected, causing ageusia or loss of taste.
Ageusia (loss of the sense of taste) is an affection in which the power to discriminate the tastes of bitter, sweet, salt, acid and alkaline substances is lost.
Etiology. — It occurs oftenest in an incomplete form in facial palsy and in hysteria. Injuries of the trigeminus and glosso-pharyngeal nerves, catarrhal diseases of the mucous membrane of the mouth and nose, are frequent causes. It is not caused by cortical brain disease so far as known.
Some ageusia is present in the imbecile, and the sense of taste is less keen in the lowly organized and criminal classes.
Symptoms. — The symptoms are subjective and may not be noticed at first by the patient. In hemiageusia from facial palsy and in hysteria it has to be looked for, as the patient does not complain. The tests are made with solutions of salt, sugar, vinegar, and quinine. A single solution of sugar usually answers. But the different parts of the
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DISEASES OF THE NERVOUS SYSTEM.
tongue differ in sensibility to different substances. Care must be taken to exclude the nose as a factor in taste.
Pathology. — Ageusia occurs as the result of disease of the roots of the trigeminus, especially of the second, or, as Krause claims, the third; also from disease of the facial when the chorda tympani is implicated and from disease of the glosso-pharyngeal root.
Disease of the trigeminus and facial usually causes ageusia on the anterior two-thirds of the tongue, with loss of taste, especially for sour and bitter substances. Sometimes, however, disease of the trige- minus or disease of the tympanum involving the tympanic plexus and chorda tympani causes ageusia of the whole tongue on the affected side.
Ageusia from disease of the glosso-pharyngeal alone is very rare, and then causes loss of taste on the posterior third of the tongue, soft palate and pillars of the fauces, with loss of taste to sweets and acids. A few cases have been reported in which paralysis of the glosso-pharyngeal caused complete ageusia on the affected side. It must be inferred, therefore, that taste fibres run sometimes wholly in the fifth, more rarely wholly in the ninth nerves, and usually in both.
The treatment depends on the cause. Locally, cleansing and stim- ulating mouth-washes and electricity may be used.
Parageusia, or perversions and imperfections in the taste sense, are very frequent. They are generally due to irritation of the taste nerves from catarrhal inflammation of the stomach or mouth. They also occur in hysteria.
Sensory Neuroses of the Upper Cervicae Nerves.
The mass of muscles at the back of the neck is made up of nine individual muscles on each side. They are innervated by the post- erior branches of the upper six cervical nerves and the spinal accessory.
Sensation is supplied to this muscular mass and to the back of the head by the anterior branches of the second, third and fourth cervical and the posterior branchs of the second cervical. The cutaneous dis- tribution of these nerves is shown in the diagrams. (Figs. 85 and 86.)
From a study of the innervation of the skin and muscles of the neck, it seems that most neck pains and paresthesias are placed in the areas supplied by the occipitalis major and minor nerves, branches of the second and third cervical nerves. (Figs. 30 and 31.)
Cervico-occipitae Pains. — There are practically only a few neu- ralgias which may be considered to have a definite clinical course and to be entitled to the name of special diseases. The chief of
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 213
these are trigeminal neuralgia, neuralgia of the brachial plexus and the neuralgia of the sciatic plexus. The other forms of neuralgia are nearly always symptomatic or reflex pains or are distinct manifestations of a neuritis or some injury or irritation of the nerves. Pains in the neck, therefore, are very rarely to be classed under the head "cervical" neuralgia or " cervico-occipital " neuralgia. Nevertheless, there is no region of the body in which pain is more frequently complained of than in the back of the neck and head, unless we except the trigeminal pains. The excessive amount of pain experienced in the neck is due to several causes. The muscles of the neck are continuously used, perhaps more than almost any other muscles, except those of the eyes. The supporting of the head in the erect posture and the movements of it which accom- pany the use of vision make the functions of the upper four cervical nerves an almost continuous performance.
Whenever a group of muscles is thus used to such a continual extent in function two morbid phenomena are apt to occur. One is an exhaustion of these muscles, with consequent pain from their fatigue ; the other is the development in the muscular bodies or their connecting tissues and nerves of rheumatic and vascular disturbances — -so that pains in the neck are, as a rule, either exhaustion pains or rheumatic pains.
There is, however, another source of pathological disturbance. Whenever a serous membrane is irritated, the muscular bodies which surround it and by their movements and arrangements support it, experience reflex disturbances. This we see exemplified by the pain in the side when the pleura is involved and the pain about the joint? when they are inflamed. So whenever the serous membranes sur- rounding the brain become congested or irritated, there results a reflex disturbance upon the muscular support of the skull in which the brain is enclosed.
Whenever, then, by a persistent overuse of the brain, there occur vascular changes of fatigue, the neck muscles show it in a reflex irri- tability and various accompanying sensory disturbances. So in the beginning of meningitis we find the pain in the neck one of the earliest of the storm signals sent out.
For some reason it does not seem to happen that the four upper cervical nerves, which supply motion and sensation to the neck muscles and the overlying skin and occiput, go through a process of simultaneous inflammation or subinflammatory action, which runs a definite course of several weeks or months, as is the case with neural- gias and neuritic disturbances of the arm and leg.
214 DISEASES OF THE NERVOUS SYSTEM.
Quite apart from the preceding group of disturbances, however, there occur in the neck certain pains due to various causes, and these are spoken of as cervical neuralgia and neck pains.
The anatomy of these nerves has been described under the motor neuroses. The sensory distribution to the skin is shown in the accom- panying figure (Fig. 85).
Cervico-occipital Neuralgia — Neck Pains — Etiology. — Pains in the back of the head and neck occur in migraine, in hysteria, spinal irritation and neurasthenia, as a result of eye-strain, as a true neuralgia and as a symptom of brain tumor, meningitis and rheumatic inflam- mation of the neck muscles and nerve. True cervico-occipital neu- ralgia is rare, is much more common in women, occurs between the ages of twenty and thirty-five, and is usually an exhaustion or toxic symptom disease.
Symptoms. — Migrainous neck pains are described elsewhere. It is a one-sided trouble and periodical in type. In spinal irritation and hysteria the pain is central or, perhaps, shifting; it is especially char- acterized by a sharp, boring pain just below the occiput. With it there may be evidences of cerebral congestion or anaemia, with vertigo and faintness, and even vomiting. The boring pain is almost pathognomonic of spinal irritation. In neurasthenia the pain is more of a tired, aching character. In typical neuralgia the pain is usually unilateral, paroxysmal and sharp, sometimes reaching the intensity of a tic douloureux. There are tender points over the exit of the nerves. The disease lasts for five or six weeks. The pains may alter- nate with or take the place of a trigeminal neuralgia. The nerves in- volved are the great and small occipital from the second pair and a branch from the third pair.
Treatment. — General constitutional treatment consists in the use of antirheumatics, such as the salicylates. The muriate of ammonia has done me good service. In women pelvic troubles should be looked for; in both sexes the eyes must be attended to. Locally, counter-irritants, cupping and leeching are useful ; mustard and capsi- cum pastes are often a great relief. The ice bag also is of service. Trephining the occipital bone has cured some obstinate case, and re- secting the occipital nerves another.
Sensory Neuroses op the Lower Cervical and Brachial
Nerves.
Brachial Neuralgia and Neuritis. — Definition and Frequency. — Neuralgia of the brachial plexus is a disease characterized by severe pains centering in the upper arm and usually involving the whole up-
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 215
per extremity and shoulder; it runs a course of several months and is due to irritation or inflammation of the trunks of the brachial plexus and its roots of origin. This irritation does not usually reach the degree of neuritis, and the disease does not show the paralysis and atrophy, anaesthesia and the vasomotor symptoms of neuritis, as a rule. This form of neuralgia, even excluding the cases of traumatic neuritis, is now by no means a rare disease. Among the neuralgias brought to the attention of the neurologist it ranks third, coming after the facial neuralgias and closely following in frequency the sciaticas.
In my private history books I find that I have a list of seventy- nine cases of arm pains of various types. These I have classified as follows, and it shows that, leaving aside all symptomatic troubles and all occupation neuroses and local troubles, there is still a large percentage of pure brachial neuralgias :
Total cases of arm pains 79
Total brachial neuralgias 41
Arm pains associated with distinct neuritis 15
Occupation neuroses with dominant arm pains 16
Hysterical arm pains 4
Palmar, digital and hand pains due to local or reflex causes 6
Etiology. — Brachial neuralgia occurs oftenest in persons in middle life, ranging from thirty-five to fifty. When it occurs in young people it is (like the other neuralgias) more often of the hysterical type, or at least associated with that class who are neurotic and over-sensitive. Besides having an hereditary neurotic history as the underlying ten- dency, there is almost always a history of severe emotional strain — loss of friends, sickness in the family or worry over complications of domestic life. Sometimes, in addition, there is some physical injury or some acute sickness. In two cases I have seen it follow profuse hemorrhages. In some cases it is due to inordinate use of the arms in simple manual work, like embroidery or knitting; but this seems to me rather unusual. A mild form of brachial neuralgia occurs as the result of carrying a heavy skirt, and it is not unlikely that this kind of work (skirt carrying) and the use of the arms in dressing the hair brings on neuralgia in a great many predisposed cases.
Neuralgias are seen relatively oftener in the better classes than in dispensary or hospital patients. They occur oftener in women than in men in the proportion of about three to one. This is contrary to most statistics, because, as I believe, these statistics include many frankly traumatic and surgical cases. There is often a predisposing neurotic or rheumatic taint, and sometimes perhaps an autotoxaemia. The brachial neuralgia of tabes dorsalis and organic disease is not in- cluded in this discussion. In fine, the etiology of brachial neuralgia
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DISEASES OF THE NERVOUS SYSTEM.
has always one or more of three factors: neurotic constitution, exhaus- tion from occupation or trauma and metabolic (rheumatic) irritation.
Pathology. — Brachial neuritis proper, non-traumatic, in which there are objective evidences of nerve degeneration, is probably only a severer and more profound type of brachialgia. The common form, then, of the arm pain is usually designated as a neuralgia, though there is probably some low grade of inflammatory process in the sheaths of the nerves; and if it is desired to use the term "neuritis," I do not know that serious objection could be made. So far as I am aware, no post-mortem examinations have been made of the nerves of persons suffering from this condition except of certain acute hemorrhagic cases. If we may infer concerning it by analogy, from conditions seen in sciatica, the existence of perineuritic irritation and perhaps slight exudate, is probable. Probably sometimes the inflammation is not so much in the nerve-sheaths as in the muscular and connective tissues about it, and that there is a myositis as well as secondary neuritis. I shall assume that in using the word "brachial neuralgia" I include both the neuralgias, with no objective symptoms, and the dis- tinctly neuritic cases, of non-surgical origin.
Symptoms. — The onset is rather sudden, but there may be severe preliminary aching in the arm for a few days, and sometimes there are short preliminary or abortive attacks. Usually, however, the patient wakes at night or notices in the morning a distinct and severe pain involving the upper arm and shoulder or perhaps the whole arm. The pain usually is most acute, however, on the inner and front side of the arm and in the back between the shoulders. It is very intense and runs down the forearm and into the fingers, involving sometimes the whole hand, but usually only the first, second and third fingers. It is not a darting, shooting pain, but seems to be one that is general and diffuse, involving for a time the whole arm with a pain of para- lyzing intensity. The pains exacerbate, coming on usually at night more severely, or more in the morning, and always more after exertion. Movement makes the suffering worse; but the arm can be handled gently without much pain. Some suffering is present nearly all the time, often robbing the patient of sleep; but it may let up for a few hours during the day and then comes back again with paroxysms of great intensity lasting for one or two hours.
Many patients try at first to work off the pain by exercise, thinking it a form of rheumatism, or they get a masseur who rubs the arm, and, as a rule, makes it worse. There are, however, at times, attacks that are short and abortive. I have known some to last three or four days, and in these instances the massage or the doctor get the , credit in
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 21 7
a therapeutic triumph entirely undeserved. Usually the pains pro- gress and continue for two or three weeks until the trouble is pretty firmly established, continuing then for two or three months.
When the pain first comes on there are no objective changes in the arm. It looks normal and is simply tender and sore to the touch. L,ater the arm becomes a little swollen and the fingers somewhat edem- atous, and there is slight flabbiness of the tissues, showing vasomotor change.
In the typical neuralgias there is rarely much more than this. If it is a genuine neuritis, of course atrophies and paralyses gradually de- velop, but itsis not of this type of the trouble that I speak. In brachial neuralgias proper the changes in the arm are only slight — the grip of
the hand becomes weak, the elbow-jerk is a little exaggerated at first, muscular irritability is increased and then diminished, and there is no anaesthesia.
Examination shows points of tenderness along the course of the nerve on the inner side of the arm at the elbow and over the deltoid. There is also a point of tenderness between the scapulae about at the level of the second or third dorsal vertebra. This is very painful and is characteristic. Stretching out the arm and drawing upon the brachial plexus brings out pain, just as it does in the extension of the hip in sciatica. Later there is also weakness of response to the elbow-jerk, just as in the tendo achillis in the later stages of sciatica. The tender points that one finds in brachial neuralgia are by no
Fig. 90. — The pains and painful points of brachialgia.
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DISEASES OF THE NERVOUS SYSTEM.
means always identical with the tender points of Valleix. They vary somewhat and only correspond in a general way to the distribution of the cutaneous nerves (Fig. 90).
It will be found, as we note the neuralgia from the back of the neck down to the hand, that pains in these different segments are associated with painful points along the course of the spine. For example: In occipital neuralgia there will be a well-marked tender point close up to the base of the skull over the second or third cervi- cal spines. With a cervicobrachial neuralgia the point moves down to near the first or second dorsal. With a brachial neuralgia the spinal tender point moves to the neighborhood of the third or fourth dorsal. These spinal tender points are quite characteristic of neuralgias of the upper cervical plexus, and the collaterals of the brachial plexus.
Diagnosis. — In the chronic and less severe types of arm pain one may be easily misled in diagnosis. The first and obvious name given to an arm pain is "rheumatism." The patient is always treated first for this.
It is safe to say that arm pains are never purely rheumatic, unless there is found some objective evidence of myositis, arthritis, or periar- thritis. The most difficult thing is to distinguish between real neuritis of high grade and neuralgia. Neuritis has become a popular diagnosis nowadays, and patients talk very glibly about the term. But a neuritis cannot be recognized except by some objective signs. There must be pain and also tenderness along the nerve; there must be definite areas of hyperesthesia or anaesthesia, muscular weakness, and perhaps atrophy and electrical changes. There must be some of these symp- toms in an inflammatory process that lasts three to six months.
In about half of the cases no such objective symptoms are present. The condition is not one of actual inflammation, but of exhaustion, and in the majority of cases brachial neuralgia is an exhaustion neuro- sis with some rheumatic or metabolic irritation of the nerves in addition.
When we find a herpes along with arm pain we know that we have an inflammation of the spinal ganglion and secondary neuritis and that the process is due to an infection.
Treatment. — The treatment of brachial neuralgia is of little if any avail without rest. This must be secured by a sling, and, if necessary, by splints. In a good proportion of cases some relief is gotten by the salicylates, especially aspirin, but they must be given in very large doses, just as for acute rheumatism.
Neither massage nor hot baths should be given at first. But after two or three weeks hot applications of mud or flannel can be used.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 210,
The patient must understand that, as a rule, it takes ten or twelve treatments to accomplish results.
After a course of salicylates, tonics can be given. I do not know any drug that is of any special value unless it is strychnine, in massive doses, and of its utility I am sure in chronic cases.
Arm pains, which once were due to neuritic conditions, sometimes become habit pains; the patient has a "constitutional armache," just as some have constitutional headache. The best and only treat ment for these patients is to ignore the trouble absolutely. When the pains are rather chronic, i.e., after four to six weeks, massage and electricity do some good. The patient gets well anyway in three to six months as a rule.
Fig. 91. — The location of reflex heart pains.
The disease may recur, but this is rather rare, and only in about ten per cent, of my cases.
It is rarely double, and when so is invariably an exhaustion or hysterical neuralgia, rather than a pure neuritis.
Pains in the Forearm and Fingers. — There are rarely any neu- ralgic or neuritic pains confined to the forearm alone. Here we have only the ulnar pains following the course of the ulnar nerve, and very characteristic radial pains following the back of the arm to the fingers, and least frequently median nerve pains. Sometimes the whole hand is involved in neuralgic pain, but this is usually hysterical. The hand and fingers have paresthesias and local pains due to local disease, but rarely any true neuralgia.
Heart- Arm Pains. — Cardiac arm pains are not very prominent factors in the ordinary heart disease of the hospitals, and do not
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DISEASES OF THE NERVOUS SYSTEM.
appear so dominant among the laboring classes. Almost always in persons who have a cardiac disease and who have pains in the arm there is a decidedly neurotic element. This means, that the nervous system being more sensitive and unstable, the irritating impulses from the myocardium radiate more freely and arouse disturbances in consciousness more easily. These cardiac arm pains, are gen- erally associated with some precordial pain, not infrequently with
Fig. 92. — Showing the relation of the third cervical and first thoracic ganglion (blended together) with the brachial flexus.
some pain near the scapula, and also pains on the inner side and middle of' the upper arm and middle of the forearm. They rarely extend into the fingers (Fig. 91). Sometimes there is distinctly a pain in the right arm instead of the left. The accompanying diagram shows the usual localization of these pains. There is generally a feeling that the pain comes from the left side and runs from there into the arm. The cause of this reflex disturbance is generally attributed to the fact that the third or lower cardiac ganglion of the cervical sympathetic sends fibres which communicate with the first dorsal nerve, which nerve supplies the inner side of the arm (Fig. 92).
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 221
The Sensory Neuroses of the Intercostal Nerves.
These are chiefly intercostal neuralgia, symptomatic side pains, herpes zoster. Paralyses and anaesthesias occur in connection with vertebral and spinal-cord diseases.
Intercostal Neuralgia; Side Pains — Etiology.- — -This is a very common neuralgia. It occurs much oftener in women (partly from corset pressure) than in men (seven to one). The favorite age is twenty to thirty-five ; the season, winter. Anaemia, neurasthenic and hysterical conditions, child bearing, pelvic disorder, dyspepsia, heart disease, malaria and lead poisoning are frequent causes. Exposure and muscular strain are rare exciting causes.
Symptoms. — The disease comes on suddenly. The pain in typical cases is sharp and stabbing, but not much increased by respiratory movements. There are tender points at the seat of pain, which is usually greatest over the side at the exit of the lateral nerve branches. Often a tender point is felt over the exit of the dorsal or of the an- terior branch. It is rare to find all three points. The disease runs two to six weeks, but it is sometimes very obstinate, lasting for many months. The sixth to tenth nerves are those oftenest involved. The left side is more susceptible than the right (three to one).
Pathology. — In most cases the nerve is irritated by poor or pois- oned blood. In a minority of cases the pain is reflex from stomach, pelvis or heart. Sometimes there is a neuritis.
Diagnosis. — Probably one-half the pains in the side are myalgic in nature, and should be classed as pleurodynia. These pains can be distinguished by the history of their origin and of rheumatic influ- ences, by their diffuseness and dullness, by the great tenderness on pressure, and the pain produced on taking a deep breath. There is another considerable proportion of cases in which the pains are mainly neuralgic, but yet there are some evidences of muscular complications. Some of these pains are reflex.
In the third class of cases there is the pure stabbing neuralgia. The diagnosis is based on the exclusion of pleurisy, tabes, rheumatic and reflex causes, by the character of the pain and the presence of tender points.
Prognosis. — The prognosis is good, except for a few chronic cases due probably to a degenerative neuritis. In some of these cases there is lead poisoning.
Treatment. — The most efficacious treatment is a blister and ferru- ginous tonics with quinine. In all cases the heart, pleura, stomach, and pelvic organs must be examined, and any disorder relieved.
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DISEASES OF THE NERVOUS SYSTEM.
Iodide of potassium and chlorodyne have cured some very bad cases in my experience. If there is a rheumatic and muscular element, give salicylates or the analgesics, apply heat and secure rest by adhesive straps.
Mammary neuralgia (mastodynia) is a form of intercostal neu- ralgia involving the anterior and lateral branches of the three or four upper dorsal nerves.
Etiology. — It is caused by local tumors, or it may be an essential or a traumatic neuralgia. The causes in the latter class are anaemia, pendent breasts, pressure from badly-fitting corsets and injury.
Mammary neuralgia also occurs in hysterical women and young girls sexually precocious; it may occur in pregnancy and during lactation. Many mammary pains are due simply to local disorder of the gland.
Symptoms . — -Mammary neuralgia is unilateral, often very severe, and if it occurs in middle life is liable to cause much mental depres- sion from fear of cancer.
The treatment depends upon the cause. It requires general tonic measures and attention to the proper support and protection of the gland.
Herpes Zoster {Shingles). — -This is an acute dermatitis, second- ary to a neuritis and ganglionitis.
Etiology. — Its predisposing causes are wounds, drug habits, rheu- matic, gouty and syphilitic poisons and emotional influences. The active cause is in most cases an infection, and the disease sometimes is almost epidemic. The trouble is not infrequent in tabes dorsalis. The inflammation affects not only the nerves, but the spinal ganglia, and especially the latter, so that the condition has been called a pos- terior poliomyelitis (Head).
Symptoms. — It begins gradually with the development of pain and a herpetic eruption upon one side of the trunk. It generally involves the lower dorsal nerves. The eruption follows the course of the nerve, rarely extending to the opposite side. The pain gradually subsides, and the disease itself runs its course in a few weeks. Some- times it leaves the patient with a chronic neuralgia lasting for months.
Treatment. — Local anodynes and protective ointments may be ap- plied. Antirheumatics and analgesics are to be administered inter- nally. The free use of the analgesic coal-tar products appears to lessen the severity and length of the disease. In chronic cases very large doses of strychnine are indicated.
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Sensory Neuroses of the Lumbar Nerves.
Lumbo-abdominal Pains. — The upper two lumbar nerves are almost entirely sensory. Neuralgias of these nerves are called lumbo- abdominal. A true neuralgia is rare ; but myalgic and reflex pains from disease of the pelvic organs are very commcn.
There is pain in the loins, back and buttocks, extending over the hypogastrium or genitals on one side. The pain in the back, however, is often bilateral. Painful points may be found after a time, as in intercostal neuralgia. Sometimes the pain is located in the side of the penis (penile neuralgia).
Neuralgia of the long lumbar branches is called femoral or crural. A common form of this constitutes what is called neuralgic thigh. When these nerves are subject to a lesser irritation, causing sensations of numbness and pricking along the thigh, the condition is called meralgia. In true neuralgia, the patient complains of pain in the front of the knee and the anterior and outer parts of the thigh, but has no pain posteriorly and none below the knee. The internal branches of the anterior crural nerve do not seem to be affected, while the middle and external cutaneous branches and the genito- crural nerve are involved.
Reflex Pains. — Disease of the hip or of the sacro-iliac joint or verte- brae may cause a reflex pain in the obturator nerve, localized espe- cially in the knee and back of this joint. The foetal head sometimes compresses these branches, causing a symptomatic neuralgia. Dis- eases of the internal genital organs are especially liable to ciuse re- flex pain in the lumbar nerves. Lesions of the appendix, kidney, ureters also reflect pains to the lumbar nerves. Diseases of the external genitals and bladder more often reflect pains upon the sacral nerves. In renal colic pains are felt in the ilio-inguinal and hypogastric nerves. Local disease of the psoas muscle or iliacus, in the neighborhood of Poupart's ligament, causes pains in the lumbar nerves.
Pains over the sacro-iliac synchondrosis occur in rheumatic inflam- mation of these joints. Goldthwaite has shown that in certain injuries there may be a slight dislocation and sprain of these joints. In these cases if the patient lie on his back and the leg lifted in the extended position, great local pain is elicited and the patient cannot voluntarily raise the extended leg.
Diagnosis. — Lumbar neuralgia is distinguished from lumbago by the unilateral position, the distribution and paroxysmal character of the pain and lack cf severe suffering on motion and pressure; the
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DISEASES OF THE NERVOUS SYSTEM.
tender points and the absence of any organic disease. Lumbago comes on suddenly, with a history of exposure, is bilateral and con- fined to a single group of muscles, which are tender on deep pressure. In lumbar strain the onset is also sudden, with a history of injury, great local tenderness and evidences of trauma. Most lumbar nerve- pains are reflex or symptomatic and the main object of diagnosis is to find the cause.
Fig. 93. — Areas of cutaneous hyperesthesia in disease of the stomach. Sixth, seventh, eighth and ninth dorsal nerve-segments. (Head — Fraenkel.)
The treatment is the removal of the cause. The frequent pres- ence of pelvic disease and cf anaemia and a rheumatic history must be borne in mind.
Head's Zones. — In cases of acute or subacute inflammation of the viscera, areas of skin hyperesthesia usually appear in certain definite segments corresponding to the organ diseased. The accompanying figures are furnished me by Dr. Jos. Fraenkel from cases observed by
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 225
him. The testing for these hyperaesthetic areas has sometimes diag- nostic value.
Sciatica (Neuralgia of the Sciatic Nerve; Sciatic Neuritis.) — This is a form of neuralgia occuring in middle life and characterized by intense pain in the course of the sciatic nerve. A large proportion of the cases is due to neuritis.
Fig. 94. Fig. 95.
Fig. 94. — Area of cutaneous hyseraesthesia in a case of cholelithiasis and cholecystitis. (J. Fraenkel.)
Fig. 95. — Area of cutaneous hyperesthesia in a case of cholecystitis. (J. Fraenkel.)
Etiology. — The disease occurs three times as often in men as in women, and is the only neuralgia of which this can be said. Most cases in this country occur between the ages of forty and fifty; next between thirty and forty.*
The gouty and arthritic diathesis, and occupations which lead
* Personal statistics (102 cases) and those of Dr. L. Putzel (53 cases) give: Males, 111; females, 43. Ages: 10-20, 4; 21-30, 30; 31-40, 43; 41-50, 44; 51-60, 18; 61-70. 12; 71-80, 4.
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DISEASES OF THE NERVOUS SYSTEM.
to exposure and strain, predispose to the disease. It is not rare, therefore, among laboring men. In younger persons a neurotic constitution predisposes to the disease, and in this class the trouble is more truly of a neuralgic character and less of a neuritis.
Most cases occur in the autumn and winter. The exciting causes are constipation, pressure from hard seats, exposure, muscular strain
Fig. 96. Fig. 97.
Fig. 96. — Area of cutaneous hyperesthesia in appendicitis. (J. Fraenkel.)
Fig. 97. — Area of cutaneous hyperesthesia in a case of salpingitis. (J. Fraenkel.)
from heavy work and pelvic disorders. Symptomatic sciatica may be caused by the pressure of pelvic tumors, injury to the nerves, vertebral and spinal disease ; sciatica occurs in paralysis agitans and in diabetes and it] phthisis. In elderly persons of a rheumatic consti- tution inflammatory processes about the hip-joint complicate or cause the neuralgia.
Symptoms. — The disease begins rather suddenly. Pain is felt in the back of the thigh, running down the leg in the course of the
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 227
nerve. Generally it is most marked in the thigh, extending up often into the lumbar region. Sometimes the disease begins like a lumbago ; more rarely pain is first felt in the calf or foot. The pain is in- creased by motion, and the patient holds himself in a constrained position. The pelvis is tilted up toward the sound side and the trunk inclined over to the diseased side. After a time this leads in some cases to a character- istic deformity (sciatic scoliosis) in which the convexity of the curve of the verte- bral spines is directed toward the diseased side. The pain is almost con- tinuous, with paroxysms of great sever- ity, which often occur at night. During these paroxysms the pain is sharp, burn- ing and lancinating. In the interval it is dull. Besides the pains the patient suffers from feelings of numbness, tingling, and a sense of coldness and weight in the affected limb. There are almost always tender points over the course of the nerve. These may be found at the sciatic notch, at the middle of the hip, behind the knee, just below the head of the fibula in the middle of the calf, behind the external malleolus and on the back of the foot (Fig. 98).
A pain running up the back of the thigh may be caused by pressure over the back of the knee when the leg is extended at a little more than a right angle. This is diagnostic (Gowers). If the patient lies on his back and the leg is kept extended, and then the whole limb brought slowly up until it is at an acute angle with the trunk, a sharp pain in the Fig. 98.— Painful points in sciatica, sciatic notch is felt ; this too is diag- nostic. Anaesthesia over the course of the nerves occurs very rarely. When present, it indicates a severe neuritis or injury to the nerve. Muscular wasting and weakness occur after a time, and in old and severe cases partial electrical degeneration reactions may be observed. The ankle- jerk is usually abolished, but the other reflexes remain
228
DISEASES OF THE NERVOUS SYSTEM.
normal or nearly so. Herpetic eruptions over the course of the nerve occur in rare cases. The affected limb usually feels colder and shows evidence of enfeebled vasomotor supply.
The disease usually lasts two or three months; not rarely it lasts six months or even a year or more. It has been known to extend slowly upward and involve the sacral plexus or even the spinal cord.
Pathology. — Only twelve autopsies are on record. There has been found in most cases a thickening of the nerve and a gelatinous exudate in the perineural sheath, the fluid not, however, containing evidence of cellular proliferation (J. R. Hunt). The arteries are thickened and sclerosed. The evidence points to the presence in ordinary sciatica of a perineuritis with exudate often of gouty or rheumatic origin.
Diagnosis.- — Sciatica has to be distinguished from hip-joint dis- ease, dislocation and sprains of the sacro-iliac synchondrosis organic disease of the cauda equina or cord, muscular pains in the hip and leg and from pains caused by tumors. Pure sciatic neuralgia ought also to be distinguished from sciatic neuritis. A consideration of the facts already given ought to make the diagnosis not difficult. Pure sciatic neuralgia occurs in early life and is not accompanied by much local tenderness. There is no paralysis or wasting of the limb. Double sciatica is most always symptomatic of diabetes or organic disease. True sciatica is rarely double.
Prognosis. — Almost all cases get well in from three to six months. Severe attacks in people over forty are the most intractable. Re- lapses occur, but not as a rule.
Treatment. — In all cases which are seen early, the most important indication is rest. The patient should be put to bed, and the whole lower extremity secured in a Thomas splint extending from ankle to axilla. Ice bags or linseed poultices or leeches should then be applied over the course of the nerve. A blue pill (gr. v.) may be given twice daily at first. In less severe or older cases large blisters should be applied over the nerve in the thigh, and the application repeated in a week. If there is a rheumatic history, postassium salicylate or iodide should be given in full doses. The bowels must be freely opened. Hypodermatic injections of morphin or cocain (gr. \) may be needed for a few days, the cocaine being repeated if necessary. When the disease has become more chronic a strong galvanic current may be given daily with large electrodes, one over, the lumbar region or sciatic notch, the other, which should be the positive pole, over the leg and foot. As so-called specific remedies we have oil of turpen- tine in doses of fifteen drops t. i. d., and this may be advantageously
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 229
combined with oil of gaultheria. Massive doses of antifebrin or anti- pyrin sometimes stop the pains (gr. x., q. 2 h.). There are a great many local remedies which at times prove useful. Among these mustard plasters, menthol, chloroform liniment, setons, acupuncture cups and the actual cautery and chloride of methyl can be recom- mended. Bandaging the limb in sulphur to which a little menthol is added is often very efficacious. Kneading the nerve with a glass rod and an anodyne ointment is sometimes beneficial. Very little can be expected from nerve-stretching, but it may be tried as a last resort. If tried, however, great caution should be exercised in pull- ing on the nerve. Not over thirty to forty pounds' pull should be used. The operation of cutting down on the nerve and dissecting off the sheath for a space of several inches may be tried. But in old cases the pain sometimes becomes a "habit" and local treatment is useless.
Plantar Neuralgia. — In rare cases the pain of sciatic neuralgia is limited to the plantar nerves, and is accompanied by paresthesia and even anaesthesia of this region. The condition here is probably a neuritis combined sometimes with arthritic changes. Erythrc me- lalgia may be regarded as a form of plantar neuralgia..
EryThromexalgia (red neuralgia of the feet, congestive neuralgia) is a disease affecting the feet chiefly, and characterized by burning pains and congestion of the parts.
The disease occurs usually in men in middle life, after some fever or severe physical exertion afoot. It is due sometimes to gouty habits, and I have seen it in patients who were diabetics.
The disease begins in the ball of the foot or the heel with burning pains. The trouble increases until nearly the whole sole in the dis- tribution of the plantar nerve is involved, and the pain, though worse at night, is almost continuous. It is much increased by exer- tion, the feet become very tender so that standing or walking is most painful. Meanwhile there has developed with the pain a flushing of the part upcn exertion. In bad cases the parts most affected are continuously marked by a dull, dusky, mottled redness, with some swelling. The hands may be slightly affected. Slight injuries may cause blisters and even ulcerations. The congestion usually disappears in the horizontal position and this also relieves the pain. The symp- toms are worse in warm weather. The disease is very chronic and, though not dangerous to life, makes life very miserable.
Pathology. — In the cases of erythromelalgia as described by Mitchell and others, there are: (1) A vasomotor disturbance; (2) a neuritis; and (3) in rare instances, spinal-cord disease; (4) probably in most
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DISEASES OF THE NERVOUS SYSTEM.
cases there is an obliterative arteritis. The condition in my experi- ence is closely associated with diabetes and arterial sclerosis.
The diagnosis must be made from alcoholic and gouty paramnesia, podalgia, local disease of bone and ligaments, and from reflex pains.
Treatment. — Elevation of the feet and applications of cold give temporary relief. Faradization has sometimes given help ; oftener it has not. There is nothing known which gives permanent relief. The physician must rely upon rest, bandaging, cold, anodyne appli- cations, hydrotherapy and tonics. The salicylates, iodides, mercury, nitrites, and mineral acids with strychnine may be given. Benefit in lithaemic cases is obtained from codein and an antidiabetic diet. Mechanical treatment by bandaging the foot for a short time in a rubber bandage may be tried. In old diabetic cases the outlook is serious.
Plantar Neuroses of Mechanical Origin.
Morton's neuralgia, so-called, is a neuralgia affecting the metatarso-phalangeal joint of the third and fourth toes, and is due, it is thought, to a slight luxation, with consequent pressure on a digital branch of the external plantar nerve. It sometimes affects other toes, however. It is not always due to a luxation. Incipient flatfoot may cause it, and I have seen a typical case in a pregnant woman, disappearing after confinement. The trouble occurs generally in women, and if there is a luxation the cause is external injury or shoe pressure. The treatment is not very satisfactory. It should be directed to giving rest to the foot, and the avoidance of lateral pressure on the joints by wearing a broad-soled shoe with support to the arch of the foot. Support may also be given by a broad flannel bandage. Amputation of the toe is a very certain remedy.
Tarsalgia (policeman's disease) is a painful affection, due pro- bably in most cases to an incipient flattening of the foot and stretching of the plantar ligaments. Some have ascribed it to a deep-seated contusion of the adipose cushion covering the os calcis. A chronic in- flammation of the sheath of the tendo achillis causes symptoms resembling podalgia. Probably the condition varies somewhat in different cases. It is observed in persons who have been in the habit of going barefoot, and have then gone into the army or taken civil positions obliging them to stand or walk a great deal.
It was noticed originally in the policemen of Paris, and cases have been seen in this country. The name tarsalgia was given by Duchenne. Painful foot may be caused by a slight shortening or contracticn of the tendo achillis and is relieved by stretching this tendon.
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Treatment, medical or surgical, seems to do little for the disorder, which is very chronic. Patients are better in cold weather, and when resting the feet. Leeches, the cautery, the iodides, and broad shoes with rubber heels are serviceable.
Coccygodynia is a neuralgia affecting the lower posterior branches of the sacral nerves. It occurs oftenest in women and is caused by exposure, injury and labor. Coccygeal pains occur in spinal irritation and reflexly from pelvic disease. The disease is a most annoying one, as it interferes with sitting and walking. There is often also pain at stool, and the parts are tender to pressure. The disease is usually one involving the fibrous structures of the coccyx, and is more an articular and bony than a nervous disorder. Surgical treatment, such as amputation of the coccyx, may be needed, and is sometimes effective, but not in the cases in which there is only a neurasthenia with spinal irritation, for here the trouble seems to be more a habit pain or psychosis.
Peripheral Vasomotor and Trophic Neuroses.
Symmetrical Angio-neurotic Gangrene, or Raynaud's Disease (Abortive Form Known as Digiti Mortui). — Symmetrical gan- grene or Raynaud's disease is a rare affection characterized by spasm of the vessels of the extremities, coldness, pallor, waxiness of fingers or toes, or by blueness, mottling, swelling, pain, followed often by a dry gangrene of some of the fingers or toes.
The name is a poor one and leads to confusion. The disease is better called a peripheral obliterating endarteritis, affecting oftenest the fingers and toes, but attacking at times the nose, ears and even proximal parts of the body, such as the legs. It is an evidence of many different morbid conditions. It is seen in some types of acute rheumatism, accompanied perhaps with purpura and ecchymoses.
The disease occurs usually in children and young adults. Women are affected oftener than men. Anaemia, chlorosis, and neurasthenic states predispose to it. Malarial infection, acute infectious fevers, fright, occupations that lead to exposure, such as washing, are causa- tive factors. Diabetes and syphilis are also causes of the disease.
Symptoms. — The disease comes on rather suddenly and affects oftenest two or three fingers of both hands. In its early and mild degree there are simply a coldness, numbness and waxy pallor of the fingers. The skin looks shrunken. There is slight anaesthesia. The extremities feel as if dead. After a few hours this passes away, but returns again and may finally become an almost constant condition. Beginning in one or two fingers, it may finally involve all. The
232
DISEASES OF THE NERVOUS SYSTEM.
toes, tip of the n3se and ears may be similarly affected, though this is rare in the milder form. Exposure to cold, even slight, is the common excitant of this form of the trouble, which is commonly known as "digiti mortui," "dead fingers," or "local syncope."
In severer grades the fingers become blue, swollen, and there are burning sensations and much pain, but no anaesthesia. This condition is known as that of "local asphyxia," and it is usually followed by gangrene.
/
\
Fig. 99. — Raynaud's disease. (Howard Fox.)
In the gangrenous stage small blisters appear on the distal pha- langes, which fill with bloody serum, then dry up, and beneath the scab ulceration begins, which is shallow and soon heals, leaving a scar. The process then stops. In very rare cases the whole tip of the finger or toe, including the bone, becomes involved. The process as stated may attack the ears, lips, tongue and even parts of the trunk. Along with this gangrenous process there is often a hematuria.
The dead-finger trouble may last but a few days or weeks or it may continue for months. The gangrenous stage lasts about three weeks. It lasts longer if it comes on in one finger after the other. The disease is one of months, and it is liable to recur.
SENSORY NEUROSES OF THE CEREBROSPINAL NERVES. 233
The diagnosis must be made from senile gangrene, frostbite, ergot poisoning, alcoholic neuritis, endarteritis and obstruction of nutrient vessels.
Pathology. — A neuritis has been found in some cases of so-called Raynaud's disease, but this is secondary. In most cases there has been found an obliterating endarteritis (Jacoby, Sachs). The trouble is in some cases apparently functional or at least temporary and due to the combination of an oversensitive nervous system and some irritant, such as impoverished blood, malaria, or other toxic agent, which causes spasm and probably some temporary endarteritis. In about seven per cent, of cases the disease is associated with scleroderma.
Prognosis. — The cases usually get well, but the course is long and there may be relapses. In only the rarest instances has death oc- curred, and then from a serious and progressive arterial disease.
Treatment. — Galvanism to the spine and limbs, warm applica- tions, anodynes, tonics, are indicated. Nitroglycerine, the iodides, chloral, may be tried. The frequent short application of rub- ber bandages giving a kind of massage to the vessels has been recommended.
CHAPTER XI.
DISEASES OF THE SPINAL CORD.
Anatomy and Physiology.
Anatomy. — The spinal cord is a slender, cylindrically shaped organ. It is from 42 to 45.7 cm. (sixteen and one-half to eighteen inches) long, being shorter absolutely and relatively in women. Its weight is about thirty-three grams (one ounce). It is suspended in the vertebral canal, where it reaches in all persons over one year of age as far down as the second lumbar vertebra. In new-born infants it extends to the third lumbar vertebra.
It is divided into cervical, dorsal or thoracic, lumbar and sacral portions, corresponding with the nerves it gives off. These are re- spectively four, ten and one-half, two, and one and one-half inches long (see Fig. 100). Its shape on cross-section is nearly round, except in the lower cervical region, where it is flattened antero-posteriorly. Its average diameter is 1 cm. (two-fifths of an inch). It has two swellings or enlargements, the cervical and lumbar. Their positions, size and extent are shown on the diagram. Its specific gravity is 1 .030.
It is surrounded by three membranes, all of which are continuous with the corresponding envelopes of the brain. They are the dura mater, the arachnoid and the pia mater (Fig. 101).
The dura mater is the external covering. It is a long sac attached to the edge of the foramen magnum above and extending down until its walls fuse together at the level of the second sacral vertebra. Its cavity, therefore, is much longer than the spinal cord. It is attached to the bony canal at its lower end, and is held loosely by twenty- two lateral ligaments (/. denticulata) throughout its length.
The arachnoid is a thin, semi-transparent membrane lying loosely over the cord and roots.
Internally, this is connected by numerous connective-tissue fila- ments with the innermost membrane, the pia mater. The latter is a thin vascular sheath applied closely to the cord and roots. The space between the dura and arachnoid is called the arachnoid cav- ity. It contains a very little cerebrospinal fluid. That between the arachnoid and pia is called the subarachnoid cavity; it contains a good deal of cerebrospinal fluid. Both cavities connect with those of the brain, and slightly by leakage with each other. The dura has a me- chanical protective function, the arachnoid a serous and the pia a vas- cular function.
The spinal cord is movable in its canal to the extent of from one- half to one inch.
The Nerve Roots. — The spinal nerve-roots are covered with the pia
234
r
DISEASES OF THE SPINAL CORD. 235
and arachnoid. They pierce the dura in two places and unite to form a mixed nerve. The dura mater is prolonged over the nerves as they pass through it, forming a tubular sheath. The anterior roots are the larger. At the point of exit of the nerves from the cord a slight con- striction is formed.
The Root Ganglia. — On each posterior root, outside the dura, is a posterior spinal ganglion. ' j The ganglia lie in the intervertebral canals,
j -g 8 except those on the sacral nerves.
5> -|| Fissures. — Throughout the whole length of
! \ the cord there are two median fissures, called
» I the anterior and posterior.
-i^ Columns. — These fissures and the lines
formed by the exit of the roots divide the cord into four columns — anterior, posterior and two lateral.
Fig. 10 1. — V, Body of vertebra; V, spinous pro- Fig. 100. — Showing the rela- cess; 1, ligament; 2, vessels; 3, dura mater with the
tive size of the different parts arachnoid lying directly beneath it; 4, anterior root;
of the cord. (After Allen.) 5, posterior root; 6, spinal ganglion; 7, ligament.
The Composition of the Cord. — -The cord is composed of white and gray matter. The white matter lies outside and is composed mainly of nerve-fibres, the gray matter mainly of nerve-cells. Each has also neuroglia, connective tissue, and blood-vessels. In the gray matter is a central canal lined with epithelial cells.
The gray matter is arranged, as shown in the figures, somewhat in the shape of a letter H. Its different parts are called the anterior and
DISEASES OF THE NERVOUS SYSTEM.
posterior horns and intermediate gray. At certain levels there are lateral horns. The gray matter changes in shape at different levels of the cord. It is greatest in amount at the lumbosacral junction (23.33 sq. mm.); next at the cervical enlargement (sixth cervical) (17.32 sq. mm.). It increases in amount relatively to the white matter from above downward (Fig. 102). The gray matter of the two
Clark's col.
Ant. Col.
Eighth dorsal. First lumbar.
First sacral. Third sacral.
Fig. 102. — The different levels of the cord. (Merkel.)
halves of the cord is connected by a bridge or commissure. The ante- rior part is composed of white medullated nerves and is called the white commissure. The posterior is composed of very fine nerve-fibres, mostly medullated collaterals, called the gray commissure. Between the two is the central canal, and surrounding it is the central gelati- nous substance, composed of neuroglia.
DISEASES OF THE SPINAL CORD. 237
The posterior horns reach to the periphery. They are divided, beginning from without, into the rim zone or Tissauer's column, the spongy zone, and the gelatinous substance of Rolando. The rim zone
First cervical. Second cervical.
Tntermed. cells Fccsc Ci/neat. F asc. grac///\s
Com mis. ce//s" Med„ motor cells
Lot. motor cells
Sixth cervical.
Clark's Co/. Subst. ge.la.tlrt.
\ Xi'oc XnTPrmed. •ubst, gelatin. \ pout. ■
co/. post. \ ^rr^r^i^750^-'' M
Cells oof..
wJk
w
JT/ssunx cint.
Commtss. cells,
-Jfiotor cells
Lateral horn.
7
First dorsal.
Fig. 103. — Showing arrangement of gray and white matter at different levels of the cord.
(Merkel.)
is composed of very fine nerve-fibres; the spongy zone and gelatinous substance are composed of very small nerve-cells, some having con- tinuous neuraxons (cells of Deiter) and some having rapidly branch-
DISEASES OF THE NERVOUS SYSTEM.
ing neuraxons (cells of Golgi). The substance of Rolando is extremely rich in nerve-cells and is not made up of neuroglia, as was once supposed.
To sum up, we have the gray matter — -
Arranged in: Composed of:
{anterior, a. A ground substance of neuroglia and con- lateral, nective tissue forming the substantia posterior. spongiosa. 2. Intermediate gray. b. Cell groups: internal, anterior, etc.
c. Plexuses of fine nerve fibres, e. g., in the
rim zone.
d. Masses of neuroglia:
(1) The central gelatinous substance.
(2) The periphery of the cord and the spongy zone.
e. Blood-vessels and connective tissue.
Now, taking up some of these factors in detail, we find that:
(a) The ground substance of the gray matter is made up of a fine mesh-work of fibres which are the processes of neuroglia cells and of nerve-cells. Besides this, there is some connective tissue, and there are prolongations from the base of the epithelial cells lining the cen- tral canal (Fig. 104).
(b) The nerve-cells are, in part, arranged in groups with the long axis parallel to that of the cord. The cells are surrounded by a rich plexus of dendrites and end brushes, as well as by the supporting neu- roglia matrix, a little connective tissue, and many small blood-vessels. The cell groups are named in accordance with their position — internal, antero-lateral, lateral median, posterior or sensory cells, and the cells of Clark's column (Fig. 105).
This nomenclature answers for ordinary anatomical descriptions. Histologically, we find two kinds of cells, the root cells and column or tract cells (Strangzellen). The former are cells whose neuraxons pass out to form the anterior roots. They form the great part of the an- terior horns. Deep in the anterior horns are a few root cells, whose neuraxons pass into the posterior roots and hence to the ganglionic system. The column cells are found in the posterior horns, intermedi- ate gray and to some extent in the anterior horns. Their neuraxons pass to the white matter of the same or opposite side, and furnish com- missural, associative and even long column fibres.
The anterior-horn root cells are arranged in groups, which overlap each other. Each group has the special duty of presiding over certain sets of muscles or other organs which have a common function. These cells are large in size, 35 to 100 fi (T^ to in.) ; they are multipolar, having five or six processes, one of which is an axis-cylinder process, which, in lower animals at least, gives off a collateral before it leaves the cord. The cells in the central parts of the horn are the smaller; the cells in the lumbar swelling are largest, because they are connected with long nerves. The cells of the cervical swelling are next in size. The cells of the posterior horn are small and multipolar. The cells of Clark's column are bipolar, 30 to 60 p. (-g-J-jj- to ^-g-in.) in diameter,
DISEASES OF THE SPINAL CORD. 239
and are arranged with their long diameter parallel to the axis of the cord. They are grouped together in a kind of nest at the inner and central part of the posterior horn (see Fig. 104). Clark's column is most distinct from the eighth dorsal to the second lumbar nerves, but extends up as far as the last cervical. An analogous group of cells is found at the level of the second and third sacral nerves. A small group of spindle-shaped cells lies in the intermediate gray matter at the base of the posterior horns. There are other minor groups of cells which it is not necessary to describe here.
The white matter of the cord is composed mainly of neuraxons and the collaterals of these running in a supporting net-work of neuroglia, connective tissue and blood-vessels. Surrounding it and lying just beneath the pia mater, is a thin layer of neuroglia 5 to 50 pt to -g1^ in.) thick. The neuraxons are medullated but have no neurilemma, and but few, if any, nodes of Ranvier. There are two kinds: the large (81 to 20 pt) and the small (2 to 3 ft) in diameter. The small fibres make up the postero-internal (Goll's) column entirely, and are numerous in the deep part of the lateral columns, but they are found in all regions. The fibres run up and down for the most part, but constantly send off branches to the gray matter. They are arranged in columns, the division being based partly on anatomical, partly on physiological, and partly on embryological grounds.
Anatomically, there is a simple and natural division, which we have already given, into the anterior, lateral and posterior columns, the divisions being made by the median fissures and the roots of the nerves.
On physiological and embryological grounds the columns are further subdivided as follows:
The anterior columns \ Direct pyramidal tract.
are divided into / Anterior fundamental column.
f Lateral fundamental column. The lateral | Lateral limiting layer, columns are \ Crossed pyramidal tract, divided into | Direct cerebellar tract.
[ A ventral cerebellar tract, or Gowers' tract. In the anterior f Rubro-spinal tract, and lateral \ Vestibulo-spinal tract, columns. [ Tecto-spinal tract.
Postero-internal column, or column of GoK.
Postero-external columns, f Burdach's column is divided into The posterior J or column of Burdach. -j Middle root zone, columns are ^ [ Posterior root zone,
divided into The ventral zone.
The comma. The oval zone. The triangular column. Rim zone, or column of Lissauer.
The fibres which make up these columns are of two kinds — long or projective, short or associative.
The long fibres connect the different levels of the cord with the brain, and the posterior spinal ganglia with nuclei in the upper part of the cord.
240
DISEASES OF THE NERVOUS SYSTEM.
Sixth Cervical Segment.
DISEASES OF THE SPINAL CORD.
241
The short or associative fibres connect different levels of the cord with each other, and also connect the two halves of the cord at the same levels. The long-fibre tracts lie on the periphery of the cord, as a rule; the short-fibre tracts lie near the central gray.
The names of the long-fibre tracts are the direct and crossed pyram- idal, the direct cerebellar, the antero-lateral ascending, or ventral cerebellar, the rubro-, vestibulo-, and tecto-spinal tracts, the postero- internal or column of Goll and the upper part of the column of Burdach.
The direct pyramidal tract lies along the anterior median fissure and extends down as far as the lower part of the dorsal cord. Its fibres
Fig. 105. — Showing the different components of the spinal cord.
cross over in the anterior commissure at various levels and connect with the cells of the anterior horns.
The crossed pyramidal tract extends down the whole length of the lateral column of the cord and sends its fibres to the anterior horns of the same side.
Both of the above tracts are continuations of the anterior pyra- mids or motor tracts of the medulla. These pyramids divide at the lower end of the medulla, about ninety per cent, of fibres crossing over to form the crossed pryamidal tract and ten per cent, continuing on the same side. Some of the fibres of the crossed pyramid rede- cussate (in lower animals) and enter the pyramidal tract of the side on which they started. 16
242
Cortico-spinal (pyramidal) tract
DISEASES OF THE NERVOUS SYSTEM.
Motor and inhibition
Rubro-spinal tract
Tecto-spinal tract
Cerebellar- vestibulo-spinal tract
Olivo-spinal (triangular) tract
Higher reflexes
Higher reflexes
Automatic and higher reflexes
Automatic and higher reflexes
Spino-tectal tract
Chained and spino-thalamic tracts
Ventral cerebellar (Gowers') tract
Direct cerebellar tract
Reflex
Cutaneous sensation and higher reflexes
Visceral and equilibrium Visceral and equilibrium
Posterior column tracts (Goll and Burdach) and chained tracts? Musculo-articular and cuta- neous ? sensations
The Long Tracts Between the Brain and the Spinal Cord.
DISEASES OF THE SPINAL CORD.
243
Antero-lateral^fundamental column
Posterior fundamental column (fasciculus proprius)
Postero-median tract (descending) including:
I The comma tract
( Oval tract
j Septo-m.trginal tract
Postero-lateral tract (descending) Median triangular tract
J
Marginal tract (Lissauer's)
Ventral commissural tract
The Short or Association Tracts of the Spinal Cord.
The direct cerebellar tract begins at the level of the first lumbar nerve. Its fibres originate in the vesicular column of Clark. They pass up to the cerebellum and go chiefly to the cortex of the vermis without crossing.
The ventral cerebellar tract, or column of Gowers, extends nearly the whole length of the cord. Its fibres come from the anterior com- missure and the sensory cells of the opposite posterior cornu. They pass through the medulla and pons, turn and enter the cerebellum to end in the cortex of the vermis. A few fibres pass directly to the optic thalamus.
The rubrospinal tract originates in the red nucleus; it crosses to the opposite side, passes down through the pons and medulla, runs in the lateral column of the cord forming a triangular or wedge-shaped tract along the anterior or ventral part of the crossed pyramid tract. It extends as far down as the sacral cord. Its fibres end in the anterior horns.
The vestibulospinal tract arises in Deiter's nucleus, crosses and runs in the lateral column of the cord in front of the rubro-spinal. It may extend to the anterior column. It passes down as far as the lumbar cord. Its fibres end in the anterior horns.
The tectospinal tract originates in the corpora-quadrigemina and take a similar course to the preceding tract, a median part running in the anterior and a lateral in the lateral columns.
244
DISEASES OF THE NERVOUS SYSTEM.
The above three small tracts degenerate downward and are spoken of collectively as the antero-lateral descending tracts.
The posterior columns are made of fibres that come from the pos- terior spinal ganglion and pass directly into the posterior columns, then ascend to nuclei near the medulla where they end. These fibres are called exogenous. The columns also receive some fibres from the tract cells in the posterior horns; and they are called endogenous fibres.
The exogenous fibres in the sacral lumbar and lower dorsal cord pass inward and form the postero-internal column or Goll's column.
I Fig. 106. II
I. Showing the connections of the anterior and posterior roots and cornua with each other (Cajal). A, anterior root; B, posterior root; a, collaterals; d, end-brushes.
II. Showing the association or short-fibre system of the cord (Cajal). a, anterior cornua cell; b, c, d, association fibres; e, posterior association fibres.
This is then small below and larger in the cervical region. They end in the nucleus of Goll's column (nucleus gracilis).
The exogenous fibres in the upper part of the cord pass up, lying to the outer side of the column of Goll. They form the column of Burdach and end in the nucleus of Burdach (nucleus cuneiformis).
The column of Burdach is thus not distinctly formed as a long fibre tract except in the upper part of the cord.
The names of the short-fibre columns are the anterior and the lateral fundamental columns, the lateral limiting layer and the lower part of the column of Burdach.
The posterior columns also contain three short-fibre columns whose
DISEASES OF THE SPINAL CORD.
245
cells of origin lie in the gray matter of the cord. The fibres in the cervical region lie in the shape of a comma (comma of Schultze), in the lumbar region in the shape of an oval (oval zone of Flechsig), in the sacral zone in the shape of a triangle (triangle of Gombault). Besides this there is a zone of short fibres lying close to the gray commissure the whole length of the cord — ventral zone or posterior fundamental column.
The Relations of the Different Parts of the Spinal Cord to the Peripheral Nerves, to the Brain, and with Each Other.— I will begin with a description of the way in which the anterior and posterior nerve-roots are connected to the cord; then describe the mode in which the different columns and cell groups are connected with each other; and finally I will indicate briefly the connections of the cord with the brain.
The anterior nerve roots are connected directly with the anterior- horn cells, of which they are processes, and together with which they form the peripheral motor neurons. It is possible that in man they send off collaterals before leaving the cord.
The cells of the anterior horn are surrounded by two chief sets of "end brushes," one coming from the pyramidal and other descending tracts, the other from the posterior horns and roots. Thus these cells are in relation with impulses from the brain and from the periphery.
The posterior nerve-roots originate in the cells of the spinal ganglia. These give an axon which divides and sends off a peripheral branch to form the sensory nerve, and a central branch which passes into the spinal cord forming the posterior root. On entering the cord the fibres of the posterior root divide like a T and pass up for one or more inches and down for a short distance only. These root-fibres, with their cells of origin and the sensory nerve-fibres, form together the peripheral sensory neurons.
The peripheral sensory neurons send their axons into the spinal cord through the posterior roots, and these axons divide, run up and down the cord for a short or long distance and then end in terminal arborizations about different groups of cells.
1. Some pass directly into the columns of Goll or Burdach on the same side and pass up to end in the nuclei of these columns.
2 . Some pass to cells in the posterior horns of both sides and there end.
3. Some pass to cells of the column of Clark of the same side.
4. Some pass to cells of the anterior horns of both sides.* The different parts of the spinal cord are connected by the short fibres
which unite different levels of the cord, and by commissural fibres which unite the different halves of the cord. These short and com- missural fibres originate in groups of nerve-cells lying in the central parts of the gray matter and which we have called column nerve-cells. They are small and multipolar. Some are distributed sparsely in the white columns. Fibres arise from them, run in the commissures and short-fibre tracts, and end in brushes which put the fibres in re- lation with various cell groups (Fig. 106).
*There are, according to Ingbert, about 650,000 sensory fibres in the posterior spinal roots; each cutaneous fibre supplies from 1 to 3 sq. mm. of surface.
-46 DISEASES OF THE NERVOUS SYSTEM.
General. — The spinal cord is made up of (1) peripheral motor and sensory neurons; (2) the secondary or encephalo-spinal neurons; (3) short associative neurons. Lesions of the peripheral motor neuron cause motor irritation or a severe, flaccid paralysis and atrophy. Lesions of the peripheral sensory neurons cause pain, paresthesia, ataxia and anaesthesia and secondary trophic troubles. Lesions of the secondary or cerebrospinal neurons cause a spastic paralysis with- out atrophy. Lesions of the secondary sensory neurons cause little pain or sensory irritation, but lead to anaesthesia, paresthesia, ataxia, etc.
Functions of the Wh ite Columns. — The white matter is a conductor of nerve impulses, and its functions are relatively simple. We have only to study the direction and kind of impulses carried by its various columns. The direct and crossed pyramidal tracts originate from cells in the motor cortex of the brain. They carry motor and inhibitory or chemically disturbing impulses downward from the brain. The crossed pyramidal tract crosses in the medulla to the side opposite to that where it originates, and passes down the lateral column to connect the motor cells of the anterior horns. The direct pyramidal tract runs along in the anterior column, and at different levels sends fibres across through the anterior commissure to the motor cells of the anterior horns. These tracts normally exercise a continual tonus or balancing influence on the motor-cell groups of the anterior horns; when destroyed there develop spasmodic conditions of the paralyzed part, owing to the unapposed cerebellar and peripheral tonic action.
The anterior ground bundle, lateral ground bundle and lateral limit- ing layer have the function of associating different levels of the cord and of connecting it also with nuclei in the medulla and centres in the cerebellum. The rubrospinal, vestibulospinal and tectospinal tracts are all efferent, and are concerned in automatic movements in equilib- rium and in effecting muscular tonus.
The columns of Goll and the upper part of the column of Burdach conduct special sensations from the muscles, articulations and tendon- ous sheaths via the root on the same side. When diseased, there is a loss of the sense of position of the limbs, of the power of estimating weights, and of co-ordination of muscular effort (ataxia). The fibres cross over in the medulla.
The posterior columns contain in the outer and ventral parts en- dogenous fibres, which are associative in character; i.e., are not long- tract fibres. These conduct tactile impressions and deep-pressure sen- sations. They mostly cross but not altogether. They also contain many associative fibres, connecting different levels. Pain-sense fibres and excito-reflex fibres from the posterior roots run through these columns to the anterior and posterior commissure; other fibres run through them to Clark's columns and the column of Goll. Hence the outer or external and ventral parts of the posterior column form a pathway for all kinds of afferent sensations, and when diseased there may be ataxia anaesthesia, pain and loss of reflexes. The long- fibre tracts of this column, however, are for the deep muscular sense
DISEASES OF THE SPINAL CORD.
247
and their injury alone causes only ataxia. The cerebellar tracts, both ventral (column of Gowers) and dorsal (direct cerebellar tract), carry impulses from the muscles, joints and perhaps viscera, and have to do also with co-ordination of movement and especially with maintenance of equilibrium.
The view that the column of Gowers is a pain-temperature tract seems incompatible with the established fact that the fibres practically all go to the cerebellum. The associative fibres in the gray matter and lateral columns have in part the function of carrying upward sensations of touch, pain and temperature. Thus all the long-fibre tracts have to do with deep sensation, and the shorter tracts carry cutaneous sensations. The long-fibre tracts of the posterior column do not cross until they reach the medulla, the long-fibre tracts of the lateral column do not cross until after they have reached the cerebellum, except perhaps Gower's tract.
The gray matter contains chiefly cell groups which act as centres and distributors of nerve impulses. In the anterior horns the cells have a motor and trophic function. The larger cells are at the outer parts of the horn and send fibres to the larger skeletal muscles. The more central cells are connected with small muscles and those having more delicate functions and adjustments. In the still more central and intermediate part, also, are separate trophic cells for the muscles, bones and joints, and cell groups which preside over vasomotor and secretory functions. Among these groups are the spindle-shaped cells, which send fibres to be the vasoconstrictors (Gaskill), through the anterior (Hill) and perhaps posterior roots (Gaskill). The cells of Clark's column receive fibres from the viscera. Impulses pass to these cells and thence to the direct cerebellar tract and cerebellum. Their function is to conduct impulses from the viscera relating to equilibrium and sense of position. They are analogous to the fibres of the column of Goll. According to Gaskill, Clark's column is a centre for the vasodilators. This is unlikely.
Automatic Centres. — The nerves and cells of the cord are arranged in complex groups which preside over certain functions or respond in a definite way to certain stimuli. These are called the spinal auto- matic centres. They are the cilio-spinal, secretory, vasomotor, genital, vesical and rectal. The important parts of these centres lie deep in the gray matter on either side of the central canal, but nearer the base of the posterior horns. Lesions of the white matter or of the anterior or posterior horns do not directly affect them.
The cilio-spinal centre lies in the eighth cervical and the first dorsal segment. Its stimulation causes the pupils to dilate.
The genital centres, including those for erection and ejaculation, reach from the first to the third sacral segment, inclusive.
The bladder and rectal centres are in the fourth and fifth sacral seg- ments, extending up and down a short distance, the bladder being perhaps a little lower.
The spinal vasomotor centres extend from the second dorsal to the second lumbar segments. The vasodilator nerves pass out by the anterior, the constrictor by the posterior root (Gaskill).
248
DISEASES OF THE NERVOUS SYSTEM.
The cells of the posterior horns are sensory in function and are con- nected with the tactile, pain, temperature and reflex fibres of the posterior roots.
The trophic centres for the joints, bones and skin apparently lie near the posterior horns. Their fibres pass out by the posterior roots.
Fig. 107. — Showing the relation of the spinous processes to the points of origin in the
cord of the spinal nerves.
Topography and Localization. — The neurologist and surgeon need to know, for purposes of diagnosis:
1. The relation of the spinal nerve-roots, at their point of origin, to the spinous processes. This is shown in Fig. 107. In general it will be seen that the different pairs of nerve-roots arise op-
DISEASES OF THE SPINAL CORD.
249
posite the spinous process of a vertebra one or two segments above those between which it makes its exit. Thus the sixth cervical origi- nates opposite the fourth cervical spine, the sixth dorsal between the third and fourth dorsal spines, the first lumbar between the eleventh and twelfth dorsal spines. There is considerable variation in these relations.
2. The next points desired are the special function of each pair of nerve-roots anterior and posterior, and the level of the various centres in the cord. This is shown in the following table, based on that originally devised by Starr, modified by Mills, Sachs and myself from personal experiments and the clinical and pathological observations of Thorburn and others.
25°
DISEASES OF THE NERVOUS SYSTEM.
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DISEASES OF THE SPINAL CORD.
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DISEASES OF THE SPINAL CORD.
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Muscles of Back and Lower Extremities.
Symptoms of Deficient Action.
Erector spinae; sacro- lumbalis ; longissimus dorsi.
Abdominal muscles.
Quadratus lumborum. Adductor muscles. Sartorius.
Quadriceps femoris.
Ilio-psoas.
Tensor fasciae latae.
External rotators: |
Pyriformis
Gemelli
Quadratus femoris. J Internal obturator. I External obturator. J
Gluteal muscles.
Biceps: semitendinosus and semimembranosus.
Gastrocnemius (also plantarius and soleus)
Anterior tibial muscles (tibialis anticus, ex- tensor digitorum, and extensor pollicis lon- gus).
Peroneus longus.
Posterior tibial muscle.
Peroneus brevis.
Interossei pedis et lum- bricales.
Adductor; flexor brevis and abductor. hallucis.
Dorsal nerves. Second to twelfth dorsal segments.
Dorsal nerves. Second to twelfth dorsal.
Lumbar nerves.
Obturator nerve, great sciatic and crural.
Crural. Third lumbar seg- ment.
Crural. Third lumbar.
Crural (lumbar plexus).
Fourth lumbar. Superior gluteal. Fourth
lumbar.
Sacral plexus (muscular branches). Fifth lumbar.
Obturator nerve (lumbar plexus).
f Inferior gluteal (sacral plexus). First and sec-
1 ond sacral.
I Gluteal superior. First
I and second sacral.
Sciatic. Fifth lumbar seg- ment.
Internal popliteal. Fifth lumbar.
Anterior tibial. Fifth lum- bar and first sacral.
Peroneal. First and second sacral segments.
Posterior tibial nerve. First and second seg- ments.
Peroneal. First and sec- ond segments.
Posterior tibial. First and second segments.
Posterior tibial. First and second segments.
Lordosis of lower spine; perpendicular line from sholder falls behind os sa- crum; unilateral palsy causes deflec- tion of spine toward sound side.
Lordosis with protrusion of nates and and abdomen ; other actions deficient ; cannot straighten up from recumbent position without assistance of hands.
Lateral movements of lower vertebrae imperfect.
No adduction; thigh rolls outward.
Flexion impaired; acts imperfectly.
Leg cannot be extended; to test it ask patient, who is lying down with hip bent, to stretch out the leg; when pa- tient is sitting down to extend leg. Flexion difficult; in bed thigh cannot be flexed; difficulty rising from th horizontal position.
Deficient outward rotation; leg turned inward.
No extension of thigh; great difficulty in climbing; no abduction of thigh; waddling gait, exaggerated move- ment of pelvis.
Deficient flexion; action of quadriceps may cause excessive extension; in standing thigh is flexed to excess; trunk moved backward.
Deficient flexion of foot; heel cannot be raised; cannot stand on tiptoes.
Deficient extension; "drop-foot," toes scrape floor; to clear this, excessive flexion at knee and hip; contracture of flexors and pes equinus or equin- ovarus.
Deficient abduction; plantar arch les- sened; increased by contracture. ] Flatfoot; walking tiresome.
y Deficient abduction or adduction; deformities result from deficiencies.
Abduction and adduction of toes defi- cient; paralysis of interossei; hyper- extension of first phalanges; second and third flexed (clawed foot).
Deficient flexion of toes; foot cannot be pushed off ground easily.
The blood-supply of The spinal cord is a subject of great practical importance; and I shall present the matter here in some detail.
The spinal cord is supplied with blood by branches from the verte- bral, ascending cervical and superior intercostal arteries above, and by the dorsal intercostal, lumbar and sacral arteries below. These send off small branches which enter the spinal canal through the foramen magnum above and the intervertebral foramina at the sides:
256
DISEASES OF THE NERVOUS SYSTEM.
they pierce the dura mater and are distributed on the pia mater and in the cord. The arteries that thus supply the cord are these:
|
Primary Arteries. |
Origin from |
Ending in |
|
Anterior spinal. Posterior spinal. Lateral spinal. Lateral spinal. Lateral spinal. Lateral spinal. Lateral spinal. |
Vertebral (from subclav.). Vertebral. Vertebral. Ascending cervical (from sub- clav.). Superior intercostal (from sub- clav.). Thoracic intercostal (from aorta). Lumbar (aorta). Lateral sacral (from int. iliac). |
Anterior median spinal artery. Anterior and posterior spinal root arteries. Anterior and posterior spinal root arteries. Anterior and posterior spinal root arteries. Anterior and posterior spinal root arteries. |
The anterior spinal arteries are branches of the vertebrals. They unite to form the anterior median artery, which runs down the whole length of the cord, receiving re-enforcements from the lateral arteries (Fig. 108). The anterior spinal arteries themselves nourish only a few upper segments of the cord. The anterior median artery is not, as has been taught, a true prolongation of the anterior spinals, but is really made up by the lateral spinals. In other words, the vertebral artery through its branches nourishes only the upper cervical region of the cord. The posterior spinal arteries are smaller than the anterior and unite on the posterior surface of the cord. They do not continue down as a posterior median artery — there is no such artery; but they help to form two plexuses on the postero-lateral surfaces of the cord.
The lateral spinal arteries are derived from branches of the sub- clavian artery as far down as the second dorsal root; below this point by the thoracic and abdominal aorta and the internal iliac. It is an interesting fact that at or a little below the point where the blood- supply changes from the subclavian above the heart to the aorta below, pathological disturbances frequently occur (transverse myelitis).
Root Arteries. — The lateral spinal arteries, after they enter the spinal canal, are called the root arteries. They pierce the dura mater and pass, some along the posterior and some along the anterior roots, to the cord. There are about eight anterior-root arteries (five to ten) and about sixteen posterior-root arteries (see Figs. 108, 169). The anterior arteries are twice as large (one millimetre in diameter) and one-half as numerous as the posterior. The root arteries of the cervical region are rather the more numerous. There is a large and constant anterior-root artery in the dorso-lumbar region. The last two lumbar, the five sacral nerves and the unpaired coccygeal nerve when it exists, are accompanied by small root arteries which do not reach up to the cord itself. The lower part of the spinal cord is sup- plied by large root arteries from the lateral spinal arteries. Hence, the theory of Moxon that the circulation here is feeble is not supported by Kadyi's investigations.
The Plexuses. — The anterior root arteries pass to the anterior median fissure, and then divide, partly to form the anterior median artery and partly to form a rich plexus between the anterior roots;
DISEASES OF THE SPINAL CORD. 257
Fig. 108. — The spinal cord, anterior surface, showing the nerve roots, root arteries, and anterior plexus (Kadyi.)
posterior surfaces which form "the postero -lateral arterial plexus. The posterior-root arteries do|not|anastomose to any extent with each 17
DISEASES OF THE NERVOUS SYSTEM.
other or form a posterior spinal artery, as is done by the anterior-root arteries. There are, therefore, three relatively independent arterial plexuses: the anterior plexus, the two postero-lateral plexuses.
Veins. — The veins of the spinal canal outside the dura mater have valves, those within it have none. The veins reach the pia mater and cord by passing along the nerve-roots. Hence, we have anterior and posterior root veins, corresponding to the root arteries, but more numerous, there being a total of forty or fifty. The anterior-root
Fig. 109. — The arterial supply. A, The artery of the posterior fissure; B, the inter- funuular; C, artery of posterior horn; D, of posterior root; E, of postero-lateral column.
veins are more numerous than the posterior, but smaller (twenty- five to twenty). The veins are a little larger than the arteries, the anterior veins being one-half to one millimetre, the posterior one and one-half to two millimetres, in diameter.
Thus we see that the posterior surface of the cord has more and smaller arteries, fewer but larger veins. The posterior surface is on the whole more richly supplied with veins, the anterior surface with arteries. The lateral surfaces are the least vascular.
Anterior root arteries | 5 to 10 1 mm.
Anterior root veins | 25 to 30 \ to 1 mm.
Posterior root arteries I 16 \ mm.
Posterior root veins | 20 to 25 1 \ to 2 mm.
Vessels of the Cord Substance. — The cord is supplied by (1) cen- tral arteries which are branches of the anterior median, and by (2) peripheral arteries which come from the plexuses on the pia mater.
DISEASES OF THE SPINAL CORD.
259
These two systems have been called also the centrifugal and centrip- etal, respectively. They are not absolutely independent, but are in a good measure so. The central arteries nourish chiefly the gray matter, the peripheral arteries the white. Both systems are made up of "end arteries;" i. e., they do not anastomose with each other. Neither the central nor the peripheral arteries are distributed in ac- cordance with anatomical relations or physiological functions. Each cell group, for example, has a vascular supply from several sources.
The central arteries are given off from the branches of the anterior median at the bottom of the median fissure and number about two hundred, each spinal segment having six or seven. The accompanying central veins are small and their total capacity is less than that of the arteries, so that the central arterial pressure must be high, on account of the poor venous outlet (Kadyi). Some of the blood escapes by the peripheral veins.
The peripheral arteries pass into the spinal cord for the most part along the various connective-tissue septa. There they branch and supply chiefly the white matter. They supply the apex and some of the deeper substance of the posterior horns and Clark's columns. The arteries of the posterior septum are the largest and most numerous, often reaching to the gray commissure. The peripheral arteries are smaller than the corresponding veins (0.04 to 0.2 mm.). The relation is just the reverse, therefore, to that of the central arteries and veins. The peripheral arteries are small, and after passing into the cord branch into minute vessels which pass up and down and soon become capillaries. The central arteries, on the other hand, continue large, and run up and down some distance before they are subdivided into capillaries.
To sum up: The arteries predominate in total capacity in the anterior plexus and central arteries; the veins in the posterior plexuses and peripheral vessels. The central arteries are larger and longer than the peripheral. Hence the blood circulates more quickly and under greater pressure in the central gray of the cord. Conditions of enfeebled circulation would affect the posterior columns and roots more than the anterior and central parts of the cord.
CHAPTER XII.
THE DISEASES OF THE SPINAL CORD.
There are about thirty diseases which may be classified as belong- ing to the spinal cord. Most of these are organic in character and come under the head of inflammatory and degenerative'or system diseases. Functional disorders referable to the cord alone are rare; while of organic diseases, those that result from injury and inflammation are the most common.
Etiology. — The causes of spinal-cord diseases can nearly all be formulated under the heads of injury, exposure, poisons, autotoxaemias, infections and excessive functioning. Persons of middle life are the most predisposed, while heredity does not play an important part.
Symptoms. — The symptoms of all disorders of the nervous cen- tres can be included under the heads of those of irritation, depres- sion, and perversion. The principal irritative symptoms in spinal- cord disease are pains and paresthesias of the back and limbs, hyper- esthesia and feelings of constriction around the waist, rigidity, spasms, exaggerated reflexes, and irritability of the visceral and vas- cular functions. The principal symptoms of depression and destruc- tion are anaesthesia, ataxia, paralysis, wasting and loss of power over visceral centres. The common form of paralysis in spinal-cord disease is paraplegia, in brain disease hemiplegia, in multiple neuritis quad- ruplegia. Symptoms of irritation and depression often accompany each other. The more superficial and meningeal the disease, the more are the symptoms irritative ; the more central and myelonic the trouble, the less the irritation and the more the paralysis and vis- ceral disturbance. Thus meningitis, meningeal tumors and hem- orrhages are extremely painful; while central myelitis is almost painless. .
Pathology. — Inflammations of the meninges of the cord are not rare; the opposite is true of primary inflammations of the cord itself. As will be shown later, most of the diseases that used to be called chronic myelitis are secondary to injuries and softenings. Degenerative diseases of the cord, which include such affections as locomotor ataxia and progressive muscular atrophy, used to be called "system diseases," because they affected certain lcng-fibre
260
THE DISEASES OF THE SPINAL CORD.
26l
tracts or systems of cell groups. The name implies restrictions which are not justified in fact, and it can be retained only as a matter of convenience. Secondary degenerations alone are always systemic. The cord is relatively free from abscesses, hemorrhages and tumors.
Diagnosis. — In making a diagnosis of spinal-cord diseases, one is most helped by a thorough knowledge of the cord functions. In no part of the economy do physiology and anatomy point out more clearly the path to the clinician.
Prognosis. — The spinal-cord tissue once destroyed can never be renewed, or only to a limited extent, and that as regards the nerve fibres, not the cells. It has considerable power of adjusting itself to damage; but, on the other hand, serious injury is likely to extend by the process of secondary degeneration. Functional diseases, vas- cular and nutritive disturbances of the cord can never be so severe or chronic as to exclude the possibility of recovery.
The special diseases of the spinal cord are the following:
1. Malformations : Myelocele, meningo-myelocele (spina bifida), meningocele, heteropia, amyelia, micromyelia, macrcmyelia, double cord.
2. Vascular Disorders: Angemia, hyperemia, hemorrhage, end- arteritis with aneurism, embolism or thrombosis, oedema. Secondary to these conditions are softenings and sclerosis.
3. Inflammations: Meningitis, myelitis, abscess. Secondarily, softenings, sclerosis.
4. Degenerations : Primary: locomotor ataxia, combined scleroses, hereditary scleroses,, progressive muscular atrophy and allied types.
5. Tuberculosis : Miliary and solitary.
6. Syphilis: Gumma, ineningo-myelitis, vascular disease.
7. Tumors.
8. Functional and toxic disorders.
MALFORMATIONS.
Spina Bifida (Rhachischisis Posterior).
Spinal bifida is a congenital hernia of the spinal membranes, and sometimes of the cord, through a cleft in the vertebra caused by absence of the vertebral arches. It is really a malformation of the vertebral canal rather than of the cord.
Etiology. — The condition is not very rare, about 1 child in 1,200 (French statistics) being affected. It is often associated with hydro- cephalus or with some other defect in development, such as ventral hernia, imperforate anus or pharynx. Hereditary influence is some-
262
DISEASES OF THE NERVOUS SYSTEM.
times a factor. It is a true developmental defect, and is not due to a primary dropsy of the cord, as was once taught. It occurs rather oftener in females.
Forms. — There are three varieties described :
1. Spinal meningocele is a condition in which the spinal mem- branes alone protrude into the sac.
2. Spinal meningo-myelocele is a form in which the membranes and cord both protrude.
3. Syringo-myelocele (hydrorrhachis interna) is a form in which the fluid is in the central spinal canal, and the inner lining of the sac is formed by the meninges and thinned-out spinal cord.
Anatomy.— -The first two forms are the most common and are called hydrorrhachis externa. The fluid here lies in the subarachnoid sac,
°-kca abca abca
a a a a a
Fig. iio. — Meningocele Meningo-myelocele. Syringo-myelocele. a, Vertebral walls; b, cord; c, membranes.
and hence the wall of the protruding cyst is lined with the dura and arachnoid. The nerves and cord protrude into the sac in two-thirds of the cases (forming a meningo-myelocele), but in some of these only a few nerves are found. These structures, when present in the sac, as in meningo-myelocele, lie on its posterior and median surface. They are attached to and form part of the wall. The spinal nerves therefore start from the wall of the sac and go back into the vertebral canal. The tumor contains cerebrospinal fluid, and occasionally con- nective tissue and fat (Fig. no). The external surface is often red and smooth, and there is sometimes a depression on its median sur- face where the cord is attached.
Symptoms.- — Spinal bifida occurs almost always in the lumbar and sacral region, the reason being that the laminae here are the last to solidify. Usually but two or three vertebrae are involved.
THE DISEASES OF THE SPINAL CORD.
263
The tumor varies in size from 3 cm. (one inch) to 15 cm. (six inches) in diameter, and may have a broad base or be pedunculated. The outer skin is often glossy, or tough, thickened or ulcerated (Fig. 111).
Children with spina bifida are usually feeble, badly nourished and poorly developed mentally. Paraplegia occurs in half the cases, sometimes with anaesthesia and involvement of the sphincters. Tal- ipes occurs quite often.
The prognosis is grave. Most subjects die unless treatment is applied, and even then the prospect is not very good. The prog- nosis is best for meningocele.
The diagnosis is easy. It is generally only necessary to exclude congenital tumors which happen to be located in the lumbo-sacral region. The most important question to decide is whether the cord and nerves are present in the sac. This may be assumed as probable if there is much paraplegia, anaesthesia and sphincter trouble, and if there is a depression on the median external surface. The introduction of an insulated needle connected with an electric battery may be tried.
The treatment is strictly surgical, and then is of avail only in meningocele. At present, in- jections of Morton's fluid (iodine, gr. x. ; potas. iodid., gr. xxx. ; glycerin, §i. Dose, 5i«) seem to be most successful. These injections should be made in the lateral portion of the sac, and
the child should be kept on the back. Puncture and withdrawal of fluid with compression is not a justifiable operation. Ligaturing or opening and excising of the sac are dangerous, especially if, as is often the case, part of the cord and nerves lie in the sac. In recent years, surgical results have been more favorable and warrant serious con- sideration. No surgical treatment should be attempted, however, until two or three months after birth.
Hktkropia is a rare malformation in which masses of gray matter are found in abnormal situations. A false heteropia may be caused (Van Gieson) by manipulation of the cord in its removal after death. The displaced masses consist of nerve-cells or neuroglia.
Amyblia, or absence of the spinal cord, can exist only when the brain is absent; but absence of the brain may occur without absence of the cord. In amyelia the spinal nerves are usually present. Amyelic monsters cannot live.
Fig. hi. — Spina bifida.
264
DISEASES OF THE NERVOUS SYSTEM.
Double Cord is a very rare defect and involves only part of the cord except in cases in which there is a double vertebral canal.
Double central canal is not rare. It usually involves only a part of the cord. The two canals are side by side.
Asymmetry of the cord, usually due to abnormality in the course of the pyramidal tracts, is not extremely rare.
Splitting of the cord and defects in development at special levels are occasionally observed.
MicromyELIA is a condition in which the spinal cord is abnor- mally short or small in size, and is not a very rare anomaly. The normal adult cord has a diameter in its various parts of 6 to 9 mm. (dorsal), 8 to 11 mm. (upper cervical), 15 mm. (cervical swelling), and 12 mm. (lumbar).
SPINAL HEMORRHAGE (SPINAL APOPLEXY).
This general name may be given to (1) spinal meningeal hemorrhage or haematorrhachis, and (2) hemorrhage into the cord substance, or haematomyelia.
1. Spinal meningeal hemorrhage is far the most common form. It may be outside or inside of the dura, the former being rather oftener seen.
Etiology. — It occurs in newly born children and in adults, and is more common in men than in women. Injuries, falls, fractures of the spine are the most frequent exciting causes. Severe convulsions from epilepsy, eclampsia, tetanus, chorea or strychnine may be a cause, also severe muscular exertion. Purpura and the blood states following malignant infectious fevers, bursting of an aortic or vertebral aneurism, and cerebrospinal meningitis are rare causes.
Symptoms. — In small hemorrhages there may be no symptoms. In large effusions there are sudden, very severe pains in the back, extending into the limbs, numbness, tingling, hyperesthesia and muscular spasm, especially of the back muscles. Later there may be weakness or paralysis and anaesthesia, with disorder of the visceral centres. If the hemorrhage is in the substance of the cord the anaes- thesia is often of the dissociated type; e. g., there is retention of tactile with loss of temperature and pain sense. The distribution of the anaesthesia is in such cases segmental unless the hemorrhage is very large. The symptoms reach their height usually in a few hours. Then amelioration may occur, followed by slow recovery or with symp- toms of chronic meningitis. Rarely death occurs early from exhaustion.
Diagnosis. — A history of injury or childbirth, sudden onset of
THE DISEASES OF THE SPINAL CORD.
265
attack, with symptoms of pain and irritation which rather rapidly subside, point to extradural hemorrhage. In haematomyelia there are less pain and irritation, but more profound paralysis and anaes- thesia. The same is true of crush of the cord from fracture or dislo- cation. In tetanus there is a slower development of the symptoms, and trismus is present.
The prognosis is grave in severe cases, but if the patient survives three or four days the prospect of partial or nearly complete recovery is good.
The treatment is perfect rest in bed and the administration of remedies to move the bowels and relieve pain; leeches and other local applications are of doubtful value. If there is distinct evidence of fracture or dislocation surgical interference may be indicated. It is of no use to give styptics except in purpura, when mineral acids or suprarenal extract may be tried. Later, one may give iodide and mercury and use blisters to the back.
2. Hemorrhage into the Substance oe the Cord (H/hmato- myelia) — Etiology. — -The condition is not very rare. It may be primary from disease of the blood-vessels or purpura hemorrhagica; or it may be secondary to myelitis and tumors. Primary hemor- rhage occurs sometimes in infancy, but usually in males between the twentieth and fortieth years. Infection, injuries, overexertion, exposure, excessive coitus (Gowers), syphilitic disease of the blood- vessel, and convulsions are causes. The disease sometimes occurs in old people with degenerated arteries, which break and lead to a spinal apoplexy, just as occurs in the brain. A special cause of spinal hemor- rhage is working under high atmospheric pressure, as in the caisson disease.
The symptoms develop rapidly, with at first feelings of numbness or weakness for one or two hours or longer. Then there is a sudden para- plegia, with anaesthesia or ataxia or both. The anaesthesia is often dissociated, there being loss of pain and thermic sense with reten- tion of considerable tactile sense. The sphincters may be paralyzed ; the urine has to be drawn. The reflexes may be abolished at first, but soon return and become exaggerated. There is considerable pain in the back. If the lesion is high up, the arms and thorax are in- volved. The acute symptoms begin usually to subside at the end of seven to ten days and the disease takes the character of a chronic myelitis. If improvement does not occur, evidences of acute myelitis appear and the patient dies.
Pathology. — The vessels involved are the central arteries, which supply the gray matter and are under relatively high pressure. The
266
DISEASES OF THE NERVOUS SYSTEM.
rupture of the vessel, when due to disease, is caused by a fatty de- generation of the coats or syphilitic endarteritis ; miliary aneurisms, such as are found in the brain, rarely develop in the cord. Hemorrhage often precedes or begins a myelitis, of which it may be the cause or the result. The clot may be absorbed, leaving a cavity as in the brain ; or the broken-down tissue may become the centre of a myelitic focus. The hemorrhage is usually single, but there may be several. Mul- tiple capillary hemorrhages occur, but usually only from asphyxia and convulsions or in caisson disease. It is possible that some of the cases of disseminated myelitis occurring after infectious fevers start from small hemorrhages. Hemorrhage sometimes results from the invasion of the cord by a new growth, as in syringomyelia.
Diagnosis. — The points to be noted are the sudden onset without long premonitory symptoms, and the absence of fever followed later by gradual improvement. There is much less pain than in meningeal hemorrhage, and the dissociation of cutaneous sensations is very char- acteristic. In acute softening there is less of the dissociation of sen- sations and usually a more extensive paralysis. The disease is often mistaken for acute primary myelitis, which does in fact often follow it. Meningeal hemorrhage is more painful, and there is less paralysis, more spasm and a more complete recovery later.
Prognosis. — This is often serious as regards life, and always serious as regards health. It depends on the extent and seat of the hemor- rhages. Dorsal hemorrhages are more favorable, cervical the least.
Treatment. — Absolute rest, ice bags to the spine, and small doses of aconite given early are all that can be tried, except the use of symptomatic remedies. Treatment must be applied at once. The late treatment is the same as that for myelitis.
THE CAISSON DISEASE (DIVER'S PARALYSIS).
The caisson disease is the name given to a group of symptoms characterized mainly by pains and paralysis which occur in persons who work in caissons or diving bells, and which is brought about by the sudden return from a condensed air to the normal atmosphere.
Etiology. — Persons employed in caissons or bells work usually under a pressure of from one to four atmospheres, which means a pressure of from fifteen to fifty pounds to the square inch. Accidents, rarely, if ever, cccur if the pressure is not over one atmosphere, and they are rare if the person has not been subjected to the pressure for at least an hour. The most effective cause, however, is too rapid decom- pression. Different persons vary in susceptibility to the effects of
THE DISEASES OF THE SPINAL CORD.
267
this change in the atmospheric pressure, and those unused to the work are more liable to be attacked. Naturally, the disease is seen only in men, and during the working period of life.
The symptoms set in usually very soon after the patient has come out from the caisson, but they may be delayed for half an hour to an hour. They consist of intense neuralgic pains in the lower extremi- ties, often affecting especially the joints. There is at the same time epigastric pain. Nausea and vomiting with weakness in the lower limbs, amounting in some cases to absolute paralysis, very soon appear. There may be headache, dizziness, and sometimes choking, coughing and oppression in breathing. If the paralysis is considerable, it is usually accompanied by anaesthesia. Disturbances in the sphincters, with retention of urine and constipation, may also be present. The symptoms vary very much in severity, from pain, weakness in the legs and nausea, up to frightful neuralgic attacks and complete paralysis, motor and sensory. The upper limbs are rarely affected. The disease lasts from a few hours up to several weeks. Death occurs in some of the very severe cases. The symptoms having reached their climax gradually ameliorate, and a complete cure is not infrequent. In some instances, however, the patient is left with a permanent paraplegia and the ordinary symptoms of a transverse myelitis. Hemiplegia occurs in about one-fifth of cases and still more rarely a monoplegia.
The disease in its mildest form is characterized mainly by sev- eral pains, with some weakness and dizziness which usually soon pass away. The symptoms are spoken of as "the bends." When vertigo and staggering and some mental confusion are dominant, it is called "the staggers," and the dyspnceic and coughing attack are called "the chokes."
Pathology.- — When the patient is under atmospheric pressure in the caisson, the blood is driven from the surface of the body, and the internal viscera, including the brain and cord, are congested. The sudden change from the abnormal to normal pressure produces a rapid flow of blood from the internal organs to the periphery. The viscera not inclosed in bony cavities are enabled to relieve themselves of this congestion without much harm, but the circulation in the brain and spinal cord is less elastic ; that in the spinal cord being less even than that in the brain. The result is that congestions and small hemor- rhages ensue, producing a destruction of the nerve-tissue. In other cases there seems to be a blocking up of some of the small vessels, with consequent softening of different portions of the cord and to a less extent of the brain. The most important element in causing the trouble is the escape of oxygen and carbonic-acid gas from the blood
268
DISEASES OF THE NERVOUS SYSTEM.
into the tissues or into the blood-vessels, forming gas emboli. It will be seen, however, that on the whole the serious organic changes consist in the vascular disturbances with rupture or obliteration of blood-vessels, and consequent destruction and necrosis of tissue. Following this is a reactive inflammation producing the phenomena of an ordinary acute myelitis.
Prognosis.— The painful and vertiginous types of the trouble nearly all get well. Over half of the paralytic cases recover.
Prophylaxis. — The safest age for caisson workers is from twenty to thirty-five. They should have a good muscular system, good eyes, ears, lungs and hearts. The Anglo-Saxons and negroes seem to stand the work better than the Latin races (Pelton). They should not have the status lymphaticus. They should drink but little. The most important precaution of all is slow decompression, about two pounds per minute. Hill and Greenwood assert that pressures up to seven atmospheres (about one hundred and forty pounds to the square inch) can be safely borne, if the decompression is slow enough. On coming out the workmen should not do any climbing, and should take hot coffee, not alcohol, as a drink.
Treatment. — The most essential thing for immediate treatment and relief is immediate recompression in a hospital-lock provided for the purpose. The pressure (Pelton) should be gradually increased to about that under which the patient has worked. Then in a few min- utes decompression is very slowly given.
When the disease has .developed it can be treated only by symp- tomatic remedies. The patient should be kept quiet, and given, if necessary, hypodermatics of morphine. Dr. A. H. Smith recommends the use of ergot. Later on, the various neuralgic and paralytic symptoms may be treated on the same principles as those employed in myelitis.
SPINAL HYPEREMIA, ACUTE AND CHRONIC.
Etiology. — Acute spinal hyperemia is produced by sexual excesses, violent physical exertion, suppression of menstrual discharges and certain poisons like strychnine. It occurs also in the first stage of acute inflammatory diseases.
Chronic spinal hypersemia is, so far as is absolutely known, a very rare condition. Chronic hypersemia of the membranes may be the residuum of a meningitis or of injury, and these are probably the most common causes. As to the causes of the chronic hyperemia of the cord substance itself independent of other diseases, we can say nothing definitely.
THE DISEASES OF THE SPINAL CORD.
269
The symptoms of acute spinal hyperemia are feelings of heaviness and weight in the limbs and around the loins, numbness, creeping sensations and actual neuralgic pains, weakness of the lower limbs, with twitchings of the muscles. There may be also some disturbances in the sphincters, though of this one can speak less certainly. The symptoms are nearly always confined chiefly to the lower limbs. The statement that they are increased by lying on the back and amelior- ated by lying on the face is not always true, since posture, unless greatly prolonged, influences but very slightly, if at all, the circula- tion in the spinal cord.
The symptoms of chronic spinal hyperemia, when this hyper- aemia involves the meninges chiefly, are probably somewhat identical with those of spinal irritation. They will be described under that head.
Pathology. — The circulation of the blood in the spinal cord, as has been shown in the article on anatomy, is one which it is difficult to disturb, but which, once disturbed, is rather slow in being brought back to its normal condition. Thus violent activity of the heart and great increase in the arterial pressure, and the opposite conditions of weakened heart and lowered arterial tension, appear but little to modify the spinal functions. Hence it is unlikely that the large number of clinical symptoms that have at times been attributed to hyperemia of the spinal cord, or rather to disturbances in the circu- lation of the spinal cord, have really been due to that cause.
Treatment. — The treatment of spinal hyperaemia consists in the application of cups to the back, quiet in the horizontal position, preferably upon the side or face, ice and counter-irritants to the spine, morphine and bromides internally. In the more chronic cases muriate of ammonium, iodide of potassium and the galvanic current may be used. The use of ergot, which has been recommended, in my ex- perience has been found of little or no value.
SPINAL ANAEMIA.
Even less is known in regard to the etiology and symptomatology of spinal anaemia than of spinal hyperaemia. Undoubtedly, severe hemorrhages or diarrhceal discharges, and an aortic obstruction which cuts off the circulation of the blood from the spinal cord, will produce a spinal anaemia, and when this is severe the functions of the cord are nearly abolished. But practically we do not know of any causes which produce an acute or chronic anaemia leading to serious and prolonged disturbances in the spinal functions, aside from dis-
DISEASES OF THE NERVOUS SYSTEM.
eases of the arteries of the spinal cord themselves, such as occur in advanced life. In the most profound anaemias, which must affect equally the spinal cord with other organs, very little special dis- turbance of this organ can be discovered. Here, too, the supposed test of improvement on lying on the back is, in the writer's opinion, a fallacious one. It has been customary to associate with spinal anaemia a class of symptoms characterized by pains in the back of the nature of spinal irritation, weakness of the legs amounting to para- plegia— a type of symptoms that has been called spinal concussion, but it is impossible in the present stage of science to say that a spinal anaemia actually underlies and causes this condition.
INFLAMMATION OF THE SPINAL MEMBRANES (SPINAL
MENINGITIS).
The meningeal inflammations are :
External meningitis. 1 An: ^
Internal meningitis. ) AffeCtmS the dura mater"
Leptomeningitis. j Affecting the pia mater.
Hypertrophic pachymeningitis. > Affecting both membranes.
External Meningitis, Pachymeningitis Externa.
Etiology. — The disease is rare, and always occurs secondarily to some other morbid process. This process is in most cases tuber- culosis causing caries of the vertebrae. Other causes are suppurative inflammation, such as a carbuncle, in the neighborhood of the ver- tebrae, psoas abscess, purulent pleurisy, and the extension of a neoplasm. Tuberculous pachymeningitis is so commonly associated with cord changes that it is described more fully under the head of Compression Myelitis.
Symptoms. — The symptoms are those of irritation of the motor and sensory roots; later, compression of them and of the spinal cord, local pain in the back, radiating pains, tenderness, hyperaesthesia, twitching, progressive development of a spastic paraplegia, exag- geration of reflexes, and involvement of the sphincters. Anaesthe- sia occurs in severe forms, which takes the distribution of a root anaesthesia.
The disease, when chronic, may extend to the other membranes and cord, causing myelitis.
Pathological Anatomy. — The inflammation, if acute, is generally a fibro-purulent one. The dura mater is covered by a layer of caseous,
THE DISEASES OF THE SPINAL CORD.
271
semisolid matter, often very thick and most extensive posteriorly. It involves the dura vertically for several inches. In chronic forms the deposit is made up of connective tissue and the cord is com- pressed. In purely suppurative forms the cellular tissue outside the dura is infiltrated with pus throughout a great part of the canal.
The diagnosis is based on the presence of the primary local dis- ease, the kyphosis, the radiating pains, and tenderness and by the combination of motor and sensory irritation and paralysis. Anaesthesia and sphincter troubles come late in the disease.
The prognosis is bad, if the original disease is a serious one. Still, surprisingly good results are often obtained when the disease is taken early, especially in tuberculosis cases.
The treatment consists in attention to the local caries or inflam- matorv focus. It is therefore purely surgical and mechanical.
Internal Meningitis, Pachymeningitis Interna, Hemorrhagic and Hypertrophic. Syphilis of the Dura.
Inflammation of the inner surface of the dura mater has been much written about, and but little seen. It is probably almost always only a syphilitic meningo-myelitis.
Etiology. — The disease occurs almost always in adult life. A few cases have been reported in children (Gibney). It usually affects males. Syphilis and trauma are the exciting causes. My own ex- perience is that the disease is always a syphilitic process.
Symptoms. — The disease begins gradually with symptoms of irrita- tion (irritative stage). The patient suffers from pain and stiffness in the neck. The pains radiate up to the occiput and down the back; numbness, prickling and pain are felt in the arms, more in one than the other. The pains exacerbate and are worse at night. Stiffness and cramps may effect the arms. Nausea and vomiting sometimes occur.
After five or six months, symptoms of paralysis appear (paralytic stage). The arms are affected. They become weak, atrophy occurs, associated with contractures and rigidity. There is still pain, and in addition anaesthesia, hypersesthesia and trophic changes occur. Later, paraplegia, with rigidity, exaggerated reflexes, and spinal trepida- tion develop. The patient becomes weaker, and finally dies of exhaustion or from some intercurrent trouble. The disease affects mainly the cervical cord. Usually it runs a long and painful course.
Pathology. — The disease starts as an inflammatory syphilitic ex- udate upon the surface of the dura. This leads to a chronic inflam-
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DISEASES OF THE NERVOUS SYSTEM.
matory process, and finally the cord is encircled and compressed by a dense connective-tissue mass, which involves the pia and to some extent the cord substance.
Prognosis. — -A few cases have been reported practically cured. More cases terminate in death. Sometimes, however, the disease comes to a standstill for a long time.
Diagnosis. — This must be made from tumor, myelitis, Pott's dis- ease, wryneck and progressive muscular atrophy. The history of injury, the slow progressive course, and the localization of the symp- toms, their bilateral character and the pain, a history and present evidence of syphilitic infection give the most help. In spinal tumor the symptoms at the beginning are more sharply localized. They develop more rapidly and the course of the disease is shorter than in meningitis. Tumor though rare is really much more common than pachymeningitis.
Treatment. — The syphilitic origin of the disease must be borne in mind. Counter-irritants, electricity, hydrotherapy and sympto- matic remedies for the pain and spasms are indicated.
Acute Spinal Leptomeningitis (Inflammation of the Pia Mater
of the Spinal Cord).
Etiology. — This is so rare a disease, occurring alone, that I leave it out of the category of independent disorders. The description of cerebrospinal meningitis covers sufficiently the ground.
Chronic Leptomeningitis and Meningomyelitis.
Etiology. — This disease, which used to be often diagnosticated, is now believed to be rare and always secondary to an acute process, such as a cerebrospinal meningitis, or to syphilis, vertebral tubercu- losis and perhaps chronic alcoholism. It occurs oftenest in adults and in males. Trauma, and especially concussion of the spine, used to be thought a frequent cause, but in most of such cases the trouble is simply a hyperemia or else neuralgic and functional.
The symptoms gradually develop after an acute meningitis or an injury, and they are the same in character as those of the acute process. There are pain in the back, increased on movement and radiating about the trunk and down the limbs ; tenderness along the spine, stiffness of the back, twitching and spasms in the limbs with some weakness, and later some paralysis, wasting and anaesthesia, with weakness of the bladder. These paralytic symptoms, if severe, however, mark an invasion of the spinal cord. Cutaneous eruptions,
THE DISEASES OF THE SPINAL CORD.
273
such as herpes, may appear. The symptoms run an irregular course, with periods of improvement. They always become less when the patient rests.
Pathological Anatomy. — The inflammation consists of a prolifera- tion of connective tissue (productive inflammation of Delafield). The result is a thickening and opacity of the pi i mater and arachnoid. The dura mater may be involved, but only in severe cases. The process may invade the spinal cord, causing an ''annular" or ring-like sclerosis, from which the disease extends into the cord in wedge- shaped masses at various parts, causing eventually an involvement of fibre systems and secondary degenerations up and down. This somewhat rare terminal condition is called meningomyelitis. In syphilitic meningitis the lesion usually involves only part of ,the spinal cord, usually some of the dorsal segments. Here there is the characteristic exudate of syphilis, not of a simple proliferative inflammation.
The diagnosis must be made from spinal irritation, locomotor ataxia, myelitis, vertebral caries and tetanus. In spinal irritation there are not the rigidity, severe radiating pains, twitchings, atrophy or paralysis; and neurasthenia or hysteria exists. In locomotor ataxia the knee-jerk is lost, there is ataxia, and there is little paralysis, nor is there local tenderness over the spine.
In vertebral caries the pain and tenderness are much more local- ized, and there is spasmodic fixation of the trunk. The pain is more continuous and dull, and is increased by lateral pressure and lessened by extension. There is usually also some deformity. If compression occurs there is exaggeration of the reflexes and para- plegia without much anaesthesia. After all, however, with Pott's disease there may be a local meningitis also.
Treatment. — As chronic meningitis is usually the product of syphi- lis or the relic of traumatism or of an acute process, the indications for treatment are simple. Rest is the essential thing. With this can be combined the systematic and persistent use of counterirritants. The hot iron is usually best, because its wounds heal so quickly. Cupping is also useful if done vigorously and often. Internally iodide of potassium, small doses of bichloride of mercury, nitroglycerin and digitalis may be given, also the salicylates and ergot. Both the latter drugs should be employed in large doses, if at all. Elec- tricity in the form of galvanism may be tried. Splints and plaster jackets are of much service in some cases.
is
CHAPTER XIII.
MYELITIS— INFLAMMATION OF THE SPINAL CORD.
Myelitis is an inflammation of the spinal cord. It may be acute, subacute or chronic.
It may affect the anterior horns chiefly, when it is called anterior poliomyelitis ; or both the gray and white matter, when it is called dif- fuse, disseminated myelitis and transverse myelitis. The forms are still further divided, in accordance with their location, into the cer- vical, dorsal, and lumbar. Myelitis is given different names also in accordance with its cause. Thus we have hemorrhagic myelitis, a form in which the initial process is due to or associated with a hemor- rhage; compression myelitis, due to vertebral caries and tuberculous peripachymeningitis; purulent myelitis or abcess of the cord; and tuberculous and syphilitic myelitis.
For practical purposes the following classification is sufficient:
Acute myelitis: f i. Anterior poliomyelitis.
■j 2. Transverse, or diffuse, or disseminated I myelitis:
With exudation and necrosis.
With suppuration. 3. Abscess of cord.
Chronic myelitis: J 4. Chronic anterior poliomyelitis.
With necrosis and proliferation. \ 5. Transverse myelitis.
\ 6. Compression myelitis.
Acute Transverse Myeutis (Acute Softening of the Spinal
Cord).
Acute, diffuse or transverse myelitis is a name given to several different pathological processes. It may be a local infection causing exudative inflammation, with more or less necrosis resembling acute anterior poliomyelitis. The difference in the symptoms here depends only on the severity of the infection, its rapidity of course and ex- tent. It may be initiated or accompanied by a hemorrhage or softening from thrombosis of arteries or veins, and the latter is often the case. Nor can we always clinically distinguish between the cases due to a primary infection and those due to a hemorrhage and soften- ing. Various pathogenic microbes have been found, but they are not always present, even in real infections; because the microbe after start- ing its work is absorbed and disappears.
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MYELITIS— INFLAMMATION OF THE SPINAL CORD. 275
Hence acute transverse myelitis may mean either an acute inflam- matory process or an acute softening.
Etiology. — Predisposing causes are a neuropathic constitution, the male sex, early adult life, occupations calling for exposure and muscular strain. Sifted down, it will be found that the primary acute myelitis is due to some acute infection or toxaemia, that the secondary acute forms are due to syphilitic disease of the vessels, and trauma, hemorrhage in the cord and the gas emboli of caisson disease.
Symptoms. — Prodromal symptoms are rarely present, but there may be slight paresthesias or pain in the back and limbs. Some- times there is a chill, and in a few instances convulsions have been noticed.
The initial symptoms consist of feelings of numbness, usually in the feet and legs, which seem heavy and weak. Some pain may be felt about the back. The patient soon finds that he cannot walk easily, that he moves his legs with an effort and that they feel stiff. In a few hours or perhaps not till one or two days, a paraplegia with anaesthesia has developed, and if the lesion is in the cervical cord the arms are paralyzed also. All these symptoms may come on in the daytime or during sleep. Retention or incontinence of urine and constipation are early symptoms. There may be some fever.
Symptoms of the Attack. — If the patient is examined when the malady reaches its height, it will be found that he cannot walk or stand, but can move his legs a little. He complains of a sensation like a band around his waist or at the level of the spinal lesion (girdle symptom). His legs feel numb and heavy, but there is little pain and no tenderness. Anaesthesia to touch, pain, and temperature exists in various degrees on the limbs, as high up as the lesion. The anaesthesia, if not total, is greatest to touch, next to temperature and least to pain. He has vesical anaesthesia; the urine is retained and he has to have it drawn. The bowels are constipated, but if enemata are given the faeces may pass away without his knowledge, owing to rectal anaesthesia. If the lesion is lumbar, there is aboli- tion of the sexual power; but if dorsal or cervical, strong erections may occur without the patient's feeling them. When the lesion is above the lumbar cord also, the bladder may automatically and forcibly contract and expel the urine. In time the bowels regain some power. The paralysis in the limbs affects the flexors of the feet and legs more than the extensors. The patient can push down his limbs better than he can draw them up.
The temperature of the limbs for a few days is raised, but after this it falls a few degrees below normal. The skin becomes rough, cold,
276
DISEASES OF THE NERVOUS SYSTEM.
congested ; and excessive perspiration may take place. The general bodily temperature is usually normal throughout the disease, but in
some cases a fever develops of 1020 to 1040 and continues. The prognosis is then bad.
Bed-sores may develop early, within a few days or weeks. They appear oftenest upon the buttocks and heels, and are due to trophic dis- turbance, combined with pressure and pyogenic infec- tion of the parts.
If the lesion is lumbar, the tendon and skin reflexes are much weakened and the paralysis is somewhat flaccid. The muscles also tend to waste and show degenerative reactions. If the lesion is dorsal, as is more often the case, the reflexes are present, and after a time become ex- aggerated; there is ankle clonus and extensor response on irritating the soles of the feet ; contractures and spasms develop; the legs become drawn up and deformities are produced. If the lesion is so complete as entirely to cut across the cord, there may still be some excessive mus- cular tension, but the reflexes will be abolished (Bastian). When the cervical region is attacked, the arms are in- volved as well as the legs, and generally to a severer extent. There may now be also unequal dilatation of the pupils from involve- ment of the cilio-spinal centre; and optic neuritis from some cause has been known to occur. In extensive involvement of the upper
Fig. 112. — Acute transverse myelitis of lumbar cord, showing distribution of anaesthesia. Area in lines = total anaesthesia and analgesia. Dotted area = analgesia only.
MYELITIS — INFLAMMATION OT THE SPINAL CORD.
277
part of the cord there will be paralysis of the intercostal muscles and disturbance of the heart's action.
The disease, having in a few days reached its height, usually remains stationary for a few weeks, and then, should the patient live, improvement slowly sets in. In some cases evidences of extension up- ward or downward occur (ascending or descending myelitis) ; the symptoms be- come more severe, and in a few weeks, or oftener months, death occurs.
As improvement begins, a return of sensation is first noticed (one to six months) ; this is followed by return of more or less motion (six to eighteen months). Spasms and contractures now de- velop, owing to secondary degenerations. A certain amount of ataxia from poste- rior column degeneration, with a little anaesthesia of the skin, may remain, so that, if the patient has sufficient motor power to walk some- what, he presents many fea- tures of "ataxic paraplegia"
Some improvement may be expected for from one to two years. A few cases get almost entirely well. The majority become more or less paraplegic and bedridden, in
which condition they are regarded as cases of chronic myelitis.
Pathological Anatomy. — The early changes found in acute myelitis are those of inflammation, hemorrhagic extravasation and softening.
Fig. 113. — Acute transverse myelitis of lumbar cord, showing distribution of anaesthesia. Area in lines = total anaesthesia and analgesia. Dotted area = analgesia only.
278
DISEASES OF THE NERVOUS SYSTEM.
Often it is impossible to say whether the primary process was inflam- matory or due to a hemorrhage or softening.
Macroseopieally, the cord at the affected part appears soft, swollen, and either red and hyperaemic or pale and anaemic. In rare cases no change is apparent to the naked eye. In later stages the parts are white or gray, shrunken and hard. The cord may be reduced to a thin shred. The meninges about the affected parts are often thick- ened, inflamed, and adherent.
Microscopically, if the process is primarily inflammatory we find intense congestion, distended blood-vessels, emigrated white blood- cells in great number, especially in the perivascular spaces, swollen axis cylinders and cedematous swelling of the myelin sheath, red blood-cells, cells filled with fat granules known as compound granular corpuscles, or Gluge's corpuscles. These are leukocytes which have taken up fat granules. A peculiar form of cell, stellate in shape, known as spider cells or Deiter's cells, may be seen. They are pro- liferated neuroglia cells. The nerve-cells lose their granular or reticular appearance, become homogeneous, swell up, fat granules appear in them, and a peculiar coagulation process attacks the cell and body. The cell processes retract, become thick and finally drop off. Vacuoles are sometimes seen in the nerve-cells; but these are very rare if the tissue is properly preserved. Besides the above evidences of vascular and connective-tissue activity and of cell destruction, one sees granular matter, broken-down nerve-fibres and other evidences of nerve disintegration. Pigment or extravasations of blood, and in later stages bodies resembling starch granules (corpora amylacea) may be seen. They are probably modifications of the myelin sub- stance. If the lesion is primarily softening there may be much con- gestion, but the blood-vessels are less distended and fewer white cells are found in the perivascular spaces. The connective-tissue cells are less numerous. The nerve-cells are swollen, glassy and stain badly. There is a great deal of granular matter and nerve detritus. It is thought that in inflammatory swelling of the nerve-cells they take up the carmine stain, but in degenerative changes they do not.*
The connective-tissue changes are most noticeable in the white matter. The axis cylinder and myelin sheath are here often so disin-
*In the different stages of acute degeneration, the nerve-cells, particularly those of the anterior horns, show various appearances. These have been described by Friedmann as : (i) homogeneous swelling, hyaline swelling; (2) sclerosis; (3) simple atrophy; (4) fatty and molecular decomposition; (5) cloudy swelling. The degeneration begins in the cell body and then involves the processes and the nucleus; finally the whole cell is involved. Nerve cells do not often undergo the acute general death called coagulation necrosis (Friedmann), though the process of sclerosis, so-called, resembles it somewhat.
MYELITIS — INFLAMMATION OF THE SPINAL CORD.
279
tegrated that in thin sections they drop out, leaving holes in the section and giving it a vacuolated appearance. The lesion, whether destructive or inflammatory, may extend up or down the cord, often in the course of certain tracts. Sometimes a destructive process ex- tends a long way through the central part of the cord (perforating necrosis) and sometimes central hemorrhages occur extending up or down the cord.
After three or four weeks, if the patient lives, the process of ab- sorption and cicatrization begins, and secondary degenerations are seen. The granular and fatty matters and leukocytes disappear, the blood-vessels are thick-walled and distended but less numerous, con- nective tissue gradually takes the place of the destroyed nerve-cells and fibres. The axis cylinder is the last to be destroyed and has the greatest power of recuperation. The nerve-cell, if once destroyed, is never developed again. The cord at the point or points inflamed or softened becomes reduced to a mass of connective tissue containing, perhaps, a few nerve-fibres and cells. In this stage the condition is one that is known as chronic myelitis. In some cases a cyst is formed in the affected region.
In fatal cases the inflammation and softening continue; fresh areas of cord are involved, much meningeal exudation takes place and finally death occurs. The process may in very rare cases be still more acute, suppuration and abscess occur, and here death ensues in two or three weeks.
The inflammatory and softening processes above referred to are described in accordance with their appearance as red softening, yellow softening or white softening. A form of so-called inflammation, known as inflammatory oedema, is also described. It is an abortive inflammation, "a lymphatic congestion," analogous to vascular congestion.
The diagnosis of acute myelitis must be made from hemorrhage, acute embolic or thrombotic softening, acute ascending paralysis, multiple neuritis, meningitis and meningeal hemorrhage, and hyster- ical or functional paralysis. Syphilitic spinal paralysis (Krb's type) sometimes begins quite acutely and is really an acute softening with specific exudate in the vessels and membranes. Spinal hemorrhage comes on suddenly and is usually not attended by fever. If menin- geal, it is attended with pain and soon remits. Acute softening cannot always be distinguished from acute myelitis, of which it is probably the starting-point in many cases. In softening there is no leukocytosis, the process is slower, there is less pain and the dissociation of cutane- ous sensations is less marked. In acute ascending paralysis the dis-
28o
DISEASES OF THE NERVOUS SYSTEM.
ease is progressive, there are no involvement of sensation, no atrophy, and little change in the electric irritability. In multiple neuritis the onset is slower, there are more pain and local tenderness and sensory disturbance, and the bladder and rectum are rarely involved. In meningitis there are pain and tenderness in the back and limbs, rigidity, cramps, a little paralysis, and no bladder trouble. In hys- terical paraplegia there are no marked atrophic changes, but little spasm or rigidity, no electrical changes, and the stigmata of hysteria may be found. The sensory disturbances are variable and somewhat characteristic (see Hysteria), and the knee-jerks are not greatly if at all exaggerated. Tapping the spinal canal will enable one often to exclude hemorrhage and to determine by the presence of a lympho- cytosis that there is an inflammatory process.
The diagnosis of the location of the lesion is made by studying the height of the anaesthesia, the skin reflexes (see p. 50), and the distribu- tion and extent of the paralysis. There is often a differentiation of the anaesthesia, as shown in Figs. 112 and 113. Lesions of the lumbar region involving the gray matter cause very complete para- plegia with sphincter troubles and degenerative electrical reactions. Lesions in the dorsal cord cause a less complete paraplegia, but, owing to secondary descending degenerations of the lateral column, rigidity, exaggeration of the deep reflexes and contractures occur. Lesions of the cervical cord cause paralysis of the arms, with degenerative re- actions in the muscles. The paraplegia is spastic and there is not much muscular wasting. If the lesion cuts entirely through the cord the limbs are paralyzed and may be somewhat rigid, but the deep reflexes are absent.
Prognosis. — The prognosis is worse the more complete and exten- sive the paralysis. It is worse in serious motor paralyses than when sensation is chiefly involved. It is best in dorsal myelitis and worse usually in cervical myelitis, other things being equal. Bed-sores and slight fever are unfavorable signs; so also is severe involvement of the bladder and rectum. Recovery of sensation gives good hope of recovery of some motion. Total absense of recovery of sensation and motion after six months is very unfavorable. Improvement may be expected up to eighteen months after the onset, and in some cases even longer. In compression myelitis there is more chance of recovery than in the other forms. Syphilitic cases have a more favorable prognosis.
Treatment. — -In the attack, the patient must be put to bed; leeches or wet cups should be applied to the spine, diaphoresis should be promoted, small doses of aconite and nitroglycerin should
MYELITIS — INFLAMMATION OF THE SPINAL CORD. 281
be given, and a calomel purge administered. The bladder should be watched. After a week, moderate doses of iodide of potassium should be given. After about three weeks, if there is no fever, electricity may be applied carefully and strychnine administered in small dcses. Bed-sores should be guarded against by the use of water-beds or cushions, absorbent cotton, bathing the parts with alcohol and weak solutions of tannin. Infusion of buchu, boric acid and tincture of hyoscyamus will often help the bladder disturbance. The frequency of syphilis as a cause of myelitis (nearly one-half the cases) should lead to the persistent use of mercurial inunctions and of iodide for a long time. After acute symptoms subside, tonics such as arsenic, iron and strychnine in small doses may be given. Suspension may be tried carefully ; mechanical appliances maybe used to help the rigid limbs; lukewarm baths, douches, and massage are helpful to some extent.
Chronic Myelitis (Transverse and Compression Myelitis, Erb's Syphilitic Spinal Paralysis).
Chronic myelitis is the name given to a disease characterized by a chronic inflammation of the spinal cord and to the chronic reparative processes which follow acute inflammation, injury and softening. Chronic myelitis is usually a mixture of inflammatory, reparative and necrotic processes and is the terminal stage of various forms of acute diseases.
Forms. — Different names are given to chronic myelitis in accord- ance with the part of the cord affected. Usually the disease affects only certain levels, and then it is called transverse myelitis. More rarely it is diffuse or disseminated, central or marginal. When caused by pressure from vertebral disease, it is called compression myelitis, though this is really a meningomyelitis.
Etiology. — The disease may be either primary or secondary- The primary form is somewhat rarer. It occurs chiefly in adults and in early and middle life, and much oftener in males. Exposure, shocks, infectious fevers, lead and syphilis are chief causes, but above all syphilis. Syphilis causes it by producing arterial disease and by setting up a specific inflammatory infiltration. Secondary chronic myelitis is a rather common form. It is really only the later stage cf acute myelitis, softening, hemorrhage or injury.
Meningitis may extend and cause a meningomyelitis. A neuritis may possibly ascend and cause myelitis, but such cases, if they o cur are very rare. Compression myelitis is usually a slowly destructive,
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DISEASES OF THE NERVOUS SYSTEM.
not an inflammatory process, and it begins as an external pachymen- ingitis. When all is said, chronic myelitis as usually seen is a syphi- litic myelitis, the result of injury, hemorrhage or vertebral caries.
Among 67 personal cases, there were 61 men and 6 women. The causes that can be assigned were: Syphilis, 23; injuries, 12; ex- posure, 3; acute infection, 6, of which 3 followed grip, 1 typhoid, 1 meningitis, and 1 mumps. Two were due to caisson disease; 4 were of arteriosclerotic origin occurring in the aged and 6 were due to tuberculosis. A study of the age shows that practically all the cases occur between the ages of twenty-one and fifty, and most of them between the ages of thirty-one and forty. Those cases occurring in the extreme of life are due to senile arterial changes or to tubercle or injury. There is not much difference in the age relations of the cases of syphilitic origin and those due to other causes. Between twenty-one and thirty, 5 out of 14 were of syphilitic origin. Between thirty-one and forty, 9 out of 23 were of specific origin, and syphi- lis may fairly be said to cause over one-half the cases of non-traumatic and non-tuberculous myelitis of adult life.
Symptoms. — When the disease begins primarily as a chronic affec- tion the symptoms are as given below. And since nearly all the cases are of syphilitic origin, the description of primary chronic myelitis is practically that of syphilitic spinal paralysis.
The patient notices that his legs seem heavy and easily get tired ; prickling and numb sensations are felt in the feet; occasionally a little pain develops in the back or there is a sense of constriction about the trunk. The legs are stiff, and tests often show that the reflexes are exaggerated, with ankle clonus and extensor response (sign of Babinski). There is but little wasting of them, however. The sexual power declines; the bladder gives some trouble, there being a ten- dency to retention ; the bowels are constipated. After a few weeks or months there is a partial paraplegia, with rigidity of the limbs and exaggerated reflexes. Some anaesthesia exists, and occasional pain, which is not severe and is felt more in the back than the legs.
The muscles have now wasted somewhat, but show no decided changes to the electrical current. The bladder becomes more in- volved, the urine has to be drawn, it is often alkaline, and unless care is taken cystitis develops. The patient is still able to walk, but he does so with a stiff, shuffling gait which is characteristic (Fig. 114). The disease may show signs of slowly extending up and down, more often up. The arms become involved ; weakness and stiffness, with some wasting, anaesthesia and pain, develop, or the disease may cease its progress and the patient remain partly paralyzed for years. The
MYELITIS — INFLAMMATION OF THE SPINAL CORD. 283
general health during the course of the disease deteriorates slowly ; the patients often become anaemic and have an unhealthy pallor. Eventually the paraplegia becomes complete, the patient is bedridden, the legs are atrophied, contractured and rigid, with more or less anaesthesia. Cystitis and nephritis develop, and the patient dies from these or other intercurrent diseases.
Chronic secondary myelitis, which is the form often seen, presents eventually much the same picture as that just described. In this type, however, the symptoms are worse at first, then improve or regress, then become stationary, and finally grow worse.
Symptoms of the Different Forms. — The usual type of chronic myelitis is the trans- verse dorsal or dorso-lumbar, and this gives symptoms as above described. It is, as stated , usually a syphilitic spinal disease. If the lumbar region is affected there are more paraplegia, wasting and involvement of organic centres. If the myelitis is cervical the arms are involved, there may be pupillary changes, and the respiratory muscles are partly paralyzed; the paraplegia is not so complete and the disturbances of sensation are likely to be more varied.
Compression myelitis, so-called, is usually only a compression atrophy. It is due, as a rule, to vertebral caries, but its cause may be a spinal tumor, aneurism and pachy- meningitis. Compression myelitis is dis- tinguished from other forms by its slow Fig. 114.— Chronic myelitis, onset and the presence at first of irritative
or "root" symptoms. The patient suffers from pain and tenderness localized at a certain point in the spine. The pain radiates about the trunk or down the limbs and is increased on movements.^ At about the same time some motor weakness develops, usually in the form of paraplegia. The muscles waste but slightly. The reflexes are exaggerated; twitchings, spasms and contractures finally occur, and there is developed a spastic paraplegia or quadruplegia. With this there is usually some anaesthesia, though it is not complete. The disease is oftenest in the dorsal or lower cervical region, and hence the sphincters escape until late. Locally, evidences of spine disease appear early in the form of a kyphosis.
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DISEASES OF THE NERVOUS SYSTEM.
A central or peri-ependymal myelitis can rarely be recognized with certainty. It produces less pain and irritation, but leads to muscular atrophy, disorders of sensations, such as thermo-anaesthesia, disturb- ance of vasomotor and secretory nerves and visceral centres.
Pathology. — The pia mater is thickened over the affected region and often throughout the cord. The cord itself has a gray, discolored look at the affected level, and is usually shrunken or distorted and hard to the touch. In severe cases of secondary character it is re- duced to a small size, and the membranes about it are thick and inflamed. In transverse myelitis a vertical area of only two or three inches is involved. The microscope shows that the prominent changes are loss of nerve structure, great increase of connective tissue and increase in the number of vessels, which often have thickened walls. In the more seriously diseased part little is seen but connective tissue. In parts less diseased some nerve-fibres are seen, many hav- ing evidences of partial disintegration. There is also a good deal of amorphous material studded with nuclei. Stellate cells, granule cells, and nerve-cells in various stages of degeneration are present. In the parts less affected the signs of congestion and vascular irritation are more pronounced. Secondary degenerations occur above and below the seat of the myelitis (Figs. 121 and 122). In the typical syphilitic myelitis the meninges are thickened and there is a predilection for the inflammatory change to affect the lateral columns of the cord.
Diagnosis. — This must be made from progressive muscular atrophy and amyotrophic lateral sclerosis, pachymeningitis and spinal tumor; from locomotor ataxia, multiple sclerosis and brain palsies.
In progressive muscular atrophy there is a peculiar atrophy with- out involvement of the sphincters or sensory disturbance. Tumors usually cause much more pain; the symptoms come on slowly and are more unilateral at first and more definitely localized. A spastic paraplegia occurs from brain disease and as a functional trouble. In either case there are no trophic or sensory troubles, nor is there involve- ment of the sphincters. In locomotor ataxia there is no great degree of motor paralysis, and there are peculiar ataxic and sensory disturb- ances. In multiple sclerosis there are eye symptoms, speech dis- turbances and tremor. In the early stages of multiple sclerosis of the spinal type, the symptoms resemble closely a very slowly develop- ing chronic myelitis. The diagnosis can only be made by remembering that in multiple sclerosis the patient is usually young, there is absence of much sensory trouble, and presence upon close examination of eye trouble, tremor and speech disturbance and paresthesias, ataxia is marked, the course is often a remitting one. Paralysis from
MYELITIS — INFLAMMATION OF THE SPINAL CORD. 285
brain disease is almost always unilateral, painless, spastic and free from disturbance of the visceral centres.
Prognosis.— Inflammatory processes have a tendency to cease when their reparatory and eliminative work is done. Chronic myelitis, however, is often, as has been stated, a destructive process due to some defect in vascular supply or to some mechanical irritation. Besides this, in the spinal cord secondary degenerations set in as soon as cer- tain tracts are interfered with. Hence chronic myelitis, after a period of improvement, generally progresses, and the prognosis is not very favorable. Still, patients may live from five to twenty-five years. Dorsal myelitis is the most favorable form ; compression myelitis from caries can also often be successfully treated. Those forms which come on rather rapidly are more likely to cease progressing (Gowers). Syphilitic myelitis and meningomyelitis are liable to take a pro- gressive course, like a locomotor ataxia, to become, in fact, a para- syphilitic disease. Serious involvement of the bladder is a bad sign, and naturally the prognosis is worse the more complete the paralysis.
Treatment. — In the earlier and progressive stage of chronic mye- litis rest is imperative. The patient should lie down much of the time. Counter-irritation in the form of fly-blisters, the cautery, or setons should be applied, and if no improvement results wet or dry cups used. The descending galvanic current along the spine should be tried; faradism and massage being used upon the limbs. Cold baths and cold applications must be prescribed carefully if at all. Lukewarm baths, 900 to 980 F., or half -baths with friction at 700 to 8o° F., are more likely to be useful, but even these must be tried cautiously. The first baths should last not over five minutes and should be repeated only three or four times weekly. In later paraplegic and bedridden stages, electrical and hydrotherapeutic applications should be followed up patiently and persistently. The patient now may be allowed to remain and exercise in the lukewarm bath for some time.
Internally, iodide of potassium and mercury should be first given. After thorough trial with these remedies for eight or ten weeks, the patient should be given courses of tonic treatment, using phosphates, iron, arsenic, and small doses of strychnine. The more surely the disease is non-luetic, the more should simple tonic and mechanical measures be applied. If, however, there is a syphilitic history, treat- ment directed against this should be given at least twice a year for a period of two or three months. There is much doubt in my mind whether the older remedies, like silver, ergot, gold, are of any benefit. For the bladder troubles, the internal use of boric acid, buchu, san-
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DISEASES OF THE NERVOUS SYSTEM.
dalwood, and the formalin compounds like urotropin is useful. Mechanical and surgical measures may be of some help. In some cases suspension does good, but it may do harm. Tenotomy is justi- fiable for the purpose of straightening contractured limbs. In compression myelitis suspension on an inclined plane and the plaster jacket or other support are indicated. Very long rest in bed six to twelve months, will accomplish the same result. Cases have been reported in which surgical operations for the relief of a supposed tumor have cured compression myelitis from Pott's disease. Sea voyages are often useful and are preferable to mountain climates. Rest, quiet, fresh air and a very regular life are the essentials in all climates.
Acute Anterior Poliomyelitis (Infantile Spinal Paralysis, Acute Atrophic Paralysis).
Anterior poliomyelitis is a disease of the spinal cord characterized by a motor paralysis of rapid onset, followed by muscular wasting, without sensory symptoms. It occurs at all ages, but vastly oftener in infancy; hence it is often called infantile spinal palsy.
Etiology. — The average age at the time of attack is two years. Most cases occur under ten, and four-fifths of these occur under three. It may be congenital; i. e., occur in intra-uterine life (Sinkler), and it may occur as late as sixty. Most adult cases occur under the age of thirty.* Rather more of the infantile cases occur in boys, and most of the adult cases occur in males. Race and climate afford no exemption, so far as is known. It affects the rich and poor and occurs both in conditions where the sanitation is good and bad. The great majority of cases occur during the hot, dry months of summer and fall. Nearly eighty per cent. (78.8) occur between June and Sep- tember, inclusive. Heredity has an influence in only one or two per cent, of cases.
Over-exercise and chilling of the body when heated are occasion- ally apparent causes, but often the child is in very good health. Bad conditions of the intestinal tract have been noted before the attack. Infectious fevers (oftenest measles or grip precede the attack in about seven per cent. The disease has several times occurred as an epidemic. In these cases the disease has been
*Among 50 personal cases, 39 were in children. Of the latter there were 24 males, 15 females. Ages: Under six months, 1; half to one year, 8; one to two years, 10; two to three years, 5; three to four years, 3; four to six years, 3; six to eight years, 3. Final result was palsy, chiefly in right leg, in 14; chiefly in left leg, in 5; in both legs, 5; in right arm, 4; all four extremities, 1; the remainder in various combinations. Disease followed scarlatina in 1, pertussis in 2, cholera infantum in 1, some "fever" in nearly all cases.
MYELITIS — INFLAMMATION OF THE SPINAL CORD. 287
known to affect two or more members of the same family, but it is not contagious. According to Holt, twenty-nine epidemics have been reported in the last sixty years. The number of cases rarely is over one hundred, but in the recent epidemic in New York over one thou- sand cases were observed. Epidemics occur rather more often on the coast board and in low-lying places. In the New York cases, most cases occurred in the second year, then the third, fourth, first and fifth years. Very few occurred in children over twelve. Denti- tion is rarely an exciting cause, as used to be supposed. Injuries and falls in a few instances appear to be the cause of the disease. The fact that the child is just beginning to walk at the period when most susceptible to poliomyelitis must be considered of importance, since the new movements call for an unusual activity of the spinal centres.
Symptoms. — -There are rarely any premonitory symptoms. The patient is taken with a slight fever, ioo° to 1020 F., accom- panied by vomiting; rarely there is diarrhoea or convulsions. In a few hours or a day paralysis de- velops ; sometimes the paralysis is as much as a week in developing ("subacute form"). The fever lasts from one to three or four days. There is some leukocytosis ; examination of the cerebrospinal
fluid by lumbar puncture gives rather negative results. The paralysis rapidly reaches its height, then remains stationary for a time; then improvement sets in, which reaches a certain point and stops.
We have consequently: (1) a stage of invasion — -a few hours or a week; (2) a stationary period — -one to six weeks, usually two weeks; (3) a stage of improvement — six months to a year; (4) a chronic stage.
1. The stage of invasion may be so sudden as to suggest hemor- rhage. Sometimes the child, after a restless, feverish night, wakes in the morning paralyzed. Usually the initiatory symptoms last less than a day. With or before the paralysis a fever of ioo° to
Fig. 115. — Poliomyelitis, late stage showing atrophied and shortened leg. (Schoenborn and Krieger.)
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DISEASES OF THE NERVOUS SYSTEM.
io2° F., vomiting, delirium and, much less often, convulsions which are not severe occur. There may be for a few days symptoms of meningitis with much pain, retraction of the head and trunk. After the general disturbance subsides, there may still be some pain in the back and limbs for a few days, and in rare cases the bladder is
involved so that there is retention of urine. But the dominant symptom is motor paralysis.
The paralysis is oftenest para- plegia, next one leg, next the arms and legs, and after this various combi- nations. The eye-muscles, laryngeal and respiratory muscles almost always escape in infants. In older persons the facial and other cranial nerves may be involved. In certain peculiar cases the cranial nerve nuclei are attacked, in association with the an- terior horns. When the eye-muscle nuclei are involved it is called "polio- encephalitis superior"; when cranial nerve nuclei lower down are involved it is called "polio-encephalitis in- ferior."
2. The paralysis reaches its height in from one to four days. It remains at its height for from one to six weeks, and then improvement gradually sets in. In two or three weeks a wasting of the paralyzed limb may be noticed. It is flabby, its temperature lowered and the reflexes are gone. Slight tenderness may be present, but there is no anaesthesia. 3. The stage of improvement lasts for from six to twelve months. There is gradual disappearance of the paralysis, beginning in the limbs least affected. This continues until the paralysis has left all but one or two limbs. As a rule, it is the legs alone that are finally left paralyzed. In a quarter of the cases both legs, and in half of the cases one leg, cftener the right, remain affected. The muscles waste and show the reaction of degeneration, viz., loss of faradic irritability, retention but lessening of galvanic irritability, sluggish
Fio. 116. — Poliomyelitis, late stage, showing atrophy of leg and deformity of foot. (Schoenborn and Krieger.)
MYELITIS — INFLAMMATION OF THE SPINAL CORD. 289
contractions, and sometimes polar changes. In the leg the anterior tibial group of muscles is cftenest affected, in the arm the deltoid and shoulder group. The atrophy, having reached a certain grade,
Fig. 116. — Anterior poliomyelitis in an adult. Chronic stage.
finally ceases, and then a slight improvement may set in. After the end of a year not much further spontaneous improvement can be expected.
4. The temperature of the affected limb is lowered several de-
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DISEASES OF THE NERVOUS SYSTEM.
grees, the skin has a reddish-purplish, mottled look. The bones as well as muscles of the affected limbs do not grow so fast as those of the healthy limb. Hence, in time, the foot becomes smaller and the leg shorter. Owing to the contraction of unopposed muscles deformi- ties occur. The most frequent are talipes equinus, talipes valgus and varus. Deformities of the knees, contraction of the plantar fascia, and lateral curvature of the spine also take place. The general health of the patient is usually good.
Pathology. — The disease is an acute exudative inflammation with destruction of tissue, but without suppuration. It affects chiefly the anterior cornua, especially of the lumbar and cervical enlargements (Figs. ii7,andn8). It is not, as a rule, diffuse, but often the brunt of
the trouble is felt only by certain cell groups. These are destroyed, and after a time connective tissue takes their place. In rare cases the larger part of the central gray and some of the white matter are involved. Later a certain amount of sclerosis occurs in the lateral columns. The anterior roots and motor nerves atrophy. The mus- cle tissue also wastes, and its place is supplied by connective tissue. The disease is probably due to some infectious organism or more likely its selective toxin, but so far this has not been found, although cultures and microscopical studies have been made of the cerebro- spinal fluid during the attacks.
Diagnosis. — The disease must be distinguished from multiple neu- ritis, spinal hemorrhage, cerebral palsies, birth palsies and progressive muscular atrophy. The diagnosis can be easily made in almost all cases by remembering these facts:
Fig. 117. Eig. 118.
E.g. 117. — Anterior poliomyelitis in lumbar segment. (Demaschino.) Fig. 118. — Same, much enlarged, showing congestion.
MYELITIS — INFLAMMATION OF THE SPINAL CORD.
291
1. The age of the patient.
2. The abrupt onset and rapid development of extreme paralysis.
3. The tendency to improve.
4. The absence of anaesthesia, bladder or rectal symptoms, rigidity and pain.
5. The electrical reactions.
6. The arrest of growth of the limb. Multiple neuritis and pro- gressive muscular atrophy rarely occur in children. Myelitis and hemorrhage are usually accompanied by sensory disorders, bedsores
Fig. 119. — Acute hemorrhagic poliomyelitis in a hen; observed in an epidemic in Ver- mont in which domestic animals were attacked as well as children.
and bladder troubles; cerebral palsies are usually unilateral and accompanied by symptoms of stiffness and exaggeration of reflexes.
Prognosis. — The patient rarely dies, either from the disease or its sequelae. In some epidemics, however, there has been a mortality of about 12 per cent. He always improves, but he hardly ever gets entirely well. The cases in which recovery is complete (about 5 per cent.) are those of simple exudative inflammation without any necro- sis. They are generally very acute in type. Much can be done by careful and persistent treatment and by the help of orthopaedic surgery, even in old cases. The usual course is for the patient to get back the use of all but one leg. He grows to adult life with this short and weakened member.
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DISEASES OF THE NERVOUS SYSTEM.
Treatment. — In the acute stage the child should be put to bed and kept there. Iodine or mustard plasters or leeches must be applied to the spine. Internally, a smart laxative and a diuretic must be given (calomel, gr. iij.; tartrat. potas., gr. xx.). Then tincture of aconite is to be administered in doses of one drop every half-hour as indicated by the fever. To this may be added sweet spirits of nitre. Rest is the most essential thing. The limbs should be kept quiet and warm. It is a question for the future to decide whether a prompt tapping of the spinal cord at the onset of the disease might not be useful in checking the progress of the disease. At the end of two weeks electrical appli- cations may be very cautiously made to the limbs three times a week, if there is no tenderness or fever. After four weeks, electrical treat- ment should be given daily for a month, each limb being treated for only two or three minutes. After a rest of a fortnight another four weeks' treatment may be given. Treatment should be thus applied intermittently till the end of a year. After this it can be continued or stopped according to the condition of the patient. In old cases daily treatment for one or two years will sometimes produce valuable results (G. M. Hammond). That form of electricity which causes muscular contractions most easily should be employed, and this is usually the galvanic current. Massage is a most important adjuvant to elec- tricity. It is best given daily for not over ten minutes to a single limb. It is imperative also that the physician overcome any contrac- tures which develop, by splints, rubber muscles, and, if necessary, tenotomy. Warmth is very useful. The leg should be bandaged in cotton at night, and, if necessary, hot-water bottles placed beside it. Many parents cannot afford prolonged electrical and massage treatment. In such cases they should be told to rub the limb twice daily with a stimulating liniment and wrap it in cotton or hot flannels at night.
The child should be taught to walk and exercise the limb as much as possible. Tricycles and gymnastic apparatus may often be brought into use. The legs and feet should be kept in proper position by braces, but these should not be worn all the time. Exercise in warm baths should be taken. Suture of nerves has been tried, but suitable cases are rare.
Medicines are of little value in the chronic stage. Phosphorus, strychnine, iron, arsenic, cod-liver oil, physostigma, have been rec- ommended and are sometimes prescribed for the improvement of the general health and solatii causa.
Chronic Anterior Poliomyelitis. — This name is sometimes given to the chronic stage of anterior poliomeyelitis. There is not yet
MYELITIS — INFLAMMATION OF THE SPINAL CORD. 293
established on a sure pathological basis, such a disease as a true chronic or subacute inflammation of the anterior horn-cells of the spinal cord. The cases so reported, are instances rather of subacute degenerative processes, secondary sometimes to vascular disease, and are probably related to the progressive, spinal atrophies. Degenerative change may occur rapidly and destroy nerve-cells in a few months as well as in five or six years, and this fact explains the reports of clinical cases, diagnosticated as "chronic poliomyelitis anterior."
Acute and Chronic Senile Paraplegia.
A rather sudden paralysis of both legs sometimes attacks old people as the result of thrombosis or hemorrhage of spinal arteries. In these
Fig. 120. — Lumbar cord in a case of senile paraplegia, showing atrophy of anterior cornua secondary to vascular disease.
cases the symptoms come on suddenly, as in acute myelitis, only there is not much pain. The patient has sometimes a decided paralysis, and sometimes moderate paralysis but very great ataxia, depending on the arteries involved, whether postero-lateral or central. The patient usually improves rather rapidly and may regain a large part of his strength, but relapse is likely to occur. Rest and proper atten- tion to diet and elimination are indicated.
A chronic form of paraplegia, slowly developing, is also seen in old people. It begins with simple weakness of the legs, followed by wasting and progressive development of a paraplegia. The sphincters eventually become involved. The disease affects the lower extremities first, but gradually extends, and finally involves the arms.
294
DISEASES OF THE NERVOUS SYSTEM.
The general characters are those of a progressive muscular atrophy; but the disease is distinguished from this by the fact that the sphincters become rather early involved, and that the paralysis and wasting go on together without any fibrillary contractions. The medulla and the facial and ocular muscles do not become involved. Clinically, the disease cannot be distinguished from a chronic anterior poliomyelitis which takes upon itself a progressive type, and has associated with it spastic and some sensory symptoms. Later in the trouble sphincter involvement occurs. On post-mortem, however, it is found that there is a softening of the gray matter in the anterior horns of the spinal cord, more marked in the lumbar swelling. This softening is apparently due to the thickening and obliteration of the blood- vessels from senile changes in them. (Fig. 120.)
Dr. Gowers describes a disease which he calls senile paraplegia in which there are simple weakness of the legs and slowness of move- ment, without any atrophy, sensory disturbance, or alteration in the reflexes. He considers it to be a form of paralysis agitans. The dis- ease which I have described, however, represents more truly a simple senile paraplegia. Very little can be done for this trouble therapeutically. The use of nitroglycerin, iodide of potassium, spartein, digitalis and general tonic and hygienic measures are indicated.
Acute Ascending. Paralysis (Landry's Paralysis).
Acute ascending paralysis is a disease characterized by a rapidly developing paralysis which begins in the legs and then involves in turn the trunk, arms, respiratory and throat muscles, usually ending in death. There is little disturbance of sensation, no atrophy or changes in electrical irritability, and no involvement of the sphincters.* It is thought to be related to acute anterior poliomyelitis. Wickmann under the name "The Heine-Medin disease" groups together:
1. Acute poliomyelitis.
2. Landry's paralysis.
3. Bulbar and pontine forms.
4. Encephalitic forms.
5. Ataxic forms.
6. Polyneuritic forms.
7. Meningitic forms.
8. Abortive forms.
^Subacute Spinal Paralysis of Duchenne. — The diseases described under this head are chiefly cases of multiple neuritis. In a very few there are both neuritis and myelitis.
MYELITIS — INFLAMMATION OF THE SPINAL CORD. 295
This assumes that the same infectious or toxic agent may attack the different parts of the nervous system. This is practically the con- ception taught in the earlier editions of this work.
Etiology. — The disease is a rare one. It occurs chiefly between the ages of twenty and forty; men are affected oftener than women. Exposure is an exciting cause, and it occurs sometimes after acute infectious fevers and syphilis. The form of rabies known as "para- lytic' ' causes a disease which is apparently identical with Landry's paralysis.
Symptoms. — -There may be slight premonitory symptoms for a few days, consisting of numbness in the extremities, pain in the back or limbs and malaise. The first definite sign of the disease is weakness in the legs, which rapidly increase, until in a day or two the patient cannot walk. The paralysis soon involves the arms and then the muscles of respiration; the medulla is last affected, and then respira- tion becomes difficult; swallowing and articulation may be impossible. In rare cases there are facial and eye palsies. During the course of the paralysis there is little pain or sensory disturbance, but some degree of anaesthesia may occur. The deep reflexes are abolished. There are no vasomotor and no secretory disturbances, no noticeable atrophy, and no degenerative reactions in the affected muscles. The bladder and rectum are involved only in rare cases. There may be slight initial fever, but none occurs after the disease has well set in. The mind remains clear.
The disease, as a rule, ends fatally, and it usually runs its course in less than a week. Death has occurred in forty-eight hours. On the other hand, death has been postponed three or four weeks.
In other cases the disease stops short of the medulla. The patient becomes totally or nearly paralyzed below the neck. He then begins slowly to improve, and this improvement continues for one or two years. Eventually a fair degree of health is obtained.
Variations. — The disease has been known to begin in the medulla or cervical region and descend.
Pathological Anatomy. — A number of different diseases have been described under the head of Landry's paralysis, and correspondingly a number of different anatomical changes have been found. Multiple neuritis, acute diffuse myelitis, and poliomyelitis existed in some cases. In others there was a dropsical exudation in the central canal of the spinal cord, or a hyaline change in the central arteries. In recent years decided changes in the anterior-horn cells have been detected by means of the Nissl stain ; and it is pretty well decided that the dis- ease is in its ordinary manifestations an acute toxaemia of the periph-
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eral motor neuron, a fulgurating type of anterior poliomyelitis. It, however, affects in some cases the gray matter of the brain also. The paralysis is probably due to a poison of microbic origin. In some cases certainly this poison is that of rabies ; but it is not impossi- ble that other infections may pick out and suspend the functions of the anterior cornual cells, or, as Gowers suggests, the "end brushes" of the motor tract which connect with these cells. This would explain the symptoms.
Sometimes the poison may be so great in amount and so irrita- ting as to set up a myelitis or perhaps a neuritis. Cases illustrating these facts have been reported (Eichberg, Rosenheim, Putnam). But in most cases the patient dies before the toxin can produce any in- flammatory reaction.
The prognosis is very grave, but not absolutely bad. If there is reason to suspect the case of being one of paralytic rabies, no hope can be offered.
Diagnosis. — This must be made from acute poliomyelitis, acute myelitis, acute multiple neuritis and periodic family paralysis.
Its acute ascending course, absence of fever, of anaesthesia, atrophy, decubitus, sphincter troubles, and especially the absence of degenerative electrical reactions, the family history and a history of previous attacks are sufficient to enable one to make the diagnosis. The age of the patient, and the presence or absence of an alcoholic history should be considered.
Treatment. — This consists of warm baths or packs, counter-irri- tation to the spine, laxatives and rest. Large doses of ergotin, gr. ij., every hour have been successful in one case. Salicylate or benzoate of sodium, iodide of potassium, and mercury may be tried, tapping the cord and washing it out seems a reasonable procedure.
CHAPTER XIV.
THE DEGENERATIVE DISEASES OF THE SPINAL CORD.
Introductory: The Nature and Types of Degeneration and Sclerosis. — The degenerative diseases of the spinal cord are some- times called "system diseases," and some are often spoken of as scleroses. There are no true system diseases, however, except loco- motor ataxia, progressive muscular atrophy, and amyotrophic lateral sclerosis. These should really be called "neuron" rather than "system" diseases, and this latter term is best not used as a basis of classifica- tion. The term "sclerosis," also, is somewhat misleading. Properly speaking, it is the fibroid (and neuroglia) induration which results from degeneration, destruction or inflammatory irritation. We speak of degenerative sclerosis, of an inflammatory and of a neuroglia sclerosis, or of a sclerosis of mixed origin, according to the nature of the primary disease which caused it. Some writers look upon certain primary de- generations as parenchymatous inflammations. There is no objection to this point of view as long as we agree upon the essential character of the process. The words "degeneration" and "sclerosis" are often used to indicate the same thing, one being the pathological name, the other the anatomical. I shall use the term "sclerosis" here in its pathological sense, meaning the process of hardening, in presenting a classification of the degenerations of the spinal cord.
Posterior spinal sclerosis (locomotor
a. Primary and degener- ative.
ataxia). Lateral sclerosis.
Combined sclerosis. Hereditary
ataxia.
Progressive muscular atrophy, amy-
Spinal scleroses.
otrophic lateral sclerosis. Ascending and descending degener-
b. Secondary.
i
ations.
Chronic myelitis and sclerosis fol-
c. Inflammatory.
lowing destruction of cord. Multiple sclerosis.
A few words of explanation are needed regarding these different conditions, since a world of confusion has been made on account of the
297
298
DISEASES OF THE NERVOUS SYSTEM.
different stand-points taken by writers when nerve pathology was young.
a. Primary degeneration or primary sclerosis, as one may say for convenience, is a process which begins in the nerve-tissue itself and ends in its atrophy, with substitution of connective tissue. As to its nature, so far as the microscope shows us, it is a gradual decay and death of the neurons. In some sclerotic processes, like locomotor ataxia, this decay is accompanied by the development of irritating products, leucomains or toxalbumins, which may produce so active a change in the connective tissue as to lead to something resembling a
Fig. 121. — Ascending degeneration in third cervical segment, from transverse lesion in second dorsal, showing degeneration of columns of Goll and cerebellar tracts.
secondary or reactive inflammation. This is never of high grade, how- ever, and in some forms of tabes is very slight.
In progressive muscular atrophy the decay and death produce few irritating products, though enough, perhaps, to account for the fibril- lary twitchings and occasional hypertonic condition of the muscles.
The ultimate cause of these degenerative processes is not known. The progressive character of the diseases like locomotor ataxia and progressive muscular atrophy would lead one to think that there is a poison at work and constantly acting on the diseased tissue. So far, all bacteriological examinations have failed to disclose any mi- crobe, but the fact that many degenerative processes follow infectious fevers or syphilis has led to the suggestion that pathogenic germs have poured into the system a poison, or have so modified the cellular
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 299
nutrition that there is a poison constantly thrown out, which irritates and destroys certain areas of nerve tissue. The normal immunity of certain neurons to metabolic poisons is lost.
All the primary degenerations or scleroses have a certain degree of kinship. Their causes are in many respects the same, the course of all is uniformly progressive, and one not very infrequently com- plicates another. The sharpest distinctions are found between those affecting the peripheral motor neurons and the peripheral sensory neurons. Degenerative processes implicating the former are much rarer and their course is more rapid and fatal.
Fig. 122. — Descending degeneration in eighth dorsal segment from lesion in second dorsal, showing degeneration in direct and crossed pyramidal tract, vestibulo-spinal and comma tracts.
The degenerations of the spinal ganglia and the peripheral sensory neurons are more common, slower in course, different in etiology, and much more varied in symptomatology. Locomotor and the hereditary ataxias furnish examples of this type.
b. Secondary Degenerations of the Spinae Cord. — When any of the long-fibre tracts of the cord are cut across or destroyed, there soon results a degeneration. This extends up or down in accord- ance with the direction in which the tracts carry impulses. Thus, when the crossed pyramidal tract is cut across the degeneration ex- tends down ; when the column of Goll is involved it extends up. The degenerative process begins almost immediately and is complete in a few weeks. The myelin sheath swells, gradually breaks up and disin- tegrates ; the axis cylinder is involved next. At the same time the connective tissue and neuroglia proliferate and take the place of the
3oo
DISEASES OF THE NERVOUS SYSTEM.
wasted nerves. Finally, long tracts of connective tissue have taken the place of the nerve tissue. The process may not be a complete one if the lesion does not entirely destroy the tract. The short-fibre tracts degenerate only a little way up and down.
Secondary degenerations complicate and add to the pathological change in all organic diseases of the cord. In brain disease, involving the motor tract, as in hemiplegia, secondary degeneration extends into the cord and adds to the seriousness of the disease. Degener- ations of the spinal cord, however, do not extend up to the brain except in the case of disease of the antero-lateral ascending and cere- bellar tracts.
Those forms of sclerosis found in chronic myelitis are similar to the connective-tissue scars following destructive inflammation elsewhere.
Fig. 123. — Showing on the right side the minor and short tracts degenerating after section of the cord. A, ascending cerebellar and spino-thalamic; B, rubro-spinal (de- scending); C, comma tract (descending); D, posterior sulco-marginal; L, Lissauer's. Those tracts lined across degenerate downward, the others upward.
A person who has a chronic myelitis has a cicatrix in his spinal cord. The sclerosis of multiple sclerosis is probably inflammatory also, but it is a neuroglia rather than a connective-tissue cicatrix.
Short Degenerating Tracts. — By the use of more delicate stains other degenerating tracts have been discovered.
In the lateral column, scattered fibres exist which degenerate downward. Others of the same kind are found in the anterior column. These belong to the rubro-, tecto- and vestibulo-spinal tracts.
In the posterior column are two small tracts called the "comma tracts," because of their shape. They lie at the junction of the columns
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 301
of Goll and Burdach in the position shown in Fig. 123. The fibres degen- erate downward in transverse lesions of the cervical and dorsal cords. Usually this does not extend more than an inch. The tract may escape degeneration in locomotor ataxia.
There is a descending tract also in the lumbo-sacral region of the cord, corresponding in a measure to the oval zone of Flechsig. As described by Bruce, it lies along the posterior median septum above, while below it spreads out along the posterior margin. Bruce has called it the posterior septo-marginal tract (Fig. 123, D).
LOCOMOTOR ATAXIA (Posterior Spinal Sclerosis, Tabes Dorsalis).
Definition. — Locomotor ataxia is a chronic progressive disease, involving primarily the posterior spinal ganglia, roots and neurons, and later the spinal cord and peripheral nerves. It is characterized clin- ically by inco-ordination, pains, anaesthesia, and various visceral, trophic and other symptoms, and anatomically by a degenerative sclerosis chiefly marked in the posterior columns of the cord and pos- terior roots, and to a less extent in the peripheral nerves.
Forms. — Besides the common and typical form, there are anomal- ous and complicated types.
Etiology. — The disease occurs oftenest in middle life, between thirty and forty, next between forty and fifty. It may occur as early as the tenth and as late as the sixtieth year. In the very early cases it is usually due to hereditary syphilis. It is much more com- mon in males. Hereditary influence is very unimportant and is only indirect; i. e., the parents may be neurotic. Diathetic influence is slight. Exposures to wet and cold, combined with muscular exer- tions, are effective causes. Soldiers, travelers and drivers are rather more susceptible. Excessive railroad traveling, excessive dancing with exposure, favor the development of the disease. Excessive sexual intercourse, combined with irregular living, is a predisposing cause. Syphilis is by far the most important single factor. A his- tory of the disease is obtained in from sixty per cent, to ninety per cent, of the cases. In my own cases sixty-five per cent, had had syphilis. The patient has usually contracted the venereal disease ten
f 1. Common form.
Complicated forms.
{ 4. With initial optic atrophy, f With muscular atrophy.
302
DISEASES OF THE NERVOUS SYSTEM.
or fifteen years before, and has rarely had noticeable secondary symptoms.* Syphilis in its ordinary sense is not a direct factor, but prepares the system for the degenerative process. Syphilis followed later by excesses — mental or physical — and by exposures especially tends to produce locomotor ataxia. Lack of proper treatment for syphilis is believed to favor the development of the disease, but a careful study of the statistics of my clinic and of his own cases by Dr. Joseph Collins seems to show that antisyphilitic treatment as formerly carried out does not prevent the disease if the other favor- ing conditions, such as exhausting work and sexual or alcoholic ex- cesses, are present.
Among exciting causes are profoundly depressing emotions, acute infective diseases like typhus, pneumonia and rheumatism, difficult labors with severe hemorrhage, prolonged lactation and excessive smok- ing. Kdinger lays great stress on the overuse of the limbs. Trauma as an apparently exciting cause is noted in a very small percentage of cases. In my experience there has been evidence that the disease was already in the system but had not been recognized in these cases.
Symptoms. — The disease is generally divided into three stages : the initial or pre-ataxic, the ataxic and the paralytic.
i. The pre-ataxic or initial stage. Previous to any other notable symptoms, the patient may have for months or years attacks of sharp pains darting through his limbs or about his trunk. With this he may have only an Argyll-Robertson pupil and loss of knee-jerk. Or instead of the pains he may have for a long time gastric crises. Occassionally nothing is noted for a long time but loss of sexual power. A gradual development of optic atrophy and blindness may also precede nearly all other symptoms. Ocular palsies and hypotonia may also be pre-ataxic symptoms. In many cases this pre-ataxia is
*The following statistics from my own experience show the physiognomy of the disease in a cosmopolitan American city. Total cases, 190; males, 173; females, 17. Ages when disease began: Twenty-one to thirty, 19; thirty-one to forty, 84; forty-one to fifty, 60; fifty-one to sixty, 22; sixty-one to seventy, 5. Average age at time of onset, 40; beginning a year or two earlier in private patients,, and in those with a history of syphilis and active antisyphilitic treatment (Collins). Percentage of syphilis, 65. This is making the most liberal allowances. In one set of eighteen hospital patients, all carefully investigated, the percentage was 70. Average period between infection and tabes, fifteen years, ranging from one and one-half to twenty-five years. This is much longer than Erb's estimate, but is reached by three independent studies of my cases. Average duration of disease when seen by me, eight years; average duration of life in five fatal cases, twelve years, ranging from five to twenty years. Complications; optic atrophy, 6 per cent; marked arthropathies, 5 per cent; with general paresis, 4 per cent; paraplegia, 1.5 per cent; hemi- plegia, 1.6 per cent; eye palsies, 8 per cent. About 10 per cent, of 300 private cases were complicated with paresis — tabo-paresis. The complication is smaller in dispensary cases. High tabes (arm tabes), 5 per cent.; cranial nerve palsies, 10 per cent.; hemiplegia oc- curred in 3 cases, ophthalmoplegin in 1 case, progessive muscular atrophy in 3, laryngeal palsies in 3.
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 303
nearly all that the patient ever has, and this is particularly true of neuralgic and optic tabes.
When the ataxic process sets in the patient notices a slight uncer- tainty in walking, especially at night; he has numb feelings in his feet, and at times darting pains in the legs or rectum. His sexual function becomes weak, his control over the bladder slightly impaired. He has temporary attacks of vertigo and of double vision. A continuous sense of profound weariness oppresses him, even though he has made no exertion. The knee-jerk is lost. Some hypotonia may be present and there is lympho- cytosis of the cerebrospinal fluid. Such symptoms may last a few months or several years.
2. The ataxic stage. The gait now becomes so unsteady that others notice it; the pa- tient has to have help, and when walking watch his feet and the ground. If he stands with his eyes closed, he totters and may fall. His feet feel as though there was a layer of cloth or cotton between the soles and the ground. Parox- ysms of lightning-like pains attack the legs, and tests show anaesthesia present in the toes Fig. 124.— Showing station in second stage of and feet or in areas on the locomotor ataxia,
legs and on the trunk. A
sense of constriction is felt around the waist. The sexual power is often lost; the bladder is weak, and care has to be taken to empty it. The bowels are constipated ; at times he has attacks of intense pain in the epigastrium, with vomiting and perhaps a diarrhoea coming on without cause. The pupils are small and do not react to light, but do react to accommodation; vision is still good. The inco-ordination
DISEASES OF THE NERVOUS SYSTEM.
and pain and anaesthesia after a time affect slightly the arms. The muscles become relaxed and atonic. This stage lasts several years.
3. The paralytic stage. After several years with various remis- sions and improvements, the patient loses altogether the power of walking. His legs are somewhat wasted, but the muscular strength is fairly good. The anaesthesia and ataxia are very great. The patient does not feel the prick of a pin or touch of the hand ; nor with closed eyes does he know where his legs are. His bladder is anaesthetic and paretic, so that the urine has to be drawn. The pains are much less, but are still present at times. The arms are more involved, but never so seriously as to make them useless like the legs. The intel- ligence remains good,, and the patient may continue bedridden for years, dying finally from some intercurrent affection.
The following table shows the prominent symptoms in the usual order of their appearance:*
|
First Stage (Half to twenty years) |
Second Stage. (Two to ten years.) |
Third Sta^e. (Two to ten years) |
||
|
Motor |
Eye palsies. Ataxia. Muscular weakness and hypotonia. |
Less. Increased. Paresis, increased. Increased. |
Increased. Paraplegia, Increased. |
|
|
Sensory |
Pains. |
Pains. Anaesthesia. |
Painless. Increased. |
|
|
Exci to-reflex j |
Loss of knee jerk. A.-R. pupil. |
|||
|
Trophic |
Arthropathies. |
More rare. |
Rare. |
|
|
Visceral . . . . - |
Sexual weakness. Vesical weakness. Constipation. Gastric crises. |
| Increased. Decreased. |
Increased. Decreased. |
|
|
Special senses ■ |
Diplopia. [Rare. Optic atrophy. Rare. Deafness. |
Increased. Increased. |
||
Analysis of Symptoms. — A study of the symptoms in tabes pro- duces enormous results in the way of clinical phenomena. The
*The disease attacks different levels of the cord in about the following proportion: Optic tabes, 5 per cent.; high (arm) tabes, 5 per cent.; dorsal (abdominal and trunk) tabes, 20 per cent. ; lumber and sacral tabes, 69 per cent. To these add tabo-paresis 10 per cent.
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 305
student should not, however, lose his sense cf proportion in going over these data. After all, the serious things in tabes are not so numerous. They are his ataxia and loss of ability to walk well, sex- ual and bladder weakness, hypotonia, anaesthesia, crises, especially gastric, arthropathies, tptic atrophy.
The symptoms must now be analyzed more closely.
Locomotor and static ataxia are present very early, but only to a moderate extent. Tests, such as making the patient walk and stand with the eyes closed, noting the position of limbs and the weight of objects, will reveal an ataxia due largely to beginning anaesthesia of the joints and tendons. By the use of the ataxigraph, one can with care assure himself that the pa- tient has an excessive degree of static ataxia. In my experi- ence, when the ataxigraph records over three inches' oscil- lation, the patient not being paraplegic or under the influence of any drug, it is abnormal.
The patella-tendon reflex or knee jerk is abolished very early in all typical cases. This con- stitutes a very important symp- tom, therefore. The ankle- tendon reflex is lost early in the disease, but not, as a rule, so early as the patella and tendon FlG- I25-~ Hypotonia in late tabes dorsalis. reflexes. Inequality or diminu- tion in the activity of these reflexes are significant when taken in connection with other symptoms. An inequality is more important than a general diminution. These reflexes of knee and ankle may disappear early in the disease and return under treatment, but this is rare and later they disappear again.
The gait and station in ataxia are characteristic. In walking, the patient keeps his eyes on the ground and on his feet. The latter he throws out rather forcibly, owing to overaction of the extensors of the foot. In watching such a patient walk barefooted, the extensor tendons can be seen to stand out with each forward movement of the limb. The foot is brought down sharply on the heel and the legs are spread apart a little. Turning, a corner, turning around and going downstairs are done awkwardly, and the patient is apt to totter and fall. Walking on a chalked line is very difficult; so also 20
306
DISEASES OF THE NERVOUS SYSTEM.
is walking backward. The gait improves after the patient walks a while, and he will generally say that the practice of walking does him good. Still, he soon gets tired (Fig. 124).
Hypotonia, or relaxation and lengthening of the tendons and mus- cles, is usually an early and always an important symptom, but is sometimes not very marked until later in the disease. This is what causes the genu incurvatum; when present in high degree it is an un- favorable sign, for it is apt to lead to deformities and promote arthro- pathies. (Fig. 129.)
Fig. 126. — Hypotonia in locomotor ataxia.
Myoclonic and tic-like spasms may affect the face and body.
Myokymia (fibrillary and wave-like contractions of the muscles) is an early and rare complication. It occurs usually in cases with rather rapid onset, and affects most the muscles of the lower limbs. Though not painful it is a very annoying phenomenon.
Severe rectal neuralgia, associated perhaps with hemorrhoids, is sometimes an early symptom. Persistent neuralgia and functional disturbance of the bladder and rectum should cause suspicion of ataxia. Lancinating or lightning pains occur and are very charac- teristic. The pains dart down the legs along the course of the sciatic,
THE DEGENERATIVE DISEASES OF THE SPINAL CORD.
3°7
or they suddenly appear as patches of pain on the foot or leg or thigh ("spot pains"). The pain comes unexpectedly and with such severity that the patient involuntarily jumps or jerks the limb. He speaks of his "jerking" and "twitching pains." The pains may affect the bowels or be felt as a squeezing sensation around the waist (girdle pains). The pains of ataxia are often the most obstinate and distress- ing symptom. They usually come on in great intensity once or twice a month, and last for two or three days. They then leave the patient
Fig. 127. — Trunk and leg anaesthesia in early stage of tabes dorsalis.
for a time. They are often worse in cold and damp weather. In some cases the pains are almost, continuous, coming on, if not every day, at least two or three times a week. Such cases are associated with much cutaneous hyperesthesia, especially during the attacks. This type of cases is called "the neuralgic." The patients rarely have as much ataxia, paresis or visceral troubles as the typical forms present, and in certain respects such cases are favorable. Sometimes the pains are accompanied with erythema or herpes zoster showing an acute involvement of the posterior ganglia.
3o8
DISEASES OF THE NERVOUS SYSTEM.
The pains of the disease continue well into the second and even third stage. Meanwhile the anaesthesia becomes much more marked. It affects most the feet and next the legs, rarely extending much over the thighs, but passing to the fingers and hands. The anaesthesia is greatest to pain, but touch and temperature sense are also involved. There is often delayed conduction and polyaesthesia ; many other curious perversions of the cutaneous sense are noted. Some anaes- thesia usually develops over the finger tips and hands, and some- times a band of anaesthesia develops about the trunk. This trunk anaesthesia may be one of the early symptoms of the disease. The facial and cranial nerves are not much affected, but there may be trigeminal neuralgia. The olfactory nerve is occasionally affected. In early stages of the disease when there is perhaps some specific disorder of the mucous membrane, parosmia occurs, the patient is annoyed with sense of a bad smell. Later in tabes the sense may be lost
Optic atrophy occurs in cbout six per cent, of cases in my experience. Optic atrophy usually develops in the pre-ataxic stage. If a patient has reached the second stage without it, he will probably escape it altogether. Cases with ocular paralyses are slightly more disposed to it (Berger). It attacks the left eye oftener than the right. The atrophy begins sometimes with increased sensibility to light, flashes of light, and muscae volitantes. With the failing vision, disturbance of color sense often and contraction of the visual field always occur. This contraction, is irregular, with sector-formed defects; not hemiopic (Fig. 125). The atrophy progresses slowly with slight remissions. It may cease its progress, but this is rare. Blindness comes in about three years. Ophthalmoscopically, there may be seen slight evidence of congestion in the early stage; later, pallor of the disc, which finally becomes grayish.
The eye muscles are implicated in some way in nearly all cases of tabes. The following are the disorders: (1) Toss of the light reflex and myosis; (2) sympathetic-nerve ptosis; (3) paralysis of branches of the third nerve; (4) Paralysis of the fourth and sixth nerves.
Paralyses of the ocular muscles (third, fourth and sixth) occur rather oftener in distinctly syphilitic cases. Other ocular troubles are not influenced by exudative syphilis. Ocular palsies are early symp- toms of the disease, occurring, as a rule, in the pre-ataxic stage.
1. Loss of light reflex and pupillary rigidity. The pupils are small and sometimes uneven; they do not respond to light, but they do lo accommodation. This condition is known as the Argyll-Robertson rupil. In early stages the light reflex may be simply sluggish. In
THE DEGENERATIVE DISEASES OP THE SPINAL CORD. 309
the late stages the accommodation reflex is also lost. The Argyll- Robertson pupil is practically found only in tabes and in general paresis. The ocular skin reflex usually disappears early. The myosis in tabes is due to paralysis of the sympathetic dilating fibres. The pupils are sometimes irregular in shape.
2. Sympathetic nerve ptosis. A slight drooping of one or both lids is not infrequent. It begins early and progresses slightly up to the later stages of the disease. It is due to paralysis of the sympa- thetic-nerve fibres of the lid.
3, 4. Paralysis of the external eye-muscles. The external rectus is oftenest affected of single muscles, but the various branches ot the third nerve taken together are oftener involved than the sixth. Of the third nerve's branches, the levator palpebral and internal recti muscles are oftenest involved. There may be multiple palsies. These occur oftener in syphilitic cases. Progressive ophthalmoplegia may be associated with tabes. The ocular nerve palsies may be transitory or permanent. Those occurring in the pre-ataxic stage are usually transitory, lasting a few hours, days or weeks. Cases have even lasted two years and got well. The permanent palsies develop usually in the later stages. The early palsies are usually due to a syphilitic exudation at the base of the brain ; the late palsies are usually due to degenerative lesions of the nuclei of the ocular nerves. Violent neuralgic pains sometimes occur in the face, but it is a rare; in fact, a characteristic of tabetic pains is that they hardly ever involve the trigeminus. However, it does occur that a typical tic douloureux may develop. Paralysis of the seventh nerve, though not infrequent in cere- bral syphilis, is not a symptom in tabes. It is not even a premonitory symptom as are the ocular palsies. Spasmodic movements of the lips and face with smacking movements similar to those seen in some forms of spasmodic tic have been noted in two of my patients.
The acoustic nerve is frequently affected, some form or degree of deafness occurring in about one-fourth of the cases ; but in the majority of instances the trouble is an accidental complication due to middle- ear disease. Primary atrophy of the auditory nerve is very rare, as might be expected, since this nerve is structurally not like the optic nerve. Its existence has been inferred on clinical grounds. There is a form of tabetic deafness of trophic origin due to a sclerotic condition of the middle ear (Treitel). The labyrinthine nerve is affected but rarely. Vertigo is not common, yet labyrinthine crises causing seizures like those of Meniere's disease have been observed.
Dysaesthesia of the throat with attacks of repeated swallowing movements form what Oppenheim calls pharyngeal crises.
3IO DISEASES OF THE NERVOUS SYSTEM.
Bulbar paralysis as well as progressive muscular atrophy are rare association diseases and generally appear late* in the course.
More common are disorders of innervation of the larynx. These con- sist of laryngeal crises and paralyses of the laryngeal muscles. The characteristic paralysis of tabes is a "posticus palsy" ; i. e., a paralysis of the posterior crico-thyroid muscles. These are abductors and their paralysis causes difficulty in breathing if both are affected and some- times when one is affected. The paralysis is usually unilateral. The laryngeal palsies are usually early symptoms and usually are not serious. I have known, however, sudden death to occur in a patient subject to attacks of this type of palsy. Anaesthesia of the throat and larynx is very rare.
Laryngeal crises consist of attacks of spasm of the adductors or paralysis of abductors, with noisy, croupy respiration. The attacks come on suddenly, the patient coughs and struggles for breath, and he may be seized with vertigo and fall down. The pulse may be very fast. The paroxysm lasts for a few minutes to several hours. The symptoms are very distressing, but not usually dangerous. Paroxysms of cough have been described as "bronchial crises."
There are also cardiac crises, in which there are dyspnoea and rapid heart beat and sense of suffocation resembling angina. A much more frequent symptom is a rapid pulse which runs about one hun- dred and is small and weak.
The heart itself sometimes is diseased, but whether from neuro- trophic disturbance or not is doubtful. The pulse is often small, rather rapid, and weak.
The most common and distressing of the crises of tabes are the gastric. These sometimes occur in the pre ataxic stage and recur for a long time before the nature of the trouble is recognized. They consist of sudden attacks of intense pain extending from the groin to the epigastrium or encircling the waist, accompanied by vomiting and sometimes diarrhoea. The attacks are often associated with pains in the legs. They last two or three days, then usually pass away, but some cases last two or three weeks and leave the patient much exhausted and often more ataxic. Sometimes instead of or between the crises the patient has a continual diffuse, dead, abdominal pain.
The arthropathies of locomotor ataxia. Degenerative diseases of the joints, technically known as arthropathies, and spontaneous frac- tures of bones form important symptoms of tabes. They occur in ten per cent. (Charcot) or five per cent, (author) of cases.
The arthropathies are three or four times more frequent than the
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 311
fractures. The joints oftenest affected are the knees, ankles and hips; but the elbow, shoulder, wrist, vertebral and pelvic bones and small joints may be attacked.
Spontaneous fractures occur oftenest in the shaft and neck of the femur, next in the legs, forearm, humerus, and clavicle. The pelvis, scapula, vertebrae and under jaw maybe fractured. Arthrcpathies are often accompanied by fractures, especially of the heads of the bones. The two sides of the body are about equally affected.
The arthropathies are char- acterized by a sudden, ap- parently spontaneous painless swelling of the joint. The symptoms may develop in twenty-four or forty-eight hours. In rare cases there is a history of some preceding rheumatic pains or of an injury. After a time there is an osseous hyperplasia of the joint, which becomes enlarged to enormous proportions. There is also a tendency to luxation of the joint. It crepitates on mov- ing. There is no tenderness on pressure; the hand finds evidence of synovial exudation, roughened surfaces, and per- haps fractures of the enlarged parts. In the milder forms there are simply swelling from FlG- 128.— Arthropathy of shoulder, synovial exudation and some
enlargement of the bones with roughened surfaces. After a few weeks this swelling may subside and the joint return to nearly its natural size. In other cases the process progresses, the ligaments relax, the bones of the joint can be moved about freely, and luxa- tions are easily produced. There is still no pain, but the limb becomes almost or entirely useless on account of the loose and re- laxed condition of the parts (Fig. 129). As time goes on, some absorp- tion takes place and the head of the bone may almost disappear. The arthropathies have been divided into benign and malignant, but no sharp line can be drawn or certain prognosis made in the early
312
DISEASES OF THE NERVOUS SYSTEM.
stage. The arthropathies appear in the prodromal and early stage of the disease in over half the eases, and are often at first unrecog- nized. One-third occur after the tenth year cf the disease.
The spontaneous fractures are usually brought on by a slight trauma, such as a fall. Violent muscular movements may produce
L_' ... ' . . j
Fig. 129. — Arthropathy of knees
them. They also are painless, as a rule. The fractures usually heal well, often with abnormal readiness, but occasionally there is delay, and often healing is accompanied by great throwing out of callus.
Pathologically, the arthropathy is a rarefying osteitis. It does not differ anatomically from arthritis deformans, except that frac- tures may accompany it. Clincially, the chief difference lies in the
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 313
J
abruptness, spontaneity and painlessness of the process. The dis- ease, on the whole, cannot be considered specifically different from arthritis deformans, modified by the analgesia of the parts. It is due probably to a degenerative change in the nerves supplying the joints and bones. The process may begin in the cartilage, bone or ligaments. Eventually all these parts are involved. There is con- gestion of the synovial membranes with hydrarthrosis, then atrophy and rarefying hypertrophy of the epiphyses, relaxation of the liga- ments, formation of osteophytes and bony stalactites. There may be a rarefying osteitis of the long bones, without much joint involvement at first
Various trophic disturbances of the skin may appear, generally late in the disease. The most common are herpes and lichen. Besides these, bullae, transitory erythema, urticaria, eczema, pemphigus, ecthyma, ulcers, ichthyosis and petechiae have been described; but they are rare and often only accidental complications. A pecu- liar round perforating ulcer sometimes develops on the sole of the foot, often as the result of cutting a corn. In rare cases the nails and teeth fall out. In distinctly syphilitic cases there is usually baldness. The skin of the tabetic patient loses its "tone" due probably to the general mus- cular atony and to weakness of the erec-
tores pilarum. At any rate, the hand has a characteristic flabby feeling which is almost diagnostic of the disease. The face and cutaneous tissue generally show the same characteristic.
A sense of great weariness and heaviness in the limbs, present con- stantly, no matter how much rest is taken, is a characteristic early symptom, and is due to an irritability of the nerves of muscular sensibility.
Muscular atrophies occur sometimes in tabes. They are of three kinds: (i) a true progressive muscular atrophy due to degeneration of trophic and motor cells; (2) localized muscular atrophies due to degenerative atrophy of nerves; (3) a general wasting. Under the first head one finds ophthalmoplegia, bulbar paralysis and spinal amyotrophy; under the second, wasting of certain groups of muscles in the legs or arms.
Fig. 130.
-Perforating ulcer of foot.
314 DISEASES OF THE NERVOUS SYSTEM.
Besides these, there is a generalized atrophy which occurs in the paralytic stage and is due probably to a slight involvement of the anterior horns in the progressive process that affects the cord.
Attacks of hemiplegia in rare instances occur in tabes. They are usually of temporary character and occur early in the disease. They may come on late and are then more likely due to acute softening due to disease of the cerebral vessels of syphilitic origin.
Acute paraplegia comes on occasionally also, and this sometimes almost disappears.
The sexual power may be at first greatly exaggerated; but this is rare, and usually there is progressive weakness and loss of desire. The bladder and sexual functions are rarely entirely lost and rarely equally impaired in the first stage; one may continue good while the other is affected moderately. Usually the sexual function goes first.
Some cerebral symptoms occur in tabes, chiefly in the early stage. They are insomnia, which may be very obstinate, and occasional vertigo. An irritability of temper and tendency to despondency, sometimes noted, cannot be considered unnatural. Apoplectiform and epileptiform attacks are described, but are very rare, and should cause a suspicion of a complication. The disease in very rare cases terminates in general paresis. Usually if there is to be paresis it comes on with the tabes.
Course. — The disease has been termed progressive, but it is not so in a large number of cases. With proper treatment the symptoms can often be kept in control for years. The first stage may last twenty years or more; the second stage five to fifteen years. The total duration of the disease varies enormously, ranging between three and thirty years. A few acute cases have been observed, run- ning a course of less than a year.
Complications. — These are acute myelitis, generally syphilitic; lateral sclerosis, progressive muscular atrophy, hemiplegia from embolism or endarteritis, general paresis and heart disease.
Tabo-paresis. — In a rather increasing number of cases one sees the symptoms of paresis and tabes develop together. In these cases the cerebral symptoms are usually more dominant and are always more serious and important. These symptoms do not differ greatly from the ordinary early symptoms of paresis. The tabetic symptoms are often of less note and consist in loss of knee-jerks, some ataxia, lancinating pains, ocular palsies and vesical and sexual weakness. Taking a group of one hundred cases of tabes I find about ten per cent, are tabo-paretic. In these cases it is the paresis that has to be
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 315
more carefully watched and dealt with. Like tabes, it can be arrested, but not so often as in the pure type.
Pathological Anatomy and Pathology. — The characteristic changes are found in the spinal cord, posterior spinal ganglia and posterior roots, and to a less extent in the peripheral nerves. The spinal cord usually is reduced in size and flattened antero-posteriorly ; the pia mater is thickened somewhat. One can see with the naked eye that the posterior columns of the cord are shrunken and have a grayish appearance.
Under the microscope it can be seen that the white matter of the posterior columns is very seriously affected; the nerve tissue has disappeared, and its place is taken by connective and neuroglia tissue through which a few nerve fibres still run. The walls of the blood- vessels are somewhat thickened, but not remarkably so, nor is there always notable evidence of congestion or excessive vascular irritation.
The part of the posterior column first affected is a vertical streak lying in the middle root zone between the posterior median (columns of Goll) and posterior external columns (columns of Burdach) (Fig. 131). The segments first and most affected are those of the upper lumbar and lower dorsal region. Besides this area the rim zone or column of Tissauer is also early involved. As the disease progresses it extends upward and spreads laterally so that finally all of the posterior column is changed into a dense connective-tissue mass through which only a few nerve-fibres run. The part last and least involved is that lying just posterior to the commissure (anterior root zone of Flechsig, ventral fundamental column) and that lying just mesial of the posterior horns (external part of the middle root zone) (Fig. 132). There is sometimes a degeneration of the antero-lateral ascending tract (Gowers' tract), and very rarely of the cerebellar tract. The pyramidal tracts are involved only in complicated cases. The cells and fibres of the column of Clark are often involved in ad- vanced cases. The gray matter of both the posterior and anterior horns may show some degenerative changes, viz., decrease of the fibre net-work and atrophy of the cellular elements.
The posterior roots are usually involved, the process extending as far as the spinal ganglia, which also show some degeneration (Figs. 134, 135), but the lesion is not strikingly marked here in all cases, and sometimes the spinal ganglia are nearly healthy, although the pos- terior columns are diseased. The anterior roots are normal.
The exact initial point of attack varies, and this accounts for the variation in the symptoms. Cases that begin with decided bladder and genital symptoms probably start low down; cases which go for a
Fig 131. Fig. 132.
Fig. 131. — Locomotor ataxia, showing areas affected in first stage at five different levels. Drawn from specimens in author's possession and from comparative study of over thirty other figures.
Fig. 132. — Locomotor ataxia, second stage.
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 317
good while with only ataxia, loss of knee-jerk, and pains begin higher ; while in the brachial or arm-type cases the process begins in the cer- vical enlargement.
The peripheral nerves are diseased in a large number of the ad- vanced cases. The nerves of the leg are most involved. The process is a degenerative atrophy or neuritis It affects the extremities of the nerves first and slowly extends upward, seldom reaching the large trunks.
Sometimes the disease begins in the optic nerves, but not in the bipolar visual cells of the retina which corresponds to the posterior ganglion cells. The process here is an atrophy beginning at the periphery and extending brainward. The third, fifth and sixth nerves are occasionally involved ; still more rarely the olfactory and auditory.
Fig. 133. — Spinal cord in tabes dorsalis, showing sclerosis of columns of Goll and Bur- dach at level of sixth cervical segment. (Photographed by Dr. C. I. Lambert.)
The vagus nerve and sometimes its nucleus and that of the glosso- pharyngeal are implicated, it may be, rather early in the disease. It is believed that these facts explain many of the laryngeal and visceral crises and the tachycardia.
Pathology. — The pathology of locomotor ataxia cannot be thor- oughly understood without a knowledge of the pathology of syphilis in its relation to the nervous system — a subject which is discussed later. It may be said here, however, that syphilis leads to two sets of changes in the nervous system: one, the earlier, is inflammatory; the other, and later, is degenerative. The inflammatory changes attack the blood-vessels and serous membranes, leading to the deposit of exudates, and these are the characteristics of secondary syphilis when it involves the nerve-centres. The degenerative changes attack the nerve-tissue directly. They follow long after the infection and are the results of the syphilitic poison which has been permeating the system.
DISEASES OF THE NERVOUS SYSTEM.
Degenerative syphilis of the nervous system is not, according to older views, syphilis at all, but rather the effects of it, just as the parched and dying turf is the result of the fire which has swept over it. The disease departs, leaving a trail behind it which sets in motion the processes of decay and death of the parts. Syphilis often invades the nerve-centres before true tabes sets in and frankly shows itself in the form of inflammatory and gummatous exudates, but often it does not betray its presence and does its final work quietly through years of apparent health. All the time, however, its poison is at work instituting a tendency to death and degeneration in certain parts of the nerve-centres. The parts which are usually first selected are the posterior spinal roots and the neuraxons which pass from the
Fig. 134. — Spinal cord in tabes dorsalis, showing lesion of posterior columns at level of fifth lumbar segment. (Photographed by Dr. C. I. Lambert.)
nerve-cells of the ganglia into the posterior roots and columns of the spinal cord. Locomotor ataxia, therefore, is not primarily a sclerosis of the posterior spinal columns, but of the peripheral sensory neurons. It is true that the posterior spinal ganglia are not always so seriously diseased as the posterior columns of the cord. This, however, is be- cause the severity of the disease is first shown in the neuraxons and their collaterals, just as in alcoholic neuritis the peripheral parts of the motor neuraxons are most and earliest affected by alcohol. As the disease extends, it involves both the peripheral and central parts of these sensory neurons ; that is to say, both the sensory fibres and the posterior spinal roots. Still later it attacks other portions of the nerve-centres, so that in the last stages much of the nervous sys- tem is diseased. The reason why the peripheral sensory neurons are especially affected rather than other parts seems to me to be
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 319
this: the syphilitic poison is brought to the nerve-centres in the blood, whence it passes into the lymphatic sheaths of the blood-vessels or is thrown out upon the serous membranes in the subdural sacs. In the attempts of nature to get it out of the spinal canal and eliminate it, the poison is carried along the serous sheaths which surround the cerebrospinal nerve roots. The nerves, as they pass out from the spinal cord, are covered with three membranes: the dura mater, the
Fig. 135. Fig. 136.
Fig. 135. — Posterior spinal ganglion in third stage of tabes. P. R., posterior root; A. R., anterior root. (Oppenheim.) Fig. 136. — Healthy spinal ganglion.
arachnoid and the pia mater. The dura and the arachnoid surround them less tightly than the other. The dura becomes fused with the connective tissue supporting the nerves as they pass out, and the arachnoid becomes fused with the epineurium and perineurium. Now, fluid injected into either the subdural or subarachnoid spaces passes readily along the nerves for some distance (Macewen), and syphilitic exudate in the subarachnoid and subdural spaces of the spinal cord will thus have a tendency to infiltrate along these sheaths, but as it passes out of the vertebral canal or cranial cavity it meets
320
DISEASES OF THE NERVOUS SYSTEM.
mechanical obstacles, owing to the constriction of the parts, and there is therefore a certain damming up or accumulation of the poisonous material at these points of exit. Generally at this point it meets with the posterior spinal ganglion a highly organized structure with special vascular supply, and it therefore naturally deposits its poison upon this part, which furnishes much more opportunity for mischief than the non-vascular anterior roots. It is a fact that many of the initial symptoms of locomotor ataxia are thus connected with such troubles as would occur from an exudate trying to get out along the course of the spinal or cranial nerves. A frequent initial symptom, for exam- ple, is palsy of one of the third nerves or of the sixth or seventh nerves, due to exudates clinging around their roots. Still more fre- quently the initial symptom is a neuralgic pain in the course of the sciatic plexus, due to the effects of this poison upon the ganglia. Re- cently it has been established that the syphilitic infection is due to an organism known as the spirochceta pallida. These organisms have even been found in tertiary forms of syphilis, and by peculiar reaction of the blood it has been made probable that the spirochceta is alive and acting upon the nervous system, even in degenerative or para- syphilitic stage. If this view is established then the degenerations of tabes are really only a later form of a constantly acting infection and all the phenomena, both of syphilis proper and so-called para- syphilis are only different stages of the same pathological process. Other writers have lately claimed that there are syphilitic anti-bodies in the blood, and that it is these which cause the lesions.
I assume in this description that it is a syphilitic poison which is always at work as a cause of tabes dorsalis. This , however, is not necessarily the case, for it may be the infection of other diseases, and the results of other poisons, such as ergot, pellagra, lead, etc., are to be explained in the same way. There is an inadequacy of the lymphatic and venous systems thoroughly to rid the spinal canal of the poisons that lie in it; the body of the cord is cleared, but the roots do not get rid of the poison. In a word, I would say that locomotor ataxia is a post-infective degeneration which first attacks the posterior spinal neurons or corresponding cells of the special senses, due to a prolonged poisoning of these parts by the toxins of the infection, whatever this may be.
As to why this process attacks some and not others, it can only be said that certain people are born with defective power of resistance as regards their nerve-centres, and that others induce this defective state by physical and other excesses.
The diagnosis is not difficult in the advanced stages. In the first
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 32 1
stage the disease has to be distinguished from hereditary ataxia, multiple neuritis, chronic myelitis, spinal tumor, spinal syphilis, general paresis and neurasthenia. In hereditary ataxia the age, the history of the disease, and the absence of lightning pains are usually sufficient to distinguish it. Multiple neuritis, in its sensory or pseudo-tabetic form, sometimes resembles closely locomotor ataxia. The differential points are given in the sections devoted to that disease. In myelitis there is more paralysis, generally exaggeration of reflexes, and an absence of disturbance of special senses.
The diagnostic criteria of locomotor ataxia in all cases are the presence of lightning pains, numbness of the feet, loss of knee-jerk, hypotonia arthropathies, ataxia of station and gait, without much loss of muscular power, the presence of the Argyll-Robertson pupil, the history of syphilis and the slow onset of the disease. In addition, examination of the cerbrospinal fluid by lumbar puncture shows the the presence of a mild grade of lymphocytosis. This is true for the early but not always for the late stage of the disease. A lost knee-jerk, lightning pains, lymphocytosis and stiff pupils are usually quite enough to assure a diagnosis.
Prognosis. — In the first stage a small percentage may have the disease stopped and get practically well. In many more the disease is arrested, and in fact tabes dorsalis, if seen early and properly treated, is no longer^a dread disease. After the second stage a cure is im- possible, but great improvement may be secured and the patient made relatively comfortable for years^
In the third stage little can be done except to relieve the symp- toms, but life may be prolonged. In some cases the careful applica- tion of mechanical therapy (the Frenkel exercises) will enable patients to walk to some extent again. Death usually occurs from some intercurrent malady or from kidney disease caused by the bladder trouble. Patients very rarely indeed die from the disease itself and its various "crises."
Treatment. — The treatment of locomotor ataxia is a subject the discussion of which cannot be made dogmatic, for the treatment de- pends very largely upon the patient and the stage and cause of the disease. My experience is that any treatment depends enormously upon one's opportunities of getting the patient in the earliest stages. Supposing this be done, the first thing is to be quite assured that there is no trace of a secondary (or exudative) syphilis underlying the trouble. If some of the symptoms are caused by such exudate, inunctions or injections of mercury, warm baths and iodide of potassium should be given vigorously and persistently.
322
DISEASES OF THE NERVOUS SYSTEM.
The iodide of strontium or sodium is often a grateful change from potash. It is to be given in daily doses of about 60 grains, but this may be increased to 600 grains or 900 grains daily, with good effect. Since it is a fact that at times there are syphilitic exudates along with the true degenerative process in tabes dorsalis, this kind of treatment will occasionally give good results. It is the practice at present to use hypodermatic injection of salicylate of mercury or the biniodide, and there seems some reason to believe that better results are obtained by this method. In the majority cf cases, however, mercury and potash do no good and they may actually do harm by hastening on the downward course of the disease ; hence mercury in particular should be given with great watchfulness, and if improve- ment does not appear within six weeks it should be suspended. It has been observed also that excessive mercurization tends to produce a neurasthenic state most prejudicial to the patient and one which may even lead to a certain amount of neuritis. When the physician has assured himself that any possibility of relief from mercurials or iodide is not to be hoped for, these drugs should be dropped ; if they do benefit the patient, they should be repeated at intervals of three to six months. Along with these first medicinal measures, the physician should prescribe something which is much more important, and that is simply rest. Kvery patient with locomotor ataxia should at once have the importance of rest strongly impressed upon him, and the prescription of sixteen weeks a large part of the time in bed is some- times advisable. Equally good results can be usually obtained, however, by obliging the patient to go through a simple and regular life, involving no walking and no work: Institutional life for three months is of enormous advantage. It is a rule to which I have seen hardly an exception that tabetic patients brought to the hospital improve in a striking way simply from the quiet routine of life there, and despite the thinness of city milk and the uncertain value of hospital eggs.
The drugs which are at this time used to help in the cure are mainly the nitrate of strychnia, the tincture of iron, the preparations of arsenic and phosphorus. I have not been able to convince myself that gold, silver or barium do any special good, although these drugs are all recommended by high authorities. The various preparations of the phosphates, such as glycero-phosphate of lime, the hypophosphite of lime, phosphoric acid, seem to me to be of some benefit. Strychnine occasionally does good in small doses, but unless very carefully increased large doses may lead to disastrous results and it should always be given with caution. A great many other drugs may be given for the relief of
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 323
symptoms. Thus ergot, belladonna, urotropin sometimes help the blad- der. For the pain, phenacetin is the drug which gives the most satisfac- tion excepting opium. It may be combined with bicarbonate of sodium, with codein, or some of the other coal-tar products, such as antipyrin, antifebrin, pyramidon, may be substituted. A teaspoonful of baking- soda internally will sometimes stop the pains. Hoffman's anodyne and cannabis indica may be tried. For the bladder trouble, the fluid extract of buchu in doses of twenty drops, combined with ten drops of the tincture of hyoscyamus, is often helpful. Small doses of strychnine may be combined with this. Small amounts of strychnine can always be given for the sexual weakness, but the dose should rarely be made a large one. For the gastric crises, nothing is so good as a hypoder- matic injection of morphine, and for the severe crises of pain an occa- sional hypodermic of morphine should be given. The locomotor ataxic, however, who becomes addicted to the use of morphine for his pains is indeed in a hopeless condition. In persistent constipation the diet should be light and mainly of liquids, such as milk, malted milk, broths, etc. In persistent diarrhoeal states I have found ichthyol of use. This drug also relieves the pains.
The annoying insomnia is to be treated by fresh air and sea-shore life. If drugs must be used, bromide of lithium with a few grains of chloral, and paraldehyde in not over thirty-drop doses and veronal are the best.
In neuralgia of the rectum and bladder, suppositories containing iodoform and belladonna, or codein, or antipyrin, may be used. Some- times simple gelatin or gluten suppositories act very well. Some of the cases of rectal neuralgia or hyperesthesia are due to insufficient clearing of the lower bowel when a movement occurs, and if the patient washes out the bowel with a pint of warm water after each movement he is very much more comfortable.
There is no diet which has a specific effect upon locomotor ataxia, but the patient should be given those foods which are non-fermenta- tive and digestible. Nitrogenous and fatty foods should be prominent.
Hydrotherapy is of considerable benefit. The most efficient of the single measures is the lukewarm bath at a temperature of about 950 F. for ten or twenty minutes daily. After the bath it is well to have a little cold water poured over the back and then the patient should be diligently rubbed. In most cases a simple lukewarm bath is quite as effective as anything. In others the patient feels better if there is added to it some slight stimulant to the skin — two tablespoon- fuls of pine-needle extract, or a regular pine-needle bath or Nauheim bath may be given. The Charcot douche given in moderate strength
324 DISEASES OF THE NERVOUS SYSTEM.
is helpful in cases that are not advanced or particularly weak. I have some hesitation in recommending any special watering-places or cures. I have had patients return benefited from the Hot Springs of Virginia and other American resorts. In Europe, the baths at Lamalou, France, and at Nauheim, Germany, have some reputation. Hot baths are sometimes injurious, and bathing may be overdone by the ataxic.
Electricity is of use from its general tonic and reflex effects, and perhaps exercises some direct influence on the diseased process. Strong galvanic currents (15 to 30 ma.) should be applied along the spine, through the trunk, and down the legs and arms. The combined galvanic and faradic current is even better, given in the same way. The faradic brush should be applied over the extremi- ties and along the back.
The actual cautery is occasionally efficient in stopping pains. It should be applied to the back as often as twice a month at least and sometimes twice a week. Large dry cups may be applied rapidly and in great number (80 to 100) along the spine and along the course of the sciatic nerves. They make the patient feel better and relieve sometimes the sense of exhaustion. In very painful cases occasional wet cups and leeches are useful. Blisters and various forms of counter-irritant sometimes do good.
Suspension by the neck and arms is helpful in some cases. It is best adapted to persons in the second stage and to those who have a good deal of bladder trouble and pain. It is of little value in the paralytic stage, and must be used with care in the early stage and when patients are large and heavy. Suspensions should be given for from one to three minutes three times a week until twenty-five or thirty are taken. After three months a second course may be given. The treatment of locomotor ataxia by Systematic exercises, known as the Frenkel method, is one that of late has been considerably used. It consists in having the patient go through regular exercises which teach him to co-ordinate the different groups of muscles of the trunk, legs and arms. The Frenkel method is one which can be used with advantage with persons who are passing into the second stage of tabes and in whom the disease is not making progress. It is especially indicated where there is not great hypotonia. It must be used very carefully and for a long time. It often enables the patient to walk better and use his arms better, but it does not especially affect the progress cf the disease.
Finally, it has seemed to me that those sufferers from locomotor ataxia do best who perisistently and courageously fight against their
THE DEGENERATIVE DISEASES OE THE SPINAL CORD. 325
malady. Those who, despite suffering and discomfort, will three or four times a year take some form of treatment, medicinal, hydro- therapeutic or electric, such as will have some beneficial effect upon their general nutrition, and such as will buoy up their hopes and improve their mental condition, are quite sure to be rewarded and after a hard fight emerge into a state of comparative relief from their symptoms and secure a measurable degree of rest from the progress of their disease.
SPASTIC SPINAL PARALYLIS (Lateral Spinal Sclerosis). Little's Disease.
This is (a) a term used to describe a form of paraplegia caused by chronic myelitis, and (b) a congenital disorder, known as Little's disease, in which there is sclerosis of the lateral columns of the cord of congenital origin, (c) A primary degenerative disease of the lateral columns.
The special interest attached to this form of disease, on account of the controversies concerning it, leads me to say a few words about its history. Between the years 1846 and 1877 an English sur- geon, Little, published a number of articles on a disorder which he termed "congenital spastic rigidity of the limbs." In 1873 and again in 1879, Dr. B. C. Seguin, of New York, described a condi- tion which he termed "tetanoid paraplegia." After the first article of Dr. Seguin, to whom priority belongs, Professor Brb and Pro- fessor Charcot independently published articles in the year 1875 upon what Erb called "spasmodic spinal paralysis" and Charcot "spas- modic dorsal tabes."
Starting from the writings of these three authorities, there de- veloped in the course of a few years a description of the disease which became known as "spastic spinal paralysis" or "lateral sclerosis." This for a long time was accepted as an independent malady by most writers. Of late years, however, its real existence has been strenu- ously denied, and the cases supposed to represent this disease were asserted to be either forms of dorsal myelitis, multiple sclerosis or the result of some cerebral defect. Recently both French and Ger- man writers have revived the work of Little, and, having supple- mented his observations with their own, have rehabilitated spastic paralysis into a separate disease again, giving it the name of "Little's disease."* %
^Sometimes a spastic paraplegia develops quickly; after a few weeks the symptoms improve and the patient gets well; this has been called "hypertonic paralysis." There is here, however, simply a slight grade of myelitis or meningomyelitis. and no separate name is required to show what is the matter.
326
DISEASES OF THE NERVOUS SYSTEM.
Etiology. — The new spastic spinal paralysis, or "Little's disease," is an affection which is always of congenital origin and is due, it is supposed, to a lack of development of the pyramidal tracts. This lack of development leads to a sclerosis of the lateral columns of the spinal cord and to symptoms of rigidity of the legs and arms, ex- aggeration of the reflexes, with some real muscular weakness and atrophy. The disease is always of prenatal or natal origin, being due to some developmental defect or, as Tittle supposed, to premature and forced deliveries, with probably some meningeal hemorrhage on the convexity of the brain. It may also be a hereditary family disease. Through these causes the pyramidal tracts cease to grow, or, at least, this process is greatly delayed.
Symptoms. — The malady appears within a short time after birth, usually within a year, but it may be delayed in family types to the fifth year or even later. Some cases of Little's disease may, it is believed, develop as late as after maturity. It is not my purpose to give a description in detail of the symptoms of this trouble, because they are given under the head of cerebral diplegia or birth palsy. The only difference between ordinary cerebral diplegia and the disease under present consideration is that in this latter form there are no marked mental defects; the child is not small headed and idiotic, nor does it have epilepsy or cranial nerve palsy or hydrocephalus. The brain seems to be spared except so far as its motor functions are con- cerned. It is convenient to separate this type of disease from the ordinary spastic cerebral palsies with mental defect, for the reason that the future of these cases is in some instances more hopeful. As they mature, the lateral columns occasionally gain in development and some increase in the strength and control of the limbs is obtained. I base this statement upon the experience of others. In several cases of Little's disease at the age of fifteen to twenty-five which I have seen, there has been no marked improvement. Mentally, however, these patients are often very bright.*
Children with this trouble on trying to walk are obliged to cross one leg in front of the other as they are helped along, giving them a characteristic "cross-legged" progression. The arms are less affected than the legs. The facial and throat muscles may be slightly involved. There is no pain. In some cases the disability increases as the child grows older, owing to the greater size and clumsiness of the patient. The arms become much stiffened and contractured, and
*It is due to American neurology to say that Dr. Seguin as long ago as 1879 said: "It is possible that tetanoid paraplegia in young children may be due to deficient cerebral development and consequently agenesis of certain tracts of the cord."
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 327
the hands are flexed so that the patient can neither walk nor help him self. Epilepsy and mental deterioration also may develop at the time of puberty or adolescence.
Prognosis. — The mild cases that learn how to walk and can use the arms and hands may grow up, slowly improving, and reach a good age and a fair degree of health. The severer cases rarely reach adolescence, but grow gradually more helpless and generally succumb to some intercurrent disease before they are twenty.
Diagnosis. — The disease is distinguished from ordinary cerebral diplegia (birth palsy) due to brain lesion by the ab- sence of epilepsy, mental defects and microcephalus.
From compression myelitis, the involvement of the arms, and the absence of pain and dis- turbance of sphincters are dis- tinctive. Hereditary spastic paraplegia runs in families, be- gins at the fourth or fifth year, and involves tchiefly the legs.
Treatment. — This is altogether one of mechanics and attention to nutrition. The limbs must be persistently massed; tenoto- mies should be performed so as to straighten the legs; constant voluntary effort to use the Fig. 137.— Little's disease with contractures. Stiffened muscles should be (Fournier and Tourette.)
made. Braces, roller crutches,
etc., should be used. Patience is often greatly rewarded in this disease.
Hereditary Spastic Spinal Paralysis. — Spastic paralysis in very rare cases is found to run in families, affecting different members of many succeeding generations. In the cases described, it begins at about the age of five, affects only or mainly the legs, runs a very slow course, is not accompanied by pain, ataxia or visceral symptoms; and runs a course lasting twenty or thirty years. Dr. Bayley, of Philadelphia, has described a family of typical cases. Drs. Leo New- mark and Wm. G. Spiller have also described cases.
328
DISEASES OF THE NERVOUS SYSTEM.
THE COMBINED SCLEROSES.
By the combined scleroses is meant those forms of degenerative sclerosis in whicn both the posterior and lateral columns are involved.
There are several diseases in which combined sclerosis exists. They are :
1. Combined scleroses of profoundly anaemic and toxic states (Putnam's type),
2. Hereditary spinal ataxia (Friedreich's ataxia).
3. Combined scleroses complicating general paresis.
4. Accidental forms (Gower's ataxic paraplegia).
There are many cases reported in literature of combined scleroses, but the clinical pictures vary very greatly. These cases are probably in the most part forms of chronic myelitis or multiple sclerosis with ascending and descending degeneration. Marie has shown that the vascular supply of the spinal cord is such as rather to favor the de- velopment by extension of sclerosis in the lateral and posterior columns from a chronic leptomeningitis, and his suggestion that many of these cases are perhaps of syphilitic origin accords with my experience and conviction. Some years ago, Gowers described a disease that he called ataxic paraplegia, the lesion in which, he believed, lay in the lateral and posterior columns. Most of the cases which belong to this clinical description I think can be properly classed either with the cases of locomotor ataxia, of multiple sclerosis or of some form of chronic myelitis. It seems to me, therefore, inadvisable to encumber our neu- rology with a description of this disease.
1. The Combined Scleroses of Pernicious Anemia and Cachectic States (Putnam's Type).
This form of disease, nGt so rare as has been supposed, was first described by Dr. J. J. Putnam, later by myself ; and in quite recent times has been expanded and placed in relation with pernicious anaemia by a number of observers.
Etiology.— The disease occurs oftener in men, according to statis- tics, but my clinical experience is against this. Nine out of my twelve cases were women. The age of most cases is between 50 and 65 (16 out of 23), and next to this between 40 and 50 (5). The youngest case was 36 (Rothmann's). There is often some family or personal neuro- pathic history. I have reported three cases occurring in one family.
The disease follows acute infections like influenza, prolonged diar- rhoeal or dysenteric attacks, possibly shock, lead poisoning, malaria, lathyrism and pellagra. One patient had a malignant tumor.
r
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 329
Syphilis is not a cause. The relationship to profound secondary and to pernicious anaemia is very important. In about 10 per cent, of cases, and perhaps more, pernicious anaemia undoubtedly exists; in others there is usually a very marked anaemic and cachectic state. Often, if not always, however, nervous symptoms develop before evidences of extreme or extended anaemia are present.
Symptoms. — The initial nervous symptom is nearly always a per- sistent paraesthesia usually of the feet, associated with some weak- ness of the part. Following this there is a good deal of ataxia, and this symptom increases with the loss of motor power. Tactile, thermic and pain anaesthesia are not marked until late in the disease, but the patient often has severe pains in the back and limbs. There may be some differentiation of sensibility as in syringomyelia. With the weakness of the legs there is at first an increase of knee-jerks, and sometimes ankle clonus and rigidity, so that the patient shows the symptoms of spastic and ataxic paraplegia. Later the spasticity may become less and the knee-jerks disappear.
The arms are involved after a few months, the symptoms begin- ning with paraesthesia, weakness, awkwardness and a very slight anaesthesia. The disease may begin in the arms, as in one of my cases. The mind may be slightly weakened, the memory becoming defective and the patient emotional. The cranial nerves are not often involved, though optic atrophy has been once noted. The blood presents evidences of anaemia. Usually this is secondary, but in about ten per cent, of cases there are the typical findings of pernicious anaemia.
The disease progresses rather rapidly, though hopeful remissions occur. In from six months to a year the process has nearly reached its height. During this time the patient emaciates, marked anaemia or pernicious anaemia sets in. The skin is sallow and pale, and pig- mentation is seen. Diarrhoea occurs at times. The bladder becomes weak and retention of urine follows: the control of the rectum is eventually lost, and the patient lies bed-ridden with paralysis and contractures of the lower limbs. The arms do not become so badly paralyzed.
Prognosis. — Death usually occurs in about two years in the progressive cases. It may be prolonged to three years, and it is not unlikely that some patients can be relieved or the process checked if seen in time.
Pathology. — The disease is a toxic one, but whether due to the failure of some glandular tissue of the body to act or to infection or poison is not known. As the disease belongs to the degenerative
33°
DISEASES OF THE NERVOUS SYSTEM.
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period of life, it may fairly be supposed that in some cases the blood- making organs undergo premature decay and that the nerve cen- tres are poisoned in consequence ; or the intestinal tract may be the origin of the poison since in some cases there is evidence of great atrophy of the mucous membranes.
As to the location of the trouble, the posterior columns of the spinal cord are the parts first and most involved. The process especially involves the postero-median parts. It appears to start in certain foci in the lower dorsal or cervical cord, and to spread thence. New foci apparently develop as the disease progresses; the lateral columns are also severely affected, and espe- cially the crossed pyramidal tracts. The other columns are involved, but less regularly and completely. .so/Waj Xhe lower dorsal and lower cervical levels of the cord are especially selected. The anterior horns are finally involved, but the change here comes later. Finally, there may be actual softenings in the cord, producing cavities. The blood-vessels are somewhat af- fected with hyaline degeneration, but I am unable to say how im- portant a factor this is. The walls are thickened, there are in places dilatations and hemorrhages, and in other places collapsed vessels. There is no evidence of inflamma- tory reaction. The meninges are not involved, nor are the anterior and posterior roots much affected. Examinations of the peripheral nerves (Rothmann) and of the brain have been negative.
Diagnosis. — The disease is easily distinguished from locomotor ataxia by the absence of syphilitic history, the rapid onset, the ansemia, the motor weakness, the absence of ocular symptoms and of lightning pains. There is usually also at first an exaggeration of the reflexes. The steady and rapid progress of the malady is also most characteristic.
Fig. 138. — Combined sclerosis of toxic origin, showing location of degeneration of original foci of disease.
I
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 33 1
Multiple neuritis is excluded by the slow onset, the marked ataxia, the absence of muscular wasting and of tenderness and pain, and the development of bladder and rectal symptoms.
One of my cases presented such a marked differentiation of cuta- neous sensations that I believed it to be one of syringomyelia. The necropsy showed a cavity in the cervical cord due to softening, and in a measure explained, if it did not justify, the diagnosis. The steady and rapid progress of the symptoms ought to exclude such a diagnosis.
The existence of marked anaemia, and especially of pernicious anaemia, is, taken with ataxia, paralysis and rapid course, pathog- nomonic.
The essential nature of this process is a primary nerve degener- ation, affecting apparently the nerve-tissue first. The same poison
Fig. 139. — The spinal cord in combined sclerosis from pernicious anaemia, dorsal region. The colu'mns of Goll, crossed pyramidal tracts, and cerebellar tracts are affected.
which causes pernicious anaemia does the work here, only it may affect the nerovus centres without causing a true essential anaemia. It seems even as if in some cases the neural change came first.
Treatment. — The use of arsenic sometimes is helpful and brings on remissions in those cases associated with anaemia. Intestinal antiseptics and colon washings, fresh air and tonic medication are at times helpful.
Hereditary Spinae Ataxia (Friedreich's Ataxia).
There are four forms of hereditary or congenital spinal and cere- bellar defects which should be grouped together: They are: (i) Hereditary spinal ataxia, or Friedreich's ataxia; (2) hereditary cerebellar ataxia; (3) hereditary ataxic paraplegia ; and (4) heredi- tary spastic paraplegia. They are quite similar in cause and mode of development. The difference in symptoms depends upon the fact
332
DISEASES OF THE NERVOUS SYSTEM.
that the defect develops in the one case mainly in the posterior and lateral columns of the cord, in the second in the cerebellum, and in the fourth mainly in the lateral columns.
Friedreich's ataxia, the most common of all the forms, is a chronic degenerative disease mainly affecting the posterior and lat- eral columns of the cord.
Clinically, the disease is characterized by ataxia beginning in the lower limbs and gradually involving the upper limbs and the organs of speech. Curvature of the spine, talipes, vertigo and finally paraly- sis and contractures appear. The knee-jerk is, as a rule, absent. There is but little pain or anaesthesia and optic atrophy and visceral troubles are usually absent.
Etiology. — The fundamental factor in predisposition is an inher- ited or connate lack of development of the spinal cord, more particu- larly of the posterior columns and pyramidal tracts. This condition is inherited directly sometimes, but indirectly as a rule ; that is to say, the parents or other members of the family usually show simply a neurotic history, and it is in only a minority of cases that there is a history of ataxia in the direct line of ancestry.
The more frequent condition is this: the parents or grand- parents have some neuroses, such as insanity, inebriety or great nervous irritability; then the ataxia occur in the children of the next generation. Sometimes in a single family the uncles and nephews or cousins may be found to have the disease. Hence the name "family ataxia," used by some writers. There are a good many cases in which the parents were apparently perfectly sound and healthy. The parents rarely have locomotor ataxia, though this has been observed in a few cases.
Syphilis in the ascendants is an element in some, probably in most cases. Habitual intemperance in parents is perhaps a factor some- times ; much more rarely consanguinity and tuberculosis act as predis- posing causes of degeneration.
More cases have been observed in America than in any other country; while the fewest have been reported from France.
Race. — The disease develops at about the time of puberty, most cases occurring between the ages of six and fifteen years. It is not very rare, however, for symptoms to develop even in infancy, though some of the cases reported at this time were probably of a syphi- litic character.
Age. — In a given family the disease, as a rule, strikes the older members first, but the younger members are attacked at a relatively earlier age. The most typical time of development is a rather late
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 333
one; i.e., after twelve years of age. The disease may come on after maturity. In American cases the age of development of the disease has been rather earlier than the average.
Sex. — The male sex slightly predominates, its proportion being about sixty per cent. In America the female sex has, however, been more affected (3 to 2).
The patients, as a rule are the children of the laboring and agri- cultural classes. They have been found in the country oftener than in crowded cities. The families have often been large, but this is not always the case, especially in American cases. Nursing at the mother's breast is thought to have been an exciting cause. Usually the disease appears after infectious fevers, such as diphtheria, variola and typhoid.
Symptoms. — The child is first noticed to have an uncertainty in the gait and some feebleness in the lower limbs. These symptoms gradually increase until they interfere seriously with progression, and force him in two or three years to leave off* active play. With this there may be some slight pains or numbness in the lower limbs, and an examination will show, within a year or earlier, that the knee-jerk is gone. After five or six years the arms become af- fected with inco ordination, and a little later bulbar symptoms, such as thick or scanning speech, and often nystagmus, appear, very much delayed. During this time the patient suffers little pain or anaesthesia and has no trouble with the bladder or rec- tum. Vertigo and headache are often present. Dorsal flexion of the toes, talipes varus or some other form of clubfoot, and lateral curvature of the spine are often observed (Fig. 140). Oscillation of the head and choreiform or inco-ordinate movements of the extremities may develop. As the disease progresses the legs become weaker, and finally paraplegia, with contractures and muscular wasting, sets in. The intelligence is sometimes diminished. The disease makes slow progress; often it remains almost at a standstill for years, and
Fig. 140. — Friedreich's ataxia show: deformities of feet.
late
These bulbar symptoms may be
334
DISEASES OF THE NERVOUS SYSTEM.
the patients usually die of some intercurrent disease, such as phthisis or an infectious fever.
Among the rarely observed symptoms are tremor of the hands and head, spasms, sluggish pupils, tachycardia, profuse sweats, impotence, slight vesical incontinence, fragilitas ossium. Many of these symp- toms occur only late in the disease. When there is marked involve- ment of the cranial nerves, the disease may be put down as a cere- bellar-spinal trouble.
The major and essential symptoms are: (i) ataxia, beginning in the lower limbs and extending to the arms and tongue; peculiar rolling, ataxic gait; (2) disturbances of speech; (3) talipes and spinal curva- tures; (4) gradual development of paraplegia; (5) loss of knee-jerk; (6) absence of cutaneous anaesthesia, of bladder troubles, of eye troubles except nystagmus, and of severe pains; (7) the development of the foregoing at about the time of puberty.
Diagnosis. — -The disease must be distinguished from multiple scle- rosis of spinal type, and from cerebellar ataxia and tumors. The family history is usually of help. The disease begins earlier in life than does multiple sclerosis. The speech disturbances, nystagmus, paresthesias, pallor of the optic discs, intention tremor, and exagger- ated reflexes also serve to distinguish multiple sclerosis. The differ- entiation from hereditary cerebellar ataxia is not important as the two diseases are really the same. The general symptoms of brain tumor are not present in hereditary ataxia.
Pathology. — The lesions of importance are found in the spinal cord and medulla only. The cord is usually small, flattened and apparently congenitally imperfect in development. In some cases two central canals have been seen. A sclerosis exists throughout the whole length of the posterior and lateral columns, sometimes extending to the anterior columns (Fig. 141). The sclerosis is most marked in the postero-median columns, which are eventually affected in toto. The postero-external column is less involved and there is often a narrow strip of healthy tissue between the posterior horn and the sclerosed area, also between the posterior gray commissure and the diseased parts. The posterior-column sclerosis is usually most marked in the lumbar region. In the lateral columns the sclerosis always affects most the crossed pyramidal tracts. The direct cerebellar tracts and the so-called ascending antero-lateral tract are diseased in some cases, but apparently not in all. In a few instances the anterior median columns are involved. A zone of healthy tissue is often found between the sclerosed pyramidal tracts and the posterior horn. There are no important changes in the gray matter except a secondary
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 335
Fig. 141. — Spinal cord in a long-standing case of hereditary ataxia, cervical, dorsal, and sacral regions. Besides the sclerosis, the cord is filled with small vacuoles which are dilated perivascular spaces, these are artefacts.
336
DISEASES OF THE NERVOUS SYSTEM.
of extension of the sclerosis, but the involvement of the cells of the hypoglossal nucleus is probably the most significant change. The brain exhibits no changes of importance in relation to the symptomatology of the disease. The posterior nerve-roots are ex- tensively sclerosed, the anterior roots less so, and the peripheral nerves show some degenerative changes. The peripheral nerves are much less involved than in tabes dorsalis. It is asserted that the sclerosis in the cord is really a neuroglia proliferation — a gliosis — and there is no doubt a large amount of neuroglia proliferation in the diseased areas.
Course and Prognosis. — The disease is a progressive one, though it may be stationary for a long time and may even show temporary improvement. The longest period of duration of the disease on record is forty-six years and the shortest two years, the average being fifteen or twenty years. Death occurs from some intercurrent disorder or from bulbar complications.
Treatment. — A quiet life, good food and favorable hygienic sur- roundings are the main therapeutic helps. Arsenic and various nerve tonics may be of temporary benefit. A course of mercurial injections or inunctions is advisable in the early stages. My cases and some of the French cases were benefited by suspension by the neck in a Sayre apparatus. If the disease appears in one member of a family, effort should be made to prevent its appearance in others. The infant should not be nursed by its mother; special care should be taken to prevent its getting any infectious fevers and to prevent it from receiving any falls or blows. Its life should be exception- ally quiet, so far as physical exertion goes.
Hereditary Spastic Paraplegia is an extreme1 y rare malady resembling in general course the ataxic types, and probably not much differentiated from it.
4. Hereditary Cerebellar Ataxia.
Though not belonging in the group of spinal diseases, hereditary cerebellar ataxia is so closely related to those hereditary forms I have just been describing that I deem it best to give an account of it here.
It is a chronic disease beginning in early life, usually of hereditary or congenital origin, and characterized by an ataxia of cerebellar type associated with symptoms indicating the involvement of some of the cranial nerves. In course and symptoms it resembles to some extent hereditary spinal ataxia and belongs to the same class of
THE DEGENERATIVE DISEASES OF THE SPINAL CORD. 337
diseases. The names of Fraser, Nonne and Marie are connected with the first descriptions of this disorder and its differentiation from hereditary spinal ataxia.
Etiology. — -The malady begins in early life J>etween the ages of ten and thirty, developing, therefore, somewhat later than Fried- reich's ataxia. The disease occurs rather more often in males than in females, but, like other family diseases, is usually transmitted by the female. Syphilis and a neuropathic constitution in the family have been noted in a number of cases.
Symptoms.- — -The first symptom is shown in a disturbance of the gait. This is indicated by clumsiness and stumbling and by a ten- dency to rolling and pitching like a drunken man. The patient tends to walk with the feet wide apart. He does not, however, show the "Romberg symptom" ; that is, he can stand fairly well with the feet together and the eyes shut. There are inco-ordination and jerkiness in the movements of the arms and sometimes choreic move- ments. He sometimes also has oscillation and jerky movements of the head. When lying down the inco-ordination is very much less- ened. The speech is hesitating, ataxic and explosive. The eyes show jerky movements somewhat like those of nystagmus. An important symptom in many cases is the development of optic neuritis followed but not always by optic atrophy and blindness. The knee-jerks are usually exaggerated. The patient has no anaesthesia and suffers little pain, although he may have some headache. There is no disturb- ance of the sphincters. Mentally, the patients are usually some- what deficient, becoming eventually either simply childish or actually demented. The malady progresses, although not always steadily, the disease sometimes remaining stationary for some years. Even- tually, however, the patient becomes bed-ridden and dies of exhaustion.
Pathological Anatomy. — The few autopsies which have been made show an atrophy of the cerebellum. In some cases this is macro- scopic, the cerebellum being reduced to one-half or one-third its size. In other cases there is no naked-eye change, but there is found microscopically an atrophy of the cortical matter.
Diagnosis. — Hereditary cerebellar ataxia is a disease which has a close kinship with hereditary spinal ataxia. In fact, it is not always possible, at least, in the early stages, to distinguish between them. It seems that the same family or hereditary taint strikes sometimes upon the cerebellum, leading to a slow progressive atrophy of that organ, and sometimes upon the spinal cord, while in some cases both organs may be affected. In the cerebellar type, the distinguishing features are the presence of the knee-jerks, the absence or late occur- 22
DISEASES OF THE NERVOUS SYSTEM.
rence only of a spastic or ataxic paralysis, the presence at times of speech defects, of nystagmus and jerky tremors, and the occurrence at times of an optic atrophy. In the spinal form we see the early and more prominent paraplegic symptoms develop, with deformities of the feet, while there is a late involvement only of the cranial nerves. Usually the knee-jerks disappear, and the gait shows evidence of a spinal type of ataxia.
Treatment. — The disease is a progressive one and little can be done but to attend to the general nutrition of the patient. In one case, however, seen by me with Dr. Stillwell, it was found that the con- tinuous use of. antipyrin was accompanied by an amelioration of symptoms and seemed really to help the patient a great deal.
CHAPTER XV.
THE PROGRESSIVE MUSCULAR ATROPHIES AND MUSCULAR DYSTROPHIES.
The anterior cornual cells of the spinal cord, the motor nerves and their terminal end organs, the muscles, form a trophic unit, and the same degenerative disease may attack either end or any part of this physiological mechanism. There is a clinical and pathological unity in all the different spinal and muscular types of atrophies. But there are sufficient differences, also, to oblige us for convenience sake to make certain classifications. Thus those disorders which attack chiefly and first the anterior horn cells are called progressive muscular atrophies; those disorders attacking first the muscle tissue and its nerves are called progressive muscular dystrophies. The progress- ive muscular atrophies of central origin may attack the motor- nerve cells of the eye, of the throat and lips, of the upper or lower spinal cord. In accordance with the level affected, the disease has received different names. Sometimes the pyramidal tracts of the spinal cord are* first and most involved and then the peripheral motor neuron is affected. This has furnished excuse for another type. Then, again, while most cases of muscular atrophy are acquired, there is one type of it which is an hereditary one. Thus we find the disease classified as follows :
The progressive muscular dystrophies have also been much subdi- vided, but they are essentially the same disease, as will be seen later.
Of the above progressive atrophies, I have already described ophthalmoplegia.
Progressive Muscular Atrophy (Progressive Spinal Amyotrophy; Duchenne-Aran's Disease).
This is a disease characterized by a slow, progressive atrophy of the muscles of the extremities and trunk, with consequent paralysis, not
Progressive muscular atrophies of spinal or nervous origin.
i Progressive muscular atrophy, j (Including hereditary types.) I Amyotrophic lateral sclerosis. [ Remittent atrophy.
339
34Q
DISEASES OF THE NERVOUS SYSTEM.
accompanied by any notable sensory disturbance, and due to a pro- gressive atrophy of the motor and trophic cells in the spinal cord. In the more typical and common form (Duchenne-Aran's) the disease begins in the upper extremity.
Etiology. — -The disease affects persons in the middle period of life (twenty-five to forty-five). The extremes are fourteen and seventy years. It is more frequent in males. Heredity is rarely, if ever, a factor. Great mental strain, exposure, traumatism, excessive use of
I. 2.
Fig. 142. — Showing: 1. Segment of spinal cord with anterior-horn cell, end brush; and lateral tracts, the parts affected in progressive muscular atrophies, and 2, the muscle and its nerves, the parts affected in progressive muscular dystrophies.
certain groups of muscles, acute infectious diseases — especially typhoid ; childbirth, acute rheumatism, syphilis and lead poisoning are causes. It may complicate locomotor ataxia. The causes, as will be seen, are much the same as those of bulbar paralysis.*
* Among 72 cases, four-fifths were in males. Syphilis was present in about one-fifth of the cases. With regard to age, I have seen two cases beginning as early as the twelfth year, of the Duchenne-Aran variety, in the most characteristic form. In one case, which I have now watched from the time it began, at the twelfth year, to the present time, the patient now being 24, the disease was arrested, after about four years of progress, the patient being left with simply an atrophy of the left forearm, in the muscles of the ulnar distribution. My oldest patient was one of a perfectly typical arm type of atrophy seen with Dr. J. Arthur Booth, occurring in a physician, aged 72, but beginning probably, before his seventieth year. The period during which the greatest number of cases were seen was that between the 30th and 40th years and next, between that of 40 and 50. In cases of bulbar paralysis the age of 53 seems to be a singularly serious year. I note the occurrence of six cases at about that period of time, among 12 in all. There were about five times as many cases among males as among females, but this does not apply to all types of the disease. In the bulbar cases there were more women, the proportion being 8 to 4.
Among the exciting causes, by far the most frequent is that of some form of strenuous occupation. Workmen who had to do very heavy work, athletes or professional contor- tionists were among the victims, and in the cases of bulbar paralysis, there was several times a history of the patients being very great and excessive talkers. Lead, as a cause of paralysis, does not seem to me, after all, as important a factor as has been supposed. It could be blamed for the disease in not over five cases, and not certainly, in all of these. Several of my patients were very heavy users of tobacco, but I fancy this was only a co- incidence. Two patients had in infancy a previous attack of poliomyelitis. In one case there was a very distinct history of an acute infection by dengue; it is not improbable that other cases have followed an acute infection. On the whole the dominant causes are a occupation strain, infections or para-infections, poison and some inherited weakness of the affected neurons.
\
MUSCULAR ATROPHIES AND DYSTROPHIES. 341
Symptoms. — The patient suffers at first from slight rheumatoid pains in the shoulder or arm, associated with some feelings of numbness and weariness. Muscular wasting then begins to appear, and usually in one hand. The adductor longus pollicis is very early affected, also the thenar muscles and the interossei. The atrophy spreads from muscle to muscle, and does not follow the distribution of nerves, al- though the ulnar-nerve supply is most seriously disordered. The ball of the thumb becomes flattened, and the patient cannot abduct or flex it well. When the radial interossei are reached the forefinger cannot be abducted, and this is often an early sign. The disease gradually extends upward, attacking the flexors and extensors of the forearm, then the upper arm and shoulder. Mean- while the hand has become thin and flattened, flexion of wrist and exten- sion of fingers are lost, and a charac- teristic "griffin-claw" appearance re- sults. After a time (three% to nine months) the other arm begins to be affected. Occasionally there is a temporary remission.
In a few cases the atrophy begins first in the shoulders and arms, attacking the deltoid, biceps and triceps, then extending downward to the hands. This constitutes the "upper-arm type." Fig. 143.— Showing wasting of hands in
If, as is usually the case, the muscular atrophy,
disease continues to progress, it
passes from the shoulder girdle to the deep muscles of the back, then downward, involving successively the hip and thigh muscles, the glutei, the crural extensors and abductors being oftenest chosen. The leg muscles may be finally involved, but they usually escape. The disease as it descends continues its progress in the trunk, in- volving the intercostals. It slowly ascends the neck also, and finally leads to paralysis of the diaphragm, or a bulbar palsy may set in.
It will be seen that the ordinary course of the disease is from the lower-arm muscle groups (ulnar and median) up to the shoulder group (middle cervical nerves), then down through the dorsal and lumbar nerves, rarely reaching the sacral groups. In very rare cases it begins in the legs and ascends.
342
DISEASES OF THE NERVOUS SYSTEM.
Along with the wasting there are a corresponding weakness and paralysis, but the paralysis is the result of the atrophy and does not precede it. Fibrillary twitchings of the muscles occur; the idiopathic muscular contraction caused by striking it a blow is very marked; myoid tumors are easily brought out. In some cases the muscles are flaccid and toneless, and the deep reflexes, knee-jerk, and arm-jerk disappear early (atonic atrophy), but in most cases the irritability and tonicity of the muscles are increased, the knee-jerks exaggerated, and we have tonic atrophy. This condition may be so marked as to make it resemble a special clinical type of progressive atrophy known as amyotrophic lateral sclerosis.
The electrical irritability of the muscles gradually lessens to both gal- vanic and faradic currents, but no marked qualitative changes occur at first. Eventually we may get partial degeneration reactions, but these occur late in the disease, unless this runs a very rapid course, when fairly typical degeneration reactions may be got.
In typical cases of progressive muscular atrophy there is no anaes- thesia ; and when such symptoms develop the presence of peripheral disease or of syringomyelia or spinal, tumor must be suspected. The patients may suffer from rheumatic-like pains and from paresthesias.
The affected parts often show excessive sweating and congestion and evidence of vasomotor disturbance. This may involve the face on one or both sides; one pupil may be larger than the other, due to irritation of the cilio-spinal centre. The iris reflex, however, is pre- served, and the optic nerve is never involved.
The sexual power is often weakened, but the sphincters are not attacked. The urine shows variations in the amount of urea. There is usually an increase of lime salts.
Complications. — The most common complication is an extension of the process to the medulla, causing disturbance of speech and swal- lowing. Muscular atrophy complicates locomotor ataxia, but is rarely complicated by it. A high degree of spasm and rigidity of the legs, particularly, may occur, causing the condition known as amyotrophic lateral sclerosis.
Course and Duration. — The disease usually progresses steadily until it has reached an advanced stage, when it may stop. Remissions may occur early, however, and even some improvement take place; the disease then ordinarily progresses again. It lasts from two years to thirty or more, but on the average not over ten or twelve years. Death usually occurs from pulmonary disease, owing to the weakness of the respiratory muscles. Sometimes the extension to the medulla
MUSCULAR ATROPHIES AND DYSTROPHIES.
343
and involvement of the muscles of deglutition and of the larynx are the cause of death.
Pathology. — The primary anatomical change is a degenerative atrophy of the cells of the central parts and anterior horns of the gray matter of the spinal cord. The atrophy gradually extends and in- volves the whole anterior horn. It also extends vertically, first down, then up. Along with this atrophy are degenerative changes in the lateral columns; consecutive to this there is atrophy of the anterior roots, peripheral nerves and the muscles. The disease begins in the deeper parts of the anterior cornua, involving the central and median groups of cells. These are more concerned in nutrition and in the finer muscular movements of the extremities. Hence atrophy always precedes, or at least keeps pace with paralysis. The levels affected are the lower cervical and upper dorsal ; but if the disease is extensive the dorsal, lumbar and sacral cord are also involved. The affected part is nearly free from nerve-cells, and those present are atrophied, their processes are short or absent, and the cell has lost its angular ap- pearance. Sclerotic and pigmentary changes are observed. The neu- roglia and connective-tissue cells are increased in number, but there are no marked changes in the blood-vessels, though these may be much dilated. There is often some degeneration of the lateral columns, and this may be very complete. It is confined chiefly to the pyram- idal tracts, but extends somewhat anteriorly into the mixed lateral column. It does not affect the cerebellar or ascending lateral tracts. The degeneration has been traced up into the brain as far as the in- ternal capsule and even to the cortex. The anterior columns may be slightly affected. The posterior horns, columns and roots are normal.
The affected muscles show various degrees of degeneration. They are pale and streaked with yellow, due to fatty deposits. Some fibres may be simply narrow and shrunken ; others have lost their striation and become granular from deposit of fat globules or degenerated muscle elements ; other fibres have lost their striations and appear as if filled with a homogeneous, glassy-looking substance containing a few fat granules (vitreous degeneration) ; others show a longitudinal striation. The interstitial connective tissue is increased and in places has taken the place entirely of the muscles. The capillaries and small vessels are distended. Healthy fibres may be seen among the diseased. Changes have been found in the sympathetic nervous system, but they are unimportant.
The diagnosis has to be made from the progressive muscular dys- trophies, chronic poliomyelitis anterior, syringomyelia, neuritis and neuritic family atrophy.
344
DISEASES OF THE NERVOUS SYSTEM.
In the muscular dystrophies there is commonly a history of heredity; the disease begins usually in childhood or adolescence. It attacks the lower limbs oftener ; it is slower in progress and the two sides are affected at the same time ; there are no fibrillary contractions, and the degeneration reaction does not occur.
Remittent spinal atrophy begins rather suddenly and, having reached its height, does not progress, but remains stationary or improves for a time. The paralysis occurs first, the wasting follows. It affects groups of muscles physiologically related, while progressive muscular atrophy attacks muscles only anatomically related. It be- comes arrested, but another attack may occur. There are cases, however, which seem to be on the border line between the two diseases.
Syringomyelia is distinguished by the presence of peculiar sensory and trophic disorders.
Neuritis caused by lead poisoning is detected by the history of the case, its tendency to affect the extensors of the arm chiefly, and the absence of a progressive tendency. Sometimes, however, lead poison- ing and palsy end in true progressive muscular atrophy.
Ordinary multiple neuritis is distinguished easily by its rapid onset ■ and the presence of sensory symptoms.
The hereditary or "leg type" of progressive muscular atrophy is characterized by its attacking first and mainly the legs and forearms, by the presence of a good deal of sensory disturbance, of typical de- generation reactions, and of a hereditary or family history.
Treatment. — The patient should be well fed and have rest, quiet and fresh air. Careful local faradization and galvanization of the spine and affected parts are indicated. Massage does no good. Hypodermatic injections of strychnine in the affected member, gr. to 3V daily, the internal use of arsenic, phosphorus, iron, quinine and cod-liver oil sometimes are beneficial.
In cases with a syphilitic history, mercury and iodide of potassium have proved useful and this treatment should be carefully tested. The essentials of treatment are rest, electricity, strychnine locally, the administration of powerful tonics, and overfeeding. Animal extracts, nitroglycerin, morphine, atropin, nitrate of silver, chloride of gold and of barium, and the nitrate of uranium are recommended but do no good.
Hereditary Muscular Atrophy of Peroneal Type (Charcot-Marie Type).
This is a hereditary or family muscular atrophy of central (and neuritic) origin, attacking the' legs and later the forearms. It
MUSCULAR ATROPHIES AND DYSTROPHIES.
345
affects males more than females, but the difference is not great. It almost always begins before the age of twenty. It attacks first the muscles of the leg, not the foot, involving the peronei, then the extensors of the toes, then the calf muscles. The thighs escape till later. After some time the forearms and small hand muscles are reached. The shoulders and arm, neck and trunk muscles escape. There are occasionally fibrillary contractions ; and always partial or complete degenerative elec- trical reactions. The patients complain of some pain and 'numbness, but there is no anaesthesia.
The characteristic, then, of this form of atrophy is that it (affects the legs, rarely going above the knee, and producing a rather symmetrical atrophy !of these limbs, so that in the later stages the legs look very spindle-like, and there is no pseudo-hypertrophy at any [period in the progress. In some cases the hands and fore- iarms are attacked in a similar way, producing a symmetrical and progressive atrophy of the parts, and rarely involving the shoulder girdle or arms. So 'that, in the complete stage of
development of the disease, the jrIG I44._Peroneal type of atrophy. (Marie.) legs and feet, the hands and
forearms are especially, if not solely, involved. Still, the disease does not always affect the forearms and hands, at least, not until very late. Gowers has observed a case in which with this charac- teristic involvement of the forearms, there was also an involvement of the face, in a fashion somewhat similar to that seen in the facio- scapulohumeral type of dystrophy.
The disease runs a long course, with remissions, and resembles i in prognosis the dystrophies. The outlook is better than in the arm type, but the disease is not curable.
Some authorities assert that the disease is due to a progressive degenerative neuritis. In the writer's opinion, the anterior horns of
346 DISEASES OF THE NERVOUS SYSTEM,
the spinal cord are primarily attacked,* a view recently confirmed by Marinesco. It is therefore a disease of the peripheral motor neurons.
The treatment is the same as for the other forms of hereditary mus- cular atrophy.
Fig. 145. — Remitting muscular atrophy. Patient is unable to raise the head.
Chronic Anterior Poliomyelitis (Remitting Spinal Atrophy).
This is a disease which goes under the name of chronic poliomyelitis, but it is rare and in most cases shows by its final outcome a closer relation to the spinal atrophies than to a simple inflammatory process.
Etiology. — In my own experience syphilis has always been present;
*The writer has seen the disease in atypical form in one member of the first generation, in two members of the second. A child of one of the latter had, at the age of two years, a typical attack of anterior poliomyelitis.
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others give as causes lead, overwork, exposure and trauma. It occurs only in adults and in men more than women.
Symptoms. — The disease comes on rather rapidly; that is to say, in one or two months. It reaches its height in three or four months and before the end of a year has come to a standstill. The symp- toms then remain quiescent or improve for two or three years, then take a fresh start and present about the same course, involving new and usually neighboring groups of cells. It may progress no further after this second attack, but there sometimes occur fresh invasions involving finally the bulbar nuclei and leading to a fatal issue. The symptoms of paralysis or weakness precede the atrophy, which is a flaccid one, but in other respects it resembles the ordinary spinal atrophies. There are slight or no symptoms of involvement of the pyramidal tracts. The disease usually affects the upper extremities and especially the shoulder girdle, but eventually involves the forearms and ascends to the cervical region.
This disease may be called a chronic anterior poliomyelitis, though there is a good deal of variation in the records of the reported cases. The tendency to cessation of progress and even to improvement has characterized cases reported by Kisenlohr, and Landouzy and Dejerine. We may therefore say that chronic poliomyelitis is a form of spinal atrophy characterized as above by early weakness followed by atrophy and running a relatively rapid course with a tendency to remission or' improvement. Few cases with autopsy have been re- ported, and these showed vascular lesions with secondary degeneration of the anterior horn cells and sometimes of the anterior fundamental column.
Treatment. — The history of syphilis in my cases leads to the recommendation of an anti-syphilitic treatment for this trouble.
Glosso-Labio-Laryngeal Paralysis (Progressive Bulbar
Paralysis).
This is a disease characterized by progressive wasting and paralysis of the muscles of the tongue, lips, palate and throat, due to an atrophy of the nuclei of the nerves supplying those parts.
Etiology. — It is a disease of the degenerative period of life, most cases occurring after forty and between that time and seventy.
The disease begins later in life than spinal atrophy. It occurs rather oftener in men than women.* A neurotic heredity is some-
*While this is the usual statement, in my experience women suffer much oftener than men.
348
DISEASES OF THE NERVOUS SYSTEM.
times noted. Exposure to cold and excessive use of the muscles in talking, mental strain, debilitating influences, lead and syphilis are causal factors.
The element of syphilis in producing both this type of atrophy and the Duchenne-Aran type, has been studied by the writer, and it has been found that in over twenty per cent, there is a history of infection, the disease appearing somewhat like a form of parasyphilis. It has occurred, for example, in two cases as a terminal symptom in locomotor ataxia.
Symptoms. — The tongue is the part first affected. The patient speaks indistinctly and cannot articulate the lingual consonants /, r, n and t. The tongue cannot be elevated and is protruded only a little distance. It looks scarred and wiinkled. The lips become weak and the patient cannot whistle nor make the consonants p, b, m, or the vowel o. The saliva begins to dribble from the mouth. Disturbance in swallowing soon develops. Hard solids are taken with difficulty, next fluids, while semisolids are generally managed best. The lips finally become so paralyzed that the mouth cannot be shut, and the lower part of the face is motionless and expression- less. The upper face wears an expression of anxiety and suffering, the saliva dribbles constantly, and the whole physiognomy of the patient becomes characteristic and pitiful in the extreme. The facial nerve may get somewhat involved. Articulation becomes almost entirely lost; the voice has a nasal twang from paralysis of the palate.
The patient has tired and uncomfortable sensations of dryness and stiffness about the throat. There is no pain or anaesthesia, but occasionally there is impairment of the sense of taste. The throat reflex is usually lost, so that tickling it causes no reaction.
Electric irritability is at first unchanged, but in the later stages partial degeneration reaction occurs. In rare cases there is a rapid pulse and still more rarely glycosuria.
The laryngeal reflex becomes weak, the adductors also, but ab- ductor paralysis is rare.
The mind is not affected, but there are often an emotional weak- ness and tendency to tears — not at all unreasonable in view of the distressing nature of the malady.
The disease is often the terminal stage of spinal muscular atrophy ; it may be associated with the latter, with amyotrophic lateral sclerosis, or with ophthalmoplegia. All these types may occur together.
It runs a progressive course, with remissions of a few weeks or months. It lasts from two to three or four years.
The termination is eventually fatal. Death occurs through inter-
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ference with swallowing, and inanition or a broncho-pneumonia or bronchitis may develop which ends the patient's life.
Pathology. — The primary lesion is found in the nuclei of origin of the hypoglossal, glosso-pharyngeal, vagus and spinal accessory nerves. The raphe fibres and the anterior pyramids are also usually somewhat involved. There is sometimes atrophy of the cells of the facial nerve and of the nucleus ambiguus, which is the motor nucleus of the vagus. The brunt of the disease falls, therefore, upon those more superficial or posterior nuclei which are representative of a con- tinuation of the anterior cornual cells. If the disease is complicated
with pigment, X2-3. To the right are four cells in different stages of degeneration, xi-6.
with amyotrophic lateral sclerosis, or progressive muscular atrophy, or ophthalmoplegia, we find atrophy in the cord or ocular nuclei. The atrophic process is similar to that observed in the spinal disease.
The muscles of the tongue, and to a less extent the orbicularis oris and the throat muscles, show evidences of degeneration and atrophy. In some cases the tongue is not shrivelled, owing to the presence of a fatty deposit, and on account of this the disease has been divided into atrophic and paralytic types, but this distinction is unnecessary.
Diagnosis. — The disease must be distinguished from polioenceph- alitis inferior, bulbar apoplexy, tumors, and softening, from multiple
350 DISEASES OF THE NERVOUS SYSTEM.
sclerosis, and from chronic lesions of the cerebral hemispheres caus- ing pseudo-bulbar paralysis. It must also be distinguished from asthenic bulbar palsy. The slow onset, the progressive course, the bilateral character, the absence of involvement of sensory nerves, and the degenerative reactions are sufficient for a diagnosis. In asthenic bulbar palsy there is great paralysis, but none of the typical atrophy of the parts. It is important always to note whether there are ophthalmoplegia and spinal muscular atrophy associated with the disease.
Treatment. — The patient should be kept quiet; he must be over- fed and given massage and electricity in moderation. The same drug treatment as in the spinal disease is indicated. Small doses of mor- phine, gr. to and of atropin may be given also. It is advised in these cases, where there is any suspicion of syphilis, to give a course of hypodermatic injections of mercury. While, personally, I have not seen any benefit result from this course, there have been some cases reported in which it has given relief (Lere). Massage to the neck and face gives temporary relief. Careful voluntary exercises of the weak parts are helpful. Electricity should be tried for a short time twice or even thrice daily, if possible. The faradic current may be used, alternating or combined with the galvanic. Galvanization of the neck and medulla appears to do no good. After a time it may be necessary to feed with a tube or even to do tracheotomy.
Myasthenia Gravis (Asthenic Bulbar Paralysis and Asthenic Bulbospinal Paralysis).
These names are given to a chronic and progressive disorder characterized by the symptoms of progressive bulbar paralysis or by the symptoms of this disease and of progressive muscular atrophy, the distinguishing features being that there is no muscular atrophy, that the cases often continue on for many years instead of going on progressively to a fatal issue, and also by the fact that on autopsy no easily distinguishable microscopical changes are found.
Etiology. — Little is known as to the cause of the disease. The majority of cases have been under the age of thirty, but a patient of my own was over fifty years of age and another over forty. It is some- times associated with anaemia and also with intestinal toxaemia. Re- cently it has been found that these are lymphoid deposits in the muscles, and in some cases the thymus is present and diseased. Those causes which are found in progressive bulbar and spinal paralysis, viz., overwork, mental strain, are sometimes found here.
MUSCULAR ATROPHIES AND DYSTROPHIES.
351
Symptoms. — The disease usually begins gradually and oftenest affects first the muscles of the throat and face and of the eyes. The patient notices first a general feeling of unnatural weakness, and gets more easily tired at his work. Then he finds that sometimes he sees double, or one of his lids droops and it is difficult to keep the eye open, then he has to give up chewing solid food because it tires the jaws. Speech soon tires him; the voice maybe nasal; and he has to swallow with some care. The arms get easily weak and they tire at their accustomed tasks. Digestion is slow and constipation may occur. With all this there is little or no pain, no emaciation or local atrophy ; no spasm or twitching. On stimulating the muscles with an electric current the responses get weaker and finally cease. After a rest they return (myasthenic, electrical reaction). The same phenomenon occurs on tapping the tendons of affected muscles. The symptoms are characterized by remissions ; after a patient has reached a point at which he is almost moribund, he begins to get stronger again and may slowly get into a state of comparative strength ; then the symptoms slowly return. In this way the disease may continue for a number of years. The patient usually dies of exhaustion, but he some- times recovers.
Pathological Anatomy. — In the half-dozen early autopsies no lesion of the nervous system was found, except microscopical changes in the cells of the motor nuclei. Later lymphoid-cellular deposits in the muscles and a persistent or diseased thymus have been found. Most careful examination of the blood, urine and intestinal contents have failed to throw light on the disease, which is due, probably, to a toxaemia of intestinal or blood-gland origin.
Diagnosis. — The clinical characteristics which distinguish this disease from progressive muscular atrophy and true bulbar palsy, are the absence of any true atrophy of the muscles of the face or tongue or extremities, the absence of fibrillary twitchings, the presence of the myasthenic reaction and the irregular course with remissions. Tike these other diseases, however, asthenic paralysis is not accompanied by any disturbance of sensibility or any impairment of the sphincters.
The treatment consists in complete rest, careful attention to feed- ing, and the use of iron and arsenic, and, possibly, of quinine. Strych- nine and muscular stimulants should be given with great care. Far- adism is not advisable, but the use of a stable galvanic current is reported to have done good. The theory that vthe disease is due to some toxaemia has led to the careful attention to diet, to irrigation of the colon, and to general disinfection of the intestinal tract, and I have seen excellent results obtained by this procedure.
352
DISEASES OF THE NERVOUS SYSTEM.
Amyotrophic Lateral Sclerosis. (Spastic Form of Progressive Muscular Atrophy.)
This disease is one which has the closest possible kinship to pro- gressive muscular atrophy which we have just described. There has been an enormous amount of discussion as to whether a distinct place should be given to the disorder. The question is a very academic one, for in everything that really constitutes a special malady it is essentially the same. However, its clinical symptoms are somewhat different, and anatomically there is a somewhat more extensive and peculiar change.
Amyotrophic lateral sclerosis, or Charcot's disease, is characterized by progressive paralysis with atrophy, rigidity and contractures of the limbs.
Etiology.— It is a rarer disease than progressive muscular atrophy, and occurs most often between the ages of thirty and fifty, involving the second part of adult life. Rare cases, however, have been re- ported as occurring in childhood. According to Marie, the female sex is rather more often affected. According to the same author, no definite exciting cause is known. It is not due to syphilis or lead poisoning, nor does it follow the acute infectious diseases. It is therefore considered a disease of involution; i. e., teratological defect, the first and second motor neurons degenerating because of inherently deficient vitality. This state of affairs, however, underlies the other atrophies also.
Symptoms. — Spinal Onset. — In its spinal and more common form there is first noticed a weakness and clumsiness of one hand and this may be associated with some subjective sensation like a numbness. No atrophy is seen but some twitchings of the muscles, and irregular jerky tremor of the fingers and hand occur. Soon the other hand and then the legs are involved, so that in a few months walking tires the patient, going upstairs is difficult, the feet tend to drop because the anterior tibials are weak. Cramps are noticed in the leg muscles, and the same paraesthesise are felt as in the hands and arms. In less acute cases the lower limbs are not involved for a long time; i. e., for one or two years. Examination after the disease has developed shows some slight atrophy of the intrinsic hand muscles and perhaps of the forearms. The fibrillary twitchings are present and sometimes are extremely marked affecting the shoulder and trunk muscles as well as the arms. The reflexes are much exaggerated, especially those of the lower extremities, there is ankle clonus and the dorsal flexion
MUSCULAR ATROPHIES AND DYSTROPHIES.
353
of the great toe (Babinski's sign) is present. The wrist, elbow and perhaps the jaw-jerk are also very active.
The disease usually progresses steadily, until the upper extremities become almost useless and the lower extremities nearly as much so. Atrophy has now set in, of the same type as that of progressive mus- cular atrophy. No special sensory symptoms except sense of weari- ness and aching are to be noted and there is no involvement of the sphincters. Finally, in about one and one half to two years the medulla becomes affected, and there is weakness, stiffness, cramp and atrophy of the tongue ; the lips are less mobile and speech and swal- lowing are affected, the symptoms are the same as in bulbar palsy except that there is evidence of a rigidity and a tendency to cramp and contracture, and the atrophy comes later.
Bulbar Onset. — Sometimes the disease begins in the medulla. There is then, on the whole, a more rapid progress, a later atrophy and a slighter tendency to remission than in the other myelopathic atrophies. The patient first notices some difficulty in speaking or swal- lowing. He feels at times a spasmodic drawing of the tongue or stiff- ness of the cheek or lips. Soon after there appear a weakness and stiffness of the legs and arms. The symptoms progress rather slowly. The speech becomes disturbed ; swallowing is difficult ; the arms atrophy and become stiff and rigid, producing characteristic deformi- ties. There is great exaggeration of the reflexes ; the legs show the presence of ankle clonus; all the arm reflexes are increased, and the jaw is stiff and has a very lively jerk when struck. The patient suffers little from pain. There are no anaesthesia and no sphincter trouble, except in the last stages of the disease.
Pathological Anatomy. — Post-mortem examinations show a very marked sclerosis involving the direct and crcssed pyramidal tracts; also some of the short-fibre systems of the lateral column. The anterior cornual cells are atrophied, as in progressive muscular atrophy. Lesions are also seen at times in the columns of Goll. In fact, the post-mortem findings resemble entirely those cf progress- ive muscular atrophy, except that there is a sharper accentuation of the disease in the lateral tracts. In the medulla the nuclei of the hypoglossal and other motor cranial nerves will be found diseased and the pyramidal tracts also. The lesion of the white columns dimin- ishes in intensity from below up, so that as one gets into the cerebral peduncles very little if any is to be seen. In a few cases, however, the process has been traced to the motor cortex and changes even in that part have been discovered. In a case of my own, which was very closely studied and repcrted upon by Dr. Joseph Collins, the
354
DISEASES OF THE NERVOUS SYSTEM.
sclerosis of the motor tracts did not reach above the medulla, and there was no lesion of any moment in the cortical motor cells.
In amyotrophic lateral sclerosis the degenerative process attacks first the terminal fibres and collaterals of the cortico-spinal motor neurons. The next part attacked is the anterior cornual cell. We have, therefore, the curious and perplexing phenomenon of a disease which attacks the cell body of one neuron and the terminal neuraxon of another neuron just above it. It is difficult to explain this upon the ordinary lines of nerve-cell pathology. Still, we have analogies, perhaps, both in locomotor ataxia and in multiple neuritis.
The diagnosis of amyotrophic lateral sclerosis must be made from transverse myelitis, multiple sclerosis, and the other forms of progress- ive muscular atrophy. The diagnosis is based upon the very striking and progressive atrophy associated with exaggerated reflexes, rigidity and contractures, and without any sensory symptoms or sphincter troubles. . The diagnosis from ordinary bulbar palsy depends upon the appearance of stiffness, cramps, exaggerated reflexes and rigidity displayed by the muscular supply of the facial, the trigeminal and the glossopharyngeal, and the tenth, eleventh and twelfth cranial nerves.
The prognosis is invariably bad, but in those types beginning in the legs and arms life may be prolonged a number of years.
The treatment is the same as that for progressive muscular atrophy.
THE PROGRESSIVE MUSCULAR DYSTROPHIES.
As I have already stated, there are various forms of progressive muscular atrophy to which the special name of "dystrophy" is given, because they are hereditary in character and because the muscular end of the motor neuron is apparently the first and the most severely attacked. Recent and closer study of the pathology of muscular dystrophy tends to show that the lesion is not in the muscle and ter- minal of the motor nerves alone, but that the peripheral motor neuron is also to some extent affected. The clinical characteristics of the muscular dystrophies, however, are pretty distinct and are sufficient to justify the separation of them into a different class.
A number of types has been described, the distinctions being based chiefly on the part of the body first affected. These types are not of great importance, but may be enumerated here for convenience. (Fig. 147):
1. Pseudo-muscular hypertrophy. Allied to it is
(a) Leyden-Mobius or hereditary type, appearing in children,
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355
beginning in the back and lower limbs. The congenital type of Hoffman is allied to this.
2. Erb's juvenile type, or scapulo-humeral type, beginning in childhood or youth, usually in the shoulder-girdle and trunk.
3. Landouzy-Dejerine type, or infantile progressive muscular atrophv of Duchenne, or facio-scapulo-humeral type. It resembles the preceding form, with the exception that it involves also the face.
Fig. 147. — Showing the parts first attacked in the different types of muscular dys- trophy and muscular atrophy. The shaded parts in A show the place of onset of pro- gressive muscular atrophy of ordinary or Duchenne-Aran type, and of types 2 and 3 in text. B shows place of onset of types 1 and (a) in text.
4. The peroneal or leg type has been classed with the dystrophies, but is probably of spino-neural origin, and has been described with the atrophies (see page 344).
The essential unity of all these different forms is shown by the fact that cases occur in which pseudo-hypertrophy takes place in the scapulo-humeral and other types, by the fact that a disease resem- bling pseudo-hypertrophic paralysis occurs without any hypertrophy, and by the fact that different types occur in the same family.
The dystrophies of the different types have many characteristics
356
DISEASES OF THE NERVOUS SYSTEM.
in common. They are, in a considerable proportion, of hereditary origin or, at least, run in families. Among twenty-nine cases ob- served by myself, there was a distinct family history in 12 per cent. They affect boys much oftener than girls, in the proportion of about one to five. In my own cases, there were twenty-four males and five females. The great majority occur under the age of twenty, and a majority under the age of ten years. Thus, among my cases, there were under five years, eleven ; under ten years, seventeen ; and under twenty years, there were twenty-two. They may develop, however, as late as the fortieth year.
The so-called "peroneal type," develops, on the whole, rather late, while pseudo-muscular hypertrophy and the allied hereditary type begin very early, often in infancy, and some children even have never known what it was to walk.
The distribution of the types probably differs somewhat in dif- ferent countries and races. In this country, in the Montefiore Home, where the patients are mainly Hebrews, the pseudo-muscular hypertrophy and the hereditary type of Teyden-Mobius is, by all odds, the most common. In the personal cases, seen by myself, both in private practice and in my clinic, pseudo-muscular hypertrophy is also most frequent. The shoulder-arm type of Erb, comes next in frequency, while the facio-scapulo-humeral type is, in my experience, extremely rare, there being only one in thirty cases. The peroneal type is not common. Among the native Americans the dystrophies often develop later in life, take a longer and less serious course, and sometimes become arrested. In fact, the common type among the native classes, which I have seen, is one in which the hip, back and shoulder-girdle become gradually affected rather symmetrically, so that the patient becomes greatly emaciated, but is still able to walk some and use the forearms, the face and threat not being affected. This form of dystrophy begins usually below and ascends, being first a hip-girdle and trunk affection, then a shoulder-girdle affection, and is, therefore, a sort of combination of the hereditary (Teyden- Mobius) and of the so-called juvenile type (Erb).
All the cases which have cc me under my observation in New York could be grouped, then, in the following way:
1. The typical cases of pseudo-muscular hypertrophy, beginning in the leg, but soon involving the shoulder and hip-girdle.
2. The root type, beginning in the hips usually and back mus- cles, and extending to the shoulder and trunk muscles, not necessarily extending to the legs and arms, at least, not until very late in the disease, and accompanied often with some pseudohypertrophy.
MUSCULAR ATROPHIES AND DYSTROPHIES.
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3. The peroneal type which, in its pure form, is extremely rare> but of which irregular types are not so uncommon.
4. The facio-scapulo-humeral type, which is the rarest of all. Among my cases there were of the first type, eleven; of the second
type, twelve; of the third type, four; and of the fifth type, one.
Pseudo-muscular Hypertrophy (Atrophia Musculorum Lipomatosa).
This is a disease beginning in childhood and characterized by a symmetrical, progressive atrophy of muscles, associated with an apparent muscular hypertrophy due to a deposit of fat in the wast- ing muscles.
Etiology. — The disease attacks boys much oftener than girls. It begins, in the vast majority of cases, under the age of ten, often at the close of infancy, very rarely not till after puberty. Heredity is a very important factor (in three-fifths of the cases), the hereditary influence being almost always transmitted by the mother. A psychopathic or neuropathic condition is often found in the ancestry. Syphilis, intemperance, consanguinity, are not factors in hereditary causation. Injury and an acute disease some- times appear to act as exciting causes.
Symptoms. — The first symptom noticed is a weakness in the legs, which shows itself in a peculiar "waddling gait" and a tendency to stumble and fall. A little later (fifth or sixth year) an apparent hypertrophy of the leg mus- cles, particularly of those of the calves, develops. "™ ~ "
Fig. 148.— Pseudo-mus-
I he extensors 01 the knee or one 01 them and cuiar hypertrophy. (Erb.) the gluteal and lumbar muscles may also be
affected. Sometimes the hypertrophy is very great, at other times it is barely noticeable. The affected part has a peculiar, hard, non- elastic feeling to the hand, not like that of normal muscle. In the upper part of the body the hypertrophy oftenest attacks the infra- spinatus, the supraspinatus or the deltoid may be somewhat involved (Fig. 149). The lower parts of the pectoralis major and latissimus dorsi are also usually atrophied, giving a characteristic appearance to the shoulders. The upper-arm muscles usually become wasted, the forearm, neck, and face rarely. The tongue may be hypertrophied.
358
DISEASES OF THE NERVOUS SYSTEM.
Along with the pseudo-hypertrophy there occurs an atrophy of certain groups of muscles'; and after a time the pseudo-hypertrophy disappears and an atrophy takes its place. In the lower limbs the muscles most atrophied are the flexors cf the hips, then the extensors of the knee and those of the hip. The calf muscles fail before the
anterior tibial. The atrophy and consequent weakness of the lower-limb muscles cause great difficulty in going up- stairs, the gait becomes more waddling, and the patient loses the power of getting up when lying on the floor. These peculiarities are due chiefly to the weakness in the extensors of the knees, the extensors of the hip, the flexors of the hip and the erector spinas. By reason of the same defects, the child when standing has an antero-posterior curvature of the spine with the concavity backward (lordosis) (Fig. 148). This is due to the weakness of the extensors of the hips, which, acting from the hips, are un- able to tilt the pelvis back. On sitting, this lordosis disap- pears and is replaced often by a curve in the opposite direc- tion due to weakness of the
Fig. 149. — Pseudo-muscular hypertrophy .
involving legs and shoulders. erectors of the spine. There
may be some lateral curvature
also. In consequence of the weakness and contractures of the leg
muscles, there early develops a talipes equinus, and later the legs may
become flexed cn the hips and the forearms on the arms.
The muscles show no fibrillary twitching and rarely any de- generative reactions, but there is sometimes a peculiar tetanic con- traction with both the faradic and the galvanic current. A myotonia occasionally develops on the basis of a dystrophy.
The knee-jerks and elbow-jerks gradually weaken and in time are lost. ;. ;
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There is no pain or other disturbance of sensibility.
The affected parts feel cold and look reddened, as if from deficient vasomotor innervation. The organic spinal centres are not involved. Intelligence is usually good.
The Leyden-Mobius type is mainly differentiated by its beginning in the back and hip-girdle, thence affecting the legs. (Fig. 150.)
Course. — The disease runs a chronic but variable course. Its prog- ress is at first slow; after walking becomes impossible it may cease to progress. It lasts from ten to twenty- five years. In a few cases patients have reached the age of fifty or sixty years, even when the disease began in youth. The earlier the disease begins the more rapidly it extends ; the more pronounced the tendency to lipomatosis, the more rapid is the course.
Pathological Anatomy. — The disease, like other forms cf dystrophy, is a de- generative atrophy, the process affecting first the muscle fibres and nerve termi- nals and the connective tissue being secondarily involved. In a simple atrophy of muscles, such as follows disuse, the muscle fibres simply grow smaller and gradually break up and disappear. In degenerative atrophy, the process is accompanied by evidences of irritation, such as swelling of the muscle fibre, proliferation of muscle nuclei, splitting cf the fibre longitudi- nally, and connective tissue proliferation. All these phenomena are seen in the pathological process which takes place in the dystrophic muscles. All the varied changes may be noted in the same muscle. In the early stages there is a true hypertrophy c f some of the fibres, a condition thought to be characteristic of the muscular dystrophies in distinction from the spinal atrophies. Besides swelling and hyper- trophy of fibres, one sees atrophy cf the fibres; the bundles are rounded; there are increase of muscle nuclei, splitting cf fibres, vacuolization, and a tendency to break up into fibrillar (Erb). The connective tissue at first shows evidence of irritation and proliferation. Finally, as the muscular atrophy progresses, connective tissue increases and takes its place, until a dense, hard myosclerosis results (Fig. 151).
Fig. 150. — Leyden-Moebius type of dystrophy. (Scboenborn and Krieger.)
3<5°
DISEASES OF THE NERVOUS SYSTEM.
In some parts there is deposit of fat in the connective-tissue cells, and this may increase until an extensive lipomatosis exists. In the later stage of the disease the fat deposits are absorbed and there are only atrophied muscle and connective tissue. The nerves and spinal
Fig. 151. — Cross-section of crural muscle in dystrophy, showing hypertrophied and atrophied fibres, increase of nuclei. (Cestan and Lejonne.)
Fig. 152. — Longitudinal section of supinator longus in dystrophy showing atrophic fibres and fat. (Cestan and Lejonne.)
cord are usually normal; when changes are found they are secondary to the muscular disease.
The process is then, first, hypertrophy of muscle fibre and in- crease of muscle nuclei, swelling and rounding of fibres, and splitting
MUSCULAR ATROPHIES AND DYSTROPHIES.
36l
of the same; then increase of connective tissue, with corresponding atrophy of muscle and deposit of fat.
The process is a primary degeneration due to an inherent nutri- tional weakness of the muscle. In a measure it is true that those mus- cles embryologically latest developed are earliest attacked.
The JUVENILE DYSTROPHY OP
Erb, or scapulo-humeral form of dystrophy (root dystrophy) begins in childhood or early youth, a little later than pseudo-hypertrophy. The shoulder-girdle is first affected, later the arm. The forearm and legs are attacked very late. Part of the pectorals, part of the trapezii, latissimus dorsi, rhomboid, upper- arm muscles and supinators are af- fected, while the supra and infra- spinati and fc rearm and hand usually escape. There may be true and false muscular hypertrophy. There are no fibrillary contractions or degener- ative reactions (Fig. 153).
As the disease progresses it gradually affects the hip-girdle and trunk, and finally the patients have an almost general atrophy. This is then the root and trunk dystrophy, which I find the common one in this country.
The PACIO-SCAPULO-HUMER A L
form, or infantile progressive mus- cular atrophy, begins in early childhood (third or fourth year) usually, but may develop late. The atrophy attacks first the face, giving a characteristic appearance
known as the "myopathic face." There is a weakness of the oral muscle, which causes the lips to protrude and produces a symptom called the "tapir mouth." The atrophy respects the eye muscles as well as those of mastication and deglutition. It extends to the
Fig. 153. — Juvenile type of progress- ive muscular dystrophy; sixth year of
362
DISEASES OF THE NERVOUS SYSTEM.
shoulders and arms next, then it pursues the ordinary course of the dystrophies.
Prognosis. — The patient never recovers, but the disease some- times comes to a standstill and there may even be some improvement, especially in cases beginning late.
Treatment. — The prophylaxis is important. It consists in pre- venting the marriage of women belonging to dystrophic families; if a dystrophy has developed in one child, it would be unwise to take the risk of bringing others into the world. Or if children are already born, they should receive the most careful nourishment, outdoor life should be secured, and the dangers from trauma and the infective diseases be prevented. Infants should not be suckled by the mother if she belongs to the dystrophic family.
The moderate use of massage and gymnastics is very important and useful. All kinds of tonic measures are indicated, such as cold baths, good nourishment, arsenic, strychnine, and phosphorus and fats. Tenotomy and other orthopaedic measures may be useful in the later stages. Feeding with thymus or other glands does no good.
Unilateral Hypertrophy of the Face.
Dr. D. W. Montgomery has collected nine cases of this affection, seven being congenital and one commencing in the second year of life. One followed a neuralgia of the fifth nerve. Dr. Mont- gomery reported a case in which the development began at the age of ten years. In this case, the hypertrophy involved the bone, and it seems to have been a type of leontiasis ossea.
Hemihypertrophy.
There have been reported in literature by Mobius, Demme, and C. B. Tilanus a few cases of hypertrophy of one side of the body. In one case, the child was born with a half-sided muscular hypertrophy. In the other cases, the disease seems to have been also of congenital origin. The trouble seems to be a kind of teratological defect, and not due to any progressive disorder of the muscular system.
Summary of the Hereditary or Family Nervous Diseases.
The student may well be confused by the large number of so- called family nervous diseases which modern neurology has dis- covered and differentiated. The practical importance of them all is, perhaps, slight, for they are extremely rare, yet it is necessary that
MUSCULAR ATROPHIES AND DYSTROPHIES.
363
they be recognized and properly distinguished, for the prognosis and degree of suffering differ very much in different cases. They are all characterized by the fact that they are found in different gen- erations and in different collateral branches of a given family, and that they are not necessarily or often passed on directly from one parent to another. The list which I append may not be entirely complete at the time of publication of this book, but it is ample.
Hereditary Chorea. — This is really a kind of hereditary paresis or brain softening. It does not develop until adult life, as a rule, and patients with it may live until middle age.
Hereditary amaurotic idiocy is a family disease first described by Dr. Sachs, characterized by a general degeneration of all the nerve- cells of the central nervous system associated with blindness and a peculiar degeneration of the optic nerves.
Hereditary Cerebral Diplegia. — This is a family disease in which children between the ages of one and five develop spastic paralysis and sometimes imbecility.
Hereditary Hemiplegia. — This is a family disease which has been referred to by Hoffmann and of which I have seen two cases in my clinic. The children are born hemiplegic and there is atrophy of the hemiplegic side, but in my cases there was no mental defect or epilepsy.
Hereditary cerebellar ataxia is a disease allied to Friedreich's ataxia, but developing somewhat later; i. e., about the time of puberty.
Hereditary spinal ataxia, or Friedreich's disease, is fully described elsewhere.
Hereditary spastic spinal paralysis is a disease which as far as is known develops before the fifth or sixth year of life, involves only the lower extremities, and is compatible somewhat with a long life. It resembles ataxic paraplegia.
Infantile hereditary progressive spinal muscular atrophy (Werdnig- Hoffmann type). This is a hereditary form of spinal muscular atrophy so rare as to be of small practical interest. It occurs in infancy and the atrophy first affects the hip-girdle, then the trunk and shoulder-girdle, and may finally involve the peripheral segments of the trunk, but not the cranial nerves. The disease runs a course in three or four years and the children generally die under the age of four. Anatomically, there is an atrophy of the spinal motor cells and nerves.
Hereditary Muscular Atrophy of Peroneal Type. — This ' has been described under the spinal atrophies.
364
DISEASES OF THE NERVOUS SYSTEM.
The hereditary progressive dystrophies include a large number of progressive diseases affecting primarily the muscles and the muscular ends of the peripheral motor neurons. Many different types of this disease are described in accordance with the muscles which are first attacked.
Arthritic Muscular Atrophy.
In inflammation of jonts the muscles moving them are affected by a simple atrophy which is called arthritic.
Etiology. — Rheumatic arthritis is the commonest cause.
Symptoms. — The shoulder-girdle muscles are oftenest affected. Whatever the joint, it is the extensor muscles which are first and most attacked ; the muscles above the joint are more susceptible to the atrophy than those below. The atrophy is rather rapid in the first few weeks and then becomes slower. The muscles affected waste throughout their whole length. They show no fibrillary con- traction and no degenerative electrical reaction. There is often an increased irritability, so that an exaggerated tendon reflex or even clonus may be produced. There is no pain or tenderness or anaesthesia in the muscles.
Pathology. — The anatomical change is a simple atrophy and shrink- ing of the muscle fibres, with some increase in muscle nuclei, little vacuolation, no swelling or splitting of fibres (Darkschewitch). There is some increase in interstitial tissue, but this is slight. The nerves and spinal cord are normal.
The atrophy is probably due to reflex action. It is a reflex tropho- neurosis. Even a neuralgic joint may cause an atrophy.
The prognosis is good. If the arthritis gets well the muscles are also restored.
The treatment consists of electricity and gentle massage and exer- cise. Internal treatment must be directed to the arthritis.
Occupation Muscular Atrophies.
As a result of constant overuse, muscles sometimes atrophy. This applies especially to the smaller muscles of the hand. Thus there occurs an atrophy of the thenar eminence in lapidaries and in persons who constantly use this group of small muscles.
Typical atrophies of this kind may be seen in persons who run eleva- tors and who have constantly to grasp the rope in one hand. Dr. J. R. Hunt describes a type of occupation atrophy of the hand due to injury of the deep (purely motor) palmar branch of the ulnar nerve. The biceps sometimes wastes in smiths and the calf muscles in ballet
MUSCULAR ATROPHIES AND DYSTROPHIES.
365
dancers. In mcst cases this occupation atrophy of muscles reaches a certain stage and steps. If the patient is given rest, recovery takes place. This is especially true if the patient is young and in vigorous health. In other instances, the simple occupation atrophy will actually pass over into progressive muscular atrophy. The trouble is usually due to a local neuritis caused by trauma. The condition is dis- tinguished from a true progressive muscular atrophy mainly by the fact that the patient has none of the aching pains and vasomotor symptoms, such as sweating in the arm, and very little if any fibrillary tremor. The electrical reactions are the same as in spinal atrophies. In ordinary occupation atrophy the seat of the disease is probably in the muscle itself. The treatment is rest and tonics, the careful ap- plication of electricity, and hypodermatic injections of strychnine.
CHAPTER XVI.
TUMORS AND CAVITIES OF THE SPINAL CORD.
Spinal Tumors.
Etiology. — Tumors rarely occur in the spinal cord. The commonest age is thirty to fifty; tubercle occurs earlier (fifteen to thirty-five — Herter) and lipoma is congenital. Males are more subject than females. Tuberculosis, syphilis and cancer predispose to the disease. Injuries and exposures appear sometimes to excite the growth of spinal neoplasms.
Symptoms. — These vary with the location, character, size and rate of the growth of the tumor. The symptoms are such as result from a foreign body slowly and progressively irritating and destroying the roots and substance of the spinal cord. Pain appears early and is a very frequent but not invariable symptom. It is severe and shooting in character. It is generally referred to nerves running out from the cord in the region of the tumor; a girdle sensation is felt; sometimes several segments lower than the lesion. Numbness, hyperesthesia and later anaesthesia occur. Tenderness over the spine and rigidity are not very frequent. The sensory symptoms are usually more on one side, but may later become bilateral. Spasm, contracture and exaggerated reflexes develop, involving one or both legs or an arm and a leg. Later paraplegia, atrophy, loss of control of the bladder and rectum, and bedsores follow, and death ensues from exhaustion.
When the disease is cervical, first one, then the other upper ex- tremity is involved, with evidence soon of implication of the pyramidal tracts. Then the legs become weak and stiff. The ciliary centre may be irritated or paralyzed if the tumor is in the lower cervical or first dorsal region. If lower down, there develops a hemiparaplegia, later a complete paraplegia, usually with exaggerated reflexes. If the tumor is in the lumbar region the reflexes are sooner lost and the sphincters early involved. In that site the tumor gradually grows and symptoms progress until the patient becomes paralyzed with anaesthesia, contractures, loss of sphincter control, bed sores and finally death from intercurrent disease or exhaustion.
A rather frequent type of symptoms caused by spinal tumors is that known as a Brown-Sequard paralysis or hemiparaplegia. In a typical
366
TUMORS AND CAVITIES OF THE SPINAL CORD.
367
case of this kind there are paralysis of motion and muscle sense with exaggeration of deep reflexes on the side of the lesion ; paralysis of cutaneous sensation, especially of pain and of temperature on the op- posite side up to just below the anaesthesic band on the side of the lesion. On the side of the lesion the temperature may be slightly raised; there is often hyperesthesia. There may be a band of anaesthesia at the level of the lesion and on the same side (Fig. 154).
The symptoms vary according as the tumor is outside or inside the dura. The extradural forms are not common, they are lipoma, cancer, gumma, and sarcoma. The greater amount of motor and sensory irrita- tion, the evidence of some vertebral disease, existence of malignant tumor elsewhere, the absence usually of hemiparaplegia, characterize extra- dural tumors. Spinal tumors are most often intradural and extra- medullary, and are usually fibromata or fibrosarcomata. The common forms of medullary tumor are glioma and tubercle. In these cases pain and spasm and rigidity are less com- mon in the early stage; hemipara- plegia is more common. A secondary myelitis sometimes develops.
The duration of the disease ranges from three to five years, the average being two or three years.
Pathology and Pathological Anat- omy.— All forms of tumor occur, but the commonest are gliomata and
sarcomata, and after this fibromata, myxomata, gummata, and tubercles. Cancer is rare; echinococcus and cysticercus are the only parasitic tumors found. Most new growths start from the membranes, the next largest number from the cord, and fewest from the vertebrae.
The meningeal tumors are mostly sarcomata and their various modifications, fibroma, enchondroma, carcinoma and lipoma. The
Fig. 154. — Showing the condition in a Brown-Sequard paralysis due to a tu- mor growing in the left side of the spinal cord. On the left side, hyperesthesia, ataxia, paralysis, exaggerated reflexes. At the upper limit is a band of anaes- thesia. On right side, anaesthesia.
368
DISEASES OF THE NERVOUS SYSTEM.
myelonic or intraspinal tumors are commonly gliomata, sarcomata, tubercle and syphilomata.
Spinal tumors are small in size, ranging from one-fifth to one and one-fifth inches (one-half to three centimetres) in diameter. The glioma may diffuse itself for a long distance through the centre of the cord, forming cavities (syringomyelia). The sarcomata may
likewise be irregularly spread along the I surface of the cord. Spinal tumors are usually single, but fibromata, the para- sites cancer and sarcomata may be multiple.
The favorite locations for spinal tumors are just below the mid-cervical, the upper and the lower dorsal regions. Wf1,. - The secondary changes produced by
tumors are softening, hemorrhages, secondary degenerations, and inflamma- tory reaction. The origin and histology of spinal tumors do not differ from that of tumors elsewhere.
The Diagnosis. — The disease has to be distinguished from vertebral caries, transverse myelitis and syphilitic menin- gitis and pachymeningitis. The points to be noted as regards caries are the absence of an external tumor or kyphosis ; the small degree of tenderness and rigidity, the age and absence of tuber- cular diathesis. The progressive course, beginning with pain, followed by motor and then sensory paralysis and the locali- zation of the symptoms exclude myelitis. Lumbar puncture may help in diagnosis by exclusion.
The character of the tumor cannot often be determined certainly. The probabilities are in favor of sarcoma or glioma, especially in middle life. Syphiloma may be suspected from the history and results of treatment. Tubercle is very rare.
The prognosis is not very good, but has been much improved by the surgeon. Medicine does little good except in syphiloma, and even here it may be ineffective. Tubercle may perhaps cease to grow. Sur-
FlG.
155. — Sarcoma of cord and cervical region.
TUMORS AND CAVITIES OF THE SPINAL CORD. 369
gical interference now saves the life of some patients and is a more hopeful form of interference on the whole than in cerebral tumors.
Treatment. — In syphilitic tumors appropriate remedies may do good and should be vigorously used. In tubercle, tonic treatment, iodine and cod-liver oil may be of service. In gliomata and sarco- mata, nitrate of silver and arsenic may be tried. In other forms, symptomatic treatment is all that can be recommended medically. In all cases of spinal tumor surgical interference should be considered ; extradural and extramedullary tumors can be removed with great benefit. Even in medullary tumors enucleation may do some good in the early stage. Exploratory operations are justifiable if there is a question as to the diagnosis of tumor. The mortality from such operations in capable hands is very low, though it is more dangerous than trephining the skull.
CAVITIES IN THE SPINAL CORD. The cavities of the spinal cord are known as:
1. Hydromyelia.
2. Myelitic cavities.
3. Syringomyelia.
There may be various combinations of those processes, the most common being a combination of hydromyelia and syringomyelia.
Hydromyelia.
This is a dilatation o f the central canal of the cord, the cavity being filled with fluid. The condition may be associated with hydro- cephalus or with spina bifida, or it may be independent of these con- ditions. The dilation may be cystic and irregular, or, as is more usual, extend throughout the cord. The dilatation extends more posteriorly, because the posterior columns are formed latest. The abnormality may be slight and give rise to no symptoms. Or a pathological process like a gliomatosis may develop upon it.
Myelitic and Hemorrhagic Cavities.
Such cavities may be found in the cord by a central excavating myelitis or by small hemorrhages. These cavities are usually small, irregularly distributed, and are lined with connective tissue. They may be formed in rare cases in connection with hydromyelia or neoplasms.
Syringomyelia.
Syringomyelia is a disease of the spinal cord characterized by a development of gliomatous tissue in the central parts with formation
24
37°
DISEASES OF THE NERVOUS SYSTEM.
of cavities. Clinically, the disease is usually characterized by peculiar disturbances of sensation and nutrition. In some cases, however, the symptoms are atypical and the disease cannot be recognized during life.
The description here given corresponds to the classic manifestation of the disease, and it is intended to refer to syringomyelia produced by gliomatosis only.
Etiology. — The disease is rare, but several hundred cases have now been reported. It is more frequent than amyotrophic lateral sclerosis and less frequent than multiple sclerosis. It occurs oftener
r. 2.
Fig. 156. — Distribution of cutaneous anaesthesia in syringomyelia. 1 shows area of analgesia, 2 shows that of thermo-anaesthesia. The darker shades show where there is anaesthesia to pain and temperature, the tactile sense being unimpaired.
in men than in women, and develops early in life, between the ages of fifteen and twenty-five.
It occurs especially in persons who follow manual occupations, such as butchers, tailors, etc. Traumatism, pregnancy, and infectious diseases seem occasionally to give rise to it. Heredity, syphilis and alcohol are not causal factors.
Symptoms. — The disease begins insidiously with some aching pains in the neck and arms and paresthesia of the hands. There is soon a muscular atrophy of the hands resembling spinal progressive muscular atrophy, with perhaps anaesthesia. As the disease progresses the weakness and atrophy of the hand muscles become more noticeable
TUMORS AND CAVITIES OF THE SPINAL CORD.
371
and gradually extend toward the trunk. The atrophy comes on in both extremities at about the same time. Fibrillary contractions and partial degeneration reaction may be observed. Cutaneous anaesthe- sia of the affected hand and arm to temperature and pain, but not to touch, takes place; and this is so marked as to be almost pathogno- monic of the disease (Fig. 156).
The legs do not become affected until late, and then generally show a spastic paraplegia. The throat and face are rarely involved. There is a scoliosis of the spine, generally in the dorso-lumbar region (Fig. 157).
Vasomotor, secretory, and trophic symptoms are prominent. The hands may be oedematous or red and congested. Sweating or dry- ness of the skin may occur. Erup- tions appear on the skin, such as bullae, herpes and eczema. Painless whitlows attack the fingers and may destroy the terminal phalanges. Erosions and ulcerations also occur. The nails become dry, brittle and drop off. Arthropathies and spon- taneous fractures have been ob- served. The pupils may be unequal and the bulb retracted.
Late in the disease symptoms of involvement of the medulla develop/ At this time also the bladder, rec- tum and genital centres are attacked. The disease progresses slowly for years, with remissions of various degree.
The cardinal symptoms are a progressive muscular atrophy, with
a peculiar partial anaesthesia, trophic disturbances, and scoliosis.
Pathological Anatomy. — The seat of the disease is the substance of the cord. On opening it by transverse sections one finds one or more cavities extending in various degrees up and down. The most frequent primary site is the cervical swelling. From here it usually
Fig. 157.
-Curvature of spine in syringo myelia. (Erb.)
372
DISEASES OF THE NERVOUS SYSTEM.
passes down and may reach the whole length of the cord. It also extends upward and may involve the medulla and the nuclei of the cranial nerves. The cavities are of irregular shape, small size and filled with a liquid like the cerebrospinal fluid. They are situated oftenest posterior to the commissure and involve one or both posterior horns, but they may be so extensive as to involve almost the whole of the centre of the cord at some levels. The walls are usually lined by a membrane and surrounded by a gliomatous tissue (Figs. 158-16 1).
This membrane is composed of a rather dense gliomatous tissue. It may be absent in some parts. The glia cells are in various stages of development and degeneration. In parts of the cord the new growth may form a large and solid mass occupying most of the centre of the cord. Small hemorrhages and foci of myelitis may be present. In some cases there is evidence of a dilated central canal, with neu- roglia hyperplasia of the walls and a gliomatous infiltration about this. The epithelium of the central canal may form part of the wall of the cavity.
Pathology. — At about the sixth week of foetal life the central canal of the cord is large, diamond shaped, and reaches nearly to the anterior and posterior surfaces of the cord. This cavity gradually contracts and unites in the middle, the anterior part forming the central and the canal and the posterior part the posterior septum. The posterior part may, through some embryological defect, fail to close, and a con- genital cavity may be left. About this a glioma may develop, and we have syringomyelia. Probably most cases of syringomyelia are de- veloped on the basis of an embryonic defect.
Gliomata were formerly thought to be practically identical with sarcomata. It is believed now, however, that they are of epiblastic origin, and like nerve-cells are modifications of epithelial tissue. This tissue in its normal state is called neuroglia. It is a nervous substance. It is composed of small cells, round or of irregular shape, with a large nucleus and fine fibrillary prolongations. In glioma these cells are relatively much more numerous, while the fibrillary net-work is less conspicuous. There is considerable variation in the relative richness of cells and fibres, however. When the former are very frequent the term gliosarcoma has been wrongly given to the tumor. The glioma is penetrated by small blood-vessels whose walls are often diseased, so that minute hemorrhages occur and the glioma becomes stained and pigmented. In other cases it is gray or yellowish in color.
The rich cellular proliferation in gliomata has suggested an analogy in its growth to that of inflammation, and the term gliosis is used as analogous for neuroglia to sclerosis of connective tissue. Gliosis differs
TUMORS AND CAVITIES OF THE SPINAL CORD.
373
Fig. 161. — Syringomyelia, lumbar segment. From microphotographs by Dr. C. I. Lambert.)
374
DISEASES OF THE NERVOUS SYSTEM.
from sclerosis, however, in the fact that in the latter process the multiplication of fibres dominates, while in the former it is the cells; besides this, in gliosis there is a tendency to softening and formation of cavities, and all nerve-fibres disappear. In sclerosis some nerve- fibres remain, and one observes the presence of granular and amyloid bodies.
Peculiar Types. — I. The disease may be latent, giving rise to very few symptoms or to none that are characteristic.
2. There may be a period of irritation and pain in the extremities followed by paraplegia, with few sensory troubles, the course suggest- ing a chronic transverse myelitis or a Brown-Sequard paralysis.
3. There is a type in which bulbar symptoms develop early, but differing from ordinary bulbar paralysis in the involvement of the trigeminus and other cranial nerves not commonly attacked.
4. There is a form characterized by a rather rapid ascending paralysis.
None of these types can ordinarily be recognized during life.
5. There is a type characterized by the symptoms of muscular atrophy with analgesia and felons (Morvan's disease). In this type there is probably a complicating neuritis. Some assert that all cases of Morvan's disease are cases of syringomyelia, but this is not proven.
Diagnosis. — The disease is distinguished in its classical form by (1) its beginning at the period of adolescence; (2) by the progressive muscu- lar atrophy combined with the peculiar dissociated disturbances of sensibility ; (3) by the trophic disturbances and scoliosis.
It has to be distinguished from progressive muscular atrophy and dystrophy, and amyotrophic lateral sclerosis, hypertrophic cervical pachymeningitis, chronic transverse myelitis, Morvan's disease and anaesthetic leprosy.
The sensory and trophic disorders and scoliosis enable one to distin- guish it from progressive muscular atrophy. In leprosy the dissociation of the sensory symptoms is not present, and the anaesthesia is distributed along the course of the nerves or in sharply circumscribed plaques. In some cases the peculiar tubercular disease of the skin and the history of the case make the diagnosis easy. In leprosy, also, there is a perineuritis, and the enlarged inflamed nerves may be felt. Portions of the skin may be excised and examined for the leprosy bacillus. As regards the differentiation from Morvan's disease, this cannot often be done. Still whitlows are rare in ordinary forms of syringomyelia. Morvan's disease begins in one hand and slowly extends, with re- missions, to the other. Usually there is loss of tactile as well as thermic and pain sense.
TUMORS AND CAVITIES OF THE SPINAL CORD.
375
The prognosis, so far as life is concerned, is bad; but the disease has often a long course, ranging from five to twenty years, and periods occur in which the progress of the disease seems arrested and improvement occurs.
Treatment. — It is not impossible ^ ^ that we may find some drug which acts specifically on gliomatous^s
tissue, checking its growth. At-^ present we know of only two things £ which may possibly do this: nitrate of silver and arsenic. These drugs should be given; and for the rest, tonic and symptomatic treatment is indicated. Intraspinal medica- tion by lumbar puncture may eventually furnish us help in con- trolling the disease.
The Recognition of Diseases of the Cauda Equina.
Anatomy. — The cauda equina is made up of five lumbar, five sacral and one coccygeal nerve-roots. They lie in the dura mater forming a thick bundle and extending down the vertebral canal for 14 cm. They are still distinct motor and sensory roots, and do not unite till they have passed out of the dura. The cauda begins at the lower tip of the cord, at the level of the lower edge of the second lumbar vertebra. The term conus is applied to the lower end of the cord and includes the parts below the second sacral segment. The cord here becomes much smaller, loses some of its dis- tinctive microscopical structure,
t 1
Fig. 162. — Showing rauda equina. Natural size. (Muller.)
376
DISEASES OF THE NERVOUS SYSTEM.
and the anterior root-fibres are smaller and less numerous than the posterior or sensory.
The arrangement of the segments and nerves is shown in Figs. 162 and 163. The arrangement of the visceral centres is given by Miiller as follows: Second sacral, erection centre; third sacral, ejaculation centre; fourth sacral, bladder (detrusor) centre; fifth sacral, sphincter-
Fig. 163. — Showing arrangement of segments and nerves of cauda equina.
ani centre. The distribution of the sensory nerves is shown in the Figs. 164 and 165.
Symptoms and Diagnosis. — The diagnosis of cauda lesions in- volves differentiation of (1) lesions of the lower end of the spinal cord; (2) cauda lesions due to compression; (3) those due to destruc- tion; (4) lesions of the peripheral nerves.
1. Lesions^of the lower end of the cord usually come on rapidly,
TUMORS AND CAVITIES OF THE SPINAL CORD.
377
i.e., in a few days (myelitis, softening, hemorrhage) ; there is little pain or sensory irritation, and later there is dissociation of sensations. Fibrillary contractions and involuntary twitchings of the leg muscles occur. Paralysis rapidly appears, involving the lower limbs in ac- cordance with the segmental distribution of the nerves. It is flacid, and is followed by atrophy. The visceral centres are involved. If
Lesion at second lumbar segment. Lesion at third lumbar segment.
Lesion at fifth lumbar segment. Lesion at first sacral segment.
Fig. 164. — Lesions at different levels of the lumbar and sacral cord, showing areas of
anaesthesia. (Miiller.)
the conus is not implicated the paralysis does not seriously involve these centres, nor the muscles of the pelvic girdle. The motor dis- turbances are more conspicuous and troublesome symptoms than are the sensory disturbances. The cord being small and destroyed, not much improvement takes place.
2. In disease of the cauda, since it is usually a tumor, the symp-
378
DISEASES OF THE NERVOUS SYSTEM.
toms come on slowly; in injury, however, the onset is sudden. There is often severe pain felt in the bladder and distribution of the sciatic nerves, and usually bilaterally. There follows after a time anaesthesia in the course of the sciatic nerves. There is little motor irritation, and paralysis follows slowly, accompanied with pain, the sensory symp- toms being all along in the foreground. The sexual, bladder, and
Lesion at second sacral segment. Lesion at third sacral segment.
Lesion at fourth sacral segment.
Fig. 16^. — Lesions at different levels of the sacral cord, showing areas of anaesthesia.
(Muller.)
rectal centres are later paralyzed. The course is progressive unless there is effective operation or medical interference.
3. The symptoms in compression of the cauda without destruction are much the same as the above, but there is less motor disturbance, and there may be no involvement of the visceral centres.
4. In lesions of the peripheral nerves, the trouble (usually neuritis
TUMORS AND CAVITIES OF THE SPINAL CORD.
379
or injury) comes on rather rapidly. There are sciatic pains, tender points, the lesion may be only unilateral, the pains are not so severe. There is little or no paralysis in ordinary sciatica, but it may occur in neuritis. There is no paralysis of the visceral centres; the sensory and motor symptoms go together, the sensory slightly predomina- ting; there is often a history of sciatica and alcoholism, or injury.
CHAPTER XVII.
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
Anatomy. — The nervous system is developed from a hollow tube formed by a folding of the epiblast. The brain or encephalon grows out from its anterior part. This swells into three cavities called the anterior, middle and posterior cerebral vesicles. From the anterior a secondary vesicle develops; the posterior divides into two; so that eventually there are five vesicles. Out of them the different parts of the brain are formed.
Fig. 166. Fig. 167.
Fig. 166. — The cerebral vesicles.
Fig. 167. — Further development of vesicles. — I2, Fore-brain or telencephalon; I1, 'tween-brain or diencephalon; II, mid-brain or mesencephalon; 77/, isthmus and meten- c^phalon or hind-brain. The isthmus is not indicated separately in the above figure. IV, after-brain or myelencephalon or medulla oblongata. (Edinger.)
1. From the anterior vesicle there grow the cerebral hemispheres, the corpus callosum and anterior commissure, fornix, corpus striatum and olfactory lobes. It includes also the anterior part of the region lying under the thalamus in which are the optic chiasm and pituitary body. These structures form the fore-brain or telencephalon.
2. From the posterior part of the primary vesicle come the thala- mus, pineal gland (or epithalamus), geniculate bodies (or metathal- ami), and some structures lying under the thalamus, viz., the corpora mammillaria and IyUys' body. These parts form the 'tween-brain or diencephalon.
380
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
381
3. From the middle cerebral vesicle there grow the corpora quad- rigemina and cerebral peduncles, with the red nuclei and substantia nigra. They form the mid-brain or mesencephalon.
4. From the fourth vesicle, which is a secondary vesicle developed from the third primary, come the "isthmus" (which includes the supe-
Fig. 168. — Still further development of vesicles. The cerebrum is being formed out of the first vesicle T2. (Edinger.)
rior cerebellar peduncles and valve of Vieussens) and the "hind- brain," composed of the cerebellum, its middle peduncles and the pons. This part is also called the metencephalon.
5. The fifth vesicle (also a development from the third) forms the medulla oblongata, or after-brain, or myelencephalon.
The development of these parts is shown in the accompanying diagrams. In man the fore-brain is enormously developed, the
Fig. 169. — Further development. The dotted lines show the original subdivision into vesicles. (Edinger.)
'tween-brain moderately developed, the olfactory lobes are atrophic, the mid-brain is almost rudimentary, the hind-brain well developed, the after-brain relatively not much developed. The divisions above given in detail seem somewhat academic and impractical as applied to adult human brains, but the main features are not so; indeed they
382
DISEASES OF THE NERVOUS SYSTEM.
are quite essential to the understanding of modern anatomy. The following simplification is sufficient for the student:
Fore-brain or telen- 1 Cerebral hemispheres and corpora striata, cephalon. J r 1
'Tween-brain or dien- 1 ^ «. « •, . ~, -u
, , > Optic thalamus and geniculate bodies,
cephalon. J
Mid-brain or mesen- 1 ^ , , , , , , .
cephalon / ^ere®rSL*- peduncles and corpora quadngemina.
Isthmus and hind-brain \ Cerebellum and its peduncles.
or metencephalon. / Pons.
After-brain or myelen- ] ,f , ,, , , J r Medulla,
cephalon J
In the process of development of the brain, the neural canal be- comes variously enlarged and constricted, until the ventricles of the brain, the foramina of Monroe, and the aqueduct of Sylvius are formed. The ventricles are the two lateral, the third, fourth and fifth. The foramina of Monroe connect the lateral and third ventricles; the aqueduct of Sylvius connects the third and fourth ventricles.
Along the mesial or inner surface of each optic thalamus runs a groove known as the fissure of Monroe. This, according to Minot, may be traced along the sides of the aqueduct of Sylvius and fourth ven- tricle into the spinal cord, where it is identical with the line of division that in embryonic life separates the dorsal from the ventral parts of the cord, forming the dorsal and ventral zones of His. Minot regards this line of demarcation as having great morphological importance. All parts of the brain and cord dorsal to it form a dorsal zone and in- clude the receptive part of the spinal cord and. medulla, as well as the cerebellum and cerebral hemispheres. This dorsal zone contains only nerve-cells whose processes never leave the nerve-centres to form nerves, and it receives all the entering sensory nerve-fibres. It is the recipient part of the nerve-centre.
The ventral zone contains all cells whose processes go out to form peripheral nerves; it has also some cells of the other type, but it does not receive any entering sensory nerve-fibres. It is the efferent part of the nerve system and it includes the anterior and part of the lateral columns of the spinal cord, as well as the ventral parts of the mid-, hind-, and after-brains.*
The different segments of the brain are composed of deposits of nerve cells forming gray matter or ganglia, and of strands of nerve- fibres connecting these ganglia. Most of the general description of these parts must be gotten from treatises on anatomy. I purpose, however, to enumerate and describe the various ganglionic deposits found in the brain ; then show the various tracts connecting them with each other and the periphery.
The sixteen ganglionic deposits of the brain are arranged in the different segments as shown here:
1. Cerebral cortex 1 ^ -brain ^ ... ( Caudate nucleus I . " , '
2. Corpus striatum j Lenticular nucleus J Telencephalon.
*I am much indebted to Prof. C. S. Minot for details regarding this conception of the structure of the nervous system.
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
383
3. Olfactory lobes.
4. Optic thalamus.
5. Corpora geniculata [ 'Tween-brain.
6. Corpora mammillaria [ Diencephalon.
7. Lwy's body J
8. Corpora quadrigemina 1 Mid.brain.
9. Red nuclei Mesencephalon
10. Substantia nigra J 1
11. Cerebellar cortex ) jjjn^ Dram
12. Cerebellar nuclei !>■ ,T , ,
, . Metencenhalon.
13. Pons nuclei J 1
14. Olivary bodies ) After_brain
ik. Nodal nuclei [,f , , i- . 1 1 • Myelencephalon.
16. Cranial nuclei J J ^
The Cortex Cerebri and the Convolutions .—The gray matter of the surface of the brain is called the cortex cerebri, and it is by far the largest and most important deposit of nerve-cells in the body. The cortex is from 2 to 4 mm. (one-twelfth to one-fifth of an inch) thick, and its total superficial area is 1,800 to 2,700 sq. cm. The area of gray matter lying in the fissures is about twice that lying on the sur- face (Donaldson). The cerebral cortex is arranged in folds or con- volutions (gyri) separated by fissures or sulci. These fissures divide the brain also into lobes. The fissures of the brain are divided into primary and secondary. The former are permanent, and present little change in size, location or direction. The latter are vari- able in all these respects, and are often called sulci for purposes of distinction.
The primary fissures of the brain are :
The longitudinal.
The transverse or choroidal.
The fissure of Rolando or central.
The fissure of Sylvius.
The parietal.
The parieto-occipital.
The calcarine.
The position of these fissures is indicated on the accompanying diagrams, which are based on descriptions of Eberstaller(Figs. 172, 174).
The secondary fissures or sulci will be enumerated in describing the lobes. They are divided into the typical or more or less constant and the atypical or tertiary fissures.
The primary fissures divide the cerebrum into various portions called lobes. The lobes are :
Frontal. The central or island of Reil.
Parietal. Olfactory. Temporal. Limbic. Occipital.
There are eight fissures and seven lobes.
The frontal lobe is limited behind by the fissure of Rolando, and on the median surface by the calloso-marginal or subfrontal fissure and its ascending branch. It has the following sulci: 1. Superior frontal and superior precentral. 2. Infe'rior frontal and inferior precentral. 3. Olfactory. 4. Triradiate.
3§4
DISEASES OF THE NERVOUS SYSTEM.
In the frontal lobe are the following convolutions: I. Precentral or ascending frontal, having on its median surface (a) the paracentral lobule. 2. First or superior frontal. 3. Second or middle frontal. 4. Third or inferior frontal (Fig. 172).
The parietal lobe is limited behind by the parieto-occipital and an- terior occipital fissures, below on its outer surface by the Sylvian fis-
Fig. 170. — Type of cell lamination in the Fig. 171. — Type of cell lamination in
post-central area. (Campbell.) the precentral area (Campbell). In the
sixth layer is seen a cell of Betz.
sure and a line drawn back from its upper end to the lower part of the anterior occipital. In front it is limited by the fissure of Rolando. On its median surface it is limited in front by the ascending branch of the calloso-marginal or subfrontal fissure, behind by the parieto- occipital, and below by the subparietal fissure. Its important sulci are: t. The parietal or interparietal. 2. The postcentral. The con-
ANATOMY AND PHYSIOLOGY OF THE BRAIN. 385
volutions formed are, on the convex surface: 1. The postcentral or as- cending parietal. 2. The superior parietal lobule, having on its median surface (a) the quadrate lobule or precuneus. 3. The in- ferior parietal lobule, which is composed of (a) the supramarginal gyrus, (6) the angular gyrus. These latter gyri are variously de- scribed, but it is best to regard one, (a) the supramarginal, as that surrounding the posterior end of the fissure of Sylvius; the other, (b) the angular, as that beginning at the upper limit of the first temporal fissure and extending back as far as the anterior occipital fissure.
The Occipital Lobe. — -This is limited on its convex surface, an- teriorly, by the parieto-occipital fissure, laterally to this by the an- terior occipital and inferior occipital fissures. These in apes form a single continuous fissure sharply separating the occipital from the other lobes. On its median surface the parieto-occipital fissure limits the lobe in front. On the under surface the anterior edge of the tentorium about marks the anterior limit.
Fig. 172. — Left hemisphere, from without.
The sulci are: i. The transverse occipital (ape fissure of some writers). 2. The superior or lateral occipital. 3. The inferior oc- cipital. On the median surface: 4. The calcarine, which joins the parieto-occipital. 5. The inferior occipito-temporal or fourth temporal.
The convolutions on the convex surface are: 1. The superior oc- cipital. 2. The middle occipital. 3. The inferior occipital. 4. The descending occipital. On the median surface we find: 5. The cuneus. 6. Descending occipital.
The Temporal Lobe.— The convex or lateral surface shows the following sulci: 1. The first temporal or parallel sulcus. 2. The second or middle sulcus. On the under and median surfaces are : 3. The third or inferior temporal sulcus. 4. The fourth temporal or inferior occipito-temporal or collateral sulcus, which extends into the occipital lobe. 5. The hippocampal sulcus.
The convolutions are: 1. The first temporal convolution. 2. The
25
386
DISEASES OF THE NERVOUS SYSTEM.
second temporal convolution. 3. The third temporal convolution. 4. The lateral occipito-temporal or fusiform convolution. 5. The median occipito-temporal or lingual convolution. 6. The hippo- campal convolution, which is an extension of 4 and 5. 7. The uncinate convolution, which is an extension of 6.
The Island of Reil (lobus centralis, lobus caudicis). — This has a circular sulcus surrounding it, and several more or less short sulci dividing it into from five to seven short gyri.
The Limbic Lobe, if described as a separate lobe, is made to include anteriorly the gyrus fornicatus or gyrus cinguli; back of this the isthmus; then the hippocampal, uncinate, and dentate convolutions (Fig. 174).
Fig. 173. — The convolutions from above.
The olfactory lobe is rudimentary in man. Its position is shown in the diagrams.
The operculum is the part of the brain that overlaps the island of Reil. It consists of a fronto-parietal part, formed by the lower ends of the two central convolutions, a frontal part formed by the base of the inferior frontal convolution, and a temporal part formed by the tip of the temporal lobe.
The cuneus, precuneus, and paracentral lobule are important sub- divisions on the median surface of the brain. Their position and boundaries are shown in the cuts (Fig. 174).
Microscopical Anatomy of the Convolutions.- — -The cortex of the cerebrum is composed of nerve-cells, a net-work of nerve-fibres and
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387
processes, and of neuroglia tissue. Superimposed upon it is a very vascular membrane, the pia mater, which sends a rich plexus of ves sels into it. We shall proceed to study: 1st, the structure and ar- rangement of the nerve-fibres and neuroglia; 2d, the arrangement of the nerve-cells. On both these points new facts are being constantly added, and the present description must be in many respects only provisional.
The Fibre Layers of the Cortex. — The nerve-fibres of the cortex are arranged in more or less definite zones or layers. Some of these layers are very well marked in one part of the cortex, less so in others. They have received different names, but adopting the nomenclature and classification of Campbell, the principle zones are:
1. An outermost fibreless or neuroglia layer.
2. Zonal or tangential layer.
3. Line of Baillarger; in the occipital cortex called the line of Glenari.
4. Radiary zone.
Passing into the cortex at right angles to these layers are the radi- ations of Meynert ; and below the cell layers are the association fibres of Meynert.
Cell Layers. — The cells of the cortex are arranged in layers more or less definitely marked, and varying in different parts of the cortex. The type of arrangement as given by Campbell is as follows:
1. The plexiform layer containing the cells of Cajal. It is also called the molecular or first layer.
2. The layer of small pyramidal cells; the cells here are closely packed, and while most of the cells are pyramidal, some are poly- morphous. This is called also the second layer. The cells are about the size of a white blood-cell ; this layer is fused with the third layer.
3. Layer of medium-sized pyramidal cells. Here the cells are all pyramidal and increase in size from above downward.
/ Gyr. descendens
Sulc. Mttremus
Fig. 174. — Mesial surface.
388
DISEASES OF THE NERVOUS SYSTEM.
4. Layer of large pyramidal cells, lying on a level with the line of Baillarger.
5. Layer of stellate cells, known also as the granule layer or Kornerschicht. This is best represented in the calcarine cortex.
6. Internal layer of large pyramidal cells; this contains the giant cells of Betz, and in the visual cortex the large solitary cells.
7. Layer of spindle cells.
The small pyramidal and spindle cells measure about 10 x 18 /*; the large, 20 x 40 n (ttoo x6~io m0- There are in the precentral con- volutions giant cells (of Betz) which measure 125 x 55 m (xhj-x sro m-)- (See Plate.)
The cortical gray matter, as will thus be seen, contains layers of nerve-cells, into which nerve-fibres penetrate. These terminate, as do all fibres, in end brushes, which surround the receptive or sensory
Fig. 175. — Under surface of brain. LOT, lateral occipito-temporal or fusiform lobe, MOT, median occipito-temporal or lingual lobe.
cells. An enormous number of fine fibres is given off by the cells; some of these form layers in the cortex and connect neighboring parts, others run out and connect more distant parts or pass down to lower levels. There are thus three kinds of fibres — afferent, associative and efferent — just as there are three types of cells, and since nerve-cells and fibres are really parts of the same unit — the neuron — there are prac- tically three kinds of neurons in the cortex.
The different convolutions and lobes of the brain are connected with each other by association fibres and commissural fibres and to the ganglionic masses below them by projection fibres. The associa- tion fibres consist of short fibres connecting neighboring convolutions and of long tracts which connect neighboring or distant lobes.
The short association fibres are numerous ; they lie close beneath the gray matter and connect convolutions of the same lobes. The course of many of the long association paths is not yet well known. The oc- cipital lobe is connected by long tracts to the temporal lobe, and per-
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
389
haps slightly to the inferior parietal lobe (H. Sachs). The temporal lobes are connected only to the occipital. The frontal lobe is ap- parently connected with the parietal. Its connection with the tem- poral is denied (H. Sachs). All the lobes of the two halves of the brain are connected with each other by commissural fibres in the corpus callosum and anterior commissures. I will describe the projection fibres later.
Thk Corpora Striata which form the second ganglionic mass to be studied are composed of two parts: the caudate nucleus and lenti- cular nucleus. These two nuclei are separated dorsally by the white fibres forming the internal capsule. Below they are continuous. Their shape and relations are not easily appreciated except by actual
Fig. 176. — Longitudinal section, showing the lenticular (In) and caudate nuclei (en) separated by the internal capsule (ic), the corpus callosum (cc), the anterior commissure (ac), the optic tract (27), the optic thalamus (th), the corpora geniculata (g), and the pulvinar (p), the crusta (cr), the tegmentum (tg), pons (P), and cerebellum (C); p, puta- men; g, globus pallidus. (G. Stewart.)
inspection of specimens. They are indicated in the accompanying figures (176-178). The head of the caudate nucleus is connected with the gray matter of the anterior perforated space. The tail extends into the temporal lobe, where it is continuous with the gray matter of the cortex, called at this point the amygdalum, and with the claus- trum. The lenticular nucleus is also continuous with the gray matter of the anterior perforated space. It is divided into three portions ; the external is called the putamen, the inner the globus pallidus. These parts are separated by white fibres. The caudate nucleus, putamen, and probably all of the corpus striatum are modifications of the cere- bral cortex.
The corpora striata are relatively rudimentary ganglia in man. They contain sparsely distributed multipolar and fusiform cells, ttoit
39°
DISEASES OF THE NERVOUS SYSTEM.
to 5~J-o in. in diameter, the larger being in the lenticular nucleus. They are chiefly of the second or Golgi type.
The Optic Thalami. — These ganglia form the chief part of the 'cween-brain. They lie at the base of the brain, posterior and in- ternal to the corpora striata. They are continuous with each other by means of the middle gray commissure. The upper or dorsal surface forms part of the wall of the lateral ventricles ; the mesial surface forms the lateral wall of the third ventricle. Externally is the band of white
Fig. 177. — Horizontal section, showing the frontal (F), temporo-sphenoidal (TS), and occipital lobes (O), with their gray and white matter, the island of Reil (R), its gray and white substance, the claustrum (cl) the external capsule (ec), the lenticular nucleus (In), the caudate nucleus (en), the internal capsule (ic), the optic thalamus (th), the pul- vinar (p), the corpus callosum (cc), the anterior and posterior commissures (ac and pc), the lateral ventricle (LV), with the choroid plexus (ch). (G. Stewart.)
fibres called the internal capsule ; below this is a rather large mass, the red nucleus, external to a small gray nucleus called the subthalamic ganglion (Luys' body). Around and below these is a complex net- work of fibres called the stratum intermedium. Below this, on the base of the brain, are the corpora mammillaria. The thalamus is composed of six nuclei — the anterior, lateral, median, ventral, posterior and the pulvinar (Fig. 178). Besides these there is on the median surface a small ganglion called the g. habenulae. The
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
391
optic tracts wind around the posterior and outer edge of the thalamus ; and connected with these and the thalamus are two other ganglia, the external or lateral and internal or median geniculate bodies. Thus the thalamus is in close anatomical relations with five small ganglia ; three below it — the corpus mamillare, red nucleus, and subthalamus; two postero-external to it, the geniculate bodies. To these may be added the corpora quadrigemina. The thalamus is composed of multipolar nerve-cells rather more numerous and larger than those in the striatum, but not grouped closely together. Starr describes a small stellate multipolar cell and a large multipolar cell. There are, besides these, cells of the second or Golgi type which have associative functions. The optic thalamus is composed of nerve-cells whose neuraxons pass
Fig. 178. — Section through the middle of the basal ganglia, precentral (AF), and part of postcentral (AP) convolutions, and (TS) temporal lobe. It shows the corpus callosum (cc), the fornix (&), the fifth ventricle (Y), lateral ventricles (LV), small part of thalamus (th), internal capsule (ic), caudate nucleus (ch), lenticular nucleus (In), external capsule (ee), claustrum (cl). (G. Stewart).
up to the cortex ; and of the terminal fibres from neurons lying in the cortex and in the sensory cranial and spinal nuclei below. Between these are the Golgi or associative cells which unite the various af- ferent and efferent neurons. The optic thalamus is a terminal station of numerous fibres of sensation coming from the medulla and cord ; it is also the place of origin of cells which send processes to the cortex and the receiving centre of fibres coming from the cortex. It is, therefore, a very important sensory-motor ganglion connected with the reception and distribution of sensory and motor influence and with the auto- matic and reflex movements.
The Corpora Quadrigemina. — The mid-brain contains as its chief ganglia the corpora quadrigemina. These consist of four tuber-
392
DISEASES OF THE NERVOUS SYSTEM.
cles, two anterior and two posterior (Fig. 179). In man they are rudi- mentary in structure and relatively unimportant in function. In man also the posterior tubercles are developed more relatively than in most
Fig. 179. — Showing the relations of the optic thalamus to other parts. (Edinger.)
lower animals. They together measure about 14 mm. (h in.) in sagittal direction. In front lie the pineal gland and third ventricle; beneath are the aqueduct of Sylvius and the structures of the upper pons and cerebral peduncles. This inferior boundary is shown by the dotted
Fig. 180. — Section through the corpora quadrigemina (Q), tegmentum (TG), and cere- bral peduncles. SS, Substantia nigra; Pp, peduncles; AS, aqueduct of Sylvius.
line in the cut (Fig. 180). Microscopically the anterior lobes consist of layers suggesting a cortical type (Spitzka) ; the outermost is made up of optic-tract fibres, the next is a thin layer of small nerve-cells, then
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
393
optic fibres again, and deepest of all a layer containing a few large cells. The posterior lobes are more homogeneous and contain small multi- polar cells and a ganglion.
The figures 176 to 178 show the relationships of the basal ganglia and other parts.
The Substantia Nigra. — Below the corpora quadrigemina and lying between the upper sensory part {tegmentum) of the peduncles of the brain and the lower motor part (crusta) is the substantia nigra. It contains large multipolar, angular, and fusiform cells deeply pig- mented (Fig. 180).
The Red Nucleus. — Dorsal to the s. nigra and at about its middle extent is the red nucleus. It is spherical or oblong, very vascular, and contains numerous small cells.
The Subthalamus (Lilys' body) lies more dorsally but in about the
Fig. 181. — Showing the lobes and other subdivisions of the cerebellum, dorsal surface. (Edinger.)
same plane as the substantia nigra. It measures about J by J inch, and contains a few cells and a very fine plexus of nerve-fibres.
The position and relation of these bodies are shown in Fig. 176.
The nuclei of the pons Varolii are irregularly distributed masses of nerve-cells lying deep among the longitudinal and transverse fibres. A special nucleus lying low down in the pons is known as the superior olive. In the after-brain or medulla we have the gray matter of the floor of the fourth ventricle and its cranial nerve nuclei, the olivary bodies, and certain deposits of gray matter called the nodal nuclei.
The Cerebellum in man consists of a median portion or vermis and two lateral hemispheres. It is connected to the cord and rest of the brain by anterior, middle, and posterior peduncles. It is com- posed of an external layer or cortex of gray matter, of central white matter, and central ganglia. The gray matter lies in very close, nar- row folds, producing with the white matter an appearance on section called the arbor vitce. The vermis and hemispheres are divided by sulci into a number of lobes and lobules. The vermis is divided into
394
DISEASES OF THE NERVOUS SYSTEM.
superior and inferior portions. Its further subdivisions and those of the hemispheres are shown in the cuts (Figs. 181, 182). In the white matter of either hemisphere is a nucleus of small multipolar cells, the corpus dentatus or ciliary body. To the median side of this, and be- longing structurally to it, is a small nucleus, the emboliform nucleus.
Fig. 182. — Cerebellum, ventral surface. (Edinger.)
In the inferior vermis is a collection of larger multipolar cells, the nucleus fastigium or tegmental nucleus; just to the outer side, between it and the emboliform nucleus, is a small collection of cells, resembling those of the n. fastigii, called the nucleus globosus.
The Cerebellar Cortex. — If a section be taken through the gray matter of the cerebellum, it will be found to be composed of two layers:
Fig. 183. — Section through middle of vermis and pons.
an outer, or molecular, and an inner, or granular, layer. Each of these layers contains a large number of peculiar-shaped nerve-cells, and very rich plexus of nerve-fibres. The molecular layer contains two kinds of cells, one large and known as Purkinje's cells, the other smaller and known as stellate cells. The cells of Purkinje lie the more
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
395
deeply, being, in fact, practically at the boundary of the molecular and granular layers. They measure 40 x 30 n (¥^o to -^\T) in.) and have large round nuclei. Each cell gives off an enormous number of branch- ing dendrites, which run up toward the surface of the cerebellum in the shape of a bush. Each little branch sends off from the side small buds, which are called the gemmules or thorns. These branching dendrites do not pass up altogether like the branches of a round bush, but are flattened like a broom. The Purkinje cells give off from their base a neuraxon which runs down into the white matter of the cere- bellum (Fig. 184, P). Lying throughout the molecular layer are the stellate cells, which are much smaller in size, and which also give off a
Fig. 184. — The different constituent elements of the gray cortical layer of the cerebellum
number of dendrites (Fig. 184, S). Each cell has also its axis cylinder (neuraxon) and this sends off collaterals which end in a fine basket- like net-work which surrounds the body of the cells of Purkinje (Fig. 184). On this account they are sometimes called basket cells. There are other stellate-shaped cells in the molecular layer which lie more superficially, and do not have this particular connection with the Purkinje cells, but appear to belong to the same type.
The granular layer contains a large number of very small granular- like cells that Golgi was the first to show were really nerve-cells. They are only about 5 p. ( 7 ^Vo in-) m diameter, and they have a number of
396
DISEASES OF THE NERVOUS SYSTEM.
short dendrites which end in clubbed extremities. (Fig. 184, G). They give off a very fine axis-cylinder process (neuraxon) which runs up into the molecular layer and there divides in a T-shaped fashion, the fibres running parallel to the surface of the convolution and passing in between the branches of the cells of Purkinje. There are besides these granular cells a few larger cells with axis cylinders that divide and sub- divide, ending in a finely ramifying plexus. These are of the type known as the cells of Golgi. They are found in other parts of the brain.
The nerve-fibres of the cerebellar cortex are, as in the cerebrum, radiating and tangential. The tangential fibres lie at the level of the Purkinje cells, among the processes of which they run. Just be- neath the pia is a thin connective or neuroglia tissue membrane which
Fig. 185. — Schematic diagram of the relations of the cerebellar cells. A, afferent fibre to basket (stellate) cell; B, neuraxon of Purkinje cell; C, afferent fibre to Purkinje cell; D, afferent (mossy) fibre to granule cell.
sends radial fibres down through the gray matter, affording it a support.
It will be seen that the general arrangement of the cerebellar cortex is analogous to that of the cerebrum. Associative and receptive cells are found in the granular and molecular layers, and they send proc- esses forming a rich net-work around Purkinje's cells, which are efferent in function (Fig. 185). The comparatively small number of the large cells is in harmony with the view that the cerebellum is an organ that receives and adjusts nerve impulses for co-ordinate distri- bution. All parts of the cerebellar cortex are anatomically alike.
The white matter of the cerebellum consists of nerve-fibres, some of which go to form the peduncles. Others form anterior and pos- terior commissures, running through the two extremities of the ver- mis and connecting the hemispheres. There is also a longitudinal commissure in the vermis. The white matter around the corpus dentatum is called the fleece.
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The Connecting Tracts.
Having described the general arrangement of the different divisions of the brain and the collections of gray matter found in them, we are prepared to study the tracts of white matter which connect the dif- ferent parts.
The white matter, as already shown, is made up of :
{ Short.
1. Association fibres \ Long.
[ Commissural.
2. Projection fibres and interganglionic tracts.
1. The association fibres of the cortex have been already described.
2. The projection fibres are those which connect different areas of the cortex with the basal ganglia and the ganglionic masses of the pons, medulla and spinal cord. Researches by Flechsig have led him to assert that the projection fibres are much less numerous than has been suspected, and that they come only from the central convolu- tions, part of the first and second temporal, part of the occipital lobe, the hippocampus, uncus, and part of the limbic lobe. These are the parts of the brain identified with the function of voluntary movement and general and tactile sensation, hearing, sight, smell and taste. The area of the cortex of the brain thus connected to parts below by pro- jection fibres is only about one-third of the whole. The remaining two-thirds of the brain cortex is not in direct connection with parts below, but is closely connected with the projection centres by associa- tion fibres. Flechsig considers the parts of the brain which are thus connected by association fibres as higher centres, identical with the more complex mental acts, and he calls them the association centres.
The association centres according to this view are the frontal lobes, part of the parietal and occipital and part of the temporal lobes. The views of Flechsig have in general been adopted, but may require con- siderable modification.
The projection fibres of the brain form the different pathways (a) by which the special and general sensations pass up to the brain cortex and (b) by which the voluntary, automatic, and psycho-reflex move- ments of the body are brought about.
(b) The Motor Tracts. — The cerebrospinal motor paths are of two kinds: the direct or voluntary and the indirect motor tracts. The direct motor tract originates from cells in the precentral convolutions ; the neuraxons of these cells pass down and are gathered together in a narrow band which passes through and occupies nearly the whole of the posterior segment of the internal capsule. The fibres continue on into the pons Varolii and medulla, and at the latter point give off some terminals which cross (except those for the sixth nerve) to the nuclei of the motor cranial nerves. The rest of the bundle passes on through the medulla, and ninety per cent, cross over at its lower portion, form- ing there the anterior pyramids. About ten per cent, of the fibres do not cross, however, but continue on the same side. The crossed bundle passes into the lateral column of the spinal cord, forming the crossed pyramidal tract, which passes on, diminishing in size as far as
398
DISEASES OF THE NERVOUS SYSTEM.
the sacral part of the cord. It gives off terminal end brushes which surround the cells of the anterior horns. The small uncrossed band of fibres continues on in the mesial part of the anterior column, form- ing the direct pyramidal tract, or column of Tiirck. The fibres of this tract cross over in the anterior commissure at different levels, and their terminals also connect with the motor cells of the anterior horns. Thus it will be seen that the direct motor tract is a long, continuous
Fig. i 86. — Diagram of the direct or Fig. 187. — Diagram of the indirect or voluntary motor tract, showing the course involuntary motor tract,
of the motor impulses from the cerebral cortex to the voluntary muscles (after Van Gehuchten).
strand of fibres, composed of single neurons putting the cortex of the central convolution directly in contact with the motor cells of the pons, medulla, and spinal cord of the opposite side. Each pyramidal tract, as it reaches the spinal cord, contains about eighty thousand fibres (Fig. 186).
The indirect motor tract arises from nerve-cells in the anterior central convolution and, perhaps, to some extent in the frontal lobe adjoining. Its fibres pass down into the internal capsule, mingling directly with
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
399
those of the direct motor tract and giving off collaterals to the optic thalamus. The fibres pass through the cerebral peduncles, occupying their inner four-fifths, or motor part, and, finally, reach certain de- posits of nerve-cells in the pons Varolii known as the pons nuclei. They surround these cells here with terminal end brushes. From these cells neuraxons cross the median line in the middle cerebellar peduncle and thence to the cortex of the lateral lobes of the cerebellum,
Fig. 188. — The direct sensory tract, showing the arrangements of the neurons. (Van Gehuchten.)
where they in turn end. From here the nerve-cells send fibres to the nucleijof the cerebellum and the vestibular nucleus, thence down into the spinal cord, where they pass along mainly in the lateral funda- mental columns, to connect finally with the anterior-horn cells. Thus it will be seen that the indirect motor tract is composed of (a) a cortico- pontine neuron, (b) a pons-cerebellar neuron, (c) a cerebello-ganglionic neuron, (d) a vestibulo-spinal and (e) the peripheral motor neuron (Fig. 187).
The direct motor tract is concerned in all voluntary movements,
4QO
DISEASES OF THE NERVOUS SYSTEM.
and when the anterior-horn cells of the cord are cut off from it by disease there is a spastic form of paralysis. The indirect motor tract is concerned in the co-ordination of bodily movement and in the higher reflex and automatic acts. It is largely through these indirect tracts that the skilled automatic movements take place. Playing musical instruments and the involuntary use of the hands and limbs in work or games of skill are under the control of this mechanism. When the spinal cord is cut off from it, there is an unsteady and disor- dered gait and arm movement. The course of the direct and indirect motor tracts is shown in the accompanying diagrams.
The Sensory Tracts. — The next important pathways in the brain and cord are the sensory, and they are concerned in bringing tactile, muscular and general sensations from the remoter parts of the body to the cortex of the brain. It will be easier to follow these tracts if we begin at the periphery and follow the course of the fibres up to their centres in the brain. Just as in the case of motor fibres, we find direct and indirect tracts, although here even the direct sensory path is more tortuous and broken than is the case with the motor tracts.
The Cutaneous Sensory Tract. — A tactile irritation of the skin passes up the sensory nerve to a posterior spinal ganglion, where the cell body from which the fibre is derived is found. It passes directly through the ganglion, enters the posterior root of the spinal cord, and passes up to a group of cells lying in the posterior horns, where it meets and sur- rounds with its end brush a second sensory cell. A second neuraxon starts from the body of this cell; most fibres cross over in the posterior commissures of the spinal cord to the posterior column of the other side where they run up in the posterior columns and gray matter by a series of chains or successive neurons, reach the medulla and pons, and pass through this to the optic thalamus. Here they send terminals to another cell, which in turn sends a neuraxon to the cortex of the post- central convolutions. Thus the cortical centres of the direct sensory path are just posterior to the area from which the motor tract started. The direct sensory tract is made up of (i) a peripheral sensory neuron, (2) a series of intraspinal neurons, (3) a spinal-thalamic neuron, (4) a thalamic-cortical neuron (see Fig. 188).
The pain and temperature fibres take a similar course except that they cross over in the anterior commissure and ascend in the lateral columns to the thalamus.
The deep sensory tracts convey impulses which originate in muscles, joints and the viscera. The impulses pass up sensory nerves through the posterior roots. Some now pass directly into the posterior column of the cord of the same side and ascend till they reach the upper end, where their end brushes surround the cells of the nuclei of the column of Goll and of Burdach. From there they cross to the other side in the sensory decussation. Some then go to the red nuclei and optic thalamus, where they terminate. Another neuron now car- ries the impulse on to the parietal convolutions. Another set of deep sensory impulses go from the sensory roots to the cells of the column of Clark, thence by the cerebellar tracts to the cere- bellum. This indirect sensory tract is thus composed of (1) a peripheral sensory neuron, (2) a spinal-cerebellar neuron, (3) a cere-
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
bellar-thalamic, and (4) a thalamic-cortex neuron. Here, too, the mechanism is not so simple. Some of the afferent fibres do not go higher than the cerebellum, but are reflected to lower nuclei of the brain stem and thus automatic movements and the higher reflexes are brought about. The direct sensory tracts carry, for the most part, the sense of touch, pain, and temperature. Some direct sensory tracts are con- cerned with the sensation from the muscles and joints which have to do with co-ordination, and also with visceral sensations. It is through the indirect sensory and indirect motor tracts that the automatic and psycho-reflex acts are performed.
Other Projection Systems. — The optic, acoustic, and olfactory pro- jection tracts have been described in connection with their peripheral nerves.
The Membranes ok the Brain. — The membranes of the brain are the dura mater, the arachnoid, and pia mater. The dura mater lines the inner surface of the skull. It is attached loosely to the con- cavity, but closely to the base. It splits into two layers to form the venous sinuses of the skull. The inner of the two layers at certain points projects inward to form membranous septa. These are known as the great longitudinal or cerebral falx, the lesser longitudinal or cerebellar falx, and the tentorium. Hence both venous sinuses and membranous septa are formed out of the inner layer. The outer layer forms the periosteum of the bone. The dura mater is supplied with sensory nerves, chiefly by the trigeminus but posteriorly by the vagus. The blood-supply will be described later.
The arachnoid is a thin, transparent, fibrous, non-vascular mem- brane lying between the pia and dura and continuous with the spinal arachnoid. It bridges over the fissures and the depressions at the base of the brain and forms between the pia and itself certain lacunae or spaces. These are the central lacuna found at the beginning of the fissure of Sylvius, the callosal, and those of the transverse fissures and of the lateral aspect of the pons Varolii. The space between the dura and arachnoid is called the subdural or arachnoid cavity. It is lined with epithelium and resembles other serous cavities. The inner sur- face of the arachnoid is connected with the pia by numerous delicate fibrous processes. The space between these membranes is called the subarachnoid space. It communicates with the subdural space by means of the foramen of Magendie, which lies in the part of the arach- noid that passes over the pons and medulla, closing in the fourth ventricle. The subdural and subarachnoid spaces contain a serous fluid. It is a secretion, not a transudate, and comes mostly from the choroid plexus of the lateral A^entricles. The arachnoid contains no nerves or blood-vessels. It is described by some as a part of the pia mater.
The pia mater lies beneath the arachnoid and is closely applied to the brain in all its folds. It is continuous with the spinal pia. It is very vascular and supplies the whole periphery and part of the interior of the brain with blood. It consists of two layers: an outer holding the larger vessels, and an inner delicate layer closely associated with the superficial neuroglia of the brain. The pia mater folds upon itself and passes through the transverse fissure into the third and lateral 26
402
DISEASES OF THE NERVOUS SYSTEM.
ventricles of the brain. These vascular folds form the velum inter- positum, which gives off a choroid plexus to the lateral and third ven- tricles. Another fold, the inferior choroid plexus, is given off to the fourth ventricle. The pia mater has vasomotor, but no sensory nerves.
Functions of the Brain Membrane. — The dura mater, by its outer layer, acts as a periosteum; by its inner layer as a lymph sac. It is also, by virtue of its sensitiveness, a protection against injury and disease. It is supplied with sensory nerves, but is not, in fact, very sen- sitive normally and may be cut and handled without pain. Strains upon it, however, do cause some pain (Cushing). The arachnoid forms the inner wall of the sac. The pia mater is a vascular and nutritive organ. It is, however, also closely connected with the lymphatic system of the arachnoid. The blood and cerebrospinal fluid of the brain vary in amount. In congestion the cerebrospinal fluid can pass into the spinal subarachnoid cavity or be rapidly taken up by the absorbents. In anaemia and atrophy there may be compensatory increase of the fluid.
The) cerebrospinal fluid is probably a secretion and comes mostly from the choroid plexuses of the lateral ventricles which are covered by the ependyma. These can if needed pour out an enormous amount which flows into the third and fourth ventricles and thence through the foramina of Magendie and Luschka into the sub- arachnoid cavities of the brain and cord. It flows under a pressure of 5 to 20 mm. of mercury and is eventually absorbed by the blood-vessels. A little of it probably leaks into the cavity of the arachnoid proper.
The cerebrospinal fluid is colorless, transparent, alkaline. Specific gravity, 1007.
Its composition is about as follows : (Halliburton) water 99, solids 1. The solids consist of: Proteids, .085; extractives, .060; salts, .865.
The proteids consist of serum globulin, and allied bodies. (Al- bumin is a normal ingredient, according to Bernstein.) A substance that reduces Fehling solution is present. There is no fibrin ferment, and the fluid does not coagulate on boiling, or there is only a trace of turbidity. Under the microscope it is practically free from cellular bodies. The normal amount of fluid ranges from 3ii to being greater in the aged.
There occur changes in the spinal fluid in inflammatory and degen- erative diseases of the spinal cord, including syphilis, parasyphilis (in its early stages) and multiple sclerosis.
The Blood-supply of the Brain and its Membranes. — The vascular supply of the scalp, skull, and dura mater comes from the external carotids; that of the eye, brain, and pia mater from the in- ternal carotids and vertebrals. The arrangement is shown here :
[ Occipital, inferior meningeal, arteries. I Posterior auricular.
External carotid gives off
f Anterior. \ Temporal \ Middle.
( Posterior.
I Ascending pharyngeal, posterior meningeal. { Internal maxillary, middle meningeal, small meningeal.
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Internal carotid gives off
Vertebra] and basilar give off
Anterior meningeal.
Anterior cerebral. \ Middle cerebral, j Posterior communicating.
Anterior choroid. / Posterior meningeal, j I Inferior cerebellar.
Anterior cerebellar.
Superior cerebellar. [ Posterior cerebral.
The general arrangement and distribution of the arteries of the scalp and dura are shown in the accompanying diagram (Fig. 189). The blood-supply of the meninges comes from the anterior, middle,
Fig. i8q. — Showing the blood-supply of the scalp and that of the dura mater by the
middle meningeal.
and posterior meningeal arteries. These all come, except the small anterior meningeal branchei and a small posterior branch, from the external carotid. The blood passes into the diploic veins, and from there passes chiefly into the lower occipital and lateral sinuses. Some of it, however, returns in the venae comites. It all returns down toward the base of the skull. The most important of the arteries is the middle meningeal, both on account of its size and its distribution above important functional areas.
404
DISEASES OF THE NERVOUS SYSTEM.
The blood-supply to the pia mater and brain substance comes from the internal carotid and the vertebral arteries. The branches of the former artery give off the anterior and middle cerebral, the posterior communicating and anterior choroid. The vertebral arteries give off the inferior cerebellar, while the basilar branch of the vertebrals gives off the transverse, anterior cerebellar, superior cerebellar, and posterior cerebral arteries.
Fig. 190. — Showing the arteries at the base of the brain. On the right side the brain is cut away, showing the cerebral arteries and the course of the posterior cerebral.
The anterior, middle and posterior cerebral arteries, are the three largest and most important. By their anastomoses the circle of Willis is formed (Fig. 190). From the circle of Willis and the beginnings of the three arteries mentioned, several groups of vessels, six in all, are given off. They enter the base of the brain and supply the great basal ganglia and adjacent white matter. They are called the central
ANATOMY AND PHYSIOLOGY OF THE BRAIN. 405
arteries, they do not anastomose, and they are the vessels usually af- fected in cerebral hemorrhages of adult life. One set of these central ar- teries, the internal striate, passes up and supplies the two inner segments of the lenticular nucleus, the caudate nucleus and anterior part of the internal capsule. Another set, the external striate, divide into an- terior or lenticulo-striate branches which supply the lenticular nucleus and posterior part of the caudate. One of these larger than the rest is called the artery of cerebral hemorrhage. The other or posterior branch is called the lenticulo-optic and supplies the anterior part of the thalamus. According to Kalisko, the lenticular nucleus is sup- plied in part by a branch from the anterior cerebral. This he calls the artery of cerebral thrombosis. It is especially effected in severe
Fig. 191. — Showing the distribution of the artery of the Sylvian fissure, a prolonga- tion of the middle cerebral. The area in front of the shaded part is supplied by the anterior cerebral, that behind by the posterior cerebral.
gas poisoning. The cortical arteries are the terminal branches of the great cerebral arteries. They anastomose with each other but slightly. They are distributed very widely and carry much more blood than the central groups. Their distribution is shown in Fig. 191. The cortical arteries are distributed in the pia, and from there they pass in two sets, a superficial and a deep, into the gray matter, and for a short distance into the white matter. They pass straight in at right angles to the surface. They have richly arborescent branches which do not anastomose ; consequently a knife plunged straight into the brain does not cut many vessels.
The capillaries are surrounded by spaces called perivascular spaces which serve as lymphatic channels. The neuroglia cells send proc- esses which connect with or form passages to the vessel walls). The blood-vessels of the brain have nerves which are probably vaso- motor, though this is denied by some.
406
DISEASES OF THE NERVOUS SYSTEM.
Local Vascular Supply of the Central Part of the Brain.
The detailed facts of this supply have recently been carefully studied by Dr. C. E. Beevor.
The caudate nucleus is supplied by the anterior and middle cerebral central arteries.
The lenticular nucleus by the anterior choroid (part of its internal and external segments), by the anterior cerebral (caudate nucleus and external and middle segments of lenticular), by the middle cerebral (caudate nucleus and middle and external segments of lenticular).
The optic thalamus, by the posterior communicating central branches (external nucleus) and the posterior cerebral.
The corpora quadrigemina and geniculate bodies are supplied by the posterior cerebral.
The red nucleus by the posterior cerebral, and the subthalamic region by the posterior communicating.
The internal capsule by the anterior cerebral and middle cerebral for its anterior part, by the posterior communicating, anterior choroid and the middle cerebral for its posterior part. None of the thalamus is supplied by the middle cerebral and none of the internal capsule by the posterior cerebral. All direct thalamus lesions must be posterior cerebral, or in minor degree posterior communicating. No direct cap- sular lesions would come from the posterior cerebral (Beevor).
The cortical areas of the brain supplied by the anterior, middle and posterior vary somewhat ; the three chief systems anastomose at the parts where one region joins the other (Duret, Beevor). The cortical arteries do not anastomose with the central or ganglionic arteries which latter supply the centrum ovale.
The blood of the convex and mesial cerebral surface, flowing up from the base, leaves the capillaries and enters veins. Thence it still passes upward, and for the most part enters the longitudinal sinus. The most of the vessels enter the posterior portion of the sinus and in a direction forward and upward; i. e., against the current in the sinus. The course of the blood-current is, therefore, opposed both to gravita- tion and to the venous flow.
The veins of the cerebrum are : i, the superficial cerebral ; 2, the deep cerebral veins; and, 3, the cerebral sinuses. 1. The superficial cerebral veins are venae comites. Those on the convex and mesial surfaces empty chiefly into the superior longitudinal sinus, as described ; those on the basal surface and temporal lobes empty into the cavernous and lateral sinuses. These veins have no valves, and their walls are very thin and without muscular fibres. 2. The deep cerebral veins, or venae Galeni, receive the blood from the lateral ventricles and from some of the central arteries supplying the basal ganglia. They empty into the straight sinus. These deep or central veins when compressed lead to stasis and internal hydrocephalus. 3. The cerebral sinuses are fifteen in number. The important ones are the superior and inferior longitudinal, the straight, the lateral, the occipital, the cav- ernous, and the superior and inferior petrosal. They carry blood for the most part in a direction from before backward, and convey it eventually to the internal jugular.
Most of the blood of the convexity and mesial surface must pass
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
407
into the longitudinal sinus, but there is a slight connection of some of the veins with the superior petrosal and straight sinuses. The superior longitudinal sinus also communicates slightly with veins of the scalp and with the facial vein. Some of the blood from the mesial surface also goes to the veins of Galen.
On the whole, however, the system of the convex and mesial cere- bral surface is a close corporation, the blood having to pass into the superior longitudinal sinus and torcular Herophili, where it meets that of the straight and occipital sinuses, and flows forward through the lateral sinuses to the internal jugular. The circulation of the basal sur- face is less isolated. All the basal sinuses communicate with each other freely, and there are slight communications between the veins of the scalp and the cavernous, lateral, and inferior petrosal sinuses. It is safe to tie any of the sinuses, except the lateral and the posterior part of the longitudinal. The cerebellar veins, superior, inferior, and lateral, empty into the straight, the lateral, and superior petrosal sinuses. None of the cerebral veins or sinuses have valves.
The pressure of the blood as it goes through the external carotid is about 150 to 200 mm. of mercury. The pressure of the blood in the cranial cavity is much less (from o to 50 mm. of Hgr. Hill). The pressure in the central arteries is greater than that of the cortical. The pressure of the venous blood is very slight and is ordinarily the same as that of the cerebrospinal fluid.
The cerebral arteries have nerves, but it is not proved that they are vaso-constrictor and vaso-dialators. The prevailing opinion is that the splanchnic nervous system regulates the blood current of the brain. When this is depressed, blood flows into the abdominal viscera and we have cerebral anaemia, and vice versa.
Both arteries and veins are more delicate than the extra cerebral vessels.
Except in gray matter, the brain is not a very vascular organ, but this gray tissue ranks in richness of blood-supply with the lungs and liver. The amount of blood in the brain at any one time is only about one to two per cent, of the total blood in the circulation, or about four ounces (Ranke).
The diameter of the common carotids is 6.7 mm. (Thorne), that of the subclavians 6.2 mm., that of the internal carotids 4 mm., and that of the vertebrals 3.55 mm. (Gerhardt).*
The Functions of the Brain — Cerebral Localization. — The brain is the seat of conscious intelligence and mental activity. It has also control and direction of voluntary movements, it is the seat of instinctive acts, and it regulates in a measure the vasomotor, trophic, and secretory mechanisms of the body.
The Prefrontal Lobes. — The prefrontal lobes, or that part of them which is in front of the precentral convolution, are concerned with the higher association processes and memories. The most anterior portion, according to Campbell, though of late development is poor in cortical
* J. Crichton Brown gives the last two diameters 2.8 and 2.2 mm. respectively.
408
DISEASES OF THE NERVOUS SYSTEM.
cells. In the posterior part are centres for movements of the head and eyes, and in the left third frontal is a centre for speech or articulation memories. Injuries of the prefrontal generally lead to changes of character, indicated by peevishness and irritability of temper, mental enfeeblement, lack of power to concentrate the mind or to control the acts or emotions. The left lobe is said to be more important psychically.
The Central Convolutions. — This part of the brain is called the sen- sori-motor area, because it is concerned in the receipt and production of nervous impulses which cause voluntary motions of the body.
Fig. 192. — Localization of functions of the cortex of the brain. Median surface.
Certain parts of this area are in relation with certain groups of voluntary muscles on the opposite side of the body. These areas preside not so much over single muscles as over those groups of muscles which act together in producing definite, purposeful acts. The motor area proper is confined to a narrow space on the posterior surface of the precentral convolution extending down to the bottom of the fissure of Rolando. It is co-extensive with deposits of the giant cells of Betz. (Sherrington and Greenbaum). The lower part of the central con- volutions, known as the central operculum, is a centre for movements of the larynx, mouth, tongue and face. This area reaches to the lower bend on genu of the fissure of Rolando, opposite which is the centre for the neck. Above and occupying the middle third of the
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409
convolution are centres for the shoulder, arm, hand and fingers, which extend up as far as the upper genu, opposite which is the centre for the tr_ nk. Above this genu are centres for the hip, leg, toes, the area extending over into the paracentral lobules. The base of the first and second frontal convolutions is the centre for movements of the head and eyes. The exact arrangement of these centres, which has been determined by experiments upon monkeys and other lower animals as well as by clinical and surgical observations on man,
Fig. 193. — Same. Superior surface.
is shown in the accompanying Figs. 192-194. The posterior central convolution is probably the centre for cutaneous sensations.
The various sensori-motor centres are not sharply limited, but lap one over the other, so that the motor area for the forearm, for example, extends over somewhat into that for the shoulder. The corresponding sensory areas are more diffuse, so that it takes a much more extensive destruction of a certain area of the cortex to pro- duce an anaesthesia of the arm than it does to produce a paralysis of the arm. According to Campbell, the strictly motor and sensory
DISEASES OF THE NERVOUS SYSTEM.
areas have closely adjacent to them psychic-motor and psychic-sensory areas, concerning in the higher elaboration of movements, sensations and reflexes.
Bilateral Representation. — Those muscles of the two sides of the body which act together have a double representation in the brain. For example, each group of muscles used in inspiration has a centre in both hemispheres; consequently, when one centre is destroyed no paralysis results, for the reason that the other centre continues its
, rRLCENTRAL cCa /POSTCENTRAL
Fig. 194. — Localization of the functions of the brain. Lateral surface.
work. In the same way some of the muscles of the face, such as those for closing the eyes, have a double representation, and a lesion destroy- ing the centre for the orbicularis palpebrarum on cne side will not usually cause paralysis, because of the continued action of the centre of the other side. The more perfect and habitual the associated action of the muscles of the two sides of the body, the more com- pletely can one centre do the work of its associate. The best examples of the muscles having the double representation are the orbicularis palpebrarum, the muscles of the vocal cords, the muscles concerned in deglutition and in respiration. The muscles of the viscera and
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
411
blood-vessels have no known representation in the cortex of the human brain.
The special sensations have a bilateral representation also; but the more specialized the sense the less can one hemisphere take the place of the other.
Parietal Lobes. — The upper portion of the parietal lobe is the centre or region, for we include both the cortex and its underlying tracts, for association of memories of form, cr more properly the perception of the form. When destroyed there is astereognosis or loss of percep- tion of form; and if with it there is some involvement of the zone of
Fig.
195. — Localization of the functions of the brain cortex, showing the psychic areas. (Campbell.) Lateral surface.
language, there is asymboly ; that is, an inability to tell what the object is even if the form is recognized. Lying anteriorly in the lower part of the parietal convolution below the parietal fissure, is an area for memories of muscular movements, and when this is injured there is ataxia, or loss of sense of position. The whole parietal lobe seems to have to do with association processes connected with the elabora- tion of sensations. It is a percipient or, as Mills calls it, a concrete memory centre, not a sensory centre. Even the ataxia which occurs here is perhaps more a loss of perception than a pure sensory loss.
The stereognostic and ataxic centres in it are not yet sharply and satisfactorily localized and the diagram given is likely to require change.
412
DISEASES OF THE NERVOUS SYSTEM.
In its posterior and inferior part, the angular gyrus, we have part of the zone of language, especially that concerned in memories of written words (reading centre). Near this is, perhaps, a centre for memories of the uses of things (Praxic centre).
V1SU0-SENS0RY
Fig. 196.— Localization of the functions of the cortex of the brain, showing the psvchic areas. (Campbell.) Superior surface.
Occipital and Temporal Lobes—Centres of Special Sense.— The special senses have two centres— the primary and the secondary. The primary centres are connected with the ganglia at the base of the brain; the secondary centres are situated in the cortex.
The primary centre for vision is in the posterior part of the optic
ANATOMY AND PHYSIOLOGY OF THE BRAIN. 413
thalamus, the external geniculate bodies, and anterior corpora quadri- gemina. The secondary centre is situated in the occipital lobe, and par- ticularly upon its mesial surface and ab jut that part of the cuneui known as the calcarine fissure. Each occipital lobe is the centre for visual impulses from the corresponding half of the retina of each eye; for example, the left occipital lobe is the centre for vision of the left half of the retina of each eye. This relation is shown in the diagram (see Optic Nerve). Total destruction of both occipital lobes, or even of a considerable part of them if the destruction involves the median
Fig. 197. — Same. Median surface.
surface, will cause blindness. Destruction of one lobe causes only half-blindness or hemianopsia.
The primary centre for hearing is in the posterior tubercle of the corpora quadrigemina and the internal geniculate body. The second- ary centre is in the cortex of the first and second convolutions of the temporal lobe. Destruction of one temporal lobe causes some deafness in the opposite ear. This deafness, however, is not complete because the sense of hearing has a bilateral representation; each ear, in other words, sends fibres to the temporal lobes of each side, al- though more fibres cross over than go to the lobe of the corresponding side. The consequence is that the loss of one temporal lobe is in a measure supplied by the other (vide Aphasia).
414
DISEASES OF THE NERVOUS SYSTEM.
The primary centre for smell is in the olfactory lobes. The second- ary centre is probably in the anterior part of the limbic lobe, the uncus and in part of the hippocampal convolution. Whether the tracts for the sense of smell are connected with the optic thalamus or other ganglia is not definitely known.
The primary centre for taste is not known, but sensations of taste may connect with the optic thalamus before passing into the second- ary centre, which is in the hippocampal convolution.
Centres for Special Associations.— -There are certain classes of sen- sations and perceptions, simple in character and frequently repeated, so that they finally get to be used almost automatically in their work. These impressions relate to the use of the muscles in speech, in writing and in gesture language; also to other frequently repeated purposeful movements of the limbs. The muscular movements in writing and speaking are so often repeated that certain areas in the cortex are set apart for the memories of these processes, memory being simply a revival or re-association of previously registered impressions. The visual sensations and the ideas elaborated from them, which are frequently repeated in learning to read, have also a centre which is set apart for them. This forms a centre for the visual memories of language. In the same way there are auditory sensations and ideas elaborated and so frequently repeated as to be used automatically in acquiring language. These are stored up as auditory memories. We have what may be called motor memories connected with speech and gesture. These special memories have been found to have a certain localization in the brain. The centre for the memories of the articular movements of speech is" in the posterior part of the third left frontal convolution; the centre for the memories of the movements of writing is not perfectly known, but is thought to be at the posterior part of the second left frontal convolution. The centre for the memories of gesture language is unknown. The centre for the memories of ordi- nary co-ordinate movements is probably in the inferior parietal lobule. The centre for the visual memories of written language is in the angu- lar gyrus, extending backward from there into the occipital lobe. The centre for the auditory memories of spoken language is in the posterior part of the first and the corresponding upper part of the second temporal convolution. In right-handed people all the memory centres are in the left cerebral hemisphere; in left-handed people they are in the right hemisphere. The centres for the memories and associa- tions connected with language together form a large area, known as the zone of language. The destruction of these memory centres pro- duces different forms of aphasia, as will be described later. In
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415
addition to that, disturbances in these centres are produced by lesions which cut off the associating fibres connecting these centres with each other or with motor or sensory centres proper.
The Centrum Ovale, Corpus C alio sum, and the Associative Functions of the Brain. — The different parts and centres of the brain are connected together by the associating tracts and with lower levels by the pro- jection fibres. The simpler and less developed centres of the two halves of the brain are closely connected by fibres that run chiefly in the corpus callosum. The more highly specialized and less simple in function a centre, the less close is its commissural connection and the more independent is one half of the brain from the other. Thus the centres for the movements of the thorax in respiration are closely bound with each other; those for the purposeful movements of the hands less so; those for receiving visual impressions are almost independent; and the centres for language associations which are still more highly specialized, are practically entirely independent. We infer that the higher mental functions, therefore, work either in one cerebral hemi- sphere or in the other, and that the two halves of the brain do not co operate with each other in much of the higher intellectual work. This, however, may not be true for the very highest mental processes, though persons have lived and shown fair intelligence without a callosum.
The corpus callosum is the great commissural tract connecting the two cerebral hemispheres and their respective centres. The ante- rior commissure does some of the same work, being more specially con- nected with the function of olfaction. The posterior commissure has comparatively few bilateral connecting fibres, its function being more to connect the thalamus with the cranial nerve nuclei and other centres below.
The Corpus Striatum. — This ganglion is in relation with the cerebral cortex slightly, with the internal capsule slightly, with the thalamus and subthalamic nuclei, and probably through these with the cerebellum and with nuclei in the pons. It is also in connection with fibres that come up from the muscle-sense tract, in the spinal cord. It seems most intimately connected with the thalamus and subthalamic ganglia. Its functions are therefore probably connected with supplementing autonomic movements. Destruction, however, of this ganglion in the human brain produces only at times motor and vasomotor dis- turbances, and local lesions of it cannot be diagnosticated. It is there- fore called clinically a latent region.
The Thalamus Opticus. — The thalamus is in relation by its projec- tion fibres with the frontal, parietal, occipital, and temporal cortex. The fibres that go to the occipital cortex are connected with the optic
DISEASES OF THE NERVOUS SYSTEM.
tract, and have to do with the function of vision. The fibres that go to the temporal lobe are connected with the auditory tract, and have to do with the function of hearing. The optic thalami have a doubtful relation to the expression of emotions. Lesions of the posterior part of the thalamus will produce hemianopsia and central pain. Other than this, lesions of the optic thalamus produce no definite symptoms which enable us to make a local diagnosis, except through injury of neighboring parts. Disturbances of hearing have not certainly been traced to lesions in the thalamus. It is probably a primary centre for sensations of touch, muscular sense, and perhaps for smell and taste, but no definite facts in human pathology have as yet satisfactorily proved this. Lesions of the thalamus sometimes produce various forms of mobile spasm, but these are generally attributed to irritation of the fibres of the internal capsule, which go close to it. Hence, aside from pain and disturbances of vision, the optic thalamus also must be considered clinically a latent region.
The Corpora Quadrigemina. — The anterior tubercles of the corpora quadrigemina, together with the external geniculate bodies, form part of the primary centres of vision. The anterior tubercles, however, have to do chiefly with reflex movements of the pupil and the ciliary muscles. The posterior tubercles of the corpora quadrigemina and the internal geniculate body are connected with the auditory nerve, and have to do with reflex movements associated with hearing and space sensations. They also appear to receive some fibres from the cerebellum; their injury or disease produces some disturbances in equilibrium and possibly in hearing. Owing to the fact that the nuclei of the third nerves and the red nuclei lie beneath the corpora quadri- gemina, lesions of these latter produce irritations and paralyses of the third nerve, disturbances in equilibrium, and forced movements. Lesions in this neighborhood sometimes cause somnolent and stuporous states.
The red nuclei are connected with the anterior cerebellar peduncles on the one hand and with the lenticular nucleus and optic thalamus on the other, and are concerned in securing equilibrium and the adjust- ment of the body in space.
The Cerebellum. — It receives impulses from the cerebral hemispheres via the indirect motor tract. These impulses go to the cortex of the lateral lobes. It also receives impulses from the vestibular and to some extent from the other sensory cranial nerves. These impulses go to the cortex of the vermis.
The cerebellum also receives impulses from the spinal cord via the direct (dorsal) cerebellar tract and the column of Gowers (cerebral
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
417
cerebellar tract), but not from the posterior columns. These impulses go to the cortex of the vermis. The efferent tracts of the cerebellum are those to the cerebrum and those to the midbrain, subthalamic region and spinal cord. The cerebello-cerebral fibres leave the cor- tex of the lateral lobe, go to the dentate nuclei, thence through the anterior cerebellar peduncles, crossing, in most part, to the red nucleus and thalamus and the central gray nucleus tecti, thence to the cortex of the brain. The cerebello-spinal fibres start from the cortex of the vermis, then to the roof and emboliform nuclei (intrinsic cerebellar nuclei) , thence to the nucleus of Deiters mainly (paracerebellar nuclei of Horsley), thence to the spinal cord in the anterolateral tracts, and anterior cornua. Some cerebello-spinal fibres go from the red nucleus and tectal nucleus to the cord. Hence we have vestibular-spinal, the rubro-spinal and the tecto-spinal tracts convey- ing impulses down to the cord, originating mainly at least in the cere- bellum. Horsley has shown that the cerebellar cortex is a receptive organ. Stimulation of it causes no movements. Its efferent impulses go to the intrinsic nuclei of the cerebellum. Stimulation of these causes tonic movements of the head. Stimulation of the paracerebel- lar nuclei (the Deiters) in the pons cause also tonic movements of the body. It is when these intrinsic and paracerebellar nuclei are irri- tated that we get cerebellar fits. L. Hill and others have shown, as Jackson first averred, that the cerebellum causes tonic fits, the cerebral hemispheres clonic fits. There is a nervous circuit between the cere- brum, brain axis, cerebellum and spinal cord. The cerebellum has thus the function of securing the higher automatic and psycho-reflex move- ments, and through its" further relations with the space-sense nerve (eighth) of enabling us to keep our equilibrium and maintain our relations in space. It also maintains a certain tonus of the muscles. The vermis or median lobe is the part which in man is most impor- tant in doing this work. The lateral lobes are concerned in acquiring new movements and dexterities. Lesions of the lateral lobes or hemispheres produce few direct symptoms, and they are called latent regions. Lesions of the cerebellum produce disturbances in equi- librium, vertigo, constrained attitude and muscular weakness, forced movements, and a peculiar form of inco-ordination in gait which is known as cerebellar ataxia. Lesions of the middle peduncles produce forced movements also, the forced movements being either toward or away from the side of the lesion, according as it is an irritating one or a destructive one. It is irritation of the intrinsic and paracere- beller nuclei which cause these movements as well as the "fits." The pons Varolii contains some of the cranial nerve nuclei and col- 27
4i8
DISEASES OF THE NERVOUS SYSTEM.
lections of nerve-cells which are connected with fibres from the cerebral cortex on the one hand and the cerebellum on the other. It also contains the long tracts of nerve-fibres that pass from the cerebrum down through into the medulla and spinal cord and transverse tracts of fibres which connect the two hemispheres of the cerebellum. Lesions in it cause disturbances in function of the cranial nerves and of the motor, sensory and commissural tracts.
The medulla oblongata contains centres of the cranial nerves, and in it also are various reflex and automatic centres controlling and regulating the vasomotor system, respiratory and cardiac rhythm, visceral movements and secretion.
The olivary bodies are connected with the cerebellum, basal ganglia, and with the spinal cord. When injured, disturbances of equilibrium and co-ordination occur.
The Latent Regions of the Brain. — -There are certain parts of the cerebral cortex destruction of which and irritation of which produce no special and distinctive phenomena in man. These are the greater part of the temporal lobe of the right side and a portion of the temporal lobe on the left side. A part of the inferior parietal lobule also may be regarded as a latent region. The frontal lobe we have already spoken of as being concerned with certain mental functions, but lesions here often produce no symptoms, and may be to a certain extent re- garded as latent. These latent regions are called by Flechsig the higher or associative centres. The corpora striata, optic thalami, portions of the centrum ovale, and the two lateral hemispheres of the cerebel- lum are latent areas.
Brain Weight. — The average weight of the male brain is 1,358 gm. ; that of the female, 1,235 Sm- The weight varies with age, sex, race, and intelligence, and with a number of other factors. The average weight of the brain at birth is 327.8 gm. ; the brain grows rapidly until the age of four, then more slowly until the age of seven, then very slowly up to the age of sixteen to twenty. At about the age of forty-five in man and fifty in woman it begins to lose weight slowly, and at the age of eighty or over it has lost about 1 20 gm. (4 oz. ) . The brain of man weighs absolutely about nine per cent, more than that of woman. Relatively to the body weight, the brain weight of man is about 2 per cent. ; that of woman is very little less. The sexual difference is extremely small.*
The brain weighs more in the civilized races, and more in certain of the civilized races than others; the brains of English, German, and Scotch weigh more than those of French, Italian, and Russian. Some of the African and Australian tribes have the smallest brain, the average negro brain weighing 1,250 gm. When a brain weighs less
*J. C. Brown finds that after making all allowances, woman's brain weighs about one ounce less than man's.
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
419
than 1,130 gm. in man or 990 grn. in woman, it is called a micro- cephalic brain; if the weight is above 1,490 gm. in man or 1,345 £m- in woman, it is called a megalocephalic brain.
Brain weight has a certain relation to intelligence, which is not, however, an absolute one. Among a hundred men of more than average intelligence, the percentage of large brains would be about 25, whereas the percentage of large brains among persons of ordinary or low intelligence would be more than 4 or 5. In estimating the im- portance of brain weight, one must consider the height, the weight or
Fig. 198. — Showing the points at which the sections are made, a, b, horizontal line from base of frontal to base of occipital lobe; A, vertical section through middle of third frontal convolution; B, through operculum o; C, through superior precentral and lower end of Rolandic fissures; D, through rc and posterior end of Sylvian fissures; E, through angular gyrus and anterior occipital fissure; F, through parietooccipital fissure. B should be about half-way between A and C; E half-way between D and F. (See Figs 199-201.)
volume of body, muscular mass, and superficial area; these are called the somatic factors. The following formula has been devised bv Snell for estimating the mental power of different animals.
In this formula P represents the psychical factor or the amount of intelligence, H the brain weight, K the body weight, S for the somatic factor. The somatic factor has been estimated to be for mammals about 0.666. Applying this formula, we find that, expressed relatively the intelligence of man equals 0.87; woman, 0.86; the ape, 0.42; the rabbit, 0.59; the birds from 0.167 to 0.09.
The relative weight of different parts of the brain is about as
420
DISEASES OF THE NERVOUS SYSTEM.
follows: frontal lobes, 28 per cent.; parietal lobes, 36 percent. ; occipi- tal, 10 per cent.; temporal, 13 per cent.; lobus caudicus or island of Reil, 9 per cent. ; pons, ih percent. The cerebellum weighs about one-
Fig. 199. — Section through line A, Fig. 198.
Fig. 200. — Section through line B, Fig. 198.
eighth as much as the cerebrum. The proportion of the gray to the white matter in adults is 60 to 40 (Vierordt).
Preserving and Cutting the Brain.
The brain should be placed in a gallon of a z\ per cent, solution of bichromate of potassium. This must be changed daily for a week, then
ANATOMY AND PHYSIOLOGY OF THE BRAIN.
421
twice weekly for a fortnight; then it should remain in the solution for three or four months, a few crystals of thymol being added. After about three months place the brain in 95 per cent, alcohol. In a few
Fig. 201. — Section through line C, Fig. 198.
Fig. 202. — Section through line D, Fig. 198.
days it will be ready for cutting. Or for permanent or temporary use the brain may be placed in a 4 per cent, solution of formalin. Later it may be changed to alcohol or Miiller's fluid.
42 2 DISEASES OF THE NERVOUS SYSTEM.
In cutting the fresh or preserved brain for the purpose of locating gross lesions, remove the pons, medulla, and cerebellum, place the brain on its base, and make sections in accordance with the directions (Fig. 198). The sectional views exposed are shown in the following series of cuts, which are based upon those of Exner.
E
Fig. 203. — Section through line E, Fig. 198.
F
Fig. 204. — Section through line F, Fig. 198.
CHAPTER XVIII.
DISEASES OF THE BRAIN AND ITS MEMBRANES.
General Symptoms.
It will add to the intelligibility of descriptions of brain diseases and their symptoms if one first makes himself familiar with certain general symptoms that underlie more or less nearly all organic dis- orders of this organ. Symptoms due to disease of the brain may be placed in four classes: first, general symptoms of brain irritation; second, general symptoms of brain pressure; third, symptoms of focal irritation or destruction; and, last, those due directly to the patho- logical process itself.
The symptoms of brain irritation are headache, vertigo, vomiting, photophobia, mental irritability, insomnia, peculiar feelings of fullness and pressure about the head, noises in the ears or in the head, tender- ness about the scalp, and in severe cases rigidity of the neck, convulsive symptoms and delirium.
The symptoms of brain compression are divided into four stages:
There is, first, a venous stasis with consequent headache and mental dullness, and perhaps some focal symptoms.
Second, there is capillary anaemia with increased headache, restless- ness, vertigo and disturbed sensorium, delirium, unnatural sleep. There is some cyanosis and oedema of the optic disk. A slowed pulse and rise of blood pressure.
Third, there occurs an increased capillary anaemia and pressure phenomena from the medulla, with a slow bounding pulse, high blood pressure and irregular respiratory rhythm. The patient is cyanotic and stuporous with snoring respiration, the optic disk more involved. In the terminal stage the coma deepens, the medullary centres become paralyzed with rapid pulse, lowered blood pressure and failure of respiration (Kocher).
The symptoms of brain irritation are associated with a hyper- aemia or a venous stasis and anaemia. The symptoms of brain com- pression may be associated with anaemia or oedema, tumors, blood- clots and foreign bodies. Often in states of malnutrition in wThich the brain is impoverished the symptoms resemble much those of com- pression. Pressure symptoms and irritation symptoms often lap one into the other and they cannot always be sharply distinguished.
423
424
DISEASES OF THE NERVOUS SYSTEM.
Focal symptoms depend almost entirely upon the location of the particular lesion. If it is in a motor area, focal symptoms of irrita- tion would be spasmodic phenomena, such as convulsions. If the lesion were destructive, the symptoms would be those of paralysis or anaesthesia.
The symptoms due directly to the pathological process itself may be very slight. Thus in case of a tumor of the brain the symptoms are mainly caused by pressure, irritation, and local disturbance of certain special parts of the brain. In suppuration, however, the process itself may produce general symptoms such as are associated usually with sepsis— chills, irregular fever, mental hebetude, prostra- tion, emaciation and sweats.
Among the symptoms produced by focal lesions there are a few which deserve some preliminary general study, because they may be caused by lesions of very different kinds and occur consequently in very different forms of diseases. Those symptoms which we wish particularly to study here are hemiplegia and aphasia. These repre- sent the two great dominating symptoms pertaining on the one hand to motor disturbance and on the other hand to sensori-motor disturbance.
Hemiplegia. — Hemiplegia is a paralysis of one-half of the body involving the side opposite the lesion. The face, arm, and leg are usually all paralyzed; the arm most, the leg next, the face least. Hemiplegia may be either acute in onset or slow and progressive. Acute hemiplegia is the result usually of hemorrhages and soften- ings of the brain, more rarely of inflammations and injuries. Pro- gressive hemiplegia begins gradually, as its name implies, and slowly increases until the height of the disease is reached. It is usually caused by tumors growing in one side of the brain, but it may be caused by a slowly developing patch of sclerosis, which sclerosis may be in turn only a part of a multiple sclerosis. Further description of the peculiarities of hemiplegia will be given under the head of Special Diseases of the Brain.
Aphasia. — -Aphasia is a disorder of the faculty of language; and it has a number of varieties, in accordance with the particular part of the brain involved and the particular portion of the mechanism of this faculty that is destroyed. By the faculty of language we include the processes by which we hear, see, and at the same time appreciate the meaning of symbols. It includes also the faculty of expressing to others by voice, writing, or gesture our ideas. It has, therefore, a receptive side and an emissive side. We may have lesions in the brain which destroy that part of the language faculty concerned in our power of seeing and understanding written words or the gesture language.
DISEASES OF THE BRAIN AND ITS MEMBRANES. 425
In reading understandingly one sees certain words ; these words revive certain visual memories connected with past perceptions. Thus one sees the word "book;" this suggests to him past memories of form, color, tactile and other sensations associated with the past perceptions of books. There is a certain centre in the brain where these visual memories for letters and words are located. When this centre is destroyed the memories are destroyed and the word "book" or any other written word conveys no meaning. The patient can spell out the letters, he can see the letters, but he cannot read any more than if he had never been taught. The condition is known as alexia or word blindness. Again a person may have learned to associate certain gestures with definte ideas, as the motion of carrying a glass to the mouth with that of drinking, or the motions of using a knife and fork with that of eating, or the motions of the deaf-and-dumb alphabet with certain words and ideas. These memories of gesture language are located in certain regions, and when they are destroyed the patient is no longer able to understand gestures or the sign language. This condition is known as sign blindness. When a person is not able to understand the significance or uses of things about him, he has apraxia. Apraxia, sign blindness, and alexia all come under the general head of mind blindness, because, though the patient can see, he does not under- stand what he sees. A person hears certain words, as, for example, the word "knife. " This conveys to him a certain idea of the form, color, and other properties associated with knife. The memories associated with the auditory perception of different words are stored up in a certain locality which is the centre for auditory memories. When this centre is destroyed the person hears spoken words, but they convey to him no meaning. All that is said to him sounds as if it were in a foreign language: he hears, but he does not understand. This condition is known as word deafness. So much for the receptive or sensory side of language.
In communicating our ideas, we speak, write, and make gestures. In speaking we make use of the organs of articulation, and this use involves the fine adjustment of a delicate muscular apparatus. In the act of expressing ideas we have to bring into play the memories of the past muscular movements of this articulatory mechanism. These movements were learned by a slow and painful process during infancy. After the power of speech is acquired, the mechanism works readily and almost automatically, because we only have to send a stimu- lus to the centre which presides over the stored-up memories of the impulses to innervate properly the mechanism of speech. There is, therefore, a centre for the memories of the movements of articulation—
426
DISEASES OE THE NERVOUS SYSTEM.
a centre which is, of course, closely connected with the motor areas that directly innervate the larynx, pharynx, and oral and facial muscles. When a lesion destroys this centre for speech memories, a person is unable to reproduce the words necessary for expressing an idea ; for example, he sees a knife, he knows what it is, but the memory of the motions neces- sary to express the word "knife" is gone. To him it seems that the name is gone, and that is the common way of expressing it. He cannot say the word ** knife." The patient may wish to express the idea of pain. He feels the pain, he knows that he has pain, but he cannot revive those motor memories which are concerned in expressing the word "pain;" he cannot tell, therefore, in words what is the matter with him. When a person is thus troubled, he is said to have a form of motor aphasia for which the particular name given is aphemia. In the same way there is a centre for the memories of the muscular movements concerned in writing; and when a lesion destroys this centre the patient is unable to write, though he may be able to speak. This condition is called agraphia. There is a centre, less well defined, for the memories of the movements used in gesture language, and when this is destroyed the person is unable to express his ideas by gesture or sign language. This condition is known as amimia. A loss of memory of musical expression is called aynusia. In some cases, patients are able to speak and write, but they skip words, repeat often, and talk confusedly. There is here a lesion of the tracts associating the language centres, and the condition is called conduction aphasia, while to his stumbling speech the term paraphasia is given.
In attempting to classify these various aphasic conditions we group together as much as possible those symptoms which we know are related to rather definite areas of the brain. The divisions are based on symptoms, yet each symptom group has an anatomical seat which in many cases can be exactly determined.
The following are the principal forms of aphasia and their ana- tomical localization:
In the examination of a case of aphasia, the questions to bring out the following points should be put to the patient :
To test his power of expression:
1. Can he speak voluntarily or use ejaculations expressive of some emotional state?
2. • Can he answer questions?
3. Can he count or repeat letters of the alphabet?
4. Can he repeat words? If he can talk, does he talk coherently, or is it a jargon, or does he skip words now and then as he talks?
5. Can he hum tunes'
DISEASES OF THE BRAIN AND ITS MEMBRANES. 427
6. Can he write voluntarily -or to dictation? Can he copy?
7. Can he indicate his wants by gestures? To test his power of understanding language :
8. Does he understand the words spoken to him, or gestures made to him?
9. Can he name things seen, heard, felt or can he name abstract things, like darkness, beauty, sorrow?
10. Can he recall to his mind objects named to him? That is, if the word "poison" is spoken, does he know what it means?
11. Can he read silently or aloud?
12. Does he know the uses of things about him, such as how to use a knife and fork, or a pencil, and what is his general intelligence?
By a series of questions of this sort we can make a diagnosis of the psychological quality of his speech defect, and say that he has "audi- tory" aphasia, or "motor" aphasia, or a "visual" aphasia, and so on.
The physician, however, can ignore this kind of diagnosis, to a large extent, and he had best try first to determine the anatomical location of the aphasia, and arteries involved in the lesion. By the method of questioning which has just been laid down, the patient is discovered to have certain peculiar defects of language, and, in accordance with these defects, the physician is able to assign the trouble to one or another portion of the brain.
1. Thus, in one very common form of speech disturbance, we have a fronto-capsular lesion, i.e., sl lesion injuring the left third frontal convolution and usually extending back so as to involve the interior capsule somewhat. ' Here, the patient has usually hemi- plegia but no sensory disturbance, and his intelligence is quite good. The main characteristic of his speech defect is that of aphemia, that is, a complete inability to say a word, or, at least, only one or two simple words. With this mutism, there is also usually a loss of ejaculatory speech, and of responsive speech, of counting or association speech, and of ability to repeat words that are spoken. Yet, though thus completely aphemic, the patient can write and read well, and understand spoken language perfectly. The lesion here is in the third left frontal convolution, extending, perhaps, somewhat into the lenticular zone (Fig. 206), and due to involvement of one of the ganglionic arteries, which does not supply much of the corpus striatum; sometimes there is an involvement of the cortical arteries. These lesions are very rare, because the third frontal convolution is not supplied altogether by a single artery but by several. Occasion- ally, however, this portion of the brain is rather extensively involved, and there may be with some hemiplegia, also evidences of difficulty in
428
DISEASES OF THE NERVOUS SYSTEM.
understanding spoken language. Sometimes the pure fronto-cortical lesion causes an aphemia, like this described, which is temporary, be- cause the lesion is so small. This form of aphasia is sometimes known as "pure motor" aphasia or "pure motor subcortical" aphasia.
2. The second type of aphasia is that known as "occipital" or " parieto-occipital " aphasia, known as "pure alexia."
In this group of cases, the patient can talk well, and understand, and write well, but he cannot read, except a few words or letters, and he cannot copy well. He has always a hemianopsia, and sometimes the alexia is wrongly attributed to this. Usually there is with it some hemiataxia or hemianaesthesia or hemiparaesthesia, and there may be a very slight temporary hemiplegia. The intelligence of the patient is good. These cases are quite rare also. They are due to a lesion in the occipital lobe, extending sometimes into the parietal. This lobe of the brain is supplied by the posterior cerebral artery. When lesions of this artery occur, they involve the visual centre often, and also the so-called association or visuopsychic centres concerned in reading. These are situated in the angular and in the second occiptal gyri. While the lesion is usually due to involvement of the posterior cerebral, alexia may be caused also by lesions of the Sylvian artery, since it supplies some of the angular gyrus, but in this case, we get some injury of the temporal cortex, and an alexia which is complicated with other symptoms.
3. A third group of aphasias is known as a "temporal or "parieto- temporal" or the "sensory aphasia of Wernicke." Here, the patient is found to have no hemiplegia, or only a temporary and slight one, but his power of coherent speech is seriously injured. He has some voluntarily speech, and responsive speech, and may use words very fairly, even loquaciously, but he talks nonsense on account of the con- fusion, repetition, and misplacement of words. He has, in other words, paraphasia or jargon. He cannot name objects shown him, and usually not objects that are heard by him, or felt, or smelt, or tasted. This constitutes a symptom known as "anomia," or "optic aphasia." He can read a little, but very poorly, and write a little and copy, but this is all done very badly. In other words, he has some alexia and agraphia. He cannot understand questions or simple direc- tions, or, if he does understand simple directions, he cannot understand complicated ones, and there is, apparently, some loss of general intelli- gence. Patients act sometimes rather childishly and emotionally. Perhaps the dominant symptom may be said to be this inability to understand, and this confusion and jargon, or paraphasia of speech. With this speech defect there may be, as already stated, a temporary
DISEASES OF THE BRAIN AND ITS MEMBRANES. 429
or slight hemiplegia. There is usually some hemianaesthesia or ataxia or astereognosis, or all three of them. There is sometimes a hemi- anopsia, which may not be permanent. Such persons improve a good deal in their intelligence, but rarely get entirely normal, and they have to be watched rather carefully. They do not show symptoms of any true dementia. The lesion, in these cases, is in the auditory and the auditory psychic area of Campbell, that is to say, in the posterior two-fifths of the first temporal convolution, extending, in severe cases, into the parietal lobe or into the second temporal. There is a cutting off, to some extent, of the association tracts between the audi-
Fig. 205. — Lesion of Sylvian artery, and softening of temporo-parietal region, with
aphasia. (Spiller.)
tory psychic and the visual area. The artery here involved is, of course, one of the terminal branches of the Sylvian, or perhaps the trunk of the Sylvian itself. In this latter case, when the lesion is extensive, the speech is apt to be very confused, and almost purely jargon, and there is more likely to be some hemiplegia. Occasionally, a very small lesion of the first temporal alone occurs, and here the patient is simply unable to name objects seen, and often objects that are felt, or heard, or smelt, or tasted. In this case, we have what is called a "pure anomia," or inability to name things. Another term for it is " optic aphasia."
4. The fourth group of aphasias is the " fronto-lenticular " or "len-
43o
DISEASES OF THE NERVOUS SYSTEM.
ticular zone" aphasia and "mixed" aphasia. This is the most com- mon of all the aphasias. We here find a very extensive aphemia, that is, inability to talk voluntarily or even to say any words. There is a good deal of inability to read ; reading is always imperfect — that is, there is some alexia and always also, some difficulty in understanding, at least, complicated sentences; that is, in other words, there is some mind-deafness. There is always a very decided hemiplegia, but rarely any sensory symptoms. The lesion here involves the branch of the middle cerebral that supplies the corpus striatum, the internal cap- sule, and the fibres converging into it from the second and third frontal
Fig. 206. — Showing the zone of language and the localization of the four types of aphasia. I. Frontocapsular aphasia, characterized by aphemia. II. Parietooccipital aphasia, characterized by alexia. III. Temporal or temperoparietal aphasia, character- ized by anomia, some mind deafness, and paraphasia. A small lesion may cause only optic aphasia. IV. Frontolenticular aphasia, the common type of aphasia, characterized by aphemia with much agraphia, alexia, mind deafness, and hemiplegia.
and praecentral convolution. In other words, the lenticular zone is involved and part of The fibres that come from the temporal and frontal areas of speech are destroyed. In some way also, the associa- tion tracts from the occipital lobe must be affected, because of the difficulty in reading.
This form of aphasia, which I call, anatomically, the "lenticular zone" aphasia, is called by Marie, an anarthria plus the aphasia of Wernicke. The accompanying diagram will show the anatomical localization of the four different groups of aphasias. And I am sure that the student and physician, in attempting to make a diagnosis of aphasia, will find the task much lightened by simply turning his at- tention to the effort to locate, anatomically, the area of the brain and the artery involved.
DISEASES OF THE BRAIN AND ITS MEMBRANES.
431
Thus we have the following groups of aphasia :
C.inical Type. Anatomical Seat.
1. Pure motor aphasia. Fron to-cortical aphasia, Pure agraphia. Fronto-cort'cal aphasia.
2. Pure alexia. Occipital or parieto-occipital aphasia. Apraxia.
3. Auditory and optic aphasia. Tempero-parietal aphasia. Anomic aphasia.
(Sensory apnasia of Wernicke).
4. Mixed and conduction aphasias. Lenticular and fronto-lenticular aphasia.
Conduction and Mixed Aphasia. — There are very few cases of pure conduction aphasia. When it occurs there is paraphasia and para- graphia; the patient repeats words over and over in a kind of verbal intoxication, or mixes things so that the speech is almost gibberish. Still he can express himself and can write, read and understand. The lesion is usually in the island of Reil or the convolutions about the fissure of Sylvius. It is a lenticular zone aphasia.
Practically, conduction aphasia is usually mixed with a visual or auditory aphasia.
Malformations of the Brain and its Envelopes.
Congenital malformations of the brain are of little practical im- portance, for in most cases the monsters cannot live and in all cases they are better dead. I shall simply give a brief enumeration of the important forms.
f Anencephaly. Abnormalities I Micrencephaly and microcephaly, of the brain. ] Porencephaly.
[ Absences or malformations of parts, e. g., cyclopia. Abnormalities [ ^rania. . of brain and Meningocele, its invelo es ' -^ncePna^oce'e- s P s* [ Hydrencephalocele.
Anencephaly is always present with acrania. In anencephaly the cerebellum and part of the basal ganglia may be present. In such case the child can live a short time.
Micrencephaly and Microcephaly. — Micrencephaly is a condition in which the brain is only partially developed. If, as is usually the case, the cranium is also abnormally small, it is called microcephaly. It is due, probably, in all cases to an inherent defect in the growth of the brain. Virchow has asserted, however, that there is a form in which the abnormality is caused by a premature growing together of the cranial bones, a micrencephaly being a result of the mechanical condition. An adult cranium whose great circumference measures less than 43 cm. will contain a micrencephalic brain. The normal minimum weight of the adult brain is 960 grams for man and 880 for woman. It should bear the ratio to the body at birth of 14 per cent., and of 2.37 per cent, in adult life (Vierordt).
432
DISEASES OF THE NERVOUS SYSTEM.
Porencephaly is often an artificial condition. It will be described later.
In cyclopia there is an undivided anterior cerebral vesicle; the orbits form a continuous cavity with a single rudimentary eye.
Meningocele is a hernia of the brain membranes, arachnoid, and dura mater through a cleft in the skull. In encephalocele the brain also protrudes. Both these forms occur usually in the occipital region and almost invariably in the median line. In hydrencephalocele there is a sac with fluid contents.
Diseases of the Membranes of the Brain.
The diseases to be considered under this head are anaemia and hyperaemia, inflammation of the dura mater or pachymeningitis and inflammation of the pia mater or leptomeningitis.
Anemia and Hyperemia of the Membranes of the Brain. — Anaemia of the membranes of the brain is a condition that cannot be separated from anaemia of the brain substance, and will be considered in connection with it. Hyperaemia of the brain membrane, so far as it relates to hyperaemia of the pia mater, must also be considered in con- nection with hyperaemia of the brain tissue. Dural hyperaemia, or con- gestion of the dura mater, is a condition which occurs as the result of injuries, sunstroke, and of ceitain infective poisons, especially that of syphilis. The symptoms are those of pachymeningitis of the slight grade, and will be described under that head. They consist mainly of pain, occasional attacks of vertigo, and sensations of fullness about the head. The treatment is that for the beginning stages of a meningitis.
Inflammation of the Dura Mater or Pachymeningitis Ex- terna.— It has been the custom to describe two forms of pachymenin- gitis, the external and the internal. Internal pachymeningitis, or haematoma of the dura mater, is properly a hemorrhagic disorder, and is described under the head of Dural Hemorrhages. A true inflam- mation confined to the internal surface of the dura alone is of extremely rare occurrence.
Pachymeningitis externa is a disease that involves, at first at least, the outer surface of the dura, and is usually of surgical origin and in- terest.
Etiology. — Accidents, injuries, osteitis, caries of the petrous bone in mastoid disease, of the ethmoid bone in ozaena, necrosis, syphilis and erysipelas are the usual causes.
The symptoms are local headache, fever, delirium, sometimes even convulsions and paralysis. In the severe cases the disease has usually extended and involved the pia. Pus is generally formed, and bur-
DISEASES OF THE BRAIN AND ITS MEMBRANES.
433
rows between the bone and dura. The disease is recognized mainly by the discovery of the local cause.
The course is acute or subacute.
The treatment is a surgical one.
Inflammation of the Pia Mater or Leptomeningitis. — In- flammation of the pia mater has the following types: simple menin- gitis due to some infection, epidemic cerebro-spinal meningitis due to a specific general infection, tuberculous meningitis, serous meningitis and syphilitic meningitis.
Most of all these forms of meningitis may be either acute or chronic, the chronic form being usually simply a sequela of the acute.
Acute Simple Leptomeningitis — Etiology. — Acute leptomenin- gitis is always due to an infective process reaching the cerebral mem- branes usually directly from without, but sometimes through the blood. Trauma, and especially acute alcoholism predispose to this. The most common source of infection is disease of the middle ear and mastoid cells. Disease of the frontal sinuses and upper nasal passages; opera- tions on those parts; disease, injuries, and fractures of the cranial bones — are also common causes. Pneumonia is the most frequent in- fective disease in which the pyogenic organisms are carried by the blood. After this come pyaemia, septicaemia, variola, scarlet fever, more rarely endocarditis, empyema, rheumatism, measles, typhoid fever and mumps. Occasionally a brain abscess reaches the surface and sets up a meningitis. Insolation can of itself not cause it. The disease is more frequent in males, and is distributed through all ages of life, though it occurs oftener in the young.
Symptoms. — The symptoms in the various types differ somewhat, but have a general similarity. They are to be broadly grouped into the prodromal, the irritative, the depressive, and the paralytic stages.
Prodromal symptoms are shorter and less marked in simple men- ingitis than in tubercular. The patient suffers from malaise, languor, headache, vertigo, irritability, loss of appetite and vomiting. Of these symptoms headache is the most notable.
In the second stage the dominant symptoms are headache, delirium, rigidity of the neck, hyperesthesia of the skin, retraction of the ab- domen, vomiting, irregular fever, contracted and often unequal pupils, sometimes optic neuritis or retinitis. The headache is usually per- sistent with exacerbations of great intensity. Rather early in this disease the patient's mind begins to wander; he mutters incoherently; he may have periods of violence alternating with stupor. In some cases there is a continuous low muttering delirium. Vomiting also occurs early and is of a violent, explosive (projectile) character. This 28
434
DISEASES OF THE NERVOUS SYSTEM.
symptom is not always present. The head is bent back and the patient can be lifted from the pillow by placing the hand under the occiput. There is sometimes a general rigidity which resembles catalepsy. Drawing a dull point along the skin causes a red line to appear (tache cerebrate). Pinching or rubbing the skin causes much pain. The abdomen falls in and assumes a characteristic "boat shape." The pupils are usually contracted and uneven. The eyes are intolerant of light. Optic neuritis occurs often when the inflammation is at the base, but it is a late symptom. Convulsions and local paralyses of the cranial nerves, causing slight strabismus, ptosis, or facial palsy, may occur. The fever is irregular in course and not high — ioi° to 1030. The pulse is usually irregular or rather intermittent. It varies greatly in frequency and may be rather slow — 50 to 70. Respiration is rather quickened and sometimes irregular. The bowels are constipated ; the urine is small in amount and sometimes albuminous.
In the paralytic stage the patient becomes stupid or comatose; there is still some rigidity, except in the very last stages. The ab- domen is still greatly retracted, the pupils may now dilate, the skin be- come moist, and the patient's bowels and bladder move involuntarily. Death then occurs in one or two days as a rule.
When the disease is mainly on the convexity of the hemispheres there are more delirium, convulsive and paralytic troubles; when con- fined to the base there is less delirium, while paralysis of cranial nerves, optic neuritis, vomiting, and retraction of the head are com- moner or more prominent.
Course and Duration. — The disease may begin suddenly, and the patient pass at once into the comatose state, dying in a few days. Usually the process lasts one or two weeks; it may be prolonged for several weeks.
The prognosis is very grave, but it is less serious than in tuber- culous meningitis and more serious than in the cerebrospinal form.
The diagnosis is based on the presence of an exciting cause, such as disease of the ear or nose, trauma, infective fevers, and upon the presence of the symptoms given. It is usually easily recognized, the main difficulty being to distinguish it from tuberculous and cerebro- spinal meningitis.
To assist in diagnosis it is permissible to make a lumbar puncture, draw off the fluid, and examine it for bacteria.
Pathology. — The disease is a fibro-purulent or purulent inflamma- tion. It involves usually the base more than the convexity, but the reverse may happen. The ventricles are often involved and may be independently inflamed. There are descriptions, therefore, of simple
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DISEASES OF THE BRAIN AND ITS MEMBRANES. 435
basilar meningitis, meningitis of the convexity, and ventricular meningitis or ependymitis. The inflammatory deposits are most conspicuous along the course of the Sylvian fissure and the vessels branching from it, about the optic chiasm, and at the posterior and under surface of the cerebellum and the sides of the pons. It may lie only in the subarachnoid cavity, but usually the arachnoid and sometimes the dura are implicated. There is increase of fluid in the ventricles and arachnoid cavities, and this fluid may be turbid. The surface of the ventricles may show an inflammatory process.
The microorganisms found in meningitis are the pneumococcus, streptococcus pyogenes, intracellular meningococcus, the pneumo- bacillus, and a bacillus resembling that of typhoid fever. Still others have been described, and the process is apparently a mixed infection, though the pneumococcus is found oftenest.
Treatment. — Prophylaxis is the most important measure, as there is no specific treatment. Chronic disease of the ear and nasal sinuses should be attended to, and injuries of the skull treated with the strict- est regard to antisepsis. The patient should be kept quiet, a dose of calomel given, and small doses of iodide of potassium administered at frequent intervals. An ice cap may be applied to the head and hot ap- plications to the feet. Hot poultices along the upper spine are useful. Opium must be given for the pain, if needed; and antipyretics or phenacetin sometimes answer, in a measure. The internal use of iodoform has been highly recommended, gr. vi. to gr. xij. daily; shaving the head and rubbing upon it an ointment containing twenty per cent, iodoform, then covering the scalp with an oiled-silk cap, is a treatment highly spoken of. Surgical intervention is sometimes justifiable.
Epidemic Cerebrospinal Meningitis (Spotted Fever). — This is an acute infective disorder and is produced by a special micro- organism. It has certain peculiar clinical characteristics which lead us to describe it separately. Anatomically the changes involve the spinal membranes as well as the cerebral.
Etiology. — The disease most frequently attacks children, about 30 per cent, of the cases occurring in persons five years of age and younger and 15 per cent, in infants, but it may occur at any age, even up to seventy. Males are affected rather more often than females in the proportion of about 2 to 1. It prevails in the form of epidemics which affect cold and temperate climates especially, and which travel from one part of the country to another, but epidemics are especially fre- quent in seaboard cities. It may occur sporadically. It most fre- quently develops during the winter season, and attacks persons who
436
DISEASES OF THE NERVOUS SYSTEM.
are living in crowded houses, tenements, or barracks. It is communi- cable by a carrier and is slightly contagious. It does not, however, infect things, so that an infected house is not directly dangerous, i. e.y a family moving into a house that has been infected does not get the disease. It does not spread in schools or hospitals. Seaport towns seem more liable to the infection, and dock-labcrers and miners are good carriers. Darkness, moisture and heat in ships and mines favor- ing the development of the germ. One attack does not confer an immunity against a second.
Symptoms. — The general appearance of a person attacked with the disease is that of one who has been poisoned by some agent which is extremely prostrating to the whole system and at the same time one
which has a specific inflammatory effect upon the meninges of the brain and spinal cord. When the disease is rapid and malignant, the patient seems to die of an acute toxaemia before any inflammatory process has time to develop. In milder cases and those of longer dura- tion the prostration is less, and the evidences of inflammation of the meninges then develop in the typical way.
The disease may begin with prodromal symptoms of malaise, or some naso-pharyngeal catarrh, but usually there are few pro- dromes. The earliest symptom is usually severe headache which may be accompanied with vomiting and in children writh convulsions. As the disease develops, the headache, accompanied by giddiness, increases, pain and stiffness in the neck become marked, pains run down the back and radiate to the limbs; there is photophobia, and delirium in many cases is present. The skin is hyperaesthetic ; the pulse rises to 120 or higher; the temperature varies very much, but
Fig. 207. — The Kernig sign.
DISEASES OF THE BRAIN AND ITS MEMBRANES.
437
is usually raised to 1030, 1040 or even more. The bowels are gen- erally constipated. In most eases there develop certain skin erup- tions, usually in the form of purpuric spots; herpes, urticaria, and erythema are occasionally seen. These eruptions vary very much in different epidemics; the purpuric spots are the most important from a diagnostic point of view, and have given to the disease the name of spotted fever. The reflexes are at first exaggerated and the peculiar phenomenon known as Kernig's sign is present; i. e., the leg cannot be extended on the thigh to more than a right angle. There is a leukocytosis of 15,000 to 30,000. Lumbar puncture shows the presence of a cloudy fluid under pressure, and the specific meningo- coccus is usually found. As the disease progresses the symptoms of irritation and pain give way to those of somnolence, stupor, emacia- tion, paralysis and contractures, the patient dying of exhaustion
(Fig. 208). Optic neuritis, acoustic neuritis, and inflammation or irritation of the oculo-motor nerves take place, and paralyses of the limbs may be added.
The meningococcus is present in the nasal secretions in the early stage — first or second week of the disease.
In the last New York epidemic the most constant symptoms were:
Stiffness of the neck.
Headache.
Vomiting.
Convulsions in the young.
Petechial eruptions and herpes in nearly one-third. Kernig's sign.
Leukocytosis, 15,000 to 35,000. Eye complications.
Fig. 208. — Cerebrospinal meningitis, terminal stage.
438 DISEASES OF THE NERVOUS SYSTEM.
The disease may run a short and malignant course, killing the person in a few hours or one or two days. In the New York epidemic the majority of deaths occurred on the second day (Billings). In moderate cases it lasts two weeks, but it may continue with remissions for eight or nine weeks.
A large number of different varieties of the disease are described, such as the abortive form, fulminating form and typhoid form. In some cases the sensorium is not much disturbed — the patient being conscious and rational. Here the spinal cord seems most affected (spinal type). In other cases the cranial nerves, oculo- motor, facial and acoustic are especially affected and the disease seems most to attack the posterior fossa (posterior basic meningitis). The disorder is often complicated with pneumonia and bronchitis, less often with inflammation of the joints and serous membranes. The disease often leaves very serious sequelae, the most important being deafness and spinal irritation or chronic spinal meningitis. A large number of deaf mutes owe their affliction to this disease.
Pathological Anatomy. — In the very acute cases the post-mortem shows nothing but the evidence of very severe blood-poisoning. In the milder and more chronic cases an inflammation involving the pia and arachnoid of the brain and cord is found. This inflammation is fibrinous or fibro-purulent in character, and may be accompanied with the exudation of a good deal of inflammatory material. Bacteriologi- cal researches show that the disease is due to the presence of a specific microorganism, which is usually the meningococcus intracellularis, though sometimes other microorganisms like the pneumococcus are found.
The diagnosis is based upon the history of an epidemic of the disease being present, upon the presence of the ordinary symptoms of acute cerebral and spinal meningitis, such as headache, delirium, retraction of the head, the sunken abdomen, hyperesthesia and pains; Kernig sign and the presence of the peculiar purpuric spots or of herpes of the face. A lumbar puncture will definitely settle the diagnosis. In tuberculous meningitis the cerebrospinal fluid is usually clear. One must learn to distinguish the disease from typhus, tetanus, uraemia, pneumonia, and from the other forms of meningitis, especially the tuberculous. It should be remembered that after a community has been once visited by an epidemic these sporadic cases are apt to crop up from time to time for many subsequent years. The so-called posterior basic meningitis is only a form of the cerebrospinal disease.
The serum of infected patients has a high opsonic index, and ag- glutinative power and their tests are useful in diagnosis of doubtful
DISEASES OF THE BRAIN AND ITS MEMBRANES.
439
cases; still a normal opsonic index does not exclude the disease (Houston and Ranken and Taylor).
The prognosis varies much with the epidemic, but the disease is always a serious one. The mortality ranges from 20 to 80 per cent. The mortality rate in the last New York epidemic was 91 per cent. ; in the Teath epidemic it was 75 per cent. It is worse when the disease comes on suddenly and severely, with early coma. It is better in persons over the age of ten and worse in very young children. Cranial-nerve complications are unfavorable, in that they are apt to leave permanent deafness. Severe spinal complications are apt to leave their mark in the form of a chronic meningeal trouble.
Treatment. — The patient should be given sustaining food, and everything possible should be done to counteract the depressing effects of the toxaemia. Opium or morphine internally, chloral and alcohol are the drugs which have been specially recommended. Frequent, even daily tapping of the cord has produced good results in my ex- perience, especially in older cases. The specific antitoxin serum, prepared at the Rockefeller Institute under the direction of Dr. Flexner promises to be of real value. Treatment by vaccine obtained by the opsonin method has not yet produced results. Surgical treat- ment is advised and has been attempted but so far without results (Cushing). Warm baths, hot moist applications, and leeches have all been tried with more or less good results.
Prophylaxis. — The disease gets into the system through the agency chiefly of the infected naso-pharyngeal secretions. It enters the system through buccal abrasions, the tonsils, intestines and per- haps by the lungs. Persons exposed to the disease and patients them- selves should have the naso-pharyngeal kept carefully disinfected. Public places in which spitting occurs should be carefully cleansed. Those living in badly infected houses should be isolated. It does no good to isolate patients and disinfect houses if the carriers are not looked after.
Tuberculous Meningitis (Acute Hydrocephalus). — This is a form of meningitis due to infection with the bacillus tuberculosis. It differs pathologically from other forms in the character of the in- fective organism; anatomically, in the fact that the inflammation is usually and chiefly basilar and never purely purulent ; etiologically, in that it chiefly affects young children ; and symptomatologically, in the presence of prodromata and a more irregular course.
Etiology. — Tuberculous meningitis occurs chiefly between the ages of two and ten, oftenest between two and five years, sometimes in in- fancy, rarely in adult life, very rarely after the age of fifty. Males are
44-0
DISEASES OF THE NERVOUS SYSTEM.
rather more subject to it. A hereditary history of phthisis, a scrofu- lous diathesis, bad hygienic surroundings, and the presence of tubercu- losis elsewhere in the body predispose to it. Tuberculous milk is prob- ably one source of the infection. The eruptive fevers, especially measles, blows on the head, and great emotional excitement appear to act as exciting causes.
Symptoms. —A knowledge of the prodromal symptoms is especially important. These are paroxysmal and intensely severe headaches and darting pains in the head, which cause the child to cry out ("the hydrocephalic cry"), vertigo, loss of appetite, explosive vomiting without nausea, the vomited matter being usually colorless and watery, constipation, an altered dispositon, and irritability. The tache cere- brate or cerebral macule, more rarely ptosis, contracted pupils and facial paralysis may appear early. The prodromal stage often lasts, with remissions, three or four weeks. When the disease sets in there is more persistent headache; vomiting, fever, and the other symptoms of meningitis already described appear. The pulse is rapid. The irri- tative stage gradually passes into the paralytic and comatose. The temperature is irregular and rarely very high. There may be ptosis and strabismus and the pupils are apt to be dilated. Erythema and herpes may occur, later convulsions may occur with optic neuritis, ocular palsies, diarrhoea and incontinence. The Kernig sign is not so common; but the Babinski reflex is present in one-fourth of the cases (Koplik). Macewan's sign is often present. It indicates the presence of hydrocephalus. (The patient sits upright, the head slightly inclined to one side ; percussion with the finger on the upper side brings out a tympanic note.) Death occurs in three or four weeks. In infants the disease often runs a very obscure course, the patient showing chiefly symptoms of brain compression with stupor.
Pathological Anatomy. — In rapidly fatal cases, with severe symp- toms, there may be only an intense congestion of the brain with nu- merous miliary tubercles in the pia mater at the base and over the con- vexity. Here we must assume that a bacillary toxin causes the symptoms. In most cases there are decided deposits of tubercles at the base, with fibrinous inflammatory deposits about the optic chiasm, along the fissure of Sylvius, at the sides of the pons, and elsewhere. Miliary tubercles are seen scattered over the convexity and in the choroid and ventricles. They are generally found in the spinal mem- branes also, especially over the cauda equina. The tubercles lie be- neath the pia surrounding the small vessels. They may coalesce into large tuberculous nodules. There is usually an increase in the arach- noid fluid, and in most cases an increase in the ventricular fluid.
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DISEASES OF THE BRAIN AND ITS MEMBRANES. 44I
Somewhat rarely there are very great distention of the ventricles and compression of the convolutions. This condition used to be called acute hydrocephalus. Small spots of softening may be seen from ob- literation of the vessels by the tubercles. The bacillus tuberculosis is found in the tuberculous nodules.
Diagnosis. — As regards the form of the disease, this is based on the hereditary history, the age, the existence of tuberculosis of the lungs or other organs, and the peculiar prodromata of the disease. Occasionally tubercles can be seen on the choroid. Lumbar puncture of the spinal canal with withdrawal of fluid and its examination fur- nishes the most certain method of diagnosis. The fluid is clear, or per- haps a little turbid and is under pressure, the tubercle bacillus is usually found if carefully looked for. The infective character of the fluid may be tested on guinea-pigs. There is an excess of protein and a large (90 per cent.) percentage of mononuclear cells.
Prognosis. — This is usually absolutely bad, yet post-mortem ob- servation of patients dying with practically no inflammatory change makes it seem possible that the disease might be checked, and a good many cases are reported in which this apparently has been done. Some of these are, however, probably cases of hereditary syphilis.
Treatment. — So far as is now known, this is not different from that given under the head of meningitis elsewhere. It seems, however, as if in time some antitoxin may be discovered which will check the progress of the poison and the development of the tubercle; mean- while the best thing to do is to give the patient fresh air, administer internally small doses of iodide of potassium at frequent intervals and use symptomatic treatment.
The prophylaxis consists in treating all tuberculous lesions, in bringing up children on non-infected milk and keeping the hereditarily predisposed away from all sources of infection — which last is perhaps as yet a council of perfection.
Hydrocephalus. — Hydrocephalus is a condition in which there is an excessive amount of fluid in the ventricles and subarachnoid cavity of the brain. When this fluid is in the ventricles it is called internal hydrocephalus, and practically most hydrocephalus is internal. Hydrocephalus is a symptom of several morbid conditions; but in general we may invite it into the chronic and the acute or sympto- matic forms.
Chronic Primary Hydrocephalus. — This is a disease of infancy, characterized by a gradual enlargement of the head, with mental deficiency and symptoms of brain irritation caused by an accumula- tion of fluid in the ventricles of the brain.
442
DISEASES OF THE NERVOUS SYSTEM.
Chronic hydrocephalus is not an inflammatory process, but one due to mechanical causes or to defects in structure or nutrition. The fluid always accumulates in the ventricles of the brain ; hence chronic hydrocephalus is always internal. The so-called external forms of hydrocephalus are inflammatory or else are secondary to meningeal hemorrhage or brain atrophy.
Etiology. — Four out of five cases begin at birth or within the first six months of life. Congenital anomaly, syphilis, alcoholism, lead poisoning in the parents, and some unknown family taint predispose to the disease. Poverty and poor nutrition and rickets are also factors. These causes mechanically block up the aqueduct of Sylvius and thus stop or check the current of cerebrospinal fluid.
Fig. 209. — Chronic hydrocephalus.
Symptoms. — The head may be so large at birth that instrumental help is needed. More often the parents notice a gradual increase in the size of the child's head, beginning soon after birth. The forehead bulges, the occiput stands out, the fontanelles and sutures widen, and pressure shows evidence of fluctuation. Meanwhile the face does not grow much and the result is to give triangular shape to the head. It may measure twenty-four, twenty-seven and one-half (Minot), thirty- two (Bright), and even forty-three inches (Klein) in diameter. These extreme measurements are reached only after one or two years. With this abnormal growth of the head, mental and physical symptoms ap- pear. The infant is restless and irritable; its appetite may be good, but the general nutrition is poor and its bodily growth is retarded. The mind does not develop ; usually it does not or cannot learn to walk. It may be unable to support the weight of its head. There is strabis- mus and sometimes optic atrophy. The pressure of the dropsy thins
DISEASES OF THE BRAIN AND ITS MEMBRANES. 443
the orbital bones and forces down the axis of the eyeballs (see Fig. 209). Vomiting, coma, and convulsions eventually appear, and the child dies of exhaustion or some intercurrent disease in two or three years.
In some cases the trouble is less serious, it ceases to progress, the bones solidify, and the child grows up with good intelligence.
The hydrocephalus which is associated with the brain atrophy of insanity and old age or with general dropsical conditions has no kin- ship with the process we are now describing.
Pathology. — The disease is due to the gradual accumulation of a serous fluid in the ventricles of the brain. The cause of this is an in- flammatory or developmental obliteration of the aqueduct of Sylvius or the foramen of Magendie and the adjacent lateral foramina of Mierziejewski. This prevents the escape of the ventricular fluid into the subarachnoid cavity. Contributing factors are congenital or ac- quired defect in the absorbents of the ventricles and a rachitic and easily yielding skull. The attempts to make chronic hydrocephalus an inflammation are failures, though there is at times a thickening of lining membrane of the ventricles and other changes of a proliferative character. The lateral ventricles when the cerebrospinal fluid is secreted are principally and often solely affected, and these are so dis- tended as to press out their cerebral walls, flattening the convolutions and turning them into a thin shell often less than a quarter of an inch in thickness. Sometimes only one lateral ventricle, and in rare cases only the fourth ventricle, is affected by the dropsy.
The diagnosis has to be made from rickets and an acute inflam- matory process. In rickets the head is square, the fontanelle does not bulge, the enlargement is less, and there are signs of the disease in the bones elsewhere.
Prognosis. — -The congenital cases usually result fatally in a few months, or at least before the third year. Those developing in in- fancy may persist for four to six years; and in mild cases the disease ceases to progress and a fairly healthy adult life is reached.
Treatment. — A great many measures have been recommended, but there is no unanimity about any one of them. In such a state' of therapeutics it is safe to say that treatment is of little use. The most rational measure is the inunction of mercury and the administration of iodide of potassium combined with tonics. The other surgical measures, such as tapping the ventricles, are irrational and need not be discussed. Quincke's method of tapping the spinal canal will not apply in these cases. Strapping the head with diachylon plaster is recommended by Trousseau and by J. Lewis Smith.
444 DISEASES OF THE NERVOUS SYSTEM.
Secondary or acquired hydrocephalus is usually caused by an attack of acute meningitis, or by tumors. It may also be due to ependymal inflammation and obstruction of the veins of Galen, by thrombosis or other mechanical cause. In acute meningitis this is a frequent condition and cause of death. In other cases infants are left with a growing head and develop symptoms later, of the primary disease. In the acute conditions the symptoms may run a varying course owing to the partial and irregular escape of the fluid. The patient, if the disease progresses, becomes stupid and comatous and develops paraplegia and rigidity of the body, retraction of the head, trunk and great emaciation. The treatment is purely surgical and consists in puncture of the ventricle and withdrawal of fluid or a de- compressive operation (Cushing). The results of surgical interference have so far not been satisfactory.
Aecohoeic Meningitis (Serous Meningitis, "Wet-Brain").
Alcoholic meningitis is a clinical term used to indicate the peculiar group of cerebral symptoms which is seen in persons who have suc- cumbed to the effects of prolonged alcoholic intoxication. The dis- ease is not a true meningitis but an acute toxaemia of the brain with serous effusion, and may be called, for the purpose of convenience, an alcoholic serous meningitis. It should not be confounded with the acute serous meningitis following infections and trauma.
Etiology. — The disease occurs oftenest in men simply because of the more frequent indulgence of the male sex in alcohol. It rarely develops until a person has been drinking eight or ten years, and, con- sequently, affects people oftenest between the ages of thirty and forty. The exciting cause is commonly alcohol and in this country whiskey or what are known as "hard drinks," but beer and ale will accomplish the same result. I have rarely seen the disease in wine drinkers. The persistent use of morphine, cocaine, and chloral may lead to much the same condition. The exciting cause is usually a continuous drink- ing bout of two or three weeks, ending in delirium tremens. The delirium tremens, however, is not by any means always present. The patient may pass from a condition of prolonged intoxication into the condition of alcoholic meningitis or "wet-brain."
Symptoms.— -In case delirium tremens has occurred, the patient after two or three days of prolonged delirious excitement gradually sinks into a semi-coma. This is accompanied by a muttering delirium. The patient is sufficiently conscious to have flitting delusions and hal- lucinations of sight and hearing. At this time he is able to drink and
DISEASES OF THE BRAIN AND ITS MEMBRANES. 445
take food; the pulse is rather rapid, the temperature is usually normal or may be raised one-half or one degree. The skin is hyperaesthetic and pressure upon the muscles of the arms or legs or abdomen causes pain. The pupils are usually rather small. Often at this time con- junctivitis and keratitis may appear. After a few days the patient's stupor becomes deeper and he can be aroused only with difficulty. The arms and legs are now somewhat stiff, the reflexes are exaggerated, the neck is stiff and slightly retracted, and attempts to move it cause expressions of pain. The Kernig sign is present. The abdomen is re- tracted and the skin and muscles are still very hyperaesthetic. The lids are closed. The pupils are small and do not react well to light. The tongue is coated and usually dry, and urine and faeces may be passed involuntarily. The patient may linger this way for several days more. The pulse becomes rapid and feeble, the extremities are stiff and cold. The skin is dry and loses its elasticity, so that when pulled up between the fingers it stays in folds. "Putty skin" is a good name for this. The coma deepens, the temperature may rise to 1030 or 1040, and symptoms of pneumonia may appear as the scene closes. On the other hand in some cases the patient does not pass into the worst stage, the mind becomes clearer, the hyperesthesia lessens, food is taken better, and the bowels are moved voluntarily. Improve- ments continues and in three or four weeks the convalescence begins.
Pathological Anatomy. — I have made autopsies and careful micro- scopical examinations in over twenty cases of the character just de- scribed. In nearly all the brain is found to be somewhat pale, the arachnoid contains two or three ounces of serous fluid, the subarach- noid space is saturated with fluid, and the ventricles are dilated. Sections through the brain sometimes show punctate hemorrhages and in rare cases spots of hemorrhagic extravasation are seen sur- rounded by softening. Occasionally the beginning of a suppurative cerebral meningitis will be seen. Microscopic examination shows in the uncomplicated cases that there is no true inflammatory process. There is often congestion, but not always; the commoner condition is an oedema of the brain tissue, the perivascular and pericellular spaces being dilated. The nerve-cells show various stages of degeneration, not pigmentary in character, however. The chromophilic granules are often unstained, or, if stained, have lost their true relations, and seem broken down. The cell outlines are irregular; the nucleus lies near the periphery of the cell, and in some cases has broken out and escaped from it. Sometimes there is a large number of neuroglia cells in the pericellular spaces. The disease is undoubtedly, primarily at least, a toxaemia not due directly to the influence of alcohol but to the
446
DISEASES OF THE NERVOUS SYSTEM.
poisons which have developed in the body as a result of the condition of inanition and the paralysis of the digestive function caused by the prolonged ingestion of alcohol and abstinence from food. The cell degeneration is more like that which is known as "degeneration from a distance," such as is seen in nerve-cells when the neuraxon is destroyed. It is a degeneration which affects especially the body of the cell and not so much the nucleus; hence the remarkable power of recovery from this condition which so many people show.
The diagnosis of the disease is to be made from ordinary suppura- tive meningitis, from acute serous meningitis due to infection, and from acute encephalitis. In most cases the history of the patient is quite sufficient to establish the diagnosis. The symptoms of them- selves are almost identical with those of ordinary acute suppurative meningitis. The only distinctions which I have been able to observe are that in suppurative meningitis there is more fever, there is less of the low delirium, hallucinations are rare, and there is an earlier and more profound coma. In other words, it is an acuter and more severe malady than alcoholic meningitis. The absence of convulsions and paralysis and the presence of hyperesthesia, rigidity, and contracted pupils, as well as the absence of pyrexia, are usually sufficient to dis- tinguish the disorder from encephalitis or encephalitis complicated by alcoholic meningitis. Lumbar puncture will settle the diagnosis and often prognosis. The cerebrospinal fluid is usually in excess and under great pressure; it is clear and colorless, unless there has been a superimposed infection as sometimes occurs, when evidence of inflam- mation will be found.
The prognosis is bad when the disease has become well developed and when decided coma and rigidity have set in. A prognostic crite- rium which I have long used and which is fairly accurate is this : if the patient has not a stiff neck and back he will get well, but when stiff* neck comes on the patient dies.
The treatment of the disorder should be instituted at the very be- ginning. If there are still any relics of the debauch, as shown in the condition of the stomach or intestinal tract, the stomach should be washed out and, at all events, a thorough purge should be given. The patient should then be fed most liberally with hot milk given every two hours; beef tea and an egg beaten up in milk may also be given, but the condition of practical starvation on the part of the patient should al- ways be borne in mind. Stimulants in the shape of whiskey should not be administered if it is possible to avoid it, but strychnine in doses of one-sixtieth of a grain every two hours is often useful. An ice cap should be applied to the head and at times leeches or large blisters
DISEASES OF THE BRAIN AND ITS MEMBRANES.
447
seem to be useful applied to the back of the neck. The patient, how- ever, should not be much depleted. Large doses of ergot have been recommended. When he becomes comatose it means that the ven- tricles and archnoid cavities are becoming filled with water. At this time tapping the spinal cord may be tried. I have done this in a number of cases and have at times removed two or three ounces of fluid with some amelioration of the symptoms and never any bad results, but I have never seen it do any permanent good.
CHAPTER XIX.
DISEASES OF THE BRAIN.
These diseases, like those of other parts of the nervous system, consist of malformations, vascular disturbances, inflammations, soft- enings, hemorrhages, degenerations and scleroses, chronic infections, tumors and functional disorders.
Cerebral hyperemia is a condition in which there is an excessive amount of blood in the cranial cavity; it may be acute or chronic, active or passive. According to Leube, while cerebral hyperemia may exist, it cannot be diagnosticated. Practically, the diagnosis is not now made by neurologists. Still it seems to the writer that we cannot deny that there may be at least a chronic passive hyperemia.
Etiology. — Hypersemia of the brain occurs physiologically under excitement and overactivity of the heart and from various stimuli; a pathological condition of acute congestion may be induced by sun- stroke, certain drugs, such as alcohol, and by injuries; also by mechanical causes which prevent the exit of the blood from the cranium. An acute congestion also occurs in mania and in many forms of fevers, as well as in the initial stage of meningitis. A chronic cerebral hypersemia may be induced by the causes already mentioned as bringing on acute congestion. The prolonged use of alcohol, prolonged mental excitement, overwork, and worry may also lead to this condition. The foregoing causes lead to what is known as active congestion, in which the blood is driven in excess into the brain through the arteries. A passive congestion may exist in which the blood is prevented from leaving the brain and is kept mainly in the intracranial veins. The causes of passive congestion are chiefly mechanical, such as cardiac disease and mechanical ob- structions about the neck from tight clothes, and an obstruction to the flow of blood from the lungs on playing on wind instruments.
Symptoms. — A great deal has been written regarding the sympto- matology of cerebral hypersemia, but many of the statements made are nothing but guesswork. Probably the main symptoms produced by an active congestion of the brain'are a sense of fullness and pressure, a feeling of constriction about the head, some headache which may be vertical, mental excitement or irritability, confusion of ideas, vertigo,
448
DISEASES OF THE BRAIN.
449
insomnia, ringing in the ears, and pulsating sounds in the head. These symptoms are perhaps due in reality to an anaemia. Geigel asserts that true hyperemia means an increased flow of blood through the capillaries, and there may easily be a poor capillary flow with a passive congestion. Examinations of the fundus of the eye and of the tympanum furnish no criteria.
Treatment. — The treatment of cerebral hyperemia, when indicated, consists in giving large doses of fluid extract of ergot and bromide of potassium. One or two drachms of the ergot three times a day and fifteen or twenty grains of bromide of potassium may be prescribed. In some cases veratrum viride relieves promptly the pressure feelings. Wet cups to the back of the neck, the cautery in the same region, ice caps, purgatives, quiet and rest, and a careful regulation of the diet and the bowels are all important measures.
Cerebral Anaemia. — This condition, according to Leube, can be diagnosticated.
It occurs among the young; more often in females than in males. It is seen in early adult life, when it is induced by the various causes producing general anaemia, and again after the climacteric, when it is due to organic changes in the cerebral arteries of the nature of an obliterating endarteritis. B right's disease and syphilis, exhausting diseases and profuse hemorrhages, and such disorders of digestion and nutrition as lead to general anaemia produce also cerebral anaemia. A potent cause of acute cerebral anaemia is fright.
Symptoms. — -The symptoms of acute cerebral anaemia are vertigo, tinnitus, obscuration of vision, acceleration of the pulse, confusion of ideas, nausea, faintness, or complete syncope, rarely convulsions. In chronic cerebral anaemia the symptoms are mental apathy and a feeling of disinclination to work, tendency to somnolence in the daytime and insomnia at night, mental depression, headaches which are usually frontal or vertical, occasionally some vertigo and tinnitus.
Diagnosis. — A chronic anaemia of the brain can hardly be recog- nized except through the evidences of cerebral arterial sclerosis and defective circulation, and some general anaemia. That the symp- toms of cerebral anaemia are due to a vascular rather than a blood condition, is shown by the absence of characteristic cerebral symp- toms in pernicious anaemia.
Treatment. — Treatment should be directed toward strengthening the heart, opening up the capillaries, enriching the supply of blood and toward improving the general nutrition. It consists, there- fore, in the administration of preparations of iron and of such tonics as the mineral acids, strychnine, quinine, and nitroglycerin. 29
450 DISEASES OF THE NERVOUS SYSTEM.
INFLAMMATION OF THE BRAIN.
The forms of acute inflammation of the brain are accute suppura- tive encephalitis or brain abscess, acute exudative encephalitis with hemorrhage, and acute polio-encephalitis. The only important form of primary chronic inflammation is multiple sclerosis.
Acute Suppurative: Encephalitis (Abscess of the Brain).
Brain abscess is a suppurative inflammation which affects the par- enchymatous and other structures of the organ. It is always a focal disease, but may be single or multiple.
Etiology. — The primary cause of all forms of brain abscess is practically a microbic infection. The form of microbe, its mode of entrance, and the part of the brain attacked vary greatly. The pre- disposing causes relate chiefly to age and sex. Brain abscess rarely occurs before the first year or after the fiftieth year of life. It is rather frequent in young people, and occurs on the whole oftenest between the ages of ten and thirty. Males are more often affected than females in the ratio of about three to one. The exciting causes are chiefly disease of the ear, of the nose, and of the cranial bones, injuries, and remote suppurative processes.* To this may be added septic and other infections. Chronic inflammation of the middle and internal ear is the most common cause of brain abscess, especially when that disease affects the tympanum and mastoid cells. Only a small per- centage of abscesses (about 8 per cent.) follow acute otitis media. This is the reason more cases occur in adults. Caries of the ethmoid and nasal bones and of the orbital cavity leads to brain abscesses in a considerable proportion of cases. After chronic ear and bone diseases injuries are the most frequent cause, making up about 50 per cent, in all. The injury may be a compound fracture with direct infection from the open wound, or the abscess may be the result of contrecoup and may develop in a part of the brain opposite to that which was in- jured, or the abscess may develop below the point injured, there being apparently healthy tissue between the surface of the brain and the diseased part. These abscesses develop through laceration of brain tissue and subsequent infection of the wound with organisms. The most common remote suppurative processes which are followed by brain abscess are tuberculous inflammation of the lungs, fetid bronchitis, and empyema. Brain abscess may develop, however, from distant points of suppuration on the extremities or in almost any part of the body. Pyaemia may lead to the production of brain abscess.
*In nine thousand consecutive autopsies at Guy's Hospital there were fifty-seven brain abscesses due to ear disease and one due to nasal disease (Pitt).
DISEASES OF THE BRAIN.
451
Among the infectious fevers which are complicated with brain abscess are diphtheria, typhoid and typhus fevers, erysipelas, small-pox, the grippe. The oidium albicans or thrush may also be a cause. Brain tumors sometimes become surrounded by a suppurative encephalitis or may break down with the formation of mixed suppurative and neoplastic tissue. Tuberculous tumors are most frequently accom- panied by suppurative encephalitis.
Symptoms. — Brain abscesses take sometimes an acute and some- times a chronic course. In acute cases the symptoms develop rapidly and the disease runs its course in a few days or weeks. The symptoms come under the general head of those of pressure, those of poisoning from the diseased focus, and local symptoms due to irritation or de- struction of certain special areas of the brain. The pressure symp- toms are those of headache which is often very severe and persistent, vomiting which is quite frequent, though not invariable, vertigo, and a condition of mental dullness which may pass into a delirium ending finally in coma. Optic neuritis often occurs. The pupils are apt to be irregular, but furnish no definite indications. The pulse is usually slow, ranging from 60 to 70, but it varies a great deal. The tempera- ture is normal or subnormal, as a rule, but this also varies, and it may rise several degrees above normal, always running an irregular course. The blood shows an increase in leukocytes and especially of the poly- morphs. The toxic symptoms are those which we get in septic poison- ing; namely, prostration, irregular fever, emaciation, anorexia, and such mental and sensory disturbances as have already been referred to. As a result of local irritation or destruction, there occur convul- sions, paralysis, aphasia, and disorders of some of the special senses. Convulsions are not very common. When they occur they are gener- ally of an epileptic character. The paralysis is usually in the form of hemiplegia. The cranial nerves are not often involved, if we except the optic. The urine is said to show a diminution in chlorides and an increase in phosphates. The patient dies finally in coma from exhaustion.
In the chronic form of brain abscess the symptoms may for weeks, months, or years remain practically latent, after the exciting cause has been at work and after the abscess has been established. The patient during this latent stage may suffer from headache, vertigo, mental irritability, and depression; he may at times have a convulsive attack. Occasionally there will be an exacerbation of the disease, at which time he suffers from intense pain, vomiting, perhaps delirium or a convulsion. From this he recovers and continues in a fairly good state of health again. After a variable period- usually of weeks or months, the ter-
452
DISEASES OF THE NERVOUS SYSTEM.
minal stage sets in. This terminal stage of the chronic form may assume very much the characters of the acute form already described. In other cases it shows itself by a sudden apoplectic or epileptic seizure or a sudden attack of coma, in which the patient sinks and rapidly dies. These terminal phenomena are due to the fact that the abscess, which has been, previously encysted and quiescent, suddenly breaks into a lateral ventricle or through the surface of the brain, or to the fact that a hemorrhage occurs into the abscess.
Complications. — The common complications of brain abscess are a phlebitis of the superior petrosal and lateral sinuses and a meningitis. The phlebitis accompanies abscesses that are caused by disease of the ear. The meningitis may be caused by ear disease, but more frequently accompanies abscesses due to injury. When phlebitis is present there will be found an oedema about the ear and neck and a hardness of the jugular veins. In meningitis there is apt to be more rigidity of the neck, more pain, and there are often cranial-nerve paralyses.
Pathology. — Acute suppurative encephalitis resembles acute sup- purative myelitis in the intimate nature of the changes that take place. There is an intense congestion of the part, which gives it a reddened appearance and which used to give to this process the name of red softening. This condition, however, is only the initial stage of the suppurative inflammation and does not deserve to be ranked as a special form of inflammatory process. As the process continues the parts become crowded with leukocytes and infiltrated with inflammatory exudate. The nerve-fibres and cells are destroyed, in part mechanically, in part by the poisonous influence of the pyogenic organisms. The nerve -cells lose their normal contours, swell up, and disintegrate; the neuroglia cells absorb the broken- down detritus and swell up, forming what are known as granu- lar corpuscles; the leukccytes increase until a purulent mass is formed. The total result is a mixture of broken-down nerve-fibres and cells, leukocytes, and granular bodies. Bacteriological tests show the pres- ence of various pyogenic microbes. Usually staphylococci or strepto- cocci; rarely the pneumococcus. The abscess thus formed varies in size from one centimetre to six or eight centimetres in diameter (two- fifths of an inch to three inches). It is generally somewhat round, and if the case is chronic a fibrous wall is formed. It takes from three to four weeks for such a wall to develop (Fig. 210). Brain abscesses are usually single, occasionally there are two or three. In some conditions thev are multiple; that is to say, there may be fifteen, twenty, or more. Multiple brain abscesses are always small and are usually due to pysemic infection.
DISEASES OF THE BRAIN.
453
Location. — Brain abscesses involve the cerebrum oftener than the cerebellum, in the proportion of about four to one (Barr), two to one (Le Fort and Lehmann). They occur rather oftener in the right cerebrum. They are very rare in the pons and medulla. The cere- bral lobes oftenest affected are the temporal and frontal. In the cere- bellum it is the lateral hemispheres that are most frequently attacked. The seat of the abscess has important relations to the cause. Ab- scesses due to ear disease are almost always either in the temporal lobe or in the cerebellum. If the ear disease is in the tympanum, the cere-
Fig. 210. — Abscess of cerebellum.
brum is usually the seat of the abscess. If the disease is in the mastoid cells, the cerebellum is usually the part affected. If the disease is in the labyrinth, the abscess is also more apt to be in the cerebellum. This distribution of the seat of the disease is due to the anatomical rela- tions of the bony parts to the temporal lobe and cerebellum, respec- tively. Brain abscesses due to injuries are more frequent in the frontal and temporal lobes. What are known as idiopathic brain abscesses — that is, those which arise without any known cause — are most frequent in the frontal lobes. This is because m> st such cases are due to an unrecognized affection of the nasal cavities and ethmoid bone. Brain
454
DISEASES OF THE NERVOUS SYSTEM.
abscesses due to suppurative processes in the lungs and pleura are probably embolic; and, as the emboli are carried up into the middle cerebral artery, the brain abscesses having this origin are situated in the field supplied by this artery. In children under ten, in whom brain abscess is usually due to ear disease, the cerebellum is more apt to be affected.
Course. — Acute abscesses last from five to fourteen days, rarely over thirty days. Traumatic cases run the shortest course. Chronic abscesses may have a latent period of weeks, months, and in rare cases even one or two years. When terminal symptoms come on, death
Fig. 2ii. — Showing the points where the trephine is usually applied and the relations of the sinus. The divisions of the lines indicate quarter-inches. (Lancet.)
occurs in a few days. In a few cases brain abscesses have been spon- taneously evacuated through the nose. Aside from this, the termina- tion is always a fatal one unless surgical interference takes place. There is sometimes a recurrence of the abscess after an operation.
Prognosis. — The outcome of brain abscess is always a fatal one unless surgical interference takes place. There is sometimes a recur- rence of the abscess after an operation.
Diagnosis. — The diagnosis of brain abscess is based upon the his- tory of injury, aural or nasal disease, remote suppuration, upon the general symptoms of sepsis, upon the presence of headache, vomiting, slow pulse, normal or subnormal and irregular temperature, a local tenderness of the scalp and rise of temperature over the seat of the
DISEASES OF THE BRAIN.
455
lesion, hebetude, delirium, optic neuritis, rapid wasting, and dimi- nution of chlorides in the urine and leukocytosis with a high poly- morph, count. Lumbar puncture may help to exclude a meningitis.
The diagnosis of the location of the abscess is based upon the history of its cause, whether from injury, ear disease, emboli from the lungs, or nasal disease; also upon the presence of hemiplegia, local convulsions, tenderness and rise of temperature of a certain area of the scalp. As brain abscesses are apt to affect latent regions like the temporal and frontal lobes, local diagnosis is usually difficult. The diagnosis must be made from tumors of the brain, meningitis, and phlebitis of the sinuses. The differential points are given under the heads of the diseases mentioned. In some cases of otitis media symptoms of abscess occur, but there is an exquisite superficial tender- ness limited to the region of the trigeminus. These cases recover and are perhaps due to a dural congestion (Cushing). Sometimes, also, in otitis symptoms of the acute serous meningitis of Quincke occur, which are relieved by lumbar puncture.
Treatment. — The actual treatment of a brain abscess after it has developed is, as already stated, exclusively a surgical one. The successes so far have not been very great, but they have been suffi- cient to justify operation and to furnish greater hope for the future, when a more exact diagnosis can be made and a wider surgical ex- perience has been obtained. According to Cushing, the percentage of recoveries now is about fifty. The accompanying figure shows the points to be located in trephining for abscess from ear disease. Some- thing is due to the patient in the way of prevention, especially in cases of persons who have chronic aural or nasal disease with carious proc- esses. These should be carefully watched and treated.
Acute Non-suppurative Encephalitis (Hemorrhagic Encepha- litis, Hemorrhagic Polioencephalitis).
Acute non-suppurative encephalitis is an exudative and sometimes hemorrhagic inflammation of the brain, characterized by symptoms of general infection or toxaemia, of severe cerebral irritation, and the signs of local cerebral lesion. It is sometimes spoken of as a non- suppurative encephalitis and as a curable encephalitis. The disease occurs as one form of the infection when an epidemic of anterior polio- myelitis occurs. In some of its forms, it is probably a rather common affection, complicating especially the acute infectious fevers of child- hood and influenza.
The disease sometimes attacks the medulla, causing symptoms of
456
DISEASES OF THE NERVOUS SYSTEM.
an acute bulbar palsy. This is termed the polio-encephalitis inferior of Wernicke, and it sometimes attacks the nuclei and association tracts of the motor nerves of the eye, causing acute ophthalmoplegia. This is termed polio-encephalitis superior. In its most frequent form, it attacks the cerebral hemispheres, sometimes, perhaps, the cortical gray matter alone, but it is more often a diffuse process.
Etiology. — The disease is caused, first, by the same infection as that of epidemic poliomyelitis ; second, by other febrile infections, especially influenza, the fevers of childhood and otitis media; third, it is one of the results and complications of serious alcoholism. It occurs most often in the young — except in that form caused by alcohol and in- fluenza. In some cases, trauma, when associated with some slight infection and perhaps laceration of the brain, causes it. Encephalitis has been known to follow a malignant endocarditis, and in connection with the infections of the puerperal state.
Symptoms.- — Symptoms of the disease, when it affects the medulla or mid-brain, are described under the heads of acute bulbar palsy and acute ophthalmoplegia. When the disease attacks the hemispheres, it begins, as in the other cases, rather suddenly and without notable premonitory symptoms. The patient is seized by headache followed by fever sometimes reaching 1050 F. This may be associated with vertigo, vomiting, photophobia and delirium. In children there may be convulsions. The symptoms of irritation disappear and are followed by a condition of semicoma or stupor. The patient can generally be patly aroused, and he does not usually have the stiff neck or the small pupils of meningitis. The respirations are shallow and frequent, the pulse is rapid and feeble. As the disease progresses, the deep reflexes are diminished and later the sphincters may be in- volved. After the patient has lain in a semicomatose condition for several days, he may become less stupid and more irritable and restless; or after two or three weeks of comparative stupor he may begin gradually to improve and, in a few weeks more, con- valescence takes place, leaving him well or with some motor or mental defect.
In some cases an epileptic convulsion may occur in the early part of the disease. Again, as the disease develops, aphasia and paral- ysis of the arm or leg, or hemiplegia, may appear. In accordance with the location of the inflammation, the patient may have disturb- ances in the motor sphere, or he may have hemianopsia, hemiataxia, or disturbance of the cranial nerves, such as nystagmus, or eye palsy, or difficulty in speech and deglutition. Children may be left with a mental defect, and probably mild attacks of this disease are often
DISEASES OF THE BRAIN.
457
the cause of defects, or of epilepsies, attributed, perhaps, to an ordi- nary infective fever. An optic neuritis may also occur.
Course and Prognosis. — The disease is always serious but is not by any means always fatal. It may in its milder form run a course of two or three weeks, the patient gradually coming out of his stupor and making a slow recovery. In other cases the coma continues to deepen and the patient dies of exhaustion, and in still other cases the disease passes into a chronic state in which he lingers for weeks and even months.
Diagnosis. — The disease in the young is probably more often mis- taken for meningitis. It is to be differentiated from this by the sud- den onset with coma, the absence of projectile vomiting, pinhole pupils, stiff neck, hyperesthesia, and rigidity of the limbs. The pres- ence of hemiplegia or local paralysis, or the occurrence of an epileptoid attack would point to encephalitis. The diagnosis from meningitis, however, with acute hydrocephalus cannot always be made. The previous occurrence of an attack of grippe in the young, or of expo- sure to the sun or acute alcoholism in the adult would lead to the probability of an encephalitis, provided the symptoms of meningeal irritation could be excluded. Tuberculous meningitis can be excluded usually by the presence of premonitory symptoms and the absence of any tuberculous focus in the lungs or intestines. By the help of lumbar puncture fluid can be withdrawn from the spinal sac, and examination of it might be of service in excluding meningitis.
Pathological Anatomy. — The pathological process underlying this disease consists of an acute inflammation with intense congestion and numerous small hemorrhages and capillary emboli. There is some hemorrhagic exudation as well as infiltration of leukocytes, with a certain amount of softening of the cerebral tissue in the neigh- borhood. The parts most frequently affected are variously stated to be the frontal and occipital lobes, the central convolutions, the semi- ovale, the temporal lobes, the base of the brain, and the corpus stria- tum. In four cases which I have examined, the process was in the temporal lobes, the parietal lobule, the mid-brain and the corpus striatum. It has been known to affect the cerebellum. If the proc- ess is a mild one, the hemorrhage and exudate are absorbed, and the injured brain tissue is gradually replaced by connective and neuroglia tissue. In this way small foci of sclerotic tissue are formed and the patient may afterward suffer from symptoms due to this condition. In the severer cases the softening becomes more extensive, larger hemorrhages occur, and in one case I have seen a massive apoplexy as the terminal stage.
458
DISEASES OF THE NERVOUS SYSTEM.
Treatment. — The patient should be kept quietly in bed and should be given an active purge. Calomel is usually employed, but croton oil has seemed to me to be much more efficient as an eliminative and counter-irritant. The kidneys should be kept active, an ice cap placed upon the head, and leeches placed at the back of the neck. The treatment after this can be only that of sedation and support. If the patient is stuporous and has a high fever, small doses of aconite should be given. If he is asthenic, he should receive strychnine. Of course, the nourishment should be carefully attended to, and, if he suffers pain, he should have morphine. Chloral and bromide seem to be the most efficient agents for relieving the restlessness and insomnia.
Mui/tiplk Sclerosis.
Multiple sclerosis is a chronic and progressive malady charac- terized by some paralysis, usually in the form of paraplegia, by coarse tremor, disturbances of speech, nystagmus, apoplectiform attacks, and various other cerebral and spinal symptoms depending upon the seat of the lesion. It is due to the development of sclerotic patches in the different parts of the brain and cord, which patches are for the most part the result of a neuroglia proliferation. The disease is probably an inflammatory rather than a degenerative one. It affects the spinal cord as well as the brain.
Etiology.- — It occurs rather more frequently in the male sex and is a disease of the first half of life. Multiple sclerosis is, in fact, one of the few chronic nervous disorders of organic origin developing at this time. Most cases begin between the age of twenty and thirty. Cases have, however, been observed in infants and children, but the trouble in its typical form does not appear in the declining years of life. While a few cases of hereditary multiple sclerosis have been reported, it is generally conceded that hereditary influence is very slight and even a hereditary tendency to degenerative disease or a neuropathic family history is rare though not unknown. Strumpell's theory that the disease is endogenous has not been sustained. There can be no doubt that the most important of all of the few causes of multiple sclerosis is infection. This is so true that it may be called a post- infectious disease. The infectious disorders which are followed by sclerosis are typhoid fever, pneumonia, malaria, and the eruptive fevers. Among these malaria and typhoid are the more important. It has been known to follow also diphtheria, whooping-cough, erysipe- las, dysentery, cholera, and even rheumatism. Workers in metallic poisons, such as lead, copper, zinc, and even manganese may develop
DISEASES OF THE BRAIN.
459
sclerosis. The influence of trauma in causing multiple sclerosis has to be very carefully weighed. It is certainly very rare, but there seem to be some few cases in which trauma appeared to be an exciting cause (Keellich) . It is generally accepted that neither syphilis nor alcoholism are causative factors.
The following data as to the frequency and cause of the disease were collected by Dr. C. W. O. Bunker, in his neurological Prize Essay:
Frequency. — Among 70,000 neurological cases in America the per- centage of multiple sclerosis was 1 in 266, or 0.058 per cent. In Scot- land it is about 2 per cent.; in Berlin, .85 per cent.; in Hamburg, 1 per cent.
Age. — It is a disease of early adult life, from 20 to 40, but it may occur in childhood and, perhaps, even in infancy. The cases occuring after 50 are doubtful or atypical. The following table, compiled by Bunker and myself, represents American cases. It makes the incidence of the disease too late. Practically, the cases occur between the fifteenth and thirty-fifth years.
-10, 9
10-20, 17
20-30, 36
30-40, 45
40-50, 32
50-60, 24
60-70, 7
70-80, 4
The disease is twice as frequent in men as women — 129 to 67 ; statistics of Jelliffe, Bunker and myself. European statistics make the numbers about even. The disease is seen more frequently in foreigners, perhaps, because our clinics are so largely foreign in popu- lation. My private cases are nearly all American.
Symptoms.— The disease begins insidiously. There may be a pre- liminary and rather short hemiplegic or paraplegic attack with some sensory disturbance. More often a comparatively short time after re- covery from malaria or some acute fever, the patient begins to suffer from weakness of the lower limbs with stiffness and some degree of numbness and ataxia. The bladder is also a little weak, and it is diffi- cult to retain the urine. In fine, the symptoms are very much like those of the onset of myelitis. These symptoms after a few weeks may disappear and the patient be well for months. If not, very soon the patient notices some unsteadiness in the gait, due not alone to weak- ness in the legs, but to an increasing degree of ataxia. He finds also that his hands are trembling and that this tremor increases upon voluntary motion. It is the type of tremor known as "intentional." He has at this time also some indistinctness in speech, it being diffi- cult for him to enunciate long words. These come out in a slow,
460
DISEASES OF THE NERVOUS SYSTEM.
syllabic utterance, as it is called, each syllable being spoken sepa- rately. He may have also a little trouble in swallowing. By this time he has had some sensation of numbness in the limbs, and some pains occasionally in the joints and extremities, but the sensory troubles are not very marked and are mainly parsesthesic. If he is examined by a physician three or four months after the beginning of the malady, it will be found that the gait is stiff and awkward, the patient walking somewhat like a drunken man; or, in other cases, it may simply be the stiff, weak gait of moderate paraplegia. The Romberg symptom will
Fig. 212. — Pallor of entire optic disc except its nasal margin. (Holcbn.)
be found to be present to some extent. The knee-jerks are exaggerated and ankle clonus may be present. The abdominal reflexes are absent in a good many cases. The hands are unsteady, and the movements are characterized by a jerky tremor, which may be so great that the patient has difficulty in dressing and feeding himself. This tremor dis- appears almost entirely if the patient lies flat upon his back. If he sits up, however, it may be seen perhaps in the muscles of the neck, causing the head to be oscillated, and constant more or less regular tremor in the arms is present. The speech becomes more thick,
DISEASES OF THE BRAIN.
461
and often is almost unintelligible in the severer cases. Examina- tion of the eyes shows a nystagmus, perhaps only when the eyes are turned to one side, but often the jerky movements are seen, even when the patient is told to look directly at an object. Evidence of impaired eyesight appear with pallor of the temporal side of the optic disc (Fig. 212). The tongue is protruded in a jerky way, and attempts at swallowing are often awkward. If a glass of water is handed him, the patient seizes it, but in carrying it to his mouth he agitates it so violently that the fluid is spilled and perhaps the tumbler drops from his hands. Examination of the mus- cular system shows some weakness of the legs or possibly some degree of hemiplegia. There is, however, no marked atrophy of the limbs, and no change of any moment in the electrical reactions. There are many cases in which the jerky tremor, syllabic speech and nystagmus are not marked or develop very slowly, and more recent studies show that the most common symptoms are :
1. Spastic paraplegia.
2. Optic atrophy.
3. Sensory defect.
4. Unsteady gait.
5. Sphincter trouble. (
6. Remittent course.*
Examination of the cutaneous sense may show some little tactile anaesthesia in the limbs, but this is not always present. There is a certain amount of ataxia, which is not so much due to the muscular anaesthesia as to inability to control and co-ordinate the movements. There is no loss of sense of weight or of pressure. Of the nerves of special sense, the eye is most frequently involved. The patient may have some diplopia from paralysis of one of the eye-muscles. The pupils react to light and accommodation. Examination of the fundus of the eye shows a certain amount of atrophy of the optic disc in the temporal half, a characteristic condition in this disease. In later stages this may go on to complete and general atrophy of the nerve. On account of this involvement of the optic nerve, the patient suffers from contraction of the visual field, central scotoma, scotomata, and weakness of vision. Examination of the cerebrospinal fluid by lumbar puncture shows the presence of a slight lymphocytosis.
Sometimes he develops the above symptoms to only a moderate extent a short time after the infectious fever. He then gradually im-
*The less frequent symptoms are vertiginous attacks, epileptiform attacks, ocular palsies, amblyopia, paraplegic attacks. Transitory attacks of hemiparaesthesiae, hemi- plegia or paraplegia, amblyopia and diplopia occur sometimes very early and even years before the onset of the disease.
462
DISEASES OF THE NERVOUS SYSTEM.
proves and gets nearly well. Some traces of the malady, however, still linger. After a number of years it begins to develop again, and it then progresses steadily. Thus an examination of the history of a case in which the disease apparently began well along in adult life will show that the beginning of the trouble dated back before the time of adolescence.
While the disease is running the course just described, the patient sometimes suffers from attacks of vertigo, and occasionally from sud- den seizures resembling apoplexy, and even epileptiform attacks may occur. The mind is usually not much affected. There may be some slight dullness of the intellect, some hebetude, or even a slight amount of melancholia. In certain cases the patients are subject to attacks of impulsive laughing; that is to say, without any sufficient cause they suddenly break out in exaggerated laughter, from which they quickly recover themselves. These attacks may be frequently repeated. As the patient gets worse the paraplegia increases and he becomes finally helpless. The use of the arms is preserved for a longer time. Some involvement of the sphincters of the bladder and rectum eventually occurs and the patient has difficulty in holding his urine and in expelling or holding the feces.
The progress of the affection is variable. It usually goes steadily on without remissions, reaching finally in two or three years a chronic stage, in which the patient remains for several years without much change. At other times the progress of the disease is hastened by re- peated exacerbations, accompanied by apoplectiform or hemiplegic at- tacks. In still other cases the amelioration continues and remains permanent, and a practical cure takes place. The various symptoms of the disease are classified as cerebral, cerebellar, and spinal. The cerebro-cerebellar symptoms consist in modifications of speech, attacks of vertigo and apoplectiform seizures, hemiplegia, intention tremor, mental changes, optic atrophy and spasmodic laughing or crying. There is sometimes also a certain amount of deafness and perversion of taste and smell. Finally, lesions in the brain may give rise to a cere- bellar ataxia, due to the development of the disease in the cerebellum.
Under the head of spinal symptoms are included the spasmodic paraplegia, with some bladder and sexual weakness, and a slight amount of sensory trouble. There may be also some weakness of the arms. Occasionally there are noted some trophic troubles, such as splitting of the nails and atrophy of the muscles.
Aborted Types or "Formes Frustes"of Multiple Sclerosis. — In some cases the nodules of sclerosis are so limited in number and so peculiarly placed that they give rise to very atypical forms of the dis-
DISEASES OF THE BRAIN.
463
ease. Perhaps the most common one is that in which the disease takes the type of a progressive spastic paraplegia. The patient suffers from weakness of the lower limbs, accompanied by stiffness, cramps, exaggerated reflexes, and disturbances in the bladder and rectal functions. Anaesthesias, pain, and the girdle symptom may develop. In addition to this, however, a close examination will show some evi- dence of disease of the optic nerves and perhaps disturbances of the eye-muscles. The patient will have nystagmus, diplopia, or other visual disorders. There will also be some attacks of vertigo or of epileptoid convulsions. The combination of the eye symptoms with
the progressive paraplegia will often reveal the true character of the disease.
Pathology. — Grayish nodules are found distributed through the brain and spinal cord (Figs. 214-215). They vary in size from a milli- metre to two or three centimetres in diameter (one twenty-fifth to one inch). They are of firmer consistence than is the surrounding brain substance, but are not quite so hard as is ordinary connective tissue. They consist microscopically of neuroglia tissue, which some assert to be connected with the walls of the blood-vessels, as a rule. Very often the axis cylinders of nerves can be seen passing through the lesion. The nodules are found most frequently in the white matter of the brain more especially in the pons, internal capsule, and centrum ovale. They rarely begin primarily in the gray matter, but may invade it secondarily. The roots of the peripheral, especially of the
464
DISEASES OF THE NERVOUS SYSTEM.
cranial, nerves occasionally contain or are surrounded by these sclerotic masses. In the spinal cord they may extend up and down the gray and white matter for a considerable distance, or they may involve the whole cord at a certain level, turning it into a fibrous mass. The blood-vessels surrounding and in connection with these diseased areas show some evidences of thickening and increased vascularity, but no true inflammatory process, as a rule. The primary pathologi- cal change in multiple sclerosis is as yet unknown ; many things point to its starting originally from small emboli or thrombi which lead
to minute softenings, with a secondary reparative and sclerotic process. The fact that the disease follows infective fevers makes such an origin of it seem probable. On the other hand, pathological anatomy does not yet bear out this view, and it is possible that the disease begins by a primary degeneration affecting first the myelin sheaths of the nerve-fibres sparing the axis cylinders and cell bodies, this being followed by a neuroglia and connective-tissue proliferation which ends in the formation of the small islands of sclerosis. An important pathological peculiarity of the process is that, while it destroys the
DISEASES OF THE BRAIN.
465
myelin sheaths of the nerves, the axis cylinders remain intact for a long time, and consequently conduction of nerve impulses takes place imperfectly, directly through the nodular masses.
The pathology of the disease is still in doubt, but the evidence increases that it is a form of inflammatory trouble; that the pernicious agent is a toxin which passes from the vessels and has a selective in- fluence upon the myelin sheaths. These are first destroyed, with en- suing neuroglia proliferations. The blood-vessels do not show much change, it is true, yet that the poison comes from the blood or lym- phatics or both seems probable. The character of the disease sug- gests that some protozoan or other microbe may yet be found under- lying it.
Course and Duration. — The disease runs a very irregular course. Its prodromal stage is long and remissions of considerable length occur. The disease may last from five to twenty years, the average duration being five to ten years. Death sometimes occurs from involvement of the nerves of the medulla, but more often from weakness and exhaustion or some intercurrent malady.
Diagnosis. — The diagnosis in typical cases is not very difficult ; but as, on the other hand, typical cases are not common, the disease has always to be studied with great care before certainty can be reached. The diagnosis is based upon the slow development of the disease, with attacks of vertigo, weakness, paresthesia and uncertainty in gait; also upon the paralysis of the extremities with intention tremor, ataxia, rigidity, and contractures; upon the disturbances of vision, nystagmus, and the speech troubles. The existence of early paralysis with sensory symptoms and then remissions is most important. The presence of headache, attacks of vertigo, apoplectiform attacks, and the peculiar mental condition often furnish help. The age of the patient and the cause should also be taken into consideration. The disease must be distinguished from Friedreich's ataxia, spastic spinal paralysis, loco- motor ataxia, dementia paralytica, bulbar paralysis, paralysis agitans, chronic meningitis, and hysteria. The points already given and those furnished under the heads of these different diseases must be utilized in making these distinctions. In the pseudosclerosis of Westphal, there are no ocular symptoms. In diffuse cerebral sclerosis, also, ocular symptoms are rare and there are progressive dementia and paralysis. In multiple sclerosis the pupils are rarely affected. The method of exclusion may be used with advantage in reaching the diagnosis of this protean malady.
Prognosis.- — The prognosis, while not favorable as regards the ulti- mate cure, is somewhat favorable as regards the remission and im- 30
466
DISEASES OF THE NERVOUS SYSTEM.
pro cement, and the disease on the whole is not so severe as is locomo- tor ataxia or the other degenerative disorders.
Treatment. — In the treatment the same measures recommended for other chronic diseases of the nervous system must be employed. Hygienic measures, electricity, and hydrotherapy have some thera-| peutic value. Internally, the use of large doses of iodide of potassion, the hypodermic injection of arsenic, the administration of nitrate of silver and of quinine and other tonics are advised. A very regular systematic, and quite mode of life, combined with the use of iodide of potassium and bichloride of mercury, has produced good results in my experience, even in cases which gave no history of syphilitic infection. The use of atropine or tincture of belladonna helps in the vertiginous attacks and seems to act favorably on the course of the disease. One of my patients got his remission while taking phos- phates and receiving daily application of the violet rays. These latter certainly do no good in advanced cases.
THE APOPLEXIES.
Apoplexy is a clinical term used to indicate a condition charac- terized by sudden paralysis, usually attended with loss of conscious- ness, and due to the breaking or blocking up of a blood-vessel in the brain.
Apoplexy is a general term. Particular forms are described in accordance with the cause of the apoplexy. These are:
1. Intracranial hemorrhage, from rupture of a blood-vessel (hem- orrhagic apoplexy.)
2. Acute cerebral softening, from embolism or thrombosis (em- bolic or thrombotic apoplexy).
Apoplexy from Intracranial Hemorrhage (Cerebral Hem- orrhage, Hemiplegia).
There are four groups of intracranial blood-vessels, those of the dura mater, those of the pia mater, and those supplying the basal ganglia and white matter. We may consider the vessels that supply the pons, medulla, and cerebellum, which are chiefly branches of the vertebrals as a separate group, subject to somewhat different mechani- cal conditions. Corresponding to this we have :
1. Dural or pachymeningeal hemorrhages.
2. Pial or subarachnoid hemorrhages.
3. Central hemorrhages.
4. Hemorrhages in the medulla, pons and cerebellum.
DISEASES OF THE BRAIN.
467
It is the central hemorrhages (No. 3), due to rupture of the blood- vessels going to the great basal ganglia, internal capsule, and white matter, that constitute the great majority of cerebral hemorrhages seen by the physician. It is this class that I have particularly in mind in the following description.
Etiology. — At the time of birth and during infancy there is a slight tendency to intracranial hemorrhage owing to the accidents and in- juries of labor. The hemorrhages at birth are usually meningeal and traumatic. After this period the liability is very small, but slowly increases up to the age of forty, when predisposition specially begins. Four-fifths of all cases occur after forty, and the tendency to hemor- rhages increases in each decade up to eighty, when it diminishes ab- solutely and relatively.* Males are slightly more predisposed than
Age.
Clinical cases in hospital and private practice.
Ten
Ten to twenty
Twenty-one to thirty. Thirty-one to forty . . Forty-one to fifty. . . . Fifty-one to sixty. . . . Sixty-one to seventy . Seventy-one to eighty Eighty-one to ninety .
27
2
19
28
35 54 28 10 1
204
females (115 males and 54 females in 169 cases). Rather more cases occur in cold weather, at high altitudes, in the temperature zone, and among civilized races.
Heredity has an undoubted though not great influence in predis- posing to cerebral arterial disease. A family tendency to early apoplexy is a stigma of degeneration. Infective fevers and maras- mic states are predisposing causes. Chronic kidney disease is pres- ent in one-third of the cases. Chronic alcoholism and gout are power- ful predisposing causes. Syphilis was present in 25 per cent, of my dispensary cases of apoplexy, but some of them were no doubt throm- botic. Still syphilis is of importance in causing cerebral hemor- rhage. Rheumatism is less important. Heart disease, fatty and atheromatous arteries, arteritis, and miliary aneurisms may be regarded as more than simply predisposing — they are determining causes. Leucocythaemia, scurvy, and purpura are conditions which also par- ticularly tend to cause hemorrhage. The so-called apoplectic habit — short, thick neck and high shoulders and florid face — has really some
468
DISEASES OF THE NERVOUS SYSTEM.
importance in the better classes. Congenital anomalies, such as a narrow thoracic aorta or inherited deficiency in the strength of the walls of the blood-vessels, also play a part. Any sudden physical exertion, such as straining at stool, the excitement at coitus or of a passion, eating a large meal and drinking a great deal of fluid, espe- cially alcohol, taking a cold bath, all may lead to rupture of an artery. Direct blows on the head may cause meningeal hemorrhage, rarely a central hemorrhage, for the central blood-vessels are well protected.*
The symptoms are the prodromal, those of the attack and acute stage, and those of the chronic stage.
Prodromal symptoms are rare except in syphilitic cases. When present the patient suffers from headache, insomnia, dizziness, numb- ness of the hand and foot on one side, and a failure of memory for words. He may have "full" feelings or even pain in the head and bad dreams at night. Nosebleed and irregular heart action sometimes occur. Often an attack comes or when the patient is feeling particu- larly well. The attack always comes on suddenly and may be accom- panied (i) by convulsions and coma, (2) by coma alone, or (3) it may come without loss of consciousness.
1. Initial convulsions are rare and generally mean a meningeal hemorrhage. They occur, however, in young children. When present they are unilateral or partial, as a rule, but may be general. 2. The common mode of onset is with coma. The patient, without warning, suddenly becomes dizzy, loses consciousness, and falls. The face is flushed, the pulse hard and rather slow, the blood-pressure may rise to over 200 mm., the breathing is labored and stertorous, the cheek on one side puffs out with each expiration, the eyes are partly closed, the eye-balls fixed or deviated to the paralyzed side, the pupils are contracted and rigid, the skin is bathed in sweat, the limbs are relaxed, but some evidence of hemiplegia is present; the urine may be retained or it and the feces involuntarily evacuated. The urine is usually of rather high specific gravity and often contains albumin, even when there is no renal disease. The temperature in severe cases may fall below normal during the first twelve hours, even to 960 F., but this is not the rule. It is the rule, however, for the temperature in a few hours to be f or i° higher on the paralyzed than on the sound side. If the case is rapidly fatal coma continues, respiration often assumes a Cheyne-Stokes character, the pulse becomes fast, the blood tension falls and symptoms of bulbar failure occur, the temperature gradually
*Among 324 cases of hemiplegia occurring in the Cornell clinic, carefully analyzed by Dr. T. W. Evans, he found less than 1 per cent, in which even the question of trauma could be raised. Among 120 private cases, there was a history of a blow and of syphilis in one.
DISEASES OF THE BRAIN.
469
rises, and usually reaches 1020 or 1030 F., until just before death, when it may sink again. Swallowing and speech become difficult, hypo- static pneumonia sets in, and the patient dies in from two to four days. In slower fatal cases the patient regains consciousness partially and then enters a condition of stupor or mild delirium. He is restless and suffers from headache. The temperature may continue normal for a time, but is usually higher on the affected side. At the end of two or three weeks it rises higher, pneumonia develops, the patient becomes unconscious, and death ensues. In the favorable cases, which constitute the majority, coma, if present, gradually passes away in from one to six hours, leaving the patient's mind somewhat weak and confused and his speech disturbed, or more rarely the intelligence may not be at all disturbed. During the first few days or weeks after the attack the physician finds that the prominent symptom is the hemiplegia. This affects the arm and leg most and the face least. Only the lower two branches of the facial nerve are involved, and the patient can shut the eyes. The tongue, if protruded, turns to the paralyzed side; the uvula is turned in various ways and its position is of no significance. There is often some evidence of cutaneous anaes- thesia of the paralyzed side, and less often hemianopsia and disturb- ances of hearing occur. In right-sided hemiplegia the patient, after recovering consciousness, is often unable to talk or to understand what is said. Examination shows that he has a motor or sensory aphasia (vide Aphasia).
The deviation of the eyes and head to one side usually disappears in a day or two. Occasionally there is a temporary ptosis. The pupils at first are contracted, that on the paralyzed side the more so; this condition disappears with returning consciousness.
The paralysis of the arm and leg is usually flaccid at first, and the limb falls heavily when lifted ; the reflexes are lessened or abolished.
Sometimes, however, rigidity sets in at once. This symptom occurs when blood has broken into the ventricles, and also in some meningeal hemorrhages. The skin reflexes are abolished or nearly so on the affected side.
The usual course of the temperature is for it to rise on the second and third day to ioo° F. or 1020 F., being ^° to i° higher on the para- lyzed side. In a few days it gradually falls, so that by the eighth to the tenth day it is normal.
If the temperature continues to rise after the fourth or fifth day, it is a sign of an extension or inflammatory reaction of the hemor- rhage. Hence the thermometer furnishes a very important criterion of the seriousness of the case.
470 DISEASES OF THE NERVOUS SYSTEM.
The varying course of the apoplexy is shown in the following diagram :
Health line.
3. Chronic stage.
4 to 8 weeks.
Improvement. \
Attack.
1. Death. 2. Death.
2 to 4 days. 2 to 4 weeks.
1 The Chronic Stage, Hemiplegia. — At the end of a month, if fever and symptoms of cerebral irritation have subsided, the chronic stage may be said to begin. The hemiplegia has improved, the patient can move the leg and arm a little, sensory symptoms have lessened, the mind is clear, headache has disappeared. Improvement con- tinues, though more slowly, for several months or even one or two years. During this time the patient is "a hemiplegic" (Fig. 216).
The hemiplegia affects the arm more than the leg, and the face least of all. The distal segments of the limbs, the feet and hands, are affected more than those near the trunk. The muscles that act bilaterally, such as those of respiration, phonation, and facial ex- pression, are but slightly involved. The paralysis is not strictly a hemiplegia, for the muscles on the sound side are somewhat weakened, as tests will show. In severe cases, especially in old people, even the visceral muscles, especially those of the bladder, are weakened. At the onset of the attack there is sometimes a temporary "initial" rigidity of the muscles on the paralyzed side, or an "early" rigidity may develop in one or two days. There always develops at about the beginning of the second week a "late" rigidity. This, which at first is slight, gradually increases, and finally contractures affect the paralyzed limbs. The superficial reflexes, which at first were absent, reappear; the tendon reflexes become much exaggerated, and clonus can be obtained in the leg and arm. The sound side shares to a small extent in these conditions. The contractures affect the ex- tensors of the foot more than the flexors, and bring the toe down and the heel up. The leg is held nearly extended, and the limb in walking is swung around, the toe scraping the ground. The shoulder is ad- ducted, the forearm flexed, and the fingers are tightly shut into the
DISEASES OF THE BRAIN.
471
palm by the overaction of the flexors (Fig. 216). The facial muscles show a slight contraction and drawing to the affected side. The muscles on the paralyzed side do not waste. In infantile hemiplegia, however, the affected limbs grow less than those on the sound side.
The paralyzed limbs may be the seat of peculiar disorders of move- ment. These consist of
Associated movements.
Tremor.
Ataxia.
Choreic movements.
Continuous or athetoid move- ments (Fig. 217).
Spastic movements and cramps.
Such movements, aside from those that are spastic, are rarely seen in the hemiplegia of adults.
The electrical irritability may be at first slightly increased or di- minished, but the change is small in amount and never reaches the degenerative stage. The direct muscular irritability is at first in- creased on the affected side (Dana).
Hemianaesthesia, if present at first, disappears to a great extent, leaving only residua about the feet and hands. Paraesthesiae are com- mon. In rare cases the patient suffers great pain in the arm and leg. This pain is generally of a burning character and very ob- stinate and distressing. Cramping _ , _t . , . . . . .
r Fig. 216. — Chronic hemiplegia with con-
pains in the legs and arms are com- tractures. (Curschmann.)
mon in the severer cases.
During the first few weeks after the onset, joint inflammations and bedsores may attack the affected side. The temperature of the hemi- plegic side is usually a very little higher than that of the sound side.
Vasomotor disturbances, sweating, skin eruptions, and increased growth of hair are some of the rarer symptoms.
The mental condition is more or less affected. The patient be- comes irritable, cries easily, and is in general more emotional. The
472
DISEASES OF THE NERVOUS SYSTEM.
memory is impaired, and the power of concentrating the attention and carrying on work is less. Sometimes a progressive mental dete- rioration sets in and epilepsy or insanity develops. The mental dis- turbance is greater in old people and depends somewhat on the size of the hemorrhage. Those forms which produce serious aphasia especially limit and lessen mental activity.
Meningeal Apoplexy. — Aside from the apoplexies due to rup- ture of the central arteries and involvement of the basal ganglia just described, there are a minor number in which the meningeal arteries, the cerebellar, or some branch of the basilar are affected ; hence we have meningeal, cerebellar, and pons hemorrhages.
Fig. 217.- — Showing athetoid movements of hands. (Curschmann.)
Hemorrhages from the vessels of the dura mater are usually due to a rupture of the middle meningeal artery or vein or some of the cerebral veins as they pass to the sinus, and this is especially true in such hemor- rhages as are the result of injuries to the head. The causes are injuries to the head, including obstetrical injuries, alcoholism, and insanity.
In dural hemorrhages the result of head injuries, the clot is some- times intradural, lying in the arachnoid space, and sometimes epidural, lying between the bone and the dural membrane. The extradural hemorrhages are perhaps a little more common in surgical experience ; the intradural in medical and especially asylum experience. In over one-half of extradural cases there is an interval of consciousness lasting from a few hours to two months, but usually only a few hours, be- tween the accident and the time when distinctive cerebral symptoms develop. Then the patient gradually becomes dull, somnolent, and
DISEASES OF THE BRAIN.
473
finally comatose. Along with the gradual or rather sudden loss of con- sciousness there develops a hemiplegia upon the side opposite the clot. This is usually not complete, though it may become so. It is much less common in extradural hemorrhage. Anaesthesia is rarely present. The reflexes are generally somewhat exaggerated, and there may be considerable rigidity. Spasmodic movements of some kind occur in nearly half the extradural cases and in more than half of the intradural. These spasmodic movements may involve the whole of the affected side, or may simply affect the eyes and the facial muscles. They consist of irregular twitchings. The pupils are usually somewhat contracted, more so upon the paralyzed- side. When there is a dilated pupil on the side of the lesion and a small pupil on the opposite side, it is known as the Hutchinson pupil, and means a severe brain compression involving the third nerve at the base. The eyes are generally both turned toward the affected side and away from the lesion. The pulse is hard, slow and full; the tension high; the respiration is rarely stertorous, though it may sometimes be so, and Cheyne-Stokes respiration may be present. In these cases the clot is very large and the compression great. Aphasia may be present if the clot is upon the left side. The temperature may be raised one or two degrees, or it may be normal. The progress of the disease is usually steadily fatal unless surgical interference is undertaken. The coma deepens, the respiration becomes stertorous and then embar- rassed, the pulse gets rapid and weak, and the patient dies. With surgical interference (since 1886), between two-thirds and three- fourths of the cases are saved (Scudder and Lund).
Dural hemorrhages occurring idiopathically are due sometimes to the rupture of a meningeal artery, but usually to rupture of the veins of the pia mater or to the rupture of new vessels formed in a pachy- meningeal exudate. This idiopathic hemorrhage is rare in ordinary practice, but is not specially so in insane asylums or in large city hospitals. This is because the two great causes of this type of hemor- rhage are insanity and alcoholism. General paresis is the form of in- sanity with which it is oftenest associated. In the case of alcoholics, it is probable that injuries from blows are an exciting factor in the production of the hemorrhage, these occurring while the patient is in a state of intoxication. The symptoms of idiopathic hemorrhage are extremely variable, owing to the complicating influences of the in- sanity and alcohol. The patient after suffering from headaches or vertigo becomes suddenly comatose and shows marked evidences of hemiplegia and even of anaesthesia. Rigidity of the paralyzed side is often present, and sometimes spasmodic movements are observed.
474
DISEASES OF THE NERVOUS SYSTEM.
On the other hand, at times the paralysis can hardly be observed, and the patient is in a semicomatose state, has a muttering delirium, and presents the general aspect of a person suffering from the oedema or "wet-brain" of alcoholics. In dural hemorrhages occurring in paresis, the patient usually without warning becomes unconscious, and he often has some convulsive symptom and a hemiplegia develops. In these cases there is often a rapid improvement, and the patient gets partly well, usually experiencing other attacks later.
Pial apopeExy occurs very rarely, and the most frequent cause is trauma associated perhaps with syphilis and alcoholism. In many instances very slight localizing symptoms occur, and no absolute diagnosis can be made. If the hemorrhage, however, is in the motor area of the cortex, local spasmodic movements and some hemiplegia are observed. The most characteristic symptoms are the sudden incom- plete hemiplegia, involving, perhaps, mainly an arm or a leg, associated with local spasmodic movements, resembling Jacksonian epilepsy.
Thalamus Apoplexy. — A hemorrhage here causes a temporary hemiplegia, with anaesthesia, which latter symptom continues. There may be later dysthesiae and pains of the extremities and perhaps athetoid or choreic movements. Sometimes there is hemianopsia and generally the deep reflexes are not exaggerated.
Hemorrhage in the Medulla is extremely rare. If of any size, it causes instant death; if small, it will leave symptoms similar to those of softening. I have never seen a case.
Hemorrhage op the pons is also rare, but they make up two per cent, of my cases. The cause is much the same as that for cerebral hemorrhages, though syphilis is less often a factor. In nearly half the cases the hemorrhage is a large and immediately fatal one. In the majority of cases it is a small focal hemorrhage from rupture of a transverse or median branch of the basilar artery.
The patient has some prodromal headaches and malaise for a few days, with vertigo and sometimes vomiting. Then he falls suddenly as if by a lightning stroke, into a coma, usually very profound. There are twitchings of the face or of the limbs or both, but rarely any general convulsion (three cases). The face is flushed, there is a fall of tem- perature and the pulse is irregular, but not markedly increased or slowed; as a rule, the respiration is slow, 4 or 6 per minute, or more often irregular, and of Cheyne-Stokes type. The pupils are con- tracted to a pin-point, and do not respond to light, but maybe uneven. There is convergent strabismus or conjugate deviation of the eyes. The limbs are at first stiff, but may be relaxed later and the reflexes increased.
DISEASES OF THE BRAIN.
475
The patient cannot be aroused, but can be made to vomit. A few hours later the temperature rises, sometimes very high — 106 to 108 — but usually not. There are involuntary movements of the bowels, swallowing is impossible; evidences of dyspnoea are shown. The pulse becomes more rapid, cyanosis develops, the patient dies in six to twenty hours, usually with evidence of paralysis of respiration.
If the hemorrhage is less severe, the coma is less profound or may be absent, the patient shows signs of agonizing suffering and difficulty in respiration, evidences of crossed paralysis, crossed anaesthesia and disturbance of equilibrium and ataxia can be noted.
In still smaller lesions we find no such severe general symptoms, but only those due to a circumscribed focus in one side of the pons, because the smaller lesions are unilateral. The syndrome of pons lesions then is :
1. Headache, malaise, vomiting.
2. Sudden and profound coma.
3. Twitching of the face and limbs or both.
4. Myosis and convergent strabismus or conjugate deviation (away from the side of the lesion).
5. Slow, irregular breathing.
6. Irregular pulse.
7. Dysphagia.
8. Paralysis of limbs or crossed paralysis and exaggerated reflexes.
9. Gradual rise of temperature, sometimes to high point.
10. Death inside of twenty-four hours.
Cerebellar ApopeExy. — Hemorrhage into the cerebellum occurs in one or two per cent, of all fatal cases. Its recognition is very diffi- cult. There is sometimes a preliminary period of severe headache, lasting several days. In other cases the patient at once falls into a state of profound coma, with stertorous respiration. Vomiting sometimes occurs. There may be some hemiplegia, and if so this is on the side of the lesion, owing to this pressure on the motor tract. Distinct evidences of hemiplegia, however, are not always observed. The condition of the pulse and arterial system is very much like that of ordinary apoplexy, but the respiratory system is usually more seriously affected. Disturbances in the movements of the eyes and in swallowing, and in fact all those symptoms which show a pressure or irritation due to blood oozing into the fourth ven- tricle may be present. Death is almost sure to occur, and is in- evitable if the hemorrhage, as is so often the case, breaks through and reaches the fourth ventricle.
If the hemorrhage is small and does not break into the ventricle,
476
DISEASES OF THE NERVOUS SYSTEM.
the patient suffers from intense vertigo which oblige him to lie per- fectly still in a horizontal position. With or preceding this he may fall down and have convulsive movements or forced and irregular tonic contractions of the muscles; in other words, a cerebellar fit. If this passes away the vertigo also may pass, and the patient gets nearly well, but is left with some feeling of uncertainty in gait, and with tinnitus cerebri and tendency to vertiginous and cerebellar attacks. Pathology and Morbid Anatomy. — Spontaneous intracranial hem-
Fig. 218. — Showing the different areas commonly involved in cerebral hemorrhage. Art. L.S., lenticulo-striate artery; Art. L.O., lenticular optic; Art. O.E., external optic.
orrhage is always due to the presence of diseased blood-vessels in the brain. This diseased condition consists of :
1. A degenerative arteritis which results in producing small aneurisms. 2. A fatty degeneration of the vessel walls. 3. Besides this, in most cases the larger blood-vessels are atheromatous.
1. The arteritis produces small or miliary aneurisms which affect only the smaller arteries, especially those of the central group. They may be fusiform or sacculated in shape; they range in size from one- fourth to one millimetre ( ytto to 2T m-) m diameter. They are usually
DISEASES OF THE BRAIN.
477
not very numerous, but there may be as many as a hundred in the brain. They are the results, not of inflammation, but of a degenera- tion which affects first an area in the internal coat; this causes local weakness and consequent dilatation; secondarily there is a periarteri- tis. These aneurisms occur almost exclusively during the degenera- tive period of life.
2. Fatty degeneration of the walls of the small cerebral arteries occurs in purpura, scurvy, leucocythaemia, marasmic conditions, and post-infective states, especially in early life, and is the common cause of hemorrhage at that time.
3. Atheroma affects the larger vessels only. It is indirectly a cause of hemorrhage by lessening the elasticity of the vessel wall. Atheroma is present in from one-eighth to one-fifth of all cases. Hypertrophy of the heart is a factor in causing hemorrhage, and such hypertrophy exists in about forty per cent, of cases. Emboli lodged in the cerebral arteries may cause hemorrhage by suddenly stopping the arterial circulation and raising the blood-pressure. Hemorrhages are found by far the oftenest (twenty per cent.) in the caudate and len- ticular nuclei and adjacent parts. The lenticular and lenticulo-striate branches of the middle cerebral are oftenest affected; next the branches of the anterior cerebral to the caudate nucleus and the lenticulo- optic branches of the middle cerebral. The branches of the posterior cerebral break more rarely. The parts affected in hemorrhage, in order of frequency, are about as follows:
The following statistics from the records of Presbyterian, New York and Bellevue Hospitals, collected by Dr. Hoobler and myself, show the site of lesion.
In
Hemorrhage
and Softening.
Frontal lobe Parietal lobe Occipital lobe Temporal lobe Corpus striatum Optic thalamus Internal capsule Ventricles Corpus collosum Corpora quadrigemina Piae and cortical Pachy meningeal Pons
Cerebellum
24 14 6
14
62
25 28
64
8 7
3
1 r
1
4 17 4 9 21 6 6
1
25s
68
478
DISEASES OF THE NERVOUS SYSTEM.
These figures show that over three-fourths of all the hemorrhages occur from rupture of the central or ganglionic arteries.
Cortex hemorrhages are generally small and may be subarachnoid or may break through into the arachnoid cavity. Ventricular hemor- rhages are almost always secondary to a rupture into the neighborhood of the basal ganglia. Pons hemorrhages occur usually in the median line. Cerebellar hemorrhages are oftenest due to rupture of the superior cerebellar artery. They usually cleave their way externally and break into the fourth ventricle. Dural hemorrhages are due to rupture of the meningeal veins and arteries and of the vessels in newly organized clots. They lie in the arachnoid cavity and flatten the convolutions.
The reparative changes after a hemorrhage take the following course: ist, Coagulation of the blood, which in a few days begins to soften and become absorbed. 2d. Formation of a fibrinous wall about the clot. This occurs from the seventh to the ninth day. 3d. Forma- tion of a cyst with transparent fluid contents, and perhaps fibrous trabecule running through it, twentieth to thirtieth day. 4th. Con- traction of the cyst wall, which begins by the fortieth day. 5th. Secondary degenerations begin from the tenth to the fourteenth day.
Physiology. — The blood pressure of the cerebral arteries is equal to about 155 mm. of mercury. The resistance or support furnished by the surrounding tissue is equal to about 10 mm. of mercury. Hence there is a special liability to rupture of intracranial vessels. The middle cerebrals are most often affected, because they are in the most direct line from the heart and are nearest to that organ. The pressure lessens as the arteries subdivide and get farther away from the heart.
The diagnosis of hemorrhagic apoplexy must be made from alco- holic coma, uraemic coma, opium coma, epilepsy and hysteria; acute softening from embolism and thrombosis.
From alcoholic coma the diagnosis is made by the odor of the breath, the incomplete coma, the equal pupils, the absence of low or unequal temperature, the absence of paralysis.
From uraemic coma by the absence of albumin and castsin the urine, though their presence does not surely indicate uraemia; by the unequal pupils, the temperature, the absence of hemiplegia and the presence of the physiognomy peculiar to cases of chronic Bright's disease.
From opium poisoning by the history, the stomach contents, the presence of equal and contracted pupils, the slow respirations, the temperature, and the absence of paralysis.
From epilepsy by the history of the onset with epileptic cry, the dilated and equal pupils, the biting of the tongue, the absence of hemi- plegia, the rather rapid return of consciousness.
DISEASES OF THE BRAIN.
479
Hysterical attacks present little semblance to that of apoplexy; hysterical hemiplegia is characterized by its flaccidity, by its not in- volving the face, and by the presence of the anaesthesias and others hysterical stigmata.
In embolic softening the earlier age of the patient, the presence of decided valvular heart disease, the parturient condition, the slighter degree and shorter duration of coma, the absence of serious disturbance of temperature, the onset first of paralysis and then of convulsive movements and coma — all lead to a presumption in favor of embolism.
The presence, on the other hand, of a congested face, tense pulse, and throbbing carotids favors the existence of a hemorrhage.
From thrombotic softening diagnosis is more difficult. The oc- currence of prodromata, consisting of slight seizures quickly recovered from, the slighter degree of coma, the advanced age, hard atheroma- tous arteries, low blood-pressure, evidence of anaemia and asthenia, weak or fatty heart, the absence of stertorous respiration, flushed face, and unequal temperature not much lowered or raised, the slight pupillary disturbance, and absence of convulsions point to thrombotic softening. Evidence of a lesion in the pons or cerebellum suggests hemorrhage, while evidence of lesion in the medulla points almost surely to soft- ening. The existence of a very high blood-pressure will often help the diagnosis in all cases, but especially from softenings.
Dr. Burr has suggested the possibility of using the X-ray in diag- nosticating cerebral hemorrhage, and he was able to demonstrate this in one case. The examination of the cerebrospinal fluid by lumbar puncture will promptly show the presence of a hemorrhage if it is meningeal or ventricular.
Several authors have found large granular macrophages containing red blood-cells in the cerebrospinal fluid in cerebral hemorrhage, but these are not always found (Hastings). The examination of the urine for haemoglobin has also been considered a diagnostic test.
The chances in any case between the ages of thirty and fifty, if there is no heart disease, are six to one in favor of hemorrhage.
The diagnosis of the location of the lesion has already been indicated, except for the central hemorrhages. These represent four clinical types :
1. That due to rupture of the lenticulo-striate branch of the mid- dle cerebral with injury of the lenticular region and internal capsule in its more anterior part. This gives the picture of the usual type of hemiplegia as described with no marked sensory symptoms.
2. If the lenticulo-optic is involved, the lesion is further back, these is less hemiplegia and the leg is more involved, there is involvement of sensation, muscular and cutaneous, and aphasia is less marked.
DISEASES OF THE NERVOUS SYSTEM.
3. If the artery to the optic thalamus is involved, which is a branch of the posterior cerebral, there is a temporary hemiplegia with hemi- angesthesia. After the paralysis has disappeared, there develop dys- thesias and pains in arm and leg of the opposite side; and some hemi- ansesthesia may continue to persist, and especially a loss of deep sensibility.
4. Ventricular hemorrhages are usually the result of a very exten- sive lenticulo-striate lesions, the blood finally bursting into a lateral ventricle and thence permeating into all the ventricles and even to the subarachnoid space, so that a diagnosis can be made or con- firmed by a lumbar puncture. The symptoms are characterized by the severity of the coma, giving way of the medullary centres, a motor restlessness, increased rigidity of the limbs, and sometimes evidence of bilateral involvement. The eyes and head are generally turned to the side of the lesion, the pupils contracted, loss of power over the sphinc- ters, sweating, vaso-motor disturbances, rise of temperature and ir- regular respirations, all are usually present, indicate a serious cerebral lesion. The blood-pressure, which is high when there is a large clot on the brain substance, falls if it breaks into the ventricles.
Prognosis. — The majority of cases get over the first attack. They are very liable to have another within one to five years. The minority recover from this. Few survive a third attack. The prognosis of the attack itself depends on the severity of the coma and paralysis, the dis- turbance of temperature and of respiration, the evidence of rupture into the ventricles, the development of decubitus, the continuance of loss of control over the bowels and bladder.
If profound coma continues four days there is little hope; if fever develops and continues steadily, of if there is initial subnormal tem- perature, or if signs of bulbar compression and paralysis occur, the prognosis is grave.
If the patient passes the first week with little or no fever and con- sciousness has returned, the prognosis is good.
The presence of renal disease and of alcoholism is bad. Develop- ment of slight delirium which continues is unfavorable.
Cerebellar and pons hemorrhages are very fatal, meningeal slightly less so.
The prognosis of the chronic stage has been given under symptoms.
Improvement continues rather rapidly for three months, then very slowly. Improvement may continue for one or two years. Com- plete recovery is very rare. The great danger after middle age is recur- rence of the attack.
DISEASES OF THE BRAIN.
Treatment of the Attack. — The patient should be laid in a horizontal position and kept quiet. Ice should be applied to the head and hot bottles at the feet. The feet and legs should be swathed in cloths wrung out in hot water containing mustard, a cupful to a pail of water. A laxative should be given, either one or two drops of croton oil or a quarter of a grain of elaterium. If there is evidence of intense cerebral congestion, the pulse being very full and hard and the heart beating strongly, bleeding eight to ten ounces is justifiable. This should be tested with an instrument for measuring blood-pressure. Ordinarily it is better to give a drop of tincture of aconite every twenty minutes for two or three hours. Pressure on the carotid of the sound side and even ligature of it have been recommended, but there is no experience yet to justify either. Administration of bromide of sodium and enemata of ergot have been advised, but are of doubtful value. After the first twelve hours, treatment must be symptomatic. Should delirium and other evidence of mental irritation appear, large blisters must be applied at the back of the neck and an elaterium purge given if the patient is not too weak. The use of iodide of potassium or mer- cury is not indicated unless the case is distinctly syphilitic. The pas- sage of a galvanic current through the brain cannot possibly do any good. Great care should now be taken that the patient does not de- velop pneumonia. The mouth and pharynx should be cleansed anti- septically, and the patient should not be allowed to remain in one position. If there is sufficient evidence of a meningeal or cortical clot, trephining should be seriously considered.
At the end of three or four weeks the faradic battery may be used carefully on the affected limbs. A seance of fifteen minutes daily for four to six weeks should be given, then treatment should be suspended for a fortnight, to be begun again and kept up systematically for a year if need be. Massage may be alternated with the electricity. When contractures develop the stabile galvanic current may be tried, though it does little good. Static sparks, however, are helpful; luke- warm baths should be tried and measures used to produce hyperex- tension of the affected parts.
Internally during this time the patient is to be given courses of iodide of potassium, tonics, and laxatives if needed. The patient should be made to live a quiet life, preferably in a warm, equable climate. The kidneys should be kept active and arterial tension low. For these purposes nitroglycerin should be given and at times small doses of chloral, and the diet should be simple and rather non-nitro- genous. Strychnine in very small doses (gr. sometimes helps the contractures; as also do the bromides and physostigma. 31
482
DISEASES OF THE NERVOUS SYSTEM.
Acute: Softening of the Brain (Embolism, Thrombosis).
Acute softening is a condition caused by the plugging of a blood- vessel with an embolus or thrombus, and is characterized by a sud- den apoplectic seizure; the symptoms eventually running a course like those of cerebral hemorrhage.
Etiology. — Embolism occurs rather more often in women, throm- bosis in men. Embolism is rare in children; it occurs oftenest be- tween the ages of twenty and fifty, thrombosis between the ages of fifty and seventy. The most important predisposing factors in em- bolism are acute or recurrent endocarditis, infectious fevers, profound anaemia, pregnancy, and blood dyscrasias; in thrombosis, arterial scle- rosis from any cause, syphilitic, lead, or gouty arteritis, fatty heart, and blood dyscrasias. Severe gas poisoning leads sometimes to thromboses The same causes which lead to the arterial disease which produces cerebral hemorrhage also predispose to thrombosis, though in the latter condition atheroma plays the important part.
Symptoms. — In embolism there are rarely any premonitory symp- toms; the onset is sudden; it may begin with some convulsive twitch- ings, then follow hemiplegia and temporary loss of consciousness. Coma, however, is rarer than in hemorrhage, and if present is usually shorter. There is rarely vomiting, nor do we find the hard, pulsating arteries, flushed face, and severely stertorous breathing. The initial temperature changes are slight, but in a few days fever may develop.
In thrombosis premonitory symptoms are frequent. In syphilitic cases there are headaches and cranial-nerve palsies. In other cases vertigo, temporary aphasia, insomnia, transient hemiplegia, numbness of the hand and foot, and drowsiness may be present. The onset is more gradual; the hemiplegia slowly develops, taking several hours, perhaps, for its completion; meanwhile the patient gradually becomes comatose. The attack sometimes is rather sudden, with no loss of consciousness, and it may occur in sleep. The temperature often has a slight initial fall, followed by a rise, just as in hemorrhage. In both embolism and thrombosis the hemiplegia tends to improve very much in a few days or weeks unless the vessel obliterated is a large one. Embolism is rather more apt to affect the left side of the brain, though the difference is not great. The middle cerebrals are most frequently affected (seventeen out of twenty-seven cases). Softenings affect the vertebrals, basilar, and posterior cerebral arteries more often relatively than do hemorrhages; then the initial symptoms may not present the character of hemiplegia, but of a bulbar paralysis. Acute softening
DISEASES OF THE BRAIN.
483
may kill within twenty-four hours, but, as a rule, the patient survives the onset, and if he dies it is not for several weeks. After the acute stage is over the patient passes into the chronic stage, which resembles in nearly all respects that of hemorrhage. After an acute softening, however, it is believed that there are more spastic symptoms and a greater tendency to mobile spasm. In embolism, owing to the youth and freedom from arterial disease, the mind is less affected; while in thrombosis the contrary is the case.
In acute softening from gas-poisoning, a branch of the anterior cerebral artery which supplies the caudate and part of the lenticular nuclei is particularly affected so that a symmetrical softening of both lenticular nuclei is almost a characteristic of this accident. This is accompanied with coma, and vasomotor and trophic disturbances of the limbs at times.
Pathology. — The embolic plug cuts off the blood-supply from a certain area of brain tissue. In twenty-four hours this begins to soften. If the area is in the cortex it becomes red (red softening) ; if in the white and less vascular part, it is usually white with a few red punctate spots. The red softening gradually becomes yellow (yellow softening). The dead tissue gradually is absorbed, leaving a cicatrix or cyst. If the embolus contains infective microbes, there may be a local encephalitis and abscess.
In thrombosis there are usually evidences of extensive atheroma or syphilitic arteritis. In those instances in which the thrombosis is caused by the blood state and a weak heart, little arterial change oc- curs. Atheroma affects chiefly the internal carotids and the large arteries at the base, viz., the middle, anterior, and posterior cerebrals and the basilar and vertebrals. The branch of the anterior cerebral supplying the caudate and part of the lenticular nucleus is called ''the artery of cerebral thrombosis."
Among seventy-two cases of cerebral softening with autopsy col- lected by Dr. B. R. Hoobler from hospital records (Neurological Prize Essay), the site of the lesion was as follows:
|
Frontal lobe |
6 |
|
Parietal lobe |
17 |
|
Occipital lobe |
4 |
|
Temporal lobe |
9 |
|
Caudate nucleus |
5 |
|
Lenticular nucleus |
16 |
|
Optic thalamus |
6 |
|
Internal capsule |
6 |
|
Corpus collosum |
2 |
|
Pons medulla |
1 |
484
DISEASES OF THE NERVOUS SYSTEM.
This shows that about half (35) involve the central and half the cortical arteries. The porterior cerebral is involved in about one- third of the cases. The parietal lobe and the caudate nucleus are most often affected. Most of these cases are thrombotic. Embolism almost always affects the great basal ganglia or some cortical branch of the anterior and middle cerebrals.
The secondary changes after thrombosis resemble those after em- bolism; a thrombus, however, may lead to supplementary embolism through breaking off of a clot, and both conditions may cause a complicating cerebral hemorrhage.
The Diagnosis. — The important points have been gone over under the head of hemorrhage. They may be tabulated in part here.
Hemorrhage:.
Age, thirty to fifty.
Hereditary history of arterial disease.
Sudden onset, with coma and paralysis occurring together, the coma deepening.
Initial and early rigidity. Very unequal pupils.
Stertorous breathing and hard, rather slow pulse, high blcod- pressure.
Peculiar alternating conjugate deviation of eyes. Early rigidity.
Peculiar disturbances of temperature, as described. Presence of decided renal disease.
Acute Softening.
Earlier or later age.
History of syphilis or of gas poisoning.
Premonitory symptoms and more gradual onset (in thrombosis), more transitory coma or absence of coma. Initial convulsive movements (embolism).
Presence of weak heart (in thrombosis) or endocarditis (in embolism).
Slight hemiplegia with anaesthesia. The puerperal state (embolism) :
Embolism is distinguished from thrombosis by the age, the pres- ence of endocarditis, of the puerperium or infective fevers, and by the sudden onset, with perhaps some convulsive movements. Throm- bosis occurs oftener in the aged, and there are prodromata — a slower onset and evidence of arterial disease and a weak heart.
DISEASES OF THE BRAIN.
4«S
The prognosis as regards the attack is somewhat better than in hemorrhage, as a rule. In embolism it is good as regards recurrence; in thrombosis, bad. The mental condition is better in embolism; usually worse in thrombosis. The recovery from attacks is more com- plete in acute softening. After the chronic stage is reached, however, the prognosis is about the same in all forms.
The treatment of the attacks consists essentially in rest and such attention to the bowels, kidneys, and heart as may be*indicated. In thrombosis it may be important to give heart stimulants and arterial depressants, and for this purpose I advise the use of alcohol, digitalis, or strophanthus with nitroglycerin. Iodide of potassium and mer- cury ought to be given if there is the slightest suspicion of syphilis. Later it is well to give courses of the iodides and mercury and of strophanthus, nitroglycerin, strychnine, and such tonics as may be indicated. The symptomatic treatment of the chronic stage is the same as in hemorrhage.
Special Forms of Thrombosis and Embolism.
Thrombosis and Softening of the Medulla. — Softening of the medulla is rare, but much more frequent than hemorrhage. It occurs at the early apoplectic age — thirty to fifty; oftener in males, and is due to lues and the arterial changes associated with alcoholism and neph- ritis. It has been observed as a result of working in compressed air. The artery involved is the vertebral or its large branch, the inferior cerebellar. The trouble may come on slowly, almost like a tumor, with symptoms of general cerebral arterial sclerosis and of slow involvement of the medullary nerve-centers; numbness, vertigo, tinnitus, headache, unilateral disturbance of hearing, ataxia and disorder of speech, and cardio-respiratory troubles. When a distinct seizure finally occurs, as is usually the case, we have a rather special syndrome. This consists of hemiplegia and cutaneous anaesthesia, with dissociation of sensation on the side opposite the lesion ; forced movements to the side of the lesion, and ataxia most marked on the side of the lesion. In other words, the sensory and motor tracts injured have not yet crossed. The cerebellum and its tracts on the side of the lesion are affected. There may be anaesthesia to some extent of the trigeminus and even of the facial nerve on the side of the lesion. There are disturbances of speech and swallowing and there is often paralysis of the sympathetic on the side of the lesion. Cardio-respiratory disturbances are present. There are no convulsions, forced movements or spasmodic tears and laughter. The cause of the symptoms is shown in Fig. 219 The tracts
486
DISEASES OF THE NERVOUS SYSTEM.
for muscular and tactile sensations have not altogether crossed in the medulla, hence we may get some evidence of ataxia on both sides. But the cerebellar tracts and peduncle injured have not crossed.
Thrombosis and Softening of the Pons. — Softening in the pons is most apt to be in the ventral part, near the raphe, and next to this, in
L - I* J Mu-ck' sense.
Fig. 219. — Showing how lesions in red areas cause focal symptoms by cutting off tracts and destroying carnial nerve nuclei. The motor tracts lie deep below the sensory and begin to cross in the lower medulla and cord.
the dorsal part of the pons. The basilar artery or its transverse branches are involved. Lesions of the pons alone are rarer than those involving also the medulla, especially its upper part, because the basilar is not plugged as often as the vertebrals and its branches. The prodromal symptoms are not so long. In a typical attack due to
DISEASES OF THE BRAIN.
487
plugging of the basilar, we get a softening of the deeper ventral parts of the pons. The patient suffers from hemiplegia, with some paresis on the opposite side to the hemiplegia. There is great exaggeration of reflexes and twitching spasms and coarse, irregular, jerky move- ments of the limbs, but no convulsion. There is usually anaesthesia and ataxia on the side of the lesion, and the anaesthesia is dissociated. There is often forced weeping and laughter, dysarthria may occur. Forced movements are not common. When the lesion is high up and involves some of the transverse branches of the basilar, we get oculo- motor palsies and spasmodic disorders on the side of the lesion and paralysis and anaesthesias on the opposite side. When lower down,
Fig. 220. — Chronic progressive softening of the brain in senility. (Marie.)
we get fifth and seventh nerve palsies on one side and hemiplegia on the other. In basilar artery lesion, the injury is large and severe, the patient develops disorders of deglutition and speech from exten- sion of the process and dies from paralysis of the bulbar centres. No great disturbances of temperature occur, nor are there marked pupillary symptoms. In the smaller lesions due to involvement of the transverse branches recovery may occur, leaving the patient with a crossed paralysis.
Chronic Progressive Softening of the Brain.
I. Sometimes thrombotic processes affect especially and successively the smaller central arteries of the circle of Willis and lead to a gradual softening. With this process there develops a gradual hemiplegia coming on perhaps in stages during the course of six months or a year.
488
DISEASES OF THE NERVOUS SYSTEM.
With the hemiplegia there may be headaches, vertigo, paresthesia and anaesthesia, dysarthria with some mental deterioration. The pro- gressive character of the disease suggests a brain tumor or abscess. In very severe cases there may be optic neuritis and atrophy. Usually, after reaching a certain stage, the disease is arrested and the patient remains about the same for some years.
II. In more senile cases, there are found through the brain many foci of softening dependent on arterial thickening and defective nutrition. In these senile cases the symptoms may include a mod- erate degree of hemiplegia, dysarthric troubles, and even some bul- bar paralysis. Especially notable is the "short-step gait" of senility. The reflexes are generally exaggerated; the mind is usually somewhat enfeebled.
III. Another type of chronic softening of the brain is that affecting especially the gray matter at the base of the frontal and temporal lobes. This is seen in senile dementia, in epilepsy, and I have observed it in old alcoholics.
There have been attempts to show that a chronic progressive softening of the brain occurs net dependent on vessel lesion. Such cases are surely very rare.
Cerebral Palsies of Children — Infantile Hemiplegia and Diplegia, Little's Disease.
The brain palsies of early life show themselves in the form of (i) hemiplegias; (2) diplegias or double hemiplegias, in which both sides of the body are involved; and (3) paraplegias, in which the lower limbs are chiefly or entirely involved. In these palsies, as in the same troubles of adult life, the loss of motor power is always accompanied by a rigidity and by some contractures and exaggeration of reflexes, in this respect distinguishing these paralyses from those of spinal origin. The seat of lesion in these cases is in the hemispheres of the brain, and it is the central motor neurons which are involved; that is to say, that part of the direct motor tract which extends from the brain cortex down to the spinal cord as far as the anterior horns. The brain palsies of children are therefore disorders of the cortico-spinal neurons, while the spinal palsies of children are disorders of the neuro-spinal neurons.
Etiology. — The disease occurs rather oftener in males than in females, though the difference is slight. The vast majority occur in the first three years of life; about one-third of them are congenital. Injuries to the mother during the time of pregnancy, possibly diseases and emotional disturbances at this time, are factors in producing the
DISEASES OF THE BRAIN.
489
congenital cases. Those cases that occur at the time of birth are due to tedious labor, the use of forceps, and other injuries at the time of parturition. After birth, the causes are those which lead to the pro- duction of intracranial hemorrhages, embolism, and thrombosis; these being injuries and the infectious fevers. Of the latter, pneu- monia, encephalitis, whooping-cough, measles, and scarlet fever are the most prominent. Syphilis is a rare cause; cerebrospinal men- ingitis and epileptic convulsions are also occasional causes.
Symptomatology. — The disorder in about one-fourth of the cases begins with a convulsion, which may be unilateral, but is usually general in character, and may last for several hours. At the same time a febrile process develops, and this continues for several days. When these acute symptoms have subsided, or before this, it is noticed that the child is paralyzed upon one side, the paralysis involving the arm, leg, and face, as in adult hemiplegia, or perhaps involving both sides. This paralysis undergoes gradual improvement, the face re- covering earliest and most, the leg next, and the arm least. As the child develops it is found that the paralyzed side fails to grow as fast as the other, and there may be from one-half to one inch or two inches of shortening in the arm or leg. The circumference of the limbs is less, the surface somewhat colder, and some vasomotor disturbance may be present. With the progress of the case a rigidity of the affected limbs develops; the heel becomes drawn up, so that there is talipes equino- varus or equino- valgus. The flexors of the forearm and of the wrist and fingers contract, as do also the adductors of the thighs. In general it will be found that there is a contraction of the flexors and adductors of the affected limbs. With this rigidity and the contractures there are exaggeration of reflexes and clonus in most cases. In the disor- dered limbs peculiar mobile spasms develop. These consist of athetoid, choreic, and ataxic movements, also sometimes tremors and associated movements. The choreic and athetoid movements are the most common (Fig. 217).
Along with the appearance of these symptoms it is noticed that there are disturbances in the mental condition of the child. It is usually backward in development, this backwardness ranging from simply feeble-mindedness to complete idiocy. Taking all cases, there is about an equal division between feeble-mindedness, imbecility, and idiocy (Sachs). Perhaps a little over one-fourth of the subjects have a fair intelligence. There is usually slowness in learning to talk, and in a small proportion of cases there is a decided aphasia. Such condition is rather more frequent with right hemiplegia than with left hemi- plegia, though the rule is not an absolute one. In connection with the
490 DISEASES OF THE NERVOUS SYSTEM.
mental defect there may develop many of the peculiar moral traits as- sociated with idiocy and low degrees of intelligence. Epilepsy very frequently complicates the disease; nearly one-half of the subjects suffer from this trouble. This epilepsy is in most cases general in character; in a few cases it takes the Jacksonian type, in a small number petit mat alone is noted. Examination of this class of suf- ferers reveals, aside from the paralysis described, various evidences of defective development. These are known as stigmata of degeneration ; though they cannot be classed strictly among such, since they are ac- quired stigmata in most cases, rather than marks which are the result of primary deficiency in development. These stigmata consist of a microcephalic or a macrocephalic skull, cranial and facial asymmetry, prognathism, imperfectly developed teeth, and a high palatal arch. It has been found that, as a rule, in cases of cerebral hemiplegias of child- hood the patient eventually has a slight flattening of the skull on the side of the lesion (Fisher and Peterson). Finally, in a few cases there may be found defects in the special senses, such as imperfect hearing, deafness, deaf-mutism, and defects in vision, such as hemianopsia, and perhaps imperfections in smell and taste. Anaesthesia is never observed.
The symptoms in the cerebral palsies of children, having passed the acute stage and having become somewhat ameliorated, enter into a chronic stage. This chronic stage begins within a few months after birth or after the onset of the disease. No great change occurs in the paralyses as the child grows older until he reaches the time of puberty, though there is a slight improvement in most cases. After the time* of puberty, if the mental condition of the child is good, the physical symptoms are apt to improve considerably.
Morbid Anatomy. — The primary changes that lead to the cerebral palsies of children are: ist, simple agenesis or lack of brain develop- ment, producing localized atrophy of the cerebrum and the condition known as porencephalies (true porencephalus is a condition in which, owing to a congenital defect in nutrition, a cavity or depression exists in the cerebral hemispheres, this cavity reaching generally into the lateral ventricle; true porencephalus is found in about one-fourth of the cases, though no definite statistics can be given, owing to the different interpretations given to this term); 2d, hemorrhage, which is probably the most frequent of the single causes; 3d, embolism; 4th, thrombosis; 5th, meningo-encephalitis and polio-encephalitis; 6th, a diffuse cortical sclerosis. Many other terms are used to de- scribe the pathological conditions found at the basis of the brain palsies of children, but the principal causes of all are undoubtedly, as has been
DISEASES OF THE BRAIN.
49 1
described, hemorrhages, embolism and thrombosis, and a defective development or agenesis. It is probable that in the hemiplegics the original lesion is generally a central hemorrhage, less often a meningeal hemorrhage. After this, probably the most frequent condition is a por- encephalus from some intra-uterine accident, which may have been defective nutrition causing anaemia and softening, or hemorrhage, or thrombosis. Acute encephalitis or inflammation of the brain of the kind similar to polio-myelitis is a frequent cause. In double hemi- plegias or diplegias of children the cause is in the vast majority
Fig. 221. — Atrophied brain with sclerosis and a cyst, from a case of infantile cerebral hemiplegia.
of eases a meningeal hemorrhage due to some injury or disturbance at the time of labor. In other cases of diplegia the lesion is a double porencephalus, which may be either the result of an intra-uterine hemorrhage or simply a defective development. In the paraplegias the lesion is probably very much the same as in the diplegias, that is to say, either a meningeal hemorrhage or a brain agenesis. Occasionally a diffuse sclerosis has been found in these cases. Not infrequently, as the result of hemorrhages, there develop cysts which fill up the atro- phied areas of the brain (Fig. 221). It is difficult to present accurately and definitely the relations between the pathological change and the
492
DISEASES OF THE NERVOUS SYSTEM.
clinical result, but it may be shown with some degree of correctness in the following table.
Original Lesions.
Hemorrhage.
Embolism.
Thrombosis.
Agenesis.
Encephalitis.
Later Pathological Condition.
Atrophy. Lobar sclerosis. Cysts.
Porencephaly. Microcephaly-
Clinical Result.
f Hemiplegia. | Diplegia. J Paraplegia. ) Sensory defects. | Mental defects. [ Epilepsy, etc.
Diplegia or Birth Palsies. — That form of the brain palsies of childhood characterized by double hemiplegias or diplegias has certain special characters which lead to its being often classed apart. These diplegias in almost all cases are congenital and are due either to in- juries at the time of birth or to some disorders of intra-uterine life. There may be convulsions or a prolonged state of asphyxia at the time of birth. After recovery from this no special trouble is noticed with the child by the mother for some weeks or months, when it will be found that it does not use its arms or legs. Other convulsions de- velop, and eventually the features of a double hemiplegia with mental impairment and epilepsy are observed (Fig. 222). In these cases the mental defect is much more decided than in the hemiplegias ; in- deed, few of these cases ever show any good amount of intelligence. Epilepsy is extremely common. The anatomical lesion in the cases is, as already stated, either a meningeal hemorrhage which has pressed upon and injured the cortical motor areas in each hemisphere, or it is a congenital porencephalic defect.
Spastic Cerebrae Paraplegia, Little's Disease. — In a few cases the brain lesion is such that there results little disturbance to the arms or face ; the paralysis is largely confined to the lower extremities. The mental condition is often very good, and there is no epilepsy. As the child gets older great improvement in the use of the limbs may occur. There are, however, rigidity and contractures of the limbs, exaggerated reflexes and spasms of the flexors and the adductors, so that the child's legs cross each other and interfere in its feeble attempts at walking. The disease is thought to be due to a developmental defect in the motor tracts, and it is possible that in some cases only the spinal cord is affected.
Diagnosis. — The clinical diagnosis of cerebral palsies is to be made from the spinal palsies. The latter are distinguished by the fact that in the infantile paralyses of spinal origin there is no rigidity or exaggeration of reflexes, and there are electrical degenerative reactions of the muscles and decided wasting of the limbs with shortening. The mode of onset in cerebral palsies and their distribution in the form of hemiplegias in which the face is involved also indicate the seat of the lesion. The pathological diagnosis is by no means an easy one. Cerebral palsies occurring at the time of birth and accompanied at that time by general
DISEASES OF THE BRAIN.
493
convulsions or asphyxia may be considered to be due to meningeal hemorrhage, especially if the delivery of the child has been brought about by the use of forceps or if the labor has been long and tedious. Diplegias and paraplegias which are congenital are probably due to true porencephalus, provided there was no difficulty at the time of labor and there were no convulsions or other serious phenomena after it. Cerebral palsies occurring after birth in the first, second, or third year of life are apt to be due to hemorrhage and encepha- litis, less often to embolism or thrombosis. Hemiplegias de- veloping after infectious fevers are likely to be due to hemor- rhage. In diagnosticating the pathological lesion in such cases it must always be remembered that hemorrhage is much more frequent than embolism, and that thrombosis as a factor has not yet been very clearly estab- lished.
Course and Prognosis. — In all types of the disease the course is chronic and perfect cure is hardly possible, although in the slighter forms of hemiplegia nearly all traces of the paralysis may be absent. In the hemi- plegic form the patient often reaches adult life, and if his in- fig 222.— Cerebral diplegia-,
telligence is not defective and he (Little's disease.)
has no epilepsy the motor trouble
improves a great deal and he may live a long and useful life. If epi- lepsy and mental defect are present, there ensues eventually a further mental deterioration, and such subjects rarely live much beyond the period of adolescence, or if they do they pass into the asylums for the idiotic and epileptic. The diplegic and paraplegic cases have a much worse prognosis both as to duration of life and as to improvement in symptoms, except occasionally the type described above as cerebral spastic paraplegia or Little's disease. The degree of intelligence and the absence of epilepsy are the two factors which measure the serious-
494
DISEASES OF THE NERVOUS SYSTEM.
ness of these cases, as they do those of the hemiplegias. As regards the significance of individual symptoms, the post-hemiplegic move- ments have a bad import; the presence of a microcephalic head or of decided marks of degeneration is unfavorable.
Treatment. — The treatment, so far as the paralysis is concerned, is largely mechanical. The patient is benefitted by occasional courses of electrical treatment which stimulate somewhat the nutrition and functions of the muscles. Massage and stretching of the contractured tendons and limbs also are helpful in my experience. The orthopaedic surgeon is able to render valuable assistance by occasional overstretch- ing the contractured limbs and placing them in splints. Tenotomy may also be resorted to with advantage, as I have had occasion to see. The child should be encouraged above all, however, to use the limb as much as possible. He should be taught gymnastic exercises; running, walking, and bicycle riding are all measures which give great help. When the child's intelligence is good and there is little or no epilepsy, a great deal can be expected in the way of improvement as the child grows older.
So far as the epilepsy is concerned, it should be treated on the same principles as idiopathic epilepsy, except that great care should be had in the use of the bromides; a thorough test must be made in order to determine how much of this drug will suppress the fits, and then its use must be graduated in the future in accordance with the knowledge thus obtained. The mental defects of the child can be helped only by proper training of the body and careful education of the mind. The question of operative interference in these cases has excited much attention. A priori it would not seem as though surgical in- terference could do good in relieving conditions in which there is destroyed or atrophic tissue. Still the subject must be dealt with em- pirically, and there have been some results which show that apparently a relief is obtained in a few cases by trephining the skull or by Lanne- longue's operation of craniectomy. If there is no microcephalus, if the case is one of hemiplegia with imbecility and epilepsy, the surgeon should simply make an exploratory opening. If he then finds any evidences of compression from the presence of a cyst, this may be very cautiously opened.
CHAPTER XX.
TUMORS OF THE BRAIN— SYPHILIS.
The kinds of tumor found in the brain are tubercle, syphiloma, glioma and sarcoma, which are the common forms; myxoma, carci- noma, fibroma, osteoma, cholesteatoma, lipoma, psammoma, neuroma; vascular tumors including aneurisms; echinococcus, and cysticercus. In fact, all forms of new growths are found in the brain; but the infec- tious granulomata, tubercle and gumma, and the sarcomatous type of tumors are the most common. As compared with other organic dis- eases of the central nervous system, brain tumors are rare.
Etiology. — Brain tumors affect males oftener than females, the ratio being about as two to one (644:320). Sarcomata alone seem to affect females about as often as males. Brain tumors occur with about equal frequency throughout all ages of life up to about fifty; one-third occur under the age of twenty (Gowers). During childhood tumors are about equally distributed throughout all ages (Starr). One-half of all the tumors of childhood are tuberculous; after this come gliomata, endotheliomata and sarcomata. The gumma, glioma and sarcoma begin to be more frequent after the age of twenty. Sarcoma and especially cancer occur in the middle and later ages of life; but brain tumors of any kind are extremely rare after the age of sixty.
To sum up in tabular form, the relative frequency of the different kinds of tumors with regard to age is shown in the following:
Childhood, tubercle, parasites.
Early life, gumma, glioma, parasites.
Early and middle life, sarcoma, endothelioma, glioma, and gumma.
Middle and late life, sarcoma, endothelioma, gumma, cancer.
Heredity has a slight influence in predisposing to brain tumors. Blows on the head and other forms of injury to the cranium are ex- citing causes in a small proportion of cases.
Seat. — Over one-third of all brain tumors are in the posterior fossa ; about one-fourth of all tumors are in the cerebellum, or cerbello- pontine angle, and about an equal number in the cortex cerebri. Very few tumors are found in the medulla or fourth ventricle.
About one-twelfth of all tumors are in the basal ganglia and lateral
495
496
DISEASES OF THE NERVOUS SYSTEM.
ventricles, and a rather smaller number in the mid-brain (corpora quadrigemina and peduncles).
The order of frequency in location, then, is about as follows:
Cerebellum — pons.
Cortex cerebri.
Centrum ovale.
Pons.
Mid-brain.
Medulla.
In children their relations are a little different. Here nearly one- half of the tumors are in the cerebellum and pons. They are very much less frequent in the cortex cerebri but more common in the centrum ovale, basal ganglia and mid-brain so that the order of fre- quency is:
Cerebellum — pons.
Cerebrum ovale.
Basal ganglia.
Mid-brain.
At the rnid-brain as well as all the contents of the posterior fossa are nourished by the vertebral arteries, and their branches; it follows that the majority of tumors in children are in this vacular area. A little over one-eighth of the total brain mass is thus especially subject to tumor growth.
Symptoms. — -The symptoms of brain tumors vary extremely in accordance with the location, the kind of tumor, the rapidity of growth, and the age of the patient. The general course of a case of brain tumor in an adult is somewhat as follows: The patient first notices a headache which is intense and persistent, and which has exacerbations of frightful severity. With the headache or between the attacks vomiting occurs, which is often not accompanied by any nausea. Sensations of vertigo, annoying paresthesias, and convulsive movements affecting one or more extremities develop, and there may even be general convulsions. Optic neuritis then appears and the patient finds that his eyesight is weak and progressively deteriorates. The mind becomes more or less disturbed, the mental processes are dull and slow, a feeling of hebetude and incapacity to attempt any mental exer- tion are present. As the disease progresses the intense pains and vomit- ing produce weakness and emaciation. Paralyses of various kinds develop and blindness ensues. Convulsions of a local or general character become more frequent, and finally the patient becomes bed- ridden and helpless.
The course of the disease is not a steady one, there being often
TUMORS OF THE BRAIN — SYPHILIS.
497
slight remissions, or there may be periods when progress seems to be arrested. After a period of time varying from one to four or five years death occurs from exhaustion or some intercurrent malady.
The symptoms thus very briefly outlined are divided into general and focal. The general symptoms are :
Headache,
Vertigo,
Vomiting,
Optic neuritis,
Mental defects.
Besides these there may be general convulsions and speech dis- turbances.
Headache occurs in from one-half to two-thirds of the cases; it is very severe and the pains are of a boring or lancinating character ; they are so horrible that they often lead the patient to think of suicide. The pains are sometimes periodical, occurring every night or every other day, and suggest by their perodicity a malarial character. They are located sometimes in the brow or in the occiput, while sometimes they are diffused all over the head ; they are rather more frequent than otherwise in the neighborhood of the tumor. They are more frequent with cerebellar tumors than with those located anywhere else. Pains are also frequent with tumors of the mid-brain and of the cerebral hemispheres. They are less frequent with tumors situated in the peduncles and at the base of the brain. The pains are due to the in- creased intracranial pressure and to irritation and stretching of the dura mater by the encroachments of the new growth. Headache occurs in about the same proportion in children and adults, and it does not seem to bear much relation to the kind of tumor, although the pains are generally less with the gliomata, and they are more frequent with rapidly growing tumors whatever their character. With the pains there is often a local tenderness of the scalp and cranium which may be elicited by percussion, and in most cases there is greater tender- ness in that part of the cranium lying over the tumor.
Vomiting is a symptom which is almost as frequent as headache. The vomiting is often of a projectile character and not accompanied by much nausea. Vomiting occurs, as does headache, more frequently with cerebellar tumors. It is associated with rapidly growing tumors, such as syphilitic or tuberculous neoplasms.
Vertigo is a general symptom which occurs in from one-third to one-half of the cases. The vertigo may be slight, such as is often felt from ordinary causes. Occasionally it is very severe and accompanied by forced movements. The severer forms and those associated with
498
DISEASES OF THE NERVOUS SYSTEM.
forced movements occur with tumors of the cerebellum and the parts closely connected with it.
Optic neuritis is one of the most frequent and important of all the general symptoms of brain tumor; it occurs at some period of the disease in at least four-fifths of the cases, more frequently in cerebellar tumors and in those of the mid-brain and great basal ganglia. It is rare in tumors of the medulla. It is less frequent and marked in the slow-growing tumors. The neuritis may run a somewhat rapid course and then improve a great deal or even for a time disappear ; but ordina- rily the course is progressive and it ends eventually in an atrophy of the optic nerve. Hence the examination of the eyes in brain tumors should be made a number of times in order to note the progress of the trouble. Primary atrophy of the optic nerve does not occur in brain tumors. The inflammation almost always affects both nerves, but it may begin with one and subsequently affect the other.
Mental defects are almost always present in tumors of the brain. These defects consist in a slowness of the mental processes, a condition of hebetude, a tendency to attacks of somnolence, and sometimes a peculiar childishness and silliness or peculiar mental irritability. The memory is also usually somewhat weakened and the power of attention lessened. Such psychical defects are more frequent with tumors of the frontal lobes and more frequent also with large tumors.
General convulsions occur in about one-fourth of the cases and more frequently when the tumors are situated in the cerebral hemi- spheres and cortex. There may be also apoplectiform attacks, from which the patient recovers in the course of a few days or weeks. More rarely there is a genuine apoplexy from the bursting of a blood-vessel in the neighborhood of the tumor.
The speech disturbances are most marked in tumors which affect the pons and medulla and the origin of the cranial nerves. Such speech disturbances, when characteristic, are shown by a confluent articulation; that is to say, the patient runs the syllables together.
The local symptomatology of cerebral tumors cannot follow always strictly the anatomical subdivisions of the brain. Practically, it is im- portant first to make a diagnosis between tumors above and tumors below the tentorium. The former include tumors of the cerebral hemispheres and anterior fossae, the latter tumors of the cerebellum, pons and medulla, which thus includes tumors of the middle and posterior fossse. Tumors of the isthmus of the rhombencephalon or hind-brain belong more to the group of subtentorial tumors also. There are symptoms distinguishing tumors in these two places because the pressure conditions are not alike. Pressure in
TUMORS OF THE BRAIN— SYPHILIS. 499
subtentorial tumors soon blocks the aqueduct of Sylvius and causes a hydrocephalus and added pressure symptoms. Tumors of the hemi- spheres cause the change more slowly.
Fig. 223. — Showing relationships of tumor in the posterior fossae of the skull.
i . Tumors of the prefrontal area. Tumors in this area often show no particular localizing symptoms, and this part of the brain is conse- quently put down as a latent one ; nevertheless, in a good proportion of cases tumors here produce peculiar mental disturbances that, taken
5°°
DISEASES OF THE NERVOUS SYSTEM.
in conjunction with the general symptoms, enable us to make a local diagnosis. The symptoms are peculiar mental hebetude, childishness, irritability, often a kind of silliness and emotional weakness, a tendency to laugh and cry and to get angry at trifling causes. The entire character and temperament of the man are sometimes changed. Be- sides this, owing to implication of the olfactory nerve, there may be loss of the sense of smell on one or both sides; implication of the optic nerves will cause hemianopsia and optic neuritis. If the tumor in- volves the orbit there will be paralyses of the ocular muscles and pro- trusion of the globe of the eye. If the tumor grows backward there is gradual invasion of motor centres with irritation, showing itself by spasms, convulsions, and later by paralyses.
2. Tumors of the central convolutions. It is in this area that we are often able to make the closest and most accurate diagnosis of the localization of new growths, owing to the involvement of the different motor centres. Through the involvement these centres are at first irritated, with the result of producing local spasms or Jacksonian epilepsy. Such spasms are often preceded by sensory symptoms or aurae. As the tumor grows, the area of involvement becomes larger, spasms become more diffused, and general convulsions may finally ap- pear, with hemiplegia. There may be simply feelings of numbness or prickling, which either are permanent or simply precede spasms, or there may be hemianesthesia of a moderate degree to pain, touch and tem- perature. The muscular sense also may be somewhat involved. In cases of slight sensory involvement the capacity for localizing sensa- tions seems to be most implicated. Besides the symptoms mentioned, there may also be motor aphasia and agraphia. If the symptoms are more distinctly motor the prsecentral convolution is most involved. If the tumor is subcortical the symptoms are more of the paralytic and spastic type than when the cortex itself is implicated. (See Fig. 194).
3. Tumors of the parietal lobe excluding the post-central gyrus. The symptoms produced by tumors in this area may be very slight. The most characteristic are disturbances of muscular sense, which occur when the supramarginal gyrus is affected ; astereognosis or loss of the perception of the form of objects felt if the superior and inferior lobules are involved, and word blindness which occurs when the left angular gyrus in the -inferior lobule are affected. If the tumor is in the post-central we get more disturbance of cutaneous sen- sation, but usually also some motor phenomena and disturbance of deep sensibility. The cortical representation of the third nerve is thought to be in the* neighborhood of the angular gyrus, and some
TUMORS OF THE BRAIN — SYPHILIS.
cases have been reported in which paralysis of this nerve resulted from tumors in that area.
4. Occipital lobes. Tumors in this region, if situated in the cuneus and calcarine fissure, produce homonymous hemianopsia. If the tumor involves the other parts of the left occipital lobe and the cuneus is not seriously involved, there may be a condition known as soul blind- ness or incapacity to understand the nature of the things which one sees. If the tumor extends up chiefly toward the angular gyrus, there may be word blindness, along with some hemianopsia. If the tumor extends farther forward into the parietal lobe, there may be hemianaes- thesia, hemiataxia, and perhaps a little hemiplegia owing to involve- ment of the fibres of the internal capsule.
5. Temporal lobes. The temporal or temporo-sphenoidal area on the right side is very nearly a latent one. On the left side, tumors involving the posterior part of the first and upper posterior part of the second temporal convolution produce sensory aphasia. Tumors in either lobe when large and extending well down toward the base may produce attacks of vertigo or forced movements, owing probably to irritation of the labyrinthine tracts. Tumors that involve the hippocampal convolution and the uncus produce some disturbances in the senses of smell and taste.
6. Tumors of the corpus callosum. Tumors situated in this area are very rare. Their symptoms have been thought to be somewhat characteristic; but in the writer's experience they correspond closely with tumors situated in the third ventricle and lateral ventricles of the brain; in other words, tumors which, beginning in the central parts of the brain, gradually extend outward toward the periphery. The symptoms credited to tumors of the corpus callosum, are, first, the general symptoms of brain tumor, to which there are superadded a gradually developing hemiplegia with later a paraplegia. At the same time there is a great deal of mental dullness, stupidity and drowsiness; the patient often sits for hours mute, refusing to speak, or lies in a half- somnolent condition. There are no paralyses of the oculomotor nerves or of the other cranial nerves. There is no anaesthesia. The disease gradually progresses and the patient dies in coma.
7. Tumors of the great basal ganglia and the capsule (the optico- striate region). The general symptoms of tumors of this region re- semble in many respects those of tumors of the corpus callosum. The stupidity, however, may be less marked. There is usually a progressive hemiplegia which may be accompanied by anaesthesia and sometimes by choreic movements, if the tumor involves the optic thalamus and adjacent part of the capsule.
502
DISEASES OF THE NERVOUS SYSTEM.
Tumors of the corpus striatum cannot be diagnosticated by symp- toms due to the irritation or destruction of this ganglion. The loca- tion can be only approximately estimated by the presence of symp- toms due to lesion of the adjacent parts, viz. , the internal capsule and the adjacent motor cortex. When on the left side, symptoms of aphasia of the motor type may appear. As the lesion extends there may be hemi- plegia and later, perhaps, irritative symptoms from involvement of the capsule and motor cortex. We might expect some cutaneous vaso- motor disturbances on the opposite side of the lesion. In all central growths there is likely to develop an apathy, slowness of thought and even somnolence. This is probably due to the pressure effects. Tumors of this region are very rare.
Tumors of the Optic Thalamus. — The same may be said of tumors here as of those of the corpus striatum. Lesions of the thalamus itself give no specific symptoms unless it is that of pain referred to the ex- tremities of the opposite side. But by extension we get hemianesthesia of various types and involvement of the ocular muscles and reflexes. There is later even homonymous hemianopsia; peculiar forced move- ments, and intention tremor may occur. The opinion that in lesions of the thalamus there is a paralysis of emotional expression has not been well sustained. The thalamic syndrome which is observed in acute lesions of this ganglion does not appear very characteristically in chronic progressive lesions. This syndrome consists of hemianaes- thesia, with slight loss of cutaneous but marked loss of deep sensi- bility; slight hemiplegia, without much of any contracture or exag- geration of reflexes; severe pains on the paralyzed side and some chronic and athetoid movements on this side.
8. Tumors of the pituitary body and optic chiasm. The thalamus and adjacent region ('tween-brain or diencephalon) is part of the brain which in man is not sharply limited. It may be divided into an upper or dorsal part, including the thalamus, geniculate bodies and pineal gland, and a lower or ventral part, hypothalamus, which includes the corpora mammillaria, the pituitary body and infundibulum and the optic chiasm. Practically, we attempt to diagnosticate tumors of the optic thalamus and tumors of the pituitary body and optic chiasm.
Tumors of the pituitary body are not extremely rare, and are often associated with acromegaly. Tumors of the hypothalamus press on the chiasm and cause optic neuritis and atrophy and a charac- teristic bitemporal hemianopsia. Sometimes acromegaly is present.
9. Tumors cf the pons. If the tumor is in the pons it will cause, if situated high up, a palsy of the third nerve on one side and hemi-
TUMORS OF THE BRAIN — SYPHILIS.
503
plegia on the opposite side. If lower down there may be a palsy of the fifth nerve on one side and hemiplegia on the other side (Fig. 224). If the tumor is extensive it may produce not only a hemiplegia but a hemianaesthesia. If situated somewhat superficially and on the lateral edge of the pons involving the peduncles, there will be forced movements of the body, either toward or from the seat of the lesion.
Fig. 224. — Showing the mechanism of crossed paralysis. Lesion at M causes paralysis of third nerve, lesion at F paralysis of fifth nerve with hemiplegia of opposite side. (After Van Gehuchten.)
i o. Tumors of the medulla are rare and generally tubercular or syphi- litic. The medulla is the seat of origin of the IX, X, XI in part and XII nerves. Through it run motor and sensory and cerebellar tracts. It is the centre for the automatic regulation of respiratory cardiac and vasomotor functions. The olivary bodies place it in close relation also with the cerebellum. Tumors here cause, therefore, paralyses of the tongue, disturbances of deglutition, speech and finally of respiration
DISEASES OF THE NERVOUS SYSTEM.
and cardiac action. Hemiplegia, hemianaesthesia and ataxic symp- toms occur.
11. Tumors of the isthmus mean an involvement, more or less, of the upper part of the pons, the superior cerebellar peduncles and valve of Vieussens, the lateral lemniscus and tract of Gowers, and the fourth nerve or superior oblique. Tumors here, therefore, are practically diagnosticated as tumors of the upper part of the pons, when such diagnosis can be made.
12. Tumors of the mid-brain (cerebral peduncles and corpora quad- rigemina. This part of the brain is, like the isthmus, relatively small in man, but it contains three important structures: the cerebral peduncles, the corpora quadrigemina and the aqueduct of Sylvius. In the base of the peduncles run the motor and sensory tracts, above which is the locus niger and above this the tegmentum with sen- sory tracts, the anterior cerebellar peduncles, the red nuclei and the nuclei and motor nerves of the eye. The anterior tubercles and genicu- late bodies have to do with the reflexes and co-ordination of the eye movements, the posterior with the functions of the eighth nerve. Tumors here are sometimes tumors of the peduncles, and sometimes of the corpora quadrigemina according as they are superficial or deeply situated. The general character of the symptoms is an association of hemiplegia with disturbances of the motility and re- flexes of the eye, often ataxic symptoms and sometimes forced move- ments and disturbances of hearing. We would expect soon to get symptoms of ocular palsies and of brain compression from closure of the aqueduct of Sylvius. A tumor at the base of or in the peduncles will cause the syndrome of Weber, i. e., hemiplegia on the opposite side and paralysis of the third nerve on the same side.
13. Tumors of the cerebellum. Tumors of the cerebellum are more often seen in children and they grow rather rapidly, so that a distinct picture of cerebellar disease may be developed in a few months. The early symptoms are vomiting and headache; and the child may be first brought to the physician for stomach trouble. The stumbling ataxic gait then develops and with it a good deal of general muscular weakness (parasthenia). Strabismus and nystagmus are often early symptoms. The patient has a weakness of one side not amounting to hemiplegia and there may be some facial nerve weakness on the same side as the lesion. There is diminution or loss of deep reflexes and some hypotonia. As the ataxia progresses, the patient may be unable to stand well, but inclines to fall backward or to one side ; when he tries to sit, even, he may fall over and the hands become awkward and ataxic, though less so than the legs. There may be sudden seizures of
TUMORS OF THE BRAIN — SYPHILIS:
violent vertigo, or loss of consciousness, with tonic irregular movements of the limbs, or perhaps only sudden short forced movements, throwing the patient to the floor (cerebellar seizures). These are less common in children than in adults. There may later be involvement of the auditory or trigeminal nerve. Choked disc is very nearly always present and is an early sign, especially in the rapidly growing tumors. As the tumor grows it usually stops up the aqueduct from the third to the fourth ventricle, and then an internal hydroce- phalus develops and we get symp- toms of brain compression. The rnind becomes apathetic and slow ; headache and vomiting are less, blindness sets in, with paralysis of some of the motor nerves of the eye and dilated and fixed pupils. The neck is retracted and stiff and Kernig sign is present. The pulse may be slow at first and later in the terminal stage may be signs cf bulbar compression and paralysis. The patient emaciates and death occurs in cne-half to two years; though in some slow-growing tumors of adults the disease may last ten years or more.
Tumor of the ponto-cerebellar angle give a rather special symp- tomatology (Fraenkel, J. R. Hunt). (Fig. 228.) It arises as a small
fibroma usually on the auditorv
Fig. 225. — Attitude in a case of nerve. It grows very slowly and cerebellar tumor.
may undergo finally sarcomatous
degeneration. As it grows it forms a hard, circumscribed, pedunculated mass pressing into the angle between the lateral anterior surface of the pons and the middle peduncle of the cerebellum. The first symptoms are simply those of irritation and compression of the eighth nerve, with tinnitus, vertigo, and progressive deafness; then there may be pains in the trigeminal nerve and later perhaps some
506
•DISEASES OF THE NERVOUS SYSTEM.
paresthesia or anaesthesia over the distribution of this nerve with loss cf conjunctival reflex. The facial nerve may become involved as the disease progresses. Usually not till after several years does the patient begin to show the general symptoms of brain tumor and then those of cerebellar diseace : headache, vomiting, choked disc, staggering, cerebellar gait, falling generally to the side of the lesion, cerebellar attitude, in about half the cases some seizures, some hemi- plegia on the side of the lesion; finally, symptoms of internal hydro- cephalus and brain pressure.
Fig. 226. — Position of head in cerebellar tumor. (Fraenkel.)
Multiple tumors. About one-seventh of all brain tumors are multiple. Hence, in making a diagnosis of the localization of tumors, this fact must be borne in mind. The tumors which are most fre- quently multiple are tubercle, cancer, and melanotic growths.
Pathology. — Tubercle is the form of tumor found oftenest in chil- dren and is altogether the most frequent of brain tumors, unless we except endothelioma. It is more often located in the cerebellum, but may appear in the pons or other parts of the brain. It may be a single or, as it is then called, a solitary tubercle, or there may be a multiple growth. The tumor is irregularly round in shape and varies in diameter from one and a half to two inches. It has a grayish-yellow appearance externally; internally, a yellowish or cheesy look. It is not vascular, but is often surrounded by softened or inflamed tissue.
TUMORS OF THE BRAIN — SYPHILIS. 507
There may be an associated meningitis. The tumors, when solitary, usually start from the central parts of the brain, but they also develop on the meninges of the convexity, particularly in the parietal region, and sometimes they develop also at the base. Tubercle always arises from infection by the tubercle bacilli, which are carried by the blood to the brain. The tumors develop usually from some infectious focus, starting in a blood-vessel of the pia mater. Microscopically, the tumor shows the ordinary appearances of tuberculous growths. It contains in its periphery many round cells, nuclei, and giant cells. In the centre there is usually an amorphous substance, the product of degeneration and the breaking down of the ordinary substance of the tumor. The characteristics of the growths are the presence of the round cells and giant cells, the caseation and softening of the centre, and the absence of vascularization, with the presence of the bacilli.
Syphiloma or gumma. Gummatous tumors of the brain are usually associated with syphilitic meningitis or some other form of cerebral syphilis, such as endarteritis, and perhaps inflammation of the cranial nerve-roots. Syphilitic growths are usually found upon the brain surface, oftenest on the base, next upon the convexity of the frontal and central convolutions. The process appears either in the form of a somewhat distinct tumor or in the form of an irregular thick- ened exudate lying upon the surface of the brain and forming what is called gummy meningitis. The gummata may attain great size. They start usually from the pia mater and are due, as in the case of tubercle, to the irritative action of the infective organism. The gumma is irregular in shape ; it has a somewhat thick, grayish periphery and often a yellowish centre, the appearances differing with the age of the tumor. Microscopically, it is found to consist of small round cells and spindle cells with various broken-down nerve-tissue elements. It pre- sents in the interior the evidence of cheesy degeneration, somewhat like that in tubercle, but less marked. There is a peculiar develop- ment of fibrous tissue in the syphilitic growths which distinguishes them somewhat. Besides this, the blood-vessels are numerous in the periphery and show evidences of endarteritis and peri-arteritis. The distinctions between gumma and tubercle are the less amount of cheesy degeneration in the centre of the former, its more irregular ap- pearance, the presence of arteritis and vascularization, the absence of giant cells and of tubercle bacilli and the presence of the spirachaete pallida.
Actinomycosis is a form of infectious tumor which sometimes ex- tends from the face and neck into the brain, leading to inflammatory processes, however, rather than to true tumors. No other neoplasms
508
DISEASES OF THE NERVOUS SYSTEM.
of infectious origin attack the brain unless glioma be found to be of that nature. Cystic tumors are not uncommon and are the result of para- sitic invasion, of injuries and of the breaking down of a glioma or from a teratoma.
Glioma may occur in any part of the brain, but is most frequently found in the cerebrum. It is the only tumor which is peculiar to the
Fig. 227.— Tumor of frontal lobe. (J. R. Hunt.)
nervous centres, being developed from the neuroglia tissue which forms the supporting structure of these centres. Glioma originates in the white matter of the nerve-centre and not from the membranes or fibrous structures. It may grow to a very large size and is the form of brain tumor which becomes the largest. Gliomatous tumors measure from three to eight or more centimetres in diameter. In appearance
TUMORS OF THE BRAIN — SYPHILIS. 509
the glioma can be scarcely distinguished from the brain substance it- self, but usually looks like either pale or congested gray matter, or it may have a yellowish or gelatinous appearance. The tumor is very vascular and it may show the results of hemorrhages. The central
Fig. 228. — Tumor of cerebello-pontine angle
part sometimes breaks down, forming cavities or cysts. The tumor may grow very rapidly, infiltrating the normal tissue. In these cases there is hardly any definite boundary between the tumor and the normal tissue. In other cases the tumor grows slowly, but rarely if
DISEASES OF THE NERVOUS SYSTEM.
ever becomes encapsuled. Microscopically, it is found to consist of small cells with delicate fibrous prolongations, these being the glia cells. The tumor is very vascular and its whole appearance is sug- gestive of an inflammatory process rather than a new growth ; the in- flammatory process being one in which the neuroglia tissue reacts to the inflammatory irritant. Gliomata may undergo certain changes, e. g., a mucous degeneration of the cells takes place, forming a myxo- glioma. When there is with the neuroglia-cell proliferation a rich proliferation of round cells from the connective tissue, it is called a glio-sarcoma. When the tumor is situated near the surface, involves the membranes, and grows slowly, with an increase in fibrous tissue, it is called a fibro-glioma. When the gliomatous growth is very firm and hard, the fibrous portion of the glia tissue predominates; it constitutes a nodule such as is found in multiple sclerosis, and these hard gliomata are sometimes called neuro-gliomata.
Sarcoma. The sarcoma and its various modifications (endothe- lioma, etc.) form perhaps the most important and almost the most frequent of the brain tumors. The sarcoma is a tumor of con- nective-tissue origin ; it develops, therefore, from the brain membranes or from the sheaths of the blood-vessels. Sarcomas may be single or multiple. They may be of all shapes and they grow to very varying sizes. They often develop a capsule. They may be either primary or secondary. Their growth is often rapid. They are white or grayish in appearance or may be somewhat yellowish, dependent on the predominance of the different kinds of cells and blood-vessels. Microscopically, they are made up of small round cells, spindle cells, and other cells of various sizes and forms. They contain often con- siderable fibrous tissue. They contain blood-vessels, but are not richly vascular. The essential characteristic of the sarcoma is the rich development of round cells and spindle cells ; in other words, its rich cellular contents. Sarcomata are peculiar in undergoing many modi- fications. Thus sometimes fibrous tissue develops largely and the tumor is called a fibro-sarcoma; sometimes the tumor undergoes mucous degeneration and is called a myxo-sarcoma. There may be a breaking down of the centre with the formation of cysts. There may be a development of pigment. Not frequently a sarcomatous process invades a glioma and we have a mixture of a sarcoma and glioma. Sarcomatous tumors sometimes have an alveolar structure. These tumors contain endothelial cells derived from the lymphatics. When sarcomata develop from the dura mater and are slow in growth there may be calcareous deposits in them and they are called psammomata.
f
TUMORS OF THE BRAIN — SYPHILIS. 51I
Endothelioma is a form of tumor often called fibro-sarcoma. It is said under this name to be the most frequent of the true tumors. It usually starts from the meninges and is often encapsulated and therefore subject to surgical treatment.
The fibroma is a very rare brain tumor, unless the pacchionian bodies, when enlarged and hardened, may be so considered. Occa- sionally fibro-neuromata are found developing on the roots of the cranial nerves.
Osteoma is not particularly rare, developing in the form of bony plates in the dura, falx, or tentorium. Osteomata in the brain sub- stance are mere pathological curiosities.
Enchondromata, lipomata, and angiomata are rare and have no practical importance.
Cancer is relatively a rare affection of the brain, especially as a primary development. It usually arises from the membranes of the brain. Cancer is not infrequently multiple and is usually of the soft or colloid character.
Parasitic growths. Parasitic tumors are extremely rare in this country. The only forms which are found are the echinococcus and the cysticercus cellulosae. The echinococcus produces hydatid cysts, which may be large or small, few or many, and are usually all upon the surface of the brain. They are much rarer than the cysticerci. These form cysts which are usually multiple, slow in growth, lie upon the surface of the brain or in the ventricles, are encapsuled, and show no symptoms.
Aneurisms are anatomically tumors, but clinically they present some special symptoms and hence are described separately.
Seat. — Tuberculomata are most common in the cerebellum. The cerebellopontine tumor is oftenest a fibrosarcoma. Syphiloma is oftenest in the meninges of the posterior or middle fossa, endothelioma in the meninges of the subtentorial region and (Cushing) cysts in the cortex.
Diagnosis. — It is necessary first to make the diagnosis of the presence of the tumor, next of its location, and finally of its nature. The existence of a brain tumor is determined by the presence of the characteristic general symptoms — headache, vomiting, vertigo, optic neuritis, mental disturbances, and progressive course. The physician must bear in mind the possibilities of meningitis, abscess, lead poison- ing, hysteria, and paretic dementia. Very often a localized basilar meningitis of syphilitic or tuberculous origin simulates closely the presence of a tumor. Besides the general points referred to, in esti- mating the probabilities of the existence of a tumor we must bear in
DISEASES OF THE NERVOUS SYSTEM.
mind the age of the patient and the existence of a tuberculous or syphilitic history, the history of an injury, of local tenderness, and the presence of some new growth in other parts of the body, particularly about the neck or thorax or in the lungs. The Calmette tuberculin test may be made.
The symptoms of brain tumor are sometimes closely simulated by serous meningitis. In these cases there may be headache, optic neuritis, vomiting, convulsive seizures and even a hemiplegia; the autopsy shows, however, an internal hydrocephalus. In these cases there has been a serous effusion in the ventricles due to an inflamma- tory process of infective or toxic origin, or the diagnosis can sometimes be made by lumbar puncture. Brain tumors are also closely simulated by a localized progressive cerebral endarteritis. A close examination of the general condition of the arteries is needed. Optic neuritis is not so prominent. In sinus thrombosis and sinus inflammation there oc- cur high temperatures, rapid pulse, frequent chills and oedema.
The diagnosis of the location of the tumor is based upon the rules already given in regard to local diagnosis. The X-ray has occasionally been of service. The diagnosis of the nature of the tumor can often be made and should be attempted. In children, for example, the chances of the tumor being tuberculous are very great, particularly if there is a scrofulous diathesis or tuberculous disease elsewhere. Syphilitic tumors of the brain are almost always accompanied by or preceded by manifestations of external syphilis. Gliomatous tumors occur in childhood and early life. They produce, as a rule, fewer irritative phenomena and are accompanied by remissions and by apoplectic or pseudo-apoplectic attacks due to the vascular nature of the tumor. Carcinomata occur late in life and are usually secondary.
Prognosis. — In extremely rare cases tumor of the brain appears to stop growing and become encapsulated and atrophied. Such tumors are of a tuberculous or syphilitic, perhaps sometimes of a gliomatous or sarcomatous character. As a rule, the brain tumor grows steadily and the symptoms of the disease become more pro- nounced until death occurs. The prognosis is best for tubercle in children and gumma in adults. It is worse in those cases of glioma and cancer. In fact, the sooner serious symptoms develop, the more rapid the general development of the disorder, the sconer does a fatal termination come. The disease lasts on an average two or three years, ranging from a month to eighteen years.
Treatment.— Something can be done medically in cases of tuberculous tumors, syphilitic tumors, and possibly in the sarcomatous variety. In tuberculous tumors a general constitutional and strengthening treat-
TUMORS OF THE BRAIN SYPHILIS. 513
ment must be resorted to; fresh air, tonics, and a large amount of food being the main reliance. The utility of any form of tuberculin is as yet doubtful. In syphilitic tumors much can be done by the usual vigorous antisyphilitic treatment. In sarcomatous tumors, if they are suspected, some help may be obtained from the internal use of arsenic. Symptomatically we must give such drugs as antipyrin, phenacetin, antifebrin, codeine, and morphine for the relief of pain. The ice cap and leeching often help the headache also. Should con- vulsions develop, the .bromides should be used, just as in idiopathic epilepsy.
In cases in which the location of the tumor can be made out, the question of surgical interference should be considered. The percen- tage of cases in which surgery can help is extremely small. It will include only those cases in which the tumor can be located; of those which can be located, only those which are in an accessible region, and finally, of those which are in an accessible region, it includes those which are either superficial, or, if lying in the brain substance, are more or less encapsulated. The removable tumors of the brain amount to less than five per cent. They are, in particular, the endotheliomata, sarcomat- ous, syphilitic, and tuberculous tumors lying in the central or occipital areas. In many cases in which there is some doubt as to the localiza- tion, an exploratory trephining is justifiable; cases in which it is known that the tumor cannot be removed, the trephining for the simple pur- pose of relieving pressure is justifiable. In cases which are not oper- able, the decompressive operation of Cushing may often be done with advantage. With the present technic tumors can now be safely removed from the cerebellum and the cerebello-pontine angle and, in fact, from nearly every superficial region of the brain. But the opera- tion in the serious cases should only be done by a specially skilled surgeon.
Intracranial Aneurisms.
Intracranial aneurisms are of two kinds — -"miliary" and those of large size. The miliary aneurisms are minute dilatations of the ves- sels and are always multiple ; they have been described under the head of cerebral hemorrhage. Large aneurisms affect only the large cere- bral arteries at the base of the brain. The arteries are affected in the following order: middle cerebral, basilar, internal carotid, and an- terior cerebral. The anterior and posterior communicating and verte- bral arteries are occasionally involved, the posterior cerebral and in- ferior cerebellar very rarely (Gowers).
Etiology. — Males are affected slightly oftener than females. Aneu- 33
514 DISEASES OF THE NERVOUS SYSTEM.
risms occur at all ages from ten to sixty; before ten and after sixty they are extremely rare. Heredity occasionally plays a part in predis- posing to cerebral aneurisms. The exciting causes are embolism, es- pecially when the emboli contain microbes, syphilitic disease, in- juries, and in rare cases senile degeneration.
The symptoms are very indefinite ; they resemble to a considerable extent those of tumor at the base of the brain; headache and vertigo, mental dullness and irritation, cranial-nerve palsies, and occasionally hemiplegia and convulsions are noted. Optic neuritis is rather rare. In a few cases the patient is conscious of a murmur or recognizes the pulsating sensation in the head. Sometimes when the aneurism is in the vertebral artery, a murmur can be heard between the mastoid process and the spinal column (Moser).
The diagnosis is often difficult; it is based on symptoms of tumor at the base of the brain pressing on cranial nerves and on motor or sensory tracts. The effect of carotid compression should be tried.
The prognosis is not good. In perhaps the majority of cases a rup- ture of the vessel occurs in a few years; however, rupture is not the inevitable event, and sometimes the disease becomes stationary or undergoes spontaneous cure.
The treatment of the disease, if it can be recognized, is the same as that for aneurism elsewhere so far as drugs are concerned; surgi- cally, the common carotid may be tied and perhaps the vertebral if the aneurism is believed to be connected with that artery or with the basilar.
Syphilis of the Nervous System.
Syphilis is an extremely important factor in the causation of the organic diseases of the nervous system. Nervous syphilis makes up over ten per cent, of all the hereditary forms, and while in adult life the specific virus attacks nerve-centres relatively less often, yet it is a factor whose importance is very great. In the previous descriptions of nervous diseases we have referred to the syphilitic element in con- nection with etiology and pathology; but syphilis produces upon the nervous system certain forms of disease which are characteristic, hence it is best to take a brief survey of the effects of this infection independ- ently. Syphilis is now established to be the result of an infection by a micro-organism, and the result of the activity of this upon the nervous centres is to produce a condition which is really at first a form of inflammation.
Syphilis begins to attack the nervous system in two ways : by round- cell infiltration and exudation affecting the blood-vessels of the men-
TUMORS OF THE BRAIN — SYPHILIS. 515
inges, more rarely of the cord and brain; and by the development of a parenchymatous degeneration of the nerve substance. In all cases there is first the exudation and proliferative syphilis, but in about half the cases this is so slight that the first noticeable effect of syphilis is a degenera- tive process. Syphilis begins to attack the nervous system in from two to twelve years after infection, but it may occur within six months or as late as thirty years. If it occurs early the exudative proc- ess dominates and may be the only one. If it occurs late, the degen- erative process is the chief and apparently only one. The causes leading the syphilis organism to attack the nervous system are chiefly a hereditary weakness or predisposition; next, overuse of the nervous system, especially in sexual excesses and in laborious occupation and, finally, the abuse of alcohol and food and a mode of life which in general tends to weaken and poison the system.
There is some evidence that mild and hardly noticeable forms of infection are those likely to attack the nervous centres later. It is a fact that in about half the cases the patients deny either knowledge of any infection or the development of secondary cutaneous symptoms (rashes, sore throat, alopecia, pains, etc.). This apparent immunity of the cases with flagrant secondary symptoms may be due in part to the fact that these latter are more vigorously treated, but it does not altogether explain it.
It has seemed to several students of the question that very vigorous and prolonged and careful treatment of syphilis does not have any effect in warding off the degenerative or even exudative processes in the nervous centres. This was the conclusion of Collins and myself and it has been reached by others. However, I believe that with the modern and more thorough and prolonged treatment, there is some prophylactic result. Of all persons affected by syphilis it is probable that in 5 per cent, to 10 per cent, the nervous system becomes involved. Men are rather more often affected than women with nervous syphilis, and rather more in proportion to the infection because they are more subject to the exciting causes. The condi- tions of modern civilization have increased the amount of nervous syphilis, and this increase is mostly in the degenerative types of paresis and tabes. Syphilis may attack nerve-roots and ganlia, but practically does not affect the peripheral nerves, except secondarily. Exudative syphilis chooses especially the vessels and membranes at the base of the brain, especially in the posterior fossa, where the blood-supply is rich but the activity of the circulation less. It is not rare, however, in the central arteries. It affects the membranes and substance of the spinal cord, especially the dorsal cord in
DISEASES OF THE NERVOUS SYSTEM.
its lateral surface. Here it involves soon the substance of th cord also. Perivascular inflammation, with thickening of the arteries and involvement of the nerve-substance is the co mmonest type of disorder, local exudates and the formation of gummata are certainly rare at the present day. The degenerative process affects the posterior columns, roots and spinal ganglia in tabes; the lateral and posterior columns in progressive types of chronic myelitis; and the cortical cells of the brain in paresis. Nervous syphilis may affect both cord and brain with exudative and degenerative processes at the same time. Thus we may have a dorsal myelitis and a tabes together or a cerebral exudative syphilis with cranial nerve palsies and gum- mata accompanying the development of paresis.
Syphilis has been shown rather definitely to be due to infection by the spirochsete pallida, and this is found not only in the initial lesions, but in the later gummata. When degenerative processes set in, how- ever, as in tabes, it is found that certain antibodies have developed from the nerve-cells or the special exudates. These antibodies, how- ever, cannot be developed unless the organism of syphilis and its as- sumed toxins are present. Hence the demonstration of antibodies in de- generative or, as it is now called, parasyphilis show that the disease is still present. In other words, parasyphilis is not anything else than syphilis itself acting in a different way. Nervous syphilis, therefore, is a single thing, having its different stages of evolution. It may be, however, that in the terminal stage the poison is finally eliminated, leaving only the dying neurons. The stages of nervous syphilis, then, are these:
1. Perivascular, vascular and parenchymatous and meningeal exudates, causing nerve palsies, hemiplegia, paraplegia, headaches, convulsions, etc. Arrest or progress to recovery.
2. Parenchymatous degenerations, leading to tabes, paresis, tabo- paresis, progressive paraplegia.
3. Arrest and disappearance of toxin and antibodies or progressive terminal degeneration.
Symptoms.- — Since syphilitic changes may attack any part of the nervous centres, the symptomatology of nerve syphilis is necessarily a varied one. The reader can perhaps best understand the way in which the disease acts by having presented, first, a series of tables show- ing on the one side the clinical symptoms, on the other the chief ana- tomical changes that underlie them. The first table presents the symptoms of syphilis of the brain, which is unquestionably the most common form. The next table shows the symptoms of syphilis of the cerebrospinal system, a form which ranks second in frequency. Third,
TUMORS OF THE BRAIN — SYPHILIS.
517
we have syphilis of the spinal cord alone, which is somewhat rarer and, last and rarest of all, we have syphilis of the nerves. In addition to these four forms of syphilitic manifestation, we have two diseases which are acknowledged to be sequelae of syphilis and which are caUed postsyphilitic degenerative processes or degenerative syphilis.
I. — Syphilis of the Brain.
Clinical Symptoms. Anatomical Change.
Severe headache, vomiting, vertigo, men- Gummatous inflammation of the base
tal dullness; and irritability, attacks of involving nerve roots, or gummatous
somnolence or coma, convulsions, era- inflammation of convexity, arteritis,
nial-nerve palsies, optic neuritis, hemi- and phlebitis, plegia, polyuria, and polydipsia.
II, — Cerebrospinal Syphilis.
Many of the brain symptoms as above, Gummatous basilar meningitis; diffuse,
spastic paraplegia with spinai pains and disseminated, or localized menin go-
involvement of sphincters. myelitis.
III. — Spinal Syphilis. Paraplegia with pains, Brown-Sequard Meningi-myelitis, gumma, localized soft-
paralysis. enings from obliterative arteritis.
Spastic paraplegia and ataxia.
IV. — Syphilis of Nerves. Cranial-nerve palsies, cauda-equina Root neuritis, gummatous neuritis,
symptoms, local palsies of peripheral nerves.
V. — Late-Syphilitic Degenerative Processes (Parasyphilis).' Locomotor ataxia; general paresis. Progressive spastic spinal paralysis.
Taking up these different forms of nerve syphilis in order, I will give some further details with regard to each of them.
I. Syphilis of the brain in its most common form shows itself by a gradual development of severe and persistent headache. This is usually associated with vertigo, sometimes with nausea and vomiting. After the headache has developed and has lasted for a time, or even without much delay, there comes on sometimes an attack of hemiple- gia. Preceding the hemiplegia, or in some cases without the hemi- plegia, there are paralyses of the cranial nerves, more especially of the nerves of the eye. Optic neuritis is somewhat frequent. There may be, before any paralyses develop, attacks of epileptic convulsions, either general or partial. Without any paralyses or with simply cranial-nerve paralyses, there may develop attacks of somnolence and coma. Even if such attacks do not appear, the patient often shows a mental irritability and weakness, a slowness of the reasoning process, and incapacity to fix the attention such as is observed in connection
DISEASES OF THE NERVOUS SYSTEM.
with brain tumors, only with nerve syphilis these symptoms are not usually so marked. Polyuria and polydipsia are symptoms which are occasionally met with. It will be seen that the syphilitic poison pro- duces very various manifestations when it attacks the brain. The characteristic features are this variability in the symptoms and their remittent character. Elaborate systems of clinical classification might be made out of these various groups, but it will be sufficient for the present purpose to call attention to the fact that the intense head- aches, optic neuritis, cranial-nerve palsies, attacks of somnolence and coma, and hemiplegia associated with some of the foregoing symptoms are characteristic of most of the forms. The reason for the peculiar symptoms in brain syphilis is manifest when it is known that the lesion most commonly found underlying them is a gummatous meningitis which has a special predilection for the base of the brain. In particu- lar it seems to attack the interpeduncular space and the neighbor- hood of the optic chiasm and the surface of the pons Varolii. This gummatous meningitis consists of a syphilitic inflammatory exudate which surrounds, presses upon, and injures cranial nerves, attacks the arteries of the base, producing an obliterating arteritis and consequent softenings, with the hemiplegia which is so often a manifestation of the disease. Much less frequently the inflammatory process attacks the convexity, and then it assumes the form of a gummatous patch which produces cortical irritation with headaches, mental disturbances, and convulsions.
II. The next form of nervous syphilis is the cerebrospinal. In this we have almost exactly the same conditions and symptoms, so far as the brain is concerned ; but in addition there are symptoms due to more or less diffuse syphilitic inflammation of the pia mater of the spinal cord. The syphilitic process often extends into the spinal cord, producing an obliteration of the arteries and softening with the symp- toms of a transverse or a central myelitis. Thus we have combined the symptoms of cerebral syphilis and paraplegia with, as a rule, consider- able pain in the back, produced by the involvement of the meninges.
III. The third type of syphilis is the spinal form. The symptoms in spinal syphilis are usually those of a transverse myelitis, involving, in the writer's experience, most often the lower part of the dorsal and upper part of the lumbar cord. This myelitis usually comes on rather slowly with the ordinary symptoms of a chronic or subacute trans- verse myelitis, there being a progressive paraplegia with spasticity of the legs and a good deal of pain. The condition is known as syphilitic spinal paralysis. It is probable that syphilis is a much more frequent factor in the production of so-called transverse myelitis than is usually
TUMORS OF THE BRAIN — SYPHILIS.
519
supposed. The anatomical process underlying it is that of a menin- gitis which passes along the septa into the substance of the cord, in- volves the arteries of the cord, and produces a more or less complete softening of the part. The only truly inflammatory process, therefore, is that which is produced in the meninges, connective tissues, and arteries. The anatomical changes in the cord substance are mainly those of softening with reactive inflammation. Spinal syphilis may show itself also by the development of gummatous nodules which grow from the meninges, press upon the cord, and produce the symptoms of a spinal tumor. Spinal syphilis may also develop itself in three or four different foci, producing the symptomatology of disseminated myelitis.
IV. Syphilis of the nerves. Syphilis rarely affects the periph- eral nerves; there are, however, occasional deposits of syphilitic exu- date producing the ordinary symptoms of irritation and compression of nerves. There is said to be a form of multiple neuritis produced by syphilis, but its actual existence has not been demonstrated. Syphilis has been known to attack the roots of the cranial nerves, producing a root neuritis ; and it is very apt to attack the roots of the spinal nerves when the spinal membranes are involved.
V. The late syphilitic degenerative processes are locomotor ataxia, general paresis, tabo-paresis and progressive spinal paraplegia. Occasionally it happens that the syphilitic deposits in the spinal cord may produce lesions somewhat like those of locomotor ataxia, and in this case there will be a train of symptoms which also resemble this disease. In true ocomotor ataxia, however, the process is a degenerative, not an exudative one. The syphilitic poison seems so to affect the nervous centres as to predispose them to the peculiar degeneration characteristic of tabes. Syphilis may also produce a chronic meningo-encephalitis which will manifest itself by symptoms resembling general paralysis.
Hereditary Syphilis. — Inherited syphilis will lead to anatomical changes and clinical manifestations resembling in all respects those of acquired syphilis. Inherited syphilis, in other words, may pro- duce headaches, cranial-nerve palsies, hemiplegia, epilepsy, mental disorders, and paraplegia. The disease probably is the cause of a considerable proportion of the cases of chronic hydrocephalus and of many of the cases of so-called tuberculous meningitis. The peculi- arities of hereditary syphilis show themselves rather more in diffuse symptoms such as would be attributed to a meningitis of the con- vexity ; in other words, convulsions and mental weakness are rather more frequent, while hemiplegia and cranial-nerve palsies are com- paratively rare. Hereditary syphilis also very rarely indeed attacks
520 DISEASES OF THE NERVOUS SYSTEM.
the spinal cord, although it is not unlikely that it is a factor in the production of some of the hereditary diseases of that organ. Heredi- tary syphilis develops at any time from birth to the eighteenth year, but most commonly under the age of five years.
Pathology. — I have already given some indications of the pathological changes produced by syphilis. The disease affects the nervous system (i) by producing a meningitis with infiltration, (2) by producing gum- matous masses, (3) by producing an inflammation of the arteries, and (4) by so influencing the nervous system as to lead to the development of degenerative diseases. Of all these forms of anatomical change it is the arteries that are most often affected, and particularly the arteries at the base of the brain.
Syphilitic meningitis is characterized by the proliferation of round cells and the preponderance of an exudate which has a tendency to infiltrate into the nervous tissues. The anatomical characteristics of the syphilitic gumma must be studied in special text-books. The in- flammation of the arteries attacks first the external coat and adventi- tia, producing there an enormous multiplication of round cells. The external coat becomes weakened, and as a result there develops be- neath it, between the intima and the elastic layer, another exudate which constitutes what is known as endarteritis. In syphilitic arteri- tis, therefore, there is both a peri-arteritis and an endarteritis; the former being usually the primary and most essential process. The endarteritis, however, as it develops gradually produces an occlusion of the arteries. This cuts off the circulation of the blood and leads to softening of the part. There is also a development of a hyaline de- generation in the arteries, which some regard as a very essential part of the anatomical change.
The cerebrospinal fluid in the majority of cases contains lymphoid cells and also syphilitic antibodies, and these are more marked the more advanced the disease and the more definite the evidence of a former syphilitic infection. Wassermann and Prout, who established this fact, think the antibodies are developed by the nerve-cells. The method of testing for these antibodies is a difficut and delicate one ; and the fact of their existence is more important in clearing up the pathology of tabes and paresis than in diagnosis.
Diagnosis. — The diagnosis of nervous syphilis is based upon the history of an infection, the irregularity and fugacity of the symp- toms, the intense headaches, the presence of an optic neuritis, the age of the patient, and the results of treatment. In estimating the im- portance of the history of infection, it should be remembered that the third year after infection is the serious one for the development in
TUMORS OF THE BRAIN — SYPHILIS.
particular of those symptoms produced by obliterating arteritis. In hereditary syphilis the presence of the Hutchinson teeth, the hazy cornea, and deafness or other ear trouble help us in diagnosis. The headache of syphilis is rather characteristic. It may attack any part of the head, but is usually unilateral or irregular, or again it may be bilateral in its distribution. The pain is very intense and sometimes exhibits a certain periodicity. It is usually worse at night. It is apt to last continuously for from five days to three or four weeks. Head- ache of this character, followed by the paralysis of one or more cranial nerves or by an attack of hemiplegia, is extremely suggestive of syphilis. Optic neuritis is very liable to occur when the disease shows other evidences of being situated at the base of the brain. This optic neuritis is associated with contraction of the visual field, and a char- acteristic feature of this contraction is that it varies a great deal from week to week. The cerebrospinal fluid may furnish the decisive test. The sex and age of the patient may be taken into consideration in weighing the evidence, and finally the prompt effects of the use of iodide of potassium should have very decided weight.
Prognosis. — It is very difficult to give definite facts regarding the prognosis of syphilis. Unquestionably, the outlook is much more favorable than it is for any other organic disease of the nervous system. When the syphilitic process has not produced so much arterial disease as to lead to obliteration of vessels and softening, a very great degree of improvement and even a recovery may be expected. So far as injuries to the nerves or nerve-roots go, we can generally expect a great im- provement or cure. Lesions of the convexity are usually amenable to treatment. Syphilitic hemiplegia has a not much better prognosis than hemiplegia from other causes. Syphilitic myelitis has a not very good prognosis, but it is better than that of myelitis due to trauma. Nervous syphilis may last from one to three or four years. The effects of the disease may, if nerve-tissue is destroyed, last a lifetime.
Treatment. — As regards the prophylaxis, it is important that per- sons who have become infected by syphilis should be treated with mer- cury and iodide of potassium in the second as well as the third stage of the disease. After the first year at least, the patient should not ne- glect to take a certain amount of iodide of potassium and mercury four times a year, each course of treatment lasting six weeks. The patient should be warned against indulging in alcohol, against all ex- cesses, mental as well as physical. A laborious life full of worry and anxiety, in which the patient attempts to help himself along with stimulants, is surely provocative of nervous syphilis.
It is stated that infection with syphilis can be prevented by
522
DISEASES OF THE NERVOUS SYSTEM.
always using after connection a local application of mercury. The treatment of the disease when it has appeared consists mainly in the administration of mercury and of the iodide of potassium or sodium. This latter should be given in beginning doses of ten grains three times a day and increased gradually until the maximum amount which the patient can bear is taken. This maximum is usually between three and four hundred grains a day. In some cases it is important to give more than this — as much, that is to say, as two hundred grains three times a day. In my own experience I have known a patient to take five hundred grains three times a day for a considerable time without harm, and indeed with benefit. Usually, however, such ex- traordinary doses are not needed. It is found that, as a rule, patients tolerate large doses of iodide quite as well as smaller ones, and sometimes the iodism produced by small doses disappears when large doses are given. The drug is best administered largely diluted with water or with Vichy or in milk, and taken after meals. Some persons bear it better before meals. It is advisable to give mercury alternating with the iodide. This may be given in the form of the bichloride or by an inunction.
In recent years the treatment of syphilis by hypodermic injections of the bichloride or salicylate of mercury has been widely adopted, and apparently better results have been obtained than by inunction or by administration by the mouth. One or two grains of the salicylate mixed with a liquid petroleum oil may be given twice a week. Mer- cury can be given with advantage in the early stage of parasyphilis, but not in the late stage.
Other drugs which are of value are the ordinary tonics, such as iron, quinine, and the bitters and mineral acids. Plenty of good food, out-door air, and all those things which will improve the general health of the patient are indicated.
CHAPTER XXI.
FUNCTIONAL NERVOUS DISEASES.
Functional nervous diseases are those in which no definite known anatomical change underlies the morbid phenomena. On this account, it is customary to classify them on a clinical basis. We can, however, also make etiological and pathogenic subdivisions. Applying such a method now, we have two broadly distinguished classes : the pri- mary, or degenerative, and the secondary, or acquired neuroses. Such a classification is suggestive and helpful, though not perfectly correct, because several factors often enter into the cause of the same neurosis, including now some distinctly recognized infections and poisons.
[ Primary neurasthenia.
Primary degenerative j Epilepsy.
7 & \ Hysteria major,
neuroses. „ A-, <
| Hereditary chorea.
( Myoclonia, and myotonia, including motor tics. { Chorea.
r? • r , i j Tetanus.
From infectious, autoch- j ^
thonous, and miner a lj R^jy*1^'
P°is°ns- I Tremor.
[ Neuralgia.
{ Neurasthenia.
Acquired , Exhaustion and shock neu- j Hysteria.
neuroses. | roses. ] Exophthalmic goitre.
[ Occupation neuroses.
A . i ■, ,. f Tic douloureux.
Acquired degenerative neu- T , ,. n ° < .Local spasmodic tics,
roses
Paralysis agitans.
( Miscellaneous. Vasomotor, trophic, and sleep disorders.
EPILEPSY.
Epilepsy is said by some to be a protean manifestation due to different morbid conditions and not to be a special disease. This may be in a sense true, but, after all, the great majority of cases have a natural history which has common features and make its descrip- tion as a single disease feasible and useful.
Idiopathic epilepsy is a chronic functional disorder characterized by periodical seizures attended by loss of consciousness and usually by convulsions. Mental disturbances may accompany or take the place of the convulsions. It has no known pathological anatomy, unless it be certain degenerative cell changes in the brain cortex.
523
524
DISEASES OF THE NERVOUS SYSTEM.
Symptomatic epilepsy is a form in which the periodic convulsive attacks are associated with gross organic changes in the brain.
Jacksonian or partial epilepsy is a form of epilepsy, usually symptomatic, and is characterized by periodic convulsions affecting only certain groups of muscles, and often unattended by loss of consciousness.
Hystero-epilepsy is not epilepsy, but a form of hysteria.
Eclampsia or acute epilepsy is the name given to a single isolated attack of convulsions. It is generally of the symptomatic type.
Idiopathic epilepsy shows itself in three rather distinct types of attacks, viz., that of severe attacks called the grand mal; that of minor attacks, the petit mal; and the rarer larvated forms charac- terized by acute mental disorder and called psychical epilepsy or the psychical epileptic equivalent.
Etiology. — Predisposing causes: Heredity is the most potent of any single influence. A history of epilepsy or insanity is found in the family in about one-third of the cases and rather more on the paternal side. In many other cases it can only be established that the family is a neuropathic one. The heredity is rarely a direct one ; i. e., the parents are not epileptic in more than about i percent, of cases. Alcoholism and the intermarriage of neurotic persons contribute to produce the convulsive tendency in children. The element of alco- holism is not as large as has been generally stated. In my experience it is only about 5 per cent. It must be remembered that many parents reported alcoholic were not so at the time of the birth of the child. Epilepsy is prevalent in the Jewish race, which is not one of alcoholic habits. Powerful emotions during pregnancy, accouchement injuries, and syphilis have some influence. Children at the time of birth may have a meningeal hemorrhage leading to injury and atrophy of one or both of the cerebral hemispheres. Later in infancy or childhood, attacks of encephalitis may occur leading to mental defects and paralysis. About 40 per cent, of infantile hemiplegics have epilepsy. More cases occur in the country than the city, more in temperate climates, and more among in-bred races. American statistics (Putzel, Hamilton, Hammond, Starr, and myself) show a slight preponderance among males.
Age. — -The epileptic age is between birth and twenty, and still more definitely between five and fifteen. In three-fourths of the cases the disease begins before the age of twenty-; in one-sixth of my cases, before the age of five. After twenty the danger of epilepsy is slight, and when it occurs it is usually due to accidental causes, like syphilis, alcoholism, or plumbism. Idopathic epilepsy, however, may develop
FUNCTIONAL NERVOUS DISEASES.
525
even after sixty. The accompanying table shows graphically the relation of age to the development of epilepsy, chorea, and neuralgias.
Exciting Causes.— Exciting causes are not present in the majority of cases. The most important are the occurrence of rickets at the time of dentition, fright, injury to the head, sunstroke, infectious diseases, especially scarlatina, masturbation, alcoholism, and syphilis. The im- portance of traumatism when there has been a real injury to the brain is considerable, amounting to about 10 per cent. Simple concussions without organic injury do little harm. Masturbation is a real but rare
|
Percentage of total cases 60% 50% 40 % 30% 20% 10/c |
up to end of 5thyear |
6 th to |
to 15 ^ |
I6'.h |
2l6.f to 30^ |
3ls.f to a nth |
41s.1 tou 50- |
51s.1 to 60- |
61 V to |
|
|
— V— |
||||||||||
|
A |
||||||||||
|
'/ |
— \f> |
\ |
Fig. 229. — Table showing percentage of cases of epilepsy, chorea, and neuralgia occurring at each half -decade and (after twenty) each decade. Double line, epilepsy; dotted line, chorea; single line, neuralgia.
cause, so also is syphilis. The so-called reflex causes are ocular and auditory irritations, worms , dyspeptic states, dental irritations, lesions involving peripheral nerves. Some American observers put much stress on the importance of ocular irritations. Probably the gastro- intestinal tract and genital organs furnish the most important exciting irritations. True idiopathic epilepsy may be brought out by peripheral irritations; more rarely there occurs only a reflex epileptiform neurosis. Much stress is now laid on intestinal auto-infection as a factor in epilepsy.
526
DISEASES OF THE NERVOUS SYSTEM.
Symptoms. The Onset. — The disease begins sometimes with attacks of petit mal which may last one or more years before anything more serious occurs. Then suddenly there is a convulsion. Occa- sionally nothing but petit mal attacks ever occur. In other cases without previous warning the patient has a severe seizure. In six months or a year he has another, in the next year he has three or four and then they come on regularly every week or two.
The Convulsion. — The patient often feels some premonitory symp- toms for a few hours or a day, consisting of general malaise, irrita- bility, or giddiness. The attack begins in about half the cases with a peculiar sensation called the aura. Often also a loud cry is uttered and the patient falls unconscious to the ground. The face is pale, the eyes are open and turned up or to one side, and the pupils dilated. The head is drawn back or to one side, and the whole body is in a state of rigidity or tonic spasm. The arms are slightly drawn out from the trunk, the forearms, and wrists extended or flexed, the fingers clinched or flexed in other ways, the legs and feet extended. This tonic stage lasts for fifteen or twenty seconds; the face becomes congested and then livid from compression of the veins of the neck and stoppage of respi- ration. Gradually jerky movements of the face and limbs begin and the stage of clonic spasm sets in. The trunk and limbs are now alter- nately flexed and extended with violent shock-like contractions, the facial and eye-muscles twitch, saliva collects in the mouth, and as the tongue is often bitten it becomes stained with blood. The movements are sometimes so violent that the patient is thrown about the bed or floor, and occasionally a limb is dislocated, usually the shoulder. The urine often, and the fseces occasionally, are passed. The temperature is raised +° or i° F., rarely more. The pulse, feeble at first, becomes frequent and tense, and then, as the attack subsides, becomes feeble again. The clonic spasm lasts from one-half to one or two minutes. It subsides gradually, and the patient sinks into a stupor, from which he can be roused with difficulty. This stupor is succeeded by a heavy sleep of several hours and a feeling of hebetude which lasts all day. Vomiting sometimes occurs as a terminal symptom. Immediately after the attack there is a temporary exhaustive paralysis, with loss of knee-jerk. The pupils contract again and often oscillate. There may be a slight amount of transient albuminuria or glycosuria. The earthy phosphates are found increased ; urea is not. There is a distinct lessening of haemoglobin in the blood (Fere) and of hgematoblasts. Sometimes the attack is followed by others, and for hours the patient passes from one convulsion into another. This condition is called status epilepticus. It usually lasts less than twelve hours, but may
FUNCTIONAL NERVOUS DISEASES. 527
last for one or more days and until finally death occurs from exhaustion. It develops only in the severer types.
Symptoms of the Minor Attacks. — In the minor attacks {petit mal) the patient suddenly stops in anything in which he is engaged, the features become fixed, the eyes open, the face is pale, the pupils are dilated, often slight twitching of the facial muscles or of the limbs occurs, and consciousness is lost. In a few seconds the attack is over, and the patient, who does not fall, resumes his work or conversation, being unconscious of what has occurred, except that he has had a "spell." Often there is a warning sensation of aura. This is felt as giddiness, sense of fear, numb sensations of the extremities, flashes of light or blindness, or choking sensations. There may be a cry uttered. The minor attacks are in rarer cases accompanied by sud- den forced movements; the patient runs a few steps, or turns round, or makes some automatic movements. This is called procursive epilepsy.
Symptoms of the Psychical Attacks. — Sometimes the minor attacks are followed by outbursts of maniacal excitement or by sudden violent automatic movements, and in these states the patient may commit crimes of violence. In rare cases the patient passes into a somnam- bulic state, during which he performs accustomed acts, such as driving and walking, automatically and naturally (somnambulic epilepsy). This form of epilepsy may come on without a preliminary minor attack, and then it is to be considered a "psychical epileptic equivalent."
Minor attacks may end in convulsions of a co-ordinate type in which the patient jumps, kicks, throws the arms about as in hysteri- cal attacks. These are called hysteroid convulsions.
The seizure may consist of only a short tonic stage and a "few twitchings of the limbs, the whole lasting but a few seconds. This is called an abortive attack. Under the influence of medication, the severe seizures are often reduced to abortive forms.
Jacksonian or partial epilepsy is a form of the disease character- ized by convulsive attacks affecting only a single group of muscles or a limb, and generally not accompanied by loss of consciousness. Jacksonian epilepsy is always symptomatic of some focal lesion affecting the cortical motor area of the brain. This may be a tumor, inflammation, or injury, or only some slight degenerative change in the cells of the cortex. This form of seizure is particularly significant of a slowly growing brain tumor or syphilis.
The aura usually consists of a sensation of numbness, prickling or of a breeze beginning in the hand or leg and passing up to the head when consciousness is lost. Still oftener there is a peculiar sensation
528 DISEASES OF THE NERVOUS SYSTEM.
starting in the epigastrium and passing upward. More rarely there are special-sense aurae, such as a ball of light in one eye, noises, or voices and peculiar tastes or smells. Besides these there occur sudden desire to go to stool, feelings of giddiness, dreamy states, peculiar sensations in the head, and indescribable general sensations.
The aurae may be divided into:
Visceral — epigastric, laryngeal, cardiac, rectal.
Cutaneous sensations.
Special senses — flashes of light, etc.
Psychical — emotions, dreamy states, etc.
Cephalic — giddiness, etc.
The aura is thought to indicate the seat of the first discharge of nerve force, and its study is of most importance in connection with symptomatic epilepsies, as will be shown later. The patient may have only the aura for a long time before the convulsion sets in. Some times there never is anything but the aura. This undeveloped form of epilepsy I have called paraepilepsy.
Relative frequency of the different kinds of attacks. The severe attacks are the most frequent, next come combinations of severe and minor attacks, and next minor attacks alone, while the psychical forms are the rarest.
Frequency of the Attacks. — The severe attacks may come on only once or twice a year, and this commonly occurs during the develop- ment of the disease. The frequency gradually increases until they occur every month, or two or three times a month. Sometimes the fits occur in groups of four or five every month or two. In very bad cases convulsions occur every day. The petit-mal attacks are more frequent and often occur daily.
Time of Attacks. — The moon and the seasons have no influence. More attacks occur during waking hours than during sleep; but two-thirds of the attacks occur between 8 a. m. and 9 p. m. Many patients have their attacks early in the morning just after awakening (matutinal epilepsy). According to Pierce Clark, the hour in which the greatest number of attacks occur is 9 p. m., and the fewest attacks occur between 4 and 7 p. m.
State of Patient Between A tta ck $.— Epileptic patients often feel better for a time after the convulsion is over. They not rarely suffer from severe neuralgic headaches; the appetite is capricious, often in children it is voracious, but in older cases there may be anorexia; the bowels are usually constipated ; the pulse is small, soft, and frequent in the young, later it is often slow.
Mental Condition. — A gradual mental deterioration occurs in the
FUNCTIONAL NERVOUS DISEASES.
529
great majority of epileptics, but it is slight in some and not very great in others. It shows itself by feebleness of memory, irritability of temper, selfishness, incapacity to concentrate the mind or to carry out a purpose. In children great mischievousness and lack of moral sense, with vicious impulses, may appear. The mental deterioration is dependent on those underlying factors which cause the disease. It is apparently in some cases due to the excessive number of the fits. This is not necessarily the case, nor is it generally true that it occurs more often with petit mal. It is more marked in cases beginning very early in life, but this is true only when there are decided marks of physical and mental degeneration present. A certain rather small percentage of epileptics become either demented or insane. True epilepsy is rarely compatible with extraordinary intellectual endow- ments. Caesar, Napoleon, Peter the Great, and other geniuses may have had some symptomatic fits, but not idiopathic epilepsy.
Physical Condition. — epileptics are usually undersized and of not very robust constitution (Fere). Some patients, however, present a very vigorous muscular development. They always present some of the marks of degeneration, physical, physiological, or mental. Such marks or stigmata are about ten times more frequent than in healthy persons. The physical stigmata are (Fere) short stature, cra- nial asymmetry (in 71 per cent.), short parietal or frontal arc, and triangular skull ; in women high prominent forehead ; bad teeth badly placed, high palatal arch; facial asymmetry; prominence of occiput and lemurian hypophysis; differences in color, size, position and shape of pupils; astigmatism (in 75 per cent, of cases); badly shaped and placed ears ; misplaced crown of scalp ; low vital capacity ; small genitals, atrophic uterus ; greater development of left side ; long fingers. Cranial deformities of pronounced type occur in epileptics associated with idiocy, hemiplegia and brain defects of early origin. Some- times, apparently from a premature ossification of sutures, there are the peculiar shapes of the skull known as scaphocephaly, or steeple skull, and plagiocephaly, or obliquely deformed skull.
The physiological marks of deterioration are a lessened muscular strength (as 35 to 50), habit choreas, a rather imperfect eye with excessive amount of astigmatism and functional muscular weakness. There is a lessened vital capacity, weak and slow digestion, often de- fective metabolism and sexual atrophy or irritabilty. The excretion of phosphoric acid is below normal as compared with urea.
The psychical stigmata are mental feebleness, moral insensibility, irritability, wayward and vicious impulses, lack of will power, and sexual aberrations. 34
53°
DISEASES OF THE NERVOUS SYSTEM.
Association Diseases. — Epilepsy may be complicated with a de- generative myoclonia (myoclonus epilepsy). It is sometimes seen in connection with torticollis and spasmodic tic of other types. Epileptic seizures occur in the cerebral syphilis and general paresis.
Epilepsy is regarded by some as not a disease, but a syndrome or symptom-complex due to various morbid conditions of the brain. It is true that epilepsy runs a varying course and presents different features in different individuals. The grouping of cases in accordance with the severity and type of attack, the degree of intelligence, the tendency toward deterioration, the physical and physiological defects, the tendency to arrest, improvement, or increased severity of the dis- ease is, therefore, desirable. Taking into account these factors, I find in my experience the following general types or groups. It is not pretended that this is an exact classification. Such a one is not yet possibl . It is fairly accurate, as I have seen epilepsy and I have watched many cases for ten to twenty-five years.
The types are :
I. The common mixed form. The attacks begin about puberty. They are of haut and petit-mal type, but it is the haut-mal that is trouble- some. The seizures settl down to fcur to six monthly and occur singly or in groups, by day or night.
The patients have some morbid mental traits ; they are of moderate intelligence and this deteriorates a little, but does not end in insanity or dementia. The physique is fairly good, often robust, as to the mus- cular system, there are generally defects of physiological function. These patients sometimes get well or get their attacks well under control.
II. The petit-mal and psychic type. The attacks are of the psychic and mild type alone, at least for very many years. They come on, as a rule, in early childhood at the fourth and fifth year or even sooner. They increase until they occur many times daily. The patients are bright, often very bright and precocious children, and they do not dement as the years go on, unless haut mat attacks set in. They may, however, develop mental peculiarities and defects. Physically, they are of delicate but sound physique. These cases do not get well. With this petit-mal type go most of the cases of cerebral automatism, psychical epilepsy, epileptic sleep attacks, etc.
III. The delayed type, epilepsia tardiva.
The attacks do not come on until after adolescence, and sometimes not till the climacteric. They are of haut-mal type, but petit mal is usually present in some degree. There is sometimes a history of cause, such as infection, trauma, syphilis, overstrain. The attacks
FUNCTIONAL NERVOUS DISEASES.
531
are not very frequent relatively, i. e.t one or two a month. They are controlled fairly easily, though not entirely. The patients are of fair intelligence, of higher degree at least than those of type I. They do not deteriorate very much. The epilepsy of advanced years is not of this type, but is a phase of senile dementia.
IV. The organic and degenerate type. The attacks come on in con- nection with gross defects or disease or injuries of the brain. There is retarded development, idiocy, imbecility, perversions and lack of bal- ance in the mental functions. Paralyses, tics, etc., are present. Here the epilepsy is only one expression of the morbid cerebral condition. The cases are hopeless, so far as cure is concerned.
Underlying all the types there is, as a cause of the seizure, some degen- erative cell or neuron change. It is very probable that this change is of very much the same character in all cases, and that the disease differs in course and symptoms because the changes vary in locality, extent and intensity. So that contrary to the views of some, epilepsy is in my opinion almost a specific disease. Even in idiots and paralytics the epileptic phenomena are due to this special finer cellular degenera- tion, for the brain may be greatly injured, badly developed, full of cysts or tumors and disease, yet the epilepsy does not always appear.
Pathology. — The body of the epileptic shows sometimes skin erup- tions and ulcers, the result of treatment. There are often evidences of local injuries and fractures due to falls. The organs may show vices of conformation. The uterus is frequently infantile or sharply flexed. Deformation of the occipital bone or the atlas so as to produce narrow- ing of the upper spinal canal has been noticed. The brain may be unduly large or small, but there is nothing constant in this, nor is there an ab- normal difference in the weight of the two hemispheres. The convolu- tions show many anomalies, but there is in them nothing specific. On the whole the convolutional type is a simple one. A pathological change found in epilepsy is an induration or sclerosis (gliosis). This affects the cornu ammonis rather often (4 to 10 per cent.), more rarely the olivary bodies or cerebellum. Besides this, small patches of induration occur in the gray matter in various parts of the cortex. Chaslin finds a diffuse increase of neuroglia tissue throughout the brain, more marked when the case is older. Others have found an increase in the neuroglia cells (Kingsbury). In old cases there is often a chronic leptomeningitis, and vascular changes due to the frequent congestions of the brain take place. These consist in varicose and fusiform dila- tation of vessels, with evidence of small hemorrhages. Slight degenera- tive changes in the nerve-fibres are also observed.
Bevan Tewis finds in epileptics with insanity a fatty degeneration
532
DISEASES OF THE NERVOUS SYSTEM.
of the nuclei of the "angular cells" of the second layer of the cortex. In the severer and later stages of the disease this nuclear degeneration is increased so that vacuoles are formed. The cells of the deeper layers are also affected, but to a less extent. The change, though not peculiar to epilepsy, is more extensive and pronounced in this disease. Recent investigators (Onuf, Collins, Clark and Pront) have found degenerative changes in the cortical cells.
To sum up : The anatomical basis of idiopathic epilepsy, so far as it has any, consists in a degeneration of the cortical cells. Also a proliferation and increase in the neuroglia tissue, this occurring most markedly in various islets or special areas of the cortex. The blood- vessels and connective tissue are involved only secondarily and later.
Toxcemia and Epilepsy. — Many elaborate investigations have been made to ascertain whether epilepsy is not due to some toxic cause or perverted metabolism. There is much in the history of the disease to lead one to hope for a solution of the problem here. Cholin, uric acid and carbonate of ammonia have been thought to be the exciting poisons. But the toxic theory is not yet proved. Even in alcoholic and lead epilepsy, the toxic agent is probably a secondary one.
Physiology. — The epileptic fits are due to sudden discharges of nerve force. The seat of the discharge is the cortex of the brain. The discharging cells are, in the severe seizures, the large motor cells, the function of which is to store up and discharge nerve force. They are under control of the cells (angular cells) of the second layer, which have an inhibitory power. These being diseased, their control is weakened and the motor cells "explode" periodically. In sensory and psychical epilepsy the same mechanism exists. The more highly organized cells with large nuclei of the second layer are congenitally or otherwise weak and diseased ; the cells below them are not main- tained in stable equilibrium and hence periodically break down and " discharge."
The diagnosis is based on the character of the attacks and has to be made from hysterical and various toxic and symptomatic convulsions.
The aura, the scream, the quick loss of consciousness, the dilated pupils, the tonic convulsion, the bitten tongue, the emptied bladder are all characteristic. The hysterical patient sometimes, but rarely, loses consciousness, the epileptic almost always. Hysterical patients do not hurt themselves in falling or bite their tongue, and their muscu- lar movements, while irregular and violent in character, are yet co-or- dinate, i. e., they throw themselves about, kick, strike, etc. Their
FUNCTIONAL NERVOUS DISEASES.
533
attacks often are produced by emotion and are ended by some power- ful mental or physical impression. They rarely occur in sleep. The slight rise of temperature in epileptics rarely occurs in hysterics. Petit mal and epileptic vertigo are distinguished by the sudden lapse of consciousness and by the sudden pallor and fixation of the eyes, dilatation of the pupils, and slight twitchings of the face. Nocturnal convulsions are usually epileptic.
Eclampsia, or acute symptomatic and reflex convulsions, cannot always be distinguished from epilepsy. The history of the case, the irregular and often prolonged character of the fit, may enable one to make the diagnosis.
Course and Prognosis. — Epilepsy shortens life to some extent; most subjects do not live beyond the age of forty or fifty. About ten per cent, become demented or insane. Five or ten per cent, get well. The remainder reach a certain stage of severity in their disease and continue in it for years. This severity depends on the treatment, the nature of the attacks, and the extent of degeneration which the organism shows. While unquestionably treatment cures or suppresses the disease in some cases, it disappears spontaneously in others. Epilepsy developing after adolescence is not easily curable, but is rather easily controlled and is not so serious, except sometimes in old people. The prognosis of petit mal is worse than that of grand mal; that of the two combined is worse still, yet not hopeless. The psychical form of epilepsy is the least amenable to treatment. Epilep- tic insanity and dementia are incurable. Death occurs rarely in the attacks except in terminal stages. Yet the status epilepticus is al- ways a source of danger.
Epileptics are said rather frequently to suffer from phthisis. This is, however, a matter of infection and can be prevented. It should be remembered that epileptics who have only a moderate number of attacks, six to fifteen yearly, can get along comfortably for years, doing their work and enjoying a fair share of the duties and pleasures of life. Finally, the following prognostic rules may be laid down: The prognosis is better in males, better if there is no hereditary history, better if the fits are nocturnal or diurnal alone, better in grand mal, better if fits occur infrequently, better if they begin after twenty, and better if due to extrinsic causes. The prognosis is very bad in post-hemiplegic epilepsy and epilepsy due to organic disease, and in persons mentally weak or deteriorated.
Treatment. — The first and essential rule of treatment is to take cases early and treat them vigorously from the start. Children who have had a few convulsions during the first three or five years of life
534
DISEASES OF THE NERVOUS SYSTEM.
should be treated as if they might develop epilepsy between the ages of ten and fifteen or earlier. The recurrence of a fit between the ages of five and ten should excite apprehension and call for the most diligent treatment. Another rule is that when epilepsy is recognized in children the case should be treated constantly for at least three years after all attacks have ceased.
Constitutional Treatment. — Along with the evolution of epilepsy there is probably a progressive diffuse neuroglia sclerosis of the brain. Whether this is primary or secondary, it is at least proper to use those measures which apparently affect this neuroglia proliferation. Mer- cury, arsenic and perhaps iodide of potassium are drugs which we have good reason for believing affect this.
Besides this, we should use measures that increase vasomotor tone and strengthen and steady the circulation. Nothing does this better than water. Epileptics should be given showers, douches, cold sponge baths, or wetpacks according to their needs and opportunities. They should also drink water freely. Again, the nervous system is greatly steadied and quieted by mental occupation that interests one. Noth- ing is more unfortunate than the idleness often enforced on epileptics. I have seen the disease absolutely checked by having a boy learn a trade that he liked.
The next most important indication is diet, the prevention of in- testinal decomposition. In petit mal particularly an absolutely non- irritating diet, such as milk, meat and bread, will quickly lessen or stop the attacks. Meats can be taken in moderation if eaten slowly. As a rule it is a little safer to keep meat out of children's diet for a time; but in adults it is not necessary, though it should be given in moderation. A practically salt-free diet is advisable part of the time.
Removal of irritating causes. Malaria, if present, promotes the convulsive tendency; so also do lead and alcohol; tobacco does not do this, but its use is better stopped, as it is liable to weaken vascular tone and impair digestion. Syphilis causes epilepsy only through pro- ducing organic changes.
The rheumatic, gouty, and so-called tuberculous diatheses do not stand in any close relation to epilepsy. The condition known as lithaemia, however, in which there are insufficient oxidation and ex- cretion of products of tissue waste, needs attention. Hence the use of bicarbonate of potassium, salicylate of sodium, the alkaline mineral waters, and a restricted diet are not rarely indicated.
The importance of reflex irritations has been much overestimated. Still they must be considered. The most serious are those arising from the gastrointestinal tract, the sexual organs, and the eyes.
FUNCTIONAL NERVOUS DISEASES.
535
Phimosis if present must be relieved, and masturbation or sexual excesses stopped, if possible. It is admitted now that removal of the ovaries, even if diseased, never cures true epilepsy, though it may help hysterical convulsions.
Astigmatism and hypermetropia should be corrected ; also ocular insufficiencies if these are pronounced.
Proper attention to the frequent constipation and dyspepsia is of course necessary. The use of hot water is often serviceable, a glass being sipped slowly before the morning and evening meals. This helps also to carry off the bromides and wash out the system generally. Still further to promote this, a purge should be given every fortnight or month. Out-door life and active physical exercise were indorsed by Hippocrates. They do not have any specific influence unless asso- ciated with some employment.
Specific Treatment. — Physicians who undertake the treatment of epileptics often do not realize the seriousness of their responsibilities. Many, I fear, simply give a little bromide, stop the meat, circumcise the boy, and say they think the child will outgrow it. But children do not outgrow it; they steadily get worse unless something definite is done, and well done for a long time. With proper, prompt, and pro- longed treatment, the attacks can be entirely controlled in 5 to 10 per cent, of cases, and I believe more. They can be greatly controlled in over one-half the cases, so that the patient may be able to continue his education and do some work in life. But this cannot be accomplished by any casual dosing or occasional consultation with some high authority. The physician should approach the responsibility of a case of epilepsy as he would that of a mortal surgical condition, in which much depends on knowledge and attention to all the details of a long technic.
It is conceded that the colony treatment of epilepsy is the one which approaches most nearly the ideal in effectiveness, but it cannot be applied to all classes — at least, for a long time — and perhaps never to a certain rather large percentage. There must always, therefore, be a good many epileptics who have to be treated at their homes, and whose care must be directed by the family physician or the specialist.
For this class of persons I have gradually evolved a conventional or routine treatment of epilepsy, which seems to produce the most satisfactory results in those cases in which a reasonable opportunity for therapeutic effort exists. That is to say, cases which are not of very long standing, and which have not already undergone serious mental deterioration, and cases in which the mental and physical degeneration, at the beginning, is not of a very high grade.
536 DISEASES OF THE NERVOUS SYSTEM.
The features upon which emphasis must be laid, in the treatment of epilepsy, are: —
First, the fact that the course of treatment about to be instituted is to last for at least two years, and that all measures prescribed must be carried out with the greatest fidelity and exactness during that time, no matter how well the patient may seem, or how unnecessary regimen and drugs may appear to be. The preparation and outlining of treatment should receive the care and attention such as is given to a capital operation.
Second, the use of the pure bromide of sodium or some other salt (there is no special merit in mixed bromides) in combination with the glycero-phosphate of soda or of soda and lime so that a patient takes on an average 60 grains of the bromide of soda and 20 to 30 grains of the phosphate, in twenty-four hours. To this combination iron and a little arsenic may be added, if needed. It has seemed to me proved, beyond any question, that by the combination of proper soluble phos- phates with bromide, the depressing effect of the bromide can be largely gotten rid of, and the patient can continue bright and active, and grow fat under a fairly large dose of the drug. This has been tested by me now for about six years, mainly in private practice, but also in my dispensary work. The maximum dose of bromide which can be taken in this combination is sometimes as high as 90 grains a day, but, rarely, more, and not often as much.
Third, I have found it a most efficacious plan in treatment to in- termit the medication for either one or two days in each seven. Dur- ing these days, e.g., a Wednesday and Sunday, the drug is stopped, and in its place is given, three times a day, before meals, a tumbler of hot water, and with it an alkaline laxative. The ordinary tablet of rhu- barb and soda, with nux vomica, usually answers this purpose. Some- times 20 grains of bicarbonate of soda is enough. Its purpose is to flush out the stomach and bowel and cleanse the gastrointestinal canal twice a week, thus preventing the accumulation of drugs and toxins. After meals on these days I sometimes give 10 drops of tincture of iron or some needed tonic. I have not seen any access of convulsion during the day or the day after such intermission. With this stomach treat- ment there should be a colon irrigation using a hot high bicarbonate of soda solution. This is given about once a week. Sometimes in its place I use an injection of a pint of warm sweet oil at night followed by a simple enema in the morning.
Fourth, an important measure which I employ in the treatment is the securing of violent physical exercise for about twenty or thirty minutes, at least, three times a week. This must be done either by
FUNCTIONAL NERVOUS DISEASES.
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some active sport, like boxing, by exercises in the gymnasium, by tennis, skating, or by the simpler methods of chopping or sawing wood or punching the bag; but the exercises of whatever kind should be short and to the point of free perspiration. After this exercise, the patient is given a cool bath. Delicate persons, women, and those who are unable to carry out such exertion, I direct to purchase a "hot-box" and take a hot-box sweat, followed by a cool bath, three times a week. These boxes are not expensive, and can be set up with little trouble.
Fifth, the question of diet is attended to, as indicated above.
The medication should be increased rather than diminished as the patient gets better*; he should take more, if possible, at the end of the sec- ond year than at the beginning of the first, if the attacks are controlled. After four years one can feel safe, not before. There are few other drugs of any importance in epilepsy, though freak cures happen under all kinds of measures. Tincture of simulo seems to have seme value in doses of 3i ter in die upward. Growing children must, as a rule, be taken from school. This is unfortunate, but it seems indisputably necessary. The education, however, should be allowed to proceed and regular fixed short hours of study be given. The child should be al- lowed to live as nearly a normal life as possible. If old enough, some can be taught a trade or some handicraft with advantage. The grown person should be urged to adopt or continue in some occupation, of which an out-door one is preferable, but any occupation is preferable to idleness.
The most difficult cases after the degenerates and those with organic brain disease are the bright, precocious children who develop petit mal alone and who have daily attacks. They are usually not amenable to bromide medication in any form. After a thorough trial of this, however, even to the point of bromidation, and using every possible tonic and eliminant, the patient is best left to hygienic measures alone.
This treatment of epilepsy always presupposes a preliminary elim- ination of all reflex* causes. If the epilepsy is local (i. e., Jacksonian) or even if it began as a local affair, it is advisable to operate, and if any source of irritation is seen in the cortex it should be removed. Occasionally this does good, but not always. Often epileptics are brought to us with scars on the scalp and a history of some old in- jury, perhaps even of fracture. If the epilepsy is general,, however, and does not seem to have started from the injured zone, operatic ns are of no use.
It goes without saying that there are many epileptics who are too advanced in the disease or too degenerate for treatment and who need only custodial measures.
CHAPTER XXII.
HYSTERIA.
Hysteria in its broadest sense is not a disease, but a morbid ten- dency in the individual by reason of which the mind reacts in an un- usual way to excitations from within and without. As seen clinically, this reaction is shown in an excess of response to ordinary stimuli, in an unusual suggestibility, and in a tendency for subconscious states to control morbid bodily functions. Hysteria has been briefly defined as a condition in which the bodily symptoms are due to a morbid state of the mind. Hysteria in this sense is a widely prevalent quality of the human character. It displays itself in brief and often trivial episodes, such as unjustifiable emotional explosions of crying and laugh- ing and motor and visceral crises. These are often to be considered only as symptoms of a natural instability, and they are often associated with other real morbid conditions. When this reaction is very severe, per- sistent and disorganizing we, have Hysteria major, which is distinctly a disease having a definite origin, course and symptoms. A slight degree of this hysterical reaction is very common and can hardly be called abnormal. Nearly all women are said to be somewhat hysterical. When the condition occurs occasionally and under stress of emotion, it is episodical or minor hysteria. When the tendency to morbid reaction and control by subsconscious states is more or less prolonged and permanent, it is major hysteria.
Etiology. — Of the predisposing causes of hysteria in general heredity is the most important. In about seventy-five per cent, there is a history of hysteria or some neurosis or psychosis in the parents. The disease is transmitted more often by the mother. Heredity is particularly apt to be important in the hysteria of children ; it is a much smaller factor in hysteria of adult males. A hereditary history of rheumatism, gout, and tuberculosis is of very doubtful im- portance. Hysteria is a disease of early adult life, most cases occur- ring between the ages of from fifteen to twenty-five in females; it occurs later in males. Hysteria attacks children between the ages of eight and fifteen, chiefly between eleven and fourteen. The trouble affects women more than men in the proportion of four to one, varying much with race, climate and occupation. Hysteria major, however, occurs oftener in men than women ; it occurs in adult
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and middle life and there is often no hereditary history. These statements are based on personal experience of this type of hysteria as seen in this country. They do not correspond with the observations of European observers or of alienists, but are certainly true for the type of disease which I am describing. Hysteria occurs in all classes of life, but rather less frequently in the middle classes than among the poor and the very rich. Male hysteria is more frequent in the poorer classes who are subjected to the influences of alco- holism, poverty, injuries, etc. Hysteria is certainly much less frequent in its severer forms in this country than in some parts of Europe, particularly France. In my experience it is much less frequent than epilepsy in the northern and eastern parts of this country. It occurs, however, quite frequently in the negroes and also in the Eatin races of this country. Bad methods of education and bad family training undoubtedly tend to promote the development of the disease.
The most important single exciting factor is powerful emotion, particularly fear. Other emotions of an allied character — excitement, sorrow, anxiety — may bring on attacks. The disease can be developed by imitation. Injuries combined usually with mental shock are fruit- ful causes of producing hysteria. The infectious fevers, syphilis, diffuse hemorrhages, the poisons — lead, mercury, and tobacco — the adminis- tration of ether, mental and bodily and sexual excesses, are all im- portant agents in developing the disease. Acute alcoholism is not very rarely followed by hysteria.
i. Hysteria minor or the hysterical temperament is characterized by an interparoxysmal condition of emotional weakness, nervousness, hyperesthesia and pains, and by crises of an emotional, motor, sensory or visceral type. In hysteria minor there are no permanent objective marks like anaesthesia and paralysis, and rarely convulsive seizures. The patient, who is almost always a girl or young woman, gradually develops an undue sensitiveness, the mind is depressed, and she gets easily alarmed. She has feelings of nervousness and lacks control over the emotions, she laughs and cries very easily and yields to every impulse. She suffers from headaches, which are usually vertical and often severe and chronic, and from spinal pains. She sleeps, as a rule, rather badly and often has disagreeable dreams. She has, under any little excitement, sensations of tickling, fullness or choking in the throat, forming the condition known as globus. Ex- citement also brings on attacks of trembling or chilly feelings which come and go. There is more rarely a considerable amount of vaso- motor instability, as shown by flushings and by coldness of the extremities.
54Q
DISEASES OF THE NERVOUS SYSTEM.
She has with more or less frequency distinct crises of an emotional character, during which she laughs or cries without apparent cause, or at least to an extent beyond her control. She may have attacks of vomiting or headache, or of intense mental excitement amounting al- most to delirium. In some cases the patient has somnambulic attacks at night, or she may have under a little excitement attacks of cere- bral automatism during which she involuntarily does things that she is entirely unconscious of when she comes out of the attack. The crises are followed by a copious discharge of very light urine. Hysteria minor is often associated with a condition of neurasthenia or of simple nervousness. Among the asthenic symptoms, are attacks of great gen- eral muscular weakness, or myasthenia. Even in hysteria minor there is a degree of that peculiar mental condition which will be described later and which is known as suggestibility. Hysteria minor is a disease which belongs especially to childhood and early womanhood. It is very apt to become ameliorated and disappear a little later in life or under the influence of proper treatment, but it may continue or pass into the major form or into some degenerative psychosis., of which a common type is that of the querulents. This consists of a not small group of persons, generally women, who have continual and unreason- able grievances, who instinctively oppose all things suggested by their friends, whose attitude is always one of being misunderstood, who are practically very selfish, and who fly into bursts of unreasoning rage or grief over trifles. In the domestic life they are a source of great trouble and unhappiness. They are generally called hysterical, but the trouble is perhaps an abortive paranoia of querulent type.
2. Hysteria major is characterized by interparoxysmal manifesta- tions of anaesthesia, paralyses, contractures, tremors, peculiar mental conditions, and by paroxysms of an emotional, convulsive, or other serious nature. Hysteria major is what is usually meant when one speaks of hysteria as a disease ; it includes also hystero-epilepsy. The onset may be gradual, but not infrequently it follows some shock, the first symptom being a convulsion, a paralysis, or some emotional outburst.
The symptoms of the crises are the most striking and will be de- scribed first. The most common of the paroxysms of hysteria are emotional outbursts of crying or laughing; after this come motor dis- turbances in the shape of convulsions of various types or of hemi- plegia or other form of paralysis. Besides this we have attacks of severe- pain, forming neuralgic crises; attacks of nausea, gastralgia and vomiting, forming gastric crises; much more rarely there are pro- longed attacks of hysterical coughing, hiccoughing, sneezing, or rapid
HYSTERIA.
541
breathing. The hysterical seizure may also take the form of attacks of trance and lethargy, catalepsy, amnesia, and cerebral automatism.
The emotional crises are characterized by appearing without any good cause; the patient laughs without reason, and the laughing con- tinues and is quite beyond her power of control. In the same way, and rather more frequently, crying attacks or attacks of furious anger and excitement come on. Associated with these outbursts there is almost always a peculiar sensation of something in the throat. It is described sometimes as being a ball or pressure or a squeezing sensation. It is called hysterical globus, and is due usually to a paresthesia of the nerves of the throat and larynx, but occasionally there is also a muscular spasm of those parts. Following the crises there is a profuse discharge of pale, limpid urine.
Hysterical convulsions have two rather well-defined types. One of them is that which comes on also in hysteria minor and is the ordi- nary form of hysterical convulsions; the other is a much more severe disturbance in every way and is known as a hystero-epileptic or hyster- oid attack. In the hysterical convulsion the patient, under the in- fluence of some excitement, injury, or acute gastric disturbance, rather suddenly falls down and begins to go through various irregular move- ments of the body, such as thrashing with the arms, kicking with the legs, throwing the head from side to side, rolling about on the bed or floor. In the more distinctively convulsive seizure the hands and arms and fingers are flexed, the legs and feet are extended, the eyes are generally closed, the eye-balls often converged or moved about irregu- larly, the pupils dilated. There is some lessening of sensation over the body and of the conjunctivae. The patient often utters noises or screams at intervals. She may bite her lips, but does not bite the tongue, nor does she ever hurt herself in her various contortions. The attack may last for half an hour to several hours, unless some measures are taken to break it up. In other forms of hysterical convulsion there is simply a general shaking or trepidation of the body as though the patient had a chill ; in other cases again the main type of movement is that of opisthotonos, the patient rising up upon the head and heels and arching the body as in tetanus. Again the attack may consist simply of a little rigidity of the body or of a series of rhythmical movements of the head or trunk or limbs, the patient sitting up and oscillating the head or swaying the trunk or moving the arms, uttering at the same time incoherent words. In still other cases the patient simply falls down and lies unconscious like a person sleeping for a few minutes or even an hour. In children the attacks may be associated with peculiar noises and movements in imitation of animals, such as the growling of a
542
DISEASES OF THE NERVOUS SYSTEM.
dog or the mewing of a cat. This condition is called therio-mimicry. In some instances the attack may be accompanied by or may end in a condition of mental excitement approaching delirium. The patients while suffering from these seizures generally appreciate what is going on about them, and will often respond to some stern order for them to cease or will be brought to a state of quietude by pressure upon some part of the body which provokes pain. In women in particular, press- ure over the ovaries or epigastrium will abort the attack ; the applica- tion of cold water or an emetic will do the same.
After a hysterical crisis or sudden shock, the patient may be found to have a paralysis of arms or legs or one side of the body.
The Symptoms of the Inter paroxysmal State. — -Between the crises of major hysteria the patient may be in a fair condition of general health, but usually presents certain definite chronic manifestations of the disease. The most characteristic are sensory symptoms, paralyses, and contractures.
Sensory symptoms. These consist of cutaneous and mucous hyperaesthesia and anaesthesia and anaesthetic disturbances of the special senses. Cutaneous anaesthesia occurs in three forms: the common form is that of hemianaesthesia involving one-half of the body; next in frequency is the segmental anaesthesia involving an arm or a leg or part of the face or head ; rarest of all the forms is a dissemi- nated anaesthesia occurring in the form of patches. These various modes of distribution are shown in the accompanying figures. The anaesthesia is a pain anaesthesia chiefly. The tactile and thermic sensations are less markedly affected. The anaesthesia is in some rare cases transferable by means of magnets or electrical irritants or by suggestion. The anaesthesia can also be lessened or removed tem- porarily by the application of magnets or coins or pieces of metal. For example, if a silver coin is fastened upon the anaesthetic area, in the course of a few minutes or a few hours there will be a zone of nor- mal sensation under and around the coin. Sometimes the temperature of the skin upon the anaesthetic part is lowered 30 or 40 F., and upon pricking the skin blood does not flow. The anaesthesia is oftener upon the left side in the proportion of three to one. Hysterical an- aesthesias are not accompanied by subjective sensations as are organic anaesthesias. The skin reflex is usually abolished. Anaesthesia of some kind occurs in a very large proportion of the serious forms of hysteria major. They are rare, however, in children, and are rarer in women than in men, in the author's experience. Anaesthesia of the mucous membranes is present chiefly in hemianaesthesia; it then involves the mucous membrane of the mouth and throat, and to a less extent that
HYSTERIA.
543
of the nose and glottis. Hemianesthesia is usually accompanied by some hemiplegia and often by some tremor. Segmental anaesthesia is also often accompanied by some degree of paralysis of the part.
Visual anaesthesias. One of the most common of the permanent stigmata of hysteria is an anaesthetic condition of the retina. The result of this is the production of a concentric limitation of the visual field and a disturbance in the color sense. Complete loss of this sense may take place or there may be a variation in the ways in which the colors are perceived. There may be also a distinct diminution in the acuity of vision or even a complete loss of sight of one eye or both eyes. This hysterical amblyopia usually comes on suddenly and may last
Fig. 230. — Hysterical loss of color sense and limitation of visual field. Color sense absent in left eye and field contracted; in right eye field less contracted; order of appre- ciation of colors from without in was yellow, violet, blue, red, green (observation by Dr. E. S. Peck).
for weeks. The visual disturbance is most common with hemianaes- thesia. It is more marked on the affected side, but exists to some ex- tent on the healthy side. The limitation of the fields is shown in the accompanying cut (Fig. 230).
Hearing. There is sometimes a diminution in the acuity of hearing of one ear, and this occurs, if present, in connection with hemianaes- thesia. There may also be a loss of hearing to high and low notes, while hearing is apparently fairly good to notes of medium range; and finally there may be a diminution in hearing by bone conduction, while hearing by aerial conduction is but little impaired, this being due probably to an anaesthesia of the acoustic nerve.
Disturbances of taste in the form of anaesthesia or paraesthesia are quite frequent and are important signs in hysteria. The loss of the
544
DISEASES OF THE NERVOUS SYSTEM.
sense of taste may involve only the back portion of the tongue and the palate ; in other words, the taste field is here limited as it is with hearing and vision.
The sense of smell may be abolished, but this usually occurs in con- nection with hemianesthesia.
Hyperesthesias and neuralgias. Pure neuralgias are somewhat rare in true hysteria, but hyperesthesia and pains of various kinds are not at all infrequent. Hyperesthesia occurs in the form of patches at different parts of the body. These sensitive points may, when pressed upon, bring on paroxysms of various kinds, and they are therefore called the hysterogenic zones (Fig. 231). The most common seat of
Fig. 231. — The three types of distribution of anaesthesia in hysteria; hemianassthesia segmental and disseminated. Z, hysterogenic zones.
these zones in women is over the ovaries; in men, in regions corre- sponding to the ovaries and on the scrotum. Hysterogenic zones, however, may be found just beneath the mammary gland, on the epigastrium, along the spine, and in other places. These zones are sensitive areas; they can be made to disappear by applications of electricity and by refrigeration and counter-irritation. Hysterical patients often suffer from local headaches, which are apt to be con- fined to the top of the head or to the sides near the temples. The pain is severe, sharp, and boring, and may exacerbate with such intensity as to produce symptoms almost resembling meningitis. The spot-like pains are known as hysterical clavus. Hysterical patients occasionally have migraine, facial neuralgia, and intercostal neuralgia. Much
HYSTERIA.
545
more often they have pains along the spine, producing symptoms of spinal irritation. Hysterical patients also have at times attacks of palpitation and pains over the heart, constituting what is known as pseudo-angina. Such troubles are much more frequent in women.
Motor symptoms. The motor symptoms of hysteria are paraly- ses, myasthenia, contractures, tremor, and choreic and ataxic move- ments. The paralyses of hysteria take the form of hemiplegia, para- plegia, and monoplegias. Hysterical hemiplegia occurs usually rather suddenly, often as the result of some severe shock. The left side is more frequently attacked. The arm is most affected, the leg next, while the face is hardly ever involved. The paralysis is not an abso- lute one, and the patient is able to drag himself along. The deep re- flexes are usually not exaggerated and they may be for a short time absent. The paralysis is thus a flaccid one. The gait of the patient is different from that of hemiplegia due to organic disease ; in hysterical hemiplegia the patient drags the paralyzed leg after him, in organic hemiplegia the patient swings the paralyzed leg around in a half-circle. This peculiarity of the gait, the absence of exaggerated reflexes, the absence of paralysis of the face, and the presence very commonly of other hysterical stigmata are sufficient to enable one to make the diagnosis. Sometimes the face on the affected side is slightly drawn by a spasm, so that it appears to be paralyzed when it really is not (Charcot). Monoplegias affect the arm or leg, very rarely indeed the face, occasionally the eye muscles, and most commonly of all the muscles of the larynx. Hysterical monoplegia is usually accompanied by anaesthesia of the affected part and by other symptoms of hysteria. There are no serious atrophic changes or disturbances of the electrical reactions. Hysterical eye palsies show themselves in the form often- est of an insufficiency of the internal recti. In hysterical palsy of the larynx the abductors are involved so that the patient cannot speak loud, and the condition is called hysterical aphonia. The trouble often comes on suddenly, the patient finding that he cannot speak above a whisper. The paralysis is not so great but that the ad- ductors can be approximated in coughing. The trouble is distin- guished from laryngeal inflammation by inspection of the affected part. The abductors of the larynx and the tongue and other muscles of articulation are in very rare cases also involved, and hysteria may produce symptoms resembling a bulbar paralysis. Paraplegia is a rather uncommon form of hysterical palsy ; it is usually brought on by emotions of depressing character, often associated with some slight injury. It may be accompanied by a good deal of pain in the back, and the form of disease which is popularly known as "spinal concus- 35
546
DISEASES OF THE NERVOUS SYSTEM.
sion" consists in many cases of hysterical paraplegia combined with hysterical neuralgia of the spine. In hysterical paraplegia there is very little wasting of the limbs and no change in the electrical reactions. The deep reflexes may be somewhat increased or normal ; they are never absent. There is never any prolonged or persistent ankle clonus, but there may be a short or spurious clonus due to a general exaggerated irritability of the nervous system. The sphincters are never involved except temporarily or through some complication.
Myasthenia is a frequent, peculiar, and interesting symptom oc- curring in the interparoxysmal stage of hysteria. It consists in a more or less temporary feeling of weakness of an arm or of the legs or whole
Fig. 232. — Hysterical contracture of hand.
body. Thus a person in lifting a dish from the table suddenly feels the arm give out, and if not careful the dish is dropped; or while walking the patients suddenly feel as though they had lost all power in the lower limbs. This myasthenic condition is generally temporary, but it may be so permanent as to produce a certain degree of monoplegia or paraplegia. The myasthenic condition generally precedes a paraly- sis. It presents no objective signs in the way of electrical reaction ; it involves a whole member, not a single group of muscles; it affects more the anaesthetic side, and when it exists the deep reflexes are usually exaggerated (Tourette).
Contractures. In some forms of hysteria there is a tendency for the muscles to undergo contracture under slight mechanical stimula- tion, such as pressure or a blow. This tendency to contracture in hysteria is called the contractural diathesis, and it is an important sign.
HYSTERIA.
547
The contractures may be temporary, disappearing soon alter the excit ing cause ceases, or they may develop independently and last for a long time. They do not always disappear in sleep, as was the case in the patient shown in Fig. 232, who subsequently recovered. They in- volve the legs, arms, and facial muscles, and may be associated with
Fig. 233. — Hysterical contracture of toe.
paralysis and anaesthesia (Figs. 232, 233). The contracture is usually a spasmodic, not a paralytic one.
Tremor occurs in hysteria in a considerable proportion of cases, more especially those in which there are hemiplegia and hemianaes- thesia. Hysterical tremor simulates all the various types. The com- mon form is one in which the oscillations occur from five and a half to seven and a half times a second, and it is therefore a tremor of slow
Repose. Movement. Fig. 234. — Illustrating intentional tremor in hysteria. (Tourette.)
rhythm. It ceases for a time when the person is quiet or lies in the horizontal position; also during sleep. It affects the head and tongue as well as the extremities, the latter more upon one side than the other. It may be chiefly in the lower limbs. Sometimes it has the type of an intentional tremor, ceasing on rest of the hand and increas- i ng when the hand is moved, as in raising a glass to the lips (Fig. 234). It then resembles exactly the tremo r of multiple sclerosis. There may
548
DISEASES OF THE NERVOUS SYSTEM.
be a slow tremor of four to five and a half oscillations per second ; this persists during rest and is but little modified by voluntary movements. It imitates the tremor of paralysis agitans. Finally, there may be a rapid tremor of eight to nine oscillations per second. This also per- sists during repose and is but little modified by movements. It imi- tates the tremor of Basedow's disease, alcoholism, and neurasthenia.
The mental state of the hysterical in general is one characterized by emotional instability and intense craving for sympathy, weakness of the will, lack of self-control, acuteness of perception, and a constant variability of moods. The hysterical mind is not a dull one, it is not a consciously mendacious or evil one. But there are an abnormal increase of sensibility and an exaggeration of the personal feeling or egotism which leads to selfishness and prompts deceit. The hysterical brain is circumscribed in its associative functions; the field of con- sciousness is limited just as is the field of \dsion. The mental activity is confined to personal feelings, which are not regulated by connotation of past experiences; hence they flow over too easily into emotional outbursts or motor paroxysms. The hysterical person does not think. Psychologists explain this further by assuming that in this condition subconscious states work upon the individual and cause the abnormal condition.
Physiologically, the condition is explained by supposing that there is a benumbing of the association fibres which normally connect sen- sory cortical centres with other parts and enable one to compare and adjust new experiences with old — in other words, to reason and form correct judgments. The mental life is mainly in the sensory-motor centres rather than in the "association centres" of Flechsig. It is a return to the unripe brain of childhood through a breaking off of associating paths.
By reason of this narrowed mental life there develops a condition of suggestibility, so that ideas and impressions easily become fixed and dominate the mind. The person becomes self-hypnotized and believes he or she is suffering from things which have no objective existence. The pains, palsies, and anaesthesias of hysteria major are pseudo-delusions, differing from delusions of the insane in that the false belief or idea is a subconscious one.
Trophic disorders. In hysterical paralysis a slight amount of atrophy occurs, but only such as would naturally follow disuse of the part. A very few cases have been reported in which a true neurotic atrophy resembling the atrophy that occurs in neuritis or progressive muscular atrophy was present, and it is even affirmed that degenera- tive reactions may be elicited. Cutaneous eruptions and dystrophies
HYSTERIA.
549
practically do not exist or, if present, are the result of complicating disorders.
Visceral symptoms. Hysterical patients often suffer from dys- pepsia and constipation, also from anorexia and in some cases from persistent vomiting or regurgitation of food. Occasionally the anor- exia and vomiting become persistent; the patient refuses food or re- jects all that is taken; she emaciates, becomes weak and bedridden, and develops into that particular phase of hysteria known as anorexia nervosa. In these cases, along with the aversion to food and vomiting, there may be a great deal of gastralgia. The urine in hysteria is apt to be of low specific gravity. Sometimes there is retention of urine; in extremely rare cases there is a condition known as ischuria and anuria, in which for several days extremely small quantities of urine are passed, owing apparently to a suspension of the functions of the kid- ney. Such cases should always be carefully investigated, to see that the patient does not deceive her attendants in regard to the amount of urine passed.
Vasomotor symptoms are very common. They consist of flushings and pallor, cold extremities, and at times an cedematous condition of one or more extremities. This oedema may be of the ordinary pale, waxy character, pitting upon pressure. In other cases it has a pecu- liar bluish tinge and it does not pit; the hands, which are the parts generally affected, are several degrees below the normal in tempera- ture, and the limb resembles in some respects the condition in Ray- naud's disease. Gangrene, however, never supervenes. This form of oedema is known as the blue oedema of hysteria.
There occur in hysteria febrile attacks, and much has been written upon the subject of hysterical fever. These so-called hysterical pyrex- ias may resemble in their course typhoid or malarial fever; as a rule, the temperature runs a very irregular course, and the fever often lasts for weeks or even months. The essentially neurotic origin of these fevers has hardly yet been established, and one can reach the diagnosis only by most carefully excluding all other possible causes.
Anaemia is a very common condition in hysterical patients.
There occur in psychasthenic persons (who are often called hysterical) slight seizures, such as feelings of fainting, rushes of blood to the head, perhaps a temporary blindness, palpitation of the heart and sudden extreme emotion of fear. These attacks often suggest the aura of epilepsy. They are called psycholeptic or paraleptic seizures.
Hystero-epilepsy. — The form of hysteria which shows itself by the
55°
DISEASES OF THE NERVOUS SYSTEM.
development of severe crises known as hystero-epileptic attacks is extremely rare in this country, at least in its typical phase. It has been particularly studied by the French writers Charcot, Richer, and others. Hystero-epilepsy, as this form of the disease is called, is a true hysteria and not epilepsy at all, nor a mixture of hysteria and epilepsy, though the name would suggest that that was the case. The typical attacks of hystero-epilepsy begin with certain prodromata consisting of a feeling of malaise and irritability which may last for several hours or a day. The attack is ushered in often with an aura, the patient utters a cry, falls to the ground, loses consciousness, and enters into the first phase, known as the epileptoid stage. During this she suffers from tonic and clonic spasms very much like those of true epilepsy, The muscles finally relax, and the patient becomes coma- tose for a moment and then enters the second stage, that of the con- tortions and grand movements. In this there is opisthotonos, the body is arched up, and there are violent movements of the trunk and limbs, which undergo flexion and extension, the movements being all of large range. The next stage is that of emotional attitudes, during which the patient seems to be experiencing intense feelings of anger, joy, or some other violent passion, which she expresses by the postures of her body, the movements of the eyes and facial muscles. This stage over, she enters into the last phase, which is known as that of delirium, during which there is a great deal of mental excitement of a depressing character, from which she gradually emerges into her normal condition. To recapitulate: we have in a typical attack, first, pro- dromata; second, the epileptoid phase, lasting from one to three minutes; third, the phase of contortions and grand movements, one to three minutes; fourth, the emotional phase, lasting from five to fifteen minutes; and, finally, the stage of delirium, lasting a variable time; the whole attack lasting from five to twenty minutes (Fig. 235). In this country we occasionally see hysterical patients exhibiting one or two of these phases, but very rarely indeed do they ever go through the whole series. The patients who suffer from hystero-epileptic attacks generally during the interparoxysmal stage present many of the stigmata of hysteria, such as paralyses, contractures, and anaesthesias.
Hysterical crises which take the form of convulsions or emotional seizures sometimes end or are associated with attacks of catalepsy or trance or attacks of amnesia and cerebral automatism. As these con- ditions all occur in other diseases than hysteria, they will be described elsewhere in connection with the subject of the disorders of sleep and of consciousness.
HYSTERIA.
551
Hysterical persons occasionally are attacked with violent and per- sistent hiccoughing or sneezing. Sometimes also there come on at- tacks of extremely rapid breathing or hysterical polypnoea, during which the respirations run up to fifty or seventy a minute. A hysteri- cal cough sometimes occurs; it lasts for a long time. (Esophageal spasm with consequent dysphagia is another one of the somewhat rare phenomena of hysteria. Indeed, all the symptoms refered to in this
VUUllUI(Lll\\\HIIUUll(lllli
1 Epil epteid
£ Grand Movements
3 Passional
4 Delirious
Fig. 235. — Showing the different stages of a hystero-epileptic seizure (After Richer.)
last paragraph may be grouped as tics or morbid mental habits, and not truly hysterical.
Pathology. — There is no known anatomical change at the basis of hysteria. We do not find the marks of degeneration as we do in cer- tain forms of insanity and epilepsy.
Diagnosis. — -The essential characteristics are the peculiar emo- tional condition of the patient, the past history of hysterical crises, the presence of the stigmata of hysteria, such as anaesthesias, limitation of the visual field, paralyses, and contractures. The variability of the
552
DISEASES OF THE NERVOUS SYSTEM.
symptoms, their susceptibility to influence under suggestion and rigorous moral measures, the absence of organic disease or of serious disturbance of nutrition, the sex and age, and the cause should also have weight in guiding us to our decision.
The test of hysteria minor is simply the presence of a morbidly easy reaction of the mind upon the body. It has no absolute criteria. What is morbid must be determined by the judgment of the physician con- sidering the education and environment. Hysterical crises and tem- porary hysterical states can be brought about by stimulants and drugs. Hysteria major is a rarer disease and has a clear-cut symptomatology. There are crises and the stigmata already described.
Hysteria and Psychasthenia (Phrenasthenia) . — If a person has an obsession or fixed idea which consciously affects his mind and actions, but not directly his body or functions, it is psychasthenia, or an obsessive psychoneurosis. The obsession in hysteria is usually a sub- conscious one ; in psychasthenia it is conscious. Hysterical patients have repeated attacks of vomiting, of pains, neuralgias, of spasms and paralyses. These are due to an abnormal state of subconsciousness acting on the body. The patient cannot use the arm if he has hysteri- cal monoplegia, because the compelling paralytic delusion is quite beyond his conscious control. It is so with most of the hysterical symptoms. But in the obsessive psychosis the patient has a morbid fear which prevents his going into the street or riding in the subway or looking people in the face. He realizes the nature and foolishness of the limitation, and struggles against it and often successfully. But the hvsterical hemiplegic does not know how to struggle. He cannot make the effort. Many so-called hystericals are thus really obsessives. They need a different treatment.
Diagnosis of special symptoms of hysterical manifestations. Hys- terical paralysis is characterized by the fact that there is no marked degree of wasting of the muscles, no electrical reactions of degeneration, the deep reflexes are preserved or exaggerated, and other marks of hysteria are present. Hysterical anaesthesia can generally be lessened over certain areas by the application of the magnet or can be made temporarily to disappear; it is peculiarly distributed in the way de- scribed under symptoms and is associated with anaesthesias of the special senses. Hysterical contractures sometimes cease during sleep and always under deep narcosis, and the use of an anaesthetic may clear up the case. They usually follow a fit, an injury, or an operation. They are somewhat increased on attempts to overcome them by force ; they are usually associated with paralysis and anaesthesia and other hysterical symptoms.
HYSTERIA.
553
Hysterical convulsions. These differ from convulsions of epilepsy in the way best indicated by the following table:
Hysterical Convulsion. Epileptic Convulsion.
Brought on by the emotion or injury; rarely The opposite in all these particulars, any aura; no initial cry; movements co- ordinate; tongue not bitten, and patient never injures herself. Duration perhaps several hours with intermissions; conscious- ness generally preserved. Micturition and defecation do not occur. No rise of temper- ature; may be stopped artificially. Rarely occurs in sleep.
The hystero-epileptic attacks are so characteristic that a mistake could not be made.
Prognosis. — -The prognosis of hysteria in children is good. They generally get well, though in some cases there is a recurrence later in life. Minor hysteria under proper treatment usually gets well or at least is of little importance. The major forms when chronic are often intractable even under the best treatment. When a severe form of hysteria occurs in a person of feeble frame who is surrounded with a sympathetic family, the task of rescuing her from her disorder is a very arduous one. Traumatic forms of hysteria which are not infrequently associated with some actual physical injury are sometimes difficult to cure. Hysteria which is associated with some organic disease, such as a severe pelvic disorder or an organic affection of the central nervous system, has a bad prognosis. Hysteria in the male is generally curable, but it requires vigorous treatment, and spontaneous cure is by no means likely to happen. Major hysteria has a vastly better prognosis if proper treatment for it is instituted early.
Treatment. — The treatment of hysteria may be divided into the mental, mechanical, dietetic, and medicinal.
By all odds, the most important factor in the treatment of hysteria is the mental treatment, and the most important measure to be taken is the isolation of the patient. She should be placed where she will not be surrounded by sympathetic friends; where her life will be a regular one; where some occupation may be given which will engross her attention, interest her mind, and call into play her physical activi- ties. In the major forms of hysteria associated with anorexia, emacia- tion, anaemia, and possibly pelvic disorders, the "rest cure" as elabo- rated and carried out by Weir Mitchell forms by all odds the most suc- cessful means of treatment. In many cases of less severe character a partial rest cure in which the patient is separated from her family
554
DISEASES OF THE NERVOUS SYSTEM.
but is not placed under such severe restrictions may be all that is needed. In the case of children removal from home is often advisable, and the discipline of well-conducted schools is a most excellent measure.
The mechanical means used in hysteria are hydrotherapy, elec- tricity, massage, and exercise. Of these measures hydrotherapy and electricity take the first rank. In hydrotherapy the douche or jet to the back, the shower and cold plunge, and the half-bath are the most efficacious. The technic of their use is given elsewhere. In the electrical treatment the static and faradic currents give the best results. The static sparks often relieve contractures and lessen or remove the anaesthesias, and both forms of electricity seem to have a generally beneficial tonic effect. Massage is of some value in pro- moting nutrition and it also has a favorable sedative effect on many cases. Exercise, particularly of an active kind, such as stimulates the mind and interests one, is a measure of extreme value and one which has perhaps not been sufficiently appreciated. The use of the bicycle, playing tennis, and horseback riding are measures which cannot be too strongly recommended to hysterical women; in fact, it is probable that some cases which are submitted to the rest-cure treatment might do better by an entirely opposite kind of procedure.
The drugs which can be recommended in hysteria are not nu- merous and their power is limited. Valerianate of zinc, turpentine, asafcetida, tincture of sumbul, iron, and the bromides are the most important of the nervines. In hysterical children a capsule containing two grains of valerianate of zinc and one of sulphate of quinine is often efficacious. Gowers places more reliance upon the oil of turpentine in doses which should be increased to the point of strangury.
In the treatment of hysterical convulsions the most efficient meas- ure is the administration of an emetic, and this can be best done by giving hypodermically one-twelfth or more of a grain of apomor- phine. Convulsions can be stopped sometimes by throwing water in the face or on the epigastrium; by firm and somewhat long-continued pressure over the ovaries; by the administration of valerian, aromatic spirits of ammonia, or compound spirits of ether.
CHAPTER XXIII.
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS (CHOREA— THE SPASMODIC TICS).
MYOCLONIA, TWITCHING SPASM.
Myoclonia is, or should be, a general name used for twitching spasms of all kinds. Strictly speaking, it includes chorea, the convul- sive tics, paramyoclonus multiplex, certain hysterical spasms, myo- clonus epilepsy; also myokimia or fibrillary and wave-like twitchings, and rhythmic myoclonia.
Myotonia, or tonic spasm is a general name which should in- clude such diseases as tetanus, tetany, the symptomatic tonic spasms of organic disease and hysteria, myotonia congenita or Thomson's disease and myotonia acquisita.
It is better to use these terms for symptoms and not to indicate a special disease. Some of the convulsive tics, however, are sometimes referred to as degenerative myoclonias.
Myoclonia is a general term meaning a disease characterized by muscular twitching. Myoclonus is a special term indicating a twitch- ing muscle. The same verbal distinctions apply to myotonia and myo- tonias (Foster's Dictionary).
The myoclonias have been much misnamed, and there is great confusion in the nomenclature. There are five more or less distinct clinical types, but these can be with sufficient accuracy reduced to three :
1. The infectious myoclonia, which is only the chorea of Sydenham.
2. Hysterical and degenerative myoclonia which includes practi- cally all the so-called spasmodic tics, hereditary chorea and even the association disease called myoclonus epilepsy.
3. The myokimia disease, which includes simple, general, fibrillary and wave-like twitchings, and the so-called myoclonus multiplex of Friedreich.
Myoclonia of the spasmodic tic types is often associated with rhythmical and even tonic spasms.
CHOREA OF SYDENHAM (ST. VITUS' S DANCE).
Infectious Myoclonia. This is the common type of chorea, and is the disease ordinarily meant when the term chorea is used. It is a subacute disorder charac-
555
556
DISEASES OF THE NERVOUS SYSTEM.
terized by irregular jerking and inco-ordinate movements. The disease is a common one, forming about one-fifth of the nervous diseases of children.
Etiology. — -Most cases occur between the ages of five and fifteen It is very rare under five. A few cases occur after twenty, and even up to old age, when a senile chorea is sometimes observed. It affects girls more than boys in the ratio of about 2.5 to i. In adult life the disproportion is less marked.
It is relatively rare in the negro race, especially in those of pure blood (Mitchell). In this country it is more common in children of recent immigrant races, probably because of the poor social conditions.
It occurs in all climates. Most cases develop in the spring months, next in the autumn, next in winter, and last in summer. The seasonal influence varies in different localities. In Philadelphia more cases occur relatively in the spring. In New York there is an almost equal increase in the autumn. School attendance has something to do with these variations. Choreic attacks appear to be related to increase in storms (Lewis). The disease is more frequent in cities, and probably in the poorer classes. Hereditary influence is slight, but it exists. In a small percentage of cases one parent has had chorea, epilepsy, insanity, or a decided neuropathic consitution. A phthisical or a gouty history in parents is also not rare.
The chief exciting causes are injury and fright, mental worry, and some infection, generally a rheumatic or tonsilar one. Fright or some emotional disturbance is a cause in about one-fifth of the cases. Acute rheumatism is given as a cause in very varying proportions, ranging from five to twenty-five per cent. In this country it ranges from fifteen to twenty per cent. (Sinkler, Starr, Sachs, and personal observations). Some authors lay much more stress on rheumatism and, counting attacks of tonsillitis as evidence of rheumatism, they find it a large causal factor. Endocarditis is developed in the course of chorea in about one-fourth of the cases. This may exist without any other manifestations of rheumatism. Pregnancy is a cause of chorea, generally in primiparae and always in young women under twenty-five. Chorea sometimes follows infectious fevers, especially measles, scarlatina and whooping-cough. Chorea is the prominent symptom in some septicemic cases. Septic chorea (Sachs), and the serious mental disorder known as chorea insaniens is undoubtedly due to infection. It has been caused in rare cases by reflex irritation from an injury, from nasal disease, and from sexual disorders. Overstudy and the worry of examinations are factors in causing chorea in pre- disposed and badly nourished children. Intestinal irritations, such
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 557
as worms, may excite chorea. Malaria also may aggravate, if it does not produce it. Hamilton describes a form of chorea caused by tobacco-poisoning. Anaemia and malnutrition underlie most cases.
Symptoms. — The disease may begin suddenly, but usually it de- velops slowly, and it is not till one or two weeks that the symptoms are decidedly prominent. It usually begins with irregular twitching of the hand or face on one side. The child winks, grimaces, jerks its head or shoulder, twitches its arms and drops things from its hand. The foot and leg become affected later and the child stumbles in walking. In two or three weeks the opposite side is involved, but usually less than the one originally affected. In three or four weeks the disease reaches its height. The patient's movements are then almost continu- ous. The hands can hardly be used and the child has to be fed and dressed ; even walking is awkward and difficult. Speech is indistinct and confused from the irregular movements of the lips and tongue. The muscles of respiration may be involved so that the rhythm is uneven. It is asserted that the heart's action is affected also; but this is unlikely.
The choreic movements usually occur both when the muscles are at rest and during volitional acts. In some cases the disease is chiefly characterized by inco-ordinate movements when purposeful acts are attempted. In other cases voluntary movements can be readily performed, and the muscles twitch only when the limbs are at rest. The movements cease, as a rule, during sleep. But the child sometimes sleeps badly on account of the movements. In severe cases attacks of mental excitement and even delirium come on for several successive nights, and this may be so marked a feature as to form what is called chorea insaniens, or maniacal chorea. Apart from such phenomena, the mind in chorea is usually dulled, the temper irritable, and the child much harder to manage.
The appetite is poor and capricious, the tongue coated, and the bowels are often constipated. The nutrition fails a little; there are anaemia and a tendency to loss of flesh.
The eyes present nothing abnormal. Hypermetropia, astigma- tism, and muscular insufficiencies exist, but not much more than in other nervous children.
The child is often worse in the morning and improves toward night. Excitement and physical exertion make the movements worse. There is rarely any pain and never anaesthesia or tenderness. The muscles are weak but not actually paralyzed. The deep reflexes are somewhat lessened and the knee jerk may be abolished. The electri- cal irritability of the muscles is, as a rule, increased, but there are no
558
DISEASES OF THE NERVOUS SYSTEM.
qualitative changes. Nocturnal enuresis occasionally occurs. The urine contains an excess of urea and phosphates and at the height of the attack the specific gravity may be increased.
Forms. — Maniacal chorea, chorea insaniens, is characterized by choreic movements and great mental excitement — especially at night, delirium, with hallucinations and delusions. The patient has in effect a toxic or infectious psychosis. After two or three weeks the excitement lessens and the patient becomes dull and apathetic. Such cases usually occur in adult women, and they are often fatal.
Paralytic chorea. In this form one arm or one side of the body becomes rather weak and powerless. A few twitching movements are observed. This form occurs only in children and runs the same course as the spasmodic type.
Chorea of adult life and senile chorea. The disease when it occurs in the second half of life attacks men rather oftener than women ; it is not related to rheumatism. There is usually a neurotic family history and even a hereditary history of chorea. The attack is usually caused by emotional disturbances. It runs much the same course as juvenile chorea, but is rather more apt to become chronic. When it occurs in old men it is called senile chorea. This type is not to be confounded with hereditary or Huntington's chorea.
Duration — Relapses. — The disease in this country lasts about ten or twelve weeks, ranging, however, from six weeks to six months. There may be great improvement followed by a relapse, and in this remittent manner the disease may last for years. If it last more than six months it should be called chronic. Relapses occur in about one-third of the cases and rather oftener in girls. Relapses occur oftenest within a year of the first attack and much oftener in the spring. After three years relapses practically cease. The number of relapses is usually but one, but the disease may recur eight or nine times. Relapses rarely occur in adults except in the chorea of pregnancy.
Pathology. — Chorea has no definite anatomical basis, though the seat of the disease is in the brain. The irritant seems to come from and act first upon the blood-vessels, causing in severe cases intense hy- peremia, with dilatation of vessels, small hemorrhages, and spots of softening. There are infiltration of the perivascular spaces with round cells and swelling and proliferation of the intima of the small arteries. In chronic cases the evidence of active vascular irrita- tion is less, but there are perivascular dilatations and increase of connective tissue. The process suggests a low grade or an initial stage of inflammation. The cause of this is probably either an infec-
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 559
tive microorganism or a humoral irritation similar to that causing the rheumatic symptoms and the heart lesions. The infection, when it exists, is not due to any special or single specific organism. In a con- siderable percent, of cases (ninety per cent. — Osier) especially in those of long duration, there are fibrinous deposits on the walls of the heart. The presence of points of irritation in the cortex and its meninges and in the deeper parts excites irregular discharges of nerve force and pro- duces the choreic movements. The interruption of the voluntary nerve impulses by diseased foci makes these movements irregular. In paralytic chorea the pyramidal tract is probably more seriously in- jured by some single large focus of congestion, exudation, or hemor- rhage. Indeed, I have seen a true hemiplegia develop in the midst of an attack. In maniacal chorea the meninges and cortex are more involved.
Diagnosis. — The disease is easily recognized by the peculiar twitching movements. It is necessary only to distinguish the dif- ferent forms. It must be distinguished from convulsive tic, electric chorea of Dubini, hysterial spasms which include saltatory chorea and chorea major. The distinctions are not difficult and are given in connection with the descriptions of these disorders.
Prognosis. — As regards life, the prognosis is very favorable. In this country death from chorea hardly ever occurs in children. It is more fatal in adults. In England the mortality from chorea is about two per cent. Nearly all non-fatal cases eventually get well.
Treatment. — The most important single factor in treatment is rest. The child should not be allowed to take violent exercise or to have any exc tement. In most cases he should be taken from school, and in bad cases he should be kept in bed.
Cold sponging or the ether spray daily along the back is useful. Nourishing food and iron are indicated.
As specific remedies, arsenic still heads the list. It should be given in doses of niv. of Fowler's solution t.i.d., increased by one or two drops daily to fifteen or twenty drops or even more. If this causes nausea and gastric pain or headache, the dose should be stopped for a day and then resumed, if possible, where it was left off. Next to arsenic come antipyrin (gr. v.), antifebrin (gr. iij.), exalgin (gr. iij.), the doses to be carefully increased if needed. Tincture of cimicifuga sometimes helps when arsenic fails. The bromide or valeri- anate of zinc is also an excellent remedy, especially when there is a hysterical element. The bromides and chloral are useful adjuvants in promoting sleep. Chloral alone is said to be curative if given in doses sufficient to prolong sleep greatly (Bastian). Exalgin given cau-
5<5°
DISEASES OF THE NERVOUS SYSTEM.
tiously with iron, in doses increased to fifteen grains a day, is often very useful. Hyoscine hydrobromate in doses of gr. T^ y is occasion- ally efficacious. Among other drugs of less value are the salicylates, cypropedium, lobeline, physostigmine, and tartar emetic. In chronic and obstinate cases hypodermic injections of Fowler's solution should be tried. Galvanization of the brain and spine is also useful. Change of air sometimes breaks up an attack.
Hereditary chorea was first described by a Long Island physi- cian, Dr. Waters, in 1842, later by Drs. Gorman and Lyon, and in 1872 by Dr. Hungtington. The American cases have been observed chiefly in New York, Connecticut, New Jersey, and Pennsylvania. Cases have been reported also from Germany, France and England. The disease rarely begins before thirty or after fifty; it occurs about equally in males and females. It is always directly hereditary, either through father or mother, usually the latter. It begins without known cause by twitchings in the face; the movements then extend to the arms and legs. It is attended by progressive mental deterioration, by a tendency to melancholia, and finally ends in dementia. Its course is chronic and usually very slow, lasting ten or twenty years. Post- mortem, chronic pachymeningitis and leptomeningitis witlrdegenera- tive changes in the cells of the cortex have been found.*
Myoclonias of Psychic or Degenerative Origin.
Spasmodic Tic (Mental Tics, Habit Tics, Hysterical Tics, Local General and Endemic Tics). — Spasmodic tic is a disease to which the name of chorea is often, but incorrectly given. It is a very chronic disorder, and shows itself in the form of quick, electric-like spasms of certain groups of muscles or single muscles. The spasmodic move- ments are violent, and several rapid contractions succeed each other, after which there is a period of rest. The twitching movements differ
* Electric Chorea is a name sometimes and wrongly given to very violent -forms of ordinary chorea of Sydenham. The term was first applied by Dubini to a peculiar and progressively fatal spasmodic affection which has been observed almost solely in Italy, and which is perhaps of a podagrous or malignant malarial origin. M. Bergeron in 1880 also described an " electric chorea" in which the patients are attacked by sudden rhythmical spasms. This latter disease has a uniformly favorable course. Neither of these diseases resembles true chorea, nor do they have the character of the t'cs.
The term electric chorea, therefore, is one that should be used, if at all, only with a qualifying explanation.
Oscillatory spasms and nodding spasms have been described elsewhere.
Procursive Chorea, or Dancing Chorea. — Laycock has described as a separate kind of chorea a rhythmical or trochaic form, which he says affects children, principally girls, and shows itself in spasmodic rhythmical contractions or in sudden rotating or procursive movements of the body. This has been called chorea procursive, or chorea festinans, by other writers. In many cases it is accompanied by vertigo, when the condition of the patient is similar to that of a person who has been whirling around a number of times. Such cases always have decidedly hysterical characters, although these procursive attacks may complicate ordinary chorea.
Chorea major is a manifestation of hysteria, and has been described under that head. It is not a chorea at all.
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 561
from those of chorea in that groups of muscles physiologically related are affected and co-ordinated movements result.
Spasmodic tics occur as the result of morbid habit, as the result of a morbid psychic condition, as the result of exhaustion or degeneration of the neural mechanism.
Habit and Local Tics. — There are many persons who go through life with some trick of speech, of gesture, or some peculiar grimace. It may be only a shrug of the shoulder, a twitching of the eyes, or a sniff. These various movements are tics of the co-ordinate kind. The spasmodic motion is of itself normal, but is inappropriate and misapplied.
Such movements are often seen in children. They sometimes rep- resent abortive attacks of chorea, and sometimes they are the residuum of old attacks. In many cases they are chronic convulsive tics from the start and have little relationship to Sydenham's chorea. The condition is to be treated both by moral and medicinal measures. Closely allied to habit tics are various local tics. Thus when the spasm is localized in the facial nerve, we have a facial or mimic tic (see p. 129). The spasm may become localized even in a single branch or twig, as that to the orbicularis, the zygomaticus, the diaphragm, or the tensor tympani. Spasmodic tic sometimes involves the muscles of expiration and the larynx, and then it has been wrongly called chorea of the larynx. Stuttering is a variant of local tic, but involving higher psychic processes.
Generalized Tics. — -The convulsive movements here take a wide range and affect a number of groups of muscles, producing quick, vio- lent movements of the body. They are sometimes accompanied by explosive disturbances of speech. In these cases the patient at the time of the convulsive movement utters some obscene or profane words {coprolalia), or involuntarily repeats the last words of the sentence spoken to him (echolalia), or spasmodically imitates a gesture made to him (echokinesis) , or involuntarily exclaims the thought uppermost in his mind, perhaps revealing some secret against his will (tic de pensee).
The peculiar disorder of the Maine "jumpers," characterized by sudden violent movements on being touched or startled, is a form of tic. Tics of this endemic hysterical type are seen in the clinics of this city. The diseases known as latah, occurring in Malay, and myriachit, occurring in Siberia and Kamchatka, are endemic and probably hysterical tics.
Generalized tic with coprolalia affects children between the ages of six and sixteen years, and by preference the masculine sex. There 36
562
DISEASES OF THE NERVOUS SYSTEM.
is almost always a neurotic family history, and the children are nervous.
The disease begins with attacks of violent and irregular movements, affecting, generally the head, face and upper extremities first, then involving the whole body. The movements can be controlled for a time by the will, only to break out with increased violence later. They cease entirely during sleep, which is generally profound.
After having suffered from the disease for a time, the patient will, with the attacks, utter inarticulate cries, or he may begin to repeat or echo the words that he overhears. All this is done automatically and suddenly, with the accompaniment of grimaces and muscular con- tortions. The special peculiarity of the disease is the sudden inter- jection by the patient of obscene words and expressions (coprolalia.)
The disorder is chronic, lasting for years. It is best treated by iso- lation, tonics, and ordinary antispasmodics.
Generalized tics sometimes occur in families and are accompanied with evidences of mental and physical deterioration ; or they may be associated with epilepsy, in which case the disease is called myoclonus- epilepsy. In these patients the spasmodic symptoms are often of various types. There may be choreic and also tonic and rhythmical movements. And in all generalized tics we often see combinations of various kinds of spasm. The degenerate tics are always very chronic and usually incurable maladies. The psychic or hysterical tics are amenable, especially to pedagogical treatment, wThich should be long and patiently practiced. In hysterical tics, an idea with emotional tinge lies in the subconscious and produces the motor effects. In these tics, a reflex or central irritation often sets up the spasm. This irritation, such, for example, as eye-strain or a nasal disorder, subsides from consciousness. The irritation is removed, but the subconscious feeling of it remains and produces a response.
In degenerative tics, there is probably an exhaustion or even or- ganic change in the mechanism of control.
Fibrillary Myoclonia (Myoclonus Multiplex of Friedreich).
The exact nature of this rare disease has been misunderstood. It is probably due to some infection, but in the original case it followed a fright. It occurs mostly in adult males. In the description of the disease one cannot improve on that originally given by Friedreich. He states that he observed a most peculiar affection of a set of muscles in the upper and lower extremities. This showed itself in the form of short rapid contractions, reappearing at short intervals, which
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 563
affected the biceps and triceps of both arms, and supinator longus of the forearms exclusively.
At the same time chronic spasms showed themselves in the upper part of the thigh involving the vastus externus and internus and especially the rectus femoris, also to a less extent the abductors, and further the biceps and semitendinosus were affected, while the rest of the muscles of the upper part of the thigh were entirely intact. Here and there a muscle of the back or face was slightly affected by this disturbance, which confined itself to that particular muscle exclusively.
Here it was not merely fibrillary or fascicular twitchings, but also spasms which involved the whole of the muscle which swelled for- ward in a mass, and bulged every time, even when the contractions of the muscles were not marked enough to produce a noticeable locomotor effect on the limbs to be moved. Only at times, at an unusually marked contraction, one could notice a slight change in the part to be moved, e. g., during many contractions of the biceps a slight flexion of the forearm or during some of the spasms of the supinator longus a slight supination of the hand.
The spasms of the rectus femoris produced a short and rapid for- ward movement of the knee-joint, that of the biceps and semiten- dinosus a rapid movement of the part affected, following the tendons which limit the popliteal space.
The spasms, however, were unrhythmical throughout and vary- ing in extent, even though they were separated from each other only by short intervals. (It may be mentioned here, by the way, that by placing the stethoscope on the affected muscles in the moment of their contraction, a loud and clear sound, resembling the first heart sound in every respect, could be appreciated.)
Although the affection offered a perfect symmetry in its art, as the very same muscles were affected on both sides, yet every muscle showed a complete independence in relation to its individual contractions. Now it was this muscle, now that muscle, which twitched all for itself without any relation to the others, and if it sometimes happened that the contraction of a muscle on one side took place synchronously with another muscle of the same or other side, or even at times if two sym- metrical muscles contracted at the same time, it was only an accidental coincidence, as it may be understood that such an occurrence was made possible by the frequency with which the spasmodic contractions appeared in each single muscle.
The frequency and intensity with which the contractions of each single muscle ensued were not the same on different days or at different times of the day, and in this regard a considerable difference could be
564
DISEASES OF THE NERVOUS SYSTEM.
noticed. During the times of greater agitation there were 40 to 50 contractions per minute in one and the same muscle, while during the hours of greater rest only 10 to 20 contractions could be counted. Only rarely did it happen that the spasm ceased entirely, or almost entirely, within one-quarter or one-half hour." This patient re- covered. In a case observed by myself the muscular phenomena were due to an infection from which death resulted.
MYOTONIA.
This is the general name given to tonic muscular spasms, which may be irregular, rhythmical, localized, or very general in distribution.
The myotonia may be acquired, and it is then called myotonia acquisita, or symptomatica. Not much is known of this as a definite malady. It occurs sometimes as an epiphenomenon in progressive muscular dystrophy. It is a not uncommon symptom of muscu ar exhaustion or deficient vascular supply.
Thomson's Disease: (Myotonia Congenita).
This is a hereditary and family disease characterized by the devel- opment of tonic cramps when the patient attempts voluntary move- ments. The disorder is very rare.
Etiology. — Congenital myotony is practically always hereditary and runs in families. It affects males by preference and develops at the time of adolescence.
Symptoms. — The patient notices that on trying to rise or walk his legs are seized with a painless cramp, which in a few seconds relaxes, but comes on again when the muscular movements have been repeated. If he closes his hands tightly a cramp occurs and he cannot relax the grip. If he shuts his eyes he cannot open them for a moment. The muscles of mastication may be affected, but the extremities are the parts most involved. The involuntary muscles are spared. The cramps are increased by cold and nervousness; they are lessened by muscular exercise. The muscles are somewhat hypertrophied, and the patient may present the appearance of a very strong man. The actual strength is fair, but less than would seem. The general health may be good, but the patients sometimes show the signs of low vitality in weak digestion, feeble sexual power, and susceptibility to cold.
The electrical excitability of the nerves is normal, that of the mus- cles is increased, and there is produced a contraction tetanus by both currents. In addition, Erb describes a peculiar reaction produced by a strong stabile galvanic current. It consists in the appearance of wave-like muscular movements passing from cathode to anode. This is not always present. The mechanical excitability of the muscles is also increased.
Pathology. — The disease is probably a primary muscular dys- trophy. A view supported by its occurrence in the wasting dystro-
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 565
phies. There may be, however, a peculiar defect in innervation re- sulting from a congenital anomaly of the motor tracts. The muscular fibres are found to be hypertrophied, the striations indistinct, and the nuclei increased.
The diagnosis is easily made by the characteristic tonic cramps. The prognosis is bad as regards cure, but the disorder does not shorten life.
Fig. 236. — Myatonia congenita. (Spiller.)
Treatment. — Dr. Thomsen, who first described the disease, states that active muscular exercise benefits patients. No specific measures are known.
Symptomatic Myotonia and Paramyotonia.
Paramyotonia is the name given to a form of myotonia in which the symptoms deviate somewhat from the typical ones that appear in Thomsen's disease.
Congenital paramyotonia is a family affection, resembling in this respect Thomsen's disease. The muscular rigidity is brought on not by voluntary movements, but by exposure to cold and often
566
DISEASES OF THE NERVOUS SYSTEM.
very slight degrees of cold. The tonic spasm is a long one and lasts for from a quarter of an hour to several hours. It affects the arms more than the legs. The facial muscles are prone to become rigid. The attacks are followed by some muscular weakness. In congenital paramyotonia the trouble is undoubtedly a primary disturbance of the muscles; in other words, a myopathy.
Symptomatic paramyotonia is noted most characteristically in a certain form of paralysis agitans. Here the patient, when attempting to walk or to rise from the sitting posture, is suddenly seized with an apparent rigidity of the muscles which prevents him from stirring. The trouble is probably more central than muscular. The myotonic condition appears in the progressive muscular dystrophies, and in spastic paralyses of spinal and cerebral origin.
Ataxic paramyotonia is the name given to a disorder characterized by transient spasms like those of Thomsen's disease, associated with distinct ataxia and also with weakness and some anaesthesia (Gowers). This disease is probably located in the spinal cord and should perhaps be considered one of the forms of symptomatic paramyotonia. No special treatment can be given for either of the two latter forms of disease, of which very few examples have been observed.
Hypotonia.
A few cases have been observed in which from birth the muscles have shown a persistent and extreme hypotonia. This forms a con- dition known as congenital myatonia (Fig. 236). Minor degrees of myatonia occur in many people and are not to be regarded as morbid conditions. Hypotonia is more marked generally in the female sex, and it is developed by acrobats and freaks.
Tetanus.
Tetanus is an acute or subacute infectious disease characterized by violent and painful tonic spasms with remissions and exacerba- tions. It is due to infection by the tetanus bacillus. It is called idio- pathic when no open wound is found and traumatic when such condi- tion is present. When it attacks infants it is called tetanus neonatorum ; when the jaws alone are involved it is called lock-jaw, or trismus. A form which affects the face and throat is called head or cephalic tetanus. The idiopathic or medical tetanus is more chronic in its course.
Etiology. — It has a special predilection for newborn children in some countries (West Indies) and to a less extent for puerperal women. It affect males more than females (4 to 1). After the first month of life there is practical immunity till after the tenth year. It then increases in frequency to about thirty. Most cases occur between the fifth and twenty-fifth year (Anders). It is rare after forty to fifty. It is much more frequent in dark races and in some tropical
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 567
climates (West Indies, South and Central America). It is more frequent in some regions of the temperate zone than others. It is more common in hot seasons.
It is caused generally by traumatism, and most often by contusions and penetrating wounds of the hands and feet. In cephalic tetanus there is traumatism of this extremity. In this country traumatism of the hands on Fourth of July causes many cases.
Symptoms. — The disease sets in from five to fifteen days after infection. It begins with feelings of stiffness in the neck and throat and sometimes with chilly feelings. Gradually tonic spasms develop which involve the trunk and head muscles, causing opisthotonos and other forms of rigid spasm. Trismus, or lockjaw, also occurs. The spasms are attended with intense pain. The patient finally lies in a state of general rigidity interrupted by painful tonic spasms. The muscles are of stony hardness. The jaws are set and the facial mus- cles are involved, the eyes being partly closed, the corners of the mouth drawn up, causing the characteristic risus sardonicus. There may be profuse sweating; some leukocytosis is present; tissue metabolism is not much changed. Fever is generally present and this may rise as death approaches to no° or 1120. The disease lasts from two to five weeks. There is evidence of irritation and congestion of the spinal cord and injured nerves, but no special anatomical changes are found. A specific bacillus producing a tetanizing poison has been dis- covered, the bacillus tetani.
The diagnosis is based on the characteristic history and the peculiar spasms. In strychnine poisoning there is no initial trismus or epi- gastric pain. In rabies there is also no trismus, but a respiratory spasm on attempts to swallow.
The prognosis is bad. About eighty per cent, of traumatic and forty per cent, of slow idiopathic cases die.
The treatment consists of complete rest and quiet in a dark room and the administration of chloral, bromide, morphine, and physo- stigma. Successful results from injection of blood-serum of an animal which has had the disease are reported. A tetanus "vaccine" has recently been used with some success.
Tetany (Tetanieea).
Tetany is a subacute or chronic spasmodic disorder character- ized by intermittent or persistent tonic contractions beginning in the extremities and associated with paresthesias and hyperexcitability of the motor and sensory nerves.
Etiology. — The disease is rare in this country, but relatively com- mon in Europe, especially in Austria. It occurs with frequency
568
DISEASES OF THE NERVOUS SYSTEM.
during the second, third, and fourth years of life and again at the time of puberty. Its rate of frequency then slowly declines and it is very rare after fifty. It affects males much oftener than females up to the age of twenty; after that the difference disappears. It occurs mostly in the working classes. In infants rickets is often noted. The exciting causes are exhausting influences like diarrhoea, lactation, sepsis, fatigue, mental shock, and fevers; also exposure to cold and wet. Alcoholism, dilatation of the stomach, and intestinal entozoa are also causes. It may be produced artificially by extirpa- tion of the thyroid gland, if the parathyroid gland is also removed. The disease sometimes appears as an epidemic.
Symptoms. — Tetany begins sometimes suddenly with symmetrical tonic contractions of the hands; at other times there are at first sen- sations of numbness, prickling or pain in the extremities, with ma- laise and perhaps nausea; then spasms begin. The attacks effect first and most the upper extremities. The flexors of the forearm and hand are usually involved; the fingers are flexed at the meta- carpophalangeal joint and extended at the other joints, and the thumb is adducted, producing the "accoucheur's hand." The fore- arm may be flexed and the upper arm adducted. The knees and feet are extended, the toes flexed, and the foot is inverted. In severe cases the muscles of the abdomen, chest, neck, and face are involved. Opisthotonos and dyspnoea may result. The muscles of the face and eyes develop contractions, and trismus sometimes occurs late in the disease. The muscles of the larynx, oesophagus, and bladder may be affected. Fibrillary tremors are observed in the contracted muscles, The attacks are accompanied by paraesthesias and cramp-like pains. There may be some abolition of sensation in the skin of the parts affected during attacks. The cramps last from a few minutes to hours or days. They occur during day and night and may wake the patient from sleep. Fever is sometimes present in epidemic cases. The disease has a tendency to recurrence.
While it lasts, both during and between the attacks, peculiar phenomena are observed as follows:
1. Increased Mechanical Irritability of Motor Nerves. — The motor nerves show an abnormal irritability, so that on striking the motor point a sharp muscular contraction is brought out. When pressure or a blow is made on the face over or near the exit of the facial nerve from its foramen, contractions of the facial muscles occur, es- pecially those of the lips. This is called the "facial phenomenon." By pressing on the artery and nerve of a limb a tetanic attack can be produced in the muscles supplied. It is probable that it is the pressure on the nerve alone which causes the phenomenon which is called "Trousseau's symptom."
2. The electrical irritability of the muscles and nerves is in- creased, especially to the galvanic current. Thus a negative-pole closure contraction (CaCC) is brought out by a very weak current; and if a little stronger it causes a tonic contraction or cathode-closure tetanus (CaCTe). The positive-pole opening contraction (AnOC) may be tetanic, i. e., AnOTe, and there may be even a cathode-opening
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS.
569
tetanus (CaOTe), a phenomenon not seen in any other disease. Ac- cording to Gowers there may be a reversal of the polar formula, so that a positive-pole closure contraction occurs earlier than a negative (AnCOCaCC). This is certainly rare.
3. An increase of irritability of the sensory nerves is shown by pressing upon them, when sensations of prickling and formication appear along their course. There is an increase also in the electrical sensibility, shown by appreciation of very weak galvanic currents. The auditory nerve reacts to the galvanic current in about fifteen per cent, of normal cases, and then only to strong currents and to only a partial extent ; but in tetany it reacts in nearly all cases, and with comparatively weak currents (2 to 5 or 6 ma.) on anode closure, anode fixed, and anode opening (AnC Klang, AnDKl, AnOKl) (Chvostek).
Fig. 237. — The Trousseau symptom.
The phenomena of hyperexcitability above described vary con- siderably and rapidly during the course ol the disease, and are not always present.
Types of the Disease. — -The disease varies in intensity and duration. This variation depends much upon the cause, and there have been a number of types of the disease based on the etiology. Thus we have :
1. Epidemic tetany.
2. Asthenic tetany due to lactation, diarrhoea, exhausting diseases,
etc.
3. Parathyroid tetany, due to removal of the parathyroid glands.*
4. Reflex and toxic tetany from gastric dilatation and intestinal worms.
When the spasms are continuous the disease lasts but a few
*A distinct form of tetany is due to disease of or removal of the parathyroid glands. It is believed that when tetany follows operations upon the thyroid gland itself, it is due to the removal of one or more of the parathyroids. These glands are, in human beings, very small objects— about half the size of a pea, four in number — the upper pair and the lower pair; and, according to Dr. Halstead, they have a definite vascular supply. In cases of tetany, due to partial or complete removal of these glands, the tetany can be kept under control by feeding the patients with these glands or the nucleo-proteid obtained from them.
570 DISEASES OF THE NERVOUS SYSTEM.
weeks; when they are intermittent it may continue for months. Epidemic cases last but a few weeks. The disease may be said in general to last from a few weeks to a few months.. It is rarely fatal. Patients are liable to a recurrence on return of the exciting cause.
Fig. 238. — The parathyroids. (Halstead and Evans.)
Pathology. — The phenomena of the disease indicate a congested and irritative condition of the gray matter of the spinal cord. The cause of this state is evidently in some cases (epidemic tetany) an infectious poison ; in other cases as auto-toxin in the blood (parathy- roid tetany), and in other cases it may be a rheumatic or some other
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 571
toxic influence. Ergot is known to produce symptoms resembling tetany.
In infantile tetany the irritation is apparently due to rickets and the reflex irritation of disordered bowels. It is doubtful if any reflex influence can be invoked in adults. Tetany is a functional disease and the symptomatic expression of a central or humoral toxin. This poison may be of different kinds, hence tetany has a claim to be called a distinct disease simply on clinical grounds. It has no such definite pathology as chorea or epilepsy. In the very few autopsies which have been made no definite organic lesion has been found.
Diagnosis. — The disease is usually easily recognized by the char- acter of the spasms, their symmetrical nature, their course, and the phenomena of hyperexcitability of the muscles and nerves. Trous- seau's symptom is found in no other disease. The "facial phenome- non," the peculiar electrical and mechanical irritability of the muscles and nerves, are very rare in other conditions. The sensory irrita- bility, and especially that of the acoustic nerve, is also characteristic. From tetanus the disease is distinguished by the intermittency of the contractions, their feebler character, the fact that they begin in the extremities and extend to the trunk, and by the absence of trismus, at least until late in the disease.
Treatment. — The cause should be removed if possible, lactation stopped, diarrhoea and indigestion corrected, the stomach washed out, worms expelled, rickets if present attended to. Rest, nourishing food, and tonics are indicated. Symptomatically, bromide of potassium in doses of 3iss. to 3ij- daily with chloral furnishes the surest relief. Hyoscine in doses of gr. t^q- may be tried. Inhalation of chloroform or injections of morphine are needed in severe cases. Lukewarm baths may be of service ; so also may ice bags to the spine. If elec- tricity is used only the weak galvanic current should be employed. 'Parathyroid feeding or injection of the nucleo-proteid should be tried.
RABIES AND HYDROPHOBIA.
Rabies is an acute specific disease, communicated to man from the lower animals, and characterized by severe and painful tetanic con- vulsions, paralysis and death. The disease occurs in nearly all parts of the world, and is common throughout the United States. All mammals are susceptible to the disease, and birds also may contract it.
Etiology. — The commonest source of infection is the bite of a dog; but the disease may be caused by the bite of cats, wolves, horses, cat- tle, and other domestic animals. The bites of wolves are the most dangerous, next those of cats, and next those of dogs. It is not com- municated by the bite of a rabid man. Bites upon the face and hands, the exposed parts of the body, are more dangerous on account of the rich nervous supply and from the lack of the protection of the clothing. The disease may be transmitted by saliva upon an abraded surface. About one-sixth of persons who are bitten by rabid dogs, become in-
572
DISEASES OF THE NERVOUS SYSTEM.
fected. Men are more susceptible than women, and nearly half of the cases occur in persons under 20. Rather more cases occur during the months from April to September, because more dogs and people are abroad during that period. The infection is contained in the saliva, and is secreted mainly by the parotid. Other glands may contain it, and it may be excreted in the milk. The blood and lymph are never virulent. In the infected person the virus pervades every part of the central nervous system, but is especially concentrated in the medulla. It exists also in the cerebrospinal fluid and large nerve trunks.
The microbe of rabies has not been discovered. Whatever it is, it makes its way into the nervous system, along the nerve trunks. It has an incubation period of about forty days, ranging between 20 and 90 days.
The characteristic histological change in rabies is certain bodies discovered in the nerve-cells by Negri. They are called the Negri bodies. These are not easily affected by external agencies, even by putrefaction, and are easily stained and observed under the micro- scope. By means of observing these bodies, the diagnosis, after death, of the disease, is very easily made.
The Negri bodies are specific of rabies, and they have been thought to be protozoa. This has not been proved, and it is still most prob- able that the virus of rabies is an ultramicroscopical body which is in some way connected with tissue proteids which form the Negri bodies.
Symptoms. — As a rule, there are no symptoms during the incuba- tion period. When the disease develops, there is some numbness, tingling, and perhaps radiating pain at the point where the bite oc- curred. The person suffers from nervousness, anxiety and depression; the sleep is disturbed, and then some symptoms referable to the throat appear. There is early a difficulty in swallowing. In a few days the symptoms of mental excitement appear. The patient becomes more restless, nervous and insomnolent. He suffers from thirst, but cannot quench it, because attempts to drink cause a painful spasm of the throat. As a result, while he desires to drink, the sight of water frightens and distresses him. Hence, the name hydrophobia. Then he begins to have attacks of convulsions, which affect first the muscles of the neck and throat, and then involve the whole body, causing symptoms resembling tetanus. At other times, there are very active co-ordinated convulsions, like those of hysteria. Slight sounds or any mental excitement will bring on an attack. Delirium and maniacal excitement complicate the condition, which lasts from one to three days. A general paralysis follows, and the patient dies in coma, from exhaustion, the temperature rising quite high. In cases where the
THE MYOCLONIAS OR TWITCHING AND TONIC SPASMS. 573
infection is very severe, the system is overwhelmed by the virus, and the patient dies very quickly with an ascending paralysis, which re- sembles a so-called Landry's paralysis. (Paralytic rabies.)
Diagnosis. — In the early stages of the disease, the mental condition of the patient, and the convulsive attacks are very suggestive of acute hysteria. The history of a bite and the person's respiratory spasms and throat spasms, will generally exclude this. In tetanus, there is more or less continuous rigid spasm between the attacks; the history of the disease, and the longer period of incubation, in rabies enable us to distinguish it.
Prognosis. — When the disease comes on, the issue is surely a fatal one. The careful application of the Pasteur treatment, by inoculation, reduces the mortality after bites from about 16 to one-half of 1 per cent.
The treatment after the attack comes on, is purely symptomatic, and consists in the use of morphine, chloroform and motor depress- ants, such as curare.
CHAPTER XXIV.
NEURASTHENIA (NERVOUS EXHAUSTION, BEARD'S
DISEASE.)
Neurasthenia may be defined as a chronic functional nervous disorder, which is characterized by a morbid nervous weakness and nervous irritability, so that the patient is exhausted by slight causes and reacts excessively to slight irritations.
It is sometimes a disorder by itself, but more often it is a condition associated with a psychosis. Hence, in describing neurasthenia be- low, we are really referring largely to neurasthenia plus a morbid mental state or, in other words, a neuro-psychosis. Pure neurasthenia as an independent general condition is rare. There are, however, local neurasthenias, as shown especially in gastric, enteric and sexual neurasthesia.
There are evidences that the neuropathic constitution existed in all ages, but coherent descriptions of clinical types, like the neuras- thenia of modern days, are not found in literature until the past century. The credit of calling attention to this condition most in- sistently, most acutely, and most successfully is due to Dr. George M. Beard.
Etiology. — Some doubt has been thrown over the question of the excessive nervousness and mental instability of the civilized nations of the present time. It is not a matter which can be fairly settled by statistics or the perusal of historical documents, but, on the whole, the evidence is, to my mind, conclusive that the human race does now suffer relatively more from nervous irritability and exhaustion, in its various types, than it did in the past. This I infer partly from the fact that the predisposing and exciting causes of psycho-neuroses are more largely present now than they used to be. The tendency of people to city rather than rural life is perhaps one of the strongest points in favor of this view, since we know it is in our urban population that neurasthenia breeds best. A larger proportion of persons now also use their brains in the struggle for existence and live upon a higher mental plane, with all the danger which that im- plies. The eighteenth-century writers attributed all the functional disorders then known under the terms "vapors," spleen," "hypochon-
574
NEURASTHENIA.
575
dria," "hysteria," to three things — luxurious living, sedentary life, and the unsanitary conditions of great and populous cities. They said nothing about the effects of overwork, continual anxiety, and mental strain, and one certainly does not gain from reading the English medical literature of this period that there was any such excess of work and worry among the people. While it thus seems certain that there is an increase in the mental and nervous instability of the modern race, it is not so much the real neurasthenic state as the allied psy- chic troubles that I have called phrenasthenia, which is the modern American disease.
At the present time, we know that neurasthenia is found more fre- quently among the highly cultivated races. I have seen it in negroes, but it is extremely rare, while hysteria and insanity are fairly common. The nervous temperament and the strenuous activity of North Americans are said to be due in part to the waves of cold air which pour down upon our Western and Northwestern States, causing quick changes of temperature and a cool, dry, stimulating temperature. Neurasthenia is said to be quite prevalent in Russia, and it is generally observed that it affects particularly often the Hebrew race. In this country we see it quite often in the Irish, but almost as often in the English, and rather less frequently in my experience in the Germans. Neurasthenia prevails rather more in dry temperate climates, but it is by no means infrequent in the tropical regions, and is to be found in the West Indies and in the republics of South and Central America in its classical forms. White people living in the tropics, unless under the best sanitary conditions, become nervous, and it is asserted that its cause is the excessive light acting upon non-pigmented skins. Neurasthenia is found rather more often in men than in women, but the difference is not great.
The neurasthenic age ranges from eighteen to fifty-five, but the larger proportion of cases is met with between the years of twenty and fifty. Occasionally symptoms resembling neurasthenia may be seen in children of the age of twelve or thirteen, and occasionally also there develops a kind of senile neurasthenia, which is, however, often associated with hypochondriasis, and some definite degenerative changes in the nervous or vascular system.
In men neurasthenia occurs more often in the single ; in women the relation is somewhat reversed, so that, taking both classes, the mar- ried and the unmarried are about equal.
Neurasthenia does not much affect the people of the country and small towns, though it does exist there. In great cities the number of neurasthenic women, among the wives of laborers and artisans, is
576
DISEASES OF THE NERVOUS SYSTEM.
rather large, and this is the natural result of the strain of living with husbands who are dissipated, and of rearing large families of children in the close quarters of a tenement house. The disease is relatively more frequent in the educated classes.
Hereditary influence plays a very considerable part in the develop- ment of neurasthenia. We can usually find that there is a history of migraine or some nervous irritability, of alcoholism or some psychosis upon one side or the other. A distinct history of the major neuroses or of severe mental diseases is rare, but there is no doubt that a very large proportion of neurasthenics come into the world with an over- sensitive and weakened nervous system. They may be strong enough to undergo the ordinary strain of life, but break down under some specially exciting cause.
The exciting causes of neurasthenia are very various, but they can most of them be classed under the head of excessive mental strain or shock, sexual abuse, and the influences of exhausting fevers, of chronic infections like syphilis, and of poisoning with bad food in early life, alcohol and tobacco or tea and coffee. The candy and soda-water habit of American children weakens digestion, impairs nutrition and brings on more trouble than many more apparently serious causes. In the larger proportion of cases of men, the trouble, if it develops during adolescence, is brought on by overwork at school and in college, combined with neglect of sleep and carelessness in diet. Frequently the abuses of the sexual function, of tobacco or of athletics are the exciting causes.
The practice of masturbation is one of the things for which neu- rasthenics very often keenly reproach themselves and over which much hypochondriacal brooding develops. Excesses of this kind, how- ever, are usually a sign of a degenerate or unbalanced nervous system rather than a cause. The actual harm done is greatly exaggerated, however strongly this practice is to be reprobated. Excessive and un- natural indulgences, such as sodomy, etc., tend to weaken the nervous system and are causal factors of neurasthenia. Bad methods of educa- tion and in particular excessive study are thought to predispose to nervous exhaustion. This is usually seen in ambitious college students or in young men who are forcing their way under great disadvantages through professional schools and into professional practice. Young women, who are excessively devoted to study and yet connot refrain from social indulgences, sometimes break down with nervous ex- haustion. The studies and training of the primary and secondary schools may prepare the way for these catastrophes, but they rarely come before the eighteenth year. It is not so much the hard work of
NEURASTHENIA.
577
schools as the bad habits of life at the period of education which break down the student. Besides, certain persons are destined to be neuras- thenic at the time of adolence.
Typical attacks of neurasthenia are undoubtedly brought on by the fright and shock incident to severe injuries or exposure to great danger, as in railroad collisions and other frightful forms of accident. A large proportion of the so-called "traumatic neuroses" are partly forms of neurasthenia; and morbid mental states can in time induce a neurasthenia just as the reverse is true. Neurasthenia can be brought on also by excessive child-bearing, the drain of lactation and domestic trouble, great excesses in eating and drinking, and the strain of hard domestic life and of sickness and nursing. Neurasthenia some- times follows an acute infection like that of typhoid fever or the grippe. It may also be induced by the infection of syphilis. It then comes on in the secondary or less often in the tertiary stage (Fournier). It is probable that in many of these cases the trouble is due to the ex- cessive use of mercury and saline purges. At any rate, antisyphilitic treatment can certainly bring on or bring out a neurasthenia. A com- bination of secondary syphilis with the excessive use of alcohol leads to a very obstinate type of neurasthenia. Still this must not be confused with the neurasthenia which precedes paresis and has an organic basis. Malarial poisoning seems also to have some influence as an exciting cause. Much weight has been laid upon the importance of eye-strain in producing neurasthenia, and, given a neuropathic constitution, there is no doubt that the defect in the refraction of the eye or in muscular equilibrium may cause, or at least keep up, a neurasthenic state. The same is probably true of severe forms of gastric disturbance, and of disease of the pelvic organs, such as subinvolution, decided displace- ments, and chronic ovaritis or salpingitis. In men the existence of prostatic irritation, of irritable strictures, and hemorrhoids and fis- sures, may start up neurasthenic symptoms. Chronic middle-ear disease and nasal stenosis are also put down as occasional exciting causes. The existence in neurasthenics of a tendency to constipation, or what is popularly known as "biliousness," accompanied by a gouty or lithaemic diathesis, has been much dwelt upon, and at one time neurasthenia was thought to be largely the expression of a disturbed state of the metabolism — a phase only of gout or lithaemia. This tendency, however, is rather the result of the weak nerve-centres than the cause, though the two often act in a vicious circle. Prolonged and severe dyspeptic disturbances, especially when associated with atony of the stomach and bowels and the condition known as gastroptosis and enteroptosis, are exciting or maintaining causes. Some writers class 37
578
DISEASES OF THE NERVOUS SYSTEM.
their patients under these heads, looking upon the neurasthenia as a secondary affair.
I would sum up the leading causes of neurasthenia thus:
1. Hereditary nerve instability.
2. Overwork and worry.
3. Severe shocks, with or without injury.
4. Infections.
5. Abuse of stimulants and narcotics.
6. Abuse of sexual functions.
7. Disorder of digestive functions and autotoxaemia.
This means that the causes are most often a bad heredity and fool- ish living.
These causes apply with almost equal force to the minor psychoses so often mistaken for or associated with neurasthenia.
A great deal of stress has been laid upon autotoxsemia as a cause of neurasthenia. There is no doubt that a great many symptoms and crises are brought about through this agency, but the attempts to prevent autotoxaemia by perpetually stimulating the liver and giving intestinal antiseptics, of using a large amount of water and the sim- plest kind of diet, do not of themselves cure the general disease, unless measures are taken to strengthen the impaired tone of the whole system.
There are, however, underlying certain neurasthenic conditions, de- fects or perversions of secretion like that of the thyroid. It may be that a defect in metabolism due to inadequate or excessive function- ing of various glands forms the basal secret of many neurasthenic conditions.
Symptoms. — The symptoms of neurasthenia, while manifold, have yet a pretty distinct general resemblance to each other, and the clinical picture of typical forms of neurasthenia is quite as pronounced as that of other nervous maladies. As already stated, a great deal of what is mental has been written into neurasthenia. In other words, the minor psychoses such as hysteria, psychasthenia, hypochondria, abortive melancholia, have been often looked upon as neurasthenia. The time has come now, however, when these conditions should be recognized and treated separately, and thus greatly limit the field of the disease. For neurasthenia is, strictly speaking, the name to be applied only to a neurosis — a morbid nervous, not a morbid mental state.
It will be easier to understand what neurasthenia is by indicating first what it is not; in other words, by excluding certain quite well- known groups of psychic disorders.
First, then, we exclude from the symptomatology of neurasthenia
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the mild types of recurrent melancholia or, as they are now termed, "the depressive phase of manic-depressive insanity." Later in life the malady takes more the form of an anxiety psychosis, with depres- sion, insomnia, a great deal of worrying apprehension, dysthesias, psychic pain and mental inactivity. The simple nervous exhausti- bility is in the background. These melancholic troubles are very often mistaken for neurasthesia.
Second, we exclude the psychosis characterized by a dominant sen- sory complex. It includes patients who are continually complaining of dysthesias, headaches and head paresthesias, stomach troubles and various bizarre forms of physical suffering and functional disturbance. This trouble is only an abortive or early type of what later may become the true anxiety psychosis or the melancholia of involution. The disease is seen in young people; it may last for years ; the patients are often looked upon as cases of hypochondria. They are, however, really representatives of the anxiety psychosis.
Third, we exclude the psychosis (psychasthenia) characterized by obsessions and fixed ideas, of apprehensions, of doubts and abulic states, and psychic tics. The patients have morbid fears of contamina- tion, or of going to certain places, of being alone. They have harass- ing doubts, and inability to decide any question by themselves. They have certain precisions, that is to say, things must be done in a certain fixed and regular way, and they are slow and dilatory in their work and conduct.
Fourth, we exclude the psychoses characterized by uninhibited emotion, the persons with exaggerated egotism, aggressive and mulish attitude, violent and unreasonable explosions of temper, morbid jeal- ousy. They are quasi-delusional in their views of people and conditions about them, and are absolutely unreasoning in the views which they hold. They are intelligent, quick-minded, but self -centered and abso- lutely selfish ; they are looked upon generally as "possessed" or hys- terical, but are related most to the paranoiac class.
Thus, we remove from the realm of neurasthenia the psychoses characterized by melancholic states, by the melancholic state associated with introspection, anxiety and persistent subjective physical com- plaints, the psychoses characterized by obsessions, morbid fears, abulia, impulsions and the psychoses characterized by excessive egotism, emotional outbreaks, jealousies and chronic querulence. Melancholia, hypochondria, psychasthenia, and "hysteria" or the querulents, are the names commonly given to typical examples of these maladies. There remains, then, to describe under the head of neurasthenia proper, two types of a neurosis, one the neurasthenia
DISEASES OF THE NERVOUS SYSTEM.
prcecox or primary neurasthenia of adolescence, and second, the neurasthenia acquisita or the neurasthenia produced by work, worry, shock, infections, toxins, sexual excesses, various acquired or con- genital disturbances of the stomach, intestines and the visual appara- tus. Besides these, or associated with them, we find occasional neurasthenia, especially localized, upon the heart, vasomotor centres, digestive or sexual organs.
The general symptoms of primary neurasthenia are very much like those of the acquired type; and the following description fairly outlines the clinical picture of each. In the prsecox type, the asthenia is of a more simple character; there is less evident trouble with special organs, less mental distress and fewer psychic symptoms. The young person gives out all along the line, but the malady is apt to be more persistent, and after apparent cure remissions may occur. It is not a frequent disorder, and it may come on long after the ordinary period which is about the eighteenth to the twenty-fifth years. Some- times this type of neurasthenia is followed later by a serious psychosis, such as melancholia or paranoia. This rarely happens except in per- sons of very bad heredity. One of the first symptoms is an annoying insomnia. It is usually an early insomnia, i.e., the patient cannot get to sleep till late, and then sleeps badly, frequently awakening. Distressing dreams occur, and the patient wakes unrested and more tired than when he went to bed. The patient complains of a general feeling of mental depression — life is not the interesting spectacle to him that it formerly was. The man who once delighted in work can hardly force himself now to go to it. He tires very quickly over tasks which were formerly easily performed, or he simply cannot work at all. He loses his power of initiative, of mapping out work. He absolutely cannot pursue I a train of thought. Even pleasures tire him, and every effort is followed by a headache or the penalty of two or three days resting in bed. He is very easily irritated at things which before caused him no annoyance, and becomes a source of domestic unrest and unhappiness. He is op- pressed with the fear that he will never get well. He suffers from a number of peculiar sensations which are called "cephalic parsesthe- siae." These are sensations of pressure on the top of the head, or a feeling of constriction around the temples, or a burning spot on the vertex, or tenderness of the scalp. Sometimes he has a sense of weakness or even pain in the back of the neck. All these things are increased by the use of the mind or by bodily exertion, and they are all more common in the acquired types. Headache occurs in perhaps one- half of the cases, the headache being usually either frontal or occipital.
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It is often very persistent, and in fact a chronic headache, not due to tumor or meningitis or syphilis, is almost invariably of neurasthenic origin. This neurasthenic headache is usually diurnal only, coming on in the morning when the patient wakes up, and lasting a good part of the day. It does not often keep him awake at night. In this point it is distinguished from the headaches of syphilis and of meningitis or of tumors. Women suffer from these headaches, and from pains in gen- eral, more often than men. They in particular have much pain in the back of the neck and along the spine. This keeps them from walk- ing or being upon their feet, and it may develop into a form of trouble known as "spinal irritation."
The special senses are not very seriously affected. The patients can often see quite well, but their eyes soon tire; they cannot read a book long because it makes the eyes smart or produces some head- ache. Examinations of the neurasthenic's eye frequently shows the existence of some refractive error, most frequently astigmatism and hypermetropia ; defects in the ocular muscles, and especially weakness of the internal recti, often occur. Patients have frequently com- plained to me of a defect in visual memory. They see a thing or face but do not remember it again as readily as they used to. There is no limitation of the visual field in true neurasthenia uncomplicated by organic disease, but there is a morbid susceptibility to fatigue, par- ticularly of the periphery of the vision, so that, after long testing, objects in the periphery become less distinct, and a sort of artificial limitation of the field may be produced. In some cases an object which is brought from without into and across the visual field is seen in wider range than an object which is placed in the centre of vision and carried gradually out toward the periphery. This is the reverse of the normal condition, and is known as "Foerster's shifting type." Peculiarities of accommodation, a slight drooping of the lids, in- equality of the pupils, and excessive mobility of the iris have been noted in neurasthenia.
Neurasthenics of the acquired type sometimes suffer from tinnitus, which is very distressing and aggravates every other nervous symptom, but this usually occurs only in connection with actual disease of the middle ear, or in old people with degenerative changes in the cerebral blood-vessels. An excessive sensibility to noises, and even the pleas- ant sounds, like those of music, may be present. Neurasthenics sometimes cannot bear even the most enchanting melodies. A similar morbid sensibility to taste and smell may be present. But these are matters of minor movement, and are much more often seen in hysteria.
There is no doubt in my mind that in neurasthenics the general
582
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muscular and nervous strength is lessened, and although the patient may not have lost flesh, and may not appear particularly weak, he tires quickly on ordinary exertion, and the tests of the dynamometer show a lessened response. A fine tremor of the hands is often present, and when the eyes are tightly closed there will be a quivering of the lids, and in very acute and exaggerated cases twitchings of the muscles of the face and tongue, almost like those in general paresis. This rarely occurs, however, unless the patient has, in addition to the neurasthenia, a considerable amount of toxaemia from alcohol, to- bacco or tea.
The reflexes are greatly exaggerated, but ankle clonus does not occur except there is some complication. Blows upon the motor points promptly bring out responsive contractions. The cutaneous reflexes are also exaggerated. These things vary considerably, however, in different cases, and are more marked in the younger patients and those of a neuropathic constitution.
The sexual function is irritable and weak and in certain acquired cases this local trouble dominates the situation. But here a psychosis is often complicating the situation.
There is a considerable disturbance of the heart function in neuras- thenia. The most frequent condition is an acceleration of the pulse beat from very slight cause, due to a weakening of the inhibition of the heart. A pressure over some painful point in the body will sometimes bring up the pulse from 80 or 90 to over 100, and it will remain there for one or two minutes. This is called "Rumpf's symptom." Ar- rhythmia and palpitation of the heart are less frequently observed. It is my belief that cardiac weakness is an important condition in many forms of neurasthenia and underlies sometimes a good many of the otrjer symptoms. This is particularly true of the neurasthenias of more advanced life. The blood-pressure is usually low and very variable. The cardiac disturbances are more frequent in women, in young people, and in neurasthenia associated with the use of tobacco and tea. The existence of some degree of thyroidism should be looked out for in patients with very irritable hearts. Ordinary valvular lesions have little to do with causing neurasthenia, but when a patient with a bad myocardium becomes neurasthenic his heart gives him much trouble. He feels its irregular beating and skipping and he hears its throb when he lies on his pillows; its action is disturbed by very slight exertion and slight anginal sensations are present.
A great deal of emphasis has been laid upon the vasomotor dis- turbances of neurasthenia, and a large number of neurasthenic symp- toms have been ascribed to a weakening of the vasomotor centre. .As
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a result of this the patient suffers from cold hands and feet, from flush- ing of the face alternating with pallor, from dermographic skin, and from those symptoms which we usually attribute to cerebral conges- tion, such as a sense of fullness in the head, headache, spots before the eye, dizziness, and noises in the head.
The condition of the urine has been studied very closely in con- nection with this subject. In fact, many of the symptoms which we now call "neurasthenic" were described by Dr. Prout and Dr. Gold- ing Bird early in the last century and were held by these gentlemen to be due to oxaluria. This was a condition characterized by flatulent dyspepsia, melancholia, and nervous irritability, and was thought to be due to defective metabolism, resulting in the production of an ex- cess of oxalic acid. More recent studies have shown that oxaluria is only one of the manifestations of lithgemia, and that while it is signifi- cant, as was then supposed, of defective nutritive changes, these are more dependent on a neurasthenic state than primarily upon dyspepsia and metabolic disorder. There are, according to Herter, few cases of neurasthenia which do not show in the urine or faeces some indication of defective metabolism. "The faeces often contain excessive amounts of urobilin or some related substance. The urine is usually concen- trated and of small volume (600 to 1000 c.c. in twenty-four hours). Frequently there is an excessive excretion of phosphoric acid (P2O5) and an alteration in the quantitative relation of urea and uric acid. In health the relation of the uric acid to the urea excreted varies be- tween 1 to 45 and 1 to 60 in adults. In neurasthenia (as well as some other conditions) the relation is often 1 to 40, 1 to 35, or 1 to 30. In- dican is often present in pathological quantities, especially in cases of sexual neurasthenia. Oxalate of lime is often present in excess in the urine" ("Diagnosis of Nervous Diseases").
In rare cases one finds in neurasthenics a temporary albuminuria ; I have observed it only once in one hundred cases, which is about the average. This albuminuria is not associated with the presence of casts or other evidence of kidney disease, and it is apparently due to a pare- sis of the vasomotor nerves of the kidneys. Transitory glycosuria is more often found. This glycosuria is usually associated with a heavy urine and evidences of lithaemia. Some authors (Hosslin, Dercum) state that there is an excess of uric acid very uniformly in neurasthenia, and that this uric acid results from a breaking-up of the nuclein of the cells. It has been ingeniously suggested, therefore, that since the nuclei of nerve- cells become smaller and irregular in shape when the cell is exhausted, it is from this source that the uric-acid excess comes. All observation, however, shows that excessive use of nervous tissue leads to an exces-
5§4
DISEASES OF THE NERVOUS SYSTEM.
sive excretion of phosphoric acid rather than of the urates. I refer specifically to brain and nervous tissue, not to psychic activity, for the old idea that excessive mental activity is attended with increased excretion of phosphorus has been disproved. My experience in studying the urine of neurasthenia is that in the younger cases, with a strong neuropathic taint, it is variable in specific gravity, but, on the whole, rather low; and that the daily amount, as Dr. Herter states, is below the average. It is of a low specific gravity also in neurasthenia, oc- curring after middle life when the arterial changes of that period begin to set in. In early adult life the urine is more often found to be con- densed, as others have observed, and to contain the products of de- fective metabolism. The urine thus shows either a weakened and slowed-up nitrogenous metabolism or a perverted metabolism. The important things to determine, then, in examining the urine, after excluding such evidences of serious change as albumin and sugar, are : the specific gravity and the daily amount, the amount of phosphates, the amount of urates and uric acid and their relation to each other, and, finally, the presence of indican or other products of perverted nutrition and digestion. I do not find indican very often, and practically never in the light urines.
The digestion of neurasthenics is often more or less affected, and a large proportion of them are probably treated mainly for their stomach conditions. I do not, however, usually find cases of serious and genuine gastric disturbance. In the majority, under proper rest treatment and proper diet, the tongue soon cleans off, and the patient complains relatively little of the stomach, though his nervous symptoms continue. The neurasthenic, it is true, has always a feeble digestion, and has to take great care of what he eats and drinks, but when he is put upon the kind of diet that he should take the stomach gives rela- tively little trouble. This is especially true of the younger cases. In a certain number a gastroptosis occurs, and the lower curvature drops below the umbilicus. The patient suffers from flatulence, acidity and epigastric discomfort, and anorexia and nervousness. There is atony of the gastric wall, but no very marked change in secretion, and no especial fermentation (Lockwood). It is only in patients who have abused themselves with alcohol or tobacco or excessive indulgence in sweets or with ravenous feeding that worse conditions are found. In people of more advanced age, feebleness of digestion is often associated with a more serious relaxation of the stomach and intestinal walls, and a great deal of atony of the whole intestinal tract. In these cases, which we find particularly often in women, the prolapse of the intes- tines, stomach, and perhaps of a kidney may cause a great many dis
NEURASTHENIA.
tressing symptoms. The condition has been described by Glenard under the name of "enteroptosis," and it undoubtedly is an important factor in keeping up the neurasthenia of some women in adult and middle life.
Among the most serious, though fortunately rare, symptoms of neurasthenia involving the digestive tract is the condition known as "mucous" enteritis. This trouble generally attacks women rather than men, and usually women between the ages of twenty-five and forty. It comes on after the patient has become exhausted by pro- longed domestic cares and fashionable dissipation, or some shock. It is one of the earlier symptoms of the nervous weakness, and begins with abdominal pain, followed by attacks of diarrhoea, in which tubu- lar casts are passed, or portions of such. This diarrhoea is painful, colicky, and alternates with periods of constipation. There is, in my experience and in that of others, a somewhat spastic condition of the bowel, as though it were irritated and closed down upon the contents of the intestine. The term mucous enteritis is not strictly a proper one, since microscopical examinations and autopsical reports show that the substances thrown off are not mucous mainly and that there is no actual inflammation. The casts that are found in the stools are com- posed principally of albuminous substances, the product apparently of the decomposition or disintegration of the epithelial cells of the in- testinal walls. While mucous enteritis sometimes occurs in persons who are profoundly asthenic without any decided neurasthenia, yet, in the great majority of cases, it is a symptom of neurasthenia, and can be successfully treated only on such a basis.
The respirations in neurasthenia are generally normal, but shallow and deficient respiratory expansion sometimes exists. In women par- ticularly I have often found that there was an actual inability properly to expand the chest and inflate the lungs.
The temperature is normal, and a very variable temperature of the skin is simply dependent upon vasomotor instability.
The composition of the blood is often quite normal. Hosslin finds that even in those patients who appear to be anaemic there is a normal amount of haemoglobin; however, anaemia certainly exists in many cases, and there is no question that the use of iron is often of great benefit.
Variations in the weight of the neurotic often occur. Neurotic patients may gain or lose ten or twenty pounds within a year or two. The secretions of the skin are usually increased, and the patient sweats easily and profusely. In other cases of a less irritative type the skin is inclined to be dry. Its nutritional condition is poor, the hair
586
DISEASES OF THE NERVOUS SYSTEM.
falls; and, according to Beard, there is a tendency to early decay of the teeth.
The foregoing description of the general symptomatology of neu- rasthenia is likely to confuse the reader on account of the multiplicity and wide extent of the symptoms. It is quite true that few neuras- thenics have all of the symptoms just described, and it is still more true that in most of them the patients have certain leading and dominant symptoms which annoy and depress them, and that the larger pro- portion of the manifestations of the disorder are trivial to them, as they are to the physician.
The Different Forms of Neurasthenia. — The peculiar type of neurasthenia depends mainly upon the age, the sex, the particular function especially disturbed, and the hereditary endowments of the individual.
Primary Neurasthenia {Neurasthenia PrcBcox). — Neurasthenia ap- pearing at the time of adolescence is much more apt to be associated with a primarily weak nervous constitution. The mental symptoms are dominant only when the malady takes the character of a psychas- thenia, with some fixed ideas or morbid fear. Naturally, also, at' this time, sexual ideas and sexual symptoms very largely predominate, but this, again, is a condition in which the psychosis is the important feature. In the pure type of neurasthenia prcecox the symptoms are chiefly those of easy exhaustion and irritability, and of incapacity for work because of this and not through any apprehension or morbid mental state. Along with this are oftenest evidence of weak and nervous digestion, perhaps some falling of the stomach and con- stipation. The patient has some headaches, but not a great deal of pain or paresthesia and is comparatively comfortable if kept quiet.
Acquired Neurasthenia. — It is during the mature, active life of men and women that the more distressing forms of neurasthenia occur. In these patients the element of heredity is less marked, while the ex- trinsic causes of neurasthenia, such as excesses in eating and drinking, shocks, injuries, poisons, syphilis, and gouty tendencies, all come much more into play. Here again we find evidence of digestion disorder, of cardiac irritability, of cerebral paresthesia, headache, neck and sacro- lumbar pains, coccydinia, thyroidism, menstrual disturbances and local irritation from the uterus and ovaries or from the genito-urinary tract.
Traumatic Neurasthenia, Traumatic Psychoneurosis. — After receiv- ing an injury which is often but slight, but which is usually accom- panied by a great deal of fright and emotional disturbance, the patient goes to his home feeling perhaps a little nervous and shaken, but not
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suffering to any great extent. He goes to bed and sleeps ; he wakes up the next morning feeling not quite so well as usual, but congratulating himself, perhaps, on having gotten off so easily. He resumes his work and finds that he can do it, though with not quite so much ease as usual and he very likely suffers from some pain due to a strain or bruise that he has received. In a few days — almost always within a week — he begins to notice that he is more nervous than usual, that little things irritate him which did not do so before, that his head seems somewhat confused, and that the effort to work is wearying. His sleep is dis- turbed, and he wakes up in the morning unrefreshed by his night's re- pose. He becomes somewhat despondent over his condition, and thoughts of paralysis or some other serious ailment annoy him. His head aches, the pain being more or less constant and diffused, and lo- cated usually over the forehead or at the back of the neck. He has unpleasant sensations in the head, such as that of constriction or press- ure or scalding feelings. His back also is continually painful, and walking increases it. His nervousness becomes more marked, and close examination shows a little, fine tremor in the hands. He has also sometimes creeping sensations over the body or numb feelings in the extremities. He tires very easily. He is emotional, and becomes more despondent as the days go on. Sometimes he has spots before his eyes, noises in his head, or ringing in the ears. Reading is laborious and increases his headache; so also does attention to work. His ap- petite becomes capricious and his bowels are constipated. He suffers somewhat from flatulency and dyspepsia. His heart palpitates easily, and the pulse is a little accelerated. Sometimes for a few days there is a little weakness about the bladder or irritability of that viscus. His sexual power is diminished ; his circulation seems rather poorer than usual. Very slight excitement produces sweating of the hands or coldness of the extremities. He loses a little flesh. The picture, as may be seen, is very much like that of neurasthenia of the acquired type plus a little more of the physical and sensory complex.
These symptoms may be several weeks in developing, and during this time he may perhaps consult a lawyer about his case. If so, the anxieties of litigation begin to add to and intensify his troubles. He consults a physician, and the physician finds the subjective symptoms that I have mentioned. Objectively, when examined, the physician will discover that the muscular power is somewhat weakened, that there is a certain amount of the fine tremor perhaps in his hands. The knee-jerks and elbow-jerks are exaggerated; there are tender points along the spine and upon the head. In making him stand with his eyes closed there is a certain amount of static ataxia discovered. The
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DISEASES OF THE NERVOUS SYSTEM.
pupils are often dilated and mobile, and examination of the visual field shows sometimes a slight contraction, at other times the "shifting type" already described. In many cases a degree of peripheral retinal anaesthesia will be discovered. The pulse will be found accelerated, and pressure on a tender point may send it up very rapidly; a slight exertion will also accelerate it. There will be something apparent in the physiognomy of the case which shows the man to be in a nervous and asthenic condition. Sometimes the pains from which the patient suffers in the back and the weariness in the limbs are so great that he remains a good deal of the time in bed. In all cases he will assert most positively that he is unable to work or to take that interest in his affairs that he has previously done. In a good many cases there will be added to the foregoing picture a number of symptoms due to some local injury; for example, the arm may have been wrenched or bruised, and the result may be a certain amount of neuritis and weak- ness or pain in that member ; in other cases the back may have been so severely sprained that the typical symptoms of spinal irritation ensue, and this is particularly apt to be the case when women are injured ; in other cases, again, the legs may have been hurt to such an extent that a sciatica or some other form of neuralgia develops.
The foregoing symptoms, varying in amount and degree, will last, with little change, for a very long period of time. If the case goes into litigation, there is added the worriment occasioned by having to go through the disturbing experiences of trial by jury so that what was at first mainly a neurosis becomes a psychoneurosis, or almost a psychosis. In many cases, after the trial has been settled and damages awarded or otherwise, the patient begins to mend, and in a certain proportion of cases he gets completely well. This is not invariably the rule.
Spinal Irritation {Spinal Psycho-neurosis). — Spinal irritation is a form of neurasthenia in which, associated with the general neuras- thenic symptoms, are certain special, painful symptoms, related chiefly to the sensory nerves of the spine. These cases have in the past been described under the head of "spinal anaemia" and "hyperaemia." They may develop in traumatic neurasthenia. The patients are usually young women, between the ages of sixteen and twenty-five. The trouble is sometimes brought on by injuries or by a physical over- strain. Sometimes it seems to be associated with a natural weakness of the spinal muscles and a consequent curvature. Sometimes it follows acute infectious diseases. The patient begins by complaining of pain in the back — usually in the lower part — and also in the back of the neck. These pains occur on standing or walking, or any exertion,
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and are so severe that the patients in the course of a few weeks or months give up attempting to walk about. They get relief and com- fort in bed, and so they go there and remain. The pains are of a heavy, aching character, increased until they become very sharp when attempts at movement of the trunk are made. There is a great deal of tenderness to pressure along the spinal processes, some of these proc- esses being much more sensitive than others. The most sensitive points are usually in the back of the neck and the upper dorsal vertebrae, and down in the lumbar region. There is some pain also upon pressure alongside of the spinal processes. Painful points often vary, and even in a single examination the patient may complain, and complain honestly, of different sensitive vertebrae. Pressure on these points does not often bring out visceral symptoms, as the brothers Griffin taught, but may cause faintness, nausea and tears from the pain. The patients suffer much from headaches. The arms are often weak, so that attempting to sew or write or hold a book causes pain in the neck and shoulders. The legs are also weak and the circulation is poor. There is sometimes palpitation of the heart and precordial distress. A certain amount of dyspepsia is always present, and constipation is the rule. The patients often have attacks of vomiting, and attempts to feed them require much care. The menstrual functions become ir- regular. The patient grows weaker and often becomes bedridden, es- pecially if little attempt is made to overcome the symptoms by volun- tary effort and attention to nutrition. These patients generally get well in from one to three years, but occasionally they sink into per- manent invalidism. The symptoms are quite as much due to mental sensitiveness and disordered cerebrum as to any local spinal trouble, and the term psycho-neurosis is a more correct one.
Local Neurasthenia. — There are cases in which the specially weak and exhausted function is the heart and vasomotor centres, or the stomach and intestinal tract, or the sexual organs. The symptoms here are much the same locally as those described above under the head of neurasthenia. But the patient with a nervously weak stomach or heart, or sexual organs may yet be strong enough to do a fair amount of work. It is only one or two of the somatic functions that are in- volved. Here the term local neurasthenia is a justifiable one. In many of these cases, however, a mental element develops and often local neurasthenias are also associated with a psychosis.
Angiopathic Neurasthenia. — Here the patient has some general symptoms of neurasthenia, but in particular he has special symptoms which consist of a sense of pulsation or beating, which involves the whole body. The tension of the pulse is low, the rate normal or
590 DISEASES OF THE NERVOUS SYSTEM.
slightly accelerated. He does not have palpitations of the heart, as in Basedow's disease, and there is no particular dyspnoea on exertion. The skin usually shows a striking degree of dermography, and there is an epigastric pulsation, as well as pulsation of the carotid. In these cases there is, no doubt, some thyroidism and perhaps most can be classed under this head. But such is not the explanation of every case. The pathology is suggested by the fact that one of my patients was rather promptly cured by adrenalin. And the trouble may be due to an inadequate activity of the suprarenal capsules.
Gastric Neurasthenia. — In many instances where a general neuras- thenia exists, a specially weak organ shows the dominant symptoms and it is often the case that this is the stomach or gastrointestinal tract. The malady is then termed gastric neurasthenia or if it occurs in a mild form "nervous dyspepsia." The trouble is characterized by an atonic condition of the muscular wall of the stomach, and perhaps also by defective secretion, so that digestion is slow and imperfect. As a re- sult, the stomach dilates and prolapses to some extent. The patient is then said to be suffering from gastroptosis. There is a persistent sense of discomfort over the epigastrium, which may be increased or relieved at first by food, but which sooner or later is made worse by the meal, especially if it is badly selected. There is difficulty in digesting any but the simplest foods, and a special discomfort from sweets, rich fatty food, large amounts of meat, alcoholic beverages, and tea and coffee. With this are some general neurasthenic symptoms, but they are not very severe, and the whole symptom-complex is relieved by a combination of rest and strict attention to diet with stimula- ting local applications and massage.
Neurasthenia Gravis. — -In instances which are fortunately very rare neurasthenia assumes a very severe and serious type of exhaus- tion. The patients suffer from the typical symptoms in much the ordi- nary way, but the degree of weakness is very much exaggerated. Such patients have not only headaches and disturbed sleep, pains in the back and paresthesias, digestive disturbances, and mental depres- sion, but they speedily emaciate to a considerable extent. They take food in fair amounts, but it gives them no strength. The most care- ful applications of the "rest cure" secure for them only temporary benefit. They cannot walk far without intense fatigue and exhaustion, with subsequently severe headaches, or even attacks of vomiting and diarrhoea. Despite closest examination, no distinct signs of organic disease can be discovered, and I have known, such patients to go on into a permanent and hopeless invalidism which has lasted for many years. In these cases there is not a hysterical or even large hypo-
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chondriacal element. No amount of suggestion or "mind cure" has much effect upon them. They are not, in other words, hysterical, bedridden women, but often men who have reached or passed the middle period of life, and the condition is one suggesting a premature senescence of the nervous tissues. Even these very worst cases may get well after a period of eight or ten years.
Pathogeny and Pathology. — Victims of neurasthenia are persons who in all cases have either inherited or acquired a nervous system with lessened power of resistance. In the vast majority of cases I believe that inheritance is the cause of this weak nerve structure. Such inheritance may be very slight, and, if the patient lives with reasonable care, he has good health and lives to an old age. Under the influence of severe and depressing agencies, or of poisons or infec- tions, however, this resisting power of the nerve-cells is weakened. The person then is ripe for an attack of nervous exhaustion.
It seems probable that an inherited tuberculous taint in a measure prepares the system for nervous prostration. Among acquired diseases syphilis undoubtedly impairs the physical strength and makes the person predisposed to neurasthenia. So, I believe, does excessive indulgence in alcohol, tea, and tobacco, and I would add an extreme indulgence in the carbohydrates, such as candies, sweets, and pastries of all kinds, when taken continuously in excess of a normal ratio.
A considerable number of cases, including most of the traumatic forms of neurasthenia, come on suddenly as the result of a single severe shock. Here we must invoke the agency of the vascular system. Under the influence of intense and sudden emotions of the depressing kind, the vasomotor centre and the whole vascular mechanism go through a kind of convulsion, and this convulsive disturbance is a thing which the vasomotor system of those predisposed to neurasthe- nia is unable to withstand. The nerve-cells connected with it are so weakened in their nutritive and functional power that the blood is not carried regularly and normally to the nerve-centres in the way to which such centres have been accustomed ; hence the nerve-cells beeome impaired in nutrition and functionating power.
Another factor undoubtedly exists in the production of neurasthe- nia, and that is the irritation of the nerve-centres by poisons generated within the body. We know that in certain forms of digestive disorder poisons are probably absorbed into the blood, and we know also that in gouty and lithgemic states the uric acid and other products of defective metabolism poison the system and induce many of the symptoms of neurasthenia. There is, therefore, this element of autotoxaemia
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DISEASES OF THE NERVOUS SYSTEM.
which enters measurably into the production of neurasthenia. The subject, however, has yet to be worked out into definite shape. When a person has suffered from neurasthenia for a considerable time, there are, no doubt, certain more or less permanent changes in the body; at least we note that catarrhal conditions of the stomach and bowels may become permanent, and that anaemia may be present. In cases occurring in persons advanced in life, arterial changes become more rapidly pronounced than in healthy persons. In fact, a prolonged neurasthenia, with the accompanying worry and mental depression, no doubt hastens and accentuates degenerative vascular changes. Dercum has suggested the name "terminal neurasthenia" for that condition of chronic nerve exhaustion in which anatomical changes have become fixed. Finally and in connection with this last point, we suspect that at the bottom of even simple and hereditary neuras- thenia, the trouble may be glandular and secretory rather than ner- vous. In other words the nervous centres are exhausted and irritable because of inadequate or excessive secretions of the blood glands or those connected with alimentary tract. The secret of the weak nerves may be in the fact that they are not fed properly by the juices of the body. It is along this line that some fruitful work has been done in treating neurasthenia and in understanding its nature. It is to be hoped that more will be learned of the subject and that neurasthenia may not have to be placed among the teratological defects.
Diagnosis. — Neurasthenia is to be differentiated from the condi- tions which have been already enumerated :
1. Mild attacks of recurrent melancholia.
2. Early and mild types of the anxiety psychosis, commonly called hypochondriasis. Primary neurasthenia may be associated with this psychosis, or may lead to its development. Then we have what is called constitutional neurasthenia, which is a neuropsychosis, with a rich subjective symptom-complex of painful and bizarre complaints.
3. The psychosis known as phrenasthesia or psychasthenia, which includes the groups of doubting manias, fears and obsessions, etc. Some neurasthenia usually underlies these morbid mental states.
4. The explosive, ill-balanced, violent-tempered, egotistic, unrea- soning types which seem to border in a degree upon paranoia, and in other respects to resemble hysteria, in their extraordinary and ill- advised reaction to their environments. For these patients are never satisfied with themselves or those about them. They are commonly called hystericals, but they rarely show the physical stigmata of hysteria.
The early stages of mild forms of melancholia simulate neuras-
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thenia. This is so much the case that some authors have described neurasthenia as an abortive form of melancholia. The distinguishing points which enable one to recognize melancholia are the persistent loss of sleep, extreme mental depression, with delusions and suicidal ideas, the mental apathy and retardation of thought and slowness of action.
In the early stages of general paresis the patients suffer from neurasthenic symptoms. They find that they are no longer able to work as they did before, their sleep is disturbed, they are excited, forgetful and nervous. They have not yet developed many of the physical symptoms perhaps, hence their condition suggests and is often mistaken for a simple nervous breakdown. The condition is much more perfectly simulated when the patient has been taking a good deal of stimulation, in order to keep himself up to the mark. The characteristics of the paretic condition are given elsewhere. The early and neurasthenic symptoms of dementia prgecox are also described elsewhere.
A patient may be suffering from a number of bodily ailments, and if this person be at the same time of a somewhat nervous constitution the condition may resemble neurasthenia. Those persons having a very feeble digestion, with dilated stomach and an atonic condition of the alimentary tract, may get depressed, fretful, and sleepless; so a person suffering from some chronic uterine or ovarian or bladder trouble may present many symptoms of nervous irritation. These patients may have only a lccal neurasthenia or a real local disease with neurasthenia as a reflex ccndition. It must depend largely upon the good sense of the physician to measure the importance of the local troubles as compared with those of the general symptoms. I believe that the fully developed type of neurasthenia is rarely brought out by local disease alone. Still, I have seen cases with neurasthenic symptoms cured for a time by washing out the stomach, and enormous relief to the nervous irritation to result from treating the condition of the blood or relieving the uterine disturbances.
It should finally be remembered that some neurasthenia compli- cates many of the minor psychoses.
Course and Prognosis.— There is such a thing as acute neurasthenia. This follows prolonged debauches and long periods of excessive mental strain, with loss of sleep. Such patients may present all the signs of neurasthenia, and get perfectly well in two or three weeks. Neu- rasthenia, however, is essentially a chronic disease, and when speaking of it we refer to this type of the disorder. It is a disease which comes on as a rule gradually, developing, however, in the course of a few 38
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DISEASES OF THE NERVOUS SYSTEM.
months. It may, however, come on suddenly after shocks and acci- dents, and it may develop or follow rapidly after an acute infectious fever. It always reaches its height in a comparatively short time, and runs a course lasting from one or two to seven or eight years. This course is a varying one, and this variation is particularly noticeable when the patient begins to get well. The patient continues to improve for a time and then suddenly falls back, then goes forward again, and thus convalescence progresses. Complete restoration to health is possible and frequent, but the patient always has to take more care of himself than before. As a result of an attack of neurasthenia, men and women who have suffered from it are apt thereafter to lead very saint like and ascetic lives, and hence they as a rule live long. It used to be said by Dr. Beard that neurasthenics would have a long and happy old age. They pass through the valley of the shadow of death, but the experience may be a profitable, if not a pleasant one.
Neurasthenia praecox has a much more serious prognosis, especially as regards duration. This is the kind that may last seven or eight years, but recovery usually occurs. Acquired neurasthenia often lasts only from six months to two years. Local neurasthenias are also curable, e xcept the sexual type, which is an obstinate affection if it is a purely neurast henic one.
Treatment. — Naturally, the measure of leading importance in the treatment of neurasthenia is rest, and the problem of how this can be obtained is the first one to confront the physician.
In the severe types of neurasthenia, especially when it occurs in young women, the application of the "rest cure," which has been so ingeniously elaborated and perfected by Dr. Weir Mitchell, is undoubt- edly the best treatment. I do not find, however, that men submit themselves readily to this measure, and it seems to me to answer best lor those neurasthenic women who suffer also from some hysteria and who are reasonably "suggestible" patients. A modified rest cure can often be secured by making the patient stay in bed until after midday lunch or lie down for an hour after each meal, and go to bed early in the evening. Business men will often cut their business hours down one-half if they are allowed still to continue some work. Experience shows that in most cases a rest is not needed for a very long period; i. < ., not for over a few weeks. Some isolation or change may be im- perative, but prolonged and absolute rest often is not. The patient is put to bed and kept there for several days, but is soon allowed to sit up for a part of the day, and in one or two weeks may begin to use exercises. And it is often part of the program to lead the patient from abnormal passivity to more than ordinary activity. To accomplish
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this, I often substitute exercise for massage, sending a trainer to the patient, and later sending the patient to a gymnasium. Many patients do best by putting on a sweater on rising in the morning, exercising vigorously, then taking a cool bath, and then the breakfast. Later the exercise habit is inculcated, and this not strenuously, but in accordance with the constitution and tastes of the patient.
Exercise is done to strengthen the muscles, help the circulation, increase the activity of the skin, and keep the mobility of the joints. But it also should be of a kind to arouse the attention, awaken interest and give a little aesthetic pleasure. This is often a difficult task, especially with women. In its place for them we must use hot boxes the masseuse, the trainer — or domestic work.
Horseback riding is an efficient form of exercise. Many persons are greatly wedded to the exercise of walking, and it seems best to fit their needs. It is, however, a kind of exercise wmich does not take the patient's mind off himself and does not develop the respiratory functions so well as other measures do. Golfing fills in this lack, and this sport is doubtless of service in neurasthenia. The nurse is a potent feature in -the situation. Some patients are almost purely exhaustion cases, and only want rest and feeding and sleep. They are better without a special nurse. In other cases and oftener a suitable nurse who is intelligent, tactful and interesting is a great help.
Change of scene is usually very beneficial to neurasthenics, but traveling is injurious to them. They should be sent to some special place and be made to stay there. A tour along the Mediterranean coast or a trip to Europe often brings them back worse than when they went. Much the same can be said of trips to various places in the South or West. Some of the sanitaria in Germany, some of the places in the Riviera, Egypt and Bermuda, Nantucket, parts of North and South Carolina and Arizona, furnish good resorts for neurasthenics. They generally do better in the mountains, if the altitude is not too high, than they do by the seashore. Dry, windy, sunny climates like those of the Colorado plateau and parts of California and the Northwestern States are too stimulating for most cases.
Much good may be obtained at the numerous sanitaria which exist in this country. Many of these are well conducted and well supplied with all the modern appliances for treatment. It is, however, always a serious thing to send a neurasthenic to a sanitarium, for the reason that if he stays there too long he becomes contaminated with the at- mosphere of invalidism about these places and develops hypochon- driacal ideas as to his diet, his liver, his stomach, his sleeplessness, and his various sensory disturbances. In sending a patient to a sanitarium
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DISEASES OF THE NERVOUS SYSTEM.
it is a wise plan to tell him not to stay, under any consideration, longer than six weeks; usually four is better. In the summer time great benefit can be secured by camping out in the woods and living a purely outdoor life, away from all the conventionalities and restraints of civilization.
The diet of neurasthenics, in particular young neurasthenics, should be chiefly a nitrogenous one, and my directions are that the patient can eat meats, fish, eggs, green vegetables, and fruits. Milk can almost always be taken, at least for a short time. There is a certain class of lithaemic patients who do best upon milk, vegetables, and fruit, with practically no meat ; these, however, are in the minority. In general, tea and coffee, alcohol and tobacco, are to be entirely prohibited, but this is not an absolute rule. In some cases coffee is beneficial, in some tea does no harm, and in others a small amount of whiskey or dry wine and a cigar are also harmless. The physician has to determine this by the reactions and habits of the patient. Neurasthenics usually drink too little water and it is wise to prescribe a certain amount for them. Four or five glasses of water, which may be either plain or alkalinized, are to be taken daily, unless the patient has a dilated stomach when he should have a dry diet. I find no special advan- tage in the various much advertised lithia and spring waters. In dyspeptic patients the meals should be small in amount and taken at frequent intervals ; three light regular meals a day and a little food in between form a regimen which usually answers well.
Hydrotherapy, electricity, and massage are all measures which prove of service to the neurasthenic. Of these, hydrotherapy is the most useful, though its value can be overestimated. The ordinary prescriptions consist in the cold sponge bath every morning, and, if it is practicable, the use of a Charcot or a Scottish douche every other day. For women wet packs with massage are sometimes helpful, particularly in cases in which there are a great deal of nervousness and motor irritation. At night a lukewarm bath, at a temperature of 950, for ten minutes, sometimes relieves the paresthesia and sleeplessness.
Massage seems to me of not very much use in men, but it is often grateful and helpful to women, and when a great deal of rest is to be enforced it is essential to employ it for both sexes. Psycho-therapy is always to be more or less consciously applied. The subject has al- ready been discussed and if I do not say more here it is because the methods of DuBois, suggestion, hypnotism, etc., apply much more strictly to the minor psychoses. Neurasthenia proper needs attention first, for when it is relieved the psychosis may disappear.
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It seemed to me that too much stress is often laid on the importance of minor troubles of the pelvic organs in women. Curetting and sew- ing up small tears rarely do any good. Serious uterine enlargement and prolapse, displacement and real disease of the ovaries require at- tention. If, in fact, the disorder of the pelvic organ gives recognized discomfort it should be corrected.
Young men who are distressed by nocturnal emissions and who have irritable urethras or distended vesicles may require some local treatment. But in these cases there commonly is also a psychosis and there is required more moral than local therapeutics.
The eyes have received and need to receive close attention for they are often even more neurasthenic than the stomach. Attention to errors of refraction and accommodation, and to poor muscular balance is imperative. Poor eyes may keep up the neurasthenia and make the patient suffer from the discomforts of being unable to read or to do close work, as well as give rise to headaches and all kinds of cephalic paresthesia. But most often the neurasthenia remains after the best oculists have done all that is possible. Many neurasthenics continue so though they have good eyes and can spend their resting hours in continuous reading. This is my experience and my answer to the insistent claims of certain ophthalmologists that profound long continued neurasthenia may be due to poorly corrected vision. Such cases are not seen by neurologists now.
The drugs of most value are the bromides, nux vomica, mineral acids, quinine, iron, valerian, the coal-tar antineuralgics, the hypnotics, and saline and alkaline laxatives, and salicylates.
The bromide of sodium or potassium should be given in small doses; it should be kept up for a limited time and then gradually re- duced. At the same time or later the patient may be given a tonic mixture containing such drugs as the symptoms suggest. Quinine must -be given carefully, as it causes increase of nervousness in many.
Phosphoric and muriatic acids are the two mineral acids most often of use. These acids are usually better given after meals. The saccharated carbonate of iron or Blaud's pills, if given, should be given generously, i. e., in doses of thirty grains daily. The best preparations of iron are the tartrate of iron and potassium, the carbonate, the ci- trate and the tincture. I find no special benefit from the albuminate or the peptonized preparations.
The foregoing covers in a general way the measures to be used in treating neurasthenics. The physician must seek to secure the com- plete confidence and docility of his patient. He then uses measures which secure some bodily and much mental rest. He gives to him
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simple and nourishing food in no excess, and prescribes measures which restore the slowed-up or perverted metabolism.
The Sexuae Neuroses and Psychoses.
Of the above disorders the neurologist has to deal chiefly with the vicious habits of masturbation (which may, however, be also a manifestation of disease) and the sexual neuroses, spermatorrhoea and impotence.
Masturbation and Spermatorrhoea. — Masturbation is the name given to the vicious habit of artifically exciting the sexual organs. It is very common among boys and less common but present among girls and adult men and women. It is usually only a vice due origi- nally to low associations and teachings among children. In some case it is a disease or the symptom of a neurotic or insane constitution.
Etiology. — It is most common between the ages of fourteen and eighteen, but may begin earlier. Even infants and very young chil- dren sometimes masturbate, usually as the result of some local irrita- tion which leads them to rub the genitals. A tight prepuce, eczema, or worms may lead to the habit, but it is usually taught by a companion. The practice sometimes attacks schools almost like an epidemic, for in every institution a certain per cent, of the boys are sexually precocious or vicious, while the others are ignorant and innocent of the evils of the practice. Masturbation is relatively rare after twenty, but is practiced by some throughout life even up to old age.
Symptoms. — Masturbation, as ordinarily practiced, leads after a time to a feeling of malaise, mental depression, disinclination to work, study, or to enjoy one's self as before. The appetite is a little impaired, the extremities easily get cold and perspire readily. Pecu- liar numb feelings are felt in the hands and feet. There are an unnat- ural nervousness and irritability, and the power of concentrating the mind is a little weakened. The patients often have dilated pupils and hyperaesthetic skin. After a time nocturnal emissions cccur. The organs become irritable and slight excitement causes erections. These symptoms may be slightly marked and pass away in a day or two, or until another indulgence occurs.
Masturbation is sometimes done to an extraordinary extent, even daily or twice daily for a considerable time. After a while the young man begins to find that he is not well and realizes that this habit is hurting him. Then if he be sensible and of healthy constitution he stops. Others are frightened out of it by friends or by reading the terrorizing stories printed in quack advertisements and circulars. Sometimes the fright thus caused leads the unhappy youth into a
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condition of hypochondriasis, which is helped on by the occurrence of nocturnal pollutions and the nervous debility resulting from his past indiscretions. In other cases in which there is a decided neurotic history, a genuine neurasthenia of a sexual type develops and annoys the patient for years.
Masturbation rarely leads to insanity and is oftener a symptom than a cause of such disorder. It is perhaps most often associated with dementia praecox It is occasionally the cause of epilepsy. When this is the case the convulsive attacks are likely to put on a hysteroid phase and are accompanied by peculiar co-ordinated convulsions and emotional disturbance. Masturbation is the common cause of hystero-epilepsy in women.
Diagnosis. — Many victims of the masturbation habit who have come to recognize its evils and tried to stop it develop a hypochondriacal condition, and feel sure that there is something in their faces which reveals to the world their trouble. This is not the case. But there is a certain physiognomy which in a measure characterizes the mastur- bator to such an extent that an experienced observer can detect it. The pale, pasty complexion, moist, furtive eye, dilated pupil, listless, restless, and depressed manner, the wet, flabby palms, and hyperaes- thetic skin, all help to tell the story. Locally, the penis is often reddened and more or less turgid, the scrotum relaxed, and a varicocele may be present. Examination of the urine may reveal spermatozoa. The urine also is almost always of rather low specific gravity, and contains a great excess of phosphates, both earthy and alkaline.
Treatment. — The patient must be told plainly the necessity of stopping the practice. He must be impressed, but not terrorized. He should be kept out of doors at vigorous physical exercise, for seden- tary and solitary work is always bad for such cases. He should be made to take cold-water baths and should sleep on a hard bed with light covering. He had better sleep with some one whose presence may exercise a controlling influence. He should not eat heartily at night, never just before going to bed. And what is still more im- portant, he should not drink before going to bed. Sometimes it is well to have him wakened at an early hour in the morning, when he should empty his bladder; for emissions occur often early in the morning and are promoted by the irritation of a full bladder.
Locally, cold-steel sounds may be introduced and allowed to remain for ten minutes three or more times a week, or the psychophor or Ultz- mann's short catheter may be used. In bad cases with a great deal of prostatic irritation, local applications of nitrate of silver are needed. Internally, a mixture of tinct. opii, tinct. camph., and tinct.
600 DISEASES OF THE NERVOUS SYSTEM.
lupulin may be given at night, the ingredients being somewhat varied in amount to suit the case. Bromides, chloral, atropine, and salix nigra are also drugs which are often useful. The mechanical measures which have been devised for preventing erections, such as rings with sharp teeth, are rarely needed and rarely useful. They may even do harm by directing the mind to the affected function.
I do not believe it right for the physician to prescribe fornication. It is not safe nor curative, apart from the moral aspect of the matter. It has always struck me also as pretty small business for a man pur- posely to select a wife to relieve him of the results of a weak will and vicious sensual indulgence, If marraige comes in the natural course of events, as it often does, so much the better. But to select a wife as a remedial agent for masturbation is unjust to the woman and a confession of moral and mental feebleness. Man is distinguished from the brute by his self-control. Let him bear this fact in mind and raise himself above the animals by a determined effort of the will. Pure thoughts and chaste associations, vigorous physical exercise, and a resolute effort to act a manly part will always be successful.
Traumatic Nervous Affections (Traumatic Neuroses and Psychoses, Spinae Concussion).
The present tendency of neurology is to deny the existence of any special nervous affection produced by trauma or shock. There may follow from these causes:
1. Surgical injuries.
2. Neurasthenic and morbid psychic states.
3. Hysterical states.
4. Hemorrhagic, inflammatory, and degenerative diseases.
5. Combinations of the foregoing.
These troubles may follow not only railway but other injuries, but are especially liable to follow those associated with intense fright.
2. Traumatic neurasthenia, or "traumatic neurosis," "railway spine," does not differ from forms of neurasthenia produced by other causes, except that with it there may be certain sprains and surgical troubles and some morbid mental condition. Its special symptoms are described under the head of neurasthenia.
3. Traumatic hysteria is a rare affection in this country. It does not differ from hysteria produced by other causes, except for its sudden onset and occasional surgical complications. It is usually a hysteria major and has the characteristic stigmata of that type. In this city
EXOPHTHALMIC GOITRE.
60 1
electrical injuries and frights have produced some classical cases of hysteria major.
4. There is considerable evidence that in some rare cases trau- matism may produce minute multiple hemorrhages throughout the nervous centres. In such cases there are usually neurasthenic or hysterical symptoms and in addition symptoms of organic disease.
Massive hemorrhages and serious mechanical injury of the nervous centres may be also produced by injury.
Finally, it is a well-known fact that traumatisms may excite in the predisposed locomotor ataxia (in the infected), inebriety, insanity or may lead to the development of a cerebral tumor.
It is the mental impression, the shock, much more than the physical injury, which produces the functional neurosis or psychosis.
The symptoms may appear soon after the accident, or, after a period of relative health lasting some weeks the neurosis gradually develops.
The most important practical point in connection with the subject is the diagnosis and the elimination of malingering. This is addition- ally difficult for the reason that the hopes and anxieties depending upon litigation tend to cause introspection, exaggeration of symptoms and unconscious bias even in the most honest. The opinion among American neurologists tends to favor the reality of traumatic neuroses. While malingering is not rare, yet if the patient has really a traumatic neurasthenia or hysteria the disease may not be a trifling one. Careful research, however, often tends to elicit the fact that previous to the injury the patient was an alcoholic, syphilitic, or neuro- tic, and perhaps had already the beginning of his alleged traumatic disorder. In no part of clinical medicine is a careful and searching examination and weighing of symptoms more urgently called for. The methods of carrying out such examinations are given elsewhere. Special methods for testing anaesthesia are sometimes needed. The two sides of the body should be tested simultaneously with concealed needles, beginning on the trunk, or the faradic current with a double- pointed electrode may be used. There are few patients who can successfully deceive in an examination covering all the special senses.
The treatment of these neuroses calls for no special notice here.
Exophthalmic Goitre (Basedow's Disease, Graves's Disease).
Exophthalmic goitre is a chronic glandular neurosis character- ized by rapid heart beat, enlargement of the thyroid gland, protru- sion of the eye-balls, tremor, and various neurasthenic and vasomotor symptoms.
602
DISEASES OF THE NERVOUS SYSTEM.
Etiology. — The disease occurs much oftener in women than men (four to one). It is a disease of early adult life, occurring chiefly between fifteen and thirty-five, very rarely in childhood, and never after fifty.* It is apparently more common in the Anglo-Saxon race, but is not very frequent in America, at least in the Eastern States. I am informed that it is rather common in the Northern Cen- tral States. There is very rarely any direct inheritance of the disease, but the family is often a neuropathic one. As a rule, the patient is of a neurotic temperament. Anaemia and debilitating diseases promote its development. Goitre and heart disease do not seem to predis- pose to the real trouble, but a goiter may lead to a symptomatic Base- dow's disease characterized mainly by cardiac disturbances and per- haps some cervical sympathetic symptoms. The most frequent ex- citing causes are powerful depressing emotions and severe physical exertion. Rarer causes are injuries and infectious diseases, such as measles, scarlet fever, typhoid fever, and pneumonia. It sometimes develops after such infections without any previous nervous strain or shock, unless it be supposed that in a severe infection there is always a certain degree of shock. I have met no cases following directly upon traumatism.
Symptoms. — -The disease usually begins gradually and the first symptom is in most cases rapid heart beat and palpitations, accom- panied with some nervousness and tremor. The next symptom is en- largement of the thyroid gland, and at about the same time the eye- balls begin to protrude. This order of development does not always take place, and occasionally one of the four principal symptoms is not present. The disease is usually one or two years in developing, the heart symptoms being those which continue by themselves longest. With the symptoms mentioned there occur many minor troubles which are more or less characteristic. The patient is usually very nervous and irritable ; a distresssing insomnia may be present. There is almost uniformly a fine tremor (eight to nine per second) of the hands less marked in the lower limbs and not present in the face or tongue. The reflexes are exaggerated. There is a tendency at times in walk- ing for the knees suddenly to give way. The patient rarely has neural- gias, but does have burning or feverish sensations and headaches. The skin is rather reddened and the patient sweats profusely. Pig- mentation and vitiligo are sometimes seen, and urticaria may develop. The electrical resistance of the body is much diminished, being 800
*Among 33 cases at the New York Post-Graduate Clinic (Fiske-Bryson) there were 8 males, 25 females. Ages: thirteen to twenty, 8; twenty-one to thirty, 8; thirty-one to forty, 6; forty-one to fifty, 5; fifty-one to sixty, 1.
EXOPHTHALMIC GOITRE.
to 1,500 ohms instead of 2,000 to 3,000. There is sometimes a dermo- graphic skin, as in other neurasthenic states. The respiratory func- tion is weakened and chest expansion often falls below one inch (Fiske-Bryson). Attacks of a persistent watery diarrhoea occur. Anaemia is usually present. There is occasionally polyuria, more rarely glycosuria. A study of the urine shows an increase in general metabolic activity. The menses are irregular and amenorrhoea often exists. A slight rise in temperature may occur.
The major symptoms of the disease are: Tachycardia and pulsating arteries. Goitre.
Exophthalmus. Tremor.
The minor symptoms are : Nervousness. Sweating. Insomnia.
Lessened electrical resistance. Subjective sensations of heat. Diarrhoea. Polyuria.
Symptoms in Detail. — Tachycardia is the most constant single symptom. The pulse beats from 100 to 120 per minute usually, but may rise to 160 or even 200. Its rhythm is usually steady; but palpitations occur easily, even without exciting cause. The patient may wake up at night with distressing attacks, something like those of angina pectoris, but the intense pain and sense of im- pending death are usually absent. The heart is dilated and a sys- tolic murmur is often heard at the base propagated along the ar- teries. Real organic disease, however, is rare. The arteries are dilated and soft. They pulsate strongly, particularly the carotids. A thrill is sometimes felt over the heart and always over the goitre. The arterial tension is normal or low; rarely it is high.
The thyroid gland is usually enlarged symmetrically; later in the disease the isthmus is affected and the three lobes of the gland stand out prominently (Fig. 239). If only part of the gland is involved it is oftenest the right lobe. A thrill is felt over it and a systolic murmur can be heard.
The bulging of the eyes or exophthalmus is usually bilateral and even. If one eye is alone or more affected it is the right. The ex- ophthalmus varies much in degree. It is not usually very great, but
604
DISEASES OF THE NERVOUS SYSTEM.
may be so excessive as to prevent closing of the lids and to expose the insertions of .the recti. The eyeball may be slightly enlarged (one- tenth). The exophthalmos is due to the overaction of certain muscu- lar bands which lie in the orbit ; they surround the globe of the eye like a sheath and are attached to the anterior border of the orbit. These fibres are innervated by the cervical sympathetic and in un- usual irritation they cause the eye to protrude. In Basedow's disease these muscular fibres become hypertrophied somewhat. The orbital space, as the eye is pulled forward, is gradually rilled by fat and connect- ive tissue. Hence section of the cervical sympathetic does not lead
Fig. 239. — Exophthalmic goitre.
to more than a slight relief of the exophthalmus. The pupils are nor- mal and vision is not impaired, though myopia occasionally occurs. The fundus and visual field are normal. Paralysis of some of the eye- muscles is a rare complication. Weakness of the internal recti and exophoria are frequent. The lids show certain peculiarities. One of these, known as Von Graefe's symptom, consists in the inability of the lid to follow the downward movement of the eye-ball. When the patient is to'd to follow the movement of the finger vertically down- ward the eye -ball moves steadily, but the lid catches, as it were, and re- fuses to follow or does so in a jerky manner. Another symptom, known as Stellwag's symptom, is a considerable retraction of the lids, especially the upper one. Both this and Von Graefe's symptom are due to a common tendency of the lids to retract — due perhaps to over-
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605
action of the muscle of Miiller. A tremor of the lids sometimes occurs.
Course. — The disease progresses slowly. After a year or two it often becomes stationary for a long time. Cases of gradual spon- taneous recovery occur. The natural duration of most recoverable cases is two or four years. In those which do not recover, the disease lasts five, ten, or more years. Eventually the patient emaciates, the heart becomes weaker, albuminuria and dropsy appear, diarrhoea sets in, and the patient dies of exhaustion or is carried off by phthisis or some intercurrent disease. Other cases, having improved up to a cer- tain point, remain in this state for years.
Complications. — Mental derangement occasionally occurs in the later stages of the disease. Hysterical crises, epileptic attacks, choreic movements, paralysis of the ocular muscles, muscular atrophy, paraly- sis agitans, Addison's disease, diabetes, locomotor ataxia, and local oedema have all been observed. With the exception of hysterical at- tacks, these complications are rare.
Abortive Forms. — This name is applied to cases in which only a part of the distinctive symptoms develop. Tachycardia always exists ; with it are tremor and moist skin, lessened electrical resistance and nervousness. Or tachycardia and goitre may alone be present.
Pathological Anatomy. — Post-mortem examination of the thyroid gland shows that it undergoes a true hypertrophy with increase of vascu- larity and of the glandular structure. The rational inference is that in life there is an increase in the secretions from this structure. After the hypertrophy has reached a certain stage, the glandular epithelium degenerates and breaks down, forming large acini filled with the colloid secretion. In the nerve-centres the changes which have been found are small hemorrhages in the medulla and degenerated nerve- cells. In one case of about a year's standing, I found a very marked pigmentation and vacuolization of the cells of the vagus and glosso- pharyngeal nuclei. In another case of six months' standing, no marked changes could be seen in these areas, but there was a spot of softening at the junction of the pons and the cerebral peduncle. This was ante-mortem and had led to crossed paralysis just a few days before death. In other cases congestion and small hemorrhages in the medulla have been found. In still other cases no lesions have been found, so that the actual anatomical change in the nervous centres cannot be said to be established. The heart is dilated and enlarged ; endocarditis is sometimes present, oftener not; the arteries are dilated.
Pathology. — Some writers now consider this disease primarily one
6o6
DISEASES OF THE NERVOUS SYSTEM.
due to a disordered function of the thyroid gland. From my own ob- servation I am led to the conclusion that Basedow's disease is primarily one of nervous origin, but that the thyroid disease leads to excessive secretion, causing the principal symptoms. The nervous tissues re- quire for their proper nourishment and natural functioning a certain supply of the secretion of the thyroid gland. If this is excessive, there is a state of nervousness and erethism, such as we see in Basedow's disease, and if it is diminished there develops a hebetude and depres- sion of nerve function, such as we find in myxoedema. Under the in- fluence of shock and powerful emotion or prolonged strain, there is a certain powerful demand upon the product of the thyroid gland by the nerve-centres. The thyroid juice is thrown out in great amount, and in persons of unstable organism a morbid impetus is given to the activity of the gland. It continues to grow and throw out its juice; the overexerted nervous system makes continually more demand upon it, thus acting in a vicious circle. So, while the symptoms of the dis- ease, the nervousness, the insomnia, and the vasomotor disturbances are due to the hypersecretion of thyroid, the primary disturbance is one in the nerve-centres. If these can be kept quiet long enough, the demand on their part for this excess of thyroid juice gradually ceases and the patient gets well. This is the rationale of the prolonged rest and the use of the bromides, phosphates and tonics, which are the agents that do most good in the treatment of the disease.
As to the special cause of the different symptoms, it may be as- sumed that the original enlargement of the thyroid is a vasomotor paresis of its vessels. It is a kind of erection of the organ, due to the sudden demand put upon it by emotional strain and exhausting work. The cause of the exophthalmus has already been explained. The deposit of retrobulbar fat is a secondary phenomenon. The rapid heart beat is probably due to impairment of the inhibitory fibres of the spinal accessory.
Prognosis. — About one-fifth of the cases get well or practically well. Probably over half the cases, if they can be properly treated, reach a fairly comfortable condition of improvement. The cases in which symptoms come on quickly have the most favorable prognosis. In those with marked exophthalmus and goitre the prognosis is not so good. The duration of the disease in recovering cases is from two to eight years.
Diagnosis. — The disease can be distinguished by the persistent tachycardia, with goitre or exophthalmus, and in its early stage by the tachycardia with tremor, moist skin, sensations of heat, nervous- ness, insomnia, lessened respiratory expansion, and electrical resist-
EXOPHTHALMIC GOITRE.
ance. A symptomatic Graves' disease may sometimes be caused by a goitre pressing on the vagus or sympathetic and causing irregular heart beat and perhaps exophthalmus. In these cases the history of a long-standing goitre exists, the heart's action is irregular, the exoph- thalmus is usually partial and one-sided. In abortive forms it is neces- sary to have tachycardia and at least one other of the four major symptoms to make a diagnosis.
Treatment. — Rest is the most important single thing. The patient should be put to bed or kept on the back for one or more months. Freedom from excitement and worry must be enjoined. No special diet is needed, nor do climatic influences or baths or mineral waters have much effect. Some cases are said to be improved, however, by removal to heights of one to three thousand feet. In most cases a sea voyage is the better change if one is made.
The drugs used are numerous. The most efficient are the phosphate of soda and the glycerophosphates, tincture of strophanthus, in doses of fifteen to forty drops daily; iodide of potassium or the syrup of hy- driodic acid ; arsenic and bromide of potassium used together; quinine, mineral acids, and iron, used together. Dilute phosphoric acid is often very useful. Other remedies are tincture of aconite in rriv. or aconitia in gr. 2-^0 doses, tincture of belladonna increased to the limit of tolerance, tincture of cactus grandiflorus in nix. to xx. and tincture of veratrum viride, n^x. to xxx. dose, and the picrate of ammonium, gr. i. to ij. t.i.d.
Of these drugs, the phosphates, strophanthus, aconite, the iodides, bromides, and iron have served me best. In certain very mild cases with small goitres, thyroid extract does good, but as a rule it is harmful.
Beebe and Rogers have obtained a specific antiserum which, in acute and serious cases where there is great thyroid toxaemia, acts most successfully. In the more chronic and quiescent cases it is not so efficient. A serum has been obtained also from thyroidectomized goats which has given some good results, but it has accomplished but little in any experience.
Electricity possesses some utility. It should be given, if possible, two or three times daily in the form of galvanism and in doses of two to six milliamperes for ten minutes. The technic is as follows: positive pole on back of neck, negative drawn along course of vagi in the neck; each side two minutes. Same with positive pole placed subaurally one minute ; negative pole over thyroid two minutes ; negative over cardiac region one minute, positive pole over eyes, negative over thy- roid one minute, two milliamperes. The faradic current may be used for general tonic effects or combined locally with the galvanic. The
6o8
DISEASES OF THE NERVOUS SYSTEM.
patient should lie down during treatment and remain quiet for an hour later.
For the palpitations, sulphate of sparteine or strophanthus with Hoffmann's anodyne maybe used. The ice bag placed over the heart and neck is helpful and may be used systematically. Tonic hydro- therapy is often useful, but should be carefully employed. Kocher, Mayo and other surgeons strongly advocate operation under local anaesthesia and partial removal of the thyroid; and in their hands this operation has been most successful.* Treatment of the nose has been said to cause disappearance of symptoms, but its utility is very doubtful. Respiratory exercises by which the patient is taught to increase his chest expansion do some good. Mild compression of the lids at night seems to help the exophthalmus, and slight and steady compression of the thyroid gland sometimes reduces its size a little.
*S. Landstrom's statistics of cases of himself, Kocher, Mayo, Riedel give cure or im- provement in about 80 per cent. ; death in about 10 per cent. Landstrom in 38 cases reports cure in 48.7 per cent., improvement in 17.1 per cent., failure in 26.8 per cent., death in 7.4 per cent. Mayo's figures on cure or improvement 87.8 per cent., death, 12.2 per cent.
CHAPTER XXV.
PROFESSIONAL NEUROSES, OCCUPATION NEUROSES (WRITERS' CRAMP AND ALLIED AFFECTIONS).
Writers' cramp is a chronic psycho-neurosis characterized by spasmodic, tremulous, inco-ordinate or paralytic disturbance when the act of writing is attempted, associated, with feelings of fatigue and pain.
Etiology. — It is a disease of recent times, and has been par- ticularly noted since the introduction of steel pens, about the year 1820. A neuropathic constitution is often present, and somet mes there is a hereditary history. Men are much more subject to the disease than women. The most susceptible age is between twenty- five and forty. It rarely occurs after fifty or before twenty. Clerks and professional writers are naturally much more subject to the disease. Excessive worry, intemperance, and all debilitating influences pre- dispose to it. The chief exciting cause is excessive writing. But this is not all. The writing that is done under strain or a desire to finish a set task is the harmful thing. The style of writing is also an impor- tant factor. Writing done in a cramped posture with movements of the finger alone or with the little finger or wrist resting on the table is most injurious. Free-hand writing done from the shoulder accord- ing to the American system is least harmful. Shaded or heavy writing with sharp steel pens is also productive of harm. Copying is much more harmful than composing. Authors seldom have writer's cramp. Albuminuria, lead poisoning, exposure to wet and cold, and local injuries are sometimes exciting causes.
Symptoms. — Writers' cramp veiy rarely attacks a person suddenly. The patient first notices a certain amount of stiffness occurring at times in the fingers, or the pen is carried with some uncertainty and jerky movements are made. He feels a sensation of fatigue in the hand and arm, and this may amount to an actual tired pain. The first symptoms may last for months or even years. The hand is rested as much as possible; new pens or penholders and new modes of holding it are tried. Often the patient, fearing the onset of the cramp, and as its result loss of employment, becomes anxious, worried, and mentally depressed. Sometimes the trouble is worse 39 609
6lO DISEASES OF THE NERVOUS SYSTEM.
when beginning a daily task, and it gradually wears off in a few hours. At other times exactly the reverse is the case. When the disease has reached its highest stage, writing becomes almost or entirely impossible. The moment the pen is taken in the hand and an attempt at using it made, spasmodic contractions of some of the fingers, or even of the arm, occur, the pen flies in any direction, and it is impossible to control or co-ordinate the movements. The rule is that, although writing cannot be done, all other complex movements are performed as well as ever. Thus the sufferer from writers' cramp may be able to play the piano, or paint, or thread a needle, or use the hand in any complex movements. This limitation, however, is not always present. Teleg- raphers, who use to some extent the same muscles as in writing, and who also often have to do a great deal of writing, are liable to suffer from both writers' and telegraphers' cramp at the same time. No evidences of actual paralysis are present in the affected muscles, and there is rarely anaesthesia, but the arm aches and is sometimes tender. Sensations of numbness and prickling are present; in rare cases vasomotor disturbances are observed ; associated muscular movements of the other arm or of the neck or face sometimes occur. The hand may tremble on attempting to write or fall almost paralyzed when the pen is taken.
The var.ous symptoms occur with different degrees of promi- nence, so that the disease has been classed under the heads of (i) the spastic, (2) the neuralgic or sensory, (3) the tremulous, and (4) the paralytic forms. These forms are, however, often more or less mixed.
1. The spastic form is undoubtedly the most common, and it has given to the disease its name. Cramp of some muscle or mus- cles is present in over half of the cases. The muscles of the thumb and first three fingers are oftenest affected, and in some cases the flexors, in some the extensors, are chiefly involved. In telegraphers' cramp it is the extensors, but in writers' cramp the flexors, that are mainly attacked. The thumb or forefinger or the little finger alone may suffer from the spasms. The pronators and supinators are quite often involved. The spasm is usually a tonic one. With the spasm there is also inco-ordination so far as writing movements are concerned, and this fact is quite as important in producing the bad writing as the spasm.
2. The neuralgic form resembles the spastic plus sensations of fatigue and pain, which are quite severe and are brought on by writing. There may be tenderness along the arm also.
3. The tremulous type, though rare, is very characteristic when
PROFESSIONAL NEUROSES, OCCUPATION NEUROSES. 6ll
present. The patient when attempting to write develops a tremu- lous movement of his hand and arm. This ceases when his attempts to write cease. The tremor usually affects most the fingers used in pen prehension, but it also spreads to the forearm and may even in- volve the entire extremity. An oscillatory or lateral tremor, due to involvement of the pronators and supinators, has been observed. The tremor is of the character known as "intention tremor," such as is observed in disseminated sclerosis. It is shorter in range and more rapid than the tremor of that disease.
4. The paralytic form, or that type in which muscular feebleness is the dominant symptom, is said to be rare by Gowers, and this accords with my experience. German writers speak of it as common. In the typical paralytic form the patient, as soon as he begins to write, feels an overpowering sense of weakness and fatigue in the fingers and arm. The fingers themselves loosen their grip and the pen may drop from the hand. Powerful impulses of the will and change in the mode of hold- ing the pen enable the sufferer to continue, but the arm aches and finally is absolutely painful, and weakness and fatigue compel the writer to desist. Sometimes the paretic condition is succeeded by the spastic. Many of the cases of paralytic writers' cramp are not true examples of the neurosis, but are rather cases of neuritis of a rheumatic or other type.
General Symptoms. — Writers' cramp is essentially a motor neurosis, and its leading symptom is the impairment of a motor function. Other symptoms, however, both general and local, are always associated with it. These are mainly (1) psychical and (2) sensory, more rarely (3) vasomotor and (4) trophic.
1. Psychical symptoms. The patient is often nervous, emo- tional, and mentally depressed at times. He suffers from insomnia and vertigo. Patients are generally unwilling to admit that there is any other trouble than the local one, and only careful examination may bring evidence of constitutional trouble. There are cases of purely mental writer's cramp.
2. Sensory troubles. These consist of pains, sense of fatigue, feel- ings of numbness, prickling, pressure, weight, tension, constriction, etc. Hyperesthesia, and more rarely anaesthesia, are also observed. The most common sensory symptom is that of aching and fatigue, and this is usually confined to the arm, and oftenest runs along the course of the radial and median nerves. The cervical vertebrae may be tender and sometimes patients have a headache in the parietal region of the side opposite the affected arm.
3. Vasomotor, trophic, and secretory disturbances. The condi-
6l2
DISEASES OF THE NERVOUS SYSTEM.
tion known as digiti mortui has been observed, coming on paroxys- mally. It is a symptom which the general neurasthenic state helps to produce. When the nerves are involved decided vascular changes may occur, such as passive congestion of the hand and arm, with swelling and turgescence of the ringers, and a sensation of throbbing. In bad cases the fingers will look as if they had chilblains. Local sweating, dryness of the skin, and cracking of the nails, all are condi- tions which may follow impairment of writing power from neuritic causes.
Electrical Reactions. — -The results of observations upon the elec- trical reactions of the affected parts are somewhat contradictory. Ordinary tests will, as a rule, reveal very little change. Sometimes there is a quantitative increase, sometimes a decrease, of irritability to both forms of current. The increase occurs in the earlier stages, the decrease in the later. An increase or modification of electro- muscular sensibility has been noted. The electrical examinations, therefore, are only of value in excluding a neuritis or possibly in deter- mining the stage of the disease.
Pathology. — -Neuritis is undoubtedly present in some forms of writers' cramp, so called. It is not present, however, so far as external tests go, in the typical neurosis. Nor are there any post- mortem observations throwing light on the anatomy of the disease. We must believe, therefore that is it a neurosis having no appre- ciable anatomical basis.
The act of writing is a very complicated one, calling into play numerous sets of delicately innervated muscles. These muscles are employed: i, in pen prehension; 2, in pen movement; 3, in holding the arm and wrist tense.
1. The muscles employed in pen prehension are the two outer lumbricales, two outer interossei, the adductor muscles of the thumb, the flexor longus pollicis ; to some extent the deep and superficial, short and long flexors, and the extensors of the thumb. These are supplied mostly by the ulnar (interossei, adductor pollicis, inner heads of deep flexor of fingers, and inner head of short flexor of thumb). The rest of the muscles are supplied by the median.
2. In moving the pen, if the writing is done mainly by finger and not by arm movements, the muscles brought into play are the flexor longus pollicis, extensor secundi internodii pollicis, flexor profundus digitorum, extensor communis digitorum, and to some extent the interossei. The musculo-spiral and ulnar nerves innervate these groups about equally. In moving the pen by the "American" or free-hand method there is a very slight play of the above muscles, while most
PROFESSIONAL NEUROSES, OCCUPATION NEUROSES. 613
of the pen movements is done by the muscles of the upper arm and shoulder, viz., the teres major, pectorales, latissimus dorsi, biceps, and triceps.
The spinal centres for these muscles are distributed along the fifth, sixth, and seventh cervical segments of the cord. The cells are larger and situated more superficially in the anterior gray horns.
3. Besides these movements involved in pen prehension and in the letter making, a certain amount of muscular tension is exercised in "posing" the forearm and hand and steadying the wrist. The biceps and triceps, the supinators and the flexors and extensors of the hand are here brought into play.
From the foregoing it will be seen that the muscles of pen prehen- sion are most used in all but the free-hand style of writing, since the same groups have a double duty, that of clasping and of moving the instrument.
While writer's cramp is often complicated with some neurotic disturbance leading to symptoms in the affected arm of pain, paraly- sis, tenderness over nerves, vasomotor disturbances, etc., there can be no doubt that the lesion in typical cases is central, and involves the psycho-reflex centres and indirect motor and sensory paths. Little more can be said of the pathology than that it is "bad habit" or an "exhaustion neurosis," allied to the spasmodic tics and especially to stuttering. The same is true of all the other forms of occupation neuroses, and nothing need be said upon this point regarding them when they come to be considered.
The diagnosis of well-marked cases of writers' cramp presents no difficulty. In the earlier stages, however, it may be confounded wit1^ a large number of disorders, viz., posthemiplegic chorea, hemiataxia, paralysis agitans, progressive muscular atrophy, progressive locomotor ataxia, various forms of tremor, lead paralysis, rheumatoid arthritis, neuritis, cerebral and nerve tumors, and tenosynovitis.
In many of these cases it is only necessary to bear in mind the his- tory of the disease in order at once to reach a safe conclusion as to its nature.
If there is a great deal of pain in the arm, with tenderness along the course of the nerves; if there is decided change in the electrical reactions; if there are sensations of tingling, numbness, etc.; and if the patient shows an absolute loss of power in the various groups of muscles, with some incapacity for doing other acts besides the one with which he is specially concerned, then the trouble is undoubtedly peripheral and due largely to an underlying neuritis. The prognosis in these cases is much more favorable. If, on the other hand, the dis-
614
DISEASES OF THE NERVOUS SYSTEM.
order comes on in persons who have done an excessive amount of writing ; if it is associated with nerve strain ; if the electrical reactions are but slightly changed, the sensory symptoms slight and the motor inco-ordination is marked, limited to the special class of work, and not accompanied with absolute paresis, the disorder is central and needs both a different treatment and prognosis. It is these cases that form writers' cramp proper, although no doubt neuritic and central forms are associated, or the former may run into the latter.
Course and Duration. — Writers' cramp is a chronic disease. It begins insidiously and attacks one group of muscles after another as each is brought into play by new methods of writing. If the left hand is used, that, too, is liable to become affected. The course varies, however; for a time progress may be arrested or improvement set in. When the disease becomes well established it will most often last a lifetime.
Prognosis. — The prognosis is unfavorable, yet not so much so as was once thought. Undoubted cases of complete recovery have been reported, even under unfavorable conditions. The prognosis is much more favorable if the patient begins treatment early and before marked spastic symptoms are present. It is more favorable in the neuralgic forms. Some patients who suffer from a mild form of the trouble manage, by the help of instruments or special pens, to do their work for years. The more acute the disease and the more evidently peripheral and neuritic its origin, the better the prognosis. In over one-fourth of the cases, patients who use their sound arm will not be affected in it.
The facts stated regarding the cause, physiology, and general symptomatology of writers' cramp apply to the other forms of occu- pation neuroses. A few special details , however, will be given re- garding these. The most common and important are musicians' cramp and telegraphers' cramp.
Musicians' Cramp. — Under this head we include pianists' cramp, violinists' cramp, flutists' cramp, and the cramp of clarionet players.
Pianists' cramp occurs usually in young women who are studying to become professionals or who are especially hard working and am- bitious. The absurd "Stuttgart method" of teaching the piano, in which the motions are confined as much as possible to the fingers, pre- disposes especially to this disease. The symptoms are those of fatigue, pain, and weakness. The pains are of an aching character. They are felt in the forearm especially, but extend up the arm and between the shoulders. Spasmodic symptoms are rare. The right hand is oftener affected, but both hands eventually become involved.
Violinists' cramp may attack the right hand which holds the bow
PROFESSIONAL NEUROSES, OCCUPATION NEUROSES. 615
or the left hand which fingers the strings, but more often the left hand is affected.
Clarionet players sometimes suffer from cramp of the tongue and of the laryngeal muscles.
Flute players suffer not very infrequently from slight laryngeal spasms. A similar trouble affects elocutionists. The term mogo- phonia is applied to this type.
Telegraphers' cramp affects especially those operators using the Morse system, which is still the one most widely in vogue. Contrary to the opinions of previous writers, Dr. Lewis believes that this neuro- sis is not a rare one and is destined to become more frequent. In this city the cramp is not rare, the proportion being about one in every two hundred. The technical name among operators for the cramp is 'loss of the grip." In telegraphing, the extensors of the wrist and fingers are called most into play, and hence are most and earliest affected. The symptoms come on very slowly, the thumb and index-finger being first affected. The victim finds that he cannot depress the key on ac- count of spasm in these muscles, and he finds most difficulty in making
the dot characters, such as h (....), or p ( ), or z (. . . .).
When the flexors are most affected, the key is depressed with undue force and a dash is made instead of a dot. Sufferers from the "loss of grip" generally have writers' cramp also. While spasm is usually pres- ent, the disease may show itself simply in pain, paresis, and incapacity to co-ordinate the muscles.
In sewing- spasm, which affects tailors, seamstresses, and shoe- makers, clonic and tonic spasms attack the muscles of the hands on attempting to use them in the regular work. Tailors who sit cross- legged sometimes suffer from a peculiar spasm on assuming this posi- tion. It is possible, however, that these are cases of tetany, and not the functional neurosis under consideration.
Smiths' spasm, or "hephastic hemiplegia" appears to have been observed only by Duchenne and Dr. Frank Smith. It occurs in per- sons engaged in pen-blade manufacturing, saw straightening, razor- blade striking, scissors making, file forging, etc. In doing this work they have to use a light or heavy hammer, with which strokes are de- livered very rapidly and carefully. After a time spasmodic move- ments occur in the arm used, and the arm falls powerless. As in the cases reported, there are generally hemiplegic symptoms, and also neuralgias, vertigo, and other cerebral troubles, the disease cannot be a pure "occupation" neurosis.
Drivers' spasm has been observed in veterinary surgeons by Dr. Samuel Wilkes.
Milkers' spasm is an extremely rare affection, which was first described by Basedow and seems to occur in milkmaids, never in milkmen.
Cigarmakers' cramp is very rare.
Watchmakers' cramp and photographers' cramp are also to be re- garded merely as pathological curiosities.
Ballet-Dancers' Cramp. — Under this name certain painful and paralytic troubles occurring in ballet dancers, especially premieres
1
6i6
DISEASES OF THE NERVOUS SYSTEM.
danseuses, have been described by Schultz, Onimus, and Kraussold. It does not appear that the trouble is really a co-ordinative functional one, but is rather neuralgic or the result of local strain upon the parts.
The list of professional neuroses is made to include, besides those above given, cramps and co-ordinative troubles affecting artificial- flower makers, billiard players, dentists, hide dressers, electrical instrument makers, stampers, turners, sewing-machine girls, money counters, weavers, painters, and pedestrians.
Prophylaxis and Treatment. — The introduction of typewriters, gold pens, and improved penholders has prevented somewhat the in- crease of writers' cramp. Stenographers rarely have it unless they write in long hand. Persons who have to write a great deal should use large cork or rubber penholders and gold or quill pens with smooth paper. The best style of writing is that done from the shoulder, but this is a method that bookkeepers and those who have to keep accounts cannot easily adopt. The vertical system of writing which is now being widely taught is to be preferred. Many nervous persons have a bad habit of gripping the pen very tightly and pressing down on the paper with excessive force. Fatigue soon results and painful sensations develop in the arm. Proper attention should be paid to the position of the paper written upon, the height of the desk, the light, and the sleeves of the coat or dress. The paper should be laid at an oblique angle to the edge of the desk, and not at a right angle as many writing-teachers are accustomed to direct. As some cases of "cramp " are undoubtedly cerebral, it is very unwise to attempt any extraor- dinary exploits in writing or to work with the ambition to put the writing-capacity to the utmost test. Cramp is often dated from days when such extra work is done.
When the cramp is fully developed, the most essential thing is rest, and it is generally best to advise the patient to change his occupa- tion at once. Some rest, however, may be secured by getting a new form of penholder, holding the pen in a different way, using the un- affected arm, or using some form of mechanical appliance. The mechanical appliances are splints, rubber bands around the wrist, and various instruments contrived to prevent spasm and throw the work of writing on new and larger groups of muscles.
Instruments for writers' cramp are very numerous. Those that are of some value are Mathieu's, Nussbaum's , and some modification of Cazenave's (see Figs. 240, 241). All the various instruments have been of service, or have even been curative in some special cases, but not too much must be expected of them. As a rule, they are only palliative. A cheap instrument that may prove satisfactory is that of Mathieu.
PROFESSIONAL NEUROSES, OCCUPATION NEUROSES. 617
In the medical treatment of writers' cramp the important agents are massage, pedagogics, and rest.
Electricity has some value in the treatment of occupation neuroses. The high-tension faradic current with long coil has done good service in some of my cases. The galvanic current has been helpful also. It should be given daily. The anode is placed over the cervical spine and the cathode over the various muscular groups affected. A stabile current of five to ten milliamperes for from ten to fifteen minutes is given. It should be combined with educational methods.
Fig. 240. — Mathieu's instrument Fig. 241. — Nussbaum's instrument,
for writers' cramp.
Lotions containing muriate of ammonium, liniments, hot and cold douches, the cautery, all have been recommended in professional neu- roses. Tenotomy was once employed, but has been abandoned. Very little can be expected of drugs. The most trustworthy are atropine, strychnine, cannabis indica, the iodides and bromides, and cod-liver oil. It should be remembered that sometimes the disease is almost purely cerebral, and then an antineurasthenic treatment is called for. But in other cases, when the disorder is largely peripheral, the usual treatment for a low grade of myoneuritis must be employed.
CHAPTER XXVI.
PARALYSIS AGITANS (SHAKING PALSY, PARKINSON'S
DISEASE).
Paralysis agitans is a chronic progressive disease, characterized by tremor, muscular rigidity and weakness, and by a peculiar attitude and gait, together with sensations of heat, pain, and restlessness.
Etiology. — The disease occurs more often among men than women in the proportion almost exactly of 2 to i (155 males 82 females). More than two-thirds the cases occur between the ages of 45 and 60, and the serious decade is the fifth. Practically no cases occur before 35 and very few before forty.
In my 127 personal cases (90 being in private patients) the age of development of the disease was :
20-30, 2 30-40, 8 40-50, 40 50-60, 46 60-70, 24
70-80, 7_
127
The disease is by no means rare in this country and affects the native stock seriously. Among 90 private cases 47 were of distinctly American stock, 13 occured among Hebrews, and 8 among Germans, 8 among Irish. It is not especially an urban disease, many cases coming from small towns. It affects educated and professional classes more than it does laboring men. Fifteen per cent, of my private cases were lawyers, doctors and clergymen, one was an engineer and one a teacher. It is a disease of brain-workers more than muscle-workers.
Hereditary influence has some importance. Among 90 cases there was a history of apoplexy, heart or renal disease in the direct ascend- ants in 19 cases, showing a weakness in the direction of the circulatory system. There was a direct heredity of paralysis agitans in 2, an in- direct (paternal uncle and aunt) in 2, and a family or senile tremor among 8. Thus about 1 5 per cent, show a hereditary weakness as re- gards tremor. Melancholia was present in the ascendants of 7 cases,
618
PARALYSIS AGITANS.
619
and a tendency to anxious depression is very common among the patients themselves, three of whom had the melancholia of involution. Diabetes was present in the ascendants in 2 cases and in 3 of the patients themselves. Not many families, aside from those re- ferred to, were neurotic or rheumatic, and the children of patients were almost uniformly reported healthy. Some heredi- tary influence thus is present in 10 to 20 per cent., shown mainly in a tendency to benign tremor, stuttering or a tic, or in arterial sclerosis leading to cardiac dis- ease and cerebral hemorrhage. Paralysis agitans is distinctly a disease of sober living. It is not caused by the ordinary depart- ures from rectitude, such as dissipation, sexual, alcoholic, tobacco or food excess or even by lues. The thing most com- monly observed is a regular, laborious and anxious life. The patient, if a man, has not been a vacation man, and his habits have been, if anything, abnorm- ally regular. It is the same kind of history as that which often precedes the anxious depressions and melancholias of involution, and there is often a certain anxious depression with this malady. Among direct causes, there can be no doubt that work and worry and psychic trauma are the most important.
Shock and injury are noted in 2 per cent, of cases, but the
influence is not important in all of them. In the working classes, exposure and hard work are cited as causes, but there is usually a mental element of anxiety also. About 10 per cent, of men are
Fig. 242. — The attitude in paralysis agitans.
620
DISEASES OF THE NERVOUS SYSTEM.
smokers, very few (4 per cent.) are drinkers, and syphilis is almost never present (2 cases).
Rheumatism has been put down as a cause, but few patients are really sufferers from articular rheumatism. Many, however, as the disease comes on, suffer from lumbago, sciatica and various forms of muscular pain, which are usually rather premonitions than causes of the disease.
Diabetes was present in three cases, but it is probably a coinci- dence.
It does not often follow acute infections and has perhaps no rela- tionship to any generally accepted diathesis, unless it be that known as rheumatoid arthritis, or metabolic and autotoxic rheumatic con- ditions. The view that it is a "parainfectious" malady, due to the late action of an infection, has been suggested.
Symptoms. — The disease is sometimes ushered in with an acute illness, or an attack of sciatica. It then develops slowly with some aching pains in the arm and a slight tremor in the fingers of one hand, oftener the right. For a period of several months the patient perhaps simply knows that he has an arm. Then the tremor becomes dis- tinctly marked in the hand. This gradually extends and involves the leg of the same side, then the other side becomes affected. The neck, face, and tongue are rarely attacked by tremor, and then to a small extent. After or with the tremor there comes on a stiffness in the arms and legs, and indeed of the whole body. With this there is a general contracturing and shortening of all the flexor groups, so that the head and body are bent forward, the fingers are straight but are flexed as a whole on the metacarpus, the forearms flexed on the arm, the trunk is flexed forward on the thighs, and the knees are slightly bent. The attitude gives the idea of extreme senility (Fig. 242)., The gait is slow, the steps are short and shufBing; the patient has trouble in starting, stopping, and turning corners, owing to the slowness in initiating new movements in the voluntary muscles. When once started, he maybe unable to stop and has to run along. The speech becomes affected in one or two years. The voice is high-pitched, weak, and piping, or senile in quality. There is a slowness in getting out words or in start- a sentence, though after it is begun the words come rapidly. The con- dition is analogous to the festination in the gait.
Along with the other symptoms, there are often, though not always, sensations of heat, burning, fever, and rarely of coldness. These sen- sations are felt most in the feet, legs, or arms diffusely. Often there is a general feeling of restlessness and nervousness. Aching pains and a sense of fatigue occur; neuralgic pains are more rare. There are al-
PARALYSIS AGITANS.
621
ways a peculiar redness and flush in the faces of the patients. Some- times they sweat profusely. The temperature in the axilla is normal, on the skin it is sometimes increased. The appetite is excellent, often abnormally great, and digestion is good. Visceral complications are rare. Muscular weakness comes on early; it slowly increases, but real muscular paralysis does not occur. The disease ends in rigidity, which makes the patient as helpless as if paralyzed, but the muscles preserve considerable functional power to the last. The deep reflexes are present and not, as a rule, exaggerated ; but exaggeration and even clonus occur in a small percentage of cases. As the disease progresses the tremor increases in extent, and continues with little remission dur- ing all the waking hours; then the limbs get more rigid and in a way this rigidity drowns out the tremor; the patient becomes weaker and suffers keenly from sensations of fatigue and exhaustion. At last he can no longer walk ; he becomes bedridden and is finally carried off by exhaustion or some intercurrent illness (Fig. 246).
It will be seen that the dominant symptoms in paralysis agitans are :
1. Tremor.
2. Rigidity, progressively increasing, with trunk deformities and contractures.
3. Muscular weakness.
4. Sensory, vasomotor and metabolic disturbances. Further details must be given regarding these symptoms:
The tremor is a coarse one. It ranges from about 6 vibrations per second to 3.7. The average rapidity is 4 or 5 per second, which is about one-half the normal muscular rhythm. But the chief character- istic of the tremor is that it continues when the hand or limb is at rest, while voluntary motion causes it to cease. As the hand rests on the knee it shakes; as it is moved the tremor stops. When held straight out there is no shaking for a moment, but it soon begins. A glass of water is carried safely to the lips. The patient can control the tremor for a moment, especially in the early stages of the disease. These facts about the tremor apply in ninety per cent, of cases, but there are patients whose tremor is slight when the limb is at rest and is in- creased on voluntary effort. The hands are affected in a character- istic Way. The fingers and thumb are slightly flexed and held about in the writing-position ; the tremor moves the fingers and thumb as a whole, and they vibrate so that the one pats the other gently. Some- times the tremor is one of alternate supination and pronation of the forearm. The neck and face muscles are not usually or extensively involved, the shaking of the head being generally the result of the
622
DISEASES OF THE NERVOUS SYSTEM.
general bodily tremor. Sometimes one sees a tremor of the lips or neck muscles. The tongue and eye muscles are practically never in- volved in tremor.
Rigidity. — The rigidity comes on early, and may be the first and even the only prominent symptom. It affects chiefly the flexors of the arms, head and trunk, and legs, producing a characteristic senile posi- tion. In rare cases the extensors of the neck are affected and the head is drawn back. Cramps occur, and there is always a sense of stiffness. The muscular movements are slow, especially the initiation of a move- ment. Once started, a motion may be quickly done. The gait is peculiar; the steps are short and shuffling; the patient may have dif-
ficulty in starting; but once started he goes very well; or while walk- ing there may be a sudden running forward. This is called "festi- nation." Rarely there is a tendency to run backward or sideways. Some muscular weakness sets in early and slowly progresses, apparently with degenerative changes in the muscles. There is, however, no real nerve paralysis, but rather a muscular palsy which is due to the rigidity and is not accompanied with degenerative electrical reactions. The innervation of the facial muscles is weakened and the movements slow, so that the face becomes smooth and expressionless and mask- like. The muscles are overirritable to mechanical irritation and the facial responses to blows are exaggerated. The voice in paralysis agitans always becomes affected. It grows weaker, the sin'ging power is lost, then capacity to talk long or loud. The lawyer has to give up try- ing cases. Speech is slow, difficult and the voice high-pitched, feeble
Fig. 243. — Festination in paralysis agitans
PARALYSIS AGITANS.
623
and piping. Examination shows sometimes tremulous movement of the cords, but more characteristic is the slow, weak movement of the cords. In other words, the intrinsic muscles become weak and stiff and a little tremulous like the other muscles of the body.
The handwriting in paralysis agitans soon shows characteristic features. The act of writing becomes difficult ; the letters are formed slowly and painfully : they are drawn rather than written. The letters get smaller and more imperfect. Sometimes the writing is distinctly tremulous ; more often the characteristic is smallness and lack of finish of the lines, the letters finally becoming obscured. From year to
Fig. 244. — Showing progressive micrography. Above, handwriting in 1900; belo^ handwriting seven years later.
Fig 245. — Showing tremor in paralysis agitans.
year the words are written more imperfectly and in smaller letters. Tremulousness, slowness, lack of finish of letters and progressive mi- crographia are the characteristic traits.
The eye movements share the general tendency to functional arrest. The patient stares steadily ahead. Lateral movements both of the eyes and of head are diffcult. But the vision remains good, and with glasses the patient continues to read though convergence is tiring. The special senses do not become involved except that pains of a neuritic or aching and rheumatic character occur. An initial attack of sciatica or brachialgia is not uncommon. The muscular sensibility is exaggerated. This causes a feeling of restlessness that is very annoying. The vaso-motor system is lacking in tone and sta- bility. A characteristic flush early shows on the cheeks of the patient.
624
DISEASES OF THE NERVOUS SYSTEM.
The skin is ruddy, there is a tendency to sweats, and sensations of heat are often noted. The patient likes a cool room. The spinal centres are gradually weakened. Constipation is the rule; sexual power is gradually lost ; the bladder after some years becomes irritable and there is a sense of frequent desire to micturate. This, of course, occurs often in the older cases, but it is a vesical not a prostatic
Fig. 246. — Terminal stage in paralysis agitans.
trouble. Examination shows a congested condition about the neck of the bladder.
The patient is often emotional, but the mind is not seriously af- fected. The urine is usually about normal, but contains an excess of phosphates. There may be polyuria and less often glycosuria.
Forms. — A not very uncommon type is one with a great deal of tremor and hardly any rigidity, at least not until very late in the dis- ease. About equally common is the type "without tremor" and with
PARALYSIS AGITANS.
625
rigidity, limitation of movements and deforming attitude alone. Almost always even in these cases a little tremor is sometimes seen. Unusual types of paralysis agitans are the hemiplegic or the monoplegic, and the retrocollic type.
Course and Duration. — The disease slowly but steadily progresses until a full development of symptoms occurs, when it may remain
Fig. 247. — Muscular changes in late paralysis agitans.
stationary. It takes about two years for the whole body to be affected, though this varies much. It lasts from three to twelve years or even more. In three cases of mine, death occurred in three, six, and eleven years. Death is due to exhaustion and may be preceded by what seems like a toxic delirium with perhaps some fever.
The Diagnosis must be made from senile tremor, multiple sclerosis, posthemiplegic tremor, writers' cramp and wryneck affecting the ex- 40
626
DISEASES OF THE NERVOUS SYSTEM.
tensors bilaterally (retrocollic spasm). Senile tremor occurs in the very old and affects the head first and most. In multiple sclerosis the tremor is more jerky and is a tremor of intention ; there are nystagmus, syllabic speech, and often apoplectiform attacks, eye trouble, and paralyses.
Posthemiplegic tremor is accompanied by a history of hemiplegia; there are paralysis and exaggerated reflexes and the disease is unilat- eral. In retrocollic spasm only the neck muscles and frontalis are in- volved. The absence of exaggerated reflexes, the peculiar voice, gait and attitude, and the sensations of heat and nervousness often help greatly in the diagnosis.
The Prognosis is favorable as regards life; unfavorable as regards cure ; and not very good as to bringing about a cessation of progress in the symptoms. The progress is of the malady, however, can be delayed.
Pathological Anatomy and Pathology. — The postmortem changes are not very marked, and are seen mostly in the spinal cord and medulla. There are congestion and dilatation of vessels in the gray matter, a diffuse increase of interstitial tissue, atrophy and pigmen- tation of cells (Fig. 248). The disease has yet no established pathological anatomy. Degenerative lesions have been found in the anterior cornual cells of the spinal cord, and these resemble somewhat those of senility, but in my observations they are more striking, and the trouble cannot be explained as just a premature senility of the peripheral neurons, for extreme old age does not present the pic- ture of paralysis agitans. Degenerative changes have been found in the muscles, but these are probably secondary and it is not possible to explain the vasomotor disturbance, the mental disturbances or even the tremor by a muscular lesion alone. The disease seems often to impair first the automatically acquired activities, so that to do an ac- customed act like writing requires a distinct and persistent voluntary effort. It seems as if there was an interference at the synapses, where the central motor neuron passes its stimulus to the peripheral. It may be that it is higher up in the mid-brain where the co-ordinating mechan- ism and higher reflexes have their seat.
It is probable that the exciting cause of the functional degenera- tion is a glandular defect but the exact source of the toxin is yet unknown. The theory that it is a premature wearing out of function would be more tenable if the disease occurred as an occupation neu- rosis— which it does not.
The view that it is caused by perverted action of the parathyroids has been advanced bv Dr. Berklev, but not vet established.
r
PARALYSIS AGITANS.
627
Treatment. — The most important measure is rest, mental and phy- sical, with plenty of fresh air. No special diet is indicated. Hot baths
Fig. 248 — Cells of the anterior horn of the spinal cord. The right row from a case of paralysis agitans, showing atrophy and pigmentation, the left row from a normal case.
and 'mild massage are agreeable and helpful. I know of no climatic cure. The galvanic current produces temporary relief ; it should be given daily. Hyoscine hydrobromate, first used by Charcot and in-
628
DISEASES OF THE NERVOUS SYSTEM.
troduced into this country by Seguin, is of much value in relieving the tremor (gr. T^ increased). Codeine and morphine give the best per- manent results. Quinine and mineral acids are of much service in re- lieving the vasomotor and sensory symptoms. Bromide often helps the insomnia and restlessness. Extract of pituitary gland in doses of gr. xxx. to xl. daily quiets the system also. The extract of the parathy- roid was tried by the writer several years ago with no success. Since Dr. Berkley, however, has prepared a trustworthy extract, the results are better, and I have seen distinct relief in about one-third of my cases. The dose is gr. \ to gr. ii. ter in die. Suspension is of some use in a minority of cases not too much advanced. The mind in paralysis agitans is sometimes in an emotional, almost hysterical con- dition, and patients are easily made better for a time by some psych- ical influence. Violent exercise does harm. Regular systematic training exercises do some good, vibration is useless and so are hypnotism and osteopathy.
CHAPTER XXVII.
TROPHIC AND VASOMOTOR DISORDERS.
Progressive facial hemiatrophy is a disease characterized by a progressive wasting of one side of the face.
Etiology. — -It begins oftenest in the young between the ages of ten and twenty. Females are more affected.* There is in rare cases a hereditary history. Injury and infectious fevers sometimes start up the trouble. The left side is oftener attacked.
Symptoms. — The disease begins very gradually and shows itself first in patches. The skin gets thinner, there is loss of pigment, hairs
Fig. 248. — Facial hemiatrophy, early stage, showing alopecia and osseous depressions.
fall out, and the areas may have a yellowish appearance. Sometimes the periosteum and bone are affected, and shallow depressions are formed which may be anaesthetic (Fig. 248). The subcutaneous tissue is most involved, the muscles suffer least, and there are no changes in electrical reaction. The muscles of mastication are usually spared. The bone undergoes general atrophy and the lower jaw may be reduced to two-thirds the normal size. The secretion of sebum ceases, but that of sweat may be increased. The temperature falls. There are a
*About one hundred cases have been reported. Among five, seen by myself, three were in females, two in males. The disease in all cases, so far as could be found, began between the tenth and twentieth years.
629
630
DISEASES OF THE NERVOUS SYSTEM.
sinking in of the eye, narrowing of the lid, and dilatation of the pupil. There is sometimes pain and rarely anaesthesia. The tongue and other parts of the body may be involved. Spasmodic movements of
the muscles of mastication have been noted (B. Sachs). Sclero- derma sometimes appears on the face or hands.
The disease progresses rather rapidly at first, but finally comes to a standstill. It does not shorten life. .
Pathology. — There has been found a degenerative neuritis involving the fibres of the tri- geminus; its descending root and the substantia nigra were atrophied (Mendel).
The diagnosis is easy. Hemi- plegia with atrophy in children, Fig. 249 — Facial hemiatrophy. (Schoenborn congenital asymmetry, and and Krieger.) atrophy from gross lesions of
the nerve are distinguished by their stationary character or the presence of severe pain.
Treatment. — There is no treatment known to be of service. Tonics, iodides, and electricity may be tried. Dercum has suggested resecting the trigeminal nerve.
Progressive faciae hemihypertrophy is an extremely rare condition, only eleven cases having been reported. It is usually congenital in origin, but may develop in connection with giantism, as in a case of my own (Fig. 250).
Acromegaly (Marie's Disease).
Acromegaly is a chronic dystrophy charac- terized by gradual enlargement of the hands, Fig. 250.— Facial hemi- feet, head, and thorax, and by a dorso-cervical hypertrophy occurring in kyphosis. Though the disease was first described a giant, only ten years ago by P. Marie, the number of
cases reported is rapidly increasing, and if one includes various abor- tive types it is not extremely rare.
Etiology. — It affects the two sexes nearly alike. It begins between the ages of eighteen and twenty-six ; recently a congenital case has been reported. No hereditary influence or definite exciting cause is
TROPHIC AND VASOMOTOR DISORDERS.
63I
known. The patients are sometimes naturally endowed with large extremities.
Symptoms. — The disease begins with a gradual enlargement of the hands, feet, and head. In women there is amenorrhea, in men, sexual weakness; slight rheumatic pains, headaches, malaise, mental hebetude, anaemia, and general weakness are present. The patient shows striking contrast between his bulk and his general strength. The skin is dry and there is polyuria.
Fig. 251. — Acromegaly.
The hypertrophy affects the soft parts as well as bones. In these latter there are periosteal thickening and hyperplasia, with the result of producing increase in width more than length. The arms are not much invo ved, nor is the shoulder-girdle, except the clavicle. The lower jaw is much more involved than the cranium. The tongue, lips and nose are enormously hypertrophied (Fig. 251). The thorax is enlarged antero-posteriorly and flattened. There is sometimes dullness over the sternum due to persistence of the thymus. The pel- vis may be enlarged, but the hip and leg bones are generally spared. The hands and feet undergo enormous hypertrophy (Fig. 252). The following are some of the measurements in the case that has come
632
DISEASES OF THE NERVOUS SYSTEM.
under my observation, reported by Adler, and in eases reported by Osborne and Packard :
Length of hand 7.6 to 8f inches.
Length of foot 1 1.7 to 12^ "
Cranial circumference 24 to 26I "
Circumference of thorax 14 "
It seems probable that nearly all so-called giants are cases of acromegaly.
The vision is sometimes impaired and there may be temporal hemian- opsia due to the pressure of the enlarged pituitary body on the optic chiasm. The muscles may be at first hypertrophied, later atrophied. There are no paralyses and rarely any anaesthesias.
Fig. 252. — Hand in acromegaly.
The disease runs a very chronic course, lasting ten or twenty years.
Pathology. — There has been found an enlargement of the pitui- tary body in nearly all cases, and it is possible that the disease is due to disorder of its function. The attempts to place the disease in relation with a persistent thymus, sclerotic changes of the sympa- thetic, and disease of the thyroid, all of which conditions have been found, are unsuccessful. The disease must be regarded as a perver- sion of nutrition accompanied by hypertrophy and perhaps defective action of the pituitary gland.
The diagnosis must be made from congenital enlargements, from so-called giant growth which affects single members, and from ostei-
TROPHIC AND VASOMOTOR DISORDERS.
633
tis deformans. In the latter disease it is the shafts of the long bones and the cranium, not the face, which are involved.
Pneumogenic osteo-arthropathy is the name given by Marie to a disease associated with pulmonary and pleuritic disease, and charac- terized by enlargement of the extremities and peculiar deformities of the terminal phalanges. The enlargements are not uniform. The tongue is not affected. The wrist and ankle bones are hypertrophied, the finger-tips are bulbous and spade-shaped.
Prognosis. — Acromegaly is incurable, but it has been arrested, or at least has ceased to progress, and it may not greatly shorten life.
Fig. 253.— The hands in pneumogenic osteo-arthropathy.
Treatment. — Cases have been reported in which iodide of potas- sium and arsenic have arrested the disease. In general, the treatment is only symptomatic, but feeding with pituitary gland in large doses (gr. 3d.) should be tried.
Myxcedema.
Myxcedema is a disease of the thyroid gland, but its symptoms are so largely nervous that a brief description of it is justified here. It is a chronic disorder, due, as a rule, to an interstitial thyroiditis, and characterized by a solid oedema of the subcutaneous tissue, dry skin, loss of hair, subnormal temperature, mental dullness, and even
634
DISEASES OF THE NERVOUS SYSTEM.
insanity and idiocy. It has two forms — the congenital and infantile — causing a condition known as cretinism; and an adult form con- stituting myxoedema proper.
It occurs most often between the ages of thirty and fifty, and oftener in women (seven to one). It is seen oftenest in temperate climates. Hereditary influence, alcoholism, and syphilis are not predisposing factors; lead poisoning may be a cause.
It begins slowly. The patient is languid and dull, and is unusually sensitive to cold. Voluntary movements are slow ; the weight increases
Fig. 254. — A case of myxcedema in a man aged forty-four years. (Murray.)
and a solid oedema which does not dent on pressure develops in the face and extremities. The skin gets dry and rough, the hair begins to fall, the temperature is subnormal, i° to 20 F. Mentally the patient is dull, depressed, and in one-fifth af the cases melancholia, mania, or dementia develops. The muscles are weak, the gait is slow, the voice hoarse and monotonous. There is considerable anaemia and the heart is weak. The pulse is slow and the arterial tension low. Albu- minuria is present in twenty per cent, of cases, and hemorrhages may occur.
The pallor, oedema, loss of hair, and mental hebetude give to
TROPHIC AND VASOMOTOR DISORDERS.
635
the face a characteristic expression (Fig. 254). The disease may run a course of six or seven years, the patient dying of cardiac weakness or some intercurrent malady.
The disorder is due in most cases to a chronic interstitial thyroi- ditis which usually causes atrophy of the gland. It may be produced by artificial removal of the thyroid. The result of this is a defective action of the thyroid, and a consequent poisoning of the system and deposit of mucin in the subcutaneous tissue especially.
The diagnosis is based upon the peculiar physiognomy due to the oedema pallor; the loss of hair, subnormal temperature, mental hebe- tude, and atrophied thyroid.
The prognosis is good if treatment is instituted.
The treatment consists in the administration of the thyroid ex- tract in daily doses ranging from five to forty grains or even more. The results are most brilliant, and humanity owes much to Dr. George R. Murray, who first instituted it.
Cretinism.
Cretinism is a form of myxoedema due to absence, atrophy, or defective function of the thyroid gland, occurring congenitally or during infantile life. The disease occurs endemically in parts of Europe, but only sporadically and happily with great rarity in America
Fig. 255. — A cretin dwarf, aged twenty. (Leszynsky.)
Hereditary and family influences are at work in endemic but not in sporadic cretinism. It develops either directly after or in the first three years of life, and shows itself in a stunted growth both of brain and body, most cretins being idiotic dwarfs. The general symptoms are much like those of myxoedema in adults plus the retarded growth of mind and body. The deposits of solid oedema cause peculiar defor- mities and lead to a characteristic physiognomy. The mind is dull
636 DISEASES OF THE NERVOUS SYSTEM.
and placid, the muscles are weak, the abdomen is protuberant, the hands and feet are broad and thick; the patients are anaemic, the temperature is subnormal. The arrested bodily growth is such that on reaching adult age the stature may be only twenty-eight to thirty- three inches (Fig. 256).
FlG. 256. — Sporadic cretinism in a patient aged twenty-eight; height, '34 J inches. (Murray.)
Cretins usually die young, but some survive to the age of thirty and forty.
The treatment is the same as that of myxcedema. Here, too, if the case is seen before adolescence, brilliant results can be obtained.
Angio-neurotic CEdema (Circumscribed CEdema).
Angio-neurotic cedema is a functional disorder characterized by the rather rapid appearance of circumscribed swellings upon different
TROPHIC AND VASOMOTOR DISORDERS.
637
parts of the body, these swellings being due to disturbances of vaso- motor innervation and not of an inflammatory character. The disease occurs oftenest in early adult life, the average age being from twenty to thirty, but it has been observed in young children and even in the aged. It occurs oftener in males than in females, except in this country, where the reverse ratio exists. Hereditary influence plays a part in some cases ; the disease has been known to run in fami- lies. It occurs oftener in winter and oftener in the early morning hours. Exhausting occupations predispose to it. The exciting causes are sudden exposure to cold, slight traumatisms, fright, anxiety, grief, and the ingestion of certain kinds of food, such as apples or fish. A peculiar form of this oedema seems to develop in connection with menstruation.
Symptoms. — The disease appears without much if any warning. In a few minutes or hours there develops a circumscribed swelling upon the face or arms or hands. This swelling varies in diameter from one-half inch to two or three inches. It may be dark reddish or rosy or it may be pale and waxy. It does not easily pit on pressure. There is sometimes a local rise, sometimes a fall in temperature. It is accompanied by sensations of tension and stiffness, scalding, burn- ing, and sometimes itching, but there is no actual pain. The swelling is usually single, but it may be multiple. It is located most often upon the face, next upon the extremities, particularly the hands; next on the body, then in the larynx and throat, and then on the genitals. The swellings last from a few hours to two or three days. Between the attacks the patient feels well. They are apt to return at intervals of three or four weeks to several months. Sometimes they are brought out only by certain peculiar exciting causes, such as indigestion or mental anxieties or emotional disturbances. When the disease attacks the larynx or throat, serious symptoms of dyspnoea and suffocation may appear; surgical interference may even be called for, and death has been known to result. It has been thought that neurotic oedema may sometimes attack the stomach, producing symptoms of nausea, vomiting, and great gastro-intestinal distress, and an acute neurotic oedema possibly sometimes attacks the lungs. This, however, is unlikely since the vasomotor innervation of the pul- monary blood-vessels is a very stable one.
The pathology of the disease is not known, except that it is unquestionably a disorder due primarily to toxaemia and a disturb- ance in nerve innervation. The nerves affected are, furthermore, undoubtedly vasomotor nerves . The oedema is precisely similar to that which is associated with attacks of tic douloureux and migraine.
638
DISEASES OF THE NERVOUS SYSTEM.
Diagnosis. — The symptoms of the disease are so peculiar that they are easily recognized. The spontaneous appearance of the oedema, its recurrence at certain intervals, and the absence of pain and evidences of inflammation are sufficient usually to enable us to recognize it. The giant urticaria is a disease resembling neurotic oedema and probably closely allied to it. The blue and the white oedema of hysterics differ in being persistent and associated with paralyses, anaesthesias, and contractures.
The prognosis, so far as cure is concerned, is not very good. The attacks, however, can be ameliorated, and the disease itself is not serious as regards life and the enjoyment of a fair degree of general health.
The treatment consists in the adoption of such measures as will give tone and stability to the nervous system : the use of cold baths, exercise, and massage is indicated. Internally, mineral acids and strychnine may be of some value. Cascara, nux vomica, the salicy- lates, arsenic, quinine, and atropine are all drugs which have been recommended.
CHAPTER XXVIII.
THE DISORDERS OF SLEEP.
Insomnia, Hypnotism, Morbid Somnolence, Catalepsy, Trance, Lethargy, the Sleeping Sickness.
Sleep is a condition in which consciousness is normally lost and in which the whole body, but particularly the brain, enjoys functional rest, while constructive and nutritive activity goes on.
Physiology. — The most conspicuous phenomenon of sleep is the subsidence of the higher cerebral functions ; yet other organs, notably the muscular system, also take part in the resting process. The brain during sleep is slightly anaemic, the deficiency in blood being a part of, but not the cause of, the phenomenon. The remote cause of sleep is inherent in the nervous tissue itself, which follows the great rhyth- mical law, common to all living tissue, of rise and fall in its irrita- bility. It is probable that the immediate cause of drowsiness is exhaustion of the irritability of the cortical cells and the benumbing of them by the circulation of waste products in the blood. Many facts in the history of the pathology of the brain point to the exist- ence of a sleep-centre, which being especially acted upon tends to inhibit the consciousness and draw the mind into a somnolent state.
As sleep is only a function, we cannot speak of its diseases, but only of its disorders, and these really form but a part of the diseases of the brain or of general diseases. It is a matter of convenience, however, to discuss some of these separately.
Classification. — Custom has established the use of certain terms for the various disorders of sleep, and such terms must be for the most part adhered to. It will be proper, however, for the sake of complete- ness, to arrange the various disturbances we are to discuss in accord- ance with the modern methods of studying the pathological changes of bodily functions. We propose, therefore, the following classifica- tion, which indicates the various depressions, exaltations and per- versions of the functions of sleep:
I. State of normal sleep. Hypnosis. Somnus. II. States of absence of sleep. Ahyp- Insomnia. nosis.
639
640
DISEASES OF THE NERVOUS SYSTEM.
III. States of perverted or artificial sleep.
Parahypnosis.
IV. States of excessive or frequent drowsi-
ness and sleep. Hyperhypnosis.
Dreams, nightmare, night terrors, sleep- drunkenness, somnambulism, hypnotism.
Morbid somnolence, paroxysmal sleep, epi- leptic sleeping-attacks, trance sleep, leth- argy, sleeping-sickness of Africa.
I. Normal SEEEp varies much in accordance with age, sex, the individual, and, to a slight extent, with occupation, race, and climate. The infant sleeps fourteen or sixteen hours out of the twenty-four, the adult needs about eight hours, while the aged live healthfully with but six. Women need half an hour or an hour more than men. A few persons, generally men, need nine, ten, or even twelve hours' sleep daily; others require only six. Brain workers, as a class, take less sleep than laborers. Sleep is sounder and longer in cold climates and among northern races.
II. Insomnia is a term given to conditions in which persons simply suffer from insufficient and restless sleep or from entire absence of sleep for a long time. Such conditions result from a great variety of causes. It is my purpose to discuss only those forms in which the trouble is functional or nutritional, leaving out of consideration the symptomatic insomnia of organic brain disease and that occurring as the result of painful diseases.
An entire absence of the capacity to sleep occurs most often and typically at the onset or in the course of insanity. It is here a promi- nent and most distressing symptom. The length of time during which a person can live without any sleep is about the same as that during which he can go without food, viz., three weeks. Many hysterical, neurasthenic, or incipiently insane individuals will assert that they have not slept for weeks, but careful examination shows that they have at least been in a drowsy, somnolent condition, which is, in a measure, physiologically equivalent to sleep.
Etiology. — The cases in which persons can get only a troubled rest of a few hours are much more numerous. It is a disorder of the third, fourth, and fifth decades of life. Women are less liable to suffer from it than men, and the laboring classes less than those engaged in business or professional pursuits. A frequent symptom of neurasthe- nia is an imperfect, and especially an unresting, sleep. In gout and in the so-called latent gout, or lithaemia, insomnia is a frequent symptom. One of the few nervous symptoms of secondary syphilis is insomnia. Insomnia may develop as a bad nervous habit in persons who are neg- lectful of themselves. It occurs sometimes as an hereditary neurosis. I am acquainted with a family in which, for four generations, one or more of the members have suffered from chronic insomnia throughout
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life. In anaemia and chlorosis there is often insomnia at night, com- bined with somnolence during the daytime. Disease of the heart and arteries may lead to insomnia, and under this head come the cases which occur in Bright's disease with tense arteries and ansemic brains. Disorders of the stomach lead to disturbed sleep oftener than to com- plete insomnia, and the liver, when inactive, causes somnolence rather than the contrary. The poison of malaria and the toxic agents of fever must be added to the list of causes of imperfect sleep.
It will be seen that the causes of chronic functional insomnia may be classed under the following heads:
1. Neurasthenic and vasomotor, including hereditary and habit insomnia.
2. Vascular and cardiac, including heart disease, arterial fibrosis, and general anaemia.
3. Autotoxic or diathetic, including lithaemia, gout, and uraemia.
4. Toxic, including syphilis, lead, malaria, tobacco, and various drugs, such as coffee, tea and cocoa.
In many cases there exists a combination of these causes.
Symptoms. — The forms and degrees of insomnia vary greatly. In children it is accompanied usually by much mental and physical disturbance. The patient is restless, excited, talkative, or querulous and irritable. The insomnic child is more ill than the insomnic adult. In neurasthenic insomnia there is a tumult of thoughts which prevent sleep, or sleep is superficial, unresting, and interrupted by dreams. In many cases of nsanity insomnia is characterized by great motor restlessness. In old people insomnia is generally of the quiet kind.
Treatment. — As insomnia in all its phases is often a symptom of some general disorder, treatment of a curative kind must be directed to this. Anaemia, lithaemia, uraemia, malaria, and the other toxic influences must be removed by remedies adapted to these conditions. But besides constitutional treatment there is a symptomatic treat- ment which will be discussed here, premising, however, that while there are many sleep-producing medicines there are no good drugs for insomnia.
The older physicians in treating sleeplessness, used to depend largely on hyoscyamus, camphor, opium, and the fetid drugs, such as asafoetida, musk, and valerian. Hyoscyamus is still used. It is to be given in large doses, such as five or ten grains of the extract, or even more, and from ten to twenty drops of the fluid extract. The hydrobromate of hyoscine, in doses of gr. to gr. ^ or more, is one of the best forms. Hyoscine is indicated in the insomnia of the in- 41
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DISEASES OF THE NERVOUS SYSTEM.
sane, especially in forms accompanied by motor activity. Chloral hydrate still holds its own as one of the surest of hypnotics. The dangers involved in its use have been somewhat exaggerated, though they are sufficiently real. Doses of gr. x. and gr. xv. are often quite large enough, but in alcoholic insomnia it may be given in twice the above amounts, guarded with ammonia and digitalis. Not a few persons find that chloral has bad effects. The patient awakes with a dull, heavy sensation in the head, slight headache, or gastric disturbance.
The various bromides are efficient and safe hypnotics if properly used. The immediate effect of them is simply sedative, and sleep is not produced unless very large doses are given. Some persons are even kept awake by average doses (gr. xv. to xx.). In insomnia, therefore, bromides are best prescribed in doses of gr. xv. three times a day. By the second evening sleep is generally secured. The bromide habit is rarely formed, and is in itself not so seriously injurious as the chloral or opium habit. The bromides alone are hardly strong enough hypnotics for alcoholic insomnia or the insomnia of insanity. I have found them to fail in the insomnia of the aged. Paraldehyde ranks close to chloral in its value as a hypnotic. In some persons it disturbs the stomach, but not in all, and it may be used as a hypnotic for months without its power being impaired. It is a disagreeable drug, and there is nothing so far as I know, that palliates its offensiveness. I prefer, however, to prescribe it in ^i. doses poured upon a teaspoonful of powdered sugar. Doses of 5SS- are sufficient to cause sleep in many cases, and collapse may be caused by 5ij- given to weak patients. Urethane, in doses of a scruple or more, is a mild and agreeable hypnotic, but not so certain as paraldehyde. Amylene hydrate is a hypnotic of prop- erties similar to those of paraldehyde, but less disagreeable. It is given in doses of about one drachm. Lupulin in large doses, gr. x. to gr. xx., is a good hypnotic. Sulphonal in doses of gr. x. to gr. xxx. given two or four hours before retiring is one of the best hypnotics. Chloral-amide is more agreeable and safer than chloral, though it acts practically in the same way. The dose is Qi. to 5)ij. Trional in doses of gr. xv. is rather better than sulphonal. Veronal sometimes acts best of all hypnotics, but it is to some depressing.
Among the antispasmodics are several drugs which occasionally answer well in the insomnia due to nervous irritability. A drachm of the fluid extract of valerian or of spirits of lavender, for example, may be prescribed. In some forms of insomnias — perhaps best in those due to fever, or pain, or some rheumatic or gouty trouble — ■ antipyrin in twenty-grain doses acts well. It is known that in
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many cases of mild types of insomnia a dose of whiskey, brandy, or beer will put the patient to sleep.
Besides drugs, there are many hygienic or mechanical measures to which the physician may successfully resort — listening to monoto- nous noises, reading dull or heavy books, counting, or keeping before the fancy some blank or wearying picture —
"A flock of sheep that leisurely pass by One after one; the sound of rain and bees Murmuring; the fall of rivers, winds and seas, Smooth fields, white sheets of water, and pure skies."
In cases of purely bad habit insomnia, psychotherapeutics even to the point of actual hypnosis acts well.
Mechanical remedies have nearly all for their purpose the with- drawal of the blood from the brain to the skin and abdominal viscera. Hot footbaths or warm general baths, cold douches down the spine, beating the limbs with rubber hammers, brisk exercise, a light meal, massage, all are at times efficient hypnotics. Persons who suffer from insomnia should sleep in cold rooms, the head should not be too high or very low, and in most cases they are better without late sup- pers, even though these be light. Mental work should be laid aside several hours before retiring and the evening devoted to quiet conver- sation and reading or amusements that do not actively excite the nerves. Many persons live in good health though they sleep in the day and stay awake at night. Journalists and editors, whose work obliges them to go to bed in the early morning, often continue for years without impairment of physical vigor. Despite this, it is true that the best time for sleep is at night, and that the old maxim, "Early to bed," is a sound one. Yet it is not the early bird that gets the worm so much as the bird that has slept well. The human system requires a certain amount of sleep and should have it. The industrious and ambitious often try to train themselves to shorter hours, but though they may succeed for a time, nature will not be cheated out of her due and health suffers in the end. It is a widespread custom in some countries to take a short nap in the daytime, and the custom is a good one. America has not adopted it, but might do so with benefit to the health of her brain-working class. Many from childhood up do not get a sufficient amount of sleep.
III. Perversions and Disturbances of Sleep. — Sleep is said to reach its deepest stage in from one to two hours after it begins. There is then after this a gradual lessening of the depth of sleep. Probably there are great variations in this rule, for many persons seem in sound-
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est slumber several hours after falling asleep. But, at any rate, there are lighter stages of sleep at its inception and toward its end. These are the favorite times for dreams, and at this period also there develop the peculiar phenomena of sleep drunkenness.
Dreams, Nightmare. — When sleep is perfect and profound, dreams afterward remembered do not occur. Dreaming is, therefore, a mor- bid symptom, although often of trivial significance, especially if it occurs at about the time of natural waking, when slumber is, in its physiological course, passing into the lighter stages. In sleep, no matter how light, the action of the regulating centre which directs thought, controls emotion, and exhibits itself in volition is suspended; the psychical mechanism, if excited to action at all, works without pur- pose, like a rudderless ship at sea. Ideas and emotions succeed each other by the laws of association, but are not properly correlated, and ju Igment and logical reasoning are gone. As a rule, dreams are made up of somewhat ordinary ideas and fancies incoherently asso- ciated, and shifting too rapidly to call up much feeling. When from some point in the body painful sensory excitations do produce dis- agreeable images, emotions of a most violent kind may be felt.
In the earlier stages of civilization, among primitive people, dreams were comparatively rare. When they did come with vividness they were regarded with importance, and often wTere considered visitations of spirits. Civilized man dreams more, but he has learned to treat Ins fancies with corresponding indifference. The attempts of scien- tific men to formulate laws regarding them have been productive of small results. Some diseases, however, cause, as a rule, dreams of a more or less peculiar kind. Thus heart disease is accompanied by dreams of impending death. Previous to attacks of cerebral hemor- rhage patients have dreamed of experiencing some frightful calamity or of being cut in two. Intermittent fever is often announced by persistent dreams of a terrifying character. Hammond has collected a large number of what he terms prodromic dreams, all going to show that before recognizable signs of disease are present morbid dreams of various kinds may occur. Albers says: "Frightful dreams are signs of cerebral congestion. Dreams about fire are, in women, a sign of impending hemorrhage. Dreams about blood and red objects are signs of inflammatory conditions. Dreams of distorted forms are frequently a sign of abdominal obstructions and diseases of the liver."
Nightmare is a disorder incident to the hypohypnotic state, or that of incomplete sleep. It is one of those minor ills that are nearly always symptomatic of an irritation in some part of the body.
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The usual causes of it are some digestive disturbance (repletion) and cardiac disease. Persons of a nervous temperament are more subject to it; and there are individuals whom it makes suffer all their lives. The popular belief that sleeping on the back favors it is, in general, a correct one. When nightmare occurs in cardiac disease a certain position, semirecumbent or on the right side, must be main- tained, or the painful fancies will awaken the patient. Healthy pecple can get sound sleep whether lying upon the back, the side, or the stomach; but light sleepers and those with sensitive abdominal viscera generally find that the position on the right side is the most comfort- able and less provocative of unpleasant dreams. Prolonged mental or physical strain, excitement, and worry predispose to nightmare. Farinaceous foods, excessive use of strong liquors, coffee, and tobacco, all have a similar tendency. Nightmare occurs also in anaemia and malaria, and it may, in fine, be excited by morbid conditions in any part of the body. It sometimes occurs about the menstrual period in women. Its most common feature is a sense of suffocation or im- pending death.
Pavor noctumus, or night-terrors, is a sleep disorder peculiar to children. It is allied to nightmare on the one hand and sleep-drunk- enness on the other. It differs from the former condition in that the child continues to suffer from the distressing fancies for some time after he is awake. Night-terrors occur usually one or two hours after sleep has begun. The child wakes up screaming with fright, and perhaps runs about the room or seeks its parents for pro- tection against some imagined harm. The disorder occurs in weakly, anaemic, nervous, or rheumatic children. It is due sometimes to lithaemia or, as the older writers put it, rheumatism or gout of the brain. Digestive disturbances, worms, dentition, hereditary syphilis, mental strain, fright, and excitement are placed among the causes. It sometimes appears to be a paroxymal neurosis allied to epilepsy. The disorder is usually harmless and the prognosis favorable.
Somnolentia, or sleep-drunkenness (Schlaf trunk en) , is a condition of incomplete sleep in which a part of the faculties is abnormally excited while the other is buried in repose. It is a kind of acted nightmare. The person affected is incoherent, t excited, and often violent. He experiences the delusion of some impending danger, and while under it acts of violence have been committed. The condition is one of medico-legal importance, therefore, and has been discussed by writers on that science (Wharton and Stille). Minor degrees of it are often noticed in children and in adults who are roused from a very profound sleep. It at times becomes a habit and a most annoy-
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ing or dangerous one. The disorder in its severe form is fortunately very rare.
The treatment of morbid dreams, nightmare, and pavor nocturnus must be directed to a removal of the causes. Tonics, cardiac stimu- lants, laxatives, antirheumatics, attention to diet, are called for ac- cording to the condition of the patient. Change in surroundings, is often necessary. Among symptomatic remedies the bromides are the best, except in lithaemia, when alkalies and salicylates may prove more serviceable. In somnolentia the patient should be prevented from getting into too profound sleep. He may be awakened once or twice during the night, or take a nap in the daytime. The head in sleeping should be raised high and the body not too heavily covered.
Somnambulism. — Somnambulism is a condition similar to hypno- tism or the mesmeric state. In it volition is abolished and the mind acts automatically under the dominance of some single idea. It is an acted dream. Sight, hearing, and nearly all the avenues of sense are closed. The sleepwalker avoids obstacles and performs ordinary acts automatically like an absent-minded man, which in reality he is. All those mechanisms which have been trained by constant repetition to act automatically like that which preserves equilibrium are active, and their powers may even be heightened, so that the somnambulist may walk along roofs or on dangerous roads and thread intricate passages without harm. The automatism of the somnambulist may continue for hours, until a journey has been per- formed or a task completed. He may carry out with success familiar mathematical calculations, write a letter, or work upon a picture, but he only follows along the lines established by constant iteration in his waking moments. He can originate nothing new. He is roused from his state with difficulty, and when out of it he remembers nothing of what has occurred.
Somnambulism usually arises from overeating. Sleeping with the head too low is another cause. Violent emotions act indirectly by disturbing digestion. The habit being once established, however, attacks occur without apparent cause. The disorder occurs oftenest in young people about the age of puberty, and it then attacks the sexes alike. Later in life women are more often affected. The disease is fostered sometimes at school by the attentions of the schoolmates. In most cases a condition of morbid sensitiveness underlies it. The patients are neurotic. Hereditary somnambulism has been observed. Its attacks have alternated with those of catalepsy. They are likely, after a time, to become periodical, occurring every week, fortnight, or month. The somnambulic state may come upon a person in the
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daytime. It is then regarded as spontaneous trance, or hypnotism. It is not the case, however, that persons who are easily hypnotized are usually somnambulists, though the reverse may be true.
Somnabulism is a term that should include not only sleep walking, but sleep talking.
The treatment of somnabulism is very much like that for sleep drunkenness. The patient's surroundings must be investigated, and unfavorable influences, such as may occur at school or from in- judicious nurses, be removed. He should be prevented from sleeping too soundly, the head should be raised, the clothing light, the diet regulated. Remedies like iron, quinine, phosphorus, and cod-liver oil may be given. When the patient is discovered in the somnambulistic state he should not be awakened, or at least not until he is safely back in bed.
Hypnotism, Trance, Mesmerism. — Major hypnotism is a morbid mental state artificially produced and characterized by (1) perversion or suspension of consciousness; (2) abeyance of volition; (3) a con- dition of suggestibility leading the patient to yield readily to commands or external sense impressions; and (4) intense concentration of the mental faculties upon some idea or feeling. Some contend that the hypnotic condition is not a morbid one and is not really different from profound sleep. There is, however, certainly some abnormal state of the subconscious attention.
Minor hypnotism is a state closely bordering on normal sleep in which there is a lowering of consciousness and a condition of suggesti- bility.
The proportion of persons of all ages found by Beannis to be hypnotizable was about eighteen or twenty per hundred. Children up to the age of fourteen are very susceptible. After the age of fifty- five susceptibility lessens. Men are almost as easily affected as women ; but persons of a docile mind and those trained to some degree of mental discipline and capacity for submission, such as soldiers and artisans, are more sensitive. In this country the percentage of hypnotizable subjects is less than it is in Europe. Hysterical and insane persons are not very susceptible. Those who have been mesmerized once are more easily affected afterward, and may even pass into the state involuntarily.
Methods. — There are two ways of inducing hypnotism: the fixa- tion method and the suggestive method. The former and older plan, devised by Braid, is to make the patient fix his eyes for five to ten minutes on some bright object at a distance of six or eight inches from the eyes and a little above the horizontal plane of vision. A modifi-
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cation of this is the fascination method of Luys, by which the patient is made to fix his eyes on revolving mirrors. Practically, it is some- times the custom among practicing hypotizers to give the patient a dose of paraldehyde.
In the "suggestive method" devised by Liebault and Bernheim the subject is placed in a chair in front of the operator. The opera- tor then talks to the subject in a firm and confident voice, assuring him that he will go to sleep in a short time, telling him to make no resistance, that his sleeping will be natural, that nothing will be done to worry or fatigue him, that he will dream pleasant dreams, that he will wake up feeling better; then that he is feeling drowsy, his eyes are heavy, objects look confused, the lids are falling, they are closed — in a moment more the patient goes off to sleep. This requires some little time — five to fifteen minutes. It may fail the first time and succeed the second.
Hypnotic states be self-induced by vigorously fixing the attention upon some object. The ecstatic states of the saints and the nirvana of the Buddhists are forms of hypnotism; so also are the 'trance states in which some clairvoyants and spiritualistic preachers place them- selves; this same curious phenomenon is at the bottom of the so-called "mind-healing" science, and it enters into rational therapeutics and orthodox religion. The capacity of the human mind for hypno- tism or semihypnotic states is, therefore, a most curious and impor- tant fact.
Symptoms of the Major Form. — The person who has been hypno- tized at first sits or lies quietly in the position he has assumed during the manipulations of the operator. No notable physiological changes occur, as, for example, in the pulse, respiration, temperature, pupils, skin, etc. Some increase in the cerebral blood-supply, however, is said to be present. The patient will now respond automatically to any outside command or will be dominated by any idea which is suggested to him. He will talk, or walk, or run, or gesticulate, assume expres- sions of fright, anger, or joy, entirely in accordance with the com- mand given. Apart from these commands, he is entirely dead to the outside world. He hears, sees, smells, tastes, and feels nothing. He can be burned, cut, or injured without showing any signs of feeling. At a suggestion he may be made cataleptic, somnambulic, or para- lytic. This state is termed somnambulistic trance. If left to himself, he gradually sinks into a deep sleep, from which he can with difficulty be roused. After a time, rarely more than one or two hours, he awakes as from ordinary slumber. This latter state is called trance coma, or lethargic hypnotism. The attempts of the Charcot school to
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divide hypnotic phenomena into three forms, the somnambulic, cata- leptic, and lethargic, are hardly successful. Sensitive subjects can be thrown at once into lethargy, catalepsy, or somnambulic states at the command of the operator.
The phenomena of hypnotism depend upon the wonderful sen- sitiveness and quickness of the subject in responding involuntarily, with all his nervous energy, to outside suggestion. Dishonest per- sons may learn the latter trick and thus simulate the hypnotic state. Traveling mesmerizers utilize such persons largely ; hence no confidence can be placed in the phenomena exhibited by them.
Minor hypnotism is produced by the "suggestive method" of hypnotizing. By this latter plan patients are thrown into various degrees of the hypnotic state from slight drowsiness to lethargy, but they are not somnambulic, and do not become cataleptic or anaesthetic.
Patients naturally come out of the mesmeric state through the channel of deep sleep or lethargy. Ordinarily they are dehypnotized by word of command, or by a pass of the hand, or any impression which the patient expects to be used for the purpose.
Hypnotized persons have been observed to have a diminution in the spinal reflexes and a muscular hyperexcitability. They sometimes show a most extraordinary exaltation of visual, auditory, or other special sense.
Pathology. — The underlying changes of the hypnotic condition are unknown and will probably long remain so. Hypnotism is no doubt associated with changes in the vascularity of different parts of the brain and with rapid breaking down of nerve-tissue. Ani- mals constantly subjected to hypnotic influence become demented (Harting, Milne-Edwards). The state of major hypnotism is prob- ably pathological. It is a neurosis. Minor hypnotic states are but slightly removed from the normal, if at all, and their production is not injurious.
Diagnosis. — As hypnotic states may be imitated and as injuries or crimes may be done in this state, it is very important to be able accurately to distinguish it. Since the phenomena are all subjective, this is very difficult. The methods of value are these:- 1. Careful examination of the general phenomena by experts while the sub- ject is in the alleged hypnotic state. 2. Testing the muscular hyper- excitability by percussing motor points. 3. Tests of alleged anaes- thesia by sudden burning, or pinching, or injuring the subject. 4. Tests of the tetanic muscular rigidity by the revolving tambour. In the hypnotic state the hand may be extended and held with perfect steadiness, while in conscious states a tremor soon appears. 5. Tests
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with glasses and other apparatus may be made to determine alleged anaesthesias of the special senses.
Therapeutics. — The practice of using major hypnotization is usually injurious, if repeated much, tending to exhaust the nervous force and weaken the will. It should be done only with the greatest care. Its utility in therapeutics I greatly doubt. It may relieve symptoms in the hysterical for a time, but it cannot be of permanent benefit and is likely to lead to actual harm.
The induction of minor hypnotic states by suggestion is not harmful if carefully and moderately employed. Its practical results, however, are not great, and the method is tedious, uncertain, and sometimes ridiculous. It has its value in pedagogy, among children, in neurasthenia and in morbid habits. The general popularization of hypnotism by means of mind cures, Christian science, etc., accom- plishes its results at the expense of mental demoralization, and faith- healing institutes are pernicious elements in society.
Morbid Drowsiness. — This is a very common symptom, which may be due to any one of the following causes: 1. Old age, when there is a weakened heart or diseased arteries, with cerebral malnutri- tion. 2. The diseased vascular conditions which precede cerebral hemorrhage. 3. The cerebral malnutrition occurring before or during certain forms of insanity. 4. Various toxaemias, e. g., malarial, urae- mic, cholaemic, and syphilitic. 5. Dyspepsia and gastric repletion. 6. Diabetes. 7. Obesity. 8. Insolation. 9. Cerebral anaemia. 10. Exhausting diseases. 11. Concussion of the brain. 12. Climatic con- ditions, cold, etc.
A very common cause of drowsiness is dyspepsia attended by some torpidity of the liver, the condition popularly known as "biliousness." Another frequent cause is malarial infection, which perhaps acts in- directly by impairing the functional activity of the liver. Drowsi- ness from these causes oftenest comes on in the afternoon. Anaemia is attended by drowsiness during the day, while there is often insomnia at night. Syphilis is more likely to cause insomnia, but in its third stage somnolent conditions may be produced which are of serious sig- nificance. Drowsiness occurs from the effects of severe cold. It some- times develops when persons change their surroundings, especially on going to the seashore, for low levels and a high degree of atmospheric pressure seem to promote sleep. The drowsy state that sometimes follows concussion of the brain is a familiar phenomenon. Some per- sons, no doubt, acquire the habit of drowsiness. At first the trouble may have been induced by indigestion, "biliousness," or malarial infection, but it persists after the cause is removed. Such persons
THE DISORDERS OF SLEEP.
can hardly sit through a lecture, a church service, or any exercise re- quiring quiet and attention. As the morbid drowsiness here described is only symptomatic, its treatment need not be discussed. Such reme- dies as coca, coffee, tea, atropine, glonoin, do not produce results equal to expectations.
Morbidly Deep Sleep. — Certain persons, when they sleep, pass into an almost lethargic slumber. Persons who sleep in this way often sleep a longer time than normal. They are awakened with difficulty and then suffer with headache or disagreeable sensations throughout the day. The symptom may be a prodroma of insanity. Instances in which persons retire at the usual hour, but can with great difficulty be roused in time for the ordinary duties of the day, are not rare. Some of these are illustrations of the vice of indolence, but in other cases there is an absolute need of nine, ten, or even fourteen hours of sleep.
This disorder of sleep is most liable to occur in the young and in those of nervous temperament. It often seems to be a congenital condition, for which nothing can be done. In other cases it results from overfeeding and indolent habits. Treatment is much the same as that indicated for sleep-drunkenness and somnambulism.
Paroxysmal Sleep, Narcolepsy, Sleep Epilepsy. — It sometimes happens that persons suffer from sudden attacks of unconquerable drowsiness; they fall off into slumber despite every effort of the will. These are more than drowy sensations, for sleep, or a state resembling it, cannot be kept off. Some of these cases are of a purely nervous character, i. e., the trouble is not due to a humoral poison or to organic disease, but to a paroxymal change in the nervous centres of a vas- cular or chemical character, causing sleep. It may be that the patient is epileptic and sleep seizure takes the place of the ordinary epileptic spasms.
Cases of epileptic sleep, or narcolepsy, and allied forms are not of frequent occurrence. Females are rather more often affected than males, and the susceptible age is from fifteen to forty. The disorder is brought on sometimes by fright, overstrain, and humoral poisons acting on a predisposed nervous system.
The course is chronic and relief is not always obtained. It should be remembered that syphilis, malaria, or anaemia, and indigestion may be elements in the trouble which are important, if not fundamental. Bromides in small doses are often useful factors in treatment. Change of occupation, of mode of life, or of climate may be essential to a cure.
Catalepsy, Trance, Lethargy. — Most of the so-called cases of pro- longed sleep, lasting for days or weeks, are cases of spontaneously
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developed mesmeric sleep in hysterical women, or cases of incipient insanity (dementia prsecox or stuporous melancholia). The phe- nomena in these cases may take the form of catalepsy, with waxy rigid- ity of the limbs, or lethargy. In cataleptic states the limbs may be placed in various positions and will remain there for several minutes (Fig. 257). In lethargy or trance states the patient may be plunged into a deep and prolonged unconsciousness, lasting from one day to several years. These are the "sleeping girls" of the newspapers. Others are persons of a too ready susceptibility to mesmeric suggestion, who get into a morbid habit of going into mesmeric sleep spontane- ously. In these states there may be a lowering of bodily temperature, slowing of respiratory and heart action, and excessive sluggishness
feL:.,. * Wi 111
Fig. 257.
of the action of the bowels. The patients can hear and may respond to suggestions, but they are apparently insensible to painful im- pressions and do not appear to smell, taste, hear, or see. The eyes are closed and turned upward, and the pupils contracted as in normal sleep. Many variations, however, occur in the physiological phe- nomena of these states.
The duration of the attacks of trance lethargy is from a few hours to ten years. Ordinarily, however, profound trance sleep lasts not more than a few days, while those cases in which the sleep is from mesmeric suggestion lasts but a few hours.
The katatonic patients after a few weeks or months gradually awake, become excited, and then pass into a condition of dementia or into catalepsy again.
Morbid Sleep from Organic Disease. — Prolonged and excessive sleep occurs as the result of syphilis of the brain, brain tumors, and
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the degenerative changes in old age and insanity. Morbid somnolence and stupor are not very frequent in cerebral syphilis, but are quite characteristic. The patient in some cases lies or sits all day in a semi soporous state; in other cases he walks about, but continually sleeps at his task. This state of partial sleep may pass off or end in complete stupor (Wood). It does not necessarily signify a serious issue, even though it last for weeks. Somnolence or sleep is a rare symptom in cases of cerebral tumors other than syphilitic. Conditions of drowsi- ness or stupor have been noted especially in tumors of the basal gang- lia and third ventricle.
Organic diseases of the brain tend to produce conditions of mental weakness, hebetude, or comatose states, rather than anything allied to sleep.
THE SLEEPING SICKNESS OF AFRICA.
The sleeping sickness of Africa is a disease, caused by a species of flagellated protozoa, belonging to the genus Trypanosoma. This parasite multiplies in the blood, and causes a slowly developing malady characterized by somnolence, mental apathy, paralysis, and finally death. The Trypanosome which causes this sleeping sickness is the T. Gambiense, first described by Dr. J. E. Dutton seven years ago.
Etiology. — The disease prevails mostly along the west coast of Africa, but it is extended, thence, to other parts of that continent, par- ticularly equatorial Africa. It was formerly confined to the negroes, but recently several Europeans have suffered from it. It affects par- ticularly natives who live upon the shore of the rivers and lakes. (Fig. 258). The Trypanosome is a worm-like parasite which is found among the red-blood cells. They are not very numerous, and several microscopical examinations may be needed to find them. In late stages of the disease, the parasite is found in the cerebrospinal fluid, while early in the stage it is found in the lymphatic glands and channels. The common way of the propagation of the Trypanosome is through the tsetse-fly, and it is conveyed back to man by the bites of these flies.
Symptoms. — It is stated by Dr. David Bruce that after the Trypano- some has infected the body, it may be from three months to three years before it gets into the cerebrospinal fluid, and begins to produce definite symptoms. During this stage of incubation, there is some enlargement of the lymphatic glands, and sometimes a fever of irregu- lar type. When the disease begins to affect the patient, he becomes apathetic and disinclined to exert himself. Headache and indefi- nite pains are complained of. The pulse is rapid and weak. The cervical and other lymphatic glands are enlarged, the gait is un-
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DISEASES OF THE NERVOUS SYSTEM.
steady and shuffling, the muscular powers diminished, the voice is weak, and tremors of the tongue are noted. The symptoms increase until after weeks or months the patient is confined to his bed, lying in a somnolent condition. The patient then begins to emaciate, be- comes semi-comatose, and in two or three weeks, the temperature falls and death occurs.
Pathology.— Dr. F. V. Mott has shown that there is a characteristic appearance in sections of the brain, which is found in no other disease. This is a condition of meningo-encephalo myelitis, which affects especially the medulla and the base of the brain. The inflammation
Fig. 258. — The blood in sleeping sickness, showing spirillum.
can be traced along the blood-vessels into the substance of the nervous system, the perivascular lymphatics being crowded with lymphocytes. Modern trypanosomiasis is, however, according to Druce, a disease of the lymphatic system, though its characteristic symptoms are shown upon the nerves and muscular system.
Diagnosis. — The diagnosis is difficult in the early stage of the disease. Later, the examination of the blood and lymphatics, and the peculiar progressive weakness of the body, tremor of the muscles, unsteady gait and somnolence are characteristic.
Prognosis. — Every case of the disease dies sooner or later, accord- ing to Dr. Bruce.
THE DISORDERS OF SLEEP.
65s
Treatment. — Up to the present time the most effective drug has been some preparation of arsenic, particularly atoxyl. The cure for the sleeping sickness is to prevent it by killing off the tsetse-fly.
Accidents of Sleep. — Owing to the fact that sleep is a resting state of the organism, and that many of its functions are lowered, or their cerebral control lessened, peculiar crises, or physiological and pathological disturbances of nervous equilibrium, occur. Attacks of gout, of asthma, and of pulmonary hemorrhage are most liable to occur during the early morning hours. Deaths and suicides occur oftener in the forenoon, but births oftener at night. Epileptic and eclamptic attacks occur with much frequency at night. Involuntary emissions of spermatic fluid, or gastric crises, and incontinence of urine are among the pathological incidents of sleep.
Disorders of the Pr^dormitium. — Sudden attacks of starting of the whole body, shock-like in character, accompanied by peculiar feeling in the head or occiput, not infrequently attack persons as they are dropping off to sleep. They are of slight significance.
CHAPTER XXIX.
CRANIOCEREBRAL TOPOGRAPHY.
The object of cranio-cerebral topography is to map out upon the scalp the underlying fissures, convolutions, and other parts of the brain. As this is for purposes of surgical operations, the mapping is done upon the shaved aseptic scalp with a soft anilin pencil dipped in strong carbolic solution (i to 4), or with a brush and carbolized tincture of iodine. The only instruments needed are a steel tape measure and an instrument of nickel-plated soft iron. This consists of a flat strip 25 cm. long and 1 cm. wide. From its middle there branches a second strip 10 cm. long making an angle of 670 with the longer strip.* Prac- tically, the principal points to be determined are the position of the longitudinal, Rolandic, Sylvian, and parieto-occipital fissures and the lower outline of the brain.
The measurements are based chiefly upon the known relations of certain landmarks on the skull to the parts beneath. These landmarks are the glabella, bregma, lambda, stephanion, asterion, and pterion, which are points at the junction of the various sutures with each other and with certain ridges or protuberances. Their position is shown in the cut (Fig. 259) except that of the glabella or prominence just above the nasofrontal suture. The inion is identical with the occipital protuberance.
The following rules are based upon the observations of Heftier, Thane, Reid, Horsley, Fraser, and myself:
I. The longitudinal fissure. This corresponds with the naso- occiptal arc.
II. The fissure of Rolando. Measure the distance from the glabella to the inion ; find 55.7 per cent, of this distance, and the figures obtained will indicate the distance of the upper end of the fissure of Rolando from the glabella. It should be about 48 mm. behind the bregma in male adults, 45 mm. in women, 30 to 42 mm. in infants and young children, respectively.
The fissure runs downward and forward for a distance of about 10 cm. measured on the scalp, the real length being about 8.5 cm. The fissure makes an angle of about 670 with the anterior part of the longi-
*Special instruments called cyrtometers have been devised by Wilson and Horsley, but are not necessarv.
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CRANIOCEREBRAL TOPOGRAPHY.
657
tudinal fissure. This direction is determined by the instrument above described or by the cyrtometer. The lower third of it is more vertical, and the lower end is 25 to 30 mm. behind the coronal suture. A line from the stephanion to the upper part of the asterion should about pass through it. The fissure is shorter in children.
III. The fissure of Sylvius runs nearly horizontally, and lies either under or a little above the uppermost part of the parieto-squamous suture. This suture, the external orbital process, and the parietal eminence are the guiding landmarks by help of which the surgeon can often operate without marking down lines on the scalp. In children the fissure is sometimes higher and more oblique.
Fig. 259. — Showing the position of the bony points on the cranium, the sutures, and the principal underlying fissures, also the basal outline of the brain.
To outline it, draw a vertical line from the stephanion to the middle of the zygoma. Draw a horizontal line from the external angular process to the highest part of the squamous suture; continue this back, gradually curving it up till it reaches the parietal eminence. The junction of the two lines will be at the beginning of the fissure of Sylvius. The vertical line indicates nearly the position of the ascend- ing or vertical branch of the fissure, which is, however, directed a little more forward, and is about 2.5 cm. (1 inch) in length. The posterior part of the line indicates the position of the posterior branch of the fissure. Reid's method of finding the fissure of Sylvius is to "draw a line from a point 1 \ inches behind the external angular process to a point f inch below the parietal eminence. The ascending branch 42
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DISEASES OF THE NERVOUS SYSTEM.
starts from a point f inch back from the anterior end of this line, and 2 inches (5 cm.) back of the external angular process."
IV. To outline the parieto-occipital fissure, find the lambda, mark a point 3 mm. anterior to it, draw a line through this at right angles to the longitudinal fissure, extending about 2.5 cm. (1 inch) on each side of the median line. This marks the position of the fissure. If the lambda cannot be felt, its position may be found by measuring the naso-occipital arc and taking 22.8 per cent, of it. This indicates the distance of the lambda from the inion or external occipital protuber-
ance. The average distance in male adults is 7.42 cm. (2 J inches). It is greater in women than in men by a little over a millimetre.
V. To outline the frontal lobes: The anterior end of the frontal lobes reaches to a point determined by the thickness of the frontal bone. This ranges from 2 to 8 or more mm. (yV to J inch). The floor of the anterior fossa reaches in front to a level a little above the supra-orbital margin (16 mm., f inch — Heftier). It slopes down and backward, its posterior limit being indicated by the lower end of the coronal suture.
VI. To outline the temporal lobe and the lower border of the cere-
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659
brum: The temporal lobe is limited above by the fissure of Sylvius, below by the contour line of the lower border of the cerebrum. This latter corresponds to a line drawn from a point slightly (about 12 mm.) above the zygoma and the external auditory meatus to the asterion,
Fig. 261. — Method of Chipault. Forty-five per cent, of median naso-inionic line = pre-Rolandic point; 55 per cent. =Rolandie point; 70 per cent. = Sylvian line; 80 per cent. = lambda; 95 per cent. =lower edge of occipital lobe. Line from external angular process, to 70 per cent. = Sylvian fissure. Junction of third and fourth tenths of this line represents point of lower end of fissure of Rolando.
and continued on along the superior occipital curve to the inion. The anterior border of the lobe corresponds to the posterior border of the orbital process of the malar bone.
The temporal lobe is about 4 cm. (if inches) wide at the external
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DISEASES OE THE NERVOUS SYSTEM.
auditory meatus. A trephine, as Bergmann states, placed half an inch above the meatus would enter the lower part of the lobe. The middle of the lobe is in a vertical line from the posterior border of the mastoid process. A line from the upper end of the fissure of Rolando to the point of the process would pass through this important sensory area (Fig. 259; see also p. 454).
VII. To find the position of the central ganglia, viz., corpus stria- tum and optic thalamus, draw a line from the upper end of the fissure of Rolando to the asterion, practically a vertical line. This limits the optic thalamus posteriorly. A vertical line parallel to the first, a little in front of the beginning of the fissure of Sylvius, limits the corpus striatum anteriorly. A horizontal plane 45 mm. (1 J inches) below the surface of the scalp at the bregma limits the ganglia superiorly. The ganglia lie about 35 mm. (ij inches) below the superior convex sur- face of the brain (Fere).
VIII. To reach the lateral ventricles: A number of routes may be taken. The lateral is recommended by Keen. Mark a point inches behind the external auditory meatus and 1^ inches above a base line made by drawing a line through the lower border of the orbit and the external auditory meatus. Trephine at this point and plunge the director into the brain in the direction of a point 2\ to 3 inches verti- cally above the opposite external meatus. The ventricle lies at a depth of 2 to 2\ inches (5 to 5.7 cm.).
Mr. Alec Fraser has devised a way of mapping out the fissures by means of a series of composite photographs, so taken as to show the relation of the underlying parts to certain tapes tacked upon the skull. One of his figures is reproduced here (Plate III.).
In applying this method the surgeon tacks the tapes on the shaved scalp. Then looking at the diagram, he finds where the point in the brain is that he wishes to reach and notes its relation to the median lateral circumferential tapes. Then as the circumference of the il- lustrated head is to the circumference of the living one, so is the posi- tion of the area on the tapes in the illustrated head to the desired posi- tion of the same area in the living one. The illustration is a com- posite of several adult heads varying in circumference from 20J to 23^ inches.
The tapes are divided into inches and half-inches. The primary tape is the circumferential passing horizontally round the vault of the head (on the shaven scalp) from the root of the nose (glabella) to the maximum occipital point, which is about one inch above the inion. The tape which thus entirely surrounds the head is divided into four equal parts. The points where the division is
NERVOUS DISEASES PLATE III. DANA.
COMPOSITE PHOTOGRAPH SHOWING RELATIONS OF CRANIAL SURFACE TO THE FISSURES AND CONVOLUTIONS (alec Phaser)
CRANIOCEREBRAL TOPOGRAPHY.
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made are at the anterior and posterior poles and midway on each lateral half of the tape. From these lateral mid-points a tape is run vertically over the top of the head, and other tapes are run anteriorly and posteriorly half-way between the point where the transverse tape crosses the sagittal suture and the anterior and posterior poles. An- other longitudinal tape is run from the anterior to the posterior pole half-way between the sagittal suture and the circumferential tape.
The cranial measurements necessary to indicate the location of the important fissures and convolutions are given above. There are a number of other methods, but no very important improvements upon the original one of Reid. I have repeatedly verified the methods given and see no reason to change them. Some surgeons, however, prefer the Anderson-Makin. According to this, the upper end of the fissure of Rolando corresponds to a point one-half to three-quarters of an inch behind the mid-point between the inion and glabella. The up- per extremity of the parieto-occipital fissure corresponds to a point one quarter of an inch behind the mid-point between the line of the Rolandic fissure and the inion. As surgical approach to brain tumors is now done by large bone flaps, Dr. Charles K. Mills has outlined the various areas to be attacked, corresponding to the prefrontal, post- frontal, parieto-temporal, mid-frontal, parietal and occipital areas. Having determined the location of the Rolandic, Sylvian and parieto- occipital fissures, the location of these flaps is easily determined. Froriep has shown that in long brains (not dolichocephalic but abso- lutely long) the Rolandic fissure lies a little further back; the variation, however, between long and short brains does not range above 40 mm.
CHAPTER XXX.
ON NEUROLOGICAL THERAPEUTICS.
It has seemed best to describe, in a separate chapter, some of the technical details of the treatment of nervous diseases.
Hydrotherapy.
The apparatus needed for applying water in therapeutics consists of a room at least sixteen by twenty feet, well ventilated, with water- proof floor and walls. The floor must be made so that it will drain away the water flowing upon it. The room should contain a stationary bathtub, a shower or rain bath, an apparatus for giving hot and cold douches at various pressures, a hot box in which a patient can take a hot bath with the head exposed, a few foot baths, ice bags and ice caps. The most important point in detail is the douche apparatus, which should be supplied with all the improvements for regulating the heat, pressure, and impact of the stream. The stationary bathtub should be of the largest possible size, rather shallow, and placed low down so that patients can move their limbs easily and get exercise while in it. Among the accessories may be a "vaporium," apparatus for subjecting the limbs or joints to very high temperature.
While all the foregoing things are needed in an institution and should be placed in every hospital and asylum, most of the hydro- therapeutic procedures can be carried out fairly well with a stationary tub and a shower above it. A cheap hot box with a hole in the top and a lamp for heating below can be easily added if needed. The common forms of hydrotherapy prescribed by myself are the luke- warm tub bath followed by cool spray or affusion, the hot box and Charcot or Scottish douches, the cold sitz bath, the drip sheet, and wet pack.
Special Applications op Hydrotherapy — Epilepsy. — Most pa- tients should be made to take cold showers or sponge baths with a vigorous rub-off daily in the morning. Once a week they should take a hot bath. A cold plunge into a tub full of water is a good substitute for the shower. Persons who have neither shower nor tub should get a foot tub and a large sponge. Standing in the tub with a basin of cold water before them, they should fill the sponge and let the water trickle over the head, back, and body generally. This is done for two
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NEUROLOGICAL THERAPEUTICS.
663
or three minutes, then the patient rubs himself down. The foot tub may have a little warm water in it at first.
With delicate persons hydrotherapeutic treatment must be begun carefully. The first applications should be dry, warm flannel packs, then wet packs gradually made cold. Finally, cold drip sheets, show- ers, plunges, and rubbing may be used.
A good method for a fairly robust person is that originally described by Fleury. This consists in giving simultaneously the rain shower and the jet. The patient standing in the shower receives a jet of water on the posterior surface of the body for fifteen seconds; then the jet alone for fifteen seconds; finally, the jet alone on the anterior surface of the body for thirty seconds.
In neurasthenia of adult life in men the patient should take the cold shower or plunge daily. In addition to this or in place of it, if needed, I strongly advise the Scottish douche thrice weekly or oftener. This may be preceded by a short hot bath. In neurasthenia of adolescence the same treatment is often indicated, but it must be applied more carefully, as many patients do not react well at first. They may re- quire wet packs and lukewarm baths as sedatives for a time. Nervous women almost always do better with a preliminary course of dry and wet packs, followed later by showers and douches. Thus I prescribe first a hot flannel pack for one-half to one hour; next day a wet pack, using a hot sheet; next day a cooler sheet, until in one or two weeks the patient takes a cold wet pack thrice weekly. If this, how- ever, does not lessen irritability and quiet the patient, I prescribe a hot- air bath followed by a shower or douche.
Insomnia. — The lukewarm bath at a temperature of 900 to 960 F. is often efficacious. It should be taken at night before retiring, and should last from fifteen minutes to three-quarters of an hour. A cold cloth may be laid on the patient's head. A simpler method of induc- ing sleep is to make the patient thrust the feet into a basin of cold water, 400 F. to 500 F. The legs are sponged up to the knees. This is done for fifteen or thirty seconds, then the feet are taken out and briskly rubbed. The most efficacious measure often is the hot wet pack. The pack is taken like a cold pack, only the sheet is wrung out in hot water. The patient lies upon this, and the sheet and then the blanket are folded about him. The duration is an hour or all night. In the rest- cure treatment a drip sheet is used (see Partial Rest-cure, p. 668).
Headache of a congestive character is helped by cold foot baths or cold douches to the feet; or a bath at 6o° F., which should last ten or twenty minutes and be accompanied by friction to the feet and legs. In anaemic headaches the head should be wrapped in thin linen ban-
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DISEASES OF THE NERVOUS SYSTEM.
dages wrung out in very cold water and covered with a few layers of flannel. After they are removed the head is to be rubbed dry and covered with a dry cloth.
In hysteria much the same kind of treatment is indicated as in neurasthenia. When there is much excitement the wet packs are indicated. In most of the major forms a half-bath at 6o° with cold af- fusions, or the shower, with Charcot or Scottish douche, should be given. The ordinary treatment at the Montefiore Home consists of:
Cold affusions while standing in warm water, or a hot-air bath fol- lowed by rain bath for thirty seconds at 850, daily reduced until 6o° is reached, this to be followed by a spray douche for five seconds at 650 or jet douche for three seconds at 650 to 550. The douche is reduced gradually to 500 or less, increasing the pressure from two pounds to thirty (Baruch).
When there is spinal irritation the filiform douche may be used as a counter-irritant, or the shower at 650 to 850.
In locomotor ataxia different patients get relief from different forms of treatment. The very painful and hyperaesthetic cases are not in my experience much helped by hydrotherapy. One may try, however, lukewarm baths, 850 F. to 950 F., for ten to twenty minutes, with or without pine-needle extract. The carbonic acid baths given after the Nauheim method act favorably in some cases. I do not find that they are much better on the whole than a luke-warm saline bath. For leg pains, hot-air baths to the legs alone, followed by affusions at 6o° to 700, are recommended. The extremities may also be wrapped in flannels wrung out in hot water and covered with dry cloths. Some patients are greatly refreshed and helped by cool affusions, 700 to 8o°, poured over the back and legs. In painful cases, relief can be ob- tained sometimes by applying the Charcot douche at a very high tem- perature to the back. The temperature at first is 900 F. This is gradually increased to about 1600 F. The duration of treatment should be only about one minute.
Spermatorrhoea. — Cold sitz baths may be given for from five to twenty minutes, 500 to 700, daily at bedtime.
Impotence. — Brief cold sitz baths twice or more daily, at 560 to 640, for from one to two minutes. The psychrophore, i.e., application to the prostate of cold by a rubber condom or bladder secured over a rectal irrigator au double courant, is sometimes helpful.
Incontinence of Urine. — In paresis of sphincter or detrusor, brief cold sitz baths, daily, 560 to 640, one to five minutes, are indicated; also cold rain baths (500 to6o°),cold wet packs applied locally with douches as general tonics. In spasmus vesicae, on the contrary,
NEUROLOGICAL THERAPEUTICS.
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prolonged lukewarm sitz baths daily for thirty to sixty minutes should be given.
The Rest Treatment.
This form of treatment was devised and perfected by Dr. S. Weir Mitchell and has been popularized by him and his pupils. Its value in many types of neurasthenia and hysteria, especially among women, is unquestioned. It seems more difficult to apply it to men, though it is often useful with them.
Among women it answers best in my experience for young women or those this side of middle life, of not very forceful character and not very strong will. The active, keen-witted, intellectual woman who suffers from headache, brain tire, and exhaustion from slight exertion does not do so well under a method which for a time renders the patient entirely abulic.
The essential features of the rest cure are: Isolation, diet, rest in bed, massage, electricity, and the energizing personality of a wise physician.
Isolation. — The patient can rarely be treated successfully at home, even if the relatives are not allowed to see her. The best place is a comfortable room in a boarding-house or private hospital. A private room in a general hospital may answer. A special nurse is necessary, and she should be young, neat, careful, sufficiently intelligent and tactful, but not overeducated or one who feels too keenly her social position. Decayed gentlewomen are sometimes very interesting char- acters, but they make poor nurses. She should be a stranger to the patient, rather than one who has nursed the patient in previous illnesses. She should preferably know how to give massage, electric- ity, and the simpler forms of water treatment. The patient sees only the doctor and a masseuse if the nurse does not know how to give this treatment.
The diet should consist as far as possible of milk. During the first two weeks this is especially important. Skim-milk is used and it should be perfectly fresh. Four ounces are given at first every two hours. This amount is increased to two or four quarts a day. The milk may be treated in various ways in order to make it more pal- atable or digestible. The addition of a little salt or lime water, or of tea, coffee, cocoa, or Vichy water, accomplishes this end. It may be varied with malted milk, dextrinized barley, Koumiss or some other form of fermented milk or it may be mixed with barley or rice water. Two or four ounces of liquid malt may be given before the milk three times a day. At the end of a week a pint of beef tea is
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DISEASES OF THE NERVOUS SYSTEM.
added. It is made (Mitchell) by chopping up one pound of raw beef and placing it in a bottle with one pint of water and five drops of strong hydrochloric acid. This mixture stands all night, and in the morning the bottle is set in a pan of water at no° F. and kept two hours at about this temperature. Strain through a stout cloth and squeeze the mass till nearly dry. The resulting fluid is given in three portions daily. If the taste be objected to, the meat may be roasted a trifle on one side, or the ordinary commercial extracts may be used. It is better at first for the patient to be fed by the nurse. The milk and other food should be taken slowly.
Rest. — It is extremely important that the patient be made to go to bed and lie flat on her back, not even sitting up to be fed. The object is not only to secure absolute rest, but to make the patient feel that she is in the hands of her physician, who is to manage her till she is well. The enforced quiet also adds to the desire later to get well and regain her freedom. She is to be kept in bed for a month; then allowed to sit up for ten minutes twice a day, this time being lengthened by five or ten minutes daily. After five or six weeks she may be allowed a drive or a short walk. About this time one must also begin systematically to enforce walking and other exercise, and the patient must be taught to ignore the slight pain and fatigue that at first ensue.
Massage. — The system of massage recommended by Mitchell is simpler than that ordinarily used by the Swedish professionals, and consists mainly of kneading and centripetal friction. It is given at least an hour after a meal and lasts at first fifteen or twenty minutes once a day. In a few days this is increased to three-quarters of an hour and an hour. The legs are massed first, then the abdomen, chest, and arms. The head and neck are not touched. No ointment or lubricating substance is used. Particular attention is paid to the abdomen in order to prevent constipation. After massage there should be a rest of an hour. After four or eight weeks, Swedish move- ments are added to the massage.
Electricity. — The faradic current is used with a primary coil and a slow interrupter. In very sensitive persons a long secondary coil with fine interruptions is better. Electrodes of the "normal" size are em- ployed and each segment of the limbs is gone over, beginning with the periphery. The two electrodes are placed over the muscles a few inches apart and each muscle is contracted four or six times. The question of poles may be ignored. The feet, legs, abdomen, back, and arms are gone over successively, then the fine secondary current is turned on. A large electrode, of the "indifferent" size, is placed on the back of the neck, and the other electrode placed on the sole of one
NEUROLOGICAL THERAPEUTICS.
667
foot; a current is passed for seven and a half minutes; then the elec- trode is shifted to the other foot and the current given for the same time. It is best for the physician himself to give the electricity if possible. The duration of each seance is from three-quarters to one hour, and it is continued for six weeks. Very mild currents are used at first.
The duration of the rest-cure should never be less than a month. It is usually about six or eight weeks. A typical schedule for a rest- cure patient as given by Dr. John K. Mitchell is the following:
7 a.m. — Cocoa.
Cool sponge bath with rough rub and toilet for the day.
8 a.m.— Milk, breakfast.
Rest an hour after.
10 a.m. — 8 oz. peptonized milk.
11 a.m. — Massage.
12 m. — Milk or soup.
Reading aloud by nurse. 1.30 p.m. — Dinner.
Rest an hour. 3.30 p.m. — 8 oz. peptonized milk. 4 p.m. — Electricity. 6 p.m. — Supper with milk.
8 p.m. — Reading aloud by nurse one-half hour.
9 p.m. — Light rubbing by nurse with drip sheet.
8 oz. malt extract with meals, tonic after meals. 8 oz. peptonized milk with biscuit at bedtime and a glass of milk during the night if desired.
Laxative: cascara, 10 to 30 drops p. r. n. Later Swedish move- ments are added to the massage.
Additional Measures.— A sponge bath is given every morning. In- somnia is to be feared at the start, and for this bromide of sodium may be given in doses of gr. xxx. at 6 and 9 p. m. and gradually decreased grain by grain ; or sulphonal (gr. xx. to xxx.) in hot water, or trional (gr. xv.). Some form of hydrotherapy may answer better than drugs, and a favorite measure is the drip sheet.
The following are the directions for its use (Mitchell) :
Basin of water at 650 F. Lower the temperature day by day by degrees to 550 F., or to still less. Put in the basin a sheet, letting the corners hang out to be taken hold of. The patient stands in one gar- ment in comfortably hot water. Have ready a large soft towel and iced water. Dip the towel in this, wring it, and put it turban-wise about the head and back of the neck. Take off nightdress. Standing
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DISEASES OF THE NERVOUS SYSTEM.
in front of patient, the basin and sheet behind, the maid seizes the wet sheet by two corners and throws it around the patient, who holds it at the neck. A rough, smart, rapid rub from the outside applies the sheet everywhere. This takes but two minutes or less. Drop the sheet, let the patient lie down on a lounge upon a blanket, wrap her in it, dry thoroughly and roughly with coarse towels placed at hand. Wrap in a dry blanket. Remove ice wrap; dry hair; put on nightdress. Bed, the feet covered with a flannel wrap.
As tonics, lactate, pyrophosphate, or subcarbonate of iron are given in doses of gr. xxx. daily. Small doses of strychnine, salicin or quinine, or dilute phosphoric acid may be useful.
The partial rest-cure is indicated in the milder cases of neurasthenia and hysteria. The following is the schedule given by Dr. Weir Mitchell:
a.m. — On awaking, cup of cocoa. Take bath. (Temperature given.) Lie down on lounge while using drying-towels; or, better, be sponged and dried by an attendant. In this process the surface to be rubbed red, or, if drying one's self, to use flesh brush. Bed or lounge again. Breakfast. Before each meal take three ounces of malt ex- tract; aperient at need in malt. Tonic after each meal. Detail as to breakfast diet. If eyes are good, may then read seated in bed. At 10 to ii a. m., one hour's massage. Rest one hour; may be read to, or read if eyes are good, or knit. At this time, ii a. m., four ounces of beef soup or eight ounces of milk. At noon may rise, dress slowly, resting once or twice a few minutes while dressing, and remain up until 3 p. m. See children, attend to household business; see one visitor, if desirable. From i to 1.30 p. m. malt, etc., and lunch. Detail as to diet. At first, as a rule, let this meal represent dinner. Tonic, and after it to rest on a lounge, occupied as above, reading or being read to. If possible, drive out or use tramway, so as to get air. Walk as little as possible. On return from drive repeat milk or soup. About 5 p. m., electricity, if used at all. Rest until 7 p. m. Supper at 7 p. m. Detail as to meal. Malt as before, with or without aperient, as occasion de- mands. Tonic. To spend evening with family as usual. Best not to use eyes at night for near view. Bed at 10 p. m. No letters to be written for two months, when most of these details have to be revised.
After two months of massage it should, in these cases, as in com- plete rest, be used on alternate days, and by degrees given up. If the nurse or masseuse is able to teach the patient the use of Swedish move- ments, it is desirable that these or some definite slowly increased system of chamber gymnastics be continued for months. Finally, walking must be resumed with slow and systematic increase. After
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669
the second month, write out a schedule of less restriction, to be fol- lowed for six months.
The Exercise Treatment of Locomotor Ataxia. — The treatment of locomotor ataxia by means of systematic exercises for training the ataxic limbs often produces some very satisfactory results. The method was elaborated first by Dr. Frankel, and still further by Dr. Hirschberg. For the convenience of students and readers, a schedule of the exercises which I prescribe, and which are based, more or less, upon those of the authors mentioned, is appended here.
The exercises are usually to be taken twice a day, and each exercise is to be done with the utmost care and precision by the patient.
Exercises for the Hands and Arms. — 1. Sit in front of a table, place the hand upon it, then elevate each finger as far as possible. Then, raising the hand slightly, extend and then flex each finger and thumb as far as possible. Do this first with the right and then with the left. Repeat once.
2. With the hand extended on the table, abduct the thumb and then each finger separately, as far as possible. Repeat three times.
3. Touch with the end of the thumb each finger tip separately and exactly. Then touch the middle of each phalanx of each of the four fingers with the tip of the thumb. Repeat three times.
4. Place the hand in the position of piano playing and elevate the thumb and fingers in succession, bringing them down again, as in striking the notes of the piano. Do this twenty times with the right hand, and same with the left.
5. Sit at a table with a large sheet of paper and pencil, make four dots in the four corners of the paper and one in the centre. Draw lines from corner dots to centre dot with right hand ; same with left.
6. Draw another set of lines parallel to the first with the right hand ; same with left.
7. Throw ten pennies upon the paper, pick them up and place them in a single pile with the right hand; then with the left; repeat twice.
8. Spread the pennies about on the table, touch each one slowly and exactly with the forefinger of right hand ; then with forefinger of left.
9. Place an ordinary solitaire board on the table, with the marbles in the groove around the holes. Put the marbles in their places with right hand; same with left hand. Patient may, with advantage, practice the game for the purpose of steadying his hands.
10. Take ordinary fox-and-geese board with holes and pegs, and , beginning at one corner, place the pegs in the holes, one after the other, using first the right hand, then the left.
These exercises should be gone through with twice a day, and should be done slowly and carefully, with a conscious effort every time of trying to do one's best.
Exercises for the Body and Lower Limbs. — 1. Sit in a chair, rise slowly to erect position, without help from cane or arms of chair. Sit down slowly in the same way. Repeat once.
670
DISEASES OF THE NERVOUS SYSTEM.
2. Stand with cane, feet together, advance left foot and return it. Same with right. Repeat three times.
3. Walk ten steps with cane, slowly. Walk backward five steps with cane, s1owly.
4. Stand without cane, feet a little spread, hands on hips. In this position flex the knees, and stoop slowly down as far as possible, rise slowly; repeat twice.
5. Stand erect, carry left foot behind, and bring it back to its place ; the same with the right. Repeat three times.
6. Walk twenty steps, as in exercise No. 3 ; then walk backward five steps.
7. Repeat exercises No. 2, without cane.
8. Stand without cane, heels together, hands on hips. Stand in this way until you can count twenty. Increase the duration each day by five, until you can stand in this way while one hundred is being counted.
9. Stand without cane, feet spread apart; raise the arms up from the sides until they meet above the head. Repeat this three times. With the arms raised above the head, carry them forward and down- ward, bending with the body until the tips of the fingers come as near the floor as they can be safely carried.
10. Stand without cane, feet spread apart, hands on hips; flex the trunk forward, then to the left, then backward, then to the right, making a circle with the head. Repeat this three times.
11. Do exercise No. 9 with heels together.
12. Do exercise No. 10 with heels together.
13. Walk along a fixed line, such as a seam on the carpet, with cane, placing the feet carefully on the line each time. Walk a distance of at least fifteen feet. Repeat this twice.
14. Do the same without cane.
15. Stand erect with cane; describe a circle on the floor with the toe of right foot. Same with toe of left. Repeat twice.
Between the fifth and sixth exercises the patient should rest for a few moments.
Lumbar Puncture of the Spinal Cord. — Paracentesis of the spinal dura mater has become an important factor in diagnosis, but as yet it has not proved of much therapeutic value. It is a simple operation and harmless if carefully done except in brain tumors.
In making the puncture, asepsis should be observed, and the operation should be done at home or in a hospital. The patient should lie on his left side with his lumbar spine flexed well forward ; the needle is cautiously inserted to a depth of 5 cm. between the arches of the third and fourth or fourth and fifth lumbar vertebrae near the spinous processes.
PART II.
DISEASES OF THE MIND.
CHAPTER I.
DEFINITIONS, CLASSIFICATION, GENERAL ETIOLOGY, GENERAL PATHOLOGY.
Definitions. — Insanity, medically speaking, is a serious alteration in the psychical functions of the brain. This leads to such departure from the normal in speech and conduct that the patient can no longer adapt himself efficiently to his environment.
Insanity, legally speaking, is a condition of mental unsoundness and irresponsibility, by reason of which a person is unable to under- stand the nature of his act or to pass a fairly rational judgment on its consequences to himself and others, and is not a free agent as far as the act is concerned. Thus the "mental unsoundness" of the law is not the same as insanity, since a person suffering, for example, from melancholia may be technically and medically insane, yet still be able to answer the legal test.
Idiocy, imbecility and feeble-mindedness are different degrees of de- fective mental development. Idiocy is the most serious defect and feeble-mindedness the least. The exact line of demarcation is an ar- bitrary one.
Dotardness is sometimes for practical purposes distinguished from insanity; it is the physiological dementia of extreme old age. Its dis- tinction from senile dementia is an arbitrary one.
Classification. — Mental disorders are divided into two great groups: the major psychoses or insanities, and the minor psychoses, or psycho-neuroses. The major psychoses include all those disorders which are commonly and technically regarded as insanities; those in which the disorder of the mind is such that the patient lacks responsi- bility and capacity to care for himself. The minor psychoses include all those disorders of the mind which are not insanities, in the strict meaning of the term, and in which the patient practically is still a
671
672
DISEASES OF THE MIND.
responsible person. These neuropsychoses include a large part of what has been known as neurasthenia, hysteria, and the majority of a group of disorders known as neurasthenic insanities, phrenasthenia, psychasthenia, also many mild forms of melancholia, dementia, and abortive types of the major psychoses.
The distinction between the major and minor psychoses is not an absolute one nor even a scientific one and one type may be grafted upon another. Some psychoses like melancholia may take a very mild course and not even interfere with the ordinary activities of life, or they may render the person absolutely irresponsible. Nevertheless, it is of practical importance to establish the general distinction, and especially for patients to understand that a person may be in a mor- bid mental condition and yet not be insane or liable to the develop- ment of any serious insanity.
The principal types of insanities are:
A. The Functional Insanities. — 1. Dementia praecox (precocious dementia), a very general term which includes all the different forms of early dementias, viz. :
Simple precocious dementia.
The excited form — hebephrenia.
The stuporous form — katatonia.
The delusional form — dementia paranoides.
2. Paranoia.
3. Phrenasthen a, sometimes called neurasthenic insanity or degen- erative insanity; now generally called psychasthenia.
4. Manic-depressive insanity, including the: Mania-melancholia type.
Simple melancholia type.
Melancholia of involution or chronic melancholia.
5. Toxic, . exhaustion, and infectious insanities (confusional
psychoses).
B. The Organic Insanities. — Paralytic dementia (general paresis). Epileptic dementia.
Senile dementia.
Insanity from gross brain lesions, such as tumors, hemorrhages, and injuries.
C. Organic Defect of Development. — Idiocy, imbecility.
Besides these terms wTe have names to indicate symptom groups or psychical syndromes: Delirium.
Maniacal excitement. Dementia.
ETIOLOGY.
673
Confusional states. Cerebral automatism, etc.
Various symptom-groups occur with hysteria, epilepsy, chorea, trauma, senility, the puerperium and the climacteric, but they do not justify forming special groups of insanities, except for purposes of con- venience in description.*
Etiology. — Sex. — Insanity occurs with about the same frequency in male and female, with a slight preponderance in favor of the male.
Age. — It occurs most frequently between the ages of thirty and forty (twenty-eight per cent.), next between twenty and thirty. It is rare in childhood (five per cent, under twenty) and old age (four per cent, between seventy and eighty). Before twenty it occurs oftenest in the eighteenth or nineteenth year.
Civilization. — Insanity is much more frequent in civilized coun- tries, ranging in frequency from one in three hundred to one in five hundred of the population. It is now slowly increasing relatively to the population, but it is most probable that this increase will be- fore long cease and the tide set in the other way through the influences of a better knowledge of how to live.
Congenital Conditions. — The existence of a nervously unstable or neuropathic constitution is the most important of the factors that pre- dispose to insanity. Many people have this constitution and live their lives sanely. Nevertheless, it probably always exists in those who develop functional insanities, and in many who suffer from the
* Among the insane received from April, 1903, to April, 1904, at the psychopathic wards of Bellevue Hospital the distribution of types was as follows: the mania and 'mel- ancholia representing various types of manic, depressive, simple, and involutional melancholia:
|
Male. |
Female. |
Total. |
|
|
131 |
223 |
354 |
|
|
Melancholia |
57 |
136 |
193 |
|
Primary and confusional |
34 |
38 |
72 |
|
60 |
86 |
146 |
|
|
64 |
36 |
100 |
|
|
201 |
36 |
237 |
|
|
32 |
47 |
79 |
|
|
94 |
99 |
i93 |
|
|
Toxic |
206 |
184 |
39o |
|
179 |
203 |
382 |
|
|
9 |
4 |
13 |
|
|
20 |
14 |
34 |
|
|
Total |
2,i93 |
||
43
6/4
DISEASES OF THE MIND.
organic forms. This predisposition shows itself by an easy exhausti- bility of the brain, by an intemperate and excited manner of using the mind, indicating a lack of inhibition; also by its over-reaction to fevers, drugs, and intoxicants, by its sensitiveness to disturbances of metabol- ism, such as result in excess of uric acid and its allies, by a lack of social feelings, excessive self-consciousness and egoism, self-inspection, desultoriness of purpose, and abnormal sexual life, moral defects, and excessive emotionalism.
Some differences in these characteristics are found according as the patient tends to develop periodical melancholia, precocious de- mentia, paranoia, or phrenasthenia.
Heredity. — In about twenty-five per cent, of the insane a distinct history of insanity or of some serious neurosis is found in the antece- dents. This represents the influence of direct heredity. But this does not indicate the real fact as to hereditary and congenital in- fluences. In the vast majority of persons who become insane the brain is congenitally defective or lacking in power of resistance. The original endowment of the patient mentally and cerebrally is the most impor- tant single factor in the causation of his insanity. It does not follow, however, that this 25 per cent, of insane must have become so by virtue of their heredity. A large number could be saved by proper attention to mental hygiene and the avoidance of exciting causes.
. 1 Icohol. — The abuse or even more than simply temperate use of alcohol is an extremely important factor in leading to degeneracy, and in producing a brain susceptible to insanity. The use of alcohol is especially important as an indirect factor in leading to the production of weakly and neuropathic offspring, and also as a direct cause of in- sanity through overuse. The excessive use of alcohol often does more psychic harm to the descendants than to the individual himself. About five per cent, of serious psychoses are directly due to alcohol. Another five per cent, or more of cases of temporary toxic conditions or degenerative disorders are due to alcohol.
Syphilis. — Syphilis is less potent as a hereditary factor, but as a cause of paresis, which now makes up ten per cent, of the insanities, it ranks among the important producers of mental disease.
Climate. — Insanity occurs with nearly equal frequency in warm and cold climates, the civilization being about the same; rather more cases occur in summer than in winter, but insanity is little influenced by the weather and not at all by the moon.
Race. — There is no very great difference in insanity among the different races, except in so far as they are subject to different con- ditions of living. Certain races have more insanity because they have
GENERAL PATHOLOGY.
67s
been subjected to more trying conditions of life. Even savage and backward races, if put under civilized conditions, develop insanity; thus the negro when he gets in urban communities shows his full proportion.
Education. — Education as a whole is a beneficent influence in les- sening insanity, just as are wealth and social prosperity. But both education and wealth can be made the means of developing psychoses and degenerative constitutions.
Urban Life. — There is rather more insanity in urban than in rural populations, and there would be still more if the cities did not draw the good blood of the country to itself. Urban life, certainly of itself, is more productive of insanity than rural life.
Occupation. — Certain callings furnish relatively more insane than others. Among the educated classes it is no doubt artistic pursuits, including the dramatic. Among the mechanic and artisan class it is railroad men and engineers, workers in the dangerous industries, such as lead, aniline dyes and mercury. According to Berkley, the lower the social stratum the greater the percentage of psychoses. According to Hill the professional class is relatively most susceptible, and farmers, including farmers' wives, are among the least.
Psychogenetic Causes. — Many cases of insanity are due to the con- tinued indulgence in morbid mental habits, the cultivation of moods and the unbalanced development of the different instincts, such as the social, sexual and religious.
The direct and accessory causes of insanity are many. They are syphilis, alcohol, narcotic drugs, severe mental strain, depressing emo- tions, shocks, trauma, operations, severe infectious sicknesses, auto- toxaemias, and certain organic diseases of the brain.
General Pathology. — The insanities fall into two principal groups: the one of functional and developmental origin, the other of organic origin ; but these groups shade into each other. The two types are represented by a developmental insanity, like paranoia, and by an organic insanity, like general paresis. The developmental insanities have no known anatomical base. Such lesions as are found are sec- ondary. There is, however, something wrong or defective in the ar- rangement and make-up of the brain neurones, or of their vascular and lymph supply. In some cases, like the non-dementing insanities, such as phrenasthenia, or paranoia, the trouble is perhaps an absence or mis- placement of certain association tracts, so that normal responses and inhibitions do not come from the ordinary stimuli. In other cases the neurone is short-lived, or, to use the word coined by Gowers, there is an "abiotrophy." Certain neurone groups become prematurelv senes-
676
DISEASES OF THE MIND.
cent and die at the age of twenty-five instead of seventy years. This condition underlies the precocious dementias.
Again, the trouble may be in defects of vascular supply or in the lymph channels. As a result, the brain neurones are not nourished sufficiently, or the products of tissue waste clog the lymphatics and the cells become poisoned, just as occurs in the later toxic insanities.
vStill further, the insane may have defective excreting organs. The products of digestion are imperfectly neutralized by the liver or carried off by the absorbents, or the kidneys may not excrete freely the prod- ucts of tissue waste. There are certain glands of the body, like the thyroid, and perhaps the adrenals, which may act a part even in in- citing the developmental insanities.
But, after all, it is the too easy exhaustibility of the neurones which seems to be the most serious trouble. Whether this be due to the in- trinsic weakness of the cell or of the vascular and lymphatic systems is not known.
The anatomical changes of the organic and specifically toxic in- sanities will be described later.
CHAPTER II.
GENERAL PSYCHOLOGY.
For a sound knowledge of neurology one must know anatomy; but for a sound understanding of mental disorders we must know, and, as far as possible, agree upon psychology and psychological terms. Neurology is based on a knowledge of structure and the significance of its changes; but psychiatry is almost purely a clinical science, and its acquirement depends on an understanding of symptoms. The symp- toms being due to a perversion of the normal activities of the mind, it follows that a knowledge of the normal is necessary. A great deal of confusion in description and classification has resulted from the dif- ference among writers in their stand-point toward psychology. Hence I make no excuse for giving some account of the elementary facts of this science. The works, especially of James, Baldwin, Marshall, Stout, Scripture, Calkins, and Cattell, are to be credited here.
In the physiology of the nervous system we find three groups of phenomena: those connected with the sensory and other ingoing im- pulses to the nerve-centres; those connected with the motor and other outgoing impulses from the nerve-centres to the different muscles, viscera, and glands; and the reflex and other intermediate and as- sociative functions, which result in combining the first and the second.
In psychology we have to deal with the phenomena which belong to the third group, that is to say, those of recording, associating, and coordinating the afferent and efferent impulses. The work of the mind lies between the sensation started by an ingoing impulse and the mo- tion produced by an outgoing impulse. All that goes on, for example, between a visual stimulus and the resulting voluntary act belongs to psychology. Such visual stimulus gives to us a conscious sensation which is elaborated into a percept, and further into an idea. It leads to associations which arouse desire and result in purposeful acts. The difference between the instantaneous reaction or "jump" of a marble as it is dropped on a stone and the volitional reaction of the brain to a sensation when a marble is dropped on the hand, is accomplished be- cause of the infinitely more complex processes possible to occur be- tween the receipt of a stimulus by the sensory nerve and the coordi- nated movement of the hand which follows.
677
678
DISEASES OF THE MIND.
Some of the phenomena of the mind are grouped especially about the ingoing, some about the outgoing, and some about the interme- diate processes.
Thus we have on the ingoing side sensation and perception. In the associative and coordinating sphere: ideation, judgment, memory, as- sociation of thought, feeling, sentiment, and emotion. On the out- going side : instinctive acts, automatic acts, and volitional acts.
Associated with all these phenomena is consciousness.
Consciousness is the name given to that quality of the mental state through which a person becomes aware of himself as one distinguished from the rest of the world. It is the distinctive characteristic of mental life. This conscious state, or state of "awareness," constantly changes, and each state represents but a moment of time. It accom- panies all our volitional acts, but there is a large amount of mental activity that goes on unconsciously.
Sensations are simple conscious states aroused by the excitation of a sense organ or other afferent stimulus. Pure sensations from the out- side world are rarely experienced, because they are at once grouped with other mental and previously recorded impressions into percepts. When sensations are referred to an external object they are called special; when referred to the body they are called general.
Perception. — On seeing or feeling an object we instantly associate the sensation with previously recorded impressions. In doing this we perform the act of perceiving, and the result is called a percept. Thus we see a round, yellow object, and immediately associate it with previous experiences, which tell us that it is rather rough to touch and of peculiar odor and taste, and we perceive that it is an orange. Thus the process of perception is the grouping of a sensation with previously recorded impressions, the result being that we recognize a particular object which, in perception, is always before us in space.
An idea is the reproduction, with a more or less adequate image, of an object that is not present to the senses. A percept, on the other hand, or a perception, as the term is sometimes used, is a representation in the mind of an object that is present before us in space. We per- ceive the orange; we have an idea of what an orange is. In the build- ing-up of an idea we group together or associate our various sensory and perceptive experiences until we finally get this special notion or idea, which comes to our mind without having the object itself before us. The term "apperception" is used by some, but its meaning is generally a vague one to most minds, and it is not a term needed in the descriptions of morbid psychology.
A concept is an abstract or universal idea, recognized apart from any
GENERAL PSYCHOLOGY.
679
special or particular qualities; the word is used with various meanings, however.
By associating various ideas together in a certain regular way so that there comes from them something new, we reach a judgment, and this process of associating ideas to an effective end is called reasoning. Reasoning, then, is a process which leads to some new fact and differs from a casual association of ideas, such as occurs in revery or in the ordinary play of association by suggestion, as when a gray horse sug- gests the presence of a red-haired girl, or when the smell of gas sug- gests a leak in the pipe. Sometimes in the association of ideas in speech we proceed from one inference to another, or one thing is sug- gested by another, until we reach a point in the discussion which may be simply the climax of a story, or the exposition of a point of view, or description of some past event. When the associations thus lead to some definite point or idea, wre speak of it as the "goal idea," and in most rational speech or description there is this goal idea. Judgments are conclusions reached by the processes of association. These may be elaborate and conscious, or simple and very largely subconscious proc- esses. Thus many conclusions and opinions develop in the mind al- most unconsciously or through slight suggestion.
Memory and Orientation. — The activities of the mind are depend- ent on the power it possesses to retain and revive impressions; that is to say, its power to remember and recollect. This faculty of the psychic cells is called memory. It means that all stimuli sent to the brain make some kind of impression on the central nerve-tissue, which im- pression can be revived at future times by other suggestions and stim- uli. This process of record and revival is a form of association, and memory revival means the reestablishing of an association (Marshall). Memory forms part in most conscious psychic life. The mind cannot work without its constant help, though the memory may be good, and yet the mind works badly. The feeling of identity or personality is dependent on memory. Memory is usually divided into the power of learning or memorizing and the power of reviving or recollecting.
The knowledge of our relation to the external world, our apprecia- tion of time and space — in other words, our sense of orientation — is dependent on the memory.
The Instincts .—Those things which we do, say, or feel automati- cally as the result of inherited habits and experiences are said to be instinctive. Man does comparatively few purely inherited instinctive acts. As an infant the instinct for food leads to emotional expression and coordinated efforts. There is an instinctive love of life and fear of danger which control us to an extent; the family affections and sex-
68o
DISEASES OF THE MIND.
ual feelings are largely spontaneous and instinctive; the feelings of right and wrong seem also to be largely inherited, and conduct is con- trolled more by instinctive feeling than by conscious ethical motives. The feeling for beauty is often instinctive. In most of these cases the instinctive feeling is automatic, but the feeling and conduct resulting are modified often by training and experience or by other artificial motives.
We may divide the instinctive feelings into the somatic, i.e., those pertaining to the body, such as love of life, fear of death, sexual and hunger instincts; the ethical, those pertaining to conduct and the family and social duties; the aesthetic, those which relate to apprecia- tion of beauty in nature and art.
In the insane there is often a disturbance in all these spheres. The patient loses his sense of what is right, loses his love of parents and child and wife; he cares no more for what is beautiful and is no longer repelled by what is ugly or base. When the sense of what is right is lost the patient lies, steals, is cruel, and perhaps has no family affection ; he is then said to suffer from a moral imbecility. A certain degree of this is present in constitutional criminals; but the most decided forms are found in certain children who seem born moral monsters. Ac- quired insanities seldom do more than dull or extinguish in part the instincts.
Feeling or affect is not a separate function of the mind, but is a quality of the different mental states; that is to say, every mental state is accompanied by some kind of feeling, either pleasurable or painful, or has some quality that can be regarded as "affective." It may not necessarily be distinctly disagreeable, but may pertain to some subjective sensation, like hunger or thirst, or some simple desire of action. In all cases feeling only qualifies or gives a certain character to the mental state.
Emotion is that distinctive feeling which is aroused by some definite cause and leads to some expression of its existence. The sentiments are the higher types of emotion connected with some ideal object, like duty, ambition, beauty. Emotion includes feeling or affect and something more. A person may have a simple pleasurable feeling. It becomes more than this when the sight of a long absent friend arouses keenness of joy and acts expressive of this state. *
The volition or will is technically the conscious realization of the strongest and therefore deciding feeling. It is usually accompanied by a motor act, although a person after consideration may reach a con- clusion and not act on it until later. The process of volition may comprehend both the formation of a definite judgment or preference
GENERAL PSYCHOLOGY.
68l
and the expression of it in action. We see an apple and a stone. The idea after comparison comes to us that the apple is more desirable than the stone. The desire for the apple and the intellectual and emotional preference for it over the stone sets to work the impulse to take it, and a volitional act is performed.
The volition function is not therefore simply or only voluntary ac- tion, but includes the whole conscious process that led to this act. In this process we are made to feel that we have a power within us that can reach any of several judgments and lead us to any one of several choices. This leads us to believe we have a free will and are respon- sible for our acts; and society holds men responsible on the basis of this view. We accept it as long as we feel that there is the ordinarily free play of association and inhibition, so that the decision or choice comes about without any artificial or abnormal interference.
When an act is done without conscious preference or desire, or in response to an impulse coming upon one from subconsciousness, it is an involuntary or impulsive act. Acts which are the expression and re- sult of habit and which are done without our having any memorable consciousness of them are called automatic. Many acts are brought about by imitation and suggestion, and these are often done with so little conscious volition that they belong almost to involuntary or complicated reflex actions.
Th£ Subconscious Activities. — A large part of the mental activ- ities go on without our consciousness. Impressions may be registered without our knowledge; associations may be formed, and emotional states be connected with them; and we may thus have certain ideas and feelings of which we are mostly unaware. Certain moods and reac- tions, certain points of view or even fixed ideas may be due to these subconscious activities. The emotional state or "emotional complex" shows itself by certain tests known as association tests. Jung has developed a method by which these subconscious emotional states and their corresponding ideas can be detected. A long series of words is made covering the things connected with the ordinary activities of life. The patient is then told that these words will be spoken to him and he is to answer as quickly as possible with a word which the word spoken to him suggests. The time between the uttered word and the reply is measured by a stop-watch. It has been found that when any word which suggests something having emotional importance to the patient is spoken there is a delay in answer. The average interval be- tween question and answer is a fraction of a second. When there is an emotional complex around it may be two or three seconds.
A similar result is obtained by means of a very mild galvanic cur-
682
DISEASES OF THE MIND.
rent passed through the body connected with a very delicate galvan- ometer so as to measure minute changes in the resistance of the body. The patient is placed with his hands on two metal electrodes and the current turned on till the galvanometer comes to rest. Then a ques- tion is asked, or a sentence or word spoken. If this has any emotional significance to the patient, the electrical resistance of the body falls and the galvanometer needle registers it. By these two methods we can obtain knowledge of emotional defects and of the presence of emotional states which are outside the patient's control, or perhaps of states which the patient did not know existed. The fact has been established that the emotional complex is very slight in dementia prsecox. It may be discovered that certain fixed ideas or hypochondriacal ideas are due to past emotional experiences that have become registered uncon- sciously. These methods are still not perfectly worked out, but it seems probable that they will be of much help in unraveling the cause of psychasthenic states, and in the diagnosis of the psychoses.
CHAPTER III.
GENERAL SYMPTOMS AND SYMPTOM GROUPS.
The functions of the mind, as described in the foregoing chapter, undergo morbid alteration in the psychoses. These alterations form the symptoms of insanity. Various technical terms are given to these symptoms.
Disorders of Perception. — An illusion is a disorder of sensation and perception in which the object is perceived as something else than what it is. Thus a feather blown along the floor is taken for a mouse, a chair is mistaken for a person, or a noise is interpreted as a spoken word.
An hallucination is a disorder of sensation and perception, in which something is perceived which is not really present. Thus a person thinks he sees a person standing before him, or that an animal is in the room, or that there are insects crawling on the wall or coming through the ceiling, or he hears a noise or smells a smell when neither exists. This perception, however, does not lead him to believe that these things are real. He sees a man before him or hears a voice, but he knows there is no man or actual voice.
Illusions and hallucinations are falsifications of sense experience. They have the quality of extreme vividness and give an emotional tinge to the mental activities of the patient. They may and often do run into delusions, whether they are the real cause or not. Thus the moment a person who has an hallucination that he sees a person in front of him believes that such a person really is there, the hallucina- tion becomes a delusion. Illusions and hallucinations are usually disorders of the perception of hearing or sight, and are aural or visual. There may, however, be hallucinations of smell, taste, and of condi- tions pertaining to the skin, or muscles, or viscera.
Disorders of Judgment. — A delusion is a condition in which the patient has a false belief or judgment incompatible with a be- lief which he should have held, considering his education and social surroundings.
Delusions are divided into two types, the systematized and the unsystematized. In the systematized delusion the patient has only one or a few delusions which he logically work's out from his false
683
684
DISEASES OF THE MIND.
premises. In unsystematized delusions, the patient has a variety of delusions which have no relation to each other, and which are fleeting, foolish, and incoherent. Delusions are indicative of a deep- seated disturbance of the mind, involving, as a rule, not only the pro- cesses of thought and association, but those of sensation and those of feeling. They receive many names, in accordance with their character. Sometimes they centre about the person, and are con- nected with grandiose ideas of the person's importance. They are then called expansive delusions. Sometimes they are connected with feelings of great depression and ideas of having done some harmful act, or having some distressing disease, and they are then known as depressive or hypochondriacal delusions. Very often they are associated with ideas of persecution, and are then called persecu- tory delusions.
Disorders of Ideation and Association of Thought. — Ideas in healthy minds occur in response to normal suggestions and succeed each other in more or less orderly sequence. When this sequence fails we get a confusion of thought. Where the process of association is weakened ideas come slowly, comprehension is imperfect, response is difficult and belated. This is called a " retardation of thought," and it occurs in dementia and in states of mental exhaustion and melancholia. When ideas succeed each other very rapidly and with- out much or any natural sequence, the condition is called a "flight of ideas," a condition characteristic of maniacal excitement.
When there is an extraordinary activity, as well as confusion in ideas and trains of thought, so much indeed that no train of thought really seems to complete itself and no idea is clearly realized in con- sciousness, the state is one of delirium. In other words, delirium is a confused and more intense maniacal excitement. In both conditions there is a general emotional and motor excitement. Maniacal ex- citement must not be confused with "acute mania," which is not a symptom, but a special form of mental disease.
The special disturbances of ideation and association then are: Delusion, confusion, retardation of thought, difficulty of thought, flight of ideas, maniacal excitement and delirium, these being asso- ciated, as are all the other symptoms, to a less extent, with emotional disturbance.
Disorders of Orientation and Memory. — Disorientation is a condition in which the capacity to realize ourselves and our where- abouts is defective. It occurs in the clouding of consciousness, in dementia, and in other forms of mental disturbance. When there is loss of orientation of the person, we may have disorders of personality.
GENERAL SYMPTOMS AND SYMPTOM GROUPS.
685
Weakness of memory is shown both in inability to learn new things and in an inability to retain things which have already been learned. A great loss of memory is termed amnesia, while a condition in which real memories and inventions are mixed up is called paramnesia. There is also a condition of memory in which the patient seems quite unable to recall things in the least correctly; his memory experiences are all more or less fabrications .
There is a particular form of memory disorder in which the patient is, to an extreme degree, unable to retain or remember, for even a moment, things presented to him. For example, he sees a friend, and a moment later forgets that he has seen him before that day; or he is told it is Friday, and a moment later thinks it is Monday. This may be called a loss of attention-memory.
Disturbances of feeljng appear very frequently and underlie a large number of the psychoses. When the affective state is pleasur- ably active, there is usually associated with it a corresponding activ- ity in the processes of thought, and we get with exaggerated feelings of pleasure or self-complacency some motor excitement. This con- dition of a morbid pleasurable state is sometimes spoken of as "euphoria" or "exaltation"; if very intense, as "mania."
When the affective state is depressed so that there is a feeling of conscious suffering, the condition is known as depression or melan- cholia. ' The emotional condition may be simply diminished in inten- sity, so that the patient no longer feels any interest in life or in him- self; his capacity both for enjoyment and for sorrow are totally lost, and there is simply emotional negation or apathy. This is often seen in exhausted conditions.
The emotional state is also disturbed by specific feelings of depression, such as fear and anxiety. It may show a childish and a silly kind of humor, or be raised to a state of morbid intensity, in the condition known as ecstasy. It may be associated with delusions, and we then have "expansive delusions" or "delusional melancholy"; or it may be associated with agitation or stuporous states.
Thus there occur in disorders of feeling:
Apathy;
Melancholia, morbid fears, etc.; Melancholia with stupor; Melancholia with agitation ; Euphoric and expansive states; Maniacal exaltation; Ecstasy.
686
DISEASES OF THE MIND.
Disorders oe Volitional Function, or Power of Reaction. — When a person loses the power of choice or initiative, it is known as aboulia, or paralysis of the will.
When the volitional activity is exaggerated so that patients con- tinually want to be doing things, it expresses itself often in what is known commonly as "nervousness"; or, if more exaggerated still, in the motor excitement shown in an intoxication.
When this volitional excitement is still greater so that patients are continually talking, planning, and doing all kinds of aimless things, or when this finally amounts to a maniacal state, in which they talk excessively, laugh and dance about, the patient is said to be under a "pressure activity." This "pressure activity" is seen not only in mania, where there is a certain kind of purposefulness, but in states in which the "pressure activity" is purposeless, the pa- tient going through all kinds of grimaces and contortions and making senseless noises.
Without actual loss of power of volition, the patient may have simply a weakness of will, and with this there is often a condition of morbid suggestibility, so that the patient is easily led to do what- ever is proposed to him — a condition known as suggestibility. Some- times there is a morbid persistence of an impulse that has once been started, leading the patient to posture in different ways, or to go through certain movements over and over again. This is called stereotypy. Stereotypy is shown in two ways: by a person taking a certain attitude and staying there for a long time, or by going through certain regular movements, such as for hours walking up and down the room over the same course, rapping on the table, hopping and jumping, or exhibiting a certain amount of mannerism, such as walking with a peculiar gait, dragging one foot, or going in straight lines or circles.
Frequently, with the condition known as stereotypy there is an- other condition known as negativism. In negativism the patients tend to do just the opposite from that which they are requested to do, no matter how simple or reasonable that request may be. It is the ulti- mate insane expression of foolish and unreasoning obstinacy, not a resistance really due to an intellectual delusion or fear, though delu- sions do exist. Verbigeration is the continual repetition of senseless words and phrases, and is a condition akin to stereotypy.
Distractability. — Attention is part of the volitional function, and is the power by which we are able to fix the mind upon given subjects. When the attention is easily disturbed, it is called a distractability, and when it is lost there is a condition of absolute incapacity to fix the attention on a single process, which has received the name of aprosexia.
GENERAL SYMPTOMS AND SYMPTOM GROUPS.
687
Obsession (besetment) is a morbid mental state due to the action of an imperative idea or feeling which persistently assails and vexes the mind. This imperative or insistent idea is thrust into the mind and cannot by any process of reasoning be got rid of.
The terms "obsession" and "fixed idea" are often used somewhat interchangeably. Strictly speaking, however, in obsession an idea is imposed upon one's consciousness despite the will; thus a mother becomes harassed, and even terrified, with the idea that she will kill her child, or that she may suddenly do him some harm. A fixed idea, on the other hand, is one, strictly speaking, that is legiti- mate and natural, and perhaps has grown out of certain reasonable conditions; it becomes fixed and dominant, and it modifies the reason- ing of the patient. It is of the nature of a delusion ; is, in fact, an abor- tive delusion. Thus a person has an idea that he is going to have an attack of a certain illness, and there are some symptoms which justify this fear, but the idea is entirely out of proportion, in force and dominance, to the cause; or a person may have an idea of vengeance, which to a certain extent is justifiable, but which is intense and over- whelming and cannot be put out of consciousness. The term fixed idea or obsession is also applied in a rather popular way to persons who nurse and exploit certain fantastic views, or allow a perhaps sane idea entirely to dominate their thoughts and activities in morbid disproportion to its importance. Obsession leads to those conditions known as morbid fears and doubts, and sometimes to morbid sensorv states and impulsive acts. Obsession and fixed ideas are on the border- land of delusional conditions (abortive paranoia).
A compulsion is a condition in which there suddenly is forced upon the mind a desire to do a certain act, which act the patient does not want to perform, but is, nevertheless, with difficulty prevented from carrying out. The compulsion is accompanied by feelings of anxiety and attempts at resistance. Thus a patient, when shaving himself, is suddenly seized with a feeling that he must cut himself. Com- pulsive acts result when, as sometimes happens, the resistance is no longer possible.
An impulsive act is one in which the patient does a certain act from an overwhelming impulse, which he is conscious of, but is not able to restrain or fight against.
The impulses which lead a person to these impulsive acts are known as "morbid impulses," and they are at the basis of such con- ditions as kleptomania, pyromania, dipsomania. They sometimes underlie homicidal, suicidal, and perverted sexual acts. On the other hand, they may be very trivial impulses, such as to touch
688
DISEASES OF THE MIND.
every lamppost, or always to put the left foot first in going out of a door. When these impulses are shown in a violent gesticulation, ejaculating inopportune words, they are called psychic tics.
Disorders of Consciousness. — When consciousness is normal we have what is termed "clearness of consciousness." When it is lost we have unconsciousness ; and when it is imperfect the state is one of "clouding of consciousness." This is the condition in which a person becomes who s confused by excessive fatigue, by illness, or by drugs, or when he is passing into a condition of sleep or stupor. It is believed that in early childhood and in idiots, even normal con- sciousness is somewhat clouded. When consciousness becomes very much dulled we have states of stupor or lethargy; and, finally, when consciousness is lost we have states of coma, somnolence, syncope, and sleep. When coordinated and apparently purposeful acts are done without the patient remembering them, we have states of cerebral automatism, or double consciousness.
In states of excitement and ecstasy there is an abnormal intensity of consciousness.
There are perversions of subconscious mental activity in many morbid mental states that have to do with the development of mor- bid impulses, obsessive ideas, and abnormal acts.
Symptom Groups. — There occur various clinical symptoms, grouped together and forming what are called syndromes. They are seen in various forms of insanity, but do not of themselves con- stitute special diseases. Such symptom groups are dementia, which is a condition of general mental enfeeblement, usually progressive; euphoria, which is a state of abnormal pleasurable feeling; melan- cholia, which represents a symptom group, but is also the name given to a special disease. Delirium and maniacal excitement are also syndromes observed in the course of many of the psychoses.
Delirium is a clinical term, used to express a morbid mental con- dition, characterized by great mental and motor excitement, with confusion of ideas, and a rapid succession of incoherent and delusional ideas, usually associated with hallucinations. The state is often com- plicated with a febrile condition and generally ends in exhaustion. Delirium, in its very highest and most intense degree, is known some- times as "acute" delirium or "grave" delirium, and this form has sometimes been described as a separate disease. In the writer's opinion, however, it is only a symptom usually of some severe infec- tious or toxaemic process.
Another severe form of delirium is known as delirium tremens, and is practically always observed as the result of the excessive use
GENERAL SYMPTOMS AND SYMPTOM GROUPS. 689
of alcohol. Delirium tremens .itself, however, is usually not the direct effect of alcohol, but the result of an auto-toxaemic state, which has been brought on by starvation and alcoholic indulgence. When the delirium is less severe and marked, when the motor excitement is slight, and the mental processes are more confused and slow, it is known as a "low delirium," and this is the kind usually seen associated with infectious fevers and certain toxic and /exhausted conditions.
In epilepsy there sometimes occurs a somewhat mild form of delirium, which is called the "anxious delirium," while after exhaust- ing exertions or severe operations, or hemorrhages, there is sometimes a "collapse delirium."
The essence of all the delirious states is a great psychomotor ex- citement, accompanied by incoherence and confusion; and the dis- tinguishing point between acute delirium and acute maniacal excite- ment is, that in the latter there is a certain coherence of ideas, and more or less power of orientation and appreciation of surroundings, and a certain completeness to the different delusions and ideas that arise in the mind of the patient.
The clinical picture of a case of delirium is familiar to all prac- ticing physicians. The condition generally begins rather suddenly, and the patient exhibits a wildness of action and demeanor that at once alarms those about him. He is restless and throws himself about, attempts to strike and destroy things, is altogether unappreciative of his surroundings or of any of the ordinary restraints or decencies of life, and has to be promptly restrained in bed. Sleep is impossible and food is generally refused. The patient often has hallucinations of hearing or sight, and is unable to answer coherently the simplest questions, often responding to them with attacks of violence. The condition goes on, if unrestrained, for several days, when exhaustion and perhaps collapse ensue; it is often accompanied by a rise of tem- perature, great sweating and congestion of the face, overaction of the heart, and neglect of the natural functions.
Acute maniacae excitement is often termed simply "acute mania" or "peracute mania." Technically, however, the term "acute mania" applies to a special disease, running a course of a num- ber of weeks or months, and marked by psychomotor excitement. Acute maniacal excitement is a condition in which the patient shows many of the symptoms of delirium, but, as already stated, has less confusion of the mind, greater orientation, and capacity for control. The bodily excitement may be nearly as great, but it is less continu- ous, and appears more spasmodically. The patient may for a time be fairly quiet and then burst out again into excitement and violence.
44
690
DISEASES OF THE MIND.
Acute maniacal excitement is often somewhat difficult to distinguish from delirium, and the two states may mingle. It is seen in connection with the onset of paresis, of dementia prczcox, or of manic-depressive insanity. Maniacal excitement, however, also occurs in the toxic psychoses, as in alcoholism and in other forms of confusional insanity in its early stages.
The Physical Symptoms of Insanity.
These are shown in modifications of structure and of function. The modifications of structure include anatomical stigmata of de- generation and structural defects.
The anatomical stigmata of degeneration usually include all con- genital defects, but many of these are simply "marks," which indicate a badly put together body, and they do not interfere with normal function. Thus a peculiarly shaped skull, high, narrow palate, badly set ears and teeth, and scarcity or abundance of hair, etc., are oftener found in the psychopathic than in the normal, but they do not inter- fere with function. On the other hand, the insane have anomalies of the cerebral arteries and of the circulatory system generally, which do interfere with normal function. It will be found that in this class as a whole also there are often defects in the kidney, liver, and glan- dular organs.
The special anatomical stigmata of degeneration have already been described under the head of Nervous Diseases. Abnormal variations of the cephalic index are frequent among the insane. Hy- perbrachycephalic skulls of 90 to 93, and hypodolichocephalic skulls, with indices of 72 to 70 are often observed (Berkley). Micro- cephalic and deformed skulls are, however, mostly seen in the idiot- imbecile class. Anomalies in the arteries of the base of the brain are particularly frequent in the insane. Excessive hairiness of the skin is common, but most often appears after the psychosis has developed.
The disorders of function include a lessened cutaneous sensibility, defects in sight and hearing, often imperfect intestinal digestion. Heart disease and arterial sclerosis are common. The blood and urine are often modified, but anaemia and nephritis are usually secondary conditions. Disorders of secretion are shown by ptyalism, and excess- ive or deficient perspiration.
The more characteristic physical symptoms of insanity are those shown in attitude and expression. Examples of these are: tooth- grinding, facial grimaces and other tics, agitated movements, anxious,
GENERAL SYMPTOMS AND SYMPTOM GROUPS.
69I
apprehensive expression, apathetic and lifeless movements, with hang- ing of the head, katatonic states and strange attitudes, senseless resist- ance to reouests or negativism, peculiar repetition of certain move- ments, or stereotypy; increased brilliancy and mobility of the eyes in mania; ocular parallelism in melancholia; muttering, senseless laughter, and verbigeration.
CHAPTER IV.
METHODS OF EXAMINATION AND DIAGNOSIS.
In the examination of a patient it is best, so far as possible, to follow some fixed line, though this is often not possible, and in some cases even is not necessary. In an ordinary examination the things that are chiefly investigated are the following:
The general physiognomy, attitude, and speech of the patient, which often, without much further investigation, tell the story. Thus the expression of drunken exaltation in mania, the profound dejection of melancholia, the sunken head and stupor in katatonia, the speech and tremors in paresis, and the dull and careless physiognomy of dementia, often at once reveal the diagnosis.
The orientation of the patient; that is to say, the discovery of how much he is aware of his present situation, surroundings, and condition. In determining this a series of questions is usually asked regarding his past history and how he came to his present place, what symptoms he is suffering from, and what his ideas and wishes for the present and future are.
The existence of any delusional ideas, or any hallucinations or illu- sions. These points are often brought out in connection with questions regarding his orientation.
A determination in general of his degree of intelligence, and of his memory and judgement.
His emotional condition, as to whether he is excited or depressed, and what the causes for that condition are.
An investigation into any organic or physiological disturbances.
Almost all of these points can be gained by acquiring the confidence of the patient, and getting him in various direct or indirect ways to tell the story of his life and present illness. If a more complete analysis of symptoms is required, a particular investigation is made along the points just mentioned and certain others. The particular points of such further investigation are these :
First. Orientation, by which we determine what insight he has into his own condition, the clearness or "clouding of consciousness," the notion of his personality, whether it has been changed or doubled, and in general the idea of his relations to the external world of time and space.
692
METHODS OF EXAMINATION AND DIAGNOSIS. 693
Second. Troubles of perception, including an investigation into the existence of illusions and hallucinations and their type, whether visual, auditory, olfactory, gustatory, dermal, visceral, or motor.
Third. Troubles of association, including the study of his memory of his reasoning powers and judgment, and of the existence of delu- sions and their character. Uuder this general head also we inquire into the existence of fixed ideas, obsessions, the power of attention, incoherence, and flight of ideas.
Fourth. The disturbances of emotions, by which we determine the existence of indifferent, apathetic, or melancholic states, of mor- bidly euphoric states, of maniacal excitement, delirium, and also of the existence of morbid fears, apprehensions, impulses, passions, and instincts.
Fifth. The disturbances of volition, the presence or absence of aboulia, or loss of will power, of morbid automatic reaction, shown in the increased degree of suggestibility, of impulsions, catalepsy, stereotypy, and negativism.
Sixth. Finally there will be the disturbances of consciousness, shown in abnormal ecstatic states, in clouding of consciousness, in weakness of the notion of personality, and of the transformation and doubling of personality.
The examination and recognition of a case of insanity in the majority of cases are very simple and easy; in the minority of cases they are somewhat difficult, and in a few cases almost impossible in one examination. In the ordinarily difficult cases it will usually take about an hour to make out some distinct evidence of an abnormal mental state, for few of the insane can feign their condition so cun- ningly that some suggestion of it will not sooner or later creep out. Certain types of monomanias and of melancholia with mutism often are the most difficult to detect. It is practically a well-known fact that suggestions about religion, hypnotism, electricity, wireless teleg- raphy, or some idea of persecution, will often bring out the delusion. In the diagnosis of insanity naturally a good deal of help is to be ob- tained from the facts furnished as to the antecendent history, and sometimes it is only by positive assurance as to the validity of these facts that a conclusion can be reached. Some help can be gained at times by a knowledge of the way in which the malady has developed. There are certain forms of insanity which practically never develop suddenly without any previous suggestion of the trouble beforehand. The physical examination of the patient will give some confirmatory evidence as to the existence of a psychosis; thus the carelessness of appearance, the unusual growth of hair, the mutilations of the person,
694
DISEASES OF THE MIND.
may all be of some help. In some dementias the evidence thus fur- nished is quite positive. It is only in general paresis, however, that the physical signs enable one to speak early and with absolute posi- tiveness regarding the condition.
After the existence in a patient of the fact of insanity is made out, the diagnosis of the particular type is to be considered. The recognition of the special type is helped by a knowledge of the prevalent insanities at given periods of life.
At the period of adolescence (fifteen to twenty-five) we meet chiefly functional insanities dependent on constitutional and de- velopmental defects: i. These are dementia praecox in its simple, hebephrenic, katatonic, or paranoid forms. 2. Manic-depressive insanity, chiefly in a simple melancholic or maniacal form (recurrent melancholia, recurrent mania, circular insanity). 3. Phrenasthenic or degenerative insanities.
At the time of maturity and active life (twenty-five to forty-five) : 1. All forms of insanity, but especially paranoia. 2. General paresis and other organic insanities. 3. Toxic, infectious, and exhaustion types.
At and after the climacteric : 1 . Chronic melancholia of involu- tion, agitated, and hypochondriacal and querulent melancholias. 2. Senile insanities.
CHAPTER V.
GENERAL PROGNOSIS AND TREATMENT.
Insanity is sometimes the result of a morbid condition inherent in the structure and function of the individual. In this case the disorder is irregularly chronic and has no very definite course. It is improved or cured only as the individual, by conserving and strengthening his constitution, manages to overcome the degenerative taint. Such insanities are paranoia and phrenasthenia.
In other cases there is superimposed on a slightly or seriously degenerative constitution a serious strain, a toxaemia, or a disordered metabolism. Here the malady runs a certain more or less definite course, like a fever, ending in permanent or temporary cure. Such insanities are manic melancholia, confusional psychoses, and demen- tia praecox. In other cases there is distinct organic disease of the brain as in paresis and senile dementia. Here the course is fairly definite.
Any general statement regarding prognosis is of little va1ue since the outlook varies immensely with the type of psychosis. It is esti- mated that about one-half the insane get well (about one-quarter per- manently well), and about one-half die sooner or later in the attack. Death occurs in about six per cent., and the average age at death is about fifty-six (Hill, Pilgrim).
The general treatment of the major psychoses has to be almost entirely institutional. This is not because an insane patient cannot be as well or better treated under private auspices, as because to do this latter requires a great deal of care and expense, much beyond the reach of most. The general opininon as to the best methods at the present time of caring for the insane is that there should be first of all in the cities and larger towns either psychopathic hospitals and dispensaries or psychopathic wards in the hospitals. In these small hospitals and special wards the acutely insane should be re- ceived, and if amenable to treatment there, retained until they are cured. If they turn out to be cases that are likely to be of long dura- tion, they are transferred to a second class of institutions, the State hospitals, or to some private hospital. Here proper provision for the care of cases likely to run a rather long course is made, and patients, the duration of whose disease is one or two or three years, are cared
695
696
DISEASES OF THE MIND.
for. When it has been determined that a certain malady is absolutely incurable, the patient is transferred to a colony or hospital for chronic cases, where he can live an outdoor life and where means are provided for his comfort and for the utilization of such physical and mental powers as he still possesses.
It is to be hoped that in time convalescent hospitals or annexes will be established to complete the scheme.
While it is true that the majority of cases of insanity are better treated in institutions than at home, this is not always the case, and not rarely patients who have only minor deviations from the nor- mal are better when kept in constant association with normal minds.
There is as yet no specific medical treatment for most of the psy- choses. Drugs may alleviate symptoms and possibly shorten some- what the duration of the disease, but nearly all drug medication is symptomatic. If there is any drug which has a specific effect it is opium and its derivatives.
Antitoxic medication is often used in certain confusional psy- choses, and of recent years the antitoxic character of some of the functional psychoses has led to the hope that we may yet find help in medication. At present the surest antitoxic agents are the purga- tives which unload the liver and clear out the bowel. Mineral foods, like iron and the phosphates, rank next in importance.
Baths, electricity, and massage have only symptomatic value.
Institutional treatment, with its auxiliaries of outdoor life and diversion, are the mainstay in the treatment. Sea-bathing, garden and farm work are especially helpful.
I have often seen patients, when near their convalescence, bene- fited by taking them away from the surroundings of a psychopathic ward or hospital. The environment of the patient's family is usu- ally bad; but to surround one insane person with many sane is some- times a very wise procedure.
CHAPTER VI.
THE MINOR PSYCHOSES AND PSYCHO-NEUROSIS.
Phrenasthenia, Psychasthenia, Hypochondriasis, Hysteria,
Psycho-neuroses.
As already stated, the minor psychoses are not as a rule insanities in the common sense of the word, and it seems advisable to use for them some distinguishing term. Psycho-neurosis is a word which may be employed for this purpose. It includes those many cases of neurasthenia which really have essentially a psychical basis, and which are often mild or abortive types of simple melancholia, hypo- chrondriacal melancholia and prodromal stages, sometimes aborted, of dementia praecox or paranoia. It includes some of the traumatic and shock psycho-neuroses, and also a group of cases variously called borderland psychoses, constitutional psychopathies, neurasthe- nic psychoses, and "degenerative insanity." These terms include the conditions characterized by morbid impulses and fears, doubting manias, impulsive acts, obsessions, kleptomanias, dipsomanias, and many forms of perversion of the sexual instinct.
They are all dependent on a minor defect of mental stability, one that is quite compatible with sanity and responsibility. They are chronic and not dementing in tendency. The word phrenas- thenia may be used for this group. The Greek, <t>ptivi is much more properly applied to the mind and its diseases than the ^vxv, which is more associated with "life," "spirit", and "soul."
PHRENASTHENIA (PSYCHASTHENIA).
(Degenerative insanity; Neurasthenic Insanity; Impulsive and Compulsive Insanity; Borderland Cases; Original Psycho- pathic Conditions.)
The term psychasthenia, suggested by Janet, has caught the attention of the profession and will doubtless supplant that of phren- asthenia which I used in my former edition. The condition has been elaborately studied and analyzed, but I find the description of phen- asthenia fits fairly well that of psychasthenia. This latter word may, however, be used in a somewhat narrower sense to include only psychoses characterized by morbid fears, panicky seizures, obsessions,
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DISEASES OF THE MIND.
fixed ideas, doubting mania, aboulia, distressing sensations of unreality of the person; and by motor disorders, such as psychic tics, and psychic seizures as described under the head of "symptoms". In my opinion, compulsive and impulsive psychoses and even some of the episodes of hysteria minor are closely related and belong to the same great group. The term phrenasthenia may still be preserved, therefore, to cover the whole group of what used to be called neurasthenic insanity. The term obsessive neurasthenia or obsessive psycho-neurosis is used by some instead of psychasthenia. Beard originally described many of these symptoms and classed them under the head of neurasthenia. Indeed, neurasthenia usually accompanies the condition, and many unstable people, when they get weakened or exhausted by work or disease, develop morbid fears and obsessions. So that part of the cure of psychasthenia is to treat the neurasthenia.
Phrenasthenia or psychasthenia is a chronic, non-dementing psychosis, characterized by the occurrence of imperative ideas, by compulsive and impulsive acts, by morbid fears and conditions of agitating doubt. The main characteristic of phrenasthenia is a defect in the inhibition, by reason of which association of ideas and the natural elaboration of thought and impulse to action are no longer under control. The constitution which is affected by this disorder is similar to that which underlies hysteria, and to the impulsions to drink, to take drugs, and to special manias, like kleptomania. In its mild form it is little more than a peculiar incapacity for self-control, and is often associated with simple neurasthenic and hysterical symptoms. In its more severe types it approaches to the condition of systematized delusion, and it is called by some "abortive paranoia." In other cases the element of anxiety and distress is so great that there is with it a certain amount of melancholia. The phrenasthenic syndrome, or symptom-complex, of impulsions and compulsions, fears and doubts, fits itself ordinarily most perfectly upon a certain peculiar inherited constitution; but this same syndrome may be seen in connection with the other psychoses or acquired degenerative states. It may form a part of paresis for a time or of paranoia, of senility, or even of true melancholia.
Etiology. — The most frequent etiological factor is heredity; nearly one-half of my cases gave a history of direct or indirect mental dis- order in the ancestry. In one case the father was a tabetic at the time of the birth of the child; in two cases the father had paresis. Alcoholism in the ancestry is present, but not so prominently as in other psychoses. The disease occurs in males and females about equally. Symptoms of the trouble may be shown in children as young as
THE MINOR PSYCHOSES AND PSYCHO-NEUROSIS. 699
eight or nine years; more than half the cases, however, develop be- tween the ages of fifteen and twenty-five; nearly one-fourth of the cases between the ages of twenty-five and thirty, while one-eighth of the cases develop between the ages of ten and fifteen. A few cases occurred between the ages of thirty-five and forty, and it is seen again with some frequency at the time of the climacteric. The earlier the case develops the more severe, as a rule, are the symptoms liable to become.
Among the most frequent exciting causes may be mentioned some kind of shock by fall or accident; next after this is alcoholic excess. Among other causes I have noted infectious fevers, an attack of chorea, a stroke of lightning, car sickness, over-education, uterine prolapse, and ovariotomy.
Symptoms. — -The symptoms develop in four different types, though these are often intermingled, and, in most cases at least, three of the characteristic groups of symptoms are more or less present.* These types are those characterized (1) by morbid fears, (2) by im- perative ideas, (3) by a doubting mania, and (4) by morbid impulses.
In 91 cases I find the order about as follows: Morbid fears, 37; obsessive and fixed ideas, 25; doubting manias, 18; compulsions and impulsions, n.
Morbid Fear, Doubting Mania. — Phobic and doubting phren- asthenia begins usually rather suddenly as a result of some slight shock, and perhaps of some casual and unimportant incident. A young man while in church or in a crowd suddenly has a feeling of disturbance of consciousness, with something akin to vertigo. He is alarmed, the head seems full, the heart palpitates, and he feels as if he would faint. These attacks may be repeated. They are called psychic seizures, or psycholepsy. After one occurs the patient becomes afraid to go in a crowd or in an enclosed place where there are people (agoraphobia) ; he may even be afraid to go out of doors without a companion. This state is accompanied with some general nervousness, depression, in- somnia, and pressure or pain at the back of the head or vertex. He is often unable to pursue his work or study, and becomes worrying and introspective. After some months or a year the secondary symptoms disappear, but he may for several years feel a dread of going in certain places.
*The condition of obsessive and fixed ideas is called by Kraepelin compulsive insanity . Compulsive acts may result, but they are accompanied by consciousness of the nature of the act, by feelings of anxiety, and by attempts at resistance. The victim of morbid im- pulses is said to be suffering from impulsive insanity. Here the impulses spring sud- denly into consciousness, and acts follow at once without the patient being able to resist them. These impulses vary in character and severity from the impulse to touch a lamp post to that to commit a murder.
700
DISEASES OF THE MIND.
Fear of storms and of lightning (astraphobia) may come on in much the same way, but this form of morbid fear is less disturbing as a rule, and often the patients are quite well as long as the sky is reasonably clear. Some patients are so sensitive, however, that they become nervous and depressed before storms, and can predict them as unerringly as do rheumatic or neuralgic sufferers.
In fear of contamination and dirt (mysophobia) the patient is constantly washing the hands, changing the clothes, and trying to avoid contact with people. The sufferer will spend an hour or more in dressing and undressing or in preparing to go out. After a time almost all activity is curtailed by dread of contact with something, and the victim sits in a chair, or lies in bed with hands enveloped in cloths to protect him from the dust. All the interests of life are lost in this one absorbing idea and fear, though the patient is not de- mented or maniacal. There is a decided depression, but not a true melancholia. If the patient, however, is driven by his attendants to do things he dreads, he cries and becomes excited and hysterical.
Fear that the heart may stop or the breathing cease are also serious types of morbid fear. The patient is always feeling the pulse and ask- ing for an examination of the heart and an assurrance of its soundness.
The number of fears is almost as great as the number of objective things. The more common types are mysophobia, agoraphobia, claustrophobia (the fear or oppression felt in closed rooms, especially in public assembly rooms), fear of riding in cars or carriages, and astraphobia. There are also many "occupation fears," such as that in which the barber fears to use his razor, the tailor to cut the cloth, the bartender to mix drinks, and the business man to sign his name to checks. These symptoms run a course of two or three years. If properly treated, the patient recovers, with a tendency to relapse.
Allied to the morbid fears are the morbid "philias" or anxious concern for the safety and health of the race, more particularly of the domestic animals. The patients cannot walk the streets without concern over the tail of the docked horse, and will get up at night to fetch in a wailing cat which seems to him in real, not amorous, distress.
While morbid fears occur in very early life, often developing at puberty, doubting mania occurs rather later, usually after adolescence. The doubting habit is often a morbid outgrowth of an original mor- bid scrupulosity and attention to detail. The patient has usually been very orderly in habit and worried when things are not arranged or done in a very orderly way. The clothes and bureau drawers and the bric-a-brac of the room have all their set place, and a discomfort
THE MINOR PSYCHOSES AND PSYCHO-NEUROSIS. 701
is felt at seeing them out of the regular spot. Then some disturbing emotion or sickness sets agoing an absolute distress of mind if things are not just so or are not perfectly clean. Then begin frequent washings, change of clothes, an hour is spent in arranging the articles on a bureau and mantel or in dressing the hair.
The doubting mania sometimes seizes girls — more rarely men — who are about to be married, and engagements are repeatedly made and broken. Often the doubting habit is shown in connection with fear of fire, of burglars, or gas. The patient gets up at night a dozen times to feel the gas jets, to see if the doors are locked, to look under the bed, and do other whimsical things. These doubting states are often not very serious, and are only exaggerations of a naturally timid and scrupulous disposition, Alcoholism is at times associated with doubting mania.
Obsession, Fixed Idea; Obsessive Phrenasthenia. — Obsessions and fixed ideas often relate to some rather trivial thing. In the obsessive constitution the association machinery seems to get clogged and ideas tend to stick pertinaciously in the mind. A slight suggestion or re- mark casually made with some especial rhythm or emphasis stays and bothers the patient. More often there are one or more ideas which stay and torment him. They are unpleasant ideas of possible mistakes made or injuries thought to have been done. They are usually absurd, and the patient knows perfectly well that they are so, but they cannot be shaken off. They dominate and harass the sufferer who feels really like one "possessed." They lead to insomnia, nervous restlessness, hysterical outbreaks, and crises of the abdominal viscera, with diar- rhoea and polyuria. Yet often the patient, if naturally intelligent and sensible, will keep himself in control, and show most of the time no emotion or evidence of suffering. These fixed ideas may attack a person periodically for years, or may after a few months disappear, not recurring at least to any extent. Shocks, acute sickness, and de- pressing experiences will bring them out.
The syndrome of the fixed idea, not reaching the intensity of a de- lusion, may be part of a melancholia of involution or of manic-depress- ive insanity.
Morbid Impulse. — Impulsive phrenasthenia is the condition in which impulses spring suddenly into consciousness, and acts follow at once without the patient being able to resist them. The morbid impulses may be very slight and harmless, such as a desire to touch every lamppost passed on the street, to step on alternate stones of the pavement, to put the left foot first in going from a room, or they may lead to violent acts, such as murder, theft, or arson. When there
702
DISEASES OF THE MIND.
is a morbid impulse to utter certain words over and over, it is called onomatomania. Psychic tics belong to the morbid impulses. A patient of mine, a clergyman, who was overworked and anxious, deve- loped impulses to an automatic coprolalia and profanity while at work in his study, and even while preaching. Another clergyman had the same tendency along with a decided doubting mania.
Morbid Compulsion. — When a morbid impulse rises into conscious- ness and is held more or less under restraint it is called rather arti- ficially a compulsion. A patient while shaving started to cut himself. He never did do this, though he was obliged to give up doing this function himself for a time. He also felt an intense desire to jump overboard while on a ship, though he had had no melancholia at any time. The compulsion is often perfectly restrained, and I have known a man who went about for years with at times an almost overwhelming fear that there would come upon him an uncontrollable impulse to assault some one.
Compulsive and Impulsive Manias. — When impulses and com- pulses involve a larger psychical sphere, we have the various conditions known as kleptomania and pyromania, homicidal and suicidal mania. But here, along with defective inhibition, there is often a morbid degree of criminal instinct or there may be present another psychosis, such as mania or melancholia or paranoia. Thus the sexual criminal acts and perversions are of the class of compulsions or impulsions, plus a congenital and hereditary instinctive defect.
A type of impulsion midway between the mild and neurasthenic and the deep-seated psychopathic cases are the impulses to drink leading to periodical inebriety. Here there occurs periodically, with or without the excitement of a single taste of liquor, an over- whelming impulse to drink, and the patient drinks until consciousness is lost.
In fine, impulsive and compulsive insanity are generally part of the psychosis phrenasthenia, but they may occur as episodes in melancholia, mania, dementia praecox, paranoia, and the criminal constitution.
Hypochondriasis and Phrenasthenia. — Hypochondriasis is a term applied to a morbid mental condition in which the patient thinks he is suffering from some physical disease. It is a much used and much abused term. Hypochondriacs vary much in the degree in which the symptoms show themselves. In some cases the belief in the disease is so foolish and extravagant as to form a real delusion, as when a patient thinks he has a worm in the head or stomach or an absolute and permanent stoppage of the bowels. In these cases
THE MINOR PSYCHOSES AND PSYCHO-NEUROSIS. 703
the hypochondria is only a manifestation of a melancholia, generally a chronic melancholia of involution.
In more cases the patient's hypochondria is simply a manifesta- tion of a morbid fear or an idee fixe or obsession. These are cases of phrenasthenia, or neurasthenic psychosis. A milder degree of hypo- chondria characterizes chronic neurasthenia. Hypochondriasis, there- fore, is only a syndrome which occurs in melancholia, phrenasthenia and neurasthenia, and is not a separate disease. The hypochondria of early life is often only a form of "anxious depression" such as characterizes the melancholias of later life.
Hysteria and Phrenasthenia, Hystericae Insanity. — Some at least of the mental traits and emotional crises of hysteria are forms of or episodes of phrenasthenia. There is here a loss of control due to the sudden explosion of fear or anger from an inadequate cause. The interparoxysmal state of the hysterical is characterized often by morbid fears and obsessions and by psychic tics. Thus the phrenasthenic, who despite his morbid fears is forced to do the things he dreads, will go into an emotional crisis resembling an hysterical attack. I con- clude, therefore, that while in hysteria there are other psychic phe- nomena, such as automatisms and emotional disturbances, and while in hysteria there may develop real major psychoses, it seems unnecessary to describe a special hysterical insanity. Most at least of the serious psychoses of hysteria can be placed in other known categories when they do not belong essentially to the present one.
Diagnosis. — The diagnosis of a phrenasthenia, characterized by morbid doubts and fears, fixed ideas and morbid impulses, is usually very easy. Sometimes the condition is associated with a great deal of mental depression and anxiety, so that it may be something like a melancholia; but there is with it none of the profound melancholia, with suicidal feelings, and the condition is one more of anxiety and alarm than of true emotional depression. There is, furthermore, no difficulty or retardation of thought, nor any of the apathy of melan- cholia. Persons with fixed ideas or obsessions may seem at first to resemble, to a certain extent, paranoiacs, and such cases have been described as abortive types of paranoia. The fixed idea, however, is one which the patient realizes to be overdominant and in a sense forced upon him; he does not accept it with sympathy and conviction as the paranoiac does; he is aware that it is, in a measure, delusional in character. In the case of morbid impulsive acts, the question of an underlying melancholia or other psychosis must be considered.
Prognosis. — A neurasthenic psychosis, associated with morbid fears and doubting manias, occurring in early life, is a serious condition.
DISEASES OF THE MIND.
If taken early in hand, however, and placed under rigid control, the patients have an excellent chance of recovering in one or two years. If, as is often the case, the patients are kept at home and not properly managed, the trouble becomes fixed, some mental deterioration de- velops, and a serious and incurable condition takes place.
When the disease develops later in life, as an episode or as the result of some shock or exhausting effort, prompt treatment is usually effective; but here again, if the trouble be neglected and becomes fixed, the prognosis is more serious.
Phrenasthenic conditions associated with fixed ideas and compul- sions are in my experience less serious. They often disappear under tonic treatment and rest in the course of a few months or a year, but there is the liability that they may return.
A phrenasthenic psychosis rarely develops in the degenerative period of life ; if it does, it is serious and is apt to run into a melancholia.
The hysterical mental state, with its crises and intercurrent ner- vous phenomena, has about the same prognosis as that of the other forms of phrenasthenia.
Treatment. — The treatment of phrenasthenia in all its forms is essentially one of restraint and pedagogical training. Without such measures very little can be done; but with them all but the worst cases can be managed with fair satisfaction. This general statement ap- plies to dipsomania and the drug habits, as well as to the kleptomanias and the morbid fears and doubts. It is as useless to treat these cases (except in the mildest forms) by medication and at home as it is to treat the drink habit or the major hysterical conditions. There are no specific drugs. In the mild types of phrenasthenia, characterized simply by fixed ideas and a certain amount of fears and doubts, hyp- notic treatment is certainly of some use. This applies especially in the younger cases.
Obsessive Psycho-neurosis, Mania for Detail, Fixed Ideas, Morbid Fears, Aboulia. Mrs. N., a woman of 40, with no children and a poor heredity. She has within the last ten years been developing more and more certain fixed ideas, so that her conduct is now almost entirely controlled by them. She cannot keep house, for the details fret her beyond bearing, and has to live at a hotel. She is physically well, has a good appetite and sleeps well, but she has to live in a certain fixed fashion. She will only wear certain clothes, and wear them in a certain way, and change them on certain days. If they are torn, she will not sew them up, but ties the holes up with little threads. She will not go out except along certain streets, and at certain times of the day, and she will eat her meals only at certain places, and in a fixed manner. She
THE MINOR PSYCHOSES AND PSYCHO-NEUROSIS. 705
is in constant fear that something will happen if she does not follow these rules, which are largely the product of certain superstitions. She will not do anything on a Friday, or on the 13th, or on any number with which 13 may be associated. She is very excitable, has bursts of temper, quarrels with her husband, lacks social and sexual instincts, has no friends, and no occupation, but occasionally amuses herself by sewing or going out driving or to the theatre. She is not unintelli- gent, is not melancholy, but is absolutely unreasonable on the subject of her obsessions without being really delusional. She prefers, hav- ing adopted a certain course, to keep upon it, and will do nothing new or make any change without extraordinary efforts. When forced to do things that are contrary to her notions, she becomes excited and hys- terical, but after having been once persuaded to do them, she will con- tinue without objection. She has no bad habits other than the mental habits, and she declares that she is very well. The course is chronic, and there is no sign of special mental deterioration. She has no stig- mata of hysteria.
Obsessive: Psycho-neurosis, Morbid Fear.
Mrs. S., a woman of 26, with good antecendents, after a confinement, was one day very much frightened by seeing her child in a convulsion. From that time, she began to suffer from sleeplessness, anxiety of mind, and she was given powders to make her sleep. After taking these for two weeks, she got an idea that she had a dangerous craving for them. She saw the vision of a sleeping-powder all the time before one of her eyes, and she became obsessed with the idea that she was a victim of the powder habit. Certain words spoken to her would stick in her mind, and she would have to say them over and over to herself, such as "powders," "powders," "powders." She became alarmed about her mind, feared she was going to lose her reason, had a sense of unreality, things seemed "way back" in her; she at times talked to herself. One day she had a pain in her back, and then thought she had kidney trouble. Later, she had a peculiar feeling in her head, and thought she had gas in it. She became depressed and almost suicidal, but was conscious all the time that her ideas were foolish and that they were in reality delusions and not true. She could not, however, argue herself out of them. She soon got well.
Obsessive Psycho-neurosis. Morbid Fears Aboueia.
Mr. M., aged 25, has for several years become more and more affected by his morbid fears. He received a good education, went to college, but left it in his junior year, because he could not control the fears that dominated him. He dreaded to go into a class-room, and was in a panic at the thought of being called on to recite. Fearing that some thing would happen to him, he always had an end seat. Leaving college, he went into his father's place of business in the city, but he had a dread of closed places, and would not go into a theatre or church. He was also afraid of large open places, and crowds, and would hug the wall when out walking on the street. He could not go into a brill- iantly lighted room, because it made him dizzy. It was difficult for him to decide on a course of conduct; he had continual doubts and
' 45
706
DISEASES OF THE MIND.
fears about the consequences of any definite course of action. He showed some mental exhaustion, in that he could not concentrate his mind long, because it produced a feeling of confusion. On the street he would sometimes get an attack of a sort of vertigo, in which things would get black before him, and he would stagger so that he was inclined to keep to his room. He knew that all these fears and doubts were unreasonable, felt himself the victim of his obsession, and tried to overcome it. His failure had made him despondent and unsocial. He is a man of good intelligence, and good physique. He cannot drink alcohol, but tobacco quiets him. He got gradually better under a tonic and pedagogic course of treatment.
Obsessive Psycho-neurosis — Querueent, Paranoid Type.
Mrs. H., aged 37, has one child, and has for several years since its birth been neurasthenic, that is, unable to do much physically or mentally, without having to go to bed with a headache or lie down and rest for several days. She is a worrier over details, discontented with her life, thinks her husband does not come up to right standards of domestic life, flies into tempers if he is dilatory at meals or absent of an evening or if he does not talk to her or amuse her as she thinks he ought. Her conscientiousness for having things done just so makes it impossible for her to keep servants. They are all soon dis- charged, and then she gets ill trying to do their work. She is, in a way, very jealous and consequently unsocial, for she cannot bear to see her husband amused with other people, whether they be men or women. She is not happy with him, and is wretched away from him ; she fabricates stories about his immoral or unkind conduct, sees the dark side of every- thing ; she craves enjoyments, but the minute she has achieved what she wants is dissatisfied again. She has bursts of hysterical anger over trifles, goes to bed with a headache if things go wrong, and threatens to leave her home or kill herself when she has these emotional storms. She finally becomes so impossible, unstable, and apparently depressed, that she seems to be truly suicidal. Her husband, under advice, finally places her in an institution, where, under discipline, she promptly regains her balance, loses all her pains and depressions, is taken home, and soon renews her querulous ideas of misuse. She is intel- ligent, capable of enjoyment and of giving pleasure, but is obsessed by her selfish view of life and is absolutely unhappy unless she can be the centre of the stage. A long term in an institution finally made her relatively well.
HEREDITARY OR CONSTITUTIONAL INFERIORITY.
This is a term applied to a condition characterized by a congenitally unstable, nervous system, and more particularly an unstable mental condition. The patients, however, as a rule, are sufficiently under control of themselves and their morbid tendencies, to be responsible or partially responsible for their acts.
The term phrenasthenia, as defined by me, includes a considerable proportion of this class. Others, however, include in it persons who suffer from "nervousness," constitutional despondency and con-
THE MINOR PSYCHOSES AND PSYCHO-NEUROSIS. 707
stitutional excitement, morbid sexual instincts, compulsive and im- pulsive insanities with their various imperative ideas, morbid fears and morbid impulses; kleptomanias, pyromanias and persons ad- dicted to alcoholic and drug habits are generally to be included under this group.
"Constitutional inferiority," then, may underlie the following list of conditions: Neurasthenia. Psychasthenia. Hysteria. Hypochondria. Dipsomania.
Compulsive and impulsive psychoses.
Kleptomania.
Pyromania, etc.
When the trouble is more especially due to weakness of will, feebleness of purpose, incapacity to decide or inhibit, or to initiate, it is in particular an "inferiority." When the mind is sound and even strong, but the person is dominated by morbid instincts (sexual or criminal), or unsocial passions, the person is more particulary a "psychopath," or psychopathic personality. Various major psy- choses may develop on this soil.
Persons with a constitutional inferiority may, under proper environment and educational guides, live fairly normal and useful lives. If, however, they are brought up in such a way as to allow indulgence in abnormal impulses or habits, if they thus acquire vicious modes of living or go to excesses in any direction, they may develop some of the formal types of insanity, such as paranoia or even dementing types of insanity, like dementia praecox.
It is often somewhat difficult to say whether a person is simply a psychopathic personality, or whether he has not a constitutional in- feriority which has allowed criminal tendencies to develop, so that a close differentiation of the two groups cannot always be made. It is often possible by the force of teaching and the influences of environ- ment, to change the morbid tendencies, so that an inferior or psy- chopathic personality may become a fairly useful man. Great care should, therefore, be exercised in applying to a patient the term "con- stitutional defect," since it may be only a slight tendency which has grown through indulgence.
CHAPTER VII. DEMENTIA PRECOX.
Dementia precox is a general name given to several forms of constitutional psychoses which appear usually before adolescence is complete. It is characterized by progressive psychic exhaustion and tendency to dementia, while in its course there develop mental excitement, motor disorders, and delusional and stuporous states. It runs a long course with remissions, and it is usually three or more years before decided dementia occurs. Rarely the progress is ar- rested and cure takes place. The types described are the simple or hebephrenic, katatonic, and paranoid.
Etiology. — Dementia praecox is a disease preeminently of the de- velopmental period of life.*
Age. — Most cases occur before twenty-five, and the most dangerous period is from twelve to twenty-one inclusive.
In one-half the cases there is no history of insanity in the ante- cedents, and direct heredity is not very often observed. The disease is due to a congenital or inherited defect of some kind, however, in about seventy per cent, of cases. Alcoholism in the father is some- times observed. The two sexes are about equally affected. The patients are often as children rather intelligent and apparently well endowed physically. But though they may have stood well in school or college, they will sometimes confess that it was always hard work. Serious attacks of children's diseases, rapid growth, exhausting study, overexertion in athletics, fright, shock, emotional excesses, childbirth with exhausting sequelae are all exciting causes. In
* Among 125 cases admitted to the psychopathic ward of Bellevue Hospital in 1903, 70 per cent, were under twenty-five years, 16 per cent, between twenty-five and thirty. It may occur, however, as late as the fourth decade of life (14 per cent, in the above cases) and even later. There is a slight preponderance of females. The hebephrenic and simple forms are most common, the katatonic next, and the paranoid form least (59, 40, 14). The last form occurs at a rather later age, there being only 2 between sixteen and twenty-five, 7 between twenty-one and thirty, 4 between thirty-five and forty (Gregory).
Among 2,197 admissions to Bellevue Hospital psychopathic wards there were 382 cases of dementia praecox, or about 13 per cent, (females 203, males 179); hebephrenic type, 103; paranoid, 78; katatonic, 54. In two-thirds of my personal cases the disease began between fifteen and twenty-five, most being between fifteen and twenty.
There was a history of insanity in the family in 8 among 30, of alcoholism in 3, of epilepsy in 1, unknown in 8, good history in 10.
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DEMENTIA PRECOX. 709
general, dementia praecox acts like an exhaustion psychosis occurring in an unstable and abiotrophic constitution.
Dementia praecox makes up about one-fourth of institutional cases.
Hebephrenic Type. Symptoms. — The disease usually begins with a prodromal period of one or two years, characterized by a neurasthenic, hyopchondriacal, and often querulent and irritable condition. Then there is a period of more or less acute mental dis- turbance, followed by a period of remission or of permanent dementia. The simple and hebephrenic type is the more quiet form, but there is not always a sharp distinction between it and the katatonic or para- noid forms.
The following description is based on a study of thirty private cases whose history in many instances I have followed for years.
The disease develops rather gradually. The patient begins to show a lack of interest in work or study; he fails to finish his task, or is unpunctual and careless at his work; he loses his activity and liveliness of spirits, and, without being actually melancholy, shows some evidence of worry, due perhaps to a consciousness that he is not mentally himself. He may become brooding, irritable, and suspicious, and suddenly perhaps break out in an unreasoning gust of passion. He gets listless and neglectful; if at school or at some regular work he gives it up and stays at home. He no longer reads his books or takes interest in former amusements. He complains of some headache or head pressure and sleeps poorly. This condition may go on for half a year or even for one or two years, the symptoms being interpreted as neurasthenia. Then some delusion or obsession, gener- ally of a persecutory kind, develops. He thinks people are watching him or working against him, or that he has made some serious mistake in his life. He misinterprets things he reads, remarks made, or even casual gestures. There may now occur some outbreak of sudden violence in which he breaks something or tries to injure those about him or to fly from some fancied pursuer. His memory becomes poor, his power of concentration and attention weak; he will sit for hours look- ing at the same page of a book or trying to finish a letter. He becomes careless of his dress and person. He breaks out at times in silly laughter; makes curious gestures or bodily movements (tics). When urged to do anything he opposes it, even the movement of his body, like rising from a chair or getting out of bed, or eating as requested. He may have sudden attacks of violence requiring restraint, in which he breaks the furniture or tries to get out of doors. Sometimes he runs away from home and walks aimlessly for miles till exhausted. After a few months or a year he may improve and get for a year or two nearly
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DISEASES OF THE MIND.
well, only to return to his former condition, which then progresses till he gets into a state of comparative dementia. His mind is then all the time filled with delusions and hallucinations; he sits all day rock- ing in a chair or staring at a wall, muttering or laughing. At times there are again outbreaks of violence. He has no sense of orientation; he recognizes only those known intimately. He is apathetic for a time then again becomes excited and uses copious, dramatic, and violent language. The reflexes are exaggerated, the pupils usually dilated, the sensibility to pain is lessened, and there may be vasomotor disturbances, cyanosis, local cedema, and dermography. The sali- vary flow is increased, The bodily temperature may become some- what lowered. Sleep is generally disturbed, the appetite and diges- tion are fairly good, the bodily weight generally decreases. Probably serious metabolic changes occur, but their exact nature and signifi- cance are not yet known. It is possible that an autotoxaemia is an important factor in the development of the symptoms, especially of the acute type. The final stage of partial or complete dementia may last for years. About seventy-five per cent, become absolutely demented and about eight per cent, get well (Kraepelin).
Simple Dementia Precox. — This is a gradually developing dementia, a milder type of hebephrenia, which comes upon persons usually just after the period of puberty, but sometimes not till as late as the third decade. These patients gradually lose interest in their work or study then drop it, become careless, listless, inattentive, cannot be aroused to any interest in life or be stimulated by any possible means to work or effort. They develop moral and sexual defects, and in accordance with their condition in life become helpless imbeciles at home, or vaga- bonds or asylum and workhouse inmates. The dementia may be arrested at a certain stage or may end in making them helpless wrecks.
Katatonia is the name given to a form of dementia praecox characterized by a progressive dementia, with periods of excitement and stupor or katatonia, with coincident phenomena of negativism, impulsive acts, stereotypy, perverseness, and suggestibility. The patient here suffers at first from depression and hypochondriacal ideas. There then develops perhaps quite suddenly a condition of delusional excitement with hallucinations, the condition resembling that of hebephrenia. About one-third of the cases, however, pass into a state of katatonia or stupor. Some cases also pass directly from a hypochondriacal and neurasthenic state into one of katatonia; or instead of passing into a real stupor or catalepsy they are simply apathetic and slightly cataleptic, or go through simple stereotyped
DEMENTIA PRECOX.
7II
movements, or assume at times fixed attitudes, at others talk inces- santly, loudly, with senseless repetitions (verbigeration). They exhibit also the foolish resistance to requests made, called negativism. Instead of stupor there may be katatonic excitement. About fifty- nine per cent, pass into a state of dementia (Kraepelin). About twenty-seven per cent, reach a moderate grade of dementia, leaving them in a condition formerly called chronic mania, with mild and often grandiose delusions. About thirteen per cent, get approximately well.
Paranoid Type. — Here the disease is ushered in by a period of depression, worrying, and suspicion. These suspicions gradually develop into some systematized delusion. This is usually one of a persecutory or grandiose type. In most respects the disease resem- bles a subacute paranoia, with evidences, however, of mental enfeeble- ment; at times explosions of violence, and finally more or less dementia. The cases of dementia paranoides are often mistaken for paranoia, and t is still a question whether they should not be grouped with this tvpe.
Variations in Onset. — The disease may begin suddenly with a very acute outburst of mania, followed by stupor, and then by symp- toms of dementia as indicated.
The patient may, after a neurasthenic and psychasthenic prodrome pass into a katatonic and stuporous state, lying in this condition for weeks, then gradually coming out and having a remission in which there is still evidence of some disturbance.
The patient may develop his early symptoms as indicated, but the disease may then cease to progress, or nearly so, and he may remain under the dominion of some rather systematized delusion or delusions, simulating a case of paranoia
The diagnosis is based on the age of the patient (practically all cases develop before twenty-five) ; upon the presence of a period of neurasthenia and hypochondria; on the development of delusions with, at the same time, mental weakness; slowness of psychomotor response; loss of initiative and of capacity to do work; occasional violent explo- sions of mania, with hallucinations concerning the body; tendency to impulsive acts, self-mutilations, violent masturbation, stereotyped movements, negativism, katatonia.
The paranoid type is distinguished from true paranoia mainly by the dementia, the earlier onset and more rapid course.
The prognosis is unfavorable, but not absolutely so. The patient usual y has a remission, and he may get partially well, and remain slightly demented and delusional for years. More often he gets seriously demented, and remains so almost indefinitely. It is in the
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DISEASES OF THE MIND
cases which are seen very early before the disease really develops that some real hope exists.
Treatment. — Children of psychopathic families should be brought up with especial care to prevent overstrain in their educational and personal life. Art and religion, and everything that tends to stimu- late the emotional side, should be inculcated with temperance. They should be made also to avoid too strenuous an athletic life. They should be taught order and self-control, regularity of habits with abundance of sleep, and freedom from alchool and tobacco. Fat- tening and non-nitrogenous food is best for them. They must until mature be kept on rather a low mental plane, competitions and exciting experiences in school and college being avoided. Masturba- tion is to be especially cautioned against. If the disease develops, rest and outdoor life are the main factors. Attention must be paid to the possibility of an autotoxaemia. Arsenic is a remedy that some- times does good. When the attack has become serious, the best thing is institutional life.
Dementia Precox. — Simple apathetic deterioration; early ideas of reference; at puberty an hallucinatory episode, followed by profound apathy and mutism.' Later an occasional transitory excitement.
S. F., admitted to the Hospital, November 25, 1903; Jewess, aet. 17.
Family History. — A maternal aunt committed suicide. The pa- tient's mother is nervous, has spells of anger, suffers from headaches.
Personal History. — The patient was an ill-tempered child, who did poorly at school; she was shy and unsocial, hid away from visitors, was not interested in sports as other children. While still at school she complained that as she passed along the streets Italians made eyes at her from the cellars; she thought that a man who lived on the op- posite side of the street was always looking at her.
At the age of 14 she failed to get promotion from the fourth grammar class. She then refused to return to school, sat about the house entirely indolent, seemed to have lost all interest, began to neglect her person. She refused to bathe or comb her hair and would get into bed with her clothes on. When remonstrated with about her idleness, she remarked: "I was not born to work." She spent most of her time reading cheap novels. At night she prowled about the house looking for food. Her parents knew nothing of her sexual habits. Menses appeared at 16, but ceased after a few months.
A month before she was brought to the Hospital she began to have "raving spells" and violent outbursts when interfered with. Occa- sionally she macte some queer remark, e.g., "I am Christ — I am going to be crucified." She overheard the people next door saying : "Chase her out." "Why don't you shoot her?"
When taken to Bellevue she was apathetic and listless, responded to questions only when urged. She said: "I could get nailed up outside the window, I hear people talking about me . . . they say this and that. I am Jesus Christ, I see a cross outside the window."
DEMENTIA PRECOX.
7*3
On admission to the State Hospital she was quiet, smiled in a simple way, said there was nothing the matter with her. She was markedly indifferent, held saliva in her mouth; her speech was low, sometimes she merely grunted when questioned and was inclined to answer "I don't know" even to the simplest questions; when urged to give infor- mation, she made contradictory statements. She denied that she had had any trouble at home, and when asked about the statement she made at Bellevue she replied: "I didn't mean to say that." She denied hallucinations; no trend of delusions could be elicited.
The physical examination showed increased knee-jerks, but no signs of bodily disease. Menses suppressed.
Following her admission, she was quiet and passive, retained saliva in her mouth for a long time then would spit upon the floor; she said nothing spontaneously except to ask a number of times in a stereotyped manner: "Can I go home?" She persisted in showing an utter disregard for consistency in her statements, would giggle in a senseless way and answer at random. There was no muscular tension or catalepsy. She soon ceased to ask about going home and refused to answer any questions; became filthy and obstinate.
At the end of two years in the Hospital she was profoundly apathetic ; she sat all day gazing in a stupid way; she never stirred from her chair unless prompted; required to be dressed and undressed; she refused to speak even to her parents when they visited her. At times there was slight muscular stiffness in the limbs.
Now, after four and one-half years in the Hospital, her condition remains unchanged. As a rule, she is silent, pays no attention to questions, is filthy in her habits. The apathy is occasionally inter- rupted by short episodes of excitement, then she will destroy cloth- ing, beat her arms, and resist when interfered with. (G. H. K.)
Dementia Precox. — Hebephrenic deterioration; loss of energy and capacity, exhibition of serious judgment defect and diversion of interest into unhealthy channels; sexual ruminations ; masturbation and noc- turnal agitation over dreams, followed by ideas of reference and feelings of shame. Peculiar senseless ideas, feelings of physical and mental influence, marked incongruity between thought and mood. Silly laughter and apathy.
M. O., stenographer, aet. 21 ; admitted to the Hospital, August, 1907.
Family History. — Father drank to excess in youth, failed to get his medical degree, developed ideas of persecution and finally at the age of 37 disappeared from home. The patient's brother is at present in the Hospital, a case of dementia praecox.
Personal History. — The patient was a healthy child, bright in her studies. At sixteen she took up dressmaking, but this did not suit her so she learned stenography and typewriting; she held one position for two years, but was dismissed in October, 1906, because of tardiness in reaching the office. She had grown indifferent and lazy and the dis- missal did not worry her at all. She tried several other positions, but could not hold one for any length of time. In addition to her waning interest and failing efficiency at work, a further change manifested itself in the patient. She became discontented at home, induced her
714
DISEASES OF THE MIND.
mother to move into a new apartment, the expense of which was be- yond their means. She talked of going on the stage and wished to take dancing lessons. She became very seclusive, did not go out ex- cept to business or church; she spent her time at home reading theatri- cal journals and romantic stories; she bought peroxide to bleach her hair and also a preparation to remove the hair from her arms.
During the summer of 1907 she spoke of attention from a young man at the office and one day she said he had put his arm around her. She then gave up her position and the following night she awoke screaming, told her mother of impure thoughts about the young man and confessed to masturbation. After this she appeared depressed, threatened to end her life. A few days later she was brought to the Hospital.
On admission the patient was quiet, spoke in a subdued voice, did not like to look directly at the physician, covered her eyes with one hand. She expressed much horror at being in an insane hospital, made anxious inquiries, but at the same time smiled easily. She thought the other patients were talking about her; she complained that her mind wandered and that her brain was turned. She reproached herself for masturbation practiced since childhood; she told of sexual longings and of her interest in a young man in the office. He had made lewd motions and she had dreamed of him. She felt ashamed to look at men. "I thought they saw what I had done in my eyes." "I know I ruined myself by the habit. I had the impres- sion that every man looked at me in the wrong way so at the end I was ashamed to go in a car." The patient was clearly oriented, her memory was good, she did calculations rapidly and correctly. Physical examination revealed nothing of importance.
Following her admission, she was languid, talked in a whining way, still complained much of her mind being mixed up, of inability to concentrate, said bad thoughts kept coming into her mind. She was inattentive to questions and her thoughts seemed to be at times markedly scattered. She covered her face and was ashamed to look at the physician. She complained often of hearing strange voices, of feeling electric shocks, of strings running through her skin. She uttered many apprehensive ideas, but showed no fear; e.g., she remarked with a smile: "I am afraid of being killed." Frequently she would whimper and throw herself on the floor, claimed the voices said she should drop dead. The voices also suggested lewd things about Christ and the devil. Once she sprang up from the chair and com- plained that people downstairs were pulling her with strings. She lounged about idle and apathetic, neglected her personal appearance. She expressed many strange ideas and described peculiar feelings: "It seems that people got possession of me some way through my ears." "Something twisted my brain — these people that walk around talk with their feet — my brains have floated down my back" (Smil- ing). She said in regard to her thoughts : " They fly out my ears and float." Other strange ideas were: "I thought I was Jesus, thought I was a bird and a parrot." She was often seen smiling and moving her lips as she sat alone. At times she would suddenly burst out
DEMENTIA PRECOX.
giggling. Frequently she was heard to call out, "No; no!" and when questioned, she said: ilThey are using my brain."
After nearly a year in the hospital she is indolent and slovenly; very inattentive when questioned, giggles and makes irrelevant remarks. (G. H. K.)
Dementia Precox (Catatonic Form). — Onset with hypochondri- acal depression, ideas of reference and worry over masturbation. Then peculiar acts and absurd notions: burned her arm to save the world; transformed into a baby. Reiteration of pious phrases. Hallucinations and episodes of excitement, followed by stuporous state, with negativism and catalepsy. Deterioration into profound apathy, with senseless stereotyped replies to questions.
C. H., admitted to the Hospital, October 10, 1904; single; set. 21.
Family History. — Father moderately alcoholic.
Personal History. — The patient was a quiet, nervous child. She did fairly well at school until her eleventh year, when she was kept at home because she was thin, nervous and complaining of headaches. Mas- turbation from the age of nine. The patient says in regard to the habit: "It spoiled my life, I wasn't like other girls, I didn't want to go out anywhere." When she went to work she was timid, had headaches and soon gave up. A spot of gray hair caused her much worry and increased her bashfulness as she feared remarks might be made. She remained at home and kept house for her father, was alone much of the time.
The psychosis developed gradually during her twentieth year. She was then hypochondriacal and depressed; she feared that she had a tape worm, suffered from constipation and hemorrhoids; the rectum was operated upon, but she grew more despondent, worried over her sexual habits and said she had wronged her father. She feared to be alone, thought people looked at her when she went out. The last few days at home she performed several peculiar acts. After praying she went to church dressed untidily, left candles burning in her room and poured kerosene on the steps for ' ' holy water. ' ' Finally, one night she ran out on the street in her night-gown and a policeman found her kneeling in front of a church. She was then taken to Bellevue where she said: "I hear angels telling me how to pray when I lose my thoughts— sisters and nuns all around me here to save and purify the world." While in Bellevue she held her arm under a stream of hot water burning herself severely. Later explained that she did this "to save the world."
On admission to the State Hospital she presented a state of beati- tude, walked with closed eyes, prayed and sang with slow gestures and theatrical pathos, passed readily from tears to smiles. She made strange statements, such as: "I have been transformed from a big girl into a small baby." (Why?) "The Lord said I was too pure to be a woman and in order to save the world I had to be once more a baby." Her limbs were slightly stiff, catalepsy in the arms.
She produced a monotonous religious jumble delivered slowly in speech or song with devotional gestures. "Divine catholic world — now and forever — a — men — glory be to the Father. Son and Holy
716
DISEASES OF THE MIND.
. Ghost as it was in the beginning .... At least you my friend sweet Jesus have pity on me — that He may lead us into a more pure, good, Irish divine catholic world."
Another sample of her peculiar talk: "Part of my hair is gray — that's what near made the whole world come to an end — they all laughed at me, even drew up a play about me called Devil's Island."
Her productions had no resemblance to a flight of ideas, but were mostly senseless utterances or moralizing and religious reiterations, with much stereotypy in form. "I want to save everybody and everybody wants to save me." "I had the worms and the worms had me." There was no evidence of delirium, the patient had a cor- rect appreciation of her environment and was clear as to time, her memory was remarkably good.
Physically, the knee-jerks were increased; dermatographia marked; pulse soft and rapid; a large burn on the right arm. Masturbation observed soon after admission.
Episodes of excitement occurred with assaults on the nurses. She sang, prayed, kneeled, ran to the window, shouting : "Save me, I am lost." At times she would not speak, held her body rigid and tried to do the opposite of what was requested (negativism). Peculiar bodily sensations and hallucinations were described. She heard angels, her dead mother's voice, received commands from God, etc. She talked of currents of electricity and felt that she was "in power."
Two weeks after entering the Hospital the patient passed into a state of partial stupor. She ceased to speak, the limbs showed waxy rigidity and catalepsy; she held saliva in her mouth, and was fed with a spoon.
After three months she was no longer stuporous but she did not speak spontaneously, was extremely inactive, stiff and constrained in manner, face immobile, hands cyanotic; she maintained her arms in given positions, walked backward instead of turning about, gave peculiar stereotyped replies to questions.
Are you interested in things here? "Peace."
Interested in your brother or sister? "Peace."
Why do you sit that way? "Peace."
The patient settled into a state of profound apathy. She responded very little to questions, sat all day with hands in her lap, head slightly forward. She frequently smiled to herself, occasionally burst out into a laugh; at other times tears streamed down her cheeks. Whenever she answered questions the orientation was found to be approxi- mately correct. No change in the past three years. (G. H. K.)
CHAPTER VIII.
MELANCHOLIA AND MANIA.
Acute Melancholia. Chronic Melancholia. Manic Melancholia. (Manic-depressive Insanity).
The Symptom Melancholia. — -Melancholia is the name applied to a certain symptom group characterized dominantly by mental depress- ion, but showing also some slowness and difficulty in thinking, slow- ness of voluntary action (psychomotor retardation), and a narrowing of the mental horizon. The term melancholia may be used to indi- cate a symptom group such as is seen sometimes in dementia, in paresis and other psychoses.
The Disease Melancholia. — Melancholia, strictly speaking, is a special disease, and occurs under three forms: (i) Acute melancholia; (2) chronic melancholia; (3) manic-depressive insanity or manic melancholia. These are all closely allied types, so much so that the term "manic-depressive psychosis" might also be used to include all three.*
In non-asylum practice the majority of cases observed are acute and chronic melancholias. In asylum practice acute mania or manic melancholia, alternating or mixed, are nearly as often seen. For the general practitioner it seems well to bear this fact in mind, for he will see and treat twenty cases of acute or chronic melancholia to one of acute mania or manic melancholia. It seems unwise to use the term "manic-depressive insanity" for a large group of cases which never show any signs of mania, and I prefer "acute melancholia" and "acute mania" when cases are such, and the term "manic-depressive in- sanity" only when there is really a combination of the two. But the groups have a fundamental connection in etiology, pathology, and course.
Acute Melancholia (simple, functional, recurrent) is a functional psychosis, characterized by great mental depression, apathy, and loss
*My experience is that there are many acute melancholias arising in middle and less often in early life which always remain melancholia, and either end in permanent recov- ery or finally take the form of chronic involutional melancholia when the patient gets older. These acute melancholias have often the mark of the anxious, worrying, hypo- chondriacal psychosis of late life. On the other hand, there are also acute simple mel- ancholias, mostly of early life, with a more decidedly bad heredity, a more certain and frequent periodicity. These are the types which show alternations and mixtures with acute mania.
717
7i8
DISEASES OF THE MIND.
of interest in the usual affairs of life, by difficulty and slowness of mental activity, and usually by some depressing delusions. It runs a course of from a few weeks to a year, or even more, but usually lasts less than a year, with a tendency to recur. It is accompanied by in- somnia, cerebral and precordial distress, loss of appetite, dyspeptic troubles, emaciation, and often by a subnormal temperature.
Etiology. — It occurs about equally often in men and women (men, 28 ; women, 35). It is seen oftenest (eighty per cent.) between the ages of twenty and thirty-five years, but may occur even in old age. It is often excited by severe domestic or financial losses, by severe illnesses and overwork; but it may come on with very little cause, especially in second attacks. Heredity is an element in twenty-five per cent, of cases.
Symptoms. — The disease begins with a tendency to loss of sleep, lack of interest in work, and a general feeling of depression. In the course of a few weeks the patient usually develops a depressing de- lusion, such, for example, as that he has made some irretrievable mis- take in business, or has neglected a duty, or has committed some sin- ful act. Sometimes the mind goes back to sexual indiscretions of earlier life, or to some lapse in religious observance. The patient be- comes entirely centered on himself and his hopeless condition. He cannot for a moment forget his trouble, and does every act with a kind of double consciousness, the background of his misery being always present to him. Attempts at work or play or diversion are futile. He either is moodily silent or he cries a great deal and talks of his suf- ferings. His conscious horizon is limited to himself. He is stupefied by his gloom, and thought is slow and attention painful and difficult. He has to be urged to eat and to go out, and he sleeps only by artificial help. Suicidal ideas appear and suicidal impulses very suddenly rise. Unless watched, he suddenly jumps for a window or rushes to a poison bottle or tries to injure himself with a knife or by strangulation. Or suicide may become an obsession and shrewd plans be laid for carrying it out. In about fifteen per cent, of cases such a suicidal tendency is present. The patient rarely has hallucinations or illusions, and often no real delusions. There are a weakness of will and hopeless indecision of purpose; there is never real dementia, though his apathy and slow- ness of thought may suggest it. The patient suffers often from dys- pepsia and constipation, and becomes somewhat emaciated and anae- mic. In women the menses are irregular and in men sexual feeling is lost. Acute melancholia soon reaches its height and then the condi- tion continues, with variations and occasionally short remissions. There is not often great agitation of body over the delusion and the
MELANCHOLIA AND MANIA.
719
suffering, but the patient may write long letters explaining his suffer- ings, or talk continually and with intense feeling of his hopeless con- dition. Slowness of thought and apathy are interspersed with keen activity of feeling and expression. In a series of one hundred conse- cutive personal cases only three showed any maniacal excitement, and this was at the beginning of the attack.
The symptoms gradually ameliorate, and in from three to nine months the patient is convalescent. But it takes several months more for the tone of the patient to be restored. Relapses occur in about one-third of the cases. These take place in from two to five years in mild cases. In the severest cases they come on every year, and grad- ually pass into a continuous and hopeless condition.
The cases that begin early, before twenty, are most apt to relapse soon and often. The later the development the longer the period of intermission, as a rule. In some cases women have one or more at- tacks at adolescence and then no more till the climacteric.
When the disease begins after thirty-five the later attacks take often the form of chronic melancholia, or melancholia of involution. Recurrence was observed in thirty-three per cent, of my cases. Thus it cannot be said to be the rule in this type of non-manic melancholia.
Variations in the onset and course of acute melancholia occur. It may start in very suddenly with a violent maniacal outburst, or it may come on suddenly but quietly. Acute melancholia takes a wide range of intensity. In very mild attacks there is really no melancholia, but only great apathy, indifference, entire lack of interest in former activi- ties, and dislike of thought and action. These cases are often called neurasthenia.
The prognosis is favorable so far as the attack is concerned. Prac- tically all get well. Recurrence is likely, but not necessary. Acute melancholia, with no associated mania, is much less serious than the manic-melancholia form.
The diagnosis has to be made from neurasthenia and from the de- pressed states seen in paresis, dementia praecox, and exhaustion psy- choses. In melancholia there is a depth and intensity to the melancholy, and a stupor of thought and activity which is most characteristic. This ought to be sufficient to distinguish the trouble from neurasthe- nia. Depressing ideas amounting perhaps to delusions are usually present. There are rarely hallucinations and never real dementia. A melancholia may be imposed upon paresis, but the paretic has physical symptoms which distinguish the case.
Chronic Melancholia (Melancholia of Involution, Hypochon- driacal Melancholia, Melancholia Agitata). — Chronic melancholia is a
720
DISEASES OF THE MIND.
form of mental disorder allied to acute and manic-depressive types, beginning after the age of forty, running a long course and character- ized especially by anxiety, depression, restlessness, agitation, and hypochondriacal delusions.
Etiology. — It occurs somewhat oftener in men than in women, and in over half the cases begins between forty and fifty. There is a de- fective heredity in a minority of cases. There is often a history of neurasthenic or mild melancholia occurring in early or early middle life. The patient is usually one who has led a life of hard work and anxiety, and is often of a naturally worrying disposition. The dis- ease is then a kind of evolution of the worrying habit. There may be decided signs of presenility and arterial sclerosis; diabetes or glycosuria is found in a percentage of cases. Exciting causes may be absent, or if present are like those in other psychoses. Chronic melancholia of a very clear, agitated, and hypochondriacal type develops sometimes on a basis of paresis.
Symptoms. — The illness begins with symptoms of irritability and insomnia, lack of interest in work, and disagreeable head symptoms. There is a feeling of anxiety about financial and domestic matters, or of concern about the health. Fear of some impending illness or bodily weakness appears, or the patient develops some particularly insistent idea, which becomes later a real delusion. He thinks he is ruined or has brought some disgrace on his family; or has done injury to some one in the past; or thinks he cannot swallow food and refuses it ab- solutely, and has to be fed by force. Sometimes his idea is that the food cannot be digested or passed through the system, and that he has grown to enormous weight in consequence. Cases in which there is a single delusion and not much depression are said to suffer from a presenile delusional psychosis; but always the delusion is one of depres- sion and hypochondria, and leads to states of worry and self-abase- ment and to convictions of absolute hopelessness of his case. There may be decided suicidal feelings, or the delusion is so annoying that it keeps the patient restless, agitated, and constantly talking and crying and wringing the hands. Thus, in accordance with the nature of the mental symptoms, the psychosis is called hypochondriacal, agitated, suicidal.
There is a complete inability to take interest in anything but self (circumscription of consciousness), although the talk may be con- tinually of the harm he has done and of expressions of sorrow for others. Hallucinations and illusions are rare; the memory is good, and there is no dementia even in the worst cases unless senile changes develop. These patients are often violent in their attempts at suicide
MELANCHOLIA AND MANIA.
721
and refusal of food, and require great care from attendants. There is usually some emaciation, and evidences of age appear; the hair be- comes very gray, the ace more wrinkled. There may appear as the result of casual trauma haematoma of the ear and resulting deformity. But on the whole the bod ly functions are well performed and there is no great weakness.
These patients often have curious delusions about their bodies, and think they cannot eat certain things or must sit in a certain way lest they be injured. They wrap themselves in cloths to protect themselves from draughts, and sit only in specially arranged chairs. They think they cannot walk or ride, and perhaps believe they cannot breathe or eat or digest their food naturally. They think if things had been done in some different way they would be quite well, and lament some fancied oversight or mistake on the part of themelves or their doctors; or perhaps they think they have originally been made wrong and never can be different.
The disease runs on for from two to six or even eight years, and the patient may even then get perfectly well, but with a probability of a relapse. Those who have decided and deeply fixed hypochondriacal delusions and who think they cannot eat, refuse food, and are actively agitated over their conditions, represent the severest type. The cases of simpler type, with ordinary delusions of depression regarding ex- ternal things or past acts, recover more quickly.
The prognosis is not nearly so good as in simple melancholia, but in my experience even the severe cases may recover.
The treatment of melancholia consists, as in most psychoses, of rest and a regular life and regimen. Traveling and active attempts at diversion are generally bad; but in seme cases of a more agitated kind long drives about the country are beneficial, and a continual out- door life is always indicated. Something can be done to alleviate the suffering by the use of codeine and opium, and these must usually be given in small doses, gradually increased to the physiological limit. Hypnotics are usually necessary for part of the time, and prolonged sleep is a very important function to establish. Full feeding and, if necessary, forced feeding are required. It must be remembered that even in severe and old cases recovery is possible as long as no dementia sets in. The danger of suicide is to be guarded against and melancholia, more than any other type, requires the most careful guardianship.
Involution Melancholia (Anxiety Psychosis). — A gitated depression at the menopause; insomnia and worry over physical health followed by fear of punishment and torture; ideas of wickedness , cursed 46
722
DISEASES OF THE MIND.
by God. Hallucinations. Development of a fantastic and hypochondri- acal trend. Born without a soul, can never die, must suffer for sins of the world. Body empty, hands turned to stone, blood dried up, not a human being. Orientation and grasp clear; no motor retardation or feeling of mental insufficiency.
E. M., seamstress, single, aet. 40. Admitted to the Hospital October 19, 1907.
Family History. — Nothing is known of the parents except that the mother, "a worrisome woman," died at the menopause.
Personal History. — The patient had a limited schooling, was left an orphan at the age of twelve. At fifteen she was seduced, had an ille- gitimate child which died when three months old. This early fall caused her much worry and remorse in after life. She was always troubled because she was "not pure like other women." She refused several offers of marriage rather than deceive anyone. She lived a quiet se- cluded life, showing a rather unsocial disposition and having practically no friends except the family with whom she lived. Religion did not interest her; she did not believe in heaven or hell. She worked steadily, took no alcohol. Menstruation ceased four months after the onset of the psychosis.
The Psychosis. — Six months before admission, the patient com- plained of dimness of vision and specks before the eyes. An oculist gave her glasses and some medicine, but she thought the medicine affected her badly, caused a lump in the throat and interfered with her swallow- ing. She suffered with insomnia, became greatly depressed, feared that she was going blind, threatened to end her life. She soon began to say that she could never die, that it would be necessary to burn her up or throw her in the water. She was anxious and restless, walked the floor and wept. At night she was sleepless, would often get out of bed and awaken the family because she did not wish to be alone. She was taken to a sanitarium and there she refused food claiming that she couldn't swallow because her throat was closed up. Other ideas now developed and hallucinations occurred. She claimed to have no soul; she must walk the earth alone or if buried she would live for ever under the ground. One night she heard chains rattling and a large black negro walked back and forth before the door of her room. She believed that a Mrs. W., another patient in the sanitarium, was "the devil in human form." Mrs. W. called her a "bitch," talked of her past life, said her eyes and heart would be cut out, she would be burned alive, etc. The patient showed much fear and agi- tation and each night she insisted that it would be her last one on earth. At the end of six weeks she was transferred to the State Hospital.
On admission the patient wras anxious and watchful, moaning, looking about uneasily and starting nervously at any noise. When asked to explain her anxiety she replied: "I am going to be put in the hot boiling water to-night, in the dark it will be." She said further that she had been wicked, that God had put a curse upon her and .she asked if Mrs. W. was not in the hospital. Later she claimed that she heard Mrs. W. screaming downstairs and thought she had followed
MELANCHOLIA AND MANIA.
723
her from the sanitarium, maintaining that Mrs. W. was the devil in human form.
Physically the patient was reduced in nutrition; knee-jerks slightly increased; pharyngeal reflex absent. Menstruation had ceased two months previously.
The patient was perfectly clear as to her whereabouts, and correctly oriented as to time; memory for the recent and remote past unimpaired. She spoke without slowness and showed no retardation in movement. The usual thinking tests (counting, simple calculations, recalling dates, etc.,) revealed no thought difficulty or confusion, but it was evident that she concentrated poorly because of her preoccupation with depres- sive and apprehensive ideas. Mental operations requiring much ap- plication were therefore performed after some delay, still the patient admitted no feeling of mental insufficiency, and she explained her fail- ure to give quick responses by saying : ' ' I am thinking of something else, thinking of what's going to take place." "My thoughts are just on the one thing," etc.
Within a few days the agitation became greater; she walked the floor groaning, picking flesh from the tips of her fingers and other parts of her body, and rubbing her abdomen. Each day she claimed that the coming night would be her last — "I am going dawn stairs to-night, going to be transferred, put into hot boiling water, thrown into the fire, my heart cut out and my eyes." She was somewhat annoyed at the questions, regarded them as foolish — "This is all non- sense because I am going straight into hell to-night — don't be writing anything down — Oh dear ! Oh dear ! If you were going where I am you wouldn't want to answer any questions." The patient gave no satisfactory explanation for her fears, merely said: "I know what's coming to me." No account of hallucinations could be obtained at this time to explain the anxiety. She denied that she heard any voices, but said. "It is what will come to pass, you may not be liv- ing when it comes, to think I was put on earth for such a curse (weeping) —I wish someone would put a stone around my neck and throw me into the water, I am not a human being, I am not like the other people here."
Under hypnotics her sleep improved and the agitation diminished, but otherwise there was no improvement. She began to claim that she was Eve, had been born without a soul, could never die, she must suffer for the sins of the whole world. She spoke bitterly and railed against God for punishing her. "Oh what has He done to me, that God they talk so much about — such a rotton God to do what He has to me — if He made me the craziest one in the house I would be thankful."
For a time she refused food, maintaining that she could live for ever without eating. It was necessary to feed her by tube. Occasion- ally she was irritable and threatening, struck other patients.
After five months in the Hospital hypochondriacal ideas became pfcpminent — she claimed that her hands had turned to stone, that her blood was all dried up, asserted that her body was empty: "I am hollow inside — I have no waist line like you have — I don't have to
724
DISEASES OF THE MIND.
eat to live — I am not insane — I have no brains — you could shoot me through the heart and I wouldn't die — I told you I was put on this earth for a curse, born without a soul, and put here to be tortured." She would allow a pin to be stuck through the skin of the hands without flinching, claiming that she was insensitive to pain.
With the subsidence of the agitation the patient showed a disposi- tion to employ herself and was quite efficient in assisting the nurses.
July 1908: The patient is outwardly calm, she works well, has a grim facial expression, rarely speaks to anyone spontaneously, but when interviewed she talks freely, weeps copiously; claims as before that she is to be tortured, that she can never die, that she was born without a soul. She remains clear in orientation and grasp; there is no slowness in speech or action. She has never admitted any feeling of mental insufficiency. Menstruation remains absent. Sleep impaired.
(G. H. K.)
Climacteric Insanity. — The period of middle life is attended in many with an increased degree of nervous instability. This occurs earlier and rather more often in women than in men. There is an in- creased tendency at this time among the predisposed to mental dis- turbance, but there is no characteristic climacteric insanity. Most of the psychoses of this period are the melancholias, but a systematized delusional insanity or a phrenasthenia may develop then.
Manic-depressive Insanity (Acute Mania or Manic Phase of Manic-depressive Insanity, Mixed Forms, Circular Insanity). — While the acute and chronic functional melancholias just described are closely allied to the general disease, manic-depressive insanity, we have a distinct group of symptoms forming acute mania, this being its mania phase.
Acute mania, functional mania, or simply mania is the active phase of manic-depressive insanity. It is a disease characterized by great mental excitement, exaltation of feeling, clearness of consciousness, mobility of attention, flight of ideas, fleeting delusions, impulsions, and great psychomotor activity. It is called acute, but it runs a course of from a few weeks to many months, and wrould, from a general medical standpoint, be called subacute. Acute mania is a special disorder and is to be distinguished from maniacal excitement, which is a symptom occurring as an episode in most psychoses.
Mania is the condition most sharply identified in the popular mind with insanity, yet it is one of the rarer phases, constituting only fifteen per cent, of cases.
Mania may occur with melancholia in what are termed mixed forms, and it may alternate with it in circular insanity.
Etiology. — The disease occurs oftener in women than in men. Most cases appear before the age of twenty-five. Defective heredity
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is a very important factor, being present in about seventy-five per cent. The patient generally shows from early life evidences of the psychopathic constitution. The first attack is often brought on by mental shock, acute sickness, childbearing, or alcoholic excesses. The later attacks often occur without noticeable cause.
The symptoms begin with prodromes of depression and irritability or of unusual nervousness. Rather suddenly then the patient be- comes excited, restless, and talkative. The speech is extravagant and irrational, and the actions become so violent and irresponsible that restraint of some kind has to be employed at once.
In most instances the very violent demonstrations are either not present or subside, and the patient shows only the exuberance, men- tal activity, and reckless physiogonomy of one who is joyously in- toxicated. The face wears a smile and is slightly flushed, the dress is disarranged, and the body at times is quiet and then suddenly springs into activity or violence. The mind is alert, orientation and memory are good, and the patient delights in what he thinks are smart replies or turbulent comments. The judgment is profoundly disor- dered and the mind is full of unstable and expansive delusions; but hallucinations are rare. There is an appearance of euphoria, together with great irritability and tendency to outbreaks of anger. The moral sense is disturbed and the patient may be erotic or vulgar in speech. The volubility of speech and activity of thought may show themselves in copious letter-writing.
The pulse is rapid and full, the breathing accelerated, the appetite good, and the patient may gain weight. There is usually insomnia, and in women the menses are suspended. The urine shows an increase in metabolism and an excess of phosphates.
Mania sometimes takes a peracute or delirious form, generally due to some complicating alcoholism or infection or specially depressing previous experience. There may also be a mania in which the ideas are confused, and there are great disorientation and hallucinations. This condition is also caused by some special somatic disturbance. Sometimes there is a hypomania with milder manifestations of the characteristic symptoms.
Attacks of mania may alternate with attacks of melancholia. In general the alterations and combinations of periodical mania and melancholia take various types termed alternate, circular, mixed, and atypical.
The melancholia, which alternates with or substitutes itself for mania in manic-depressive insanity, is characterized, by much the same symptoms as those described under the head of acute melan-
726
DISEASES OF THE MIND.
cholia. These cases cannot in fact often be practically distinguished, nor is it necessary. The manic-melancholia, however, occurs early in life. There are more marked heredity, more serious original mental defect, and more profound mental disturbance, more certain and frequent periodicity.
In circular insanity the patient has a continuous cycle first of mania (or melancholia), then directly a melancholia (or mania), then a period of health. The attacks last from six to twelve months and the periods of health for a rather longer time. A cycle of this character may at- tack those who have acquired degenerations, such as the alcoholic and the paretic.
The disease runs a course of a few weeks or months, and it may last a year. Usually there is a sharp beginning, a slight subsidence, and finally gradual clearing up. Sometimes the course is interrupted by a day of quiet or even of depression.
Prognosis. — The patient usually recovers from the attack, but recurrence of either a mania or melancholia is to be expected. Al- though the disease is not a dementing psychosis, yet if the attacks are numerous the mind becomes permanently affected.
Diagnosis. — The disease must be differentiated from symptomatic maniacal excitement. This may occur at the beginning of dementia prsecox, or in the mania of alcoholism and the exhaustion or toxic psychoses. Maniacal excitement may usher in general paresis. In dementia praecox the mania rapidly subsides and katatonia and evi- dences of dementia appear; in alcoholism the hallucinations and dis- orientation are characteristic, and in exhaustion delirium the cause is present. In general paralysis the physical symptoms are indicative of the nature of the trouble.
Treatment. — -The indications for treatment in mania and melan- cholia are much the same. The patient must be put under watchful care to prevent violence and suicide. Full feeding with laxatives to prevent autotoxgemia is necessary. Often the nasal tube must be used for a time.
The continuous warm bath is used for maniacal conditions in some institutions, but it is very expensive and requires much personal at- tention. Hyoscine and scopalamine are useful, but they should be given with care. A mixture of morphine gr. J, hyoscine hydrobro- mate gr. y^, apomorphine gr. TV» *s often very effective. The ordinary hypnotics, trional, veronal, sulphonal, usually have to be employed. The best form of physical restraint is obtained by the use of roller bandages, sheets, and intelligent attendants. Canvas jackets and other forms of special apparatus are rarely needed. There is some
MELANCHOLIA AND MANIA.
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question whether any abortive treatment of manic-depressive insanity is possible or wise. The main object of the physician, after all, should be to see that after recovery future attacks be warded off. Therapeu- tic measures for this purpose have not been sufficiently worked out.
Manic-depressive Psychosis. — Recurrent mania, no attacks of depression. Excitement characterized by motor restlessness , loquacious- ness, flight of ideas and distractibility. Numerous grandiose ideas and a pronounced tendency to fabrication. Mild persecutory trend.
C. B., admitted to the Hospital February 14, 1908; house-wife; aet. 44.
Family History. — Father alcoholic. Mother weak-minded. A paternal uncle insane. A maternal aunt peculiar. Two sisters sexually immoral.
Personal History. — The patient has always been rather nervous, but she was bright and ambitious, efficient at her work and sociable indisposition. Married at the age of twenty-nine; no children; tem- perate habits except for occasional excesses during the attacks of mental trouble.
At the age of thirty-five, patient was upset over her sister's immor- ality; she became nervous, restless and unusually talkative. She out- lined grandiose plans for a home for cats and dogs and claimed that she knew all the wealthy people in America. She spent money fool- ishly, accused her husband of infidelity, locked him out, threatened to burn the house, imagined the food was poisoned and threatened some neighbors whom she disliked. When brought to the Hospital she was restless and loquacious, said she was going to help Parkhurst clean up the city, asserted that she had been persecuted since the age of three, accused her mother of having used her as a harlot. She calmed down quickly, the delusions were given up, she went home recovered at the end of six months and remained normal for two years.
The second attack, at the age of thirty-seven, was an excitement very similar to the first one. When again brought to the Hospital, she was talkative, self-assertive, abused the nurses, claimed that she was going to start a hospital herself. The excitement gradually subsided and she returned home at the end of ten months.
The next attack at the age of fourty-one was precipitated by the death of her mother. The following day the patient began to talk ex- travagantly and all of her grandiose plans cropped up again; she talked of her musical ability ; said her husband was to be President of the United States, she was going to write a book, etc. When re-admitted to the hospital she was talkative and boastful; claimed that she was "mag- netic," said her husband was a hypnotist; she accused her mother of incest, called her a fiend who had tormented her long enough. Fabrications abundant. "If I rub a sick person's back I can take away their pain — If he (husband) rubs me all my body is paralyzed — I have always been a nurse, not a trained nurse, a practical nurse — I should like to study men's diseases — I'm sick of studying women's diseases ... I cured myself of small-pox . . . my husband is an alderman and my brother is running for President — Hetty Green the
728
DISEASES OF THE MIND.
richest woman in the world is my aunt and she lives in Hoboken." Numerous persecutory ideas were uttered but these were largely inci- dental remarks, often clearly fabrications or exaggerations of some occurrence in her previous life. She claimed for instance that her mother-in-law had tried to kill her, that she was chloroformed at night, that attempts had been made to poison her, etc. There were no systematized delusions. Orientation, memory retention and grasp of general information were excellent.
Physically the knee-jerks were active; pupils react to light; no tremors or speech defect.
After admission she became more restless, showed much distracti- bility and commented in a humorous manner. In her talk she ex- hibited flight of ideas and sound associations. Pulling out a book from under the mattress of her bed she said : "I know how to press my pants under the mattress — -I like ghost stories — blood and thunder — ■ Shakespeare — do you know I am an Indian — well that's something new for you — Indian blood in me — you know all the poor old Indians died — are you going to* raise a beard?" (Physician unshaved.) Upon hearing the physician mention the word "iodide" she said: "My eye died — could my eye die — have you been painting something green?" (sees green stain on physician's gown). Her mood was one of exaltation intermingled with considerable irritability; she exhibited a fondness for annoying the other patients; she collected trash and was very unclean in her habits.
This excitement lasted five months; she then returned to a per- fectly normal condition; the persecutory ideas which she had expressed had originated largely out of family friction. Upon her return home the patient was industrious, social and in every way normal.
The present and fourth attack developed rapidly about Christmas 1907. After a few days of unusual quietness, during which she talked a good deal about her dead mother the patient became rather suddenly talkative, happy, sang, whistled, claimed that they owned the house in which they were boarding, called her husband a doctor, a professor in Bellevue, asserted that he was destined to be President. She talked constantly day and night, used much bad language, tried to fight her husband and made such a disturbance that her commitment was again necessary.
On reaching the Hospital the patient was in a state of mild elation, showed typical flight of ideas and much distractibility; she was perfectly clear as to her whereabouts; her memory and general grasp unimpaired. The following samples will show the disorder in the stream of thought and the distractibility. When the physician pulled out his watch she said: "They put those in hock — they have those in the hock shop — - didn't you ever run a hock shop and get a pint of beer — now you know damn well you did in Staten Island . . . you think you know a whole lot don't you — he is a regular reporter — (money dropped on the table) —the dollars, eagles and bank notes — we burn up all of our money- Maggie will make twenty-five dollars — pounds — crowns — he don't know nothing yet — he is an old Turk." In her talk she mixed in numerous fabrications — claimed that her mother and father were on
MELANCHOLIA AND MANIA.
729
the stage, that she herself had been with a circus; asserted that she had beaten her husband— " I smashed him twice in the face and kicked him down stairs." No hallucinations were present. Her physical condition was good.
She was very destructive, tore her bedding, broke the plants on the ward, collected trash, threw articles out the windows. The excitement continued for three months when convalescence set in. She quickly gained insight, denied that she had any hallucinations dur- ing the attack. At the end of four months she went home recovered.
(G. H. K.)
Manic-depressive Psychosis.- — Three attacks of depression, no excitements. Attacks characterized by sadness, loss of initiative in speech and action, feeling of motor insufficiency, mental dulness, dearth of thought, self reproach and ideas of sin. A few hallucinations.
A. McG., admitted to the Hospital November 12, 1907; a widow, set. 30.
Family History. — Both parents intemperate. One brother has had recurrent attacks of insanity.
Personal History. — The patient was normal in childhood; married at the age of seventeen and was treated badly by her husband.
In 1896 at the age of eighteen following child-birth, she was de- pressed for about three months. She rarely spoke, sat all day in a dull inactive state, spoke regretfully of her marriage. She made a good recovery.
In 1897 a second child was born and this precipitated another de- pression marked by great reduction in her activity and a feeling of men- tal dulness. She recovered in six months and subsequently bore two children without any further attacks, remaining well for ten years.
The present or third attack came on rapidly in April, 1907; there was no assignable cause. The patient says that while at work she was quite suddenly overcome by a sad, lonesome feeling; a few days later her head felt very badly, "^he mind seemed to be blank." She lost all energy and ambition, sat idly in the house, spoke little, felt that she was sinful, thought other people knew she had done wrong and a few times she heard remarks from strangers such as: "There she goes, she is a sinner." This feeling of wrong-doing referred to sexual irregularities two years previously soon after her husband's death.
In the Hospital the patient exhibited a quiet depression ; she had no inclination to talk spontaneously or desire to move voluntarily and while not actually retarded in speech or slow in action, this lack of initia- tive was accompanied by a pronounced feeling of mental and motor insufficiency. She complained of numbness in the head; explained her silence by saying that she did not know what to talk about, she could not think of anything so say, felt that she had no life in her body; there seemed to be a kind of " dead feeling " over her; she felt that she no longer had any affection for those whom she should love; she believed that she was an unworthy and wicked person and worried over immoral acts in the past. She claimed that she had been guilty of stealing, had neglected her church duties and thought God would punish her for her wickedness.
DISEASES OF THE MIND.
She frequently had visions of a serpent in the sky and sometimes saw shooting stars — these she took to be warnings that she should lead a better life in the future.
In orientation and grasp the patient was clear, her memory good, although she complained of being forgetful. She was slow in doing simple calculations and made errors. Power of retention diminished.
Physically there was nothing of importance except dulling of the pain sense over the entire body.
In six weeks the patient began to improve; her interest and am- bition returned; she became bright and cheerful, all of the feelings of inadequacy disappeared and she gained good insight into the attack. At the end of four months she went home recovered. (G. H. K.)
Manic-depressive Psychosis. — Two depressions and two excite- ments. In the depressions dull, quiet, feeling of stupidity, slowness in thinking, motor retardation and tendency to mutism.
In the excitements hyperactivity, exaltation, productivity in speech, flight of ideas, distractibility, rhyming and word association.
A. McH., admitted to the Hospital October, 1906, in her fourth attack. A single woman, get. 28.
Family History. — Negative.
Personal History. — The patient developed normally but has al- ways been of a retiring disposition and had no occupation except help- ing her mother with the housework. Habits good.
In 1895, at the age of seventeen, eight days after an operation for floating kidney she began to talk in a rambling way, was restless and tore up the bed covering. When brought to the hospital she was talkative and elated ; for two months she was excited, voluble in speech, boisterous and disorderly in conduct. She went home recovered at the end of six months.
In 1902, at the age of twenty-four, following the death of her father she had an emotional outburst and then for four months was de- pressed, quiet, talked very little, was dull and sluggish.
Since 1903 she has had attacks of pain in the right side. In Jan- uary, 1906, during one of these attacks of pain she became despondent, dull and inactive. Although the pain subsided she did not brighten up but sat quietly gazing and refused to speak. A few days later she was brought to the Hospital. She was very slow in her movements, gave no answers to questions, showed a tendency to hold her limbs in whatever position they were placed. When visited by her mother she answered some questions in whispers and explained that she wished to talk but could not "bring it out."
Physical examination negative, no signs of appendicitis.
At the end of six weeks she began to improve, but she was slow in thinking and described her feelings in the following words: "I wish I was not like this, so dumb, so stupid, I used to be lively." She cleared up rapidly and was bright and cheerful when she left the hos- pital at the end of six months.
At home she remained well for only six weeks, then she began to get restless, could not be kept in the house, was quarrelsome and scold- ing. Within a month she became highly excited, yelled and screamed,
MELANCHOLIA AND MANIA.
731
required to be held on the bed, would not allow a physician to examine her and her recommitment to the Hospital was necessary.
On admission she was exalted and boisterous, kept up a continuous stream of loud talk which showed flight of ideas, rhyming and word association. The following samples will illustrate this and also show the distractibility and tendency to make comments.
"I always like dogs — I always kicked cats because my father was a democrat ... is that justice— that ain't right — fight for the right ■ — that's the statute of liberty — three cheers for the red, white and blue."
To the physician she said: "Your glasses are gold — your head is black — your mouth is pure ... a fat cat — a democrat — republican — God damn Doctor H's flat . . . you have a blue shirt on — your veins are blue — your heart is red," etc. The following is a sample of the many rhymes that occurred in the stream of talk:
" We are leaders of the black hand We want you to understand That we command."
There was no delusion formation, no hallucinations. She was clear as to place and time and apparently had a good grasp on the re- mote past although her flippancy and refusal to give serious answers precluded accurate tests. When asked to do calculations she gave mostly random replies and her grasp on school knowledge and general information could not be tested because of her irrelevant replies, e.g., when asked to name five large cities she said: "White and blue — you are not true." The patient was in excellent physical health.
She continued hilarious and restless and at times showed much pressure of activity. She would turn and twist in bed, make a great variety of gestures, open the mouth and protrude the tongue, roll the eye-balls upward, make gyrations with the arms, strike the sides of the bed, raise the arms, spread the fingers, put the thumb to the nose, slap the forehead, etc., all done in a playful and free manner; no signs of stereotypy or constraint. With this flight of activity there was at times relatively little speech productivity. As a rule her language was very profane; she was filthy in her habits, assaulted the nurses and tore up everything she could get her hands on.
The excitement lasted for fifteen months, then the patient calmed down and rapidly recovered. (G. H. K.)
CHAPTER IX.
PARANOIA.
(Monomania, Delusional Insanity, Foue Raisonnante, Deeires SystematisES, Wahnsinn, Die Verrucktheit.)
Paranoia is a chronic psychosis, characterized by the develop- ment gradually and soon after maturity of systematized delusions, without other serious disturbances of the mind and without much tendency to dementia. It forms about six per cent, of the insanities, being seen rather oftener relatively in a non-asylum practice (six per cent, of 2197 Bellevue cases were paranoiacs).
Etiology. — It occurs oftener in females, the proportion being about ten to seven in the Bellevue Hospital psychopathic wards. In my personal experience it occurs oftener in males. It develops generally after the age of twenty-five and rarely after forty. It is an insanity based essentially on a congenital or hereditary taint, but there is often no direct inheritance of insanity. It is rarely brought out by a special exciting cause. The patient on maturing and trying to cope with the world finds himself unequal to it, and delusions begin gradually to evolve.
Symptoms. — The disease generally begins very slowly, with a long period of hypochondria or depression. Then comes a stage of delu- sions, usually persecutory, and finally a stage of grandiose delusions and mental deterioration. There may be a remission at any period in its course, followed, as a rule, by a relapse.
The patient has usually been a rather eccentric child, morbidly conscious and specially egotistical. As he develops he learns to believe that he has great talent in some direction, and is very confident that at bottom he is better than most people. Withal he is very selfish, and expects much but gives little, easily becomes offended, and develops surly dislikes of people, even of his best friends. He thinks he can be a great artist or writer, or can accomplish wonders in some business, but he fails through incapacity and lack of tact or steadfastness of purpose. He gives up and perhaps wanders off to some foreign field; or he may now develop a distinct delusion that a certain person has harmed him, and he concerts measures for revenge, writes insulting letters, and may even commit a homicidal act (paranoia persecutoria).
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PARANOIA.
733
Some patients continually find themselves, as they believe, cheated and misused in their business, and are constantly in litigation (para- noia persecutoria). Some get an idea that a wealthy woman is in love with them, or if women, have a similar delusion regarding an actor, a public man, or their physician.
With some the systematized idea takes a religious turn, and the patient thinks he has some divine mission or has received some in- spiration directly from God; or the idea may take a devotional turn and the patient become an ascetic. It is not, however, to be assumed that all the promoters of new religions and novel social ideas are paranoiacs. Many of these are simply the natural developments of ignorance and a somewhat emotional and unbalanced temperament. The characteristic of the paranoiac is that his work is ineffective, his influence brief and trivial, his ideas really too absurd and impracticable for even ignorant men long to receive. I do not class successful prophets and organizers like Joseph Smith or great apostles of social reform like Rousseau as paranoiacs. Insane minds are not creative, but are essentially weak, and lack persistence in purpose or power of execu- tion. The paranoiac often develops hallucinations; he is at first rather depressed, but he is not subject to real melancholia, although a person may have two or even three psychoses, and so the paranoiac may develop as an accident a melancholia, a toxic insanity, or a paresis.
After he has developed his delusions, he is apt to become excited and foolish, and even dangerous in his actions. He is generally then sent to an institution. Here his troubles gradually quiet down, and he may become almost or entirely rational. This period of remission may last some years, especially if he is made to lead a sequestered and regular life. If he is thrown upon the world again his insanity is lighted up and his former delusions return.
He may live in this way with exacerbations and remissions until old age, but usually his delusions become chronic and fixed and he is re- manded to an institution for life. Some patients become finally quiet, harmless monomaniacs, living a narrow routine life, in the care of an attendant or at home.
Forms. — There are described the dementing form or dementia paranoides, original paranoia, acute hallucinatory paranoia, and secondary paranoia.
Dementia paranoides is classed by Kraepelin as one of the forms of dementia praecox. It acts to all intents and purposes like a para- noia, beginning earlier in life and running a shorter course and ending in dementia.
734
DISEASES OF THE MIND.
Original paranoia is a degenerative psychosis which occurs in young children.
Acute hallucinatory paranoia is a paranoia with episodes of a rather acute character, characterized by hallucinations as well as by sys- tematized delusions. It is an episode in the paranoiac constitution and development.
Secondary paranoia may develop on the basis of any acquired degenerative mental condition; thus it is seen in chronic alcoholism and morphinism, in paresis and in senility.
Folie-d-deux or imposed insanity is a term given to a condition in which a mental disturbance affects two or more persons who have been intimately associated together. The psychosis in the second indi- vidual tends to disappear when separation occurs. The form the psychosis takes is generally that of a paranoia.
Prognosis. — The prognosis is almost uniformly bad, in the sense that the disease is not entirely cured. In mild cases, however, remis- sions occur in which the patient becomes very nearly normal, and under proper environment continues so for years. In the severer cases a chronic delusional condition is established, and the patient continues in it, with periods of some improvement, and then again relapses. In the most typical and clear-cut cases of paranoia there seems to be but little tendency to dementia, but experience shows that in the great proportion of what are called paranoiacs this tendency is to some degree present.
Pathological Anatomy. — As paranoia is not so much a disease as teratological defect, we find in the brain of these cases only secondary changes. There have been described, however, certain anomalies in the conformation of the brain, and it is probable that behind all the symptoms are certain defects in the normal arrangement of the pro- jection systems and association paths.
Treatment. — The treatment of paranoia is altogether one of en- vironment. If the patient can be placed in such surroundings that very little excitement or strain is put upon him, if he is made to live a simple, regular outdoor and indoor life, with such amusements and interests as do not at all tax the brain or nervous system, he almost invariably gets better, at least for a time. Institutional treatment is, therefore, the simplest and usually most effective way of dealing with paranoia.
Paranoia. — Suspicions and ideas of reference followed by system- atized delusions of persecution. Hallucinations doubtful. No expan- sive ideas or retrospective interpretation. Conduct natural. No signs of deterioration after ten years duration.
PARANOIA.
735
A. H., a widow fifty-six years old; admitted to the Hospital October 31, 1904.
Family History. — Patient's father had delusions of persecution and died in an insane hospital. A brother peculiar from youth has also ideas of persecution. A paternal uncle suffered from senile dementia.
Personal History. — The patient, a native of Germany, was a healthy child, bright mentally, received a good education. In youth she was sensitive and seclusive and was regarded by her family as rather eccen- tric and lacking in balance. At the age of twenty-three she married a man socially her inferior with whom she lived unhappily. When forty years old she came with her husband to America and settled in Philadelphia. Habits strictly temperate. Menopause at the age of fifty-one.
The Psychosis. — The patient claims that the conspiracy of which she is the victim started in 1897 when she became acquainted with a Mr. X. She met this man in a store where they both traded and they formed a casual acquaintance. She soon realized that people were taking notice of these chance meetings and of her conversations with Mr. X., they imputed a bad intention to her and seemed to think that she was attempting to attract Mr. X. by a show of interest. She heard no remarks but based her conclusions upon "impressions" drawn from the behavior of the clerks and people in the store. She noticed they would smile, change their facial expression, give significant glances and refer to her by various "hints." The patient then ceased visiting the store and moved to another part of the city, but she was still annoyed by "hints," and she saw notices in the newspapers containing ac- cusations that she had attempted to have improper relations with a married man. Her husband lost his position through her enemies; she was followed by detectives and an attempt was made to poison her child. "Everything was done to bring me to despair and suicide, to expel me from the country." At the end of a year the patient, in order to escape further persecution left America and returned to her home in Germany. The "intrigue," however, followed her across the ocean and she continued to see notices referring to her in the newspapers. While she was in Germany her husband died (the patient claims that he was poisoned). Finally she saw notices in the newspapers sug- gesting her return to America and hinting at the possibility of marriage with this Mr. X. whose wife, she was informed, had also died.
November, 1902, she returned to New York where she soon found out that Mr. X. had no intention of marrying her and that his wife was not really dead. From this time on he assumed the leading part in persecuting her and the cause of the persecution was due to the fact that she knew certain secrets concerning Mr. X. and because of this knowl- edge a plot was developed to kill her or to drive her to despair and suicide. This criminal plan included the ruin of her son and the dis- grace of her relations. Mr. X. came to New York under an assumed name, he followed her about, took rooms in front of her residence and watched closely all of her movements. By tipping the post office officials he read her letters and by means of a phonograph in the cellar,
736
DISEASES OF THE MIND.
managed by an accomplice, he received reports of everything spoken in the house. She tried to get work but her persecutors always man- aged to interfere and prevent her from obtaining a position. On two occasions they attempted to poison her.
The patient became greatly depressed and finally she jumped into the river; the water was shallow, however, and she was rescued and brought to the Hospital. Patient claims that she was being watched at the time she jumped into the river and that her persecutor's agents pulled her out of the water with the desire to bring her to prison and later to death.
On admission to the Hospital, patient was depressed and tear- ful, did not want to give any information about herself, believing that it would come into the hands of her enemies. She claimed that she had attempted suicide in order to escape a worse fate; her persecutor seemed to be able to influence everyone against her; she had seen him several times in different disguises and by having many people in league with him he is informed of all that she does and says. She gave as a reason for all the persecution the fact that this man once caused her to believe that he was in love with her. "I confided in him and he converted the matter into a great crime." Her persecutor fears that she will reveal some secret that she is in possession of. She refuses to tell what this secret is.
The patient was perfectly clear in orientation; her memory was ex- cellent; she was found to be well informed on general topics and gave evidence of good education. She denied emphatically that she was mentally affected.
Physically there was nothing of importance.
For some weeks the patient remained much depressed; she quickly became suspicious of the Hospital authorities and asserted that they were in league with her persecutor. She declined to answer any more questions because it was all a "humbug."
After three months in the Hospital the patient's anxiety had disap- peared and she showed a disposition to make herself useful by assisting the nurses. To the physician she said: "I don't blame you, you are misled by the intrigue — you all work out my ruin by helping my perse- cutor." She said that her persecutor had traced up her whole life and brought up some foolish acts of her youth. When asked how she had obtained her information regarding the doings and plans of her persecu- tor she replied that she had the faculty of knowing things that were never told to her. "It is like a revelation." She would not admit that she had heard any "voices." It was an ability to foretell. "Forme everybody has a looking-glass on his forehead." When asked if this in- formation came to her in the form of thoughts she replied: "It is a conviction in a moment."
After this she was again more reserved and refused to answer questions. "These matters are not to be spoken about any more — I know you are all together in connection with my persecutor." When asked if she had any proof of this she replied: "No visible, but I am quite sure he has misled you altogether — I would not have believed such a thing possible with educated and scientific persons you may
PARANOIA.
737
turn it out as insanity but you make yourselves instruments in the hands of my persecutor."
Since December, 1905, the patient has absolutely refused to discuss her case with the physicians. She keeps aloof from the other patients, maintains a dignified reserve. She has always shown a willingness to occupy herself and for two years she has had charge of the ward clothes room. She feels that she is earning her board by her work. In her conduct one notices nothing peculiar. She remains clear in orienta- tion; her memory is excellent. She talks with perfect saneness on general topics.
July, 1908: Letters to her relatives show that the system of delu- sions remains unchanged. (G. H. K.)
47
CHAPTER X.
CONFUSIONAL INSANITY.
(Exhaustion Psychoses, Infection Psychoses, Toxic Psychoses.)
Confusional insanity is a general term which answers for a type of insanity that is usually divided into three groups.
These groups consist of the exhaustion psychoses, of the infection psychoses, and of the toxic and autotoxic psychoses. While such sub- division is often useful because of its etiological help, and because sometimes rather distinct clinical features develop in accordance with the special causes, yet there are certain general fundamental characters belonging to them all. These are, that in all cases the course of the malady is relatively short; that it begins usually with some delirium or very marked confusion, and that a mental confusion at some time always dominates the scene; that there are associated with it a great degree of hallucinatory disturbance and a great variety of unsystema- tized delusions; and that usually late in the course there is some stupor or mental enfeeblement. In accordance with the constitu- tion of the individual or the character of the case the delirium varies in character, being at times hardly present, at others so acute as to prove fatal. The character of the hallucinations and of the delusions and certain peculiarities in memory disturbance and orientation occur in some groups more than in others. Underlying them all, however, as already stated, there are certain fundamental qualities which are common.
The terms applied to the different groups of confusional insanity are indicated in the following grouping, showing the types and special subtypes.
1. The Exhaustion Psychoses. — (a) Collapse delirium, acute de- lirium, (b) Acute dementia, primary dementia, amentia, acquired dementia.
2. The Infection Psychoses. — (a) Febrile delirium, (b) Multiple neuritic psychosis or Korsakoff's psychosis.
3. Toxic Psychoses. — (a) Alcoholism, including delirium tremens, alcoholic insanity, and alcoholic dementia, (b) Morphinism, cocain- ism. (c) The autotoxic psychoses, including thyroid insanity and myxcedematous insanity.
■738
CONFUSIONAL INSANITY.
739
Etiology. — The disease is essentially one of the active period of life, between twenty-five and forty-five. It occurs rather oftener in men. Hereditary influence is less marked than in most other psychoses, ex- cept in those cases due to alcohol and the drug habits. Here a con- fusional psychosis develops on a phrenasthenic constitution. The exciting causes have been already indicated.
Symptoms. — The patient suffers at first from evidences of some infection or poisoning. It may be a pneumonia, an attack of grip, a typhoid fever, a puerperal sepsis, or most often a long period of alco- holic indulgence ending with some symptoms of neuritis. On recover- ing from a shorter or longer febrile state, the patient begins to show signs of irrationality and mild delirum. This may end in an attack of acute delirium. When this subsides, the mind, instead of clearing, be- comes clouded and confused. There now appear hallucinations of sight or hearing, with various unstable delusions, especially as regards the place he is in or the people about him. He is perhaps depressed or apprehensive that he has been placed in a hospital or prison. He does not always recognize those about him. He talks incoherently at times, describing objects on the wall or people in the room, or appears to hear voices of people outside. At times he may be so alarmed and excited that he tries to get out of bed and has to be restrained. His lack of orientation and defects of memory are great, and he wTill insist he was out walking the previous day, or that he has received visitors whom he has never seen. He often responds well to direct and simple inquiries, and at times seems fairly lucid, but soon lapses into incoher- ence. If quite ill physically, the confusion and irritability are shown in a low delirium, which is usually worse at night. He sleeps badly, and is at night especially apt to be disturbed by his hallucinations and delusions. The confusion may be so great that he fails to respond sen- sibly to any inquiries, and he may become stuporous and even some- what katatonic — being then in a condition called "amentia, " a name given by some to the exhaustion psychosis.
There are then present disorientation of person, time, and place, hallucinations and various delusions, some mental excitement at times, defect in memory, mental cloudiness, retardation of thought, and stupor.
After a few weeks, or perhaps even a few days, the mind begins to clear, the delusions and hallucinations disappear, there is lucidity of thought and response. But for some time the mind is weak, thought is slow and difficult, memory defective, and there may be some apathy and depression. In some cases, particularly in con- nection with multiple neuritis, there remain a very distinct and chronic
740
DISEASES OF THE MIND.
defect of memory and a loss of orientation. This is shown particularly with reference to recent events and in the power of recording things seen or heard. The patient is shown the title of a book or a picture. It is taken away and at once he forgets that he has seen it. He does not remember whom he has just seen, or what he has just done, or where he has just been. This condition may, and usually does, in its typical form (Korsakoff's psychosis) remain permanent, and renders the patient practically unfitted to do his work or be at large without an attendant.
In the ordinary cases, however, the patient gradually recovers in a few weeks or months.
Choreic Insanity. — A form of insanity is associated sometimes with chorea. It is usually, however, really only an infectious psy- chosis, and is characterized by some delirium or maniacal excitement, followed by confusion and exhaustion. Sometimes the psychomotor excitement is very slight, and the patients develop only a kind of con- fused condition in which they do not know where they are, and have various irrelevant delusions with occasional hallucinations.
Puerperal insanity is a psychosis which occurs shortly after childbirth. It takes the form of either manic-depressive insanity or an exhaustion or toxic psychosis. Occasionally pregnancy and puer- perium are the exciting causes of dementia praecox. In any case the malady has no clinical characters which stamp it as a special puerperal disease. It used to be more often a toxic or infectious psychosis, but puerperal insanity of this type has largely disappeared under the in- fluence of modern obstetric methods.
The Alcoholic Psychoses. — Among the toxic insanities that as- sociated with the excessive use of alcohol is the most common. The use of alcohol leads to a variety of morbid mental conditions, which may be grouped under the following heads:
i st. Simple inebriety, or morbid alcoholic habit, which is charac- terized by a persistent indulgence in alcohol and a besottedness of mind and morals which gradually follows.
2d. Dipsomania, which is a periodical psychosis, belonging to the phrenasthenic insanities, in which the patient suddenly, at various periods, develops an intense desire to drink. After a prolonged de- bauch of a few days or weeks he returns to his usual vocations, and does not have a recurrence for some months. The inebriety habit or besottedness may be complicated with dipsomania.
3d. There is an insanity of several weeks' duration which is a form of confusional insanity, running about the ordinary course of this psychosis. When associated with multiple neuritis it has a some-
CONFUSIONAL INSANITY.
741
what special physiognomy, and has been given the name of the multiple neuritic psychosis or the Korsakoff' s psychosis.
4th. There is an acute delirious condition, known as delirium tremens, which is simply a form of acute poisoning due to prolonged use of alcohol, insufficient food, and autotoxaemia.
5th. There is a terminal condition which comes upon the alcoholic after many years of indulgence, which is known as alcoholic dementia or alcoholic pseudo pare sis.
Drug Insanities. — Confusional states may be caused by the use of drugs, in particular opium, cocaine, chloral, and in Eastern coun- tries of hashish. The victims of drug habits, however, belong, as a rule, to the more degenerate types of phrenasthenia, and a true confusional psychosis is episodical.
The Exhaustion Psychoses. — Confusional insanity is sometimes initiated by a form of acute or "collapse" delirium. After an attack of some acute disease, like rheumatism, erysipelas, influenza, puer- peral fever, or after operations and severe hemorrhages, the patient in two or three days goes into a condition of profound mental disturbance, with maniacal excitement, flight of ideas, and active hallucinations of all kinds. The sense of orientation is lost, the patients do not know who or where they are or recognize their friends. They often become extremely violent, tear their clothes, and try to get out of bed, refuse food, do not sleep, and neglect their person. With this sometimes develops a febrile condition, and the general state is one which is known as "acute delirium" or even of "grave delirium. " The condi- tion resembles a good deal that of delirium tremens, and often a diag- nosis can be made only by knowledge of the cause and previous history.
Amentia. — A form of confusional insanity occurs after profound exhausting mental strain, such as results from shock or long anxious vigils with the sick. It is seen not infrequently in prisoners who have suddenly presented to them the possibility of a trial and an execution. Here the patients develop at first some hallucinations and delusions regarding their surroundings and the people about them. They be- come dull and confused, answer questions rather at random, lose their sense of orientation, are careless of their person, and will often sit for a long time motionless in their rooms, or even lie in an apathetic state in their beds. Sometimes a condition of katatonia develops or a sort of stupor. This condition is more chronic than the former and lasts often for several months. It has been called amentia, primary dementia, and curable dementia.
Prognosis. — The course of many confusional psychoses is relatively short; they last from six to eight weeks. In cases of very acute onset
742
DISEASES OF THE MIND.
and acute delirium the course is rather shorter. In the alcoholic in- sanity, coming on gradually or following delirium tremens, the dis- ease may last three or four months. In the multiple neuritic insanity, which is generally a form of alcoholic insanity plus neuritic inflam- mation, the course is about the same. The prognosis, in so far as the psychosis is concerned, is almost always favorable; if the patient sur- vives the somatic disorder the insanity gets well. In collapse delirium and in acute delirium (grave) the prognosis is very serious; in fact, the so-called "grave" delirium is almost uniformly fatal. In the multiple neuritic psychosis the outlook is usually serious on account of the in- flammatory complication and the tendency for pneumonia to develop. If the patient survives there is very apt to be left some permanent de- fect of memory. In the infection psychosis associated with chorea, chorea insaniens, the prognosis is also serious. There is apt, after a week or two, to develop a delirium, and with this a fever, leading to a fatal issue.
The treatment of the confusional psychoses is almost purely symp- tomatic, and belongs essentially to the realm of general medicine. The delirium has to be quieted by hyoscine or morphine, or some of the sleep-producing drugs, the bodily strength sustained by frequent feeding, and autotoxaemia prevented by a careful attention to the excretions. The use of small doses of castor oil, frequently repeated, often is helpful in this respect.
Polyneuritic Psychosis (Korsakoff Syndrome). — Disorienta- tion, grave retention disorder, fabrications , suggestibility and misidenti- fication of place and persons. Amnesia for some events preceding the psychosis; severe polyneuritis; alcoholic excesses not established, onset during puerperal period. Psychosis followed by permanent defect symp- toms, viz., impaired power of retention, diminution in initiative, sim- plicity and emotional lability.
C. G., admitted to the Hospital August 15, 1906; aet. 35, single.
Family History. — One brother drinks to excess.
Personal History. — The patient was normal in childhood, received . a common school education and then remained at home to keep house for her father and brother. It is known that she took beer regularly for several years. Accurate information, regarding the amount of alcoholic beverages consumed could not be obtained as the patient spent most of her time alone.
The Psychosis. — About two months before admission the patient complained of pains in the legs. She was at this time illegitimately pregnant, and June 30, 1906, she gave birth to a seven months old, still- born child. While in the Tying-in Hospital the mental symptoms developed. When her brother called there to see her she fabricated about visits from relations, some of whom were long since dead. When she returned home three weeks after confinement she was still
CONFUSIONAL INSANITY.
743
complaining of pains in the legs and had considerable difficulty in walk- ing. She then spoke of her father as if he were still living, she worried about getting his meals ready and invented stories of visits from friends and relations. There were no suspicions or ideas of persecu- tion; no evidence of ' hallucinations. Because of her increasing help- lessness she was taken to Bellevue.
At Bellevue the patient said: "I was across the street this morning to see my brother (untrue), then I came back again — I was down stairs this morning — I live right across the street" (untrue).
On August 15, 1906, the patient was brought to the State Hospital on a stretcher; she was unable to walk; her arms and legs were very tender and she cried out with pain when any effort was made to move her. She remained quietly in bed, was affable and readily com- municative when questioned. She then wept a good deal, was de- spondent over her physical condition, complained especially of pains in the limbs and of heaviness in the arms. She spoke gloomily of family affairs, mentioned the death of her mother and she felt worried because her father must work so hard in his old age (father dead two years). It was found that the patient mistook almost completely the situation and had no recollection of recent occurrences. She thought she was in Mrs. M's. house just across the street from her own home on 46th Street; she mistook the physician for Mrs. C's. son; she was disori- ented as to time; fabrications were readily elicited; she forgot from one moment to another what she had just said ; could not remember a name or a number for ten seconds and her replies were easily influenced by the way the questions were put. The following samples show the tendency to confabulation and bring out glaring contradictions.
What day it this? "Sunday" (Friday) — "I know I was at church this morning." (Patient helpless in bed.) When did you come here? "I came early before dinner." (Two days in hospital.) Wnerefrom? " My own house across the street" (untrue). Didn't you do something this morning? "I think I wanted to sweep the yard and your mother wouldn't let me." Are you married? "No sir." What is your husband's name? "I forget." How long have you been married? "About six weeks." (Not married.)
The patient's memory for remote events was less seriously involved and she could give many correct statements regarding her early life. Her grasp on topics of elementary education was poor; simple calcu- lation defective; there was no evidence of delusions or hallucinations. In mood she was depressed — whined a good deal, fretted over her father's situation and expressed regret that she was making so much trouble for the people who cared for her.
Physical Examination. — The patient is an obese woman, presenting the physical signs of a severe polyneuritis, viz., absence of tendon reflexes, muscular weakness, tenderness of the nerves and muscles, pains in the extremities; the muscular weakness is more highly de- veloped in the extensor muscles, the legs are more severely affected than the arms. A characteristic foot-drop is present and the paraly- sis renders locomotion quite impossible. Pain sensibility is dimin- ished over the skin of the extremities. The eyes show in lateral
744
DISEASES OF THE MIND.
positions a well-marked nystagmoid twitching; slight fever and high pulse (90 to no).
Following her admission, muscular atrophies developed in the legs and fore-arms and although passive motion and massage were given the joints became quite stiff.
Within a month the physical condition improved, but the mental state showed no change. She remained completely disoriented, rather depressed in mood, slept much of the time during the day.
During the second month, her appreciation of the surroundings was better; she was usually able to give the physician's name and knew that she was in a hospital, but thought it was just across the street from her own house; she still fabricated as to recent doings.
After seven months in the hospital the polyneuritis had subsided, leaving the patient with some stiffness in the hands and feet. Orien- tation satisfactory and memory for events of her early life good. Her power of retention remained, however, much impaired and there was nearly complete amnesia for the period of the psychosis and also some important events antedating the onset had been forgotten. She had, for instance, no recollection of her father's death two years previously, nor did she know anything of her pregnancy and child-birth.
July, 1908: The patient is a contented and cheerful occupant of one of the chronic wards. She is simple in manner, ultra-affable, unduly emotional. Retention remains extremely poor. She now knows that her father is dead, but she is mistaken as to the time of his death. Has no recollection of the Lying-in Hospital and birth of her baby. (G. H. K.)
Hallucinatory Delirium Following Infection.
S. M., admitted to the Hospital, January, 1908; house-wife; aet. 40.
Family History. — Negative.
Personal History. — The patient's early development was normal. Married at nineteen; four children. Temperate habits.
She was well until November 28, 1907, when symptoms of acute middle ear disease developed. She refused to allow operation and two weeks later while the ear was discharging she had an attack of facial erysipelas. The psychosis developed rapidly during the infection; the patient became excited and fearful, lost clearness as to her where- abouts, talked in a rambling manner, mostly on religious topics, spoke of leading the Jews to salvation, of saving the world, at the same time uttering many apprehensive ideas — believing that her head was to be cut off, etc.
At Bellevue she was excited, screamed and struggled, showed much fear. She shouted such remarks as the following: "Saint Joseph stop the devil — all the devils with him — protect me, they will kill me."
On admission to the State Hospital the patient was somewhat quieter, but still suspicious and apprehensive. Her attention was difficult to hold and one observed rather striking fluctuations in her clearness; at times she was fairly lucid particularly when fixed with questions, but she drifted very readily into an hallucinatory delirious state. After answering some questions quite sensibly she suddenly said : "Say, look at the building moving — Oh ! the building is going to
CONFUSIONAL INSANITY.
745
fall in, but it is not going to touch us — my father died a saint on Christmas day — my mother is out in the hall there." The following are further samples of her utterances:
"The Lord talked to me, listen! you must not reveal it to the out- side people because they may be Jews — He was here with me two hours — He told me, our Tord did, that I should hit the devil an awful blow between the two eyes — I see the glimmer of the light of Heaven on the wall — I see the Holy Mother with white hair — her hair is very white."
At times the patient seemed to realize she was in a hospital; again she talked of being in the Hotel Astor. Frequently she feigned death, especially when anyone entered the room suddenly. She thought she was to be killed, claimed the food was poisoned. At times she threat- ened and strongly resisted the nurses.
The patient had a fever of 102 ; pulse, 1 10; leukocyte count, 14,000; purulent discharge from the left ear.
When her husband visited her she showed much agitation, thought he came to kill her. She recognized the patient in the next bed as her daughter, mistook the physician for her family doctor. Within a few days the patient became calmer, the sensorium clearer, but she was still very suspicious, would at times become fearful, accuse various ones of trying to poison her and of concealing knives with which to kill her; claimed that she was called bad names and that she heard threats against her life. She prayed a great deal and at times talked of having special divine power. With the abatement of the fever the patient's mental condition improved although hallucinations and misidentifica- tions continued for about a month. On one occasion she thought fine pepper was falling from the ceiling into her eyes. At the end of three months the patient was clear in orientation, but she was still irritable in mood and exhibited occasional outbursts of anger and suspicion. She would then abuse both physicians and nurse, claim that she was unjustly detained in the Hospital. At the end of five months the patient had recovered. She had no clear recollection of events just prior to her admission to the Hospital or immediately afterward. The hallucinatory experiences appeared to her as dreams. In some of these she had seen St. Joseph and he had talked to her. She seemed to be travelling in England, France and Germany with the saint; the stars were shining and everything was bright. She had misinterpreted many actual occurrences and incorporated them in her delirious trend.
(G. H.K.)
Acute: Alcoholic Hallucinosis (Alcoholic Insanity). — Sudden onset during a spree; auditory hallucinations leading, accom- panied by sense deceptions in other fields. Fear and agitation in reaction to defamatory and threatening "voices." Orientation and grasp not disturbed. Quick recovery.
W. McG., admitted to the Hospital, February 22, 1908; set. 44; clerk.
Family History. — Father drinks moderately.
Personal History. — The patient's early life was normal. He was industrious and temperate until about the age of thirty-two when he began to drink. Since then he has not held his positions so well.
746 DISEASES OF THE MIND.
He drank steadily, and because of his intemperance he lost his last position shortly before the onset of the psychosis.
During Christmas week, 1907, he went on a spree; at that time he was ill with a cold. Early in January, 1908, there was an hallucinatory episode which lasted only a few days. He continued to drink and two weeks later he again imagined he heard people talking; they accused him of crimes and threatened to kill him. He was fearful and de- pressed, and finally became so greatly terrified that he was removed to Bellevue. There he said: "I imagine that I hear people talking in the room — that I hear voices like — they talk of everything — religion — they threaten me — accuse me of crimes — I hear them now." The patient was depressed and anxious.
When admitted to the State Hospital, he was more composed, still heard threatening voices which made him nervous and uneasy. He gave a good account of his hallucinatory experience, relating how about Christmas time, after excessive indulgence in alcohol, he began to hear talking outside of the house; voices of both men and women; some claimed to be devils and others said they were angels. He heard threats against his life, they said his soul was lost, accused him of vari- ous crimes, and at times he had a sensation as if some one was injecting poison into the top of his head and sticking needles into his body. His legs felt as if they were on fire, or as if he were receiving electric shocks, he felt ants crawling over his skin and smelled bad odors. At night he was especially anxious and then he saw forms of devils and angels. The sensorium remained clear; there was no delirium or tremor; the patient at all times realized where he was and gave correctly the data of personal identification. Physical condition was good, he com- plained merely of headaches and of a feeling of general nervousness.
The hallucinations continued for a few days after admission, but from the first he seemed to have a partial realization that ''the voices" were only imaginations; the talking seemed to come into his ears as if from a telephone, whatever he said was repeated and if he read any- thing it was repeated after him. "They knew what was going on in my mind."
At the end of six weeks the hallucinations had disappeared, his sleep had improved and he had full insight into his morbid experiences, attributing his trouble to excessive indulgence in alcohol. (G. H.K.)
Aecohouc Delusion of Jealousy.
W. F., admitted to the Hospital April 22, 1908; set. 43; drug clerk.
Family History. — Negative.
Personal History. — The patient was born in Germany, was normal in youth, came to the United States at the age of nineteen; married at the age of twenty-four, has two children. No history of syphilis.
Ten years ago the patient began to drink immoderately, taking as much as a pint of whiskey a day. After drinking for five years he had an attack of delirium tremens from which he made a good recovery. When he began to drink again it was noticed that he was more sensi- tive to alcohol than formerly; he drank, however, half of a pint of whiskey daily and a considerable amount of beer. For several years
CONFUSIONAL INSANITY.
747
he has worked irregularly, was inefficient, his memory less reliable and finally he had difficulty in obtaining employment.
The Psychosis. — About a year before admission the family cir- cumstances were so poor that patient's wife had to go out to work as a laundress. The patient unreasonably opposed this; he became sus- picious of his wife, ideas of jealousy arose which soon led to open ac- cusations of infidelity. When his wife left the house to go to her work he would watch and follow her, and although he never obtained any evidence to support his suspicions they gradually grew stronger. He kept a diary of all that transpired, noting down carefully what his wife did and said. He evidently misinterpreted many remarks both from his wife and his associates in the saloons. It is possible that his com- panions knowing his jealous disposition teased him, but it is probable that there were occasionally auditory hallucinations. In the saloon they would say to him: "Your wife ain't going out washing." On another occasion he claimed that a stranger in the saloon said to him : "If you go to i nth Street, you can see your wife on a lounge with another man."
He felt that he was losing his sexual power, thought his wife sneered at him and made such remarks as — "Go away with your little thing, I can get something better." At times when quarrelling with his wife, he thought he heard some of the neighbors calling out at him: "You are crazy." He thought his wife wore better clothes than their means afforded and he believed that some one else was supplying her with money. He became suspicious of the food, imagined that his wife desired to poison him in order to get him out of the way; he even claimed that his children were not his own. He suspected his wife of having immoral relations with his brother, and also thought the brother had instructed men in the saloons to say things which would annoy him.
On admission to the Hospital the patient was poorly nourished, showed a strong, fine tremor of the tongue, hands and facial muscles, and moderate arteriosclerosis.
He was quiet and somewhat depressed by his commitment to the Hospital, talked freely, told of his suspicions and jealous ideas, at the same time remarking that his wife was a good woman who worked hard and he did not really believe that she had been unfaithful to him. He was clear in orientation and mental grasp, his memory for both recent and remote events was good.
Improvement began soon after admission and the patient quickly gave up his jealous ideas, acknowledged that he had accused his wife unjustly and said that her treatment of him and the remarks she had made were justified by his behavior. He still maintained that people had remarked about his family affairs in the saloons and he thought his brother had instigated this talk because he had previously had a quarrel with him over the division of some property. He was some- what unstable emotionally, wept easily when talking of the past. He emphasized his affection for his wife, claimed that she was the best woman a man could have and he only wanted another chance to show his appreciation. At the end of three months he was discharged from the Hospital. (G. H. K.)
748
DISEASES OF THE MIND.
Acute Confusion Following Infection. — Dazed state with com- plete disorientation, fear and perplexity; wandering of attention and marked difficulty in thought elaboration. Speech low with fragmentary irrelevant utterances. Hallucinations not prominent. Exhausted phys- ical state; slow recovery.
E. S., admitted to the Hospital April 18, 1904; house-wife; set. 25. negress.
Family History. — Negative.
Personal History. — Patient was a bright healthy child. At the age of seventeen she had typhoid fever with delirium; there were no sequellae and two years later the patient married. She has borne three children, the youngest twenty months old, and the patient was still nursing this child when the psychosis developed.
The Psychosis. — Six weeks before admission the patient had an at- \ tack of sore throat and bronchitis which her physician diagnosed as "grippe." While convalescing from this illness rheumatism developed with tenderness and swelling of the joints. She became despondent over her condition, complained that her head "felt funny;" she grew noticeably absent-minded and in conversation she would forget what she was talking about and repeat what she had just said. A week be- fore admission she accused herself of religious omissions, insisted that she was going to die, became restless, walked the floor, moaned and passed rapidly into a state of confusion.
At Bellevue she seemed terrified, talked in whispers; her utterances were broken and expressed her apprehensive state of mind: "Oh — Oh —Oh I am going to die — I wish I were dead— Oh! what is it!"
On admission to the State Hospital the patient was deeply con- fused; she seemed fearful, constantly tried to leave her bed, showed great distress and anxiety. "Lord have mercy on me — let me die — wouldn't I rather die than be here — I'm afraid they are going to hurt my father— Jesus have mercy on me." She was completely disori- ented; her attention was difficult to fix; the simplest questions seemed to puzzle her and her replies were mostly disjointed, confused utter- ances. The prevailing mood was one of fear and perplexity. She clung to the nurse as if for protection, feared that she would be killed.
Physical Examination. — Temperature 99.5; pulse quick and rapid, running up to 120. Aloud mitral systolic murmur. Conjunctivae injected. Mouth and tongue dry. Exhausted appearance. Haemo- globin 70 per cent.
For six weeks the patient's sensorium was extremely hazy and there was a very striking difficulty in thought elaboration. She seemed unable to express herself, she hesitated and stumbled, seemed to grope aimlessly for words, picked up remarks which she chanced to hear, her utterances being of a most fragmentary and irrelevant nature. A few samples of her replies to questions will show the incoherence. What place is this? "This is — first one to hop — hop — up for blister- ing something." What day is this? "This is the — a — good morning — strike out the light — march out — life dying boy — now." What did I ask you? "Thank the Lord — thank — thank the Lord— thank — much obliged."
CONFUSIONAL INSANITY.
749
She was unable to do the simplest calculations and in attempting to write she put down unintelligible symbols among which a few letters could be recognized. She seemed utterly incapable of making any mental application; her attention lapsed even under continuous stimulation. Her whole demeanor indicated that she was deeply per- plexed and confused. Her speech was low, with long pauses between words. A further sample of her disjointed talk is shown in the follow- ing reply to a simple question. What is the year? "A — please — ■ stream — light — killing — pleased outlet a stream of light come in — kick him out like I did."
The data of personal identification was markedly disturbed. She showed great confusion over such things as her age, birth-place, mar- riage, number of children, etc. The following sample exhibits her perplexity.
"Where have I been? Listen to me! Am I dead? Was I dead? What is the matter with me? Did they have salt on me? So I have been dead really? Where did I come from?"
She thought the other patients talked about her, heard her relatives outside of her room, complained of hearing dogs barking, etc. She sat up in bed looking about in a dazed, uneasy fashion. At times she failed to answer any questions, merely moving the lips without pro- ducing any sound. She appeared exhausted, her movements were slow and languid ; no muscular tension. For some days her heart acted badly; pulse 140 and weak.
Improvement occurred slowly after the second month. She be- came oriented, but was still unable to grasp the situation and would ask such questions as : "Do I have to die to get out." "It don't seem right yet, I don't seem to understand." She was still unable to do simple calculation and was slow in answering questions. In explana- tion she said: "I can't think, I can't get my mind together to think what you are talking about."
At the end of seven months recovery was complete. For the early part of the psychosis the patient had no recollection. She recalled that at home she had been depressed over her physical health and that her head had felt "queerly." She had no recollection of coming to the Hospital and was amnesic for the first few weeks after admission. She did not recall any hallucinations, except when clearing up she had im- agined she heard cursings and thought the others talked about her.
(G. H. K.)
CHAPTER XI.
ORGANIC PSYCHOSES.
The major psychoses which are distinctly dependent on organic changes in the brain are: Paralytic dementia. Senile dementia. Alcoholic dementia. Epileptic dementia.
Psychoses connected with gross lesions, such as brain tumors, hemorrhage, softening, brain injuries (traumatic insanity), syphilitic exudates, abscess, multiple sclerosis.
Idiocy.
Imbecility.
GENERAL PARESIS. (General Paralysis of the Insane — Dementia Paralytica.)
General paresis is a progressive disease of the brain running a course of about three years, characterized by abnormal mental symp- toms, ending in dementia, associated with physical weakness and certain characteristic physical symptoms.
Etiology. — Paresis, as it is usually termed, is a disease of modern civilization, and, as Krafft-Ebing states it, of syphilization. It was a medical curiosity a hundred years ago; now, it is extremely frequent in our asylums, in neurological clinics, and in private practice. It has become much more common of late years in this country. It is found in nearly all the civilized races of Europe and America, but is rare in Africa and Asia. It affects even the inferior races living among civil- ized people, and is found, for example, among the negroes of the United States.
It is not a disease which is directly inherited, but the neuropathic constitution predisposes to it, and occasionally cases are seen in early life which may be said to be of congenital origin and are due to syphilis or degeneration in the parent. It occurs much more often in men than in women, the proportion being about 5 to 1. The proportion of women is slowly becoming greater. In private practice among the better class the number of women wTho have the disease is very small.
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ORGANIC PSYCHOSES.
751
In my own case the number of paretic women is about six in one hundred cases.*
The excessive use of alcohol is a predisposing cause, as is also excess- ive mental exertion, particularly if combined with emotional strain and excitement. Sexual excesses and abuse are also predisposing causes, but the common view, that the disease is the result of perverted sexual indulgence, is not correct. Syphilis is, no doubt, the most es- sential of all the predisposing causes, and paresis must be put down, with tabes dorsalis, as one of the parasyphilitic diseases, or, as I prefer to call it, one of the forms of degenerative syphilis.
Statistics do not yet give more than twenty to fifty per cent, of cases with undoubted histories of syphilis ;f in my own experience this per- centage is fifty, and the disease is so often and so distinctly traced to syphilis in a large number of cases that we must infer its relationship in the rest. Injuries, sunstroke, exposure, acute diseases may be put down as exciting causes, but their importance is not very great. An almost sure recipe for producing a case of paresis is this: Let a man of nervous constitution acquire syphilis between the ages of twenty and thirty, then let him work as hard as possible without vacation under great mental strain, drink a great deal of alcohol, and indulge excessively sexually. This will be pretty sure to bring on paresis in ten or fifteen years.
The disease occurs most often between the ages of twenty and forty, but it is seen both earlier and later than these ages. It occurs oftener in married men and women. It is seen oftener in the city than in the country.
Symptoms. — In its typical manifestation the disease shows two stages: one of excitement or irritation; the second of dementia and paralysis. In place of the excitement of the first stage the patient may have a prolonged period of hypochondriasis or melancholia, or, with no preliminary stage of excitement or depression, may pass gradually into dementia, the mental and bodily feebleness going on together.
The first type is the most common of all, although the opinion is growing that the types characterized by little active mental disturb- ance and by more marked physical symptoms are becoming more fre- quent; in other words, that paresis is becoming more a disease of the
* Among 85 private cases there were 78 men and 7 women (1 to 15). There was a distinct history of syphilis in 43 (nearly sixty per cent.). The nationalities were: United States, 49; Hebrews, 20; Irish, n; Germans, 3; Italian, 1; Cuban, 1. Total, 85.
t Among personal cases Regis found eighty per cent, syphilitic. Krafft-Ebing found, among 175, fifty-six per cent, gave a history of syphilis. In 41 cases of paresis in chil- dren syphilis could be traced in 87.8 per cent. (Zappert). Among 24 cases in adults, 16 gave a history of syphilis (F. Mott).
752
DISEASES OF THE MIND.
brain and spinal cord proper and less a disease of the mind, the mental symptoms* being almost from the first more of a progressive dementia.
Excited Type. — In the first type of cases the patient begins by show- ing unusual irritability of temper; trivial things annoy him, and his bad humor and change of disposition become noticeable in his family relations and in his business. He is fretful; complains of being easily fatigued; loses interest in his affairs, and is unable to fix his attention for any length of time upon them. He makes occasional mistakes of judgment, and does some extravagant or foolish thing in the way of purchasing or selling. This condition of irritability is followed by one of great mental exaltation. The patient becomes very happy and cheerful and confident; he feels better than he ever did before in his life. He talks excessively, and is effusive and jocose when he used to be sober and reserved. He develops great schemes for the future, he lavishes money uselessly in making presents to his family and friends, or in some extraordinary business venture, and imagines himself pos- sessed of immense wealth or great power. He has, in other words, de- lusions of grandeur, or megalomania. This condition of exaltation is interrupted by outbursts of violence, especially if it leads him to in- dulge in drink, as is often the case. In the course of three or four months the symptoms become so marked that the family recognize the seriousness of his state, and he is confined in some institution where he can do himself and others no harm. Under institutional regime he now becomes somewhat more quiet; his exaltation softens down. His symptoms may even remit, and for a time he becomes nearly or quite rational. But after some months he begins to show distinct signs of dementia; the memory becomes weak, he forgets recent events, mis- lays things, makes mistakes in his accounts, is unable to add cor- rectly; he cannot write a letter coherently, or if he does there are mistakes in spelling and elisions of letters.
During or before the exaltation there gradually appear physical symptoms which are very characteristic. The patient's hands become tremulous, and his handwriting is so affected that his signature often cannot be recognized. There is distinct and decided facial tremor, particularly apparent if the patient is made to close the eyes and stretch the muscles of the lips so as to show the teeth.
There is marked tremor of the tongue, all this tremulousness being much more exaggerated than is seen in other diseases, except occa- sionally in acute alcoholism. The speech becomes stuttering and thick,
* Among 40 private cases the types were: excited, 15; hypochondriacal, 10; dement- ing, 15. Mendel, among 194 cases of all kinds observed since 1880, found 37 of the typical form and 70 of the dementing type.
ORGANIC PSYCHOSES.
753
and he cannot pronounce long words clearly. On examination of the reflexes it is usually found that the knee-jerks are exaggerated. The pupils are almost always uneven and, as a rule, react badly to light though fairly well to accommodation, showing, in other words, the Argyll-Robertson pupil; sometimes they do not react either to light or accommodation. The fundus oculi is normal.
There is an early and decided weakness of the sexual function. The bladder may also become weak. The appetite and vegetative organs remain in fairly good condition. The patient often suffers from persistent insomnia. During this time he also has occasionally vertigi- nous, syncopal, or apoplectiform attacks. In the latter he falls down and perhaps has hemiplegia lasting for a, few days or a few weeks. An epileptic convulsion may occur.
In some cases the knee-jerks are abolished, and there are some ataxia and evidence of a posterior sclerosis. The general muscular power is much diminished, and the patient is unable to take long walks or do any great amount of physical exercise.
In the second stage the most striking feature is the gradual onset of dementia. The patient now becomes more quiet and is inclined to sleep during the daytime. He takes little interest in affairs about him, is extremely forgetful, and is often unable to recognize even his intimate friends. He no longer knows the day of the month nor the year, and cannot tell one anything about current events of the day. He becomes gradually careless about his person, and has to be watched while at his meals lest he spill food on his clothes, and at the toilet lest he soil himself. Finally, he needs to be cared for as if he were a child.
At this late period, again, attacks of an apoplectiform character may come on, leaving him temporarily or perhaps permanently hemiplegic. His appetite often continues good, sometimes voracious, and he may gain flesh. He is apt at this time to have periods of excitement in which he has delusions of persecution, or he may have some slight de- lusions of grandeur. One patient of mine used to weigh himself every day and think he was gaining ten pounds each time. He kept on till he thought he weighed nine hundred pounds.
In the last scene of all he becomes bedridden and helpless, and finally dies of exhaustion. The somatic symptoms during this last period consist of increased tremor, disturbances of speech, and gradual muscular weakness until the patient becomes helpless.
The average duration of the disease is about three years. There are some acute, galloping forms in which the patient dies within a year, and there are some cases in which the patient reaches a stage of partial 48
754
DISEASES OF THE MIND.
or complete dementia and remains in this condition for ten or fifteen years.
The Hypochondriacal Type. — In this form the disease begins with symptoms resembling those of neurasthenia and hypochondriasis. The patient complains of disagreeable sensations about his head, hemicrania, pain in his limbs and back, inability to sleep, disorders of the stomach, and vague sensations of discomfort and oppression which he is unable distinctly to describe. These patients are often treated as neurasthenics for a long time, and at first show hardly any physical or mental symptoms suggestive of the real trouble. Careful examination, however, almost invariably reveals rigid pupils, or a history of syphilis which should always put one on guard.
After a period of perhaps a year, evidences of mental disturbances begin to appear, and they are mostly those of dementia with perhaps delusions of persecutions and suspicion. These delusions may be ac- companied by occasional outbreaks of excitement and violence, but the paretic is rarely homicidal, and, it may be added, rarely suicidal. After dementia has set in, the physical symptoms of tremor, scanning speech, and tremulous handwriting all become noticeable, and the final stage resembles that of the other form.
Dementing Type. — In a third type there is a primary dementia. The disease begins without any excitement or any special depression, with symptoms of forgetfulness, lack of attention to business, and incapacity to do work. The patient makes mistakes in his calcula- tions, mislays and forgets things, and soon is found by his employer to be of no use. He is often good-natured, not unhappy, and without distinct delusions of any kind. The somatic symptoms of tremor, fixed pupils, and exaggerated reflexes appear and become finally characteristic. Scanning speech is not always present, or comes on later.*
Syphilitic Pseudo-paresis. — There are some cases of paresis in which symptoms of exudative syphilis introduce the disease. The patient has at first eye palsies or attacks of hemiplegia with intense headache followed by convulsions. It is recognized that he has a syphilitic exudate pressing upon some part of the brain, either the base or the convexity, usually the former. Under proper treatment, this resolves and he gets over the paralysis and the seizures, but it is now found that his mind is slightly affected. He has no delusions perhaps,
*The urine in the excitable stage shows abnormal increase of solids; in the quiet and dementing stage abnormal diminution (Laiilier). The red blood-cells and haemoglobin fall below the normal, the latter more than the former; most cases show a slight leuko- cytosis. There is a decrease in the lymphocytes and increase of large mononuclear cells (J. A. Capps).
ORGANIC PSYCHOSES.
755
and no immediate exaltation, but his memory is impaired, judgment weakened, his emotional condition is one of excitability, and he has to give up business and live a quiet, inactive life. If he does this, in some cases the disease becomes arrested, and he remains fairly well for a number of years. Cases of apparent recovery even have been reported, but in my experience dementia finally sets in, though it may not be till six or seven years have passed.
Alcoholic Pseudo-paresis. — Persons who have for long periods of time continuously and excessively indulged in alcohol may develop, and generally do, a condition of mental weakness which to a certain ex- tent simulates paresis. If these patients have not had syphilis, how- ever, it is not a true paresis. The patients become weak-minded, feeble in judgment, poor in memory, their moral instincts get out of control, and acts of extraordinary selfishness, bestiality, and besotted- ness are manifest. They gradually become more enfeebled, and finally enter a condition of dementia if they are not carried off by some intercurrent disease, as is often the case. These cases, however, do not present the physical symptoms of paresis. They do not have the speech disturbances, the paralyses, or the apoplectiform seizures that characterize the true disease, and if they can be kept from alcohol they may remain in a state of partial dementia for many years.
Diagnosis. — The diagnosis is based on the history of syphilis, the facial and tongue tremor, irregular and rigid pupils, exaggerated or irregular knee-jerks, the disturbance of speech, weakness of memory, loss of power to work, irritability and change of character, change in the handwriting, and presence of convulsive or apoplectiform attacks. Examination of the cerebrospinal fluid usually shows the presence of mononuclear lymphocytes instead of a normal clear fluid. Cholin is usually found in this fluid.
Pathology. — The disease, as I have already stated, is essentially a parasyphilitic one, and is due to a degenerative change which sets in in the cells of the brain as the result of poisoning by syphilis. These syphilitic changes produce thickening of the membranes and arteries of the brain, with proliferation of the perivascular tissue. It is believed by some that the vascular changes precede the cell degeneration. It is, however, a more general opinion that the cell degeneration is pri- mary, just as it is in locomotor ataxia.
Examination of the brain of the paretic shows that the dura mater is thickened and vascular, and the membrane is adherent to the cranial bones. The brain itself has undergone atrophy (two to six ounces), and there is increase of the cerebrospinal fluid. The pia mater, es- pecially in the fore and mid regions of the brain, is congested, and
756
DISEASES OF THE MIND.
there is thickening of it and of the arachnoid, and the whole membrane is adherent to the cortex beneath it, which is found to be cedematous.
Microscopical examination shows increase of connective tissue around the blood-vessels of the cortex, thickening of the vascular coats, and degeneration of the nerve-cells in all grades. The disease in- volves not only the gray cortex, but the cells and nuclei of the medulla. There are often associated with paresis lesions of the posterior and lateral columns of the cord; in fact, it is not very uncommon to have a certain amount of locomotor ataxia associated with the lesions of paresis. A small percentage (three to five) of the cases of locomotor ataxia end in this disease.
Prognosis. — The prognosis is usually said to be invariably bad. If one sees the patient, however, in the very earliest stage and removes him at once from all forms of excitement, and makes him live quietly for a year, using anti-luetic or tonic treatment, one can sometimes check the disease, at least for a time. I have several patients who seem in this way to have been apparently cured. When the disease has well entered upon its course it is incurable by any measures as yet known. It is probably true that the disease shows remissions oftener than it used to do, and that it is less refractory to treatment than it was formerly.
Treatment. — As some of the early symptoms of the disease are often associated with a history of syphilitic infection, it is wise to put the patient promptly under anti-syphilitic treatment, preferably by hypodermic injections of mercury. At the same time, he should be sent to some quiet place in the country or to some institution where he can be made to lead an extremely regular and quiet, even life. These two measures, if applied early, have produced remissions which have lasted for from three to six months, even a year. After the anti-syphi- litic treatment, the patient should be given various tonic measures, such as the glycero-phosphate of lime in doses of thirty grains a day, with iron and strychnine.
The use of a cold wet pack and ice cap applied daily for a period of about one to two hours, followed by massage, sometimes produces decided relief, especially in the more excitable cases. The tonic form of hydrotherapy, such as cold baths and douches, is indicated in the depressed types.
CHAPTER XII.
THE PSYCHOSES OF SENILITY.
And the Psychoses of Epilepsy, Psychoses of Gross Organic Brain Disease and of Trauma.
There are generally described under this head several types of mental disturbance, more particularly one called "senile insanity." In the writer's opinion there is no need of making such a separate divi- sion. The insanity that is specially characteristic of senility is senile dementia. The other psychoses of this period are some type of the in- sanities already described, modified perhaps somewhat by the physical condition and deterioration of the patient. Thus old people are sub- ject at times to attacks of confusional insanity due to the ordinary causes of this psychosis, but running a rather milder course. They often clear up perfectly, but at times leave the patient somewhat mentally impaired. The senile are also subject to melancholia and mania in all its types. Even a neurasthenic insanity with obsessions, and a para- noia may develop at this period.
Senile insanities make up about eight per cent, of all cases. About one-third of the cases are some type of melancholia. Taking all kinds of senile insanity, about thirty-three per cent, get well. Nearly all types that develop after the age of seventy-five are dementias.
Senile dementia is a chronic dementing psychosis characterized by progressive enfeeblement of memory and general mental deteriora- tion, with occasional episodes of excitement.
Etiology. — The disease occurs most often between the ages of sixty and seventy-five years. It affects the two sexes about alike. There is some hereditary taint in about one-half the cases, and the exciting cause is often some mental or physical overexertion or some acute and exhausting disease.
Symptoms. — -The trouble begins with defect in memory, and this is most marked in memory for recent events. The patient es- pecially forgets names and places, mislays things, makes mistakes in dates and appointments, and finds the transaction of business difficult. There is an increasing lack of interest in new things and inability to ac- quire fresh knowledge. Attention is weak. The interests of life be- come narrowed, and the patient employs himself in going over and over the same things and in spending the time in trivial details. Sleep is
757
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DISEASES OF THE MIND.
usually disturbed, and the patient gets up at night and wanders rest- lessly about the room or house. Delusions are apt in time to appear, and under their influence the patient gets excited and says or does foolish things. As the disease increases, general childishness becomes manifest. The patient is irritable and peevish, or excited and perhaps expansive in his ideas about business and plans for the future. In time he becomes unable to orient himself, and if he goes on the streets, wanders about and gets lost. Sometimes he is attacked with short periods of delirious excitement in which he attempts to do some violent act, or perhaps breaks away from the house and runs screaming into the street. The patient gets careless of his dress and person; he shows hebetude and tendency to sleep in the daytime. Finally, a condition of apathy and semi-coma and complete and helpless dementia sets in.
Along with this mental change there is generally some emaciation and loss of appetite, tremor develops, and the speech becomes dis- turbed. This disturbance of speech is one of aphasia and paraphasia rather than syllabic disturbance of paresis. Usually the patient has some headache, tinnitus, and dizzy attacks. The tendon reflexes are increased and the skin reflexes abolished. The pupillary reflexes are sluggish. There are insomnia, restlessness, emaciation, physical feebleness, increase in the normal wrinkling of the skin, baldness, and loss of acuteness of special senses, dryness and glossiness of the skin, evidence of tortuous and thickened arteries, tendency to turgescence or flushing of the face on excitement, fainting attacks, and tremors of the head and hands.
The disease lasts ordinarily from three to five years. It may be interrupted or terminated by an attack of hemiplegia, due sometimes to cerebral hemorrhage, sometimes to a pachymeningeal effusion.
Diagnosis. — The main characteristics are a forgetfulness and mix- ing of old with new memories, repeating stories and questions; weak- ness of the power of attention and inability to remember or acquire recent things, and mental fatigue; the lack of interest in new things, apathy, with variability of moods; peevishness, suspiciousness, temper. At times conduct is easily influenced, and at other times the patient is obstinate and perverse. Change of character, episodes of maniacal excitement, and delusions of grandeur or suspicion distin- guish the condition from simple dotage.
The prognosis is almost uniformly bad, though one occasionally sees a patient who seems to be threatened with senile dementia im- prove and remain fairly well for a long time.
Treatment is the treatment of old age and arterial sclerosis. Oc- casionally some of the symptoms are relieved by securing sleep,
THE PSYCHOSES OF SENILITY.
759
strengthening the heart, and improving the digestive and eliminating functions.
Senile Deterioration. — Loss of memory, disorientation as to time and place, marked retention defect. Collecting worthless articles, mild restlessness. Pronounced arteriosclerosis; transitory hemiplegic attack which accentuated the decline.
M. Gy., admitted August 21, 1906, get. 65.
Family History. — Negative for two generations.
Personal History.— The patient was born in Ireland; came to the United States about forty years ago. She has always been a healthy, temperate woman, not considered in any way peculiar. Married and has borne two children.
Nine years before admission a gradual change manifested itself in the patient. She grew forgetful, seemed absent-minded about her housework, exhibited a stubborn, peevish mood. Two years before admission she suffered a paralytic stroke affecting the left side. The paralysis disappeared in a few weeks, but it left her in a much worse mental condition. The memory defect became more serious. She no longer recognized her surroundings; she wandered about, in an aimless fashion and talked of "going home" while she was really in her own house. She collected worthless articles and old rags, hid them carefully away, fearing they might be stolen. She strayed away from home and got lost on the street.
On admission to the Hospital the patient was quiet and well be- haved, greeted the physician pleasantly and remarked: "You have nice rooms here." She was completely lost as to her whereabouts and said : "This is Ireland ; this is a good place, we are all right here." Although it was August she thought it was winter time. She could give no account of her recent movements, or how she came to the Hospital. Every few minutes she would rise and start for home. She talked in a simple manner of her home conditions: "Everyone is very nice and good down where I am — we get along all right — we never ask anything of nobody — we have enough to eat and drink — ■ that's all we want." She had no delusions.
Physical Examination. — The patient is a white-haired old woman; skin dry and wrinkled; hearing slightly impaired; gait rather feeble. The pupils are small, irregular in outline, but react well to light; the tongue deviates slightly to the right and the right-hand grip is not so strong as the left; knee-jerks exaggerated; no residuals of the previous left hemiplegic attack. The superficial arteries feel thick and beady; the pulse is often irregular; the second sound of the heart accentuated.
In the Hospital the patient's condition has shown little change; she is quiet and inoffensive, wanders about the wards and wishes to start for home. When greeted she is pleasant and good natured, fabricates as to her recent doings, e.g., talks of [having been in the fields yesterday playing with the children. Concerning her age, she makes ridiculous statements, varying in a few minutes all the way from nine to fifty years. Her grasp on the remote past is greatly confused and she contradicts herself repeatedly. Says she was born in 67th Street, but a moment later assents that she was born in Ireland. One
760
DISEASES OF THE MIND.
moment she claims that she was never married, a little later she talks about her husband and says she had ten or twelve children (really two). She cannot recall a name or a number a few seconds after it is given to her. She has never gained any clearness as to her whereabouts, the length of time in the Hospital or the date. She seems to realize to some extent her memory deficiency, but she does not appreciate the contra- dictions which she makes. She explains her failure to recollect things by saying: "I don't know anything any more since my boy got killed." She spoke of this event having occurred eight months ago whereas it was almost ten years ago. (G. H. K.)
The Psychoses of Epilepsy.
The mental disturbances of epilepsy can be grouped under three divisions :
1 st. Psychical seizures.
2d. Chronic mental deterioration and dementia.
3d. Accidental or episodical psychoses of various kinds.
The Psychical Seizures. — The epileptic is subject not only to motor disturbances in the nature of convulsions, but to purely mental dis- turbances which take the place or are associated with these convulsions and which are known as "psychical equivalents." They consist in simple brief periods of clouding or loss of consciousness, or of temporary attacks of mental excitement and wandering, accompanied by auto- matic movements, or by distinct attacks of cerebral automatism and periods of simple mental confusion and dreamy states. These at- tacks, sometimes called "larvated epilepsy," have already been de- scribed in a previous section of the book.
There also come under the observation of the alienist epileptics who have attacks of acute and even furious delirium, in which the patients show extraordinary psychomotor activity and evidences of delusion, so that the state is with difficulty distinguished from that of ordinary very acute delirium due to other causes. Transitory mania or transi- tory frenzy are terms applied to some of these attacks, and it is probable that most of the cases which have been described under these heads are epileptic in character.
Besides this there occur in connection with these disordered mental states violent impulsive acts, during which serious injuries may be done. These cases of delirium, mania, frenzy, and of impulsive acts are relatively rare, except in the more advanced and serious cases. Usually they occur either after a distinct aura or as an episode in the morbid mental state that follows a distinct seizure.
Epileptic Deterioration and Dementia. — Although some epileptics have good minds and a few are of brilliant capacity, yet, as a rule, the
THE PSYCHOSES OF SENILITY.
761
epileptic is rather below the average in intelligence, moral force, and emotional control. Furthermore, as his malady progresses there is a tendency for this mental deterioration to increase. This is particu- larly the case when the convulsive attacks are rather numerous; and while it is true that the petit mal type of epilepsy is very incurable, the mental deterioration that goes with it seems to be somewhat less than in the motor forms. This mental deterioration may reach a certain stage and then cease to progress, just as the malady itself may some- times be entirely arrested. If the disease does progress, however, deterioration goes on until a distinct dementia, characterized by feebleness of memory, general weakness of intellect, and a defective moral sense, ensues. There is usually with this condition a somewhat depressed mental state.
The tendency to mental deterioration is sometimes helped by bromides and other depressing drugs; but this is not always the case, and many patients feel actually better and less depressed when taking medication in proper amount. While the dementia of epilepsy cannot itself be cured, it can often be lessened or checked by short daily per- iods of active exercise, constant employment in the open air, and care- ful attention to elimination.
The Psychoses of Cerebral Hemorrhage and Softening.
After a hemiplegia there is usually some diminution in mental power and emotional control. This may amount to a real dementia, especially in the very aged. Thrombotic hemiplegia is more often followed by these changes, because in these cases the patient is usually older and there is more arterial disease. When aphasia complicates the case there is more apparent and usually more real mental disturbance.
Arteriosclerotic psychoses are hardly to be distinguished from those of senile dementia and of the deterioration following hemor- rhage and thrombosis. In fact, there is always something more than ordinary arterial sclerosis in a mental disorder, for the severest grades of this condition are seen without notable mental change.
The mental changes in brain tumor and multiple sclerosis have been referred to in the description of these conditions.
Traumatic Psychoses.
Trauma with injury of the head may be followed by various mor- bid changes in the mental condition. It may be the exciting cause of some functional psychosis to which the patient is predisposed, such as dementia prsecox or a melancholia.
762
DISEASES OF THE MIND.
It may cause a psycho-neurosis of the type of hysteria or some im- pulsive insanity like a dipsomania.
It may in an epileptic especially lead to a dementia, or at least to mental deterioration.
By producing contusions and lacerations of the brain and by the shock it may lead to a rather special morbid mental condition which would be classed best probably as a psycho-neurosis.
My observation coincides with that of Bannister, who says that the patient after the accident "is in a semi-dazed state, in which he seems unable to get rid of some besetting idea often connected with the ac- cident. He asks over and over again in regard to it, apparently for- getting the replies received." Sometimes the patient appears at first to get over the accident, though he does not do so entirely. "After a longer or shorter time, secondary results begin to appear: vertigo, tinnitus, weakness of attention and memory, sometimes a peculiar obstinacy and wrong-headedness, together with morbid impulses and moral deterioration."
This condition rarely happens to the young and pure-blooded.
The hysterical psychoses following head injury have already been described.
INDEX.
Abducens nerve, anatomy of the, 114
paralysis of the, 122 Abiotrophy, 675 Abortive paranoia, 687, 698 Aboulia, 686, 704 Abscess, cerebral, 450 Acoustic nerve, anatomy of the, 203
neuroses of the, 203 Acquired dementia, 738 Acrania, 431 Acrodynia, 103 Acromegaly, 630 Acroparesthesia, 170 Actinomycosis of the brain, 507 Activities, subconscious, 681 Acute dementia, 738 delirium, 688, 738 mania, 689, 724 melancholia, 717
diagnosis, 719
etiology, 718
functional, 717
prognosis, 719
recurrent, 717
simple, 717
symptoms, 718 /Esthesiometer, 57 iEsthesioneurosis, 34, 35 Affect, 680 After-brain, 381
Age as a factor in the etiology of mental diseases, 673 of nervous diseases, 25 Ageusia, 211 Agnosia, 40 Agoraphobia, 700 Agraphia, 426 Akinesia, 38 Akinesis, 38
Alcohol, as a factor in the etiology of mental diseases, 674
of nervous diseases, 27 Alcoholic dementia, 741
delusion, of jealousy, case of, 746
hallucinations, acute, case of, 745
insanity, case of, 745
pseudoparesis, 741, 755
psychoses, 740 Alcoholism, 738
effect of, 27 Alexia, 425, 428 Allochiria, definition of, 41 Amaurosis, 185
Amblyopia, 185 Amentia, 741 Amimia, 426 Amnesia, 685
hysterical, 550
tactile, 40 Ampere, definition of, 78 Amperemeter, 78 Amputation neuroma, 112 Amusia, 426 Amyelia, 263
Amyotrophic lateral sclerosis, 352 Amyotrophy, progressive spinal, 339 Anaemia, cerebral, 449 meningeal, 432
pernicious, combined spinal scleroses of, 328
spinal, 269 Anaesthesia, definition of, 39
hysterical, 542
muscular, 40
trigeminal, 193 Analgesia, definition of, 39 Anarthria, 430
Anatomy, general of the nervous system, i, 2
of the brain, 380
of the spinal cord, 234 Anencephaly, 431 Aneurisms, intracranial, 511, 513
miliary, of the brain, 476, 513 Angio-ataxia, 41
-neurosis, 35, 41
-paralysis, 41
-spasm, 41
Angioneurotic gangrene, symmetrical, 231
oedema, 636 Angiopathic neurasthenia, 589 Anidrosis, definition of, 41 Ankle clonus, 55 Anomia, 428, 429 Anorexia nervosa, 549 Anosmia, 178 Anxiety psychosis, 721 Anxious delirium, 689 Aphasia, 424
auditory, 425, 427
clinical types of, 431
conduction, 426, 431
fronto-lenticular, 429
lenticular zone, 429, 430
mixed, 430, 431
motor, 427, 428
764
INDEX.
Aphasia, occipital, 428
optic, 428, 429
parieto-occipital, 428
parietotemporal, 428
sensory, 428
subcortical, 428
temporal, 428
visual, 427 Aphemia, 426, 430 Aphonia, hysterical, 545 Aphthongia, 146 Apoplexy, cerebellar, 475
diagnosis of the varieties of, 478, 484
dural, 472
embolic, 482
hemorrhagic, 466
medulla, 474
meningeal, 472
pial, 474
pons, 474
spinal, 264
thalamus, 474
thrombotic, 482 Apperception, 678 Apraxia, 425 Aprosexia, 686 Arachnoid, cerebral, 401
spinal, 234 Arbor vitas of the cerebellum, 393 Arborization, terminal, 7 Arcades, interfascicular, 15 Argyll-Robertson pupil, 55
in locomotor ataxia, 308 Arm, cord centres for muscles of the, 253
palsies of the, 154
spasmodic disorders of the, 154 Arteries, cerebral, 402, 404, 406
spinal, 256, 258 Arteriosclerotic psychoses, 761 Arthropathy of locomotor ataxia, 310
pneumogenic osteo-, 633 Associated movements, 37 Association centres in the brain, 397
of thought, disorders of, 684 Associative functions of the brain, 415 Astereognosis, 40
Asthenopia, muscular, of the eye, 124 Astraphobia, 700 Asymbolia, 40 Ataxia, cerebellar, 40
definition of, 39
family, 332
Friedreich's, 331
hereditary cerebellar, 331, 336, 363 spinal, 331
Ataxia, locomotor, 301, and see Locomotor Ataxia. motor, 39, 301
testing for, 64 static, 39
testing for, 63 tests for, 62 Ataxiagraph, 63
Athetosis, 37
Atrophia musculorum lipomatosa, 357 Atrophies, progressive muscular, 339 Atrophy, arthritic muscular, 364
hereditary muscular, of peroneal type,
344
occupation muscular, 364 optic, 184
progressive muscular, 339 facio-scapulo-humeral, 361 hereditary, 363
of peroneal type, 344 infantile, 361
hereditary spinal, 363 remitting spinal, 346 spastic form of, 352 Auditory nerve, anatomy of the, 203 neuroses of the, 203 vertigo, 207 Aura, epileptic, 527 Automatism, cerebral, 688
in hysteria, 550 Autotoxic psychoses, 738 Axis-cylinder, 6, 10, 11 Axon, 6
Babinski, sign of, 51
Back, cord centres for muscles of the, 255
Baillarger, line of, 387
Ballet-dancers' cramp, 615
Basal ganglia, tumors of the, 501
Basedow's disease, 601
Baths in the treatment of nervous diseases,
71, 74 Batteries, electric, 78 Beard's disease, 574 Bell's palsy, 132 Bends, the, 267 Beriberi, 103 Besetment, 687 Birth palsies, 489 Blepharospasm, 131 Blindness, mind, 425 word, 425
Blood-supply of the brain, 402, 404, 406
of the spinal cord, 255 Blood-vessels of the peripheral nerves, 14 Borderland cases, 697 Brachial nerves, diseases of, 154 paralysis of, 154 «
plexus, anatomy of the, 152 sensory neuroses of the, 214 Brachycephalic, definition of, 46 Brain, abscess of the, 450 chronic, 451
actinomycosis of the, 507
anaemia of the 449
anatomy of the, 380
aneurisms of the, 476, 511, 513
association centres, 397
associative function of the, 415
bilateral representation, 410
blood-supply of the, 402, 404, 406
INDEX.
765
Tain, cancer of the, 511 cells of the cortex, 387 central lobe, 386 centres in the, 407 centrum ovale, 415 cerebellum, 393, 416 compression symptoms, 423 connecting tracts of the, 397 convolutions of the, 383 corpora quadrigemina, 391, 416
striata, 389, 415 corpus callosum, 415 cortex of the, 383, 387 cranio-cerebral topography, 656 cutting the, 420 cystic tumors of the, 508 cysticerci of the, 511 development of the, 380 diagram of the divisions of the, 2 diseases of the, 423, 448 echinococcus of the, 511 embolism of the arteries of the, 482 endothelioma of the, 511 fibres of the cortex, 387 fibroma of the, 511 fissures of the, 383 focal symptoms, 423 frontal lobe, 383 functions of the, 407 ganglionic deposits of the, 382 glioma of the, 508 gumma of the, 507 hemorrhage in the 466
in the membranes of the, 466, 472 hyperemia of the, 448 inflammation of the, 450 irritation symptoms, 423 island of Reil, 386 latent regions of the, 415, 418 limbic lobe, 386 lobes of the, 383 localization in the, 407 Luys' body, 393 malformations of the, 431 meninges of the, 401
diseases of the, 432
hemorrhage in the, 466, 472 miliary aneurisms of the, 476, 513 motor tracts in the, 397 occipital lobe, 385, 412 olfactory lobe, 386 operculum, 386 optic thalami, 390 osteoma of the, 511 palsies, of children, 488 parasitic growths in the, 511 parietal lobe, 384, 411 preserving the, 420 projection system, 397 red nucleus, 393, 416 sarcoma of the, 510 sensori-motor area, 408 sensory tracts in the, 400
Brain, softening of the, acute, 482
chronic progressive, 487 substantia nigra, 393 symptoms of disease of the, 423 syphilis of the, 514, 517 syphiloma of the, 507 temporal lobe, 385, 412 thalamus opticus, 415 thrombosis of the arteries of the, 482 topography of the, 656 tubercle of the, 506 tumors of the, 495
general symptoms of, 497
local symptoms of, 498
multiple, 506
of the basal ganglia and capsule, 501
of the central convolutions, 500
of the cerebellum, 504
of the cerebral peduncles, 504
of the corpora quadrigemina, 504
of the corpus callosum, 501
of the corpus striatum, 502
of the isthmus, 504
of the medulla, 503
of the mid-brain, 504
of the occipital lobes, 501
of the optic chiasm, 502
of the optic thalamus, 502
of the optico-striate region, 501
of the parietal lobe, 500
of the pituitary body, 502
of the pons, 502
of the ponto-cerebellar angle, 505 of the prefrontal area, 499 of the temporal lobes, 501 veins of the, 406 weight of the, 418 wet, 444 Brauch-Romberg symptom, 63 Bulbar paralysis, asthenic, 350 progressive, 347 upper, 123 Bulbo-spinal paralysis, asthenic, 350 Burdach, column of, 241, 244
Caisson disease, 266
Cajal's cells, 8
Cancer of the brain, 511
of the spinal cord, 367 Capsule, tumors of the, 501 Caput obstipum, 141 Catalepsy, 651
hysterical, 550 Cauda equina, anatomy and diseases of, 375
lesions at different levels, 376 Causes of mental diseases, 673
of nervous diseases, 24 Cells, Cajal's, 8
cerebral cortical, 387
Deiter's, 8
Golgi's, 8
nerve-, 2, 5, 16
766
INDEX.
Cells, neuroglia, 13
of the spinal cord, 238 Purkinje's, 394 scavenger, of Lewis, 14 spider, 13
stellate, of the cerebellar cortex, 394 Central convolution, tumors of the, 500 Central nervous fibres, 12 Centres, cerebral, 407
spinal, 247 Centrum ovale, 415 Cephalalgia, 193
hydrotherapy in, 663
location of pain according to the cause, 196
Cephalic index, 690
Cerebellar ataxia, hereditary, 331, 336, 363 Cerebellum, abscess of the, 453
anatomy of the, 393
functions of the, 416
gray matter of the, 394
hemorrhage in the, 475
tumors of the, 504
white matter of the, 396 Cerebral automatism, 688
hemorrhage, psychoses of, 761
peduncles, tumors of the, 504
softening, psychoses of, 761 Cerebrospinal fluid, 402 examination of, 65
meningitis, 435
multiple sclerosis, 458 aborted types of, 462
nerve, sensory neuroses of the, 169
syphilis, 518 Cervical nerves, motor neuroses of the, 151, 154
sensory neuroses of the, 212, 214 Cervico-occipital pains, 212 Charcot's disease, 352
Charcot-Marie type of progressive heredi- tary muscular atrophy, 344 Chemistry of the nervous system, 22 Chiasm, optic, 180 Choked disc, 182 Chokes, the, 267 Chorea, dancing, 560
electric, 560
hereditary, 363, 560
Huntington's, 560
insaniens, 557, 558, 742
major, 560
maniacal, 557, 558
of the larynx, 561
paralytic, 558
procursive, 560
senile, 558
Sydenham's, 555, and see Sydenham' s chorea Choreic insanity, 740
movements, 37, 50 Chromophilic granules of the nerve-cell, 8 Chronic melancholia, 717, 719
Chronic melancholia, etiology, 720
prognosis, 721
symptoms, 720
treatment, 721 Cigarmakers' cramp, 615 Circular insanity, 724 Circumflex nerve, paralysis of the, 161 Civilization as a factor in the etiology of mental diseases, 673
of nervous diseases, 27 Clarionet players' cramp, 615 Clarke's column, 238 Claustrophobia, 700 Climacteric insanity, 724 Climate as a factor in the etiology of mental diseases, 674
of nervous diseases, 27
in the treatment of nervous diseases, 75 Clonus, ankle, 55 Coagulation necrosis, 32 Cocainism, 738 Coccygodynia, 231
Cold, effect of in the treatment of nervous
diseases, 72 Collapse delirium, 689, 741 Collaterals, 6, 12
Columns of the spinal cord, 235, 239, 240,
244, 246 Coma, 688
apoplectic, 468 Compulsion, 687
morbid, 702 Compulsive insanity, 697, 699
manias, 702 Concept, 678
Condition, as a factor in the etiology of mental diseases, 673 of nervous diseases, 26 Confusion, acute, case of, 748 Confusional insanity, 738 etiology, 739 prognosis, 741 symptoms, 739 treatment, 742 types of, 738 Conjugate deviation of the eyes, definition of, 119 spasmodic, 126 Consciousness, 678 clearness of, 688 clouding of, 688 disorders of, 688 double, 688 Contraction, paradoxical, 56 Contracture, definition of, 37
hysterical, 546 Convergence, paralysis of, 122 Convolutions, cerebral, 383
tumors of the, 500 Convulsions, 36 atonic, 36 epileptic, 526, 553
INDEX.
767
Convulsions, hysterical, 553
tonic, 37 Convulsive tic, 37 Coprolalia, 561 Cord, spinal, see Spinal cord. Corpora quadrigemina, 391, 416
tumors of the, 504 Corpus callosum, 415
tumors of the, 501
striatum, 389, 415 tumors of the, 502 Cortex, cerebellar, 394
cerebral, 383, 387 Cramps, 37
occupation, 614
writers' 609 Cranial nerves, afferent origin of the, 115
sensory neuroses of the, 176 Cranio-cerebral topography, 656 Cranium, table of measurements of the, 47 Cretinism, 635
Crises in locomotor ataxia, 310 Current, electrical, employed in the treat- ment of nervous diseases, 77
primary induced, 79
secondary induced, 79
strength, definition of, 77 Cyclopia, 432 Cyclopegia, 122 Cyrtometers, 656 Cystic tumors of the brain, 503 Cysticerci of the brain, 511 Cytoplasm, 7, 23
Dead fingers, 232
in writers' cramp, 612 Deafness, nervous, 204
word, 425 Debiles, 25 Degenerates, 25
Degeneration, anatomical stigmata of, 690
as a factor in the etiology of nervous diseases, 25
neuritic, complicating forms of, 108
of nerves, 94
of nervous tissue, 31, 32
of optic nerve, 184
reactions of, 83
stigmata of, 44
varieties of, 32
Wallerian, 94 Degenerative insanity, 697 Deiter's cells, 8 Delires systematise^, 732 Delirium, 684, 688
acute, 688, 738
anxious, 689
collapse, 689, 741
febrile, 738
grave, 688
hallucinatory, case of, 744 low, 689
tremens, 688, 741
Delusion, 683
expansive, 685 Delusional insanity, 732 Dementia, acquired, 738
acute, 738
alcoholic, 741
epileptic, 760
paralytica, 750
paranoides, 711
praecox, 708 age, 708
apathetic deterioration, 712
cases of, 712
catatonic form, 715
census in psychopathic ward, 708
diagnosis, 711
etiology, 708
hebephrenic deterioration, 713
type, 709 paranoid type, 711 prognosis, 711 simple, 710 symptoms, 709 treatment, 712 variations in onset, 711 primary, 738 senile, 757 Dendrites, 6, 15, 17
cellupetal impulses in the, 15, 17 Depression, 685 Detail, mania for, 704 Deterioration, epileptic, 760 Development, organic defect of, 672 Deviation, 25
Diagnosis, methods of, in insanity, 693 electro-, 83
in nervous diseases, 42 Diaphragm, paralysis of the, 152 Diathesis as a factor in the etiology of nervous diseases, 27
contractural, 546 Diencephalon, 3, 380 Diet employed in the rest treatment, 665
for the neuropathic, 68 Die Verrucktheit, 732 Digiti mortui, 231
in writers' cramp, 612 Diplegia, definition of, 38
hereditary cerebral, 363
infantile, 488 Diplopia, definition of, 119 Dipsomania, 740 Diseases of the brain, 423, 448
of the mind, 671
of the nervous system, 1 Disorders of association of thought, 684
of consciousness, 688
of function, in insane, 690
of ideation, 684
of judgment, 683
of perception, 683
of volitional function, 686 Disorientation, 684
768
INDEX.
Distractability, 686 Divers' paralysis, 266 Dizziness, 206
Doliocephalic, definition of, 46 Dorsal nerves, anatomy of the, 165
motor neuroses of the, 166 Dotardness, 671 Double consciousness, 688 Doubting mania, 699, 701 Douche, use of, in the treatment of nervous
diseases, 72 Dreams, 644 Drivers' spasm, 615 Dropfoot, 168 Drowsiness, morbid, 650 Drug insanities, 741 Drunkenness, sleep-, 645 Duchenne-Aran's disease, 339 Duchenne, muscular atrophy of, 355 subacute spinal paralysis of, 294 Dura mater, cerebral, 401
inflammation of the, 432 spinal, 234
inflammation of the, 270
syphilis of the, 271 Dynamometer, 48 Dysacusis, 211
Dysesthesia, definition of, 40
Dysphagia, 138
Dystrophies, hereditary, 364 juvenile, of Erb, 361 progressive muscular, 339, 354
Ear, neuroses of the, 203 Echinococcus of the brain, 511 Echokinesis, 561 Echolalia, 561 Eclampsia, 524 nutans, 151 Ecstasy, 685
Education, as a factor in the etiology of
mental diseases, 675 Electricity, appliances for the therapeutic use of, 78 employed in the rest treatment, 666 in the treatment of nervous diseases, 76, 86
methods of application of, 82 terms employed in relation to, 77
Electrodes, 82
Electro-diagnosis, 83
Electromotive force, definition of, 77
Electrotherapeutics, 80, 86
Electro tonus, 22
Embolism, cerebral, 482
special forms of, 485
Emotion, 680
Emotional complex, 681 state, 681
Encephalitis, acute non-suppurative, 455 acute suppurative, 450 chronic suppurative, 451 hemorrhagic, 455
Encephalocele, 432 End-brush, 7
-buds, 7
-organs, 20 Endoneurium, 10 Endothelioma of the brain, 511 Enteritis, mucous, 585 Enteroptosis, 585 Epicritic fibres, 20
sensation, 20
sensibility, 19, 20 Epilepsia tardiva, 530 Epilepsy, 523
abortive attack, 527
acute, 524
association diseases, 530 aura, 527
complicating infantile hemiplegia, 490
convulsion, 526, 553
course, 533
diagnosis, 532
etiology, 524
grand mal, 524, 530
haut mal, 530
hydrotherapy in, 662
hystero-, 524, 549
idiopathic, 523
Jacksonian, 524, 527
larvated, 760
laryngeal, 210
matutinal, 528
mental condition in, 528
minor attack, 527
myoclonus-, 562
onset, 526
partial, 524, 527
pathology, 531
petit mal, 529, 530
physical condition in, 529
physiology, 532
procursive, 527
prognosis, 533
psychical, 524, 527, 530
psychoses of, 757, 760
severe attack, 526
sleep-, 651
somnambulic, 527
symptomatic, 524
symptoms, 526
treatment, 533
types, 530 Epileptic dementia, 760
deterioration, 760 Erb's juvenile dystrophy, 355, 361
palsy, 156
syphilitic spinal paralysis, 281 Erroneous projection, definition of, 119 Erythromelalgia, 229 Esophoria, definition of, 124 Etiology of mental diseases, 673
of nervous diseases, 24 Euphoria, 685 Exaltation, 685
INDEX.
769
Examination, methods of, in mental diseases, 692 in nervous diseases, 42 Exercise, for the neurasthenic, 70
in treatment of locomotor ataxia, 669 Exhaustion psychoses, 738, 741 Exophoria, definition of, 124 Exophthalmic goitre, 601 Eye, attachment of the muscles of, to the globe, 117 motor nerves of the, 114 muscles which move the, 117 muscular asthenopia and insufficiencies
of the, 124 neuroses of the, 181 spasmodic diseases of the muscles of the, 126
Face, unilateral hypertrophy of, 362 Facial hemiatrophy, progressive, 629
hemihypertrophy, progressive, 630
herpes, 193
nerve, anatomy of the, 127
diseases of the, 129, 203 palsies, 131 spasm, 129 Facio-scapulo-humeral muscular atrophv,
36i
family diseases, 24
nervous diseases, 362 Faradic medical batteries, 79 Febrile delirium, 738 Feeble-mindedness, 671 Feeling, 680
disturbances of, 685 Feelings, instinctive, how divided, 680 Festination, 622 Fever, hysterical, 549 Fibres, nerve-, 9, 10 Fibrillary tremor, 50 Fibroma molluscum, 109
of the brain, 511 Finger pains, 219 Fingers, dead, 231
in writers' cramp, 612 Fixed ideas, 687, 701, 704 Flechsig, oval zone of, 245 Fleece of the cerebellum, 396 Flight of ideas, 684 Flushing, 193 Flute-players' cramp, 615 Folie-a-deux, 734 Folie raisonnante, 732 Forced movements, 37
Forearm, cord centres for muscles of the, 253 Forearm pains, 219 Fore-brain, 380
Foot, cord centre for muscles of the, 255 Fothergill's neuralgia, 190 Fourth nerve, anatomy of the, 114
paralysis of the, 122 Fractures, spontaneous, in locomotor ataxia,
3ii
Friedreich's ataxia, 331
myoclonus multiplex, 562 Function, disorders of, in the insane, 690 Functional insanities, 672
nervous diseases, 523
Galvanic batteries, 81 Ganglia, cerebral, 382 Gangrene, symmetrical, 231 Gastroptosis, 590
Gemmules in the cerebellar cortex, 395 General paralysis of the insane, 750, and
see General paresis paresis, 750
census, of, 751, 752
dementing type, 754
diagnosis, 755
etiology, 750
excited type, 752
hypochondriacal type, 754
pathology, 755
prognosis, 756
symptoms, 751
treatment, 756 Gerlier's disease, 210 Giddiness, 206 Glioma of the brain, 508
of the spinal cord, 372 Gliosis, 33
Globus hystericus, 539, 541
Glossopharyngeal nerve, anatomy of the, 137 motor neuroses of the, 138 sensory neuroses of the, 211
Glossoplegia, 146
Gnosia, 40
Goal idea, 679
Goitre, exophthalmic, 601
Golgi's cells, 8
Goll, column of, 241, 244, 246 Gombault, trangle of, 245 Graves' disease, 601 Gray matter, central, 16
cortical, 16 Groups, symptom, 683 Gudden, commissure of, 180 Gumma of the brain, 507 of the spinal cord, 367
Habit as a factor in the etiology of nervous
diseases, 26 Haematomyelia, 265 Hasmatorrhachis, 264 Haemidrosis, definition of, 41 Hallucination, 683 Hallucinatory delirium, case of, 744 Hallucinosis, acute alcoholic, case of, 745 Hand, cord centres for muscles of the, 253 Head, cord centres for muscles of the, 250 Head's zones, 224 Headache, 193
bilious, 200
hydrotherapy in, 663
location of pain in, according to the cause, 195
49
77°
INDEX.
Headache, morning, 197
sick, 199 Hearing, centres for, 413
hysterical disturbances of, 543 Heart-arm pains, 219
Heat, effects of in the treatment of nervous
diseases, 73 Hebephrenic type, 709 Heine-Medin disease, 294 Hemianopsia, 186 Hemiatrophy, facial, 629
progressive lingual, 147 Hemicrania, 199 Hemihypertrophy, 362
progressive facial, 630 Hemiopic pupillary reaction, 187 Hemiplegia, 424, 466, 470
definition of, 38
hephaestic, 615
hereditary, 363
hysterical, 545
infantile, 488
organic, 545 Hemorrhage, cerebral, 466
meningeal, 466, 472
spinal, 264 Henle, sheath of, 9 Hephasstic hemiplegia, 615 Hereditary nervous diseases, 362 Heredity in the etiology of mental diseases, 674
of nervous diseases, 24 Herpes, facial, 193
zoster, 222 Heterophoria, definition of, 124 Heteropia, 263
spinal, 263 Hiccough, 151 Hind-brain, 381 Hunt syndrome, 203 Hutchinson face, 124
pupil, 473 Hydrencephalocele, 432 Hydrocephalus, 441
acquired, 444
acute, 439
chronic, 441
secondary, 444 Hydromyelia, 369 Hydrophobia, 571 Hydrorrhachis externa, 262
interna, 262 Hydrotherapy in nervous diseases, 71, 662
sedative, 73
tonic, 71
Hygiene of the nervous system, 67 Hyperacusis, 211 Hyperaemia, cerebral, 448
meningeal, 432
spinal, 268 Hyperesthesia, auditory, 211
definition of, 40
hysterical, 544
Hyperesthesia, ocular, 185
retinal, 185 Hyperalgesia, definition of, 40 Hyperidrosis, definition of, 41 Hyperosmia, 179 Hyperphoria, definition of, 124 Hypertrophy, pseudo-muscular, 357
unilateral of face, 362 Hypnotism, 87, 647
lethargic, 648
major, 647
minor, 647 Hypochondriasis, 702 Hypoglossus nerve, anatomy of the,
motor neuroses of the, 146 Hypotonia, 64, 566 Hysteria, 538, 703
amnesia, 550
anaesthesia, 542
aphonia, 545
blue oedema, 549
catalepsy, 550
cerebral automatism, 550
contractures, 546
convulsions, 541, 553
crises, 550
diagnosis, 551
etiology, 538
fever in, 549
globus, 539, 541
hearing in, 543
hydrotherapy in, 664
hyperaesthesia, 544
major, 540
mental state in, 548
minor, 539
motor symptoms, 545
myasthenia, 546
neuralgia, 544
paralyses, 545
pathology, 551
prognosis, 553
psychasthenia, 552
sensory symptoms, 542
suggestibility in, 548
symptoms, 539 of the crises, 540 of the interparoxysmal state,
taste disturbance in, 543
trance, 550
traumatic, 600
treatment, 553
tremor in, 547
trophic disorders, 548
vasomotor symptoms, 549
visceral symptoms, 549
visual disturbances in, 543 Hysterical insanity, 703 Hystero-epilepsy, 524, 549
Idea, 678
Ideation, disorders of, 684 Idiocy, 671
INDEX.
77I
Idiocy, hereditary amaurotic, 363 Ignipedites, 103 Illusion, 683
aural, 683
visual, 683 Imbecility, 671
moral, 680 Impotence, hydrotherapy in, 664 Impulse, morbid, 687 Impulsive act, 687
insanity, 697
manias, 702 Incontinence of urine, hydrotherapy in, 664 Inebriety, 740
Infantile spinal paralysis, 286
Infection as a factor in the etiology of
nervous diseases, 27 Infection psychoses, 738 Inferiority, constitutional, 706
hereditary, 706 Inflammation, classification of, 31
exudative, 31
of brain, 450
of spinal cord, 274
of nervous tissue, 30
productive, 31
proliferative, 31
purulent, 31 Insane, general paralysis of, 750 Insanity, accessory causes, 675
age, 673
alcoholic, 738
alcohol in, 674
choreic, 740
circular, 724
civilization, 673
classification, 671
climacteric, 724
climate, 674
compulsive, 697, 699
confusional, 738
congenital conditions, 673
definition, 671
degenerative, 697
delusional, 732
diagnosis, 693
direct causes, 675
education, 675
etiology, 673
general pathology, 675
heredity in, 674 ,
hysterical, 703
imposed, 734
impulsive, 697
in urban life, 675
legally speaking, 671
manic depressive, 717, 724
medically speaking, 671
methods of examination in, 692
myxcedematous, 738
neurasthenic, 697
occupation, 675
physical symptoms of, 690
Insanity, prognosis, 695
psychogenetic causes, 675
puerperal, 740
race, 674
senile, 757
sex, 673
syphilis, 674
thyroid, 738
types of, 672
symptoms of, 683
syndromes, 688
treatment, 695 Insanities, drug, 741
functional, 672
neurasthenic, 672
organic, 672 Insomnia, 640
hydrotherapy in, 663 Instinctive feelings, how divided, 680 Instincts, 679
Insufficiencies, muscular, of the eye, 124 Intention tremor, 36
Intercostal nerves, sensory neuroses of the, 221
Involution melancholia, case of, 721 Iridoplegia, 121 Irritability of a nerve, 22 Irritation, spinal, 581, 588 Isthmus, 3, 381
tumors of the, 504
Judgment, disorders of, 683 Jumpers, 561
Kakke, 103 Karyochromes, 8 Katatonia, 710 Kernig's sign, 437 Kinesioneurosis, 34, 35 Kleptomania, 687 Knee-jerk, 52
Korsakoff's psychosis, 101, 738, 741 syndrome, 742
Lacrymation, 193
Landouzy-Dejerine muscular dystrophy,
355
Landry's paralysis, 294
Larvated epilepsy, 760
Laryngeal epilepsy, syncope, or vertigo, 210
Larynx, chorea of the, 561
Latah, 561
Latent regions of the brain, 415, 418 Lateral sclerosis, 325
amyotrophic, 352 Lead palsy, 161, 168 Leg, cord centres for muscles of the, 255 Leptomeningitis, cerebral, 433
lumbar puncture in, 434
spinal, acute, 272 chronic, 272 Lethargic hypnotism, 648 Lethargy, 651, 688
Levator palpebral, paralysis of the, 122
INDEX.
Lewis, scavenger cells of, 14 Leyden-Mobius muscular dystrophy, 354, 356
Light reflex, 54 Lisping, 146 Lissauer's column, 237 Little's disease, 325, 488 Localization, cerebral, 407
spinal, 248 Lockjaw, 126 Locomotor ataxia, 301
ankle-tendon reflex in, 305
arthropathies of, 310
ataxic stage, 303
brain symptoms in, 314
complications, 314
course of, 314
crises in, 310
definition, 301
diagnosis, 320
etiology, 301
exercise treatment of, 669 eye symptoms of, 308 forms, 301 fractures in, 311 gait in, 305 gastric crises of, 310 hearing in, 309 hydrotherapy in, 323, 664 initial stage, 302 joint affections in, 310 laryngeal crises in, 310 muscular atrophies in, 313 neuralgia in, 306 optic atrophy in, 308 pains in, 306 • paralytic stage of, 304 patellar-tendon reflex in, 305 pathological anatomy, 315 pathology, 317 pre-ataxic stage, 302 prognosis, 321 sexual power in, 314 skin disorders in, 313 spot pains in, 307 symptoms, 302
syphilis in the etiology of, 301 treatment, 321 trophic disturbances in, 313 Lower extremities, cord centres for the
muscles of, 255 Lumbar nerves, anatomy of the, 166 motor neuroses of the, 166 sensory neuroses of the, 223 Lumbo-abdominal pains, 223 Luys' body, 393
Macewan's sign, 440 Major psychoses, 671 Mania, 685
acute, 689, 724
compulsive, 702
for detail, 704
Mania, functional, 724 impulsive, 702 mixed forms, 724 peracute, 689 Maniacal excitement, 684
acute, 689 Manic-depressive insanity, 724 diagnosis, 726 etiology, 724 manic phase of, 724 prognosis, 726 symptoms, 725 treatment, 726 psychosis, case of, 727 Marie's disease, 630 Massage in the rest treatment, 666
in the treatment of nervous diseases, 75 Mastodynia, 222 Masturbation, 598
Mechanisms of the nervous system, 21 Median nerve, paralysis of the, 162 Medulla oblongata, functions of the, 418 position of the cranial nuclei in the, 127 thrombosis and softening of the, 485 tumors of the, 503 Medullary sheath, 10 Melancholia, 685, 717 acute, 717
diagnosis, 719
etiology, 718
functional, 717
prognosis, 719
recurrent, 717
simple, 717
symptoms, 718
agitata, 719 chronic, 717, 719
etiology, 720
prognosis, 721
symptoms, 720
treatment, 721 delusional, 685 hypochondriacal, 719 manic, 717
of involution, 719, 721 the disease, 717 the symptom, 717 Memories, centres for, 411 Memory, 679
disorders of, 684 Meniere's disease, 207 Meninges, cerebral, 401
anaemia of the, 432 blood-supply of the, 403 diseases of the, 423, 432 functions of the, 402 hemorrhage in the, 466, 472 hyperasmia of the, 432 spinal, 234
hemorrhage in the, 264 inflammation of the, 270 Meningitis, alcoholic, 444 cerebral, 432
INDEX.
773
Meningitis, epidemic cerebrospinal, 435
external cerebral, 432 spinal, 270
internal spinal, 271
lepto-, 272, 433
lumbar puncture in, 670
pachy-, 270
serous, 444
spinal, 270
syphilitic, 520
tuberculous, 439 Meningocele, cerebral, 432
spinal, 262 Meningo-myelitis, 272 Meningo-myelocele, 262 Meralgia, 171, 223 Mesencephalon, 3, 381 Mesmerism, 647 Mesocephalic, definition of, 46 Metencephalon, 3, 381 Micrencephaly, 431 Microcephaly, 431 Micromyelia, 264 Mid-brain, 381
tumors of the, 504 Migraine, 199
fulgurating, 199 Milkers' spasm, 615 Milliampere, definition of, 78 Milliamperemeter, 78 Mind, diseases of the, 671 Miners' nystagmus, 126 Minor psychoses, the, 671, 697 etiology, 698 symptoms, 699 Mixed forms, 724 Monomania, 732 Monoplegia, definition of, 38 Monro, fissure of, morphological import- ance of, 382 Moral imbecility, 680 Morbid compulsion, 702
fear, 699, 704
impulses, 701 Morphinism, 738 Morton's neuralgia, 230 Morvan's disease, 164 Motility, disordered, examination for, 48 Motor tracts in the brain, 397 Movements, associated, 37 choreic, 37 forced, 37
Fraenkel's, in the treatment of nervous diseases, 75
Mucous enteritis, 585
Multiple neuritic psychosis, 741
Multiple neuritis, 98, and see Polyneuritis sclerosis, 458
aborted types of, 462
Muscles, tables showing innervation, func- tions, etc., of the, 250
Muscular asthenopia and insufficiencies, 124
Muscular atrophy, arthritic, 364
Muscular atrophy, facio-scapulo-humeral 361
hereditary progressive, 364 of the peroneal type, 344 in peripheral nerve disease, 97 infantile progressive, 363 occupation, 364 progressive, 339
spastic form of, 352 Muscular dystrophies, progressive, 339, 354 Landouzy-Dejerine type of, 355 Leyden-Mobius type of, 354, 356 Musculo-spiral nerve, paralysis of the, 161 Musicians' cramp, 614 Myasthenia gravis, 350 Myelencephalon, 3, 381 Myelin sheath, 10 Myelitis, 274 acute, 274
definition, 274 diagnosis, 279 disseminated, 274, 281 etiology, 275 forms, 274
pathological anatomy, 277
prognosis, 280
symptoms, 275
transverse, 274
treatment, 280 central, symptoms, 284 chronic, 281
diagnosis, 284
etiology, 281
forms, 281
pathology, 284
prognosis, 285
symptoms, 282
syphilis in etiology of, 281
syphilitic spinal paralysis, 282
treatment, 285 classification of, 274 compression, definition, 274
symptoms, 283 diffuse, definition, 274 disseminated, definition, 274 hemorrhagic, definition, 274 marginal, definition, 281 peri-ependymal, symptoms, 284 purulent, definition, 274 syphilitic, definition, 274 transverse, definition, 274, 281 tuberculous, definition, 274 Myoclonia, 37, 555 fibrillary, 562 degenerative, 560 hysterical, 560 infectious, 555 psychic, 560 Myoclonus, 555 -epilepsy, 562 multiplex, 562
of Friedreich, 562 Myoidema, definition and significance of, 50
774
INDEX.
Myokymia, 36, 50 Myotonia, 555, 564
acquisita, 564
congenita, 564
symptomatica, 564, 565 Myotonus, 555 Myriachit, 561 Mysophobia, 700 Myxoedema, 633 Myxedematous insanity, 738
Narcolepsy, 651
Neck, anatomy of muscles and nerves of the, 149
cord centres for muscles of the, 250 Neck pains, 214 Necrosis, 32
coagulation, 32 Negativism, 686
Nerve, abducens, anatomy of the, 114
paralysis of the, 122 acoustic, anatomy of the, 203
neuroses of the, 203 afferent, 19 anaemia of, 92 angina of, 92
auditory, anatomy of the, 203
neuroses of the, 203 brachial plexus, anatomy of the, 152, 153
paralysis of the, 154
sensory neuroses of the, 214 cerebrospinal, sensory neuroses of the,
169
cervical, anatomy of the, 148, 149, 152 motor neuroses of the, 148, 151, 154 sensory neuroses of the, 212, 214
centrifugal, 19
centripetal, 19
circumflex, paralysis of the, 161 cranial, apparent origin of the, 115
sensory neuroses of the, 176 degeneration of, 94
neuritic, 96
primary, 94
secondary, 94
toxic, 96
Wallerian, 94 diseases of special, 114 dorsal, anatomy of the, 165
motor neuroses of the, 166 efferent, 19, 20 eighth, anatomy of the, 203
neuroses of the, 203 Nerve, electrical currents in, travelling with
impulses, 22 eleventh, anatomy of the, 138
diseases of the, 140 excito-refiex, 19 extero-ceptive, 17, 19 facial, anatomy of the, 127
diseases of the, 129, 203 fifth, anatomy of the, 188
motor neuroses of the, 126
Nerve, fifth, sensory neuroses of the, 190 fourth, anatomy of the, 114
paralysis of the, 122 glossopharyngeal, anatomy of the, 137
motor neuroses of the, 138
sensory neuroses of the, 211 hyperasmia of, 92 hyperplasia of, 109 hypertrophy of, 109 hypoglossus, anatomy of the, 145
motor neuroses of the, 146 inflammation of, 92, and see Neuritis ingoing, 19 inhibitory, 18, 20
intercostal, sensory neuroses of the, 221
lumbar, anatomy of the, 166 motor neuroses of the, 166 sensory neuroses of the, 223
median, paralysis of the, 162
motor, 18, 20
musculo-spiral, paralysis of the, 161 ninth, anatomy of the, 137
motor neuroses of the, 138
sensory neuroses of the, 211 nuclei of origin of, 91 oculo-motor, anatomy of the, 114
paralysis of the, 119 olfactory, anatomy of the, 176
neuroses of the, 178 optic, anatomy of the, 179
diseases of the, 181 origin of, 91 outgoing, 19 pathic, 19
peripheral, blood-vessels of the, 14
diseases of the, 91 phrenic, anatomy -of the, 147
paralysis of the, 152 pneumogastric, anatomy of the, 138
diseases of the, 140 posterior thoracic, paralysis of the, 160 proprio-ceptive, 17, 19 regeneration of, 97 sacral, anatomy of the, 167
motor neuroses of the, 167
sensory neuroses of the, 231 sciatic, neuralgia of the, 225 secretory, 20 sensory, 19
seventh, anatomy of the, 127
diseases of the, 129, 203 sixth, anatomy of the, 114
paralysis of the, 122 special sense, neuroses of the, 176 spinal, anatomy of the, 148, 149
motor neuroses of the, 148 spinal accessory, anatomy of the, 138
diseases of the, 140 suprascapular, paralysis of the, 161 sympathetic, of the eye, paralysis of
the, 122 syphilis of, 519 tenth, anatomy of the, 138
INDEX.
Nerve, tenth, diseases of the, 140 thermic, 19
third, anatomy of the, 114
paralysis of the, 119 thoracic, anatomy of the, 165
motor neuroses of the, 166
sensory neuroses of the, 221 trigeminus, anatomy of the, 188
motor neuroses of the, 126
sensory neuroses of the, 190 trochlear, anatomy of the, 114
paralysis of the, 122 trophic, 20 tumors of, 109 twelfth, anatomy of the, 145
motor neuroses of the, 146 ulnar, paralysis of the, 163 vagus, anatomy of the, 138
diseases of the, 140 vaso-motor, 20 Nerve-cells, 2, 5, 16 body of the, 7 central, 8
connection of, with nerve-fibres, 1 2
in the cord, 238
Nissl granules, 7
nucleus of the, 7, 8
peripheral, 8
physiology of the, 21
processes of the, 5
reproduction of, does not take place, 33 Nerve fasciculi, 9 Nerve-fibres, 9, 10
central, 12
connection of, with nerve-cells, 12 epicritic, 19 medullated, 11 non-medullated, 11 protopathic, 19 size of, 11 Nervous deafness, 204
diseases, causes of, 24
diagnosis of, 42
functional, 523
methods of examination in, 42
symptoms of; 34
treatment of, 67 exhaustion, 574 system, anatomy of the, 1, 2
arrangement of, 2
blood-vessels of the, 14
chemistry of the, 22
histology of the, 5
hygiene of the, 67
mechanism of the, 21
neuronic architecture of the, 15
pathology of the, 30
peripheral anatomy of the, 91 divisions of the, 3
physiology of the, 19
sympathetic, 16 ganglia of the, 4
syphilis of the, 514
Nervousness, 686, 706 Neuralgia, 173
brachial, 214
cervico-occipital, 214
congestive, of the feet, 229
epileptiform, 190
FothergilPs, 190
hallucinatory, 175
hysterical, 544
in peripheral nerve disease, 98
intercostal, 221
lumbo-abdominal, 223
mammary, 222
Morton's, 230
penile, 223
plantar, 229
red, of the feet, 229
reminiscent, 175
sacral, 231
sciatic, 225
trigeminal, 190 Neuralgic thigh, 223 Neurasthenia, 574
acquired, 580, 586
angiopathic, 589
course, 593
diagnosis, 592
etiology, 574
forms of, 586
gastric, 590
gravis, 590
hydrotherapy in, 663
local, 589
pathogeny, 591
pathology, 591
prEecox, 580, 586
primary, 580, 586
prognosis, 593
spinal irritation, 581, 588
symptoms, 578
traumatic, 586, 600
treatment, 594 Neurasthenic insanity, 697 Neuraxon, 6, 15
cellufugal impulses in the, 15, 17 Neurilemma, 10 Neuritis, 92
ascending, 93
brachial, 159, 214
complicating forms of, 108
degenerative, 94
descending, 93
diffuse, 92
disseminated, 93
interstitial, 92
migrating, 93, 164
multiple, 98, and see Polyneuritis
peri-, 92
retrobulbar, 183
segmental, 93
ulnar, 164 Neurofibromata, 109 Neurofibromatosis, 109
776
INDEX.
Neuroglia, 13 Neuromata, 109
etiology, 112
false, 109, 112
fibro-, 109, 112
multiple, 109, no
plexiform, 112
symptoms, 113
treatment, 113
true, 109 Neurons, 2, 5, 7, 15
associative, 20
central, 20
collaterals of the, 6
diagram showing the arrangement of the, 18
end-brush, 7
end -buds, 7
intercentral, 20
peripheral, 19
terminal arborization, 7 Neuropathic condition or diathesis, 24 Neuro-retinitis, 181 Neuroses, acquired, 523
definition of, 34
degenerative, 523
mixed, 35
occupation, 609
plantar, of mechanical origin, 230
professional, 609
secretory, 35
sexual, 598
traumatic, 600
trophic, 231
vaso-motor, 231 Nictitating spasm, 131 Nightmare, 644 Night terrors, 645 Nissl granules, 7 Nose, neuroses of the, 178 Nuclein, 23 Nucleo-keratin, 23 Numbness, waking, 172 Nyctalopia, 186 Nystagmus, 126
Obsession, 687, 70 r
Obsessive phrenasthenia, 701, 704
psychoneurosis, 704 Occipital lobes, tumors of, 501 Occupation as a factor in the etiology of mental diseases, 675 of nervous diseases, 26 fears, 700
muscular atrophies, 364
neuroses, 609 Ocular hyperesthesia, 185 Oculo-motor nerve, anatomy of the, 114
paralysis of the, 119 (Edema, angioneurotic, 636
blue, of hysteria, 549
circumscribed, 636 ( )hm, definition of, 78
Ohm's law, 77
Olfactory nerve, anatomy of the, 176
neuroses of the, 178 Olivary bodies, functions of the, 418 Operculum, 386 Ophthalmoplegias, 119
partial, in myasthenic paralysis, 123 progressive, 123 Optic atrophy, 184
in locomotor ataxia, 308 chiasm, 180
tumors of the, 502 nerve, anatomy of the, 179
diseases of the, 181 thalami, 390
tumors of the, 502 tract, 180
Optico striate region, tumors of the, 501 Organic defect of development, 672 Organic insanities, 672 Organic psychoses, 750 Orientation, 679
disorders of, 684 Original psychopathic conditions, 697 Orthophoria, definition of, 124 Osteo-arthropathy, pneumogenic, 633 Osteoma of the brain, 511
Pachydermatocele, no, 112 Pachymeningitis, externa, cerebral, 432 spinal, 270
hemorrhagic, spinal, 271
hypertrophic, 271
interna, 271 Pack, the wet, 73 Pain, sense, testing the, 59 Pains, cervico-occipital, 212
finger, 219
forearm, 219
heart-arm, 219
lancinating, of locomotor ataxia, 306 lumbo-abdominal, 223 neck, 214 side, 221
spot, in locomotor ataxia, 307
transferred, diagram of location of, 174 Palate, cord centres for muscles of the, 250 Pallor, 193 Palsy, Bell's 132
bulbar, progressive upper, 123
Erb's 156
facial, 131
lead, 161
night, 172
leg, 168
sacral, 168
shaking, 618 Papillitis, 181
Paradoxical contraction, 56 Para-epilepsy, 528 Paresthesia, 169
aero-, 169, 170
cephalic, 170, 580
INDEX.
777
Paresthesia, definition of, 40 local, 170 trigeminal, 193 Parageusia, 212 Paraleptic seizures, 549 Paralysis, abducens, 122 acoustic, 204 acute ascending, 294
atrophic, 286 agitans, 618
course, 625
diagnosis, 625
duration, 625
etiology, 618
eye movements in, 623
festination in, 622
forms of, 624
handwriting in, 623
muscular weakness in, 622
pathology, 626
prognosis, 626
rigidity in, 622
symptoms, 620
treatment, 627
tremor in, 621 alcoholic, 99
analgesic, with whitlow, 164 arsenical, 99 asthenic bulbar, 350
bulbo-spinal, 350 brachial, 154 bulbar, asthenic, 350
progressive, 347
upper, 123 bulbo-spinal, asthenic, 350 cererbal, of children, 488 compression, 161 definition of, 38 diphtheritic, 99
distribution of, from injury or disease of the motor nerves, 114
divers', 266
Erb's, 156
syphilitic spinal, 281
facial, 131
glosso-labio-laryngeal, 347 hypertonic, 325 general, of the insane, 750 hysterical, 545
in peripheral nerve disease, 97
infantile spinal, 286
Landry's, 294
lead, 161, 168
lingual, 146
oculomotor, 119
of convergence, 122
of the anterior crural nerve, 167
of the cervical muscles, 152
of the ciliary muscle, 121
of the circumflex nerve, 161
of the diaphragm, 152
of the iris, 121
of the levator palpebral muscle, 122
Paralysis of the lumbar nerves, 167 of the median nerve, 162 of the musculo-spiral nerve, 161 of the obturator nerve, 167 of the phrenic nerve, 152 of the posterior thoracic nerve, 160 of the sacral nerves, 168 of the spinal accessory nerve, 145 of the suprascapular nerve, 161 of the sympathetic nerves of the eye, 122
of the ulnar nerve, 103
progressive bulbar, 347
spastic, spinal, 325 hereditary, 327, 363
subacute spinal, 294
testing for, 48 Paramnesia, 685 Paramyotonia, 565
ataxic, 566
congenital, 565
symptomatic, 566 Paranoia, 732
abortive, 687, 698
acute hallucinatory, 733
cases of, 734
etiology, 732
forms, 733
original, 733
pathological anatomy, 734
persecutoria, 732
prognosis, 734
secondary, 734
symptoms, 732
treatment, 734 Paranoid type, querulent, 706 Paraphasia, 426 Paraplegia, definition of, 38
hereditary ataxic, 331 spastic, 331, 336
hysterical, 545
progressive spastic, 463
senile, 293
spastic cerebral, 463
tetanoid, 326 Paresis, definition of, 38
general, 750 Paridrosis, definition of, 41 Parietal lobe, tumors of the, 500 Parkinson's disease, 618 Parosmia, 179 Patella-tendon reflex, 52 Pathology, general, of the nervous system, 30 Pavor nocturnus, 645 Pedo-dynamometer, 48 Peduncles, cerebral, tumors of the, 504 Percept, 678 Perception, 678
disorders of, 683 Perineuritis, 92 Perineurium, 10
Peripachymeningitis, tuberculous, definition of, 274
778
INDEX.
Peripheral nerves, diseases of the, 91 muscular atrophy in, 97 pathology, 92 symptoms, 97
Pernicious anaemia, combined spinal scle- roses of, 328
Pharynx, cord centres for muscles of the, 250
Philias, 700
Photographers' cramp, 615 Phrenasthenia, 552, 575, 697, 702
diagnosis, 703
doubting, 699
etiology, 698
hypochondriasis and, 702
hysteria and, 703
impulsive, 701
obsessive, 701, 704
phobic, 699
prognosis, 703
symptoms, 699
treatment, 704 Phrenic nerve, anatomy of the, 147
paralysis of the, 152 Physical symptoms of insanity, 690 Physiology, general, of the nervous system,
Pia mater, cerebral, 401
inflammation of the, 433
spinal, 234
inflammation of the, 272 Pianists' cramp, 614 Pituitary body, tumors of the, 502 Plantar neuralgia, 229
neuroses of mechanical origin, 230 Plexus, brachial, anatomy of the, 152
sensory neuroses of the, 214 Plexuses, vascular, of the spinal cord, 256 Pneumogastric nerve, anatomy of the, 138
diseases of the, 140 Pneumogenic osteo-arthropathy, 633 Poison, as a factor in the etiology of nervous
diseases, 27 Policeman's disease, 230 Polioencephalitis, hemorrhagic, 455
inferior, 288, 456
superior, 456 Poliomyelitis, acute anterior, 286
chronic anterior, 292, 346 Polyaesthesia, definition of, 62 Polyneuritic psychosis, case of, 742 Polyneuritis, 98
acute pernicious, 104
alcoholic, 101
complicating forms, 108
diagnosis, 106
diphtheritic neuritis, 102
endemic type, 103
epidemic type, 103
etiology, 98
forms of, 98
malarial, 104
pathology, 105
prodromata, 100
Polyneuritis, prognosis, 107
pseudo-tabetic type, 103
sensory-motor type of, 99
sensory type of, 103
symptoms, 102
treatment, 108 Pons Varolii, functions of the, 417
hemorrhage in the, 474
nuclei of the, 393
thrombosis and softening of the, 486
tumors of the, 502 Ponto-cerebellar angle, tumors of the, 505 Porencephalus, 490 Porencephaly, 432
Posterior thoracic nerve, paralysis of the, 160
Post-syphilitic degenerative processes, 519 Potential, difference in, defined, 76 Power of reaction, disorders of, 686 Prsedormitium, disorders of the, 655 Prefrontal area, tumors of the, 499 Pressure activity, 686 Pressure sense, testing the, 58 Primary dementia, 738 Professional neuroses, 609 Prognosis, general, in insanity, 695 Projection system of the brain, 397 Prosencephalon, 3 Prosopalgia, 190 Protagon, 23
Protopathic fibres, 19, 20
sensation, 20
sensibility, 19, 20
system, 19, 20 Protoplasm of the nerve-cell, 7 Pseudo-muscular hypertrophy, 357 Pseudo-paresis, alcoholic, 741, 755
syphilitic, 754 Pseudo-tabes, 103 Psychasthenia, 552, 697
hysteria and, 552 Psychiatry, 677
Psychic analysis, method of, 87 Psychical epileptic equivalent, 524. 527 Psychic tics, 687, 702 Psychogenetic causes of insanity, 675 Psycholeptic seizures, 549 Psychology, general, 677 Psychoneuroses, 35, 671, 697 Psychoneurosis, obsessive, 704
spinal, 588
traumatic, 586 Psychopathic conditions, original, 697
wards, annual census of patients, 673
Psychoses, 35
alcoholic, 740 arteriosclerotic, 761 autotoxic, 738 exhaustion, 738, 741 infection, 738 major, 750 minor, 697
INDEX.
779
Psychoses of cerebral hemorrhage, 761 softening, 761
of epilepsy, 757, 760
of organic brain disease, 757
of senility, 757
of trauma, 757, 761
organic, 750
toxic, 738
traumatic, 761 Psychosis, agitated, 720
anxiety, 721
definition of, 34
hypochondriacal, 720
Korsakoff's, 101, 741
manic-depressive, 727
multiple neuritic, 741
presenile delusional, 720
sexual, 598
suicidal, 720
traumatic, 757, 761 Psychotherapy, 87
by emotional shock, 89
irregular, 89
philosophic, 89 Ptosis, 120
waking, 122 Puerperal insanity, 740 Pupil, Argyll-Robertson, 55
hemiopic reaction of the, 187 in locomotor ataxia, 308
Hutchinson, 473 Purkinje's cells, 394
Rabies, 571
paralytic, 573 Race, as a factor in the etiology of mental
diseases, 674 Radiant energy, 86 Railway spine, 600 Ranvier, nodes of, 10 Raynaud's disease, 231 Reaction at a distance in nerve degener- ation, 95
electrical, diagnostic table of, 86
of degeneration, 83
time, 22 Reasoning, 679
Red nuclei of the brain, 393, 416 Re-education, method, 88 Re-enforcement of the knee-jerk, 54 Reflex action, 22, 38 Reflex causes of nervous diseases, 29 Reflexes, 38 deep, 52
examination of the, 50
skin, 50
superficial, 50
tendon, 52 Reil, island of, 386 Reintegration, 87 Remak, fibres of, 11 Representation, bilateral, 410 Rest treatment, 665
Rest treatment, partial, 668
schedule for, 667 Retardation of thought, 684 Retina, anatomy of the, 179 Retinal hyperesthesia, 185 Retrobulbar neuritis, 183 Rhachischisis, posterior, 261 Rheostat, definition of, 78 Rhinencephalon, 176 Rhombencephalon, 3
Rhythmical spasm of ocular muscles, 126 R L S people, 146
Rolando, gelatinous substance of, 237 Rumpf's symptom, 582
Sacral nerves, anatomy of the, 167 motor neuroses of the, 167 sensory neuroses of the, 231
St. Vitus' dance, 555
Salivation, 193
Sarcoma of the brain, 510
Scavenger cells of Lewis, 14
Sclerosis, arterial, 71
Schultze, comma of, 245
Schwann, sheath of, 10
Sciatica, 225
Scleroses, combined, of the spinal cord, 328
lateral spinal, 325 amyotrophic, 352
multiple, 458
aborted types of, 462
of nervous tissue, 31, 32
of the spinal cord, 297
Putnam's type of combined spinal, 328 Senile dementia, 757
deterioration, case of, 759
insanity, 757
paraplegia, 293 Senility, nerve degeneration in, 109
psychoses of, 757 Sensations, 678
cutaneous, methods of testing, 57
delayed, 40, 62
disorders of, examination for, 56
double, 62
general, 678 nerves of, 19
referred, 40
reflex, 40
special, 678 nerves of, 19
time for perceiving, 62
transferred, 40 Seizures, psychical, 760
psycholeptic, 549 Senses, special, centres for the, 412 neuroses of the nerves of, 1 76 Sensori-motor area in the brain, 408 Sensory tracts in the brain, 400 Sentiments, 680 Sewing-spasm, 615
Sex as a factor in the etiology of mental diseases, 673
780
INDEX.
Sex as a factor in the etiology of nervous
diseases, 26 Sexual neuroses and psychoses, 598 Shaking palsy, 618 Shingles, 222
Shock as a factor in the etiology of nervous
diseases, 27 Shoulder and upper extremity, cord centres
for muscles of the, 251 Shower baths in the treatment of nervous
diseases, 71 Side pains, 221 Simple dementia praecox, 710 Singultus, 151 Sinuses, cerebral, 406
Skin, distribution of the sensory nerves of the, 60, 61
reflexes, 50 Skull, dimensions and shape of the, 46
landmarks of the, indicating the under- lying parts of the brain, 656 Sleep, 639, 688
accidents of, 655
disorders of, 639
the praedormitium, 655
disturbances of, 643
morbid, from organic disease, 652
morbidly deep, 651
normal, 640
paroxysmal, 651
perversions of, 643
physiology of, 639 Sleep-drunkenness, 645 Sleep-epilepsy, 651 Sleeping-sickness, 653 Smell, centres for, 414
disorders of, 178 Smiths' spasm, 615 Softening of the brain, 482, 487 Somatochromes, 8 Somnambulism, 646 Somnolence, 688 Sonmolentia, 645 Spasm, facial, 129
idiopathic muscular, 50
lingual, 146
nictitating, 131
nodding, 151
ocular, 126
of levator palpebrae, 126
oscillating, 151
salaam, 151
smiths', 615
tonic, 555 facial, 131 of neck, 151
twitching, 555
winking, 131 Spasmodic tics, 560 Spasmus nutans, 151 Spastic spinal paralysis, 325
hereditary, 327 Special associations, centres for, 414 Special senses, centres for, 412
Spermatorrhoea, 598
hydrotherapy in, 664 Spider cells, 13 Spina bifida, 261
Spinal accessory nerve, anatomy of the, 138
diseases of the, 140 Spinal cord, acute softening of the, 274
abscess of the, 274
amyotrophic lateral sclerosis, 352
anaemia of the, 269
anatomy of the, 234
arteries of the, 256, 258
asymmetry of the, 264
automatic centres, 247
blood-supply of the, 255
cancer of the, 367
cavities in the, 369
cells of the, 238
central arteries, 259 canal, 236 double, 264
centres in the, 247
columns of the, 235, 239, 240, 244, 246
combined scleroses of the, 328 composition of the, 235 degeneration of the, primary, 298
secondary, 299
short tracts of, 300 diseases of the, 260 double, 264 fibres, 239 fissures, 235 glioma of the, 372 gray matter of the, 235, 238 gumma of the, 367 hemorrhage in the, 264 hemorrhagic cavities in the, 369 horns of the, 236 hyperaemia of the, 268 inflammation of the, 274, and see
Myelitis levels of the reflexes in the, 52 localization in the, 248 lumbar puncture of the, 670 malformations of the, 261 meninges of the, 234
diseases of the, 270
inflammation of the, 270 myelitic cavities in the, 369 nerve-roots, 234, 245 paracentesis of the dura mater, 670 physiology of the, 239, 246 plexuses of the, 256 pyramidal tracts, 241, 246 relations of the different parts of the,
to the peripheral nerves, brain, and
each other, 245 root arteries of the, 256
ganglia, 235 sclerosis of the, 297
amyotrophic lateral, 352
combined, 328
lateral. 32^
INDEX.
781
Spinal cord, sclerosis of the, posterior, 301 primary, 298
Putnam's type of combined, 328 secondary, 299
spindle-shaped cells, 247
splitting of the, 264
syphilis of the, 518
system diseases of the, 297
topography of the, 248
tracts of the, 241
tumors of the, 366
veins of the, 258
white matter of the, 239 Spinal dura, syphilis of the, 271 Spinal irritation, 581, 588 Spinal nerves, motor neuroses of the, 148 Spine, railway, 600 Spongioblasts of Cajal, 9 Spotted fever, 435 Staggers, the, 267 Stammering, 146 Static electrical batteries, 78 Status epilepticus, 526
vertiginosus, 207 Stellwag's symptom, 604 Stereognosis, 40 Stereognostic sense, 62 Stereotypy, 686 Stigmata of degeneration, 44 Stupor, 688
Stuttering, 146, 151, 561 Subconsciousness, 681 Substantia nigra, 393 Subthalamus, 393 Suggestibility, 686 Suggestion, 87
Suprascapular nerve, paralysis of the, 161 Sydenham, chorea of, 555
diagnosis, 559
duration, 558
etiology, 556
forms, 558
pathology, 558
prognosis, 559
relapses, 558
symptoms, 557
treatment, 559 Smybolia, 40
Sympathetic nervous system, 16
ganglia of the, 4 Symptoms, cerebral of nervous disease, 35 compression, 423 general, of cerebral disease, 423 general, of mental disease, 683 general, of nervous disease, 34 general, of peripheral nerve disease, 97 irritation, 423
mental, of nervous disease, 35 motor, of nervous disease, 35
examination for, 48 secretory, of nervous disease, 41 sensory, of nervous disease, 39
examination for, 56
Symptoms, trophic, of nervous disease, 41 vasomotor, of nervous disease, 41
Syncope, 688
laryngeal, 210
local, of the extremities, 232 Syphilis, cerebrospinal, 518
hereditary, 519
of the brain, 514, 517
of the dura, 271
of the nervous system, 514
degenerative processes following, 519 Syphilitic pseudoparesis, 754 Syphiloma of the brain, 507 Syringomyelia, 369
neuritic type of, 164 Syringomyelocele, 262
Tabes dorsalis, 301, and See Locomotor
Ataxia Tabes, pseudo-, 103 Tache cerebrate, 434 Tachycardia, in Graves' disease, 603 Tactile sense, testing the, 57 Tarsalgia, 230 Taste, centres for, 414
disorders of, 211
fibres of the facial nerve, 128
of the glossopharyngeal nerve, 137
hysterical disturbances of, 543 Telegraphers' cramp, 615 Telencephalon, 3, 380 Temperature sense, testing the, 59 Temporal lobes, tumors of the, 501 Tendon reflexes, 52 Tension, electrical, defined, 77 Terminal buttons, 7 Tetanilla, 567 Tetanoid paraplegia, 326 Tetanus, 566 Tetany, 567
asthenic, 569
epidemic, 569
infantile, 571
parathyroid, 569
reflex, 569
toxic, 569 Thalamus opticus, 415 Therapeutic talk, method of, 88 Therapeutics, neurological, 662 Therio-mimicry, 542 Thermo-anassthesia, definition of, 39 Thermoneuroses, 35 Thigh, neuralgic, 223 Thomsen's disease, 564 Thoracic nerves, anatomy of the, 165
motor neuroses of the, 166
sensory neuroses of the, 221 Thrombosis, cerebral, 482
of medulla, 485
of pons, 486
special forms of, 485 Thyroid insanity, 738 Tic, convulsive, 37
782
INDEX.
Tic, de pensee, 561
douloureux, 190
endemic, 560
general, 560
generalized, 561
habit, 560, 561
hysterical, 560
local, 560, 561
mental, 560
mimic, 129
spasmodic, 560 Tinnitus aurium, 205
cerebri, 205 Tongue, cord centres for muscles of the, 250
hemiatrophy of the, 147
paralysis of the, 146
spasms of the, 146 Topography, cranio-cerebral, 656 Torticollis, 141, 151
Toxic factors in the etiology of nervous diseases, 27 psychoses, 738 Tracts of the spinal cord, 241 Trance, 651
hysterical, 550 somnambulistic, 648 Transferred pains, diagram of location of, 174
Transitory mania, 760
Trauma, as a factor in the etiology of
nervous diseases, 27 Traumatic hysteria, 600
neuroses, 600
psychoses, 600, 761 Treatment of nervous diseases, 67, 662 Tremor, 36
fibrillary, 36, 50
hysterical, 547
intention, 36
of paralysis agitans, 621
passive, 36
testing for, 49 Trigeminus nerve, anatomy of the, 188 motor neuroses of the, 126 sensory neuroses of the, 190 Trismus, 126
Trochlear nerve, anatomy of the, 114
paralysis of the, 122 Trophic disorders, 629 Trophoneuroses, 35
definition of, 41
peripheral, 231 Tubercula dolorosa, 109 Tuberculosis, meningeal, 439
of the brain, 506 Tumors, cerebral, 495
local symptoms of, 498
of nerves, 109
spinal, 366 'Tween-brain, 380
Ulnar nerve, paralysis of the, 163
neuritis, symmetrical spontaneous, 164
Unconsciousness, 688
Upper extremity, cord centres for muscles of the, 251
Urban life, as a factor in the etiology of mental diseases, 675
Vagus nerve, anatomy of the, 138
diseases of, 140 Vasomotor disorders, 629
neuroses, peripheral, 231 Veins, cerebral, 406 Verbigeration, 686 Vermis, of the cerebellum, 393 Verrucktheit, die, 732 Vertigo, 206
arteriosclerotic, 209
auditory, 207
bilious, 208
laryngeal, 210
lithaemic, 208
mechanical, 209
neurotic, 208
ocular, 208
paralyzing, 210
psychical, 208
senile, 209
stomachal, 208
stumbling, 208 Violinists' cramp, 614 Vision, centres for, 412
double, 119
hysterical disturbances of, 543 Volition, 680
Volitional function, disorders of, 686
Volt, definition of, 78
von Graefe's symptom, 604
Wahnsinn, 732
Wallerian degeneration, 94
Watchmakers' cramp, 615
Water, use of, in the treatment of nervous
diseases, 71 Watt, definition of, 78 Weir-Mitchell treatment, 665 Werdnig-Hoffmann type of muscular
atrophy, 363 Wernicke's hemiopic pupillary reaction, 187
sensory aphasia, 428 Wet-brain, 444
Whitlow, analgesic paralysis with, 164 Will, 680
Winking spasm, 131 Word blindness, 425
deafness, 425 Work as a factor in the etiology of nervous
diseases, 26 Wristdrop, 161 Writers' cramp, 609
treatment, 616 Wryneck, 141
treatment, 144
X-ray tests, 65, 86
i
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