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Full text of "Handbook of Severe Disability: A Text for Rehabilitation Counselors, Other Vocational Practitioners, and Allied Health Professionals"










SEV 
DISA 









Edited by 

WALTER C. STOLOV, M.D. 

MICHAEL R* GLOWERS, Ph.D. 



U.S. Department of Education 
Rehabilitation Services Administration 




M.C. MIGEL MEMORIAL LIBRARY 
American Foundation for the Blind 

15 West 16th Street, New York, New York 
10011 



Digitized by the Internet Archive 

in 2011 with funding from 

Lyrasis Members and Sloan Foundation 



http://www.archive.org/details/handbookofsevereOOwalt 



HANDBOOK OF 
SEVERE DISABILITY 



For Sale by the Superintendent of Documents, U.S. Government Printing Office 
Washington, DC., 20402 



HANDBOOK OF 
SEVERE DISABILITY 

A Text for Rehabilitation Counselors, 
Other Vocational Practitioners, and 
Allied Health Professionals 



Edited by 

WALTER C. STOLOV, M.D. 

Professor 

MICHAEL R. CLOWERS, PH.D. 

Director, Vocational Unit 

Department of Rehabilitation Medicine 
University of Washington 
A Rehabilitation Research and Training Center 
Of the National Institute of Handicapped Research 

Assisted by 

Sandra R. Blair, Technical Editor 

Phyllis Wood, Illustrator 



1981 

U.S. Department of Education 

Rehabilitation Services Administration 






Prepared and supported, in part, by the 
Rehabilitation Services Administration, 
U.S. Department of Education 
Contract No. 105-76-4115 
and the University of Washington 
Graduate School Research Fund 

© 1981 by the Department of Rehabilitation Medicine, 
University of Washington 

Library of Congress Cataloging in Publication Data 

Main entry under title: 

Handbook of severe disability. 

Includes index. 

1. Physically handicapped— Rehabilitation. 
2. Rehabilitation. I. Stolov, Walter C, 1928- 
II. Clowers, Michael R., 1942-1979 [DNLM: 
1. Counseling. 2. Handicapped. 3. Rehabilitation, Vocational. 
HD7255 H2346] 
RD797.H36 616 80-607924 

For sale by the Superintendent of Documents, 
U.S. Government Printing Office 
Washington, D.C. 20402 
Stock No. 017-090-00054-2 



To those severely disabled by disease and injury, 

in the hope that their burden may be eased 

and their objectives achieved, 

and to 

Michael R. C lowers, PkD., 

who, in his short professional life, 

achieved a level of excellence 

in the service of the handicapped 

that is a model for all of us. 



CONTENTS 



Contributors xi 

Preface xv 

1 Comprehensive Rehabilitation: Evaluation and Treatment 1 

Walter C. Stolov, M.D. 

2 Psychosocial Adjustment to Chronic Disease and Disability 13 
Gary T. Athehtan, Ph.D. 

3 Significant Body Systems 19 

Nervous System 
Musculoskeletal System 
Cardiovascular System 
Pulmonary System 
Visual System 
Auditory System 
Joel DeLisa, M.D. and Walter C. Stolov, M.D. 

4 Disability Consequences of Bed Rest 55 
Paul J. Corcoran, M.D. 

5 Spinal Cord Injury 65 
William H. Donovan, M.D. 

6 Neuromuscular Diseases 83 
Paul J. Corcoran, M.D. 

7 Peripheral Neuropathies 101 
Joseph Goodgold, M.D. 

8 Multiple Sclerosis 1 1 1 
George H Kraft, M.D. 



9 Stroke and Cerebral Trauma: Medical Aspects 119 

Thomas P. Anderson, M.D. 

10 Stroke and Cerebral Trauma: Psychosocial and Vocational Aspects 127 
RoyS. Fowler, Jr., Ph.D. 

11 Cerebral Palsy 137 

Jessie K.M. Easton, M.D. and Daniel Halpern, M.D. 

12 Epilepsy 155 

Arthur A. Ward, Jr., M.D., Robert T. Fraser, Ph.D., and Allan S. Troupin, M.D. 

13 Amputation 169 
Lawrence W. Friedmann, M.D. 

14 Rheumatic Diseases 189 
John J. Nicholas, M.D. 

15 Organic Musculoskeletal Back Disorders 205 
Rene Cailliet, M.D. and Loren A. Helberg, Ph.D. 

16 Chronic Pain 219 
Wilbert E. Fordyce, Ph.D. 

17 Alcoholism 231 

Vemelle Fox, M.D., James P. Conway, M.S., andjeri Schweigler, M.S. 

18 Drug Abuse 241 
R. Raymond Knowles, MB. 

19 Mental Illness 253 

H. Richard Lamb, M.D. and Ceale Mackota 

20 Mental Retardation 265 
Andrew S. Halpern, Ph.D. 

21 Sexual Adjustment to Chronic Disease and Disability 279 
Theodore M. Cole, M.D. and Sandra S. Cole, B.A. 

22 Cardiovascular Diseases 289 
Henry L. Brammell, M.D. 

23 Pulmonary Dysfunction 309 
Ben V. Branscomb, M.D. 



vi n 



24 Diabetes Mellitus 321 
Leona V. Miller, M.D. 

25 End-Stage Renal Disease 329 
Alvin E. Parrish, M.D. 

26 Hemophilia 341 
Shelby L. Dietrich, M.D. 

27 Sickle Cell Disease 349 
Cage S. Johnson, M. D. 

28 Cancers 363 

John E. Healey.Jr., M.D. and Jack M. Zislis, M.D. 

29 Blindness and Visual Impairments 377 
Richard E. Hoover, M.D. and C. Warren Bledsoe 

30 Hearing Impairments and Deafness 395 
Jerome D. Schein, Ph.D. 

31 Burns 409 
Edward J. O'Shaughnessy, M.D. 

32 Plastic and Reconstructive Surgerv 419 
Robert V. DeVito, M.D. 

Glossary 429 

Index 437 



CONTRIBUTORS 



THOMAS P. ANDERSON, M.D. 
Professor 

Department of Physical Medicine and Rehabilitation 
University of Minnesota 

GARYT. ATHELSTAN, Ph.D. 
Professor and Director 
Counseling Psychology Service 

Department of Physical Medicine and Rehabilitation 
University of Minnesota 

C. WARREN BLEDSOE 

Principal Consultant on Blindness (retired) 
Rehabilitation Services Administration 
Department of Health, Education and Welfare 

HENRY L. BRAMMELL, M.D. 
Associate Professor of Medicine and Physical Medicine 

and Rehabilitation 
Project Director 

Rehabilitation Research and Training Center 
Department of Rehabilitation Medicine 
University of Colorado 

BEN V. BRANSCOMB, M.D. 
Professor of Medicine 
University of Alabama in Birmingham 
School of Medicine 

RENE CALLIET, M.D. 
Professor and Chairman 
Department of Rehabilitation Medicine 
University of Southern California 
School of Medicine 

SANDRA S. COLE, B.A. 
Health Educator 
Department of Psychiatry and 

Department of Physical Medicine and Rehabilitation 
University of Michigan Medical School 



THEODORE M. COLE, M.D. 
Professor and Chairman 

Department of Physical Medicine and Rehabilitation 
University of Michigan Medical School 

JAMES P. CONWAY, M.S. 

Training Coordinator, Alcoholism Service 
Long Beach General Hospital 
Long Beach, CA 

PAULJ. CORCORAN, M.D. 
Professor and Director 
Rehabilitation Research & Training Center 
Department of Phvsical Medicine and Rehabilitation 
Tufts University School of Medicine 

JOEL DeLISA, M.D. 
Assistant Professor 

Department of Rehabilitation Medicine 
University of Washington 

ROBERT V. DeVITO, M.D. 
Clinical Professor of Surgery 
Head, Division of Plastic Surgerv 
University of Washington 

SHELBY L. DIETRICH, M.D. 
Director 

Hemophilia Rehabilitation Center 
Los Angeles Orthopaedic Hospital 

WILLIAM H. DONOVAN, M.D. 
Associate Professor 

Texas Institute for Rehabilitation and Research 
Bavlor University 

JESSIE K.M. EASTON, M.D. 
Assisant Professor 

Department of Phvsical Medicine and Rehabilitation 
Universitv of Minnesota 



WILBERT E. FORDYCE, Ph.D. 

Professor and Director of Research 
Rehabilitation Research and Training Center 
Department of Rehabilitation Medicine 
University of Washington 

ROYS. FOWLER, JR., Ph.D. 
Associate Professor 

Department of Rehabilitation Medicine 
University of Washington 

VERNELLE FOX, M.D. 
Chief, Alcoholism Service 
Long Beach General Hospital 
Long Beach, CA 

ROBERT T. FRASER, Ph.D. 
Research Associate 

Department of Rehabilitation Medicine and 
Department of Neurological Surgery 
University of Washington 

LAWRENCE W. FRIEDMANN, M.D. 
Chairman 

Department of Rehabilitation Medicine 
Nassau County Medical Center 
Professor of Rehabilitation Medicine 
State University of New York at Stonv Brook 

JOSEPH GOODGOLD, M.D. 

Professor and Director of Research and Training 
Department of Rehabilitation Medicine 
New York University Medical Center 



Ph.D. 



ANDREW S. HALPERN 
Director 
Rehabilitation Research 

Mental Retardation 
University of Oregon 



M.D. 



and Training Center in 



DANIEL HALPERN, 
Professor 

Department of Physical Medicine and Rehabilitation 
University of Minnesota 

JOHN E. HEALEY,Jr., M.D. 

Professor of Oncology and Orthopaedics and 

Rehabilitation 
Universtiy of Miami School of Medicine and 
Deputy Director 

Division of Cancer Control and Rehabilitation 
Comprehensive Cancer Center 
for the State of Florida 



LOREN A. HELBERG, Ph.D. 
Associate Clinical Professor 
Department of Rehabilitation Medicine 
University of Southern California 
School of Medicine 

RICHARD E. HOOVER, M.D. 
Chief of Ophthalmology 
Greater Baltimore Medical Center 
Assistant Professor of Ophthalmology 
The John Hopkins University School of Medicine 

CAGE S.JOHNSON, M.D. 
Assistant Professor of Medicine 
University of Southern California 
School of Medicine 

R. RAYMOND KNOWLES, M.B. 
Clinical Professor 

Department of Mental Health and Behavioral Science 
St. Louis University 

GEORGE H. KRAFT, M.D. 
Professor 

Department of Rehabilitation Medicine 
University of Washington 

H. RICHARD LAMB, M.D. 
Associate Professor 
Department of Psychiatry 
University of Southern California 
School of Medicine 

CECILE MACKOTA 
Director 

Vocational Rehabilitation Services 
San Mateo Countv (California) 
Department of Public Health and Welfare 

LEONA V. MILLER, M.D. 
Director 

Diabetes Institute of Southern California 
Los Angeles, CA 

JOHNJ. NICHOLAS, M.D. 

Associate Professor of Clinical Medicine 

and Clinical Orthopedics 
Division of Rehabilitation Medicine 
Presbyterian University Hospital 
Pittsburgh, PA 

EDWARD J. O'SHAUGHNESSY, M.D. 
Assistant Professor 

Department of Rehabilitation Medicine 
University of Washington 



ALVIN E. PARRISH, M.D. 

Professor of Medicine and Director 

Division of Renal Diseases 

The George Washington University Medical Center 

JEROME D. SCHEIN, Ph.D. 
Professor and Director 
Deafness Rehabilitation 
Deafness Research & Training Center 
New York University 

JERI SCHWEIGLER, M.S. 

Training Coordinator, Alcoholism Service 
Long Beach General Hospital 
Long Beach, CA 

WALTER C. STOLOV, M.D. 

Professor and Director of Training 
Rehabilitation Research & Training Center 
Department of Rehabilitation Medicine 
University of Washington 

ALLAN S. TROUPIN, M.D. 
Assistant Professor 
Director, Seizure Clinic 
Department of Neurological Surgery 
University of Washington 

ARTHUR A. WARD, JR., M.D. 
Professor and Chairman 
Department of Neurological Surgery 
University of Washington 

JACK M. ZISLIS, M.D. 
Assistant Professor 

Assistant Director, Rehabilitation Center 
Department of Orthopaedics and Rehabilitation 
University of Miami School of Medicine 



PREFACE 



The Rehabilitation Act of 1973, with its emphasis on 
the severely disabled, found many State vocational 
rehabilitation agencies ill prepared. Not enough of the 
counseling staff had the knowledge and the skills to 
deal with the vocational rehabilitation of the severely 
disabled. As a result, a number of training courses 
were developed throughout the country to equip the 
counselors with the necessary basic information that 
might allow them to successfully deal with their 
severely disabled clients. 

In the State of Washington, the Division of 
Vocational Rehabilitation, through its chief medical 
social work consultant Mr. Thayne Wright, asked the 
University of Washington Rehabilitation Research 
and Training Center to develop and offer a course to 
upgrade the education of the Washington counselors in 
the area of severe disability. The first 5-day course was 
held May 20-24, 1974. Subsequently, four additional 
courses were held, which were not only presented to 
every counselor within the State of Washington, but also 
to counselors from the States of Alaska, Idaho, and 
Oregon. 

As chairmen of these courses, we came to 
understand more completely the needs of the counselor 
in the area of severe disability. Preparation of a 
training manual for the courses became the initial 
framework for this text. 

When the Rehabilitation Services Administration 
indicated interest in developing a new book on severe 
disability oriented toward the vocational rehabilitation 
counselor, we expressed interest in this area and 
subsequently accepted the offer to edit such a text. We 
viewed the project as a natural extension and 
continuation of our work in assisting with the training of 
rehabilitation counselors. 

Following acceptance of the contract, an advisory 
committee was established and met with RSA rep- 
resentatives in Washington, D.C. on December 6, 1976. 
Persons serving on the advisory committee were: 
Raymond Ehrle Ph.D., President, American 
Rehabilitation Counseling Association; Leslie McCoy, 
M.D., Chief Medical Consultant, Division of 
Vocational Rehabilitation, West Virginia; William 



Graves, Ed.D., President, National Council on 
Rehabilitation Education; Ann Crumpton, Past 
President, National Rehabilitation Counseling 
Association; Paul Corcoran, M.D., Professor and 
Chairman, Department of Physical Medicine and 
Rehabilitation, Tufts University; Ruth Savlowsky, 
Rehabilitation Group, Arlington, Virginia; Ekkehard 
Petring, Ph.D., Director, Region X Rehabilitation 
Counselor Education Program; and Guy Hubbard, 
Director, Division of Vocational Rehabilitation, 
North Carolina. Representatives of the Rehabilitation 
Services Administration at the meeting included: 
Harold Shay, Director, Division of Manpower 
Development; Sterling Brinkley, M.D., Chief Medical 
Officer; Joseph Fenton, Ed.D., Chief, Special 
Centers; Gregory March, Vocational Rehabilitation 
Consultant; Frederick Sachs, Assistant Commissioner 
for Program Management; William Usdane, Ph.D., 
Director, Bureau of Demonstrations and Manpower 
Development; Katherine Lloyd, M.D., Medical 
Officer; Elizabeth Bush, Manpower Development 
Specialist; and Richard Melia, Vocational 
Rehabilitation Program Specialist. 

At the advisory committee meeting, decisions were 
made on the topics to be covered in the text, the 
authors to be invited to participate, the general format 
of the chapters (see p. 8), and the overall character 
and orientation of the text. It was decided to orient the 
book toward the first-year graduate student in rehabil- 
itation counseling as a text for his course on the medical 
aspects of severe disability, and to the vocational 
counselor in the field as a reference. 1 1 became clear as the 
text evolved that it would also have value for others within 
the entire field of vocational rehabilitation, as well as for 
manv allied health professionals and medical consul- 
tants. 

As we invited authors to contribute to the project, 
we felt ourselves quite fortunate. All authors contacted 
entered into the project with enthusiasm, were 
reasonably prompt with their submissions, and 
adhered to requests and efforts to ensure that the text 
would be a uniform volume, rather than a collection of 
disjointed unrelated contributions. We are most grateful 



xv 



to all the authors. 

We are also thankful for the fine technical editing by 
Sandra Blair. She was instrumental in achieving 
uniformity of style and presentation format, made 
many author contacts, monitored the production of the 
figures, and prepared the glossary. Editorial assistant 
Cheryl Dineen performed exceptional service in our 
bibliographical search of the literature for those key texts 
and articles added to the annotated bibliographv at the 
end of each chapter. In addition, we are thankful to her 
and to Wilma Dlouhy for typing and retyping manu- 
scripts during the review process. 

Phyllis Wood, of the University of Washington Health 
Sciences Illustration Studio, produced the line illustra- 
tions for the text. We are most grateful for her fine work 
and her unfailing care and patience in drawing and 
revising the illustrations to ensure clarity and accuracy. 

During our review of inital drafts, second drafts, 
and, in some cases, third drafts, we consulted 
numerous individuals within the University of 
Washington, the Northwest Region, and the country 
at large. We wish to express our appreciation to the 
following reviewers for their assistance in enhancing 
the final text: Marjorie Anderson, Ph.D., University 
of Washington; Sandra Belcher, Ph.D., University of 
Washington; David Beukelman, Ph.D., University of 
Washington; Stirling B. Brinkley, M.D., 
Rehabilitation Services Administration; JoAnn 
Brockway, Ph.D., University of Washington; Alan 
Dralle, CPO, University of Washington; Joseph 
Fenton, Ed.D., Rehabilitation Services 
Administration; William Graves, Ed. D., Mississippi 
State University; John L. Hampson, M.D., University of 
Washington; Noreen R. Haupt, State of Wisconsin 
Department of Health and Social Services; David Hooks, 
M.Ed., University of Washington; Kathleen Lloyd, M.D., 
Rehabilitation Services Administration; Steve Lund, 
C.P.O., University of Washington; Leslie McCoy, M.D., 
State Board of Vocational Education, West Virginia; Greg 
March, Rehabilitation Services Administration; Willman 
Massie, Rehabilitation Services Administration; 
Ekkhard Petring, Ph.D., Seattle University; Donald 
E. Rawe, Rehabilitation Services Administration; 
Frederick Sachs, Rehabilitation Services 
Administration; Martin Spickler, Rehabilitation 
Services Administration; J. Paul Thomas, 
Rehabilitation Services Administration; Susan 
Tollefson, R.N., University of Washington; Ann B. 
Trotter, Ph.D., University of Wisconsin-Milwaukee: 
William Usdane, Ph.D., Rehabilitation Services 
Administration; Johan Verhulst, M.D., University of 
Washington; David Wenner, Rehabilitation Services 
Administration; Boyce R. Williams, Rehabilitauon 
Services Administration; and Robert J. Winn, Jr., 
Rehabilitation Services Administration. 

We hope the counselor will find within these pages 
the information that may allow his clients to achieve 



and successfully maintain a desired employment. 



Walter C. Stolov, M.D. 
Michael R. Glowers, Ph.D. 



Addendum 



Following completion of the text but before final 
publication, my co-editor, Michael R. Clowers, Ph.D., 
passed away on October 22, 1979 after an extended 
illness. Both the professional and the patient-client 
rehabilitation communities lost a trusted colleague, 
advocate, and friend. Marks of his insistence that 
excellence prevail in the production of this text can be 
seen throughout its pages. It is hoped that this last major 
project of Dr. Clowers can serve as a living monument to 
his memory. 

Walter C. Stolov, M.D. 



1 COMPREHENSIVE REHABILITATION: 
EVALUATION AND TREATMENT 

Walter C. Stolov, M.D. 



DISABILITY 

Definition 

Consider the following scenario: 
A colony of 100 Earth men and women, selected from the most 
able-bodied and intelligent applicants, lands its space ship on the 
planet Soil of a nearby star system. The planet is very dark due to a 
persistent cloud cover. The friendly Soil people quickly establish a 
successful method of communication. Despite some similarities and 
differences, both groups agree to be examined by each other's 
physicians. 

Two days later, the Earth physicians report to their group: 
The Soil people, although mildly grotesque looking and 
somewhat smaller than us, are quite nice and friendly. They 
see on this dark planet because their eyes respond to infrared 
light, but not to visible light. The frequency range of their vocal 
cords includes ours, and also extends into the inaudible high- 
frequency ultrasound range. The foods here consist of highly 
complex compounds, and their digestive system includes a 
double stomach. The first stomach, much like our cow's, 
predigests the food. The rest of the digestive system is the same 
as ours. 

Like us, they have two arms, but theirs are shaped m the 
form of wings, which allow them to fly, and instead of having 
five fingers, the ends of their arms are flat pads. All their 
various utensils, of course, interface successfully with these 
pads. They write with special pens that lay down infrared 
radiation sources which, though invisible to us, they can read. 
They have only one leg which is as broad as our two and ends 
in a round, fiat, pad-like structure. Walking involves a rolling 
motion of this pad. 

The Soil people live in high-rise apartments which look 
almost like ours, but there are no stairs or elevators. They 
simply fly up into their apartments and pass through doors 
located on the outside walls. A combination of high-frequency 
sound signals, which they emit, open the door locks. 

All manual labor on Soil is done by computers and by robots 
with physical configurations like ours. Computers are pro- 
grammed and triggered by combinations of the high-frequency 
sounds. All children gradually learn these sound combina- 
tions in school. 

The Soil physicians presented the following report to the 
Soil President: 

The Earth people are blind; they have no infrared vision. 



They speak of being able to see light, but we are not sure what 
they mean. They are partially aphasic, as their vocal cords 
do not have vibration capacity at our highfrequencies. Of more 
immediate consequence is the fact that the Earth people 
cannot eat. Their digestive system is incapable of breaking 
down our food products. We will have to tackle this problem 
soon, as their own food supply will run out in a week. Of 
course, as you noticed, they are dependent in flving and, 
because of their size, they are also dependent in transfers 
in and out of our cars. 

It appears that the Earth people cannot live anywhere on 
our planet. They say our apartments look like theirs, but asked 
if we had something called "stairs" or "elevators." Except 
perhaps for the first floor in some of our buildings, these people 
really have no place to live. 

At first glance, the Earth people will be unable to workon 
our planet. Without high frequency vocal cords, they cannot 
learn to operate our computers and robots. Some of them look 
strong enough to work alongside our robots, but I suspect they 
will consider this to be menial employment. Also, 25 percent of 
them are severely depressed. 

Mr. President, the Earth people are clearly severely and 
totally disabled. What shall we do? 
The President replied, "Hospitalize them in our Comprehensive 
Rehabilitation Center, and report back to me in 6 months the 
outcome of the treatment." 

This scene graphically illustrates the definition of 
disability. Disability is present, or a. person is disabled, 
when a set of functions, either desired or required, 
exists, which cannot be independently performed when 
attempted in a specific environment. While the desired 
functions may be specific preferences of the person, the 
required functions may be totally dictated by the 
environment. 

The above Earth -Soil illustration of the influence of 
the environment on disability has exact parallels on 
Earth. Our environment has been a continuing end 
result of the activities of perhaps one -third or less of 
our population who, by virtue of power, a high level of 
intelligence, and superior health, direct the creation of 
our physical plants, our institutions, our jobs, and our 
recreation. These institutions, jobs, and physical 
plants are created for use bv the majority, who are 
much like the creators — people "in their own image." 



2 Stolov 



These insitutions, jobs, and physical plants were, 
however, not created for use by those less able-bodied, 
less intelligent, sick, or disabled — the people the 
creators hardly ever see. 

As the creators have become more sophisticated, 
systems have been developed to accommodate this 
disabled group, but somewhere "off to the side," away 
from the mainstream. Only recently has the concept 
that the disabled can be brought into the mainstream 
been considered, and the environment has begun to be 
modified to accomodate them. 

Characteristics of Disability 

Let us return again to the planet Soil and listen to 
the report given to the President 6 months after the 
Earth people entered the Rehabilitation Center. 

Mr. President, I am happy to report that 30 percent of 
the Earth people have been ' 'closed rehabilitated. " All of 
these learned to use the apparatus we developed to convert 
infrared to visible light. They also learned to manage the 
high-frequency ultrasound radiator we developed, which 
they wear around their waists. Our drug industry 
developed a pill which allowed this group to digest our 

food products, although the pill is unsuccessful with the 
others. Half of the 30 percent became independent in 

flying with rocket devices, while the other half chose to 
have their arms amputated and prosthetic wings applied. 
Automobiles were built to accommodate the Earth people, 
who actually helped in the design. The cost of the 
automobiles, however, is quite high. This group is now 
living in the community alongside our people and, in 

fact, some of the Earth men and women are in love with 
Soil people and have talked about marriage. We have 
therefore initiated sex education and counseling in an 
effort to achieve sexual compatibility. 

Twenty -five percent are still in the Rehabilitation 
Center and we are hopeful that they, too, will succeed. 
The residual problem for most of them is digestion. They 
all continue to need the predigestion machine, which is 
not yet generally available throughout the planet. 

Twenty percent of the Earth people have also been 
discharged "partially rehabilitated, " but they are not 
living among our people. They were unable to master the 
eating problem or the flying skills, and were not 
intelligent enough to handle the ultrasound machine. We 
built a handicapped housing community nearby for them. 
The building has stairs, elevators, and their type of 
lights. A sheltered workshop is also in the building, 
where the Earth people repair computers. As you know, 
this has been a task that only a small number of our Soil 
people care for. Some of them have chosen to work 
alongside our robots and appear happy in this menial 
labor. 

Another 10 percent of the Earth people have been 
"closed unrehabilitaled" and have been transferred to a 
nursing home. They were able to overcome only a few of 



their functional disabilities and require attendant care. 
The attendant turnover is quite high for, as you know, 
few Soil people are interested in this activity. 

Unfortunately, 15 percent of the Earth people have 
committed suicide, some indirectly and some directly. 
Initially, some refused to be admitted to the 
Rehabilitation Center, claiming that they were not 
disabled. They were cared for briefly in our acute 
hospital, where food could be prepared, but they soon died 
from a combination of malnutrition and disease. The 
others simply found the stress too great, discharged 
themselves from the Rehabilitation Center against 
medical advice, and soon thereafter took an overdose of 
one of the Earth medicines they brought with them. 
The above scene illustrates three important 
characteristics of disability: 

1. There is no one-to-one correlation between a 
disease and the spectrum of associated disability 
problems. The same disease can produce separate 
sets of disability problems in different 
individuals. 

2. There is no one-to-one relationship between a 
disease and the amount of residual disability. 
Disability problems can be removed even though 
the disease is unchanged. 

3. The ability of a patient and the total health care 
team to remove disability in the face of chronic 
disease is dependent upon the patient's residual 
capacity for physiological and psychological 
adaptation. Residual strength must be evaluated 
and enhanced to "work around" an impairment 
in order to remove disability. 

In our Earth -Soil scenario, the disease was "able- 
bodied human." On Earth, the disease is any of the 
many different conditions discussed in this textbook. 
Thus, in the case of multiple sclerosis, for example, no 
two patients have the same problems. Their individual 
disabilities will depend upon the functions desired or 
required, the environments in which these functions 
are to be performed, and the extent of the attack on the 
central nervous system by the multiple sclerosis 
lesions. Even if the amount of destruction within the 
nervous system is not altered, the functional losses can 
be removed in several ways. The capacity of the 
unaffected body systems can be enhanced, the 
environment can be modified, and the interface 
between person and environment can be altered to 
match residual skills to environmental demands. 

Patient - Environment Interaction 

Figure 1 - 1 illustrates the interaction between an 
individual (the patient or client) and his environment 
through the interface between the two. On the patient 
side is the basic chronic disease interacting with basic 
psychological characteristics that identify the patient as 
a personality. The psychological factors include those 



Comprehensive Rehabilitation 3 



Patient 



Interface Environment 



Disease 



IE 



Psychology 




w 



TOTAL 
DISABILITY 



FIGURE 1-1. Schematic representation of the interaction of the 
patient with his environment. On the left, disease factors are 
reciprocally influenced by psychological factors. On the right, 
social factors are mutuallv influenced by vocational factors. The 
patient and his environment mutuallv influence each other 
through their interface. The Total Disabilitv is fed by all areas. 

emotional responses that may be reactive to the stress 
of the chronic disease. The disease and the patient's 
psychology are in dynamic interaction. In most of the 
conditions discussed in this text, changes in the basic 
disease can affect the patient's psychology, and 
changes in the patient's psychology can influence and 
alter the extent of disease, particularly its 
complications. 

On the environment side of figure 1-1 there are also 
two components in dynamic interaction: the social 
sphere (the patient's home, family unit, social 
responsibilities, and interpersonal contacts) and the 
vocational sphere (the place of work, the breadth of 
responsibilities, and the financial and personal 
rewards, and also avocational and recreational 
pursuits). The dynamic interaction is clear. In many 
situations, disturbances in the equilibrium of the social 
sphere can interfere with vocational performance, and 
vice versa. Alterations in either sphere can cross the 
patient -environment interface and modify the 
patient's disease and level of psychological 
performance. 

A patient's total disability (his disability diagnosis or 
complete list of disability problems) derives from 
factors specific to himself and to his environment. 
Problems may relate to the disease; to the patient's 
psychology, social functioning, or vocational 
functioning; and to the interface between the patient 
and the environment. Removal of disability is achieved 
through therapeutic attacks on the disease, through 
enhancement of the patient's psychology, through 
modification of the interface, and through direct 



modification of social and vocational environmental 
factors. 

Consider the following brief case history and list of 
disability problems of a patient recently admitted to a 
rehabilitation center for comprehensive rehabilitation: 

A 19 -year- old woman fractured her cervical spine in a 
small - plane accident, with resultant quadriplegia. Her male 
companion, with whom she had been living for the previous year 
and a half, was killed in the crash. Their relationship had been 
close and family oriented, since she served as "stepmother" for 
her companion 's two small children from a prior marriage. 
Following the accident, responsibility for the children was 
legally assumed by their natural mother. 

The patient was hospitalized for a short period on an acute 
neurosurgical service and then transferred to a comprehensive 
rehabilitation center for inpatient care. Her problem list 
following a complete comprehensive evaluation shortly after 
admission to the rehabilitation center included: 

1 . C7 (seventh cervical vertebra) fracture dislocation 

2. C7 complete quadriplegia 

3. Ambulation dependent 

4. Transfer skills dependent 

5. Eating, dressing, personal hygiene skills dependent 

6. Bowel incontinence 

7. Bladder incontinence 

8. Decreased respiratory function 

9. Potential for pressure sore 

10. Potential for thrombophlebitis 

11. Immature personality 

12. Reactive depression 

13. Home architecture incompatible with paralysis 

14. Financially dependent 

15. Estranged from parents 

16. Unemployed, no prior work history 

17. Homemaking skills deficient 

18. Transportation dependent 

Comparing the patient's problem list with the 
scheme in figure 1-1 identifies problems 1, 2, 6, 7, 8, 
9, and 10 as directly involved with the patient's 
disease. Problems 3, 4, 5, and 18, while also a direct 
result of the disease, constitute her physical disabilties. 
Problems 11 and 12 relate to the patient's psychology, 
and problems 13, 14, 15, and 17 to the social sphere. 
Problem 16 succinctly and precisely identifies the 
vocational disability. 

Severe Disability 

The Rehabilitation Act of 1973 and subsequent 
amendments charged the State- Federal Vocational 
Rehabilitation Program to concentrate its efforts on 
patients and clients with severe disability. Some efforts 
have been made to quantify disability by defining a 
large number of functions and assessing a patient's 
degree of independence or dependence in these 
functions according to a numbering system. The 
numbers can then be totaled for each patient, and an 



Stole 



arbitrary cutoff point can be established. Patients with 
total numbers above this point may be called severely 
disabled and those with total numbers below this point 
may be called minimally or mildly disabled. 

Such an approach, while seemingly solving the 
problem of definition, is fraught with difficulties 
because disability is so highly individualized. Further, 
the relative importance of one set of functions 
compared to another (e.g., physical vs. social) is lost 
when an arbitrary numbering system is used. Applying 
a "weight factor" which scales the relative importance 
of one function to another (assuming a consensus on 
such a factor) would also be inadequate because the 
importance of one function relative to another usually 
varies from patient to patient. 

Rather than a quantitative definition, an operational 
approach to defining the severity of disability may 
perhaps be more useful for a particular counselor and 
client. As already discussed, disability is removed via 
therapeutic techniques applied to the patient, to the 
environment, and to the interface. Disability problems 
can be characterized by the therapeutic techniques 
available, or perhaps unavailable. 

1. A disability problem in a particular patient may 
be one for which a therapeutic technique is 
immediately available ("on the shelf") and is 
well-known to be capable of removing the 
disability problem. 

2. A disability problem may be one for which a 
therapeutic technique is available, but it is not 
certain that the technique can indeed remove the 
disability problem. 

3. A disability problem may be one for which no 
therapeutic technique is available that is known to 
be capable of removing the problem, but it is 
believed that one can be created. 

Given these concepts of disability problems and 
therapeutic techniques, a definition of severe disability 
can be stated which may be operationally useful for 
any particular client or patient. 

A minimally or mildly disabled individual may be viewed 
as one for whom therapeutic techniques, which will 
remove the disability and complete the rehabilitation, 
are well known and immediately available. The 
following short histories are useful examples. 

A 45 -year -old editor notices increasing difficulty seeing the 
print of the texts and manuscripts he is asked to review. He 
visits his ophthalmologist, who determines that the editor suffers 
from the commonplace farsightedness caused by the loss of 
elasticity in the lens of the eye with age. The physician prescribes 
reading glasses. The glasses are purchased, the reading 
difficulty is eliminated, the disability is removed, and the 
rehabilitation is complete. 

In this example, the eyeglasses constitute the readily 
available therapeutic technique with a well-known 
capability to remove the reading disability due to this 
type of eye problem. 



A 35 -year- old outdoor carpenter suffers a fall and ruptures 
an intervertebral disc in his lower back. Spinal nerve irritation, 
back and leg pain, and leg weakness result. Back surgery is 
recommended to remove the pressure on the nerve. The patient 
has the surgery, pressure on the nerve is removed, and the back 
and leg pain and weakness disappear. The physician advises 
that the heavy carrying loads associated with outdoor carpentry 
work will cause early degeneration of the spine and are too 
strenuous for the residual capacity of the back. Lighter work 
activities are recommended, but are unobtainable in the outdoor 
carpentry field. 

Vocational evaluation of the unemployed carpenter indicates 
that hand dexterity, which is required for indoor light 
cabinetmaking and refimshing, is excellent, fobs in indoor 
carpentry are available, and the counselor knows that the local 
vocational school has a good Tyear course in cabinetmaking, 
refinishing, and other indoor carpentry skills. The counselor 
develops an educational plan with the unemployed carpenter, 
who completes the course, finds employment, and succeeds in his 
new occupation. 

In this example, the readily available therapeutic 
technique was the local carpentry course which, when 
applied, removed the disability problem, "unem- 
ployment." 

A 45 -year old married male with fairly stable adult -onset 
muscular dystrophy manages quite well at home and in his office 
job as long as his wife assists him with dressing. The patient's 
upper extremity weakness interferes with shirt buttoning, belt 
buckling, and putting on socks, shoes, and ties. When his wife 
unfortunately dies, the patient is totally disabled in his dressing 
skills and is consequently unable to maintain employment. He is 
admitted to a rehabilitation center where, through clothing 
modifications, the use of adaptive equipment, and technique 
training, he achieves full independence in dressing. The 
disability removed, he returns home and resumes employment. 

In this example, the readily available techniques 
were those adaptive devices and methods of 
performance known to enable achievement of dressing 
independence. Of significance in this example is the 
fact that it was a change within the patient's social 
sphere of activities that created the disability and the 
potential for unemployment. 

In the above examples, the therapeutic techniques 
were readily available and known to be capable of 
removing the disability problems. Hence, these 
patients were minimally or mildly disabled. 

Severe disability can also be characterized in relation 
to available therapeutic techniques. At least three 
different types of situations signify severe disability. 

1. As in the above examples, the therapeutic 
techniques are available and known to be capable of 
removing a disability problem. However, the patient 
has several disability problems, and more than one 
technique must be applied. These various disability 
problems require simultaneous solutions and hence 
several professionals from different fields working 
together. A simple sequential application of each 



Comprehensive Rehabilitation 5 



technique will not be successful because a particular 
solution to one of the problems may interfere with the 
solution of another. All the professionals must 
therefore work together in a single setting to solve the 
problems effectively. In this case, the disability 
qualifies as severe because of the need for a center 
where various professionals can interact in the 
application of therapeutic techniques to remove the 
patient's disability. 

2. A second situation signifying severe disability 
occurs when a number of possible techniques are 
available, but it is not known in advance that they are 
capable of removing the several disability problems, 
and therefore an extended evaluation and treatment 
period is necessary. In this case, there might also be 
several techniques potentially available for each of the 
individual problems, but much trial is necessary. 

3. Finally, disability can be considered severe 
when the therapeutic techniques are not readily 
available, but evaluation suggests that they can be 
treated in therapeutic settings, working closely with the 
patient on their design. Some of these techniques 
might relate directly to the interface between the 
patient and the environment. 

Using these three operational definitions of severe- 
disability and the operational definition of minimal or 
mild disability, the counselor can decide whether or 
not a particular client's disabilitv is severe. The 
counselor first performs and /or obtains a careful 
evaluation of the client's total function and a listing of 
all the client's disability problems. This review can be 
compared to the operational definitions and the 
examples cited above to decide whether severe or 
minimal disability is present. The review also serves to 
create the rehabilitation plan. 

For example, the problem list of the 19-year-old 
quadriplegic on page 3 can be considered in light of 
the above definitions. The patient's condition can be 
identified as severe disability from several points of 
view. Available "on the shelf" therapeutic techniques, 
useful for dealing with problems 1-10 and possibly also 
17 and 18, do exist. Their successful application, 
however, requires the coordinated, simultaneous 
efforts of physicians, rehabilitation nurses, physical 
therapists, occupational therapists, and possibly also 
orthotists. 

Further, while some therapeutic techniques do exist 
that might be applied toward the solution of the 
problems 11-16, they do not have the promise of 
certain resolution, and several new techniques need to 
be created. Thus, all three operational definitions of 
severe disability apply in the example. 

REHABILITATION 

Comprehensive Evaluation 
Medical evaluation. As the understanding of 



causes and treatment of diseases and injury increased, 
a number of specialties evolved within the medical 
profession. Some of these specialties were organized 
according to known causes of the disease (e.g., allergy, 
oncology) and others as disciplines dealing with a 
particular system of the body (e.g., neurology, 
ophthalmology). The medical evaluation of a disease 
may therefore involve one or more specialists, each 
with a specific set of skills and knowledge. 

All medical evaluations include a history of the 
symptoms, a review of the past medical history, a 
historical review of each of the systems in the body 
capable of producing symptoms, and a review of the 
social, vocational, and psychological history of the 
patient. A physical examination of all the systems is 
also performed. While almost all physicians carry out 
the above, the different specialists bring additional 
insights, depending upon their expertise. Medical 
evaluation also includes a number of different 
laboratory and X-ray examinations designed to clarify 
a patient's problems, as well as to suggest or monitor 
treatment. 

The current widely used medical- record system was 
introduced and became popular about 10 years ago. 
Under this new problem -oriented format, medical 
evaluation results in a list of patient problems, rather 
than simply a diagnosis of the suspected disease. 
Included in such a list might well be the known disease 
itself. The list, however, will also include: (a) 
significant consequences and complications of the 
disease; (b) physical function problems, which 
comprise the extent of the physical disability; and (c) 
problems in the psychological, social, and vocational 
spheres, as in the 19-year-old quadriplegic patient. 

With this approach, therapeutics can be directed at 
each of the problems on the list, rather than exclusively 
at the disease itself. This is particularly important 
when the disease is chronic. In chronic disease, 
therapeutics do not exist for eliminating the pathology 
of the disease; i.e., the pathology itself may be 
permanent. Functional problems which might be a 
direct result of the disease can, however, be identified 
and solved through appropriate therapeutic- 
techniques. Physicians specializing in physical 
medicine and rehabilitation ( phy siat rists . 
rehabilitation medicine specialists) have particular 
expertise in the identification of functional, 
psychosocial, and vocational problems which might be 
overlooked by other specialists whose interest is 
focused more on the organ system or on specific 
therapies. 

Allied health evaluation. As already discussed. 
the breadth of disabilitv problems includes not only the 
disease, but also issues in the patient's psychology and 
in his social and vocational spheres. The clear 
identification and need for treatment of all these has 
resulted in a large number of medically related health 



6 Stolov 



professionals who both participate in the evaluation 
phase and work in medical settings with attending 
physicians. Examples of such professionals include 
physical therapists, occupational therapists, 
rehabilitation nurses, prosthetists and orthotists, 
medical social workers, clinical psychologists, speech 
and language therapists, recreation therapists, 
vocational counselors, work evaluators, rehabilitation 
teachers and mobility instructors for the blind, 
bioengineers, sex therapists, and audiologists. These 
professionals add their own special expertise, not only 
to help achieve a thorough evaluation of the disability 
problems, but also to ensure that all available 
therapeutics are applied. 

Comprehensive Rehabilitation Centers 

A rehabilitation center is the facility that has evolved 
for the management of patients and clients with 
chronic disability problems. These institutions are 
usually associated with general hospitals, but they may 
be "free standing." Most of the centers that have the 
capacity to treat severe disability also have inpatient 
facilities for 24 -hour -a- day intensive treatment. 
Some rehabilitation centers are only outpatient 
facilities and, as such, cannot handle as wide a range of 
problems. 

Usually, each rehabilitation center develops an 
expertise in certain areas and certain diseases, but 
many are able to handle all types of problems. Highly 
specialized rehabilitation centers include those related 
to blindness, deafness, severe mental retardation, or 
alcoholism, to name a few. All rehabilitation centers 
have a certain commonality. Their staffs all include 
full-time physicians with special interest and training 
in disability and expertise in rehabilitation treatment. 
All also have full-time staffs of allied health 
professionals representing those disciplines necessary 
for the rehabilitation treatment of the type of patient 
they serve. Most offer vocational rehabilitation 
services by full-time vocational rehabilitation 
counseling staff. They are not necessarily counselors 
for the State Vocational Rehabilitation agency, 
although in many instances staff from the State agency 
are assigned full-time to such institutions. Vocational 
rehabilitation counselors in such institutions who are 
not actually State agency staff do relate directly to State 
agency personnel to ensure continuity of vocational 
rehabilitation after discharge from the center. 

The hallmark of all rehabilitation centers with 
regard to any patient admitted is an initial evaluation 
to identify the full range of disability problems. 
Evaluation invariably involves participation by all the 
relevant professionals for the individual situation. 
After the evaluation, the rehabilitation team, together 
with the patient, develops a treatment plan for removal 



of the disability problems. For each problem, goals are 
established which characterize the anticipated end 
result of treatment. Formulation of goals and a 
treatment plan, in concert, ensures that all 
professionals are working with the patient toward a 
common end. 

During the course of treatment, the rehabilitation 
team meets frequently to assess progress, modify goals, 
and alter treatment programs, as indicated by the 
patient's progress. Efforts are directed toward 
minimizing the disease, maximizing residual capacity, 
establishing suitable interfaces between the patient and 
the environment, and modifying the environment to 
secure an adequate match between what the patient 
may be able to achieve and what his environmental 
circumstances may demand. 

Referral Information Sources 

Physicians are licensed to practice, based on 
examination by the state in which they work. 
Minimum competency in a specialty, however, is not 
associated with any governmental license, but rather is 
assessed by examinations prepared by peers. These are 
organized by the various Specialty Boards of the 
American Medical Association. The Boards admit to 
examination applicants who have met certain min- 
imum training standards for their specialty. 
Certification is then achieved through examination, 
usually written and oral. The Directory of Medical 
Specialists, a yearly publication available in almost all 
libraries, lists the training and certification of medical 
specialists. Appendix A lists those Specialty Boards in 
which certification by examination under the auspices 
of the American Medical Association exists. 

Allied health professionals have similar standards of 
competency, and directories are available of licensed 
individuals and those who may have met local or 
national examinations. Appendix B presents the name 
and address of the major national organization of each 
of the allied health professional groups. Most have 
branches in each of the states, which publish directories 
of licensed practitioners. Some have national 
directories only. Each organization can, on request, 
indicate whether a particular person has met the 
standards established for the profession. 

Appropriate rehabilitation centers for severely 
disabled clients within any given community can 
usually be identified by several sources. A voluntary 
organization called the Commission for Accreditation 
of Rehabilitation Facilities maintains a directory of 
rehabilitation centers for members of the group 
(Appendix C). Additional sources of information listed 
in Appendix C include the NIHR Medical Rehabilita- 
tion Research and Training Centers scattered 
throughout the country, and the American Congress of 



Comprehensive Rehabilitation 7 



100% 



INTENSITY 
OF SERVICE 




Onset 



Discharge 
TIME 



Employment 



FIGURE 1-2. Schematic description of the intensity of medical 
and specific vocational services applicable for acute-onset 
severe disability requiring hospitalization. At onset, medical 
services dominate. At discharge, vocational services dominate 
until employment. Both medical and vocational services may 
need to be sustained for a period of time at a low level to 
maintain health and employment. 



Rehabilitation Medicine, an organization of medical 
and allied health professionals actively engaged in the 
'rehabilitation of the disabled. The Easter Seal Society 
in each city is also usually able to identify and 
recommend appropriate facilities. 

Medical- Vocational Transition 

Particularly for acute-onset, relatively 
"incurable" disease, or acute recurrences or 
complications of an ongoing chronic disease, a 
reciprocal relationship exists with regard to the 
intensity of medical services and specifically vocational 
services. Figure 1-2 illustrates this reciprocal 
relationship. At first, the medical services are intense 
and vocational services are minimal. With time, the 
intensity of the medical services decreases, while the 
intensity of vocational services increases. Medical 
services rarely decrease to zero because they usually 
are required to maintain achieved goals. The same, in 
effect, also applies to specific vocational rehabilitation 
services. After job placement or independent living is 
achieved, the intensity of vocational services tapers off. 
In many instances, sustaining success may require 
services at a lower level over an extend :d period of 
time. 

In relatively few instances is a patient exposed to the 
rehabilitation treatment process only once. Alterations 
in the disease, in the environment, or in the interface 
may cause new or previously removed disability 
problems to resurface and require additional 
intervention. This is particularly likely for those 
diseases with uncertain courses or prone to develop 
complications. Further, as new techniques develop, 
patients may re-enter the treatment process to seek 
solutions to disability problems not previously 
solvable. 



The Individually Written Rehabilitation Plan 

The term "vocational rehabilitation services," 
under the State- Federal program, has come to mean 
"any goods or service" necessary to render a disabled 
individual employable. The term is not, therefore, 
limited to specific counseling with regard to job 
selection or job placement. Rather, it includes the full 
range of medical and allied health services which, when 
delivered, are expected to remove a sufficient number 
of disability problems to allow for employment to 
result. It therefore includes those services which the 
counselor can provide for, but might not personally be 
able to deliver. 

Vocational rehabilitation services also include 
services or processes associated with extended 
evaluation to determine the existence of vocational 
potential. In such situations, the availability of 
therapeutic techniques may be relatively uncertain, 
and the extended evaluation determines whether actual 
therapeutic services are likely to render a disabled 
individual employable. Recently, there has been 
increasing emphasis on the value of rehabilitation 
services to a severely handicapped individual for whom 
the goal may be limited to independent living and a 
satisfactory avocational life. 

The individually written rehabilitation plan 
(IWRP), a concept introduced in the Rehabilitation 
Act of 1973, fits in very nicely with the problem- 
oriented format of medical evaluation discussed above. 
The IWRP is, in essence, a contract or agreement 
between a rehabilitation counselor and a client with 
regard to any evaluation or treatment program that 
commits the counselor to restorative services, 
counseling services, and other appropriate services to 
treat disability problems. An IWRP attempts to 
identify a long-range rehabilitation goal, which may 



8 Stolov 



actually include a specific area of employment. It also 
identifies intermediate objectives to be accomplished 
on the road to the long-range goal. The intermediate 
objectives relate quite closely to the expectations of 
treatment for the various disability problems that may 
be uncovered in the evaluation of a client by a 
comprehensive rehabilitation team. 

In addition to the listing of the long-range goal and 
intermediate objectives, an IWRP spells out the 
processes and the programs by which services will be 
delivered, by whom, and how. Such plans are not cast 
in stone. Periodic review between counselor and client 
may lead to modification of intermediate objectives, 
long-range goals, and techniques of treatment that 
prior experience may dictate. Currently, with regard 
to expenditure of Federal funds, long-range 
rehabilitation goals require an employment objective. 
In time, it is anticipated that the independent living 
objective for those very severely disabled may be a 
permissible long-range goal. 

CHAPTER ORGANIZATION 

All but five of the chapters in this text deal with those 
specific diseases known from experience to be 
instrumental in causing severe disability. The five 
chapters deal with issues common to most of the others 
and have their own special format. These five (chaps. 
2, 3; 4, 21, and 32) deal with psychosocial 
adjustment, sexual adjustment, consequences of bed 
rest, reconstructive surgery, and the normal function 
of the six body systems which, when deranged by 
disease, result in severe disability. The reader is 
encouraged to become familiar with the normal 
function of these systems to enhance his understanding 
of the diseases themselves. 

All the disease -oriented chapters are organized in a 
similar way. They begin with a disease description, which 
includes its natural history of evolution, its primary 
characteristics, and its potential complications. The 
description is followed by a section on the junctional 
disabilities, both physical and psychosocial, associated 
with the disease. The physical disabilities indicate how 
the particular disease limits the patient's performance 
as a whole with regard to ambulation, transfer skills, 
eating skills, dressing skills, and personal hygiene 
skills, the group of activities usually referred to as 
activities of daily living (ADL). Further, if the disease 
produces disability in transportation and commun- 
ication skills, these are also discussed. In the 
section of psychosocial disabilities, those potential 
interferences in a patient's psychological or social 
functioning that may be a direct result of the disease, 
or associated with it, are presented. Rehabilitation 
potential is then described, incorporating factors related 
to the disease itself and the usual expected results of 
treatment. 

The next section in each chapter deals with standards 



of evaluation. Included in this section is what currently 
constitutes an optimum evaluation of the patient and 
his condition. It describes the physicians and allied 
health professionals who should be consulted to obtain 
a full characterization of the disease and identification 
of the breadth of disability problems. Significant 
laboratory examinations are also described, where 
pertinent. 

The chapters then deal with the total treatment of the 
condition. Included is the current "state of the 
art"with regard to the medical -surgical management 
and treatment by allied health professionals known to 
be capable of modifying the disease pathology and/or 
affecting and removing physical and psychosocial 
disability problems. Maintenance management is also 
included if it is recommended to maintain achieved 
rehabilitation goals. With the information in this 
section and with his knowledge of his client, the 
counselor should be able to determine whether his 
client has had the benefit of all available treatment. 

The final section in each chapter deals with specific 
vocational implications of the condition that a 
rehabilitation counselor should keep in mind when 
planning employment goals with his client. This 
section may deal with factors of education, aptitudes, 
interests, the environment, and physical demands as 
they may influence or be influenced by the disease in 
question. 

Physical demands generally include lifting, 
carrying, pushing, or pulling. Lifting and carrying 
implies that the arms have certain capacities, and that 
the person has certain transport capability. Pushing or 
pulling implies arm or leg abilities. All four 
characteristics generally parallel each other, and 
therefore physical demands can be characterized by the 
lifting function alone. 

With regard to physical demands, the following 
definitions are used throughout the text: 

1. Sedentary work means that the lifting requirements 
are on the magnitude of 10 pounds (4.5 kg) 
maximum and usually involve smaller objects. 
The work is largely done sitting, although some 
walking or standing may be required. 

2. Light work implies a lifting capacity of up to 20 
pounds (9 kg), with significant walking or 
standing requirements. A heavy requirement for 
pushing, pulling, or leg control is also consistent 
with most sitting work. 

3. Medium work implies a lifting maximum of 50 
pounds (22.5 kg), with a usual lifting requirement 
of about 25 pounds (11.3 kg). This type of work 
and the two types that follow usually require 
walking and standing capacities. 

4. Heavy work requires a lifting maximum of up to 
100 pounds (45 kg), with the usual requirement 
50 pounds (22.5 kg). 

5. Very heavy work requires a lifting maximum of 
greater than 100 pounds (45 kg), with the usual 



Comprehensive Rehabilitation 9 



carrying capacity of 50 pounds (22.5 kg). 

The chapter sections on physical demands will also 
discuss interferences in lower extremity functions, such 
as climbing, balancing, stooping, and kneeling, and in 
upper extremity functions, such as reaching, handling, 
fingering, and feeling. 

Each chapter includes an annotated bibliography, 
which references only books and articles readily 
available in most libraries. A glossary is included at the 
end of the book. Words which have been defined in the 
text do not appear in the glossary, unless they occur 
frequently. The glossary is therefore not an exhaustive 
dictionary, and the counselor may wish to have a 
medical dictionary as well. 

CONCLUSION 

The challenge of providing maximal services for the 
severely disabled is great. The acceptance of the 
challenge requires knowledge, teamwork, coordin- 
ation, planning, and patience. Assimilation of the 
material in this handbook can, at best, only equip a 
counselor with a few basics on which to build. Medical 
and rehabilitation research will soon modify these 
basics, eliminate some, and add others not yet 
imagined. The counselor must not only develop, 
through experience, the skill to minister successfully to 
the disabled, but must also maintain a continuing 
education program to update his knowledge and his 
techniques. 



Pulmonary Disease 

Rheumatology (Committee) 
American Board of Neurological Surgery 
American Board of Nuclear Medicine 
American Board of Obstetrics and Gynecology 
American Board of Ophthalmology 
American Board of Orthopaedic Surgery 
American Board of Otolaryngology 
American Board of Pathology 
American Board of Pediatrics 

Pediatric Cardiology 

Pediatric Endocrinology 

Pediatric Hematology-Oncology 
(Special Competency) 

Nephrology (Special Competency) 

Neonatal-Perinatal Medicine 
(Special Competency) 
American Board of Physical Medicine 

and Rehabilitation 
American Board of Plastic Surgery 
American Board of Preventive Medicine 
American Board of Psychiatry and Neurology 

Psychiatry 

Neurology and /or Child Neurology 

Psychiatry and Neurology 

Child Psychiatry 
American Board of Radiology 
American Board of Surgery 
American Board of Thoracic Surgery 
American Board of Urology 



APPENDIX A 



APPENDIX B 



SPECIALTY BOARDS 

The following is a list of the American Boards of the 
American Medical Association which certify specialists 
through examination. The address of their executive 
offices can be obtained from the American Medical 
Association, 535 North Dearborn Street, Chicago, 
Illinois 60610. Those currently certified are listed in 
the Directory of Medical Specialists, Vols. 1 & 2, 
Chicago, Marquis Who's Who. The Directory is 
updated annually. 
American Board of Allergy and Immunology 
American Board of Anesthesiology 
American Board of Colon and Rectal Surgery 
American Board of Dermatology 
American Board of Family Practice 
American Board of Internal Medicine 
Cardiovascular Disease 

Endocrinology and Metabolism (Committee) 
Gastroenterology 
Hematology (Committee) 
Infectious Disease (Committee) 
Medical Oncology (Committee) 
Nephrology (Committee) 



NATIONAL OFFICES 

OF ALLIED HEALTH ORGANIZATIONS 

American Association for Respiratory Therapy 
7411 Hines Place, Suite 101 
Dallas, TX 75235 

American Association of Sex Educators, 
Counselors and Therapists 

5010 Wisconsin Avenue N.W., Suite 304 

Washington, D.C. 20015 

American Association of Workers for the Blind 
1511 K Street N.W. 
Washington, D.C. 20005 

American Dental Associaton 
211 East Chicago Avenue 
Chicago, IL 60611 

American Nurses' Association 
2420 Pershing Road 
Kansas City, MO 64138 



10 Stolov 



American Occupational Therapy Association 
6000 Executive Boulevard, Suite 200 
Rockville, MD 20852 

American Optometric Association 
7000 Chippewa Street 
St. Louis, MO 63119 

American Orthotic and Prosthetic Association 
1444 N Street N.W. 
Washington, D.C. 20005 

American Physical Therapy Association 
1156 Fifteenth Street N.W. 
Washington, D.C. 20005 

American Psychological Association 
1200 Seventeenth Street N.W. 
Washington, D.C. 20036 

American Rehabilitation Counselor Association 
1607 New Hampshire Avenue N.W. 
Washington, D.C. 20009 

American Speech and Hearing Association 
9030 Old Georgetown Road 
Washington, D.C. 20014 

Commission on 

Rehabilitation Counselor Certification 

6th Floor, 162 North State Street 

Chicago, IL 60603 

National Association of Social Workers 
1425 H Street, Suite 600 
Washington, D.C. 20005 

National Rehabilitation Counseling Association 
1522 K Street N.W. 
Washington, D.C. 20005 

APPENDIX C 

VARIOUS REHABILITATION ORGANIZATIONS 
AND RESEARCH AND TRAINING CENTERS 

Organizations 

American Congress of Rehabilitation Medicine 
30 North Michigan Avenue 
Chicago, IL 60602 
Phone: (312) 236-9512 

Commission for Accreditation of Rehabilitation 
Facilities 

2500 North Pantano Road 

Tucson, AZ 85715 

Phone: (602) 886-8575 



Easter Seal Society 
2023 West Ogden 
Chicago, IL 60612 
Phone: (312) 243-8400 

International Association of Rehabilitation 
Facilities 

5530 Wisconsin Avenue, Suite 955 

Washington, D.C. 20015 

National Rehabilitation Association 
1522 K Street 
Washington, D.C. 20005 

Department of Education- NIHR Rehabilitation 
Research and Training Centers 

New York University 

Medical Rehabilitation R&T Center 

400 East 34th Street 

New York, NY 10016 

University of Minnesota 

Medical Rehabilitation R&T Center 

860 Mayo Building 

Minneapolis, MN 

University of Washington 

Medical Rehabilitation R&T Center 

15th Avenue N.E. 

Seattle, WA 98195 

Baylor College of Medicine 
Medical Rehabilitation R&T Center 

1333 Moursund Avenue 

Houston, TX 77030 

Emory University School of Medicine 
Medical Rehabilitation R&T Center 

1431 Clifton Road 

Atlanta, GA 30322 

Tufts University 

Medical Rehabilitation R&T Center 

171 Harrison Avenue 

Boston, MA 02111 

Temple University 

Medical Rehabilitation R&T Center 

Suite 201, 12th and Tabor Road 

Philadelphia, PA 19141 

The George Washington University 
Medical Rehabilitation R&T Center 

Room 714, Ross Hall 

2300 Eye Street N.W. 

Washington, D.C. 20037 



Comprehensive Rehabilitation 1 1 



University of Colorado Medical Center 
Medical Rehabilitation R&T Center 

4200 Ninth Avenue, Box C242 

Denver, CO 80262 

University of Wisconsin 

Mental Retardation Rehabilitation R&T Center 

Waisman Center on Mental Retardation 

and Human Development 

2605 Marsh Lane 

Madison, WI 53706 



University of North Carolina at Chapel Hill 
Blindness Rehabilitation R&T Center 

210 N. Columbia Street 

Chapel Hill, NC 25714 



Boston University 

Rehabilitation R&T Center in Mental Health 

1019 Commonwealth Avenue 

Boston, MA 02215 



University of Arkansas 

Vocational Rehabilitation R&T Center 

346 West Avenue Annex 

Fayetteville, AR 72701 

University of West Virginia 
Vocational Rehabilitation R&T Center 

Suite E-l Dunbar Plaza 

Dunbar, WV 25064 

University of Oregon 

Mental Retardation Rehabilitation R&T Center 

212 Clinical Services Building 

Eugene, OR 97403 

New York University 

Deafness Rehabilitation R&T Center 

80 Washington Square East 

New York, NY 10003 

University of Alabama 

Medical Rehabilitation R&T Center 

1717 Sixth Avenue South 

Birmingham, AL 35233 

Northwestern University 

Medical Rehabilitation R&T Center 

Rehabilitation Institute of Chicago 

345 East Superior Street 

Chicago, IL 60611 

Texas Tech University 

Mental Retardation R&T Center 

P.O. Box 4510 

Lubbock, TX 79409 

University of Wisconsin-Stout 
Vocational Rehabilitation R&T Center 
Menominie, WI 54751 

University of California, San Francisco 
Deafness and Mental Health Rehabilitation 
R&T Center 

1474 Fifth Avenue 

San Francisco, CA 94143 



2 PSYCHOSOCIAL ADJUSTMENT 

TO CHRONIC DISEASE AND DISABILITY 

Gary T. Athelstan, Ph.D. 



ADJUSTMENT TO DISABILITY 

Definition 

Adjustment to disability has been defined by social 
scientists in various ways. Some definitions focus upon 
the individual and the behaviors that are learned or the 
roles that are assumed after onset of disability. Others 
adopt a sociological perspective and consider the 
individual in relation to society and societal units such 
as the family or community. This chapter will present 
some of the major phenomena of psychosocial 
adjustment to disability as they are reported in the 
literature and encountered in rehabilitation practice. 
Sexual adjustment receives special attention in chapter 
21, since it is a relatively new topic in the rehabilitation 
literature and has been the subject of increasing 
interest in recent years. 

Much of the research on adjustment processes in 
rehabilitation has been done from the perspective of a 
single academic discipline or to develop and test one or 
another theory. Nearly all such studies have 
contributed to our understanding of the effects of 
severe disabilities and how people cope with them. 
However, the discussion of psychosocial adjustment to 
disability developed in this chapter does not emphasize 
any particular disciplinary or theoretical viewpoint. 
Rather, the problems of adjustment are presented in 
the pragmatic terms which are familiar to disabled 
individuals and the counselors who work with them. 

In the sections that follow, a working definition of 
psychosocial adjustment is presented. Special emphasis 
is given to developing a conceptualization of 
psychosocial adjustment which is consistent with the 
philosophy of rehabilitation. This is followed by a 
discussion of the process of adjustment that highlights 
some of the landmarks, problems, and possible phases 
in the process. Finally, the implications of these issues 
for counselors and some of the ways that counselors 
can intervene in the process to facilitate adjustment are 
discussed. 

In a study of spinal cord injured patients which is 
currently underway at the University of Minnesota, 
patients were asked to tell the staff what adjustment to 
disability meant to them. Most of the answers 
expressed variations on a theme of "learning how to 
live with it." Others said that adjustment meant 



"making the most of what you have" or "trying to 
lead as normal a life as possible." 

Rehabilitation professionals tend to define 
adjustment in terms of achieving the individual's 
maximum potential. Although this seems to be a 
reasonable approach, it imposes the crucial 
requirement of accurately measuring "potential" in 
order to determine how much has been realized and, 
consequently, how "adjusted" the individual is. 
Considering the drastic changes that severe disability 
may produce in a person's life style and functioning, 
correct assessment of potential can be very difficult. 

Most traditional definitions of adjustment to 
disability in our society emphasize some form of 
productivity. Thus, a disabled person who is employed 
is generally assumed to be better adjusted to his 
disability than an unemployed person. However, 
counselors frequently encounter (a) employed disabled 
persons whom they consider poorly adjusted 
psychologically or socially; (b) poorly adjusted disabled 
persons who are unemployed but productive in 
nonvocational ways; and, occasionally, (c) seemingly 
unproductive and inactive disabled persons who 
appear to be very well adjusted. It is apparent, then, 
that employment, adjustment, and disability are 
separate, though interrelated, issues. Clearly, 
adjustment to disability is a very complex issue and 
cannot be solely defined in terms of employment; other 
factors must be considered as well. 

A definition of psychosocial adjustment should take 
into account the disabled individual's satisfaction with, 
or acceptance of, his circumstances. This approach has 
the major advantage of allowing a highly 
individualized assessment of adjustment to disability. 
However, it has the disadvantage of determining the 
level of adjustment by what the client says rather than 
by what he does. Since no good methods are available 
to assess the validity of these self- reports, the unit of 
measurement becomes subjective in nature. There- 
fore, individuals who report being content with their 
circumstances, whatever they may be, must be 
considered well adjusted. Yet, this view can be in 
direct conflict with very reasonable traditional 
definitions. In the study mentioned above, clients were 



13 



14 Athelstan 



asked to rate their degree of satisfaction with their 
living arrangements, financial and employment status, 
health, social life, and sex life. In general, persons who 
were most active and productive, often those who were 
employed, tended to report being least satisfied with 
their situation in life. 

In other words, the study found that individuals who 
would be considered best adjusted to their disability by 
traditional measures tended to be least well adjusted in 
terms of their self- reported acceptance of their 
circumstances. This could reflect, in part, a tendency 
of the employed and more active persons to measure 
themselves against a "normal" standard rather than to 
take satisfaction from their accomplishments in 
relation to other severly disabled persons. 
Nevertheless, the findings suggest that it would be 
appropriate to take into account some measure of 
psychological functioning or emotional status in any 
assessment of adjustment to disability. This means that 
rehabilitation counselors ought to consider the impact 
of their services not only on the employment status and 
activity level of their clients, but also on their 
psychosocial adjustment in the broadest sense. 

When all of the theoretical formulations, research, 
and clinical experience with disabled persons are 
considered, adjustment to disability seems to be a 
complex, but not at all mysterious, process that normal 
persons undergo in trying to cope with the severe 
psychological stress of a major life crisis. It should be 
viewed as an evolutionary, changing, and highly 
individualized process rather than a stable state. The 
problems faced by the newly disabled person are those 
of coping with physical and psychological loss, changes 
in body image, social status, and earning capacity, the 
anxiety and grief which often accompany these 
changes, and the need to learn new behaviors and 
make concrete plans for an uncertain future. Each 
individual will seek personal solutions to these 
problems and thus define his own adjustment to 
disability. 

PSYCHOSOCIAL EFFECTS OF DISABILITY 

Since many of the psychological effects of disability 
are a result of the social implications, it is necessary' to 
first consider some of the immediate consequences of 
disability onset. One of the most predictable effects of 
a visible physical disability is a change in the social status 
of the individual. Disabled persons in our society 
assume a special kind of minority status and occupy a 
socially devalued role. Numerous studies have shown 
that people tend to attribute negative characteristics to 
the disabled and that this tendency generalizes well 
beyond the direct effects of the disability. Visibly dis- 
abled persons are often assumed bv others to be less 
attractive, less desirable, and less capable in ways 



which are totally unrelated to their disability. 

A loss of social status may also result from the in- 
direct effects of disability, such as its economic consequences. 
The costs of acute and long-term medical care for 
chronic disease or disability may be so great as to 
deplete all of a family's assets. Furthermore, the impact 
of disability on an individual's earning capacity can 
produce a long-range change in socioeconomic status. 
Recent studies have shown that when the principal 
breadwinner of a family is disabled, an average reduc- 
tion of 40 percent in total family income results. 

Disability usually causes profound changes in close 
social relationships. Nagi and Clark (1964) have 
demonstrated the effects of disability on the family, 
including divorce rates, which sometimes rise, but may 
also decline. Also, Cogswell (1967) has shown that it is 
typical for predisability friendships to dissolve, an 
observation which is often confirmed in rehabilitation 
practice. In fact, in the early stages of social 
adjustment for paraplegics after their initial 
hospitalization, there is often a marked reduction of (a) 
social contacts; (b) frequency of entering community 
settings; and (c) the number of roles played. It has 
been suggested that much of the individual's 
predisability mode of social adjustment must be 
discarded before new roles can be assumed and a new 
adjustment worked out. 

It should be clear from this brief discussion that the 
social impact of disability is great enough in itself to 
have psychological consequences. However, disability 
also has some very direct effects on behavior which 
may produce a variety of emotional reactions and 
psychological adjustment problems. A useful way to 
determine the impact of disability is to measure the loss 
of the individual's capacity to perform certain behaviors. The 
greater the loss or impairment of behaviors produced 
by the disability, the greater the loss of reinforcers or 
rewards available to the individual, and the greater the 
need for social relearning. Thus, adjustment to 
disability can be defined in operational terms relating 
to old behaviors which must be discarded and new 
behaviors which must be learned. A behavioral 
approach to rehabilitation is helpful in focusing on 
what the individual must learn to do in order to live 
effectively with disability. 

PHASES OF ADJUSTMENT TO DISABILITY 

Considering the many negative effects of disability, 
it is only natural that the onset of a major disability will 
often be accompanied by significant emotional 
reactions. A useful way of describing the reactions that 
can occur is to examine the response to crisis occurring 
over time. Shontz (1965) has proposed a series of five 
phases of adjustment: shock, realization, defensive 
retreat, acknowledgement, and adaptation. Each of 



Psychosocial Adjustment 15 



these phases is accompanied by a characteristic 
emotional experience, and the predominant emotional 
reaction changes as a person progresses through the 
phases of adjustment. 

The phases and their accompanying emotional 
reactions, which are described in the following 
paragraphs, are not absolute and unvarying. The stage 
formulation provides a reasonable basis for categor- 
izing the behavior one often sees, but the counselor 
should realize that individuals' reactions are variable 
in time, intensity, and sequence, and some phases may 
occur in combination while others do not appear at all. 
Nevertheless, it is helpful to be alert to these reactions 
and sensitive to the different forms they may take, 
since they can affect progress in rehabilitation. 

Shock 

During the first few hours or days after onset of 
disability, the individual is usually feeling and reacting 
minimally and may have little awareness of what has 
happened. This initial phase is called shock and usually 
involves only muted emotional reactions. The 
immediate effects of serious physical illness or 
extensive injury may be part of the cause of shock. The 
counselor has little but friendship to offer during the 
shock phase, and that may be perceived by the client as 
irrelevant. 

Although counseling intervention usually has little 
direct impact at this time, it is very helpful for the 
counselor to meet the client (or patient, since this 
initial meeting will usually be in a hospital) and begin 
to establish a relationship. Because of the relative lack 
of emotional reaction, the client may be easier to 
approach at this time than later, when great fear, 
anger, depression, or other reactions can interfere with 
an initial contact. This early intervention should be 
directed toward laying the foundation for effective 
counseling later, especially since most clients move 
relatively quickly into the next phase. 

Realization 

Realization is the phase in which some recognition 
of the reality and seriousness of the disability begins to 
develop. Anxiety, possibly even panic, may be the 
predominant emotional reaction. This fear is based on 
anticipation of possible death, critical losses, or 
unpredictable change. Its extent may or may not be 
consistent with the seriousness of the disability, but it is 
important for the counselor to recognize that the client 
typically feels great fear. Depression and anger may 
also occasionally appear during this phase. 

The counselor can be helpful at this time by 
acknowledging and accepting the fear in an 
understanding way. Casual reassurance is likely to be 
rejected, but sincere reassurance by a counselor who 



has already begun to establish a relationship with the 
client will provide effective support for the client as he 
works through this crucial phase. 

Defensive Retreat 

The anxiety that normally follows onset of disability 
could easily overwhelm the individual if there were no 
defense mechanisms available to help him cope with 
the situation. Predominant among these defenses is 
denial. The person defends against the threat of 
disabilty by denying its existence or seriousness. Thus, 
the newly injured person may insist, "I am going to 
recover fully and walk out of this place," or, "Yes, I 
know my paralysis won't improve, but it won't make 
any difference." Such denial may persist or reappear 
occasionally long after onset of disability, usually in the 
form of the individual refusing to make reasonable or 
realistic allowances for the disability, or to accept the 
limitations imposed. 

The counselor must recognize that denial is serving 
an important psychological purpose for the disabled 
person. Denial can be one of the most difficult barriers 
a counselor may meet because of the important role it 
plays in helping the individual control or avoid 
anxiety. If the counselor has not previously established 
a relationship with the client, even suggesting the need 
for counseling at this point may be very threatening. 
For the client to accept help would be to acknowledge 
that something has happened and help is needed. 
Insisting that the disabled person begin planning for a 
changed future, or in some other way directly 
challenging the denial of reality, may lead to a total 
rejection of counseling that could delay or rule out 
effective intervention later. It is probably also 
important to avoid agreeing with the client and thereby 
reinforcing or encouraging denial, since that could 
strengthen it as much as direct confrontation. If denial 
is not interfering with the patient's active participation 
in his rehabilitation program, it is usually best to leave 
it alone. 

A very effective way for the counselor to handle 
denial is to work around it and try to alleviate the 
anxiety that produces it. The client should be 
informed, accurately and fully, what limitations and 
problems the disability creates, but this should be done 
in a supportive way, accompanied by realistic 
information about the possibilities of amelioration and 
rehabilitation. In other words, the bad news should not 
be avoided, but it need not be delivered without 
sensitivity. 

In the later stages of adjustment, a client who 
recognizes and accepts the maximum level of 
functioning that can reasonably be achieved may still 
exhibit hope for total cure. This hope should not be 
confused with denial. Hope may in part maintain a 
person's motivation for rehabilitation, and should not 



16 Athelstan 



be destroyed by the counselor. There is no cause for 
concern if the client is making plans and acting in a 
manner consistent with the limitations imposed by his 
disability and is making satisfactory progress toward 
reintegration into the community. 

Acknowledgment 

Acknowledgment refers to the phase in which the 
individual achieves an accurate understanding of the 
nature of the disability and the imposed limitations. 
Some persons may demonstrate a very thorough 
intellectual understanding of the disability prior to this 
time, but still not display full appreciation of its 
implications. Acknowledgment is usually marked by 
the onset of some degree of depression. 

The depression which often accompanies 
recognition of the reality and seriousness of a disability 
is a very natural grief reaction to the losses that result. 
The disabled individual is generally removed for 
prolonged periods from his customary environment, 
and may abruptly lose many desired activities and 
behaviors and, thus, their rewarding consequences. 
However, rehabilitation professionals frequently 
assume that there is an underlying pathology or "lack 
of motivation" at the base of any significant 
depression. Consequently, they tend to attribute 
responsibility for depression to the disabled individual 
and may lack initiative or imagination in finding ways 
to counter it. What is worse, by providing sympathy or 
attention, the professional may inadvertently 
strengthen a maladaptive pattern of behavior. 

On the other hand, professionals sometimes 
mistakenly insist that depression must occur. One 
occasionally hears the argument that if a person has not 
gone through a period of depression after the onset of 
disability, it must be due to adjustment being halted in 
the denial phase. The unfortunate implication of this 
argument is that the counselor must forcibly 
precipitate a depression so that adjustment can proceed 
to the next phase. In fact, there is no evidence that 
every person's adjustment must go through each of 
these phases, or that the phases must be experienced in 
any particular order. However, if the counselor has 
evidence that denial is present and significantly 
interfering with rehabilitation progress, it may be 
necessary to begin gently but firmly confronting the 
client with reality, even though this may precipitate 
anxiety and even some depression. Sensitive support 
that recognizes the stress the client feels, coupled with 
short-term goals successfully achieved, may well make 
it easier for the client to give up denial, make plans, 
and execute efforts that incorporate the disability. 

Adaptation 

Adaptation is the final phase of adjustment to 
disability. The term means simply that the individual 



has worked through any major emotional reactions to 
the disability, is realistic about his limitations, and is 
psychologically ready to make use of his potential. This 
is sometimes referred to as the "acceptance" phase, 
but it should be noted that accepting a disability does 
not imply a willingness to accept a diminished life or to 
be happy about being disabled. Rather, acceptance or 
adaptation means learning to live with certain limi- 
tations and to make the best use of remaining assets. 

FACILITATION OF ADJUSTMENT TO DISABILITY 

The emotional reactions and efforts to cope with 
disability shown by an individual will be determined 
by a combination of personal characteristics, learning 
history, and current circumstances. It is important for 
the counselor to avoid stereotyped approaches to the 
client. An athlete or manual laborer may reasonably be 
expected to experience a more adverse reaction to 
disability than a person whose work is mainly 
sedentary. However, many exceptions exist and the 
counselor is cautioned to set aside assumptions and 
perform a careful assessment of the present and 
potential functioning of each client in an effort to 
establish individual adjustment goals. 

Counseling the severely disabled must be regarded 
as a means of helping them overcome many of the 
obstacles to realizing their full potential and living 
effectively with their disabilities. Therefore, counseling 
is not done primarily to produce insight, to promote 
self- actualization, or to restructure the personality. It 
may be provided partly for emotional support and to 
promote emotional growth, but it is mainly directed at 
helping the client solve some of the practical problems 
of adjustment. The counselor must therefore be aware 
of the usual course of adjustment, and intervention 
should be designed to facilitate the client's movement 
along that course. 

Effective counseling is not likely to be limited to 
verbal interaction between the counselor and the 
client. It includes teaching, consultation, advising, and 
environmental manipulation, and may involve 
extensive work with many persons in addition to the 
disabled individual. 

Resocialization 

Often the first few months or even years after initial 
hospitalization appear to be relatively uneventful for 
the disabled person. Nevertheless, for many persons a 
subtle but critically important process of resocialization 
is taking place. Initially, after the onset of disability, 
there may be a period of apparent decline in social 
functioning as the client discards predisability roles 
and relationships. Later, the person begins a gradual 
process of developing new relationships and 
experimenting with new roles. Then gradually he 
begins to venture out into the community again, first 



Psychosocial Adjustment 17 



visiting relatively nonthreatening, anonymous public- 
places which are easy to leave. Subsequent outings 
generally involve increasing risk due to greater 
intimacy of the settings and the increased difficulty of 
leaving abruptly. Usually the last steps in the 
resocialization process involve settings like private 
parties, public places such as bars, and places of 
employment. 

There are a number of things the counselor can do to 
promote resocialization. Before developing a 
resocialization plan, it is important for the counselor to 
determine the client's predisability social patterns. 
Since pre-existing social problems are more resistant 
to change than new disability -related problems, the 
counselor should differentiate between longstanding 
characteristics and problems occurring as a direct 
result of the disability. After determining the client's 
pre-existing social involvement, the counselor's efforts 
are best directed toward environmental manipulation 
rather than traditional counseling strategies. 

The first thing the counselor should do to facilitate 
resocialization is to determine by observation and 
interview the specific social activities the client 
currently participates in. Then the counselor can judge 
what stage in the resocialization process the client has 
reached and what a reasonable next step would be. 
Since the stages are arranged in a hierarchy of 
difficulty, it is important that the counselor not 
encourage the client to progress too far in any one step. 
In addition, the client's efforts should be directed 
toward one area of resocialization at a time, such as 
development of an avocational activity or an 
independent living arrangement. 

The counselor should define manageably small steps 
and assist the client directly, if necessary, in making 
the arrangements that will result in progress toward the 
stated goals. For example, the first contact with the 
community that some severely disabled persons might 
undertake could involve a visit to the counselor's 
office. If even that step proves to be too great at first, 
the counselor may want to arrange a home visit in 
order to establish some rapport with the client and 
lower the barriers to the initial effort. After a visit to 
the counselor's office, the next step may be to have 
social contacts with other disabled persons. This could 
be accomplished by encouraging the client to attend a 
club or special interest group for the handicapped. It is 
important for the client to know that he can achieve 
each step, and that the culmination of the steps is a goal 
he wants to attain. If there is something desirable to be 
gained from each step, and if the requirement for social 
and physical effort is minimized, the client is more 
likely to succeed in the plan. 

Later, more demanding steps, such as enrolling in 
school, may be taken. Enrollment in school might be 
intended to provide the client with a marketable 
vocational skill; to provide a basis for supportive 
retraining, even if the vocational objective is initially in 



doubt; or in some cases, to provide resocialization as 
an end in itself. If the counselor recognizes the 
progressive and hierarchical nature of resocialization 
and is willing to be flexible and do whatever is 
necessary to accomplish these steps, it should be 
possible to start with a client at any stage and produce 
movement to whatever ultimate step may be set as a 
goal. 

Timing 

In order for the counselor's intervention to be 
effective, he must appreciate that a considerable 
amount of time might be required for the rehabilitation 
plan to be completed, and thus realize that progress in 
resocialization and other aspects of adjustment can be 
painfully slow. Although some clients might adapt and 
adjust to disability relatively quickly, others might 
return to work after intervals exceeding 10 years after 
onset of disability and, naturally, others not at all. 

Timing of counseling intervention is important 
mainly in relation to the readiness of the disabled 
individual to work toward rehabilitation goals. This 
readiness will vary over time. In an early contact, the 
rehabilitation counselor may find the disabled person 
mainly engaged in readjusting roles, struggling to 
"accept" the disability or "still trying to get used to 
it." Although these efforts may involve a very active 
process of adjustment, the disabled person may see this 
as a period in which restriction of his social life is the 
only obvious change taking place. Unless the counselor 
is sensitive to this process, neither he nor the client is 
likely to realize that anything significant is happening, 
and the client may report that he is "not ready" to 
undertake planning or other rehabilitation efforts. This 
apparent lack of readiness may persist over several 
contacts and, as a result, the counselor may finally 
mistakenly close the case due to the client's lack of 
motivation. 

If, instead of closing the case, the counselor realizes 
that the seemingly uninterested client may be presently 
preoccupied with social and psychological adjustment 
processes, two avenues of effective intervention are 
open. The first is active intervention and provision of 
services explicitly aimed at facilitating psychosocial 
adjustment without reference to vocational goals. A 
second possibility is assuming a more passive stance 
and simply recontacting the client periodically over an 
extended period of time, waiting for "readiness" to 
develop. This approach can be more effective than is 
immediately apparent if the counselor takes care to 
make the personal contacts supportive, non- 
threatening, and reasonably comfortable for the 
client. When the client does mature to the point of 
being ready for professional assistance, these services 
are much more accessible than would be the case if a 
new contact had to be self- initiated at that time. 

Only two demands are placed on the counselor who 



18 Athelstan 



takes this approach: first, he must take the little time 
needed, once, twice, or four times a year, to visit with 
the client; and second, he must persuade the 
counseling supervisor that this strategy can be effective 
and that the prospects for success warrant keeping a 
case open with no change in status for a longer period 
than usual. The result of this strategy can be a sudden 
and remarkable surge of "motivation" and 
rehabilitation progress on the part of a client who may 
have appeared for quite some time to have had limited 
rehabilitation potential. 

The psychological and social phenomena described 
in this chapter and the techniques suggested for 
facilitating adjustment represent a synthesis of research 
and extensive professional practice. It is hoped that the 
guidelines and suggestions presented here will assist 
rehabilitation counselors in the course of working with 
their most severly disabled and most challenging 
clients. 

BIBLIOGRAPHY 

Albrecht GL (ed): The Sociology of Physical Disability and 
Rehabilitaton . Pittsburgh, University of Pittsburgh Press, 
1976. 

This book contains 11 major articles presenting 
psychological and sociological conceptualizations of issues 
and problems in rehabilitation. Although mainly 
academically oriented, the papers include much 
information of practical value to the rehabilitation 
counselor. WE Fordyce's chapter "A Behavioral Per- 
spective on Rehabilitation" presents, a behavioral 
formulation of disability, from which the reader can glean 
practical suggestions for the application of behavioral 
techniques in rehabilitation. 

Athelstan, GT: A follow-up study of the psychological, social 
and vocational adjustment of spinal cord injured adults. In 
the Fifteenth Annual Progress Report of Rehabilitation 
Research and Training Center No. 2, Minneapolis, 
University of Minnesota, 1976. 

Since this research is still in progress, its findings have not 
yet appeared in the formal literature. However, the annual 
progress reports, particularly the report of 1976, contain 
much useful descriptive information on a large sample of 
persons who have been spinal cord injured for a long 
period of time. 

Cobb AB: Medical and Psychological Aspects of Disability. 
Springfield, IL, Thomas, 1973. 

Written mainly for the vocational rehabilitation counselor, 
this book is a collection of papers by physicians and 
psychologists. Its purpose is to familiarize the professional 
practitioner with the principal medical and psychosocial 
aspects of 12 different disabilities, such as heart disease, 
cerebral palsy, amputations, epilepsy, deafness, and 
blindness. 

Cogswell BE: Rehabilitation of the paraplegic: Processes of 
socialization. Sociological Inquiry 37: 1 1-26, 1967. 



This is a report of a rather intensive study of the postinjury 
social adjustment of a small group of spinal cord injured 
men. A conceptual formulation of the process of 
resocialization is presented. 

Malikin D, Rusalem H (eds): Vocational Rehabilitation of the 
Disabled: An Overview. New York, New York University 
Press, 1969. 

Just as the title states, this book provides an overview of the 
field of vocational rehabilitation: its history and legislative 
basis, philosophical viewpoints, the system, the process, 
and issues of practice and technique. 

Nagi SZ, Clark DL: Factors in marital adjustment after 
disability. Journal of Marriage and the Family 
26:215-216, 1964. 

A short article summarizing the factors that appear to 
influence marital stability after physical disability. Some of 
the factors are age, education, and occupation. 

Neff WS (ed): Rehabilitation Psychology. American 
Psychological Association, Inc., 1200 Seventeenth Street 
NW, Washington, D.C. 20036, 1971. 
This book consists of a set of papers presenting the state of 
the art in rehabilitation psychology, especially in the last 
12 years. Many different points of view are presented. 

The Severely Disabled. A Special Issue of the Rehabilitation 
Counseling Bulletin, the Journal of the American 
Rehabilitation Counseling Association, 18, No. 4, 1975. 
Designed to assemble current information relating to 
vocational rehabilitation of the severely disabled, this issue 
has 12 articles reporting recent research, analysis of issues, 
and new program developments. 

Shontz FC: Reactions of crisis. Volta Review 67:364-370, 
1965. 

This paper provides a conceptual view of the stages in the 
process of adjustment to crisis and a framework for 
understanding the emotional reactions that may 
accompany disability. 

Wright BA: Physical Disability — A Psychological Approach. New 
York, Harper and Row, 1960. 

This book presents a very thorough social -psychological 
analysis of disability and suggestions of techniques that the 
rehabilitation counselor can use to facilitate achievement of 
rehabilitation goals. 



3 SIGNIFICANT BODY SYSTEMS 



Joel DeLisa, M.D. 
Walter C. Stolov, M.D. 



NERVOUS SYSTEM 

For descriptive purposes, the nervous system is 
divided into the central nervous system (CNS) and the 
peripheral nervous system (PNS). The CNS consists of 
the spinal cord and the brain, namely, the cerebrum, 
cerebellum, and brain stem, and the PNS consists of 
the cranial and spinal nerves. The autonomic nervous 
system is somewhat specialized and may be viewed as 
part of both the CNS and the PNS. Sometimes the 
term somatic nervous system is applied to those parts of the 
CNS and PNS under voluntary control and associated 
with the extremities and structures of the body wall 
(e.g., skin, bones, joints, and skeletal muscle). In 
contrast, the involuntary autonomic nervous system is 
associated with internal organs, blood vessels, smooth 
muscle, and cardiac muscle. 

The main functions of the nervous system are (a) to 
receive sensory information from the environment and 
the body itself through various receptors and to 
transmit this information to the brain (afferent system); (b) 
to send impulses down the spinal cord and out the peri- 
pheral nerves to the various end organs to effect a motor 
response (efferent system); (c) to integrate the information 
obtained from all the receptors. An understanding of the 
nervous system is particularly relevant to chapters 5,6,7, 
8, 9, 10, 11, 12, 15, 20, 24, and 27. 

The basic functional unit of the nervous system is 
the neuron, which is responsible for transmitting this 
information to and from the brain. The neuron 
consists of a cell body and two distinct types of 
processes, the numerous dendrites and a single axon (fig. 
3-1). The terms "axon" and "nerve fiber" are 
synonymous. Most axons are long and encased in a 
sheath of fat -like substance called myelin which acts as 
an insulator and aids in the rapid transmission of the 
nerve impulses away from the cell body. Dendrites are 
short, branched processes which receive impulses from 
the terminal branches of axons of other neurons and 
conduct them toward the cell body. The cell body 
contains the cell nucleus and is responsible for 
maintaining the health of the neuron. 

The length of the path traveled by afferent or 
efferent information is longer than any single axon. 
Therefore, chains of neurons are necessary to convey 



Cell body 



Terminal 
branches 
of axon 




Dendrites 

FIGURE 3-1. Basic myelinated nerve cell. Dendrites receive 
input from terminal branches of other axons. When sufficiently 
activated, the cell bodv transmits an electrical impulse down its 
axon. Myelin protects the axon and, together with nodes of 
Ranvier, allows for verv fast conduction. Nonmyelinated, 
slower-conducting axons also exist. Axon diameters range from 
1 to 60 xlO~ J cm. Arrow indicates direction of impulse travel. 

messages through the entire nervous system. The 
message is an electrical impulse (action potential) 
produced by a chemical change that progresses along 
the axon. The system for transfer from one neuron to 
the next is the synapse (fig. 3 -2). The synapse is the site 
at which the axon of one neuron ends near the dendrite 
or cell body of the next neuron. Here the electrical 
impulse in the axon causes the release of a chemical such 
as acetylcholine or dopamine, and this, in turn, causes a 
sequence of chemical events that results in an impulse in 
the next neuron. 

Nerve cell bodies are usually located in groups. 
Within the brain and spinal cord these groups are 
called nuclei and constitute the gray matter, so-called 
because they have a grayish color. Outside the brain 
and spinal cord the groups are called ganglia. The 
remaining areas of the nervous system consist 
primarily of tracts of many axons which constitute the 
white matter, so-called because the myelin sheaths have 
a whitish color. Tracts that carry information of a 
specific type, such as pain, touch, or vision, usually 
have specific names. 

CENTRAL NERVOUS SYSTEM 

The cerebrum, cerebellum, and brain stem are 
contained and protected by the skull. This complex 



19 



20 DeLisa and Stolov 



mass of nervous tissue contains over seven billion 
nerve cells. The spinal cord is contained and protected 
by the vertebral column. All four structures have three 
membranous coverings known as meninges which afford 
further protection. From the outside in, these are the 
dura mater, arachnoid mater, and pia mater (fig. 3-3). The 
CNS literally floats in a bath of cerebrospinal fluid 
which occupies the space between the arachnoid mater 
and pia mater [subarachnoid space). The CNS is also 
cushioned internally by the cerebrospinal fluid located in 
cavities (ventricles) within the cerebrum and brain stem 
(see fig. 3 - 5 and 3 - 6). The fluid also serves as a medium 
through which nutrients and wastes can be exchanged 
between the blood and the CNS. 

Cerebrum 

The cerebrum, consisting of two cerebral 
hemispheres, is the largest part of the brain and 
represents about seven -eighths of the total weight of 
the CNS. The surface of these two cerebral 
hemispheres has numerous convolutions gyri. 
Between gyri lies an intervening furrow, the shallow 
sulcus. A very deep sulcus is a. fissure (fig. 3-4). The 
outer surface of the cerebral hemispheres consists of a 
thin layer of gray matter, the cortex. The interior 
portion of the cerebrum consists of white matter, 
tracts, and nuclei (gray matter) where synapses occur. 
Different lobes of the hemispheres have different 
functions, and the cortex varies in structure from 
region to region depending upon the varying function. 
The bones of the skull have the same names as the 
lobes lying beneath them. 

The two cerebral hemispheres are "twins," each 
with centers for receiving sensory (afferent) 
information and for initiating motor (efferent) 
responses. The anatomical structures of both 
hemispheres have the same names. Certain intellectual 
functions are concentrated in either the right or the left 
hemisphere (see chaps. 9 and 10). The left cerebral 
hemisphere mostly receives afferent input from, and 
initiates efferent activity on, the right side of the body, 
and the right cerebral hemisphere mostly receives 
afferent input from, and initiates efferent activity on, 
the left side of the body. 

Frontal lobe. The frontal lobe is the anterior part 
of each hemisphere and contains the nerve cells (Betz 
cells) that initiate the impulses which travel through 
one or more synapses to produce fine, isolated, and 
versatile muscle and extremity movement. These cells 
are referred to as the upper motor neuron for motor 
activity. The Betz cells are spatially organized in the 
precentral gyrus of the frontal lobe, often referred to as 
the motor strip (fig. 3-4). Their axons pass downward to 
the spinal cord first as the internal capsule and then as the 
pyramidal tract. Anterior to the motor strip are areas that 
are felt to deal with higher intellectual functions such as 




FIGURE 3-2. Schematic representation of a svnapse. (A) Single 
axon synapsing with a single cell bodv. (B) Two axons synapsing 
with a single cell body. (C) One axon synapsing with two cell 
bodies. Arrows indicate direction of impulse travel. 



Anterior root 



Posterior 
root ganglion 



Spinal nerve 



Dura mater 




Arachnoid 
mater 



Pia mater 

overlying 

spinal cord 



Spinal cord 



FIGURE 3-3. Posterior aspect of spinal cord showing posterior 
spinal roots and the coverings. Dura matercontinues into sleeve 
of spinal root. Cerebrospinal fluid is located between the 
arachnoid mater and the pia mater. Note that a spinal root is 
really several rootlets. 

abstract thinking and judgment. The cell bodies of the 
neurons of the first cranial nerve, the olfactory, are in 
the base of the frontal lobe. 

Parietal lobe. The parietal lobe is the middle 
portion of each hemisphere (fig. 3-4). The postcentral 
gyrus is the major sensory receptive area for the highest 
integration and coordination of afferent information 
dealing with pain, temperature, form, shape, texture, 
pressure, and position. Some memory functions are 
also subserved by the parietal lobe. 



Significant Body Systems 21 



Postcentral gyrus 
Central sulcus 



Precentral gyrus 




FIGURE 3-4. A left cerebral hemisphere and cerebellum show- 
ing the distribution of the cerebral cortex of the frontal, parietal, 
temporal, and occipital lobes. The central sulcus divides the 
precentral gyrus from the postcentral gyrus, and hence the 
frontal lobe from the parietal lobe. The location of Wernicke's 
area over the temporal and parietal lobes and Broca's area over 
the frontal and temporal lobes is shown. 



Temporal lobe. The temporal lobe is under the 
frontal and parietal lobes (fig. 3-4). Its cortex is the 
primary area where auditory stimuli are received. It is 
also one of the centers for dreams, memory, and 
emotions. 

Occipital lobe. The occipital lobe is in the posterior 
part of each hemisphere (fig. 3-4). Its cortex is the 
primary area where visual stimuli are received. (See 
p. 48 on the optic nerve and the nerve pathways for 
vision.) 

Basal ganglia. The basal ganglia are aggregates of 
cell bodies (gray matter) located within the cerebral 
hemispheres (fig. 3-5). These ganglia constitute part 
of the extrapyramidal system which is concerned with 
postural adjustment and gross voluntary and 
automatic movements, as opposed to the fine 
movements controlled by the motor strip of the frontal 
lobe. The basal ganglia receive afferent fibers from the 
cerebral cortex and the thalamus. The axons from 
the basal ganglia synapse in the brain stem and then 
again at the spinal cord. The extrapyramidal system is 
part of an elaborate feedback system for controlling 
movement. Close to the basal ganglia lies the internal 
capsule which contains the important efferent motor 
tracts descending from the frontal lobe on their way 
to the spinal cord. 

Thalamus. The thalamus is a large, bilateral, egg- 
shaped mass of gray matter also located deep and near 
the midline of the cerebral hemispheres (fig. 3-5). It is 
the main synaptic relay center for sensory messages as 



they travel from the peripheral sensory receptors to the 
sensory areas of the postcentral gyrus of the parietal 
cortex. 

Hypothalamus. The hypothalamus is a collection 
of ganglia located below the thalamus and intimately 
associated with the pituitary gland (fig. 3-5). It has a 
variety of crucial functions: 

1. It controls the autonomic system and hence 
regulates parasympathetic and sympathetic 
function (see below). 

2. It is part of the pathway by which emotions 
influence body functions. 

3. It secretes hormones influencing the posterior 
pituitary gland for maintaining body water 
control. 

4. It secretes hormones that influence the anterior 
pituitary gland's release of sex, thyroid, and 
adrenal stimulating hormones. 

5. It is part of the arousal mechanism for 
maintaining the waking state. 

6. It is an essential part of the mechanism for 
regulating appetite. 

7. It is crucial for the maintenance of normal body 
temperature. 

Language areas. Language function requires an 
integration of visual and auditory afferent input 
(receptive function) with efferent motor output 
(expressive function). The frontal, parietal, and 
temporal lobes are involved. The language function is 
located in the left hemisphere in essentially all right- 



22 DeLisa and Stolov 



Connections to lateral ventricles 
Right Left 



Internal capsule 



Fissure 
of Sylvius 



Region of 
hypothalamus 



4th ventricle 




Thalamus 
Putamen 
Globus pallidus 

3rd ventricle 
Pons 

Pyramidal tract 



Cerebellum 



Medu 

FIGURE 3-5. Schematic section through the cerebrum, pons, and medulla demonstrating the deep 
basal ganglia and the four ventricles. Note the thalamus on either side of the third ventricle, the 
location of the fourth ventricle, and the pyramidal tract originating in the precentral gyrus passing its 
fibers through the internal capsule and crossing over to the opposite side of the medulla. The 
hypothalamus is located below the basal ganglia in the region of the third ventricle. The Fissure of 
Sylvius is a deep sulcus that separates the parietal lobe from the temporal lobe. 

Subarachnoid space 



Arachnoid 



Dura mater 




3rd ventricle 



Cerebrospinal fluid 



4th ventricle 



FIGURE 3-6. Midline section through the cerebrum, cere- 
bellum, and brain stem. This section passes through the third 
and fourth ventricles, both of which are midline structures. 
Note cerebrospinal fluid bathing the brain. 



Significant Body Systems 23 



Frontal lobe 




CRANIAL NERVES 
I Olfactory 

II Optic 

III Oculomotor 



IV Trochlear 

V Trigeminal 



VI Abducens 

VII Facial 

VIII Acoustic 

IX Glossopharyngeal 

w 



FIGURE 3-7. Underside view of the brain showing the brain stem and the underside of the frontal 
lobe. Cranial nerves I through XII are exposed in this view. 



handed individuals and about 85 percent of all left- 
handed individuals. The receptive function is largely 
centered over the parietal and temporal cortex (fig. 
3-4) and is called Wernicke's Area, Two areas of the 
frontal cortex contribute to the expressive function. 
The main one, Broca's Area, is located just anterior to 
the temporal lobe (fig. 3-4). All of these areas are 
intimately connected to each other by nerve fibers. 
Higher mental and emotional processes, such as 
memory and learning, require many interconnections 
between areas of cortex within a single hemisphere, or 
between the two hemispheres. The term association areas 
is given to the regions where interconnections occur. 

Cerebellum 

The oval -shaped cerebellum is located under the 
occipital lobe and posterior to the brain stem (fig. 3 - 4). 
Like the four lobes of the cerebrum, the cerebellum has 
an outer cortex of gray matter with numerous sulci and 
gyri. It also has right and left hemispheres and a 
central section. Almost all information to and from the 
cerebellum is transmitted by way of the midbrain of the 
brain stem. The cerebellum has three main functions: 

1 . Maintenance of equilibrium and balance of the 
trunk. Afferent input comes from the vestibular 



portion of the eighth cranial nerve. Efferent 
messages leave the cerebellum and connect with 
the reticular formation of the brain stem 
concerned with vestibular function 
(equilibrium). 

Regulation of muscle tension, spinal nerve 
reflexes, and posture and balance of the limbs. 
Afferent information arrives from muscles and 
tendons of the limbs. Efferent messages synapse 
in the brain stem to influence the extrapyramidal 
system to effect fine motor control. 
Regulation of the coordination of fine limb 
movements originally initiated by the frontal 
lobe. Afferent information comes from the 
cerebral cortex via the pons of the brain stem. 
Efferent information goes back to the cerebral 
cortex via the thalamus for fine motor control. 



Brain Stem 

The brain stem is interposed between the cerebrum 
and the spinal cord. It contains the midbrain (cerebral 
peduncles), the pons, and the medulla oblongata (fig. 
3-6). Of these three, the medulla is perhaps the most 
important. Vital centers for cardiac function, 
respiratory function, and control of the diameter of 



24 DeLisa and Stolov 



TABLE 3-1 
CRANIAL NERVES AND THEIR FUNCTIONS 



Nerve Number 



Name 



Function 



i 

ii 

in 

IV 

V 

VI 
VII 

VIII 

IX 



XI 



XII 



Olfactory 1 

Optic 2 

Oculomotor 

Trochlear 
Trigeminal 

Abducens 
Facial 



Vestibulocochlear 
(acoustic) 

(a) Vestibular portion 

(b) Cochlear portion 

Glossopharyngeal 



Vagus 

Spinal accessory 
Hypoglossal 



Sense of smell 

Sense of vision 

Movement of eye up, down, and 
in toward the nose; constriction of 
the pupil 

Movement of eye down and out 

Muscles of mastication; sensation 
of skin of face, teeth, and lining 
of mouth and nose 

Movement of eye outward 

Muscles of facial expression; taste 
to the anterior 2/3 of tongue; 
salivary glands secretion 



Equilibrium and balance 
Sense of hearing 

Swallowing and speech sounds; 
taste to the posterior 1/3 of tongue 

Swallowing and speech sounds; 
heart rate; gastrointestinal 
movement 

Neck muscles (trapezius and 
sternocleidomastoid) 

Tongue movement; speech; 
swallowing movement 



■From frontal lobe 
2 From occipital lobe 

small arteries, and hence blood pressure, are located in 
the medulla. Reflexes controlling heart rate and 
breathing rate are centered here, as well as reflexes 
involved with vomiting, coughing, sneezing, and 
swallowing. The pons also participates in control of 
respiration and, together with the midbrain, is a reflex 
center for eye pupil reflexes and other eye movements. 
Also scattered throughout the brain stem are cell 
masses termed the recticular formation, which is 
associated with initiating and maintaining wakefulness 
and alertness generally. Further, nerve fiber tracts to 
and from the cerebrum and spinal cord must pass 
through the brain stem. 



All the functions of the brain stem are intimately 
associated with cranial nerves III-XII. The cell 
nuclei for these nerves are located in the brain stem 
(fig. 3-7). Unlike the cerebrum, where the left 
hemisphere controls right body movements and the 
right hemisphere controls left body movements, the 
cranial nerves on the left control the left side, and those 
on the right control the right side. Table 3 -1 lists the 12 
cranial nerves and their functions. 

Blood Supply to the CNS 

The blood supply to the brain is derived from the 



Significant Body Systems 25 



Frontal lobe 



Anterior communicating 

Middle cerebral 
Internal carotid 
Posterior communicating 
Posterior cerebral 




FIGURE 3-8. Underside view of the brain demonstrating the basilar arterial system, the carotid 
arterial system, and the interconnections through the Circle of Willis. Note the take-off of the main 
branches, namely the anterior, middle, and posterior cerebral arteries. The anterior spinal artery 
branches off the vertebral artery to carry blood down into the spinal cord. 



two internal carotid arteries and the two vertebral arteries (see 
fig. 3-26, p. 41). The two vertebral arteries enter the 
CNS between the skull and the first cervical vertebra. 
They join to form the basilar artery which continues up 
toward the cerebral hemispheres in front of the brain 
stem (fig. 3-8). The two internal carotid arteries enter 
the skull through two holes at its base. 

On the underside of the frontal and temporal lobes, 
the carotid and basilar systems connect together as the 
Circle of Willis. This interconnection (anastomosis) 
allows the two systems to share blood, an important 
function. From the Circle, and mostly from the carotid 
system,, arise the anterior and middle cerebral arteries. 
The posterior cerebral artery is derived mostly from 
the basilar system. 

Figure 3-9 demonstrates that the middle cerebral 
artery is the most important because it supplies the 
cortex of all four lobes. In particular, it supplies the 
precentral and postcentral gyri, the most important 
areas for movement and sensation in the frontal and 
parietal lobes, and also the cortex areas associated with 
language and vision. Figure 3-9 also shows that the 



anterior cerebral artery supplies the inner surface of 
the frontal and parietal lobes, and the posterior 
cerebral artery supplies part of the temporal and 
occipital lobes. The blood supply to the brain stem and 
the cerebellum comes mostly from branches of the 
basilar system. 

The blood supply to the spinal cord comes from the 
vertebral arteries which connect to form the anterior 
spinal artery (fig. 3-8), as well as from branches off the 
aorta at the level of the chest and abdomen. 

Spinal Cord 

Afferent information from the trunk and the 
extremities enters the CNS through the spinal 
peripheral nerves at various levels of the spinal cord. 
To reach the cerebrum, cerebellum, and brain stem, 
impulses travel up the spinal cord in fairly distinct 
tracts of nerve fibers. Similarly, to effect trunk and 
extremity movement, efferent impulses from the 
cerebrum, cerebellum, and brain stem also travel in 
distinct tracts within the spinal cord. 



26 DeLisa and Stolov 



Well-defined areas of gray matter are also contained 
within the spinal cord where synapses occur for 
transmission of these afferent and efferent impulses. 
Anterior projections of the gray matter, termed ventral 
or anterior horns, are the location of the final synapse for 
efferent impulses leaving the spinal cord. The posterior 
projections, termed dorsal or posterior horns, are the 
location of many initial synapses for afferent 
information entering the spinal cord. 

Figure 3-10 shows a cross -section of the spinal cord 
at a typical level, demonstrating the organization of 
anterior and posterior horns and three main 
tracts — the spinothalamic tract, which carries pain and 
temperature impulses to the thalamus for synaptic relay 
to the postcentral gyrus of the parietal lobe; the posterior 
columns, which carry position sense and pressure sense 
impulses to the thalamus, also for relay to the parietal 
lobe; and the corticospinal or pyramidal tract, which 
carries impulses from the precentral gyrus through the 
internal capsule (see fig. 3-5) of the frontal lobe to 
initiate muscle activity. These upper motor neurons 
synapse in the anterior horns with cells of the lower 
motor neurons whose axons leave the spinal cord via the 
spinal nerves. 

PERIPHERAL NERVOUS SYSTEM 

Somatic Nervous System 

At the spinal level, the peripheral nervous system 
begins with the spinal nerves. Corresponding to each 
of the vertebrae there is a pair of spinal nerves, one for 
each side. A total of 30 pairs exist (8 cervical, 12 
thoracic, 5 lumbar, and 5 sacral). Each nerve has two 
roots, the anterior, or ventral, motor root, and the 




Middle cerebral arter 




Anterior cerebral artery 
Posterior cerebral artery 

FIGURE 3-9. Distribution of anterior, middle, and posterior 
cerebral arteries. (A) Lateral (outer) aspect of left cerebral 
hemisphere showing middle cerebral artery serving frontal, 
parietal, temporal, and occipital lobes. (B) Medial (inner) aspect 
showing anterior cerebral artery serving frontal and parietal 
lobes, and posterior cerebral arterv serving occipital and 
temporal lobes. 



Posterior column 



Posterior horn 



Corticospinal tract 



Spinothalamic 
tract 



White matte 



Anterior horn 



Posterior root 



Sensory neuron 

Cell body 




Spinal nerve 



Motor neuron 



Synapse 



Anterior root 



FIGURE 3-10. Cross-section of spinal cord showing the anterior and posterior horns of gray matter 
and three tracts: the corticospinal, spinothalamic, and posterior columns within the white matter. 
The afferent (sensory) and efferent (motor) neurons of a monosynaptic reflex arc and the single 
synapse can be seen. Arrows show direction of impulse. 



Significant Body Systems 27 



posterior, or dorsal, sensory root. The anterior root 
contains the axons of the cell bodies in the anterior horn 
within the spinal cord. The posterior root has its cell 
bodies outside, but close to, the spinal cord in the sensory 
nerve ganglion. The posterior root, therefore, actually has 
two axons (fig. 3-10). 

Each spinal nerve is numbered to correspond to the 
number of the vertebra near which it exits from the 
vertebral canal. The vertebral canal, however, is 
longer than the spinal cord itself. Since there are essen- 
tially the same number of spinal nerve pairs as there 
are vertebrae (except for the cervical segments where 
there are eight nerves and only seven vertebrae), some 
of the spinal nerves, particularly at the lower levels, 
have to travel down a significant distance before ac- 
tually leaving the vertebral canal. In particular, begin- 
ning with the L2 vertebral level and extending 
downward to the S5 vertebral level, spinal nerves have 
to travel an increasing distance within the vertebral 
canal below the end of the spinal cord before they exit. 
From the L2 vertebral level downward, therefore, up 
to eight pairs of spinal nerves occupy the space within 
the vertebral canal. This nerve complex is referred to 
as the cauda equina (horse's tail). 

All the cervical spinal nerves control movement of 
the shoulder and arm muscles, and receive all the sen- 
sory input from the skin, muscles, bones, and joints of 
the upper extremities where special receptors exist. 
After leaving the vertebral canal, they combine in a 
special way. Out of this merger, new peripheral nerves 
are formed, each of which may contain axons that 
originated from cell bodies at more than one spinal 
level. The merger of the cervical nerves in the neck 
region is called the brachial plexus. Table 3-2 lists the 
functions of the main peripheral nerves of the brachial 
plexus. 

The thoracic spinal nerves do not recombine into 
plexuses. The anterior roots of the thoracic spinal 
nerves control the muscles of the abdomen and the 
back, and the posterior roots receive sensory informa- 



tion from the abdominal organs, and the skin, muscles, 
and joints of the chest, abdomen, and upper back. 

The lumbar and sacral nerves, like the cervical 
nerves, also combine after leaving the vertebral canal. 
This merger, which occurs mostly in the pelvis, is 
called the lumbosacral plexus. The lumbosacral plexus 
governs movement of, and receives sensory 
information from, the lower extremities, bladder, and 
anal sphincter. Table 3 - 3 lists the functions of the 
main peripheral nerves of the lumbosacral plexus. 

A reflex is an automatic involuntary action of a part 
of the body in response to a particular stimulus. 
Reflexes serve a very important protective function for 
the body (e.g., the immediate withdrawal of a hand 
from a hot surface is a reflex action). A reflex has an 
afferent component, a nerve carrying the information 
of the stimulus toward the CNS, and an efferent 
component, a nerve carrying the impulses away from 
the CNS to effect the action. If only a single synapse 
exists between the afferent and efferent nerve, the 
reflex is termed a monosynaptic reflex (fig. 3-10). If 
several nerves, and hence synapses, are interposed 
between the afferent and efferent nerve, the reflex is 
termed a. polysynaptic reflex (fig. 3-11). 

Reflexes are not dependent for their existence on the 
cerebrum, cerebellum, or brain stem. However, nerve 
impulses from these centers exert a controlling 
influence. 

The simplest spinal reflex is the tendon (stretch) 
reflex, a monosynaptic reflex. When the tendon is 
tapped, a sensory receptor in the muscle is excited and 
an afferent impulse travels in a sensory nerve to the 
spinal cord, where it enters through the posterior root. 
The sensory nerve axon synapses with an anterior horn 
cell at the same level in the spinal cord as it entered. 
The anterior horn cell axon carries the impulse 
through the anterior root of the spinal nerve and 
travels to the muscle whose tendon was tapped, 
causing the muscle to contract. A simple reflex, 
therefore, has an afferent limb (the fiber conducting the 



TABLE 3-2 
MAIN ACTION AND SENSATION FUNCTION OF BRACHIAL PLEXUS NERVES 



Peripheral Nerve 



Action 



Skin Sensation 



Axillary 

Musculocutaneous 

Radial 



Flex and abduct shoulder 

Flex elbow 

Extend elbow, wrist, 
and fingers 



Shoulder 

Front of forearm 

Back of hand and 
forearm 



Median 



Flex wrist and fingers 



Palm and thumb, index 
and middle fingers 



Ulnar 



Dexterity of fingers 



Palm and little finger 



28 DeLisa and Stolov 



White matter 



Gray matter 



Sensory neuron 




Motor neuron 



FIGURE 3-1 1. Cross-section of spinal cord showing example of a polysynaptic reflex. The impulse in 
the afferent sensory neuron synapses with a second neuron (interneuron), wholly contained within 
the gray matter, which then subsequently synapses with the efferent motor neuron to deliver the 
action response to the muscle in the periphery (compare with fig. 3-10). More than one interneuron 
mav exist in a polysynaptic reflex. Such interneurons can also transmit an impulse up or down 
several levels in the spinal cord. 



tendon -tap impulse), a single synapse within the spinal 
cord, and an efferent limb (the axon conducting the impulse 
to the muscle to produce contraction; fig. 3-10). 

Reflexes also exist which govern the action of the 
various body organs. The dominant influence of the 
higher centers is to inhibit reflexes from being too 
active. Thus, if the spina] cord is anatomically 
separated from the higher centers, reflexes below the 
level of the separation will be hyperactive. 

Autonomic Nervous System 

The autonomic nervous system, while intimately 



connected to the brain and spina] cord, is basically a 
peripheral nervous system. It is fundamentally a 
system not under voluntary control, and it functions to 
regulate the activities of structures such as the stomach 
and intestines, the heart, the smooth muscle around 
arteries, the sweat glands, the salivary glands, and the 
bladder. The autonomic nervous system consists of two 
subsystems, the sympathetic and the parasympathetic. As 
table 3-4 indicates, the two systems are usually 
antagonistic. 

Sympathetic nerve fibers leave the spinal cord 
through the anterior roots of all the thoracic spinal 
nerves and the first three lumbar spinal nerves. After 



TABLE 3-3 

Main action and Sensation Function 
of lumbosacral plexus nerves 



Peripheral Nerve 



Action 



Skin Sensation 



Femoral 

Sciatic 
Tibial 
Peroneal 

Pudendal 



Extend knee 

Flex knee 

Flex ankle and toes 

Extend ankle and toes 



Anal and urethral 
sphincters and erection 



Front of thigh, and 
inside of leg 



Sole of foot 

Outside of leg and 
instep of foot 

Skin around genitals 
and anus 



Significant Body Systems 29 



Sympathetic trunk 



Postganglionic 
sympathetic neuron 




Preganglionic 
sympathetic neuron 



FIGURE 3-12. Cross-section of spinal cord with demonstration of the preganglionic and post- 
ganglionic neurons of the sympathetic nervous system. Note the synapse in the sympathetic ganglion 
and the postsynaptic neuron carrying the impulse back into the spinal nerve for distribution to the 
extremities. Postganglionic sympathetic neurons may also go direcdy to internal organs. A 
preganglionic sympathetic neuron may bypass its "own" ganglion and not synapse until reaching 
ganglia at other levels. 



nerve 



exiting from the vertebral canal, these nerve fibers 
branch off the spinal nerves to join a chain of ganglia 
that lies on either side of the vertebral column where 
they have their first synapse. The second sympathetic 
fiber (postganglionic) then returns to the spinal nerve to 
be distributed with it throughout the extremities. The 



second sympathetic fiber can also proceed directly to 
blood vessels and to the various organs rather than 
returning to the spinal nerve (fig. 3-12). 

The parasympathetic nervous system has basically 
two parts: the cranial part, which leaves the central 
nervous system with cranial nerves III, VII, IX, and 



TABLE 3-4 

FUNCTIONS OF AUTONOMIC NERVOUS SYSTEM 

ON VARIOUS ORGANS 



Organ 



Sympathetic 



Parasympathetic 



Heart 

Bronchi of lungs 

Digestive tract 
Sphincters 
Peristalsis 

Sweat glands 

Eye 

Arteries of heart 

Peripheral arteries 

Bladder 



Increase rate 
Dilate walls 

Increase tone 
Decrease movement 

Increase sweating 

Dilate pupil 

Dilate walls 

Constrict walls 



Decrease rate 
Constrict walls 

Relax tone 
Increase movement 

Decrease sweating 

Constrict pupil 

Constrict walls 



Contract wall 



30 DeLisa and Stolov 



Tendon 



Muscle belly 



Tendon 




Bone 



Cartilage 



Joint capsule 

and ligaments 
FIGURE 3-13. Schematic representation of a typical arrange- 
ment of the tissues of the musculoskeletal system. 



X, and the sacral part, which leaves the spinal cord 
with sacral spinal nerves 2,3, and 4. Perhaps the most 
important of the parasympathetic cranial nerves is the 
tenth, the vagus nerve, which, when activated, slows 
the heart rate and speeds up gastrointestinal activity. 
The sacral parasympathetic nerves are important for 
the innervation of the bladder wall muscle and for 
sexual function, such as erection in the male. Both the 
sympathetic and parasympathetic nervous systems can 
be influenced by hormones and the emotions. 

MUSCULOSKELETAL SYSTEM 

The musculoskeletal system consists of the skeletal 
system (bones and joints) and the skeletal muscle system 
(voluntary or striated muscles). These two systems are 
interdependent, working together to support and move 
the body. The functions of the musculoskeletal system 
are: 

1. To provide a rigid support framework for the 
body 

2. To protect vital internal body organs 

3. To manufacture blood cells (the hematopoetic 
function) 

4. To store minerals such as calcium and 
phosphorus 

5. To provide a series of lever arms on which 
muscles act across joints to produce force and 
resulting body movement. 

An understanding of the musculoskeletal system is 
particularly relevant to chapters 5, 8, 11, 13, 14, 15, 
and 26. 



TISSUES 

The musculoskeletal system is composed of various 
forms of connective tissue: cartilage, ligament, tendon, 
bone, and muscle. Each of these is composed of the 
following four basic components of connective tissue: 



1. The fibroblast is the principal cell. It produces the 
building blocks that develop into the other 
components. 

2. Collagen is the principal protein manufactured by 
the fibroblast. It forms long, thin fibrils which 
intertwine into fibers that have great strength and 
are difficult to stretch. Fibers can be organized 
into various configurations. 

3. Elastic fibers, unlike collagen, are highly elastic 
and can be easily stretched. Thev are particularly 
abundant in the walls of the arteries. 

4. Proteoglycans are sometimes referred to as the 
"ground substance." They make up the matrix, 
a nonliving, extracellular substance, in which 
fibroblasts, collagen fibers, and elastic fibers are 
embedded. Proteoglycans consist of complex 
sugars and proteins. 

Depending on how these four components are 
combined and arranged in specific ways, cartilage, 
ligament, tendon, bone, muscle, or loose connective 
tissue is formed. Figure 3-13 shows all of these 
structures in their usual arrangement. 

Cartilage 

Cartilage has a high content of proteoglycans 
combined with water which gives it a gel -like 
consistency. It has essentially no blood supply and 
receives its nourishment by diffusion from nearby 
capillaries. 

There are three types of cartilage: fibrocartilage, 
elastic cartilage, and hyaline cartilage. Fibrocartilage 
has a large concentration of collagen fibers and is 
dominant in intervertebral discs. Elastic cartilage has a 
large concentration of elastic fibers and is found in the 
external ear and epiglottis. 

Hyaline cartilage, dominated mostly by water and 
proteoglycans, is the most important cartilage. It 
serves three main functions: (a) it forms the "original" 



L 



Significant Body Systems 31 




Myel 
Motor end plate 



FIGURE 3-14. Very schematic representation of the terminal 
branch of a motor neuron attaching to a muscle fiber via the 
motor end plate. When the nerve's electrical impulse reaches 
the motor end plate, acetylcholine is released. The impulse then 
travels in both directions along the fiber. During contraction, 
the strands of actin and myosin slide past each other, thus 
shortening the fiber. 



skeleton in the embryo, from which bone later 
develops; (b) it is responsible for the growth of long 
bones; and (c) it lines the opposing surfaces of the most 
important joints in the body, the synovial joints. These 
opposing surfaces have a protective layer of collagen 
fibers. 



Ligaments 

Ligaments are composed largely of collagen fibers 
and some elastic fibers arranged in a somewhat 
irregular pattern. They attach bone to bone, 
strengthen and stabilize joints, and determine the 
motions allowed. The ligaments are usually rein- 
forced parts of the joint capsule, which is also a 
connective tissue structure. 

Tendons 

Tendons are also composed largely of collagen 
fibers, but have fewer elastic fibers than ligaments. 
The fibers are arranged in thick, parallel, closely 
packed bundles to form tough cords. They attach the 
contracting part of muscles to bone. 

Muscles 

The contracting unit of skeletal muscle, the muscle 
fiber, is a long, narrow, specialized cell with many 
nuclei. The many muscle fibers that make up a muscle 
are attached to tendons at each end. The force that 
develops in a muscle transmits to bone via the tendon 
to produce movement. Within a muscle fiber are two 
main proteins, actin and myosin. These proteins are long 
narrow strands oriented in the direction of the fiber. 
Where they overlap, the fiber appears dark. Where 
they do not overlap, the fiber appears light. These dark 
and light bands alternate and give the fiber, and the 
whole muscle, a striped or striated appearance under 



the microscope. 

When a muscle contracts, the actin and myosin 
strands slide past each other and shorten the fiber. The 
trigger to start this contraction comes from the motor 
nerve attached to each muscle fiber. The attachment 
occurs at the motor end plate. Acetylcholine is released at 
the motor end plate when the electrical impulse 
travelling in the motor nerve reaches the muscle fiber 
(fig.3-14). One nerve fiber branches at its end and 
connects with many muscle fibers. A single anterior 
horn cell, its axon, the terminal branches of the axon, 
and all the muscle fibers attached to these branches 
constitute a motor unit. 

Bone 

Bone is a very specialized form of connective tissue. 
It derives its hardness from the production and 
deposition of calcium crystals on the collagen fiber 
framework. The special cells responsible for this 
process, while similar to fibroblasts, are called 
osteoblasts. Bone is not a static structure. There is a 
dynamic balance between calcium in the bone and 
calcium in the blood. Constantly, new bone is 
produced and old bone is removed. In osteoporosis, 
the balance is disrupted, the removal of bone exceeds 
its production, and the bone becomes thin, less hard, 
and more easily fractured. The intestines, vitamin D, 
the kidney, the parathyroid gland, and sex and adrenal 
hormones also have important roles in this bone 
balance. 

Bones are of two main types, flat bones (e.g., the 
skull), and the long bones of the extremities. Figures 
3-15 and 3-16 illustrate the structure of long bone. 
The strength of the long bones comes from the compact 
cortical bone of the outer layer. The bone of the inner 
medullary cavity is spongy rather than compact. 

The red marrow, where blood components are 
produced, exists within the medullary cavity of all 



32 DeLisa and Stolov 



Epiphysis 



Diaphysis < 




Articular cartilage 



-^ Spongy bone 



Articular 
cartilage 



Epiphyseal 
plate 



Periosteum 
Compact bone 
Endosteum 



Medullary 
(marrow) cavity 



Epiphysis 



FIGURE 3-15. Longitudinal section of a 
long bone showing its structure. The 
cartilaginous epiphyseal plate is the site 
of long bone growth. 




Red marrow 
in spongy bone 



Remnmant of 

epiphyseal 

plate 



Medullary 

(marrow) 

cavity 



^j- Yellow marrow 

Compact 
Periosteum 




Epiphyseal 
plate 



capsule 



Synovial 
membrane 



Joint fluid 

Hyaline 
cartilage 



FIGURE 3-16. Cutaway section of an 
adult long bone showing the medullary 
cavity containing yellow marrow. In this 
section, only a residual line remains of 
the epiphyseal plate. 



FIGURE 3-17. A typical synovial joint. 



bones in the newborn child. During growth, the red 
marrow in most bone changes to yellow marrow, which 
is fatty and does not produce blood components. 
Blood -producing red marrow remains, in the adult, in 
the vertebrae, ribs, sternum, skull, femur, and 
humerus. 

Each end of a long bone is called an epiphysis, and the 
diaphysis is the long shaft between them. At the junction 
of the epiphysis and the diaphysis at each end lies the 
epiphyseal plate of hyaline cartilage which is the site of 
long bone growth (figs. 3-15 and 3-17). The hyaline 
cartilage converts to bone at the diaphysial side of the 
plate, and reproduces itself at the epiphyseal side of the 
plate. Thus, the shaft gets longer. Long bone growth 
ceases when the hyaline cartilage stops reproducing 
itself and fully converts to bone. 

Periosteum, a tough connective tissue with cells capable 
of producing compact bone, overlies the entire shaft. 
Flat bones are formed from periosteum alone. 

Loose Connective Tissue 

Loose connective tissue is a network of very loosely 
arranged fibroblasts, collagen, and elastic fibers in 
"ground substance." It lies between muscles that slide 
on each other, and between bone and muscle. In areas 



where there is much movement, such as at the shoulder 
and hip, identifiable spaces, or bursae, surrounded by 
loose connective tissue exist. 

JOINTS 

A joint, or articulation, is a union of usually two, but 
sometimes more, bones. Joints are classified into three 
groups according to the method of union. 

Immovable (fibrous) joints, such as those between skull 
bones, unite bones by rough collagenous connective 
tissue. Slightly movable (cartilagenous) joints, such as the 
intervertebral disc, unite bones by cartilage. Freely 
movable (synovial) joints, such as the joints of the 
extremities, unite bones in a complex and specialized 
manner. 

Figure 3-17 illustrates a typical synovial joint and its 
four constant features: 

1. The joint capsule fully surrounds the joint. It is 
composed of connective tissue reinforced by 
ligaments. The capsule extends from the cortex 
and periosteum in the region of the epiphyseal 
plates of the two opposing bones. 

2. The synovial membrane, a continuous sheet of loose 
connective tissue, lines the inside of the capsule, 
and has a thin layer of specialized synovial cells on 



Significant Body Systems 33 



Maxilla 

Mandible 
Clavicle 



Scapula 

Sternum 

Xiphoid process 

Costal cartilage 
Vertebral column 
Pelvis 
Ilium 

Ishium 



Greater 
trochanter 



Humerus 




Carpals 
^~~- Metacarpals 

Phalanges 



Metatarsals 



Phalanges 



Tarsals 



<SPL,UuV~/ 



FIGURE 3-18. Anterior view of the skeleton with major bones 
labelled. 



its surface. The synovial membrane covers all 
structures within the capsule except the articular 
cartilage. 

The hyaline articular cartilage surfaces at the ends of 
each of the bones that make up the joint are in 
constant contact during movement. The cartilage 
receives its nourishment directly from the synovial 
fluid within the joint cavity. The joint cavity, 
therefore, has as its borders the hyaline cartilages 
and the synovial membrane. 

The viscous synovial fluid is produced by the 
synovial membrane. It provides a high level of 
lubrication for the opposing hyaline cartilage 



surfaces and for the synovial membrane itself 
during joint movement. In the normal joint, very 
little fluid (less than 5 cc) exists in the cavity. The 

major component of synovial fluid is hyaluronic acid, 

a proteoglycan. 

SKELETAL SYSTEM 

The 206 bones of the skeleton (fig. 3-18) are divided 
into two subgroups, axial and appendicular. The axial 
skeleton, namely the head and trunk, includes the 
skull, the lower jaw {mandible), the breastbone 
{sternum), the 12 ribs, the 24 vertebrae of the vertebral 



34 DeLisa and Stolov 



Coronal suture 



Lamboidal 
suture 



Occipital 
bone 

\ 

External 
auditory meatus 




Orbit 

Nasal bone 
Zygomatic bone 



Mastoid process 
Styloid process 
Condyloid process 
Pterygoid process 
Coronoid process 



FIGURE 3- 1 9. Major bones of the skull and facial skeleton. The 
sutures are immovable nonsvnovial joints. 



column, and the 5 fused vertebrae of the sacrum. The 
appendicular skeleton consists of the bones of the 
upper and lower extremities. 

Axial Skeleton 

Skull. The skulls has two parts, the bones of the 
cranium, which enclose and protect the brain, and the 
facial skeleton (fig. 3-19). The upper teeth are 
embedded in the maxilla and the lower teeth are 
embedded in the mandible. The mandible is the only 
freely movable bone of the skull. 

Thorax. The thoracic cage consists of the sternum 
anteriorly, the 12 thoracic vertebrae posteriorly, and 
the 12 pairs of ribs, which are connected posteriorly to 
the thoracic vertebrae. Anteriorly, 10 ribs are 
connected to the sternum by cartilage and two ribs are 
"floating." The thoracic cage protects the lungs and 
the heart. 

Vertebral column. The vertebral column transmits 
body weight from the head, thorax, and abdomen to 
the lower extremities and encloses and protects the 
delicate spinal cord. There are 7 cervical vertebrae, 12 
thoracic vertebrae, 5 lumbar vertebrae, 5 sacral 
vertebrae, and 4 small vertebrae of the vestigial 
coccyx. Each vertebra has essentially the same basic 
components, although some special differences exist 
based on location and allowed movements. 

Figure 3-20 shows a top view of a typical vertebra. 
The vertebral body anteriorly and the neural arch 
posteriorly encircle the vertebral foramen. Stacked one 
on top of the other, the vertebral foramina form the 



vertebral canal wherein the spinal cord is contained. The 
neural arch is made up of the pedicles, the neural arch 
joints, and the laminae. 

Figure 3-21 shows a side view of two typical 
vertebrae, including the intervertebral disc and the 
spinal cord in the vertebral canal. The anterior stacked 
bodies of the vertebrae form the primary weight - 
bearing structure of the vertebral column. 

The strength of the attachments between vertebrae 
is achieved by several structures: (a) a long anterior 
longitudinal ligament in front of bodies and discs; (b) the 
disc itself; (c) a long posterior longitudinal ligament 
immediately behind bodies and discs; (d) the 
interlocking hyaline cartilage surfaces of the neural 
arch joints; and (e) the ligaments attaching spinous and 
transverse processes. Spinal nerves leave the vertebral 
canal through the intervertebral foramina between the 
vertebrae. 

The intervertebral discs provide a shock -absorbing 
function. The disc consists of a ring of tough 
fibrocartilage, the annulus fibrosis, surrounding a 
central gel matrix, the nucleus pulposus. The nucleus 
pulposus gives the disc its resilience, flexibility, and 
shock - absorbing characteristics . 

The vertebral column in the normal standing 
posture adopts specific curvatures (see fig. 15-3, p. 
207). The orientation of the neural arch joints allows: 
(a) the cervical spine to rotate, flex forward, flex 
sideways, and extend backward; (b) the thoracic spine 
to rotate only; and (c) the lumbar spine to flex forward, 
flex sideways, and extend backward. 
Sacrum. The sacrum has a dual character. It is at 



Significant Body Systems 35 



Spinous 
process 



Intervertebral 
foramen 




FIGURE 3-20. Top view of a typical vertebra. The vertebral 
foramen containing the spinal cord is bordered by the vertebral 
body in front, pedicles on the side, and laminae behind. 



the same time part of the vertebral column and part of 
the pelvis. It therefore transmits the upper body weight 
to the lower extremities. 



Appendicular Skeleton 

Upper extremity. The arms are ultimately attached 
to the thorax via the collarbone {clavicle) and shoulder 
bone {scapula). The upper arm bone {humerus) is united 
to the scapula at the shoulder joint. The scapula is 
attached to the thoracic cage only by muscles. The end 
of the clavicle close to the shoulder unites with the 
scapula at a synovial joint. The ultimate joint 
attachment of the extremity to the thorax is achieved as 
the other end of the clavicle connects to the sternum at 
the base of the neck. The elbow joint unites the 
humerus with the two forearm bones {radius and ulna). 
Three sets of joints connect the radius and ulna to the 
bones of the palm {metacarpals) via the eight small carpal 
bones of the wrist. Further, the knuckles 
{metacarpophalangeal, or MCP, joints) connect the 
metacarpals to the proximal phalanx of the fingers. 
Each finger has three phalanges, proximal, middle, 
and distal, except the thumb, which has two. 

Lower extremity. The pelvis (innominate bone) 
transmits the upper body weight from the sacrum to 
the legs. It begins as three hipbones {ilium, ischium, and 
pubis) which fuse together when growth is completed. 
The sacrum connection is the sacroiliac joint. The hip 
joint unites the pelvic bone to the thigh bone {femur); 
the knee joint, which also includes the knee cap 
{patella), unites the femur to the two bones of the lower 
leg {tibia and fibula). The ankle joint unites the tibia 




Spinous 
process 



FIGURE 3-21. Side view of two typical vertebrae. They are joined 
in front by the intervertebral disc and in back by synovial joints 
(under articular capsule). Their connection is reinforced by 
anterior and posterior longitudinal ligaments and interspinous 
ligaments. A spinal nerve root exits from the vertebral canal 
through the intervertebral foramen. 

and fibula to the talus. The body weight is then trans- 
mitted to the heel {calcaneous), and to the balls of the feet 
via the other foot bones {tarsal and metatarsal). The toes 
have a phalangeal structure like the fingers. 

SKELETAL MUSCLE SYSTEM 

The two ends of a muscle are attached to different 
bones. All muscles, therefore, cross at least one 
synovial joint. Thus, when a muscle shortens, one bone 
is moved in relation to the other and the axis of 
movement is at the joint connecting the two bones. The 
muscle end attached to the bone closest to the body or 
closest to the head (proximal attachment) is usually 
called the origin of the muscle. When a muscle short- 
ens, the origin is usually stationary. The distal attach- 
ment at the other end of the muscle is called the 
insertion. When a muscle shortens, the insertion moves. 

Each muscle has its own special name. They are, 
however, also described by their functions. Muscles or 
groups of muscles which bend a limb are flexors, and 
those which straighten the limb are called extensors 
(e.g., elbow flexors, elbow extensors). Muscles which 
move a limb to the side away from the midline of the 
body are called abductors, and muscles which move a 
limb toward the midline are called adductors (e.g., hip 
abductors, hip adductors). Other functional groups are 
elevators, depressors, rotators, doriflexors, plantar flexors, and 
palmar flexors (see glossary). 

Table 5-1 (p. 67) lists the major muscles and their 
functions, together with the spinal nerves that control 
the muscles. Tables 3-2 and 3-3 (p. 27, 28) list the major 
movements of the upper and lower extremities, 



36 DeLisa and Stolov 



Mitral valve 




Extremeties, abdominal and pelvic organs, 
head, CNS, skeletal muscles, and bones 

FIGURE 3-22. Schematic representation of pulmonary and systemic circulatory systems. Arrows 
indicate direction of blood flow. Oxygen from the lungs enters the system via the pulmonary vein. 
Carbon dioxide is delivered to the lungs for removal by the pulmonary artery. Food products enter 
the system from the digestive organs into the portal vein. Waste products are removed by the liver and 
kidney. 



together with the peripheral nerves serving the 
functions. 



CARDIOVASCULAR SYSTEM 

The cardiovascular system distributes food, oxygen, 
and hormones to all the living cells and carries waste 
products and carbon dioxide away from the cells. The 
vehicle for this distribution is the blood which travels in 
channels, the blood vessels. The driving force for 
moving the blood is provided by the pumping action of 
the heart. 

There are actually two circulations and in effect two 
"hearts." The pulmonary circulation, driven by the 
"right heart," delivers blood containing carbon 
dioxide to the lungs. The systemic circulation, driven by 
the "left heart," delivers blood containing oxygen, 
food, and hormones to the rest of the body. 

The blood vessels leading from the heart are arteries 



and the blood vessels returning blood to the heart are 
veins. The veins of the systemic circulation return 
blood carrying carbon dioxide to the right heart. This 
oxygen -deficient blood is then pumped to the lungs in 
the pulmonary circulation where the carbon dioxide is 
removed and oxygen is replenished. This oxygen -rich 
blood returns to the left heart and is pumped in the 
systemic circulation throughout the body. Figure 3-22 
is a full schematic of the two circulations. 

An understanding of the cardiovascular system is 
particularly relevant to chapters 9, 10, 22, 24, and 27. 

BLOOD 

Adults have five to six liters of blood. Forty -five 
percent of the blood consists of red blood cells, white 
blood cells, and platelets. There are five types of white 
blood cells: neutrophils, basophils, eosinophils, 
lymphocytes, and monocytes. The neutrophils and 



Significant Body Systems 37 




etal 
cardium 

Pericardial 
avity 

Visceral 
pericardium 

Myocardium 

Endocardium 



FIGURE 3-23. Schematic representation of the fourchambers of 
the heart, the three layers of the heart wall, and the two layers of 
the pericardium. 



lymphocytes are the most important of these five types. 
Some characteristics of the major cells are summarized 
in table 3-5. 

Fifty -five percent of the blood is the plasma, a 
straw-colored solution of 90 percent water and 10 
percent solutes. Protein constitutes 6-8 percent. The 
remaining solutes include the food substances (e.g., 
glucose and amino acids), waste products of 
metabolism (e.g., urea, creatinine), and regulatory 
substances, such as hormones and enzymes. Of the 
proteins, globulin is essential for immunity, and 
albumin and fibrinogen play key roles in blood 
clotting. 

The red blood cell (erythrocyte) is filled with 
hemoglobin, a compound of protein and iron, and is the 
main carrier for the transport of oxygen. The 
erythrocyte has a life of 80-120 days. 

HEART 
Structure 

The heart is about the size of a man's fist. Two- 



thirds of it lies to the left of the midline within the chest 
cavity between the lungs. The heart is enclosed by a 
double -layered loose sac, the pericardium. A small 
amount of fluid between the two layers lubricates the 
surfaces to allow the heart to change its shape without 
much friction as it pumps. The wall of the heart has 
three distinct layers: the outer thin membrane, the 
epicardium; the thick middle layer of cardiac muscle, the 
myocardium, which is responsible for the heart's ability 
to pump without stopping to rest; and the inner layer, 
the endocardium (fig. 3-23). The pumping action is 
achieved by contraction of the myocardium, a special 
form of muscle somewhat like skeletal muscle, but not 
under voluntary nervous control. 

Chambers. The four chambers are the right atrium, 
right ventricle, left atrium, and left ventricle (fig. 3-23). 
Blood from the systemic circulation enters the right 
atrium through the inferior and superior vena cava. It 
passes through the tricuspid valve into the right ventricle. 
Right ventricular contraction propels the blood 
through the pulmonary semilunar valve into the pulmonary 
artery for pulmonary circulation. 



TABLE 3-5 
SOURCE, NUMBER, AND FUNCTION OF MAJOR BLOOD CELLS 



Tv]»- 



Number/mm' 3 



Source 



Function 



Red blood cell 

Neutrophil 

Lymphocyte 



5 million 

3,000-6,000 

1,500-3,000 



Bone marrow 

Bone marrow 

Bone marrow 
spleen, lymph 
nodes 



Oxygen transport 

Phagocytosis 

Production of 
antibodies 



Platelet 



250,000 



Bone marrow 



Blood clotting 



38 DeLisa and Stolov 



Aorta 



Pulmonary 



Pulmon 
vein 



Superior 
vena cava 




monary vein 



ral valve 
Aortic valve 



Inferior 
vena cava 

•' n 



Tricuspid 
valve 



Pulmonary 
valve 



FIGURE 3-24. Schematic of the heart, showing direction of 
blood flow during a complete cardiac cycle. Note the location of 
the mitral, tricuspid, and semilunar valves. 



Blood from the lungs enters the left atrium through 
the pulmonary vein and passes through the mitral valve 
into the left ventricle. Left ventricular contraction 
propels the blood through the aortic semilunar valve into 
the aorta for systemic circulation. All valves — tricuspid, 
mitral, and semilunar— can only pass blood in one 
direction in the normal situation. 

The walls between the two ventricles and the two 
atria, the interventricular septum and the interatrial septum, 
block mixing of the two circulations in the normal 
condition. 



Cardiac Cycle and Heart Sounds 

Each cycle of the heart consists of two parts, diastole 
and systole. During diastole, all four chambers are 
relaxed and both atria receive and fill with blood. 
Systole begins first with right and left atrial contraction 
propelling blood through the tricuspid and mitral 
valves into the right and left ventricle respectively 
(fig.3-24). Because a great deal of force is not needed 
for this, the myocardium of the atria is relatively thin. 
In contrast, the myocardium of the ventricles is thick. 
Ventricular systole, the main pumping action, forcefully 
propels blood into the pulmonary artery and aorta 
through the pulmonary and aortic semilunar valves. 
When this contraction occurs, the tricuspid and mitral 
valves slam shut to prevent the flow of blood back into 
the atria. The closure of the valves produces the first 
heart sound that can be heard with the stethoscope. 
This rush of blood out of the ventricles causes the pulse 
beat that can be felt at the wrist and other areas of the 



body where arteries are prominent. The rush of blood 
into the pulmonary artery and aorta distends the walls 
of these vessels. When ventricular contraction stops, 
the vessels recoil and the semilunar valves slam shut to 
prevent the flow of blood back into the ventricles. The 
closure of the semilunar valves produces the second 
heart sound. 

Murmurs are sounds other than the two normal heart 
sounds which can sometimes be heard, and usuallv 
signify disease. Murmurs usually mean excessive 
blood turbulence caused by valve dysfunction, such as 
failure to open wide enough (stenosis) or failure to 
close completely (insufficiency). 

Blood pressure recorded in the arm or leg consists of 
two numbers which refer to the systolic pressure, 
associated with ventricular systole, and the diastolic 
pressure, associated with diastole. Pressures are 
recorded in millimeters of mercury (mm Hg) and are 
usually recorded as a fraction — systolic pressure/ 
diastolic pressure, e.g., 120/180. The magnitudes of 
the pressures are dependent not only on the force pro- 
duced by the thick myocardium of the ventricles, but 
also by the resistance to flow produced by the progres- 
sively narrowing peripheral arterial blood vessels. 

The cardiac output is the amount of blood pumped by 
the heart per minute. This is calculated by multiplying 
the volume ejected with each ventricular contraction 
(stroke volume) by the number of beats per minute (heart 
rate). Stroke volume varies from about 60-70cc, and 
heart rates can rise as high as 200. In a young 
individual at rest, the cardiac output is approximately 
five liters per minute. 



Significant Body Systems 39 



Sinoatrial 
node 

Atrioventricular 
node 



Bundle 




Left bundle 
branch 



ght bundle 
branch 



FIGURE 3-25. Schematic of the heart, showing the cardiac 
conducting system. The rhythm is paced by the sinoatrial node. 



Cardiac Rhythm 



Specialized muscle fibers within the myocardium 
provide the heart with an inherent capacity to contract 
rhythmically. Each cycle begins in a special bundle of 
myocardial cells {sinoatrial node, the "pacemaker") in 
the wall of the right atrium (fig. 3-25). These cells, 
without any stimulation, initiate electrical impulses at 
regular intervals. These impulses travel in the walls of 
the atria, causing atrial contraction, and rapidly reach 
the atrioventricular node, which also lies in the right atrial 
wall near the interatrial septum. Impulses delay here 
slighdy until atrial systole is completed. From the 
atrioventricular node, impulses travel down two 
bundles of special muscle fibers (bundle of His) on the 
right and left side of the interventricular septum. The 
two bundles branch out as Purkinje fibers and spread 
over both ventricles, causing ventricular contraction. 
The electrocardiogram (ECG) records the travel time 
for these impulses, as well as the electrical events 
associated with atrial and ventricular contraction. 

Pacemaker rates can be altered by the autonomic 
nervous system and certain hormones. Parasympa- 
thetic fibers from the vagus cranial nerve cause the 
sinoatrial node to slow down and sympathetic nerve 
fibers can cause the sinoatrial node to speed up. A 
number of chemicals and the hormones epinephrine 
and thryoid accelerate the heart rate. 



VASCULAR SYSTEM 

Arterial System 

Major branches of the aorta distribute blood to the 
head, abdomen, and extremities (table 3-6). Arterial 
walls are composed of three layers: (a) the intima, a 
smooth thin layer of endothelial cells on which blood 
cannot adhere; (b) the media, a relatively thick middle 



layer comprised of smooth muscle and elastic 
connective tissue fibers which can distend and recoil 
during systole; and (c) the adventitia, the outer fibrous 
layer. 

As these arteries further branch they become 
progressively thinner walled and smaller in diameter. 
The smallest divisions of the arteries are the arterioles, 
whose main component is smooth muscle. The 
diameter of arterioles can be altered even to the extent 
of closure by the sympathetic nervous system. The 
changes in the diameter of these vessels determine the 
quantity of blood delivered to capillaries. In addition, 
arterioles influence the blood pressure since, as their 
diameter changes, the resistance to blood flow changes. 

The pulmonary artery and its distribution system is 
discussed on p. 43. 

Of special importance are the right and left coronary 
arteries that supply the heart itself, in particular the 
myocardium (fig. 3-26). These vessels branch off the 
aorta as it leaves the heart. The right coronary artery 
serves mostly the right atrium, right ventricle, and the 
bottom of the left ventricle. The left coronary artery 
supplies the left atrium and the remainder of the left 
ventricle. There is, however, an interconnection 
(anastomosis) between the two coronary arteries, such 
that each vessel alone can deliver blood to the 
myocardium of all four chambers. Most of the blood 
flow in the coronary arteries occurs during diastole 
when the heart walls are relaxed and the aortic 
semilunar valves are closed. 

Capillaries 

The arteries lead into the microscopic capillaries 
which are multiply branched and lie in close 
approximation to the fluid bathing the living cells 
(interstitial fluid). Capillary walls are very thin, 
consisting only of intima, a thin lining of endothelial 



40 DeLisa and Stolov 



TABLE 3-6 
MAJOR BRANCHES OF SYSTEMIC CIRCULATION 



Name 



Derived From 



Serves 



Head 
Abdomen 



Upper Extremity 



Lower Extremity 



Common carotid 


Aortic arch 




Brain & skull 


Mesenteric 


Descending 


aorta 


Intestines 


Celiac 


Descending 


aorta 


Stomach, liver; 
spleen 


Renal 


Descending 


aorta 


Kidney 


Iliac 


Descending 


aorta 


Pelvis 


Brachial (axillary) 


Subclavian 




Upper arm 


Radial & ulnar 


Brachial 




Forearm & hand 


Femoral 


Iliac 




Thigh 


Popliteal 


Femoral 




Leg 


Dorsal pedis 


Popliteal 




Foot 


Posterior tibial 


Popliteal 




Foot 



cells. This thin wall permits the passage of blood 
plasma containing dissolved nutrients and oxygen into 
the interstitial fluid in the vicinity of the cells, and 
permits the passage of waste products and carbon 
dioxide from the cells back into the blood stream. The 
outflow occurs at the arteriole end of the capillary and 
the inflow occurs at the venule end of the capillary. 
Only about 40 percent of the blood plasma that leaves 
the capillary at the arteriole end returns to the blood 
stream at the venule end. The remainder returns to the 
blood via the lymphatic system (see below). 

Venous System 

The capillaries lead into venules, which in turn unite 
to form veins that return blood to the heart. Veins are 
thin walled and the blood pressure in them is very low. 
Their walls have the same three layers as the arteries, 
but the media is much thinner. Veins from the legs, 
which return blood up to the heart against gravity, 
contain valves that function to keep the blood moving 
and to prevent pooling of blood and edema in the legs. 
The main collecting veins of any organ or part of the 
body are called by the same name as the main artery 
delivering the blood. The exceptions are the superior 
vena cava, which collects blood from the head and 
upper extremities, and the inferior vena cava, which 
collects blood from the rest of the body. These two 
vessels correspond to the aorta. 

Portal System 

The portal system (see fig. 3-22), while basically 



part of the venous system, has a unique function with 
regard to the liver, a most vital organ. The portal vein 
collects all the blood within the walls of the intestine. 
Nutrients derived from digestion diffuse into 
capillaries in the wall of the intestines and are therefore 
contained in the blood collected by the portal vein. The 
porta] vein enters the liver and branches into venules 
and then into capillaries intimately associated with 
liver cells. In the liver, the capillaries recombine into 
venules and then into the hepatic vein which enters the 
inferior vena cava. This system allows the liver cells to 
immediately deal with the food products, performing 
three main functions: 

1 . Execution of a number of important steps in the 
utilization of proteins, fats, and carbohydrates 

2. Storage of important substances, such as iron and 
various vitamins 

3. Neutralization of potentially toxic products of 
digestion 

Oxygen is delivered to the liver by the hepatic artery. 

Lymphatic System 

The lymphatic system is a special second pathway by 
which the individual cells of the body communicate 
with the blood. It begins as open-ended microscopic 
lymphatic capillaries in intimate contact with the 
interstitial fluid bathing all cells. The lymphatic 
capillaries collect interstitial fluid (lymph), including 
that part of the blood plasma that does not return to the 
blood capillaries and various proteins which cannot 
pass through the walls of blood capillaries. The 
lymphatic capillaries join to form lymphatic vessels of 



Significant Body Systems 41 



Right common 
carotid artery 

Vertebral artery 



Right 

subclavian 

artery 



Superior 
vena cava 



Left common 
carotid artery 



Vertebral artery 




Pulmonary 
artery 

Right 

coronary 

artery 



Left 

coronary 

artery 



FIGURE 3-26. The two coronary arteries encircle the heart and 
supply blood to all portions of the myocardium. Each coronary 
artery can supply both atria and both ventricles, thus providing 
the heart with a safety factor in the event of disease in one of the 
coronary arteries. The vertebral and carotid arteries supply 
blood to the brain. 



increasing diameter, and finally form two main 
vessels: (a) the thoracic duct, which enters the left 
subclavian vein after draining the interstitial fluid for 
most of the body; and (b) the right lymphatic duct, which 
enters into the right subclavian vein after draining the 
right arm and right upper trunk areas. Dispersed 
throughout the lymphatic system are lymph nodes, 
sometimes called glands, located in the neck (cervical 
nodes), in the floor of the mouth (submaxillary nodes), 
under the armpit (axillary nodes), and in the groin 
(inguinal nodes). The lymph nodes manufacture and 
contain lymphocytes (see table 3-5), which produce 
antibodies. Other cells in the lymph nodes filter out 
bacteria and other injurious particles, such as cancer 
cells. 

The main forces moving lymph up toward the heart 
are contraction of skeletal muscles which squeeze the 
vessels, and the inspiration phase of respiration which 
provides a suction action. Further, backflow is 
impeded by valves in the lymphatic vessels. 

PULMONARY SYSTEM 

The function of the pulmonary system is to deliver 
oxygen to the blood and to remove carbon dioxide 
from the blood. Inspired air contains 21 percent 
oxygen and essentially no carbon dioxide, while 
expired air contains 16 percent oxygen and 4.5 percent 
carbon dioxide. Further, the temperature and water 



vapor content (humidity) of inspired air is that of the 
immediate environment, while expired air is at body 
temperature and is saturated with water. Finally, 
inspired air may contain bacteria, dust particles, and 
other particulate matter, while expired air is generally 
free of such substances. 

The actual exchange of oxygen and carbon dioxide 
occurs in the walls of the alveoli deep within the lungs. 
The nose and mouth, pharynx, larynx, trachea, right 
and left main bronchi and their branches, and the 
bronchioles distribute inspired air to the alveoli, 
establish appropriate air temperature and humidity, 
and control particulate matter. The lungs and part of 
the distribution system lie within the chest cavity. 

Contraction of muscles in the wall of the chest cavity 
produces inspiration. Expiration occurs as these 
muscles relax. The rhythmicity of respiration is 
controlled by the respiratory centers in the upper part 
of the pons and in the medulla (see p. 23). 

Dysfunction of the pulmonary system is discussed in 
chapter 23. 

AIR DISTRIBUTION 

On inspiration, air enters the nose through the 
nostrils (nares). Nasal hairs filter out bacteria and dust 
particles. As the air passes through the nose, it is 
warmed and humidified by the moist lining of the nose 
(nasal mucosa). The moist sticky mucus produced by the 



42 DeLisa and Stolov 



Eustachian 
ube opening 



Pharynx 




Mandible 



Larynx 



FIGURE 3-27. Section through 

distribution system above the 

ynx, and larynx. 

mucosa also traps dust and bacteria. Less warming, 

humidification, and filtering occurs when air is 

inspired through the mouth. 

Air inspired through the nose and mouth enter a 
common channel, the throat, or pharynx, a five-inch- 
long tube (fig. 3-27). Further warming and 
humidification occur as the air passes through the 
pharynx. The tonsils and adenoids lie within the 
pharynx, as does the opening of the auditory (Eustachian) 
tube which connects to the middle ear (see p. 52). 

At the bottom of the pharynx, two openings exist, 
one into the esophagus for passage of food, and the other 
into the larynx for continued flow of inspired air. When 
food is swallowed, the opening of the larynx 
automatically closes. When air is inspired, it cannot 
enter the esophagus because its walls are collapsed. 
The larynx, which contains the vocal cords, is also 
lined with mucous membrane and has a fairly rigid 
wall composed of cartilage. 

Inspired air continues through the larynx into the 
trachea ("windpipe"). The trachea begins in the neck 
and ends in the chest cavity where it branches into the 
right and left main bronchi. At this point, inspired air is 
considered to have entered the lungs. Multiple tree -like 
branches of the main bronchi subsequently distribute 
inspired air through the lung substance. The smallest 
branches are the bronchioles . 

Rigidity of the trachea and bronchi is achieved by 
cartilagenous walls, which disappear at the bronchiole 
level. Bronchiole walls contain smooth muscle similar 



Epiglottis 

Opening 
of larynx 



Esophagus 



the face and neck showing the air 
trachea: the nose, mouth, phar- 

to that found in the walls of arterioles (see p. 39). The 
muscle is sufficient to collapse the bronchioles. Smooth 
muscle in the tracheobronchial tube may alter the 
diameter of the tubes, but collapse is prevented by the 
cartilage in the walls. 

The mucosa of the trachea, bronchi, and most of the 
length of the bronchioles contain special cells with 
hairlike projections (cilia). The cilia "beat" 
rhythmically, driving mucus (which may contain 
bacteria and particles not previously filtered) toward 
the pharynx. By the time inspired air has reached the 
bronchioles, it is at body temperature, contains 100 
percent humidity, and hopefully has been completely 
filtered. 

The inspired air finally enters through alveolar ducts 
into alveolar sacs. The walls of each sac have many 
pockets, called alveoli (fig. 3-28). Some 200 million 
alveoli make up the bulk of the lung substance. It is in 
the walls of these alveoli that oxygen and carbon 
dioxide are exchanged with capillary blood. The total 
area in the walls of the alveoli in contact with the 
capillaries is about 753 square feet (70 m 2 ). Nearly a 
liter of blood is in contact with these walls at any time. 

THORAX 

The thorax is enclosed by the sternum and ribs in 
front, ribs on the side and top, the vertebral column 
and ribs in back, and the diaphragm on the bottom. 
The diaphragm separates the chest cavity from the 



Significant Body Systems 43 



Bronchiole 
Alveolar duct 




Alveoli 



Alveolar sac 



FIGURE 3-28. Schematic showing the structure of the alveolar 
ducts, alveolar sacs, and alveoli. 

abdominal cavity and is pierced by the esophagus 
where it enters the abdominal cavity to connect with 
the stomach. The space between the ribs contains the 
intercostal muscles. 

The thorax is firmly divided into the right and left 
chest cavities, which contain the right and left lungs. 
The left lung has two sections (the upper and lower 
lobes) and the right lung has three sections (the upper, 
middle, and lower lobes; fig. 3-29). 

The septum that divides the two cavities is called the 
mediastinum. In addition to the trachea, esophagus, and 
various lymph nodes, the mediastinum includes the 
heart. 



Pulmonary Circulation 

As indicated on figure 3-22 (p. 36), oxvgen-deficient 
blood carrying an excess of carbon dioxide returns to 
the heart through the superior and inferior vena cava. 
The right ventricle subsequently pumps blood through 
the main pulmonary artery (see fig. 3-24, p. 38), 
which immediately branches into the right and left 
pulmonary arteries to distribute this oxygen -deficient 
blood to the right and left lungs. Within the lung, 
multiple branching of the pulmonary artery occurs 
until the arteriole level is reached (see p. 39). The 
arterioles open into very small capillaries that run in 
the walls of the alveoli. After the gas exchange, the 
capillaries recombine into venules and veins. 
Ultimately two right pulmonary veins and two left 
pulmonary veins containing oxygen -rich blood enter 
the left atrium for distribution into the systemic 
circulation. 

Gas Exchange 

The thin walls of both the alveoli and the capillaries 
permit the passage of dissolved carbon dioxide and 
oxygen (fig. 3-30). The effective concentration of 
oxygen in the alveolus, and hence in the alveolar wall, 
is greater than the oxygen concentration in the 
capillary blood plasma. Oxygen therefore diffuses into 
the blood. The effective concentrations of carbon 
dioxide in the alveolar wall and the capillary blood 
plasma are reversed, and therefore carbon dioxide 
diffuses from the blood to the alveolus. 

As oxygen diffuses into the plasma, the hemoglobin 
molecule in the red blood cell immediately takes up the 



Trachea 



Left main 
bronchus 



Right main 
bronchus 




Visceral 
pleura 



Pleural 
cavity 



Parietal 
pleura 



Pleural 
space" 



FIGURE 3-29. Main respiratory structures of the thorax: trachea, 
right and left main bronchi, and the lungs. Note the three lobes 
of the right lung and the two lobes of the left lung. 



44 DeLisa and Stolov 



oxygen, permitting more to flow into the plasma. This 
unique oxygen -carrying capacity of hemoglobin 
within the red blood cell allows the blood to carry over 
70 times more oxygen than it could carry if oxygen were 
simply dissolved in the plasma. Therefore, the total 
amount of oxygen that can be taken into the blood 
each minute depends mostly on: (a) the oxygen con- 
centration difference between the alveolus and the 
blood; (b) the total healthy functioning surface of the 
alveoli; and (c) the rate of respiration. 

Pleura 

The right and left chest cavities are lined by a thin 
pleural membrane adherent to the ribs, intercoastal 
muscles, diaphragm, and mediastinum. The surface of 
this pleural membrane facing the lung {parietal pleura) 
is smooth and contains cells that secrete a thin layer 
of fluid. 

A similar pleural membrane surrounds the lobes of 
each lung (visceral pleura). The outer surface of the 
visceral pleura facing the wall of the chest cavity also 
contains fluid -secreting cells. The smooth, glistening 
surfaces of the parietal and visceral pleurae minimize 
friction as the lungs expand and relax relative to the 
wall. Although these surfaces are usually in intimate 
contact, they present a potential space that can be filled 
under abnormal circumstances. 

MECHANICS OF RESPIRATION 

Inspiration 

Stimuli from the respiratory center in the brain stem 
initiate impulses in the nerves that work the diaphragm 
(phrenic nerve) and the intercostal muscles (spinal 
nerves T2 through T12). The contraction of these 
muscles increases the volume of the thorax. An 
immediate decrease in pressure below atmospheric 
pressure occurs in the thorax outside the lungs. Since 
the pressure inside the bronchi is equal to atmospheric 
pressure, air rushes in as long as the nose and /or 
mouth are open. This same reduction in pressure 
within the chest cavity to below atmospheric pressure 
also contributes to increasing the flow of blood, 
particularly in the inferior vena cava into the right 
heart. 



Expiration 

At the height of inspiration, the respiratory center 
ceases to cause impulses in the phrenic and intercostal 
nerves. The stretched, elastic fibers of the lung 
connective tissue simply recoil. Lung volume 
diminishes and the air passively flows out. Expiration 
can be actively enhanced by contraction of abdominal 
muscles. 

A cough is a forced expiration usually induced by an 



Alveolus 



Diffusion of 
carbon dioxide 




Diffusion 
of oxygen 



Capillary 



Red blood cell 

FIGURE 3-30. Schematic representation of the exchange of 
oxvgen and carbon dioxide through the thin walls of an alveolus 
and a red blood cell of a capillary. 

irritation in the lining of the air passages. It is usually 
preceded by an automatic inspiration. Immediately 
and automatically, the vocal cords close and seal the 
larynx, blocking the transfer of any air into or out of 
the lungs. Simultaneously, the abdominal and chest 
muscles contract. This contraction builds up a very 
high pressure within the thorax on the outside of the 
lungs. This pressure greatly exceeds atmospheric 
pressure. The vocal cords then reflexly open and air 
rushes out the mouth and nose. The rush of air drags 
the mucus lining the walls of the trachea, bronchi, and 
bronchioles into the pharynx. 



Lung Volumes 

Figure 3-31 describes the various lung volumes of 
importance: 

1. The tidal volume, with the body at rest, is the 
amount of air (about 500 ml) taken in with each 
breath. 

2. The inspiratory reserve volume is the maximum 
additional amount of air (about 3,000 ml) that 
can be taken in with a maximum inspiration 
effort, such as occurs at the height of maximum 
physical activity. 

3. The expiratory reserve volume is the amount of air 
that can be further expired after passive 
expiration (about 1,000 ml). 

4. The vital capacity of the lung is the sum of the 
above three volumes (about 4,500 to 5,000 ml) 
and is one of the main volumes to characterize 
a person's breathing capacity. 

5. The residual volume is the amount of air that 
stays in the lung even after maximum expiration. 
The rigidity of the cartilage surrounding the 
trachea and bronchi does not permit their collapse, 
and hence the expulsion of all of the air. 

Additional lung volumes which are often measured 
include the forced expiratory volume (FEV) and the 
maximum voluntary ventilation (MVV). The FEV is 
the volume of air that can be forcefully expired. Forced 
expiration is often timed to measure how fast the total 
FEV is exhaled. The volumes of air expired forcefully 
during the first, second, and third seconds are FEVi, 
FEV 2 , and FEV 3 , respectively. The MVV is a 
measure of the maximum volume of air breathed in 



Significant Body Systems 45 



Resting State 
(normal breathing) 



Greater Activity 
(forceful inspiration & 
forceful expiration) 




Vital Capacity 
(4,500 ml, 
theoretical) 



FIGURE 3-3 1. Diagram of lung volumes during rest and during 
increased activitv. 



and out as rapidly and forcefully as possible for 12 
seconds. This value is multiplied by five to give the 
MVV in liters of air per minute. 

Regulation of Respiration 

Several factors participate in the regulation of the 
rate of respiration, the depth of respiration, and the 
rhythmicity of the inspiratory and expiratory efforts. 

The controlling respiratory centers are located 
within the pons and medulla of the brain stem. The 
dominant controlling factor is the concentration of 
carbon dioxide (C0 2 ) in the blood. If the blood 
circulating within the respiratory centers has a high 
concentration of CO2, it will stimulate the center to 
initiate more rapid and deeper breathing. The rapid 
and deeper breathing so initiated causes more CO2 to 
diffuse out of the pulmonary capillaries into the alveoli 
and out of the system. A low concentration of CO2 
reciprocally slows down the rate and depth of 
respiration. 

The second most important influence, particularly 
on the depth and rhythmicity of respiration, is nerves 
that are sensitive to pressures within the lung tissue. 
These nerve impulses reach the respiratory centers 
through cranial nerve X (vagus nerve). Expansion of 
the lungs stimulates these receptors, signaling the 
respiratory centers to "turn off inspiration. When the 
lungs have collapsed at the end of expiration, the same 
receptors signal the respiratory center to begin 
inspiration again. This pressure reflex is called the 
Hering-Breuer inspiratory reflex. Other regulators of 
respiration are: (a) a drop in the acidity of the blood, 
which stimulates respiration; (b) an increase in blood 
pressure, which slows down respiration; and (c) a 



sudden drop in blood pressure, which stimulates an 
increase in the rate and depth of respiration largely for 
the purpose of returning more blood to the heart. 

Voluntary action initiated in the frontal lobe of the 
cerebrum can also increase or decrease the rate and 
depth of respiration, but a person cannot voluntarily 
stop breathing indefinitely. When respiration is 
stopped through a conscious effort, the carbon dioxide 
in the blood increases in concentration. This increase 
in carbon dioxide stimulates the respiratory center to 
overcome the conscious effort and forces the 
resumption of inspiration. 

VISUAL SYSTEM 

The function of the visual system is easily 
understood by a comparison with a camera. A camera 
has a shutter with a small opening in the center 
through which light from an object enters. If the object 
is bright, ; smaller hole is created than if the light is 
dim. The camera lens is necessary to bend the light 
rays. For objects that are in close, a fatter, more 
convex ("zoom") lens is needed to bend the light 
more. For objects that are far away, a thinner lens is 
needed. The need for different shaped lenses exists 
because the photographic film on which the images are 
focused is at a fixed distance behind the lens. The 
camera case encloses and protects the shutter, lens, and 
film. 

In the eye, the shutter function is performed by the 
iris, the colored part of the eye. The hole in the center 
of the iris is the pupil, through which the light enters. In 
bright light, the iris makes the pupil very small, and in 
dim light, the iris makes it large. 

The lens of the eye corresponds to the lens of the 



46 DeLisa and Stolov 



Muscle 



Conjunctiva 



Chorid 



Anterior cavity 
Anterior chamber 
Posterior chamber 



Canal of 
Schlemm 




Aqueous 
h 



Pupi 



Cornea 



Optic nerve 



FIGURE 3-32. Schematic section of the human eye. The lens, 
suspensory ligament, and ciliary muscle divide the eye into 
anterior and posterior cavities. The iris further divides the 
anterior cavity into anterior and posterior chambers. Note the 
two extraocular muscles attached to the sclera. 



camera. It has the capacity to assume a fat or thin 
shape through the action of the ciliary muscle to which it 
is attached. 

The retina, at a fixed distance behind the lens, 
corresponds to the film of the camera, and the 
"developing process" is a set of complex operations 
that convert the image focused on the retina into nerve 
impulses. These impulses reach the occipital lobe of the 
brain where they are interpreted. 

The protective camera case function in the eye is 
performed by the sclera, which is white and opaque 
except for the transparent cornea in front of the pupil 
and iris. The eyeball is further protected by a cushion 
of fat that lines the surrounding skull bones that make 
up the orbits (see fig. 3-19, p. 34). 

In front, the sclera, cornea, and eyelids are lined by 
a thin, clear, skin Tike layer, the conjunctiva. Eyelashes 
serve to partially keep foreign material out of the eye. 

The lacrimal glands , above the eyeball and behind the 
eyelid, secrete the tears. The fluid enters through the 
holes in the outer aspect of the upper eyelid and drains 
across the eye for cleansing and lubrication. The fluid 



drains into the nose through the nasolacrimal duct 
located in the inner aspect of the lower eyelid. 

A defect in any of these structures can result in 
abnormal vision. In the following sections, individual 
components are discussed in sufficient detail for 
understanding the more common eye disorders. 
Chapter 29 details the treatment, rehabilitation, and 
vocational consequences of severe visual impairments 
and blindness. An understanding of the visual system 
is also relevant to chapters 10, 24, and 27. 

CORNEA AND SCLERA 

The cornea is about 10-12 mm in diameter and is 
curved, and hence protrudes from the rest of the 
eyeball (fig. 3-32). Because of this curvature, light 
begins to bend as it enters the cornea, even before it 
reaches the lens. The cornea is very clear and without 
blood vessels. It contains five layers and is richly 
supplied with nerve cells. Injuries to the cornea 
through disease or trauma may produce corneal 
opacities. If opacities are severe, corneal transplants 



Significant Body Systems 47 



from cadavers are possible because the cornea is 
nonvascular. 

The white sclera, which is about 1 mm thick, 
completely encloses the internal structures of the eye 
except posteriorly, where it is pierced by the optic nerve. 
Its firm, inelastic, fibrous character maintains the form 
of the globe. No light passes through it. 

The sclera is lined internally by the choroid, which 
consists mainly of blood vessels enmeshed in delicate 
connective tissue and pigmented cells, giving it a color 
that may range from orange to red to brownish grey. 
The blood vessels of the choroid nourish the lens, the 
posterior cavity behind the lens, and part of the retina. 

IRIS AND PUPIL 

The iris hangs behind the cornea immediately in 
front of the lens and is attached to the choroid (fig. 
3-32). Its pigmented cells provide the color of the eyes. 
The circular muscle fibers of the iris constrict the pupil 
to reduce the amount of light allowed in, and radially 
oriented muscle fibers dilate the pupil to increase the 
amount of light. Reflexes initiated when light strikes 
the retina control these muscle contractions via cranial 
nerve III (oculomotor nerve). The pupil appears black 
because all of the light striking the retina is absorbed 
and none is reflected out of the eye. 

LENS AND CILIARY BODY 

The ciliary body, a muscular structure, also attaches 
to the choroid. In fact, the ciliary body and the iris are 
essentially one structure. From the ciliary body 
extends the suspensory ligament which attaches to the lens 
and maintains it in its proper position behind the pupil 
(fig. 3-32). 

The lens, which is crystalline and transparent, 
consists of an outer cortex and an inner nucleus. The 
cortex is softer than the nucleus. In the young, the 
nucleus is small, and hence the lens is more pliable. 
When gazing at near objects, the ciliary muscle 
contracts, relaxing the suspensory ligament. The 
tension on the lens is thereby released, and the lens 
fattens. This increased curvature of the lens causes the 
light rays to bend more in order to focus an image on 
the retina. For far objects, the ciliary muscle relaxes, 
the suspensory ligament tightens, and the lens thins 
out. 

With advancing age, the lens is unable to increase its 
curvature (presbyopia) when the suspensory ligament 
relaxes, and hence near objects (e.g., printed words) 
cannot be focused on the retina. Further, with 
advancing age and in some diseases, lens degeneration 
can occur, resulting in opacities (cataracts) which 
prevent light rays from reaching the retina. 

The ciliary body, suspensory ligament, and the lens 
divide the eye into distinct anterior and posterior 
cavities (fig. 3-32). 



ANTERIOR AND POSTERIOR CAVITIES 

The anterior cavity has two chambers, anterior and 
posterior, separated by the iris. It contains aqueous 
humor, a fluid secreted in the posterior chamber by the 
ciliary body. The fluid passes through the pupil into 
the anterior chamber where it is absorbed at the canal 
of Schlemm (fig. 3-32). This secretion and absorption is 
balanced to maintain the appropriate pressure in the 
entire eye. A chronic interference resulting in too 
much production of aqueous humor and not enough 
absorption increases eye ball pressure (glaucoma). The 
increased pressure, if undetected, can lead to retinal 
damage and loss of vision. 

The posterior cavity behind the lens contains the 
vitreous humor, a transparent, colorless gel, which 
consists of a mesh of clear liquid and cells but has no 
blood vessels. The cells are nourished from the 
surrounding tissues, namely, the choroid, the ciliary 
body, and the retina. The vitreous humor may also 
develop opacities which may affect vision by blocking 
light rays. Opacities in the vitreous humor, if present, 
are usually floating, and hence may not always be in 
the line of sight. 

RETINA 

The retina, a two -layered structure, is attached to 
almost all of the choroid. In particular, the pigmented 
layer is firmly fixed to the choroid. The inner layer of 
the retina, which contains the light-sensitive cells and 
the retinal blood vessels is, in essence, an expansion of 
the optic nerve. 

The optic nerve enters the back of the eye at the optic 
disc, which itself does not contain light-sensitive cells 
and is therefore sometimes called the blind spot. Two 
main types of cells, rods and cones (fig. 3-33), make up 
the light-sensitive character of the retina. 

Rods contain rhodopsin, a protein made up in part 
of a derivative of vitamin A. It is highlv light sensitive 
and breaks down rapidly when exposed to light. This 
chemical breakdown initiates nerve impulse 
conduction. A short period of darkness is necessary for 
the rhodopsin to re-form and the rod to function 
again. Continuous bright light, therefore, interferes 
with rod function. When entering a dark environment 
from a bright environment, a certain amount of time 
for adaptation is necessary, during which rhodopsin 
re-forms and the rods become active again. Rods, 
therefore, operate most effectively in continuous dim 
light (night vision). 

Cone cells contain iodopsin, a protein which is less 
sensitive than rhodopsin and requires a brighter light 
to break down. Daylight and color vision is therefore 
mostly mediated by cones. 

Generally, rods are more numerous than cones, 
except at the macula (fig. 3-34). In fact, the center of 
the macula, the fovea, contains no rods and is the point 



48 DeLisa and Stolov 



Inner limiting 
membrane 53? 

Optic nerve 
fibers 



Ganglion _ 
nerve cells 



Direction ot light 




Retina 



Bipolar . 
nerve cells 



Photoreceptor 

cells 

Rods (gray) 

Cones (red) 

Pigment 

epithelium 



FIGURE 3-33. Layers that compose the retina. The inner 
limiting membrane lies nearest the inside of the eyeball and 
adheres to the vitreous humor. The pigment epithelium lies 
farthest from the inside of the eyeball and adheres to the choroid 
coat. Note relay of three nerve cells in the retina: photoreceptor, 
bipolar, and ganglion cells. Light rays pass through the vitreous 
humor and various layers of the retina to stimulate the rods and 
cones. All the individual optic nerve fibers collect at the optic 
disc and pierce the sclera as the optic nerve. 



of clearest vision in good light. When desiring to look 
at a specific object in good light, the eyeball is so 
moved to focus the image on the fovea. To see an 
object better in dim light, eyeball movements occur to 
form the image more toward the periphery of the retina 
where rods are more plentiful. Diseases that produce 
macular degeneration therefore affect mostly direct 
object viewing in daylight. A vitamin A deficiency will 
affect mostly night vision or vision in dim light. 

The main retinal artery and its branches nourish the 
retina and enter the eye at the optic disc. Diseases 
affecting retinal arteries may affect both day and night 
vision because of poor nutrition of rods and cones. A 
hemorrhage of a branch of a retinal artery may affect 
the function of rods or cones, depending on the 
location of the hemorrhage. 

The distance between the lens and the retina 
(literally the size of the eyeball), coupled with lens 
factors, contribute to the common visual disturbances 
of near-sightedness (myopia) and far-sightedness 
(hyperopia). A near-sighted individual has a 
lengthened eyeball. Light rays from far objects focus 
before they reach the retina and hence appear blurred. 



A far-sighted individual has a shortened eyeball. Light 
rays from near objects focus behind the retina and 
hence also appear blurred. Eyeglasses modify the 
bending of the light rays as necessary to ensure that 
focusing occurs at the retina. 

Nerve impulses initiated at the rod and cone cells 
synapse first at the bipolar neurons within the retina 
(fig. 3-33). These in turn synapse with the optic nerve 
cells, which ultimately connect with the brain. 

NERVE PATHWAYS TO THE BRAIN 

When both eyes are directed toward an object, the 
image of the object is focused on corresponding 
portions of both retinas. A system then is required for 
the resulting impulses in each optic nerve to come 
together for proper interpretation by the brain. 

Figure 3-35 indicates the labeling used to describe 
fields of vision. For each eye, that half of the retina 
close to the nose is called the nasal portion of each retina. 
The other half is called the temporal portion. Light from 
the tree in figure 3-35, which is on the right side of the 
picture (right visual field), focuses on the left side of 



Significant Bodv Systems 49 



Temporal portion Nasal portion 
of retina i of retina 




Blood vessels 



Optic disc 
(Blind spot) 



FIGURE 3-34. The retina as seen through the pupil with an 
ophthalmoscope in a normal human right eve. Sharpest vision 
occurs when the image falls on the macula, in particular on the 
fovea in the center. The nerve fibers are exiting at the optic disc, 
which is not covered bv the retina. Images that focus on the optic 
disc are therefore not visualized ("blind spot"). Blood enters 
and leaves the eve at the optic disc through the central arterv and 
the central vein. 

Left visual field Right visual field 




Right eye 



Nasal portions 
of retinas 



-Temporal portions of retinas 

FIGURE 3-35. Note that corresponding points of focus for the 
tree are on the temporal portion of the retina of the left eye and 
the nasal portion of the retina of the right eve. Corresponding 
points of focus for the car are on the temporal portion of the 
retina of the right eye and the nasal portion of the retina of the 
left eve. 



each retina. As the figure shows, this corresponds to 
the nasal portion of the right retina and the temporal 
portion of the left retina. Light rays from the car, 
which is on the left side of the picture (left visual field), 
focus on the temporal portion of the right retina and 



the nasal portion of the left retina. 

Figure 3-36 describes how images from the right 
visual field will all ultimately reach the left occipital 
lobe and how images from the left visual field will all 
ultimately reach the right occipital lobe. The figure 



50 DeLisa and Stolov 



Left visual field 
Rays 



Right visual field 




Optic nerve 
Optic chiasm 
Pituitary gland 

Brain stem 
Optic tract 

Lateral 

geniculate 

body of 

thalamus 



Optic 
radiation 



Left 

occipital 

lobe 

FIGURE 3-36. Note crossover of 

nasal retinas at the optic chiasma 

visual field can project onto the 

versa. 

two sides of the retina 



shows the fibers from the 
traveling in each optic nerve. The two optic nerves 
come together under the frontal lobe and in front of the 
pituitary gland and join at the optic chiasma. 

At the optic chiasma, the fibers from the nasal 
portion of each retina cross over and join the fibers 
from the temporal portion of the retina on the opposite 
side which carry the corresponding image. These 
combined fibers form the optic tract. Thus, the left optic 
tract contains nerve impulses of images from the right 
visual field and the right optic tract contains those from 
the left visual field. These fibers then synapse in the 
right and left lateral geniculate bodies of the thalamus. 
Fibers from the geniculate bodies continue as the optic 
radiations to terminate in the cortex of the right and left 
occipital lobes. In addition, fibers in the optic tract 
send branches to the brain stem to achieve 
coordination between the two eyes and to interact with 
cranial nerves III, IV, and VI (oculomotor, trochlear, 
and abducens) controlling the extraocular muscles. 

Complete loss of the right optic radiations, such as 
may occur in stroke, for example, blocks vision from 



optic nerve fibers from both 
, with the result that the right 
left occipital cortex, and vice 

the left visual field for both eyes (left hemianopsia). 
Similarly, complete loss of the left optic radiations 
blocks vision from the right visual field for both eyes 
(right hemianopsia). 

The three-dimensional perception of depth is 
achieved in several ways. The two eyes each see a 
single object from an ever-so-slightly different angle. 
When their two corresponding images register in the 
occipital lobe, these slight differences are interpreted to 
mean depth. Depth perception is also a learned 
phenomenon as we come to understand shadows, slight 
color changes with distance, the blocking of one object 
by another in front of it, and relative movement 
between near and far objects (parallax) as our eye 
surveys a scene. Thus, individuals who lose one eye 
still perceive depth, although not quite as accurately 
as with two eyes. 

EYE MOVEMENTS 

Each eye is moved within its orbit by six muscles 
that are attached to the sclera. These six muscles are 



TABLE 3-7 
MUSCLES OF EYE MOVEMENT 



Significant Body Systems 51 



Muscle 



Movement Produced 



Cranial Nerve 



Superior rectus 
Inferior rectus 
Medial rectus 
Lateral rectus 
Superior oblique 
Inferior oblique 



Up 

Down 

In toward nose 

Away from nose 

Down and in 

Up and out 



Oculomotor (III) 
Oculomotor (III) 
Oculomotor (III) 
Abducens (VI) 
Trochclear (IV) 
Oculomotor (III) 



governed by cranial nerves III, IV, and VI. The 
superior rectus and inferior rectus muscles mostly turn the 
eyeball and direct the pupil up and down, respectively. 
The medial rectus and lateral rectus muscles mostly turn 
the eyeball and direct the pupil toward the nose and 
away from the nose, respectively. The superior oblique 
and the inferior oblique muscles have more complex 
actions, mostly down and in and up and out, 
respectively. Table 3-7 summarizes these movements 
and the nerves operating them. 

A disturbed balance in eye movements can lead to a 
failure of images to hit corresponding portions of the 
retinas and may result in double vision (diplopia). In 
severe losses of balance, such as might occur with 
paralysis, one eye might not even fix on the object at all 
and vision would therefore be monocular rather than 
binocular. 

AUDITORY AND VESTIBULAR SYSTEMS 

Hearing and equilibrium are usually discussed 
together because they derive from the same structure, 
the ear. The auditory system is concerned with the 
detection of sound waves, and the vestibular system is 
concerned with body balance. 

Sound waves do not travel in a vacuum. They 
require a medium for their transmission. What 
actually travels is a succession of increased pressure in 
the medium, followed by decreased pressure, and then 
increased pressure again. These alternations of 
increased and decreased pressure (vibrations) can 
occur at various frequencies. Figure 3-37, using the 
example of a tuning fork, illustrates the traveling 
pressure wave. Sound waves travel 742 miles (1,187 
km) per hour in air and 3,424 miles (5,478 km) per 
hour in water. These speeds are much slower than 
speeds of light. Hence, we see lightning before we hear 
the thunder. 

The auditory system functions to conduct the sound 
wave to a set of special cells that sense the vibration and 
convert it into a nerve impulse. When the nerve 



impulse is transmitted to the brain, the sound is finally 
perceived. Not all sound vibrations can be perceived 
by the human ear. Only those frequencies ranging 
from about 20 to 20,000 cycles per second (Hz) can be 
perceived. 

The vestibular system senses both static position and 
movement (acceleration and deceleration). One set of 
special cells senses position and a second set, movement. 
When stimulated, the cells produce nerve impulses 
which are then transmitted to the CNS. 

The ear divides anatomically into three portions, 
termed the external ear, the middle ear, and the inner 
ear, which are located in special cavities within the 
temporal bone in the base of the skull on the underside 
of the cerebral hemispheres. The external and middle 
ears are concerned with conduction of sound waves. The 
inner ear contains the set of special cells for hearing 
and the two sets of special cells for position and 
movement. The nerve impulses created by these 
special cells reach the brain through the vestibulocochlear 
nerve (cranial nerve VIII). This is really two nerves 
somewhat joined together, the cochlear nerve for hearing 
and the vestibular nerve for equilibrium. 

The following sections deal with the significant 
structures which, if impaired, can lead to the reduction 
in hearing and the sense of balance. Hearing 
impairments and deafness, together with their 
treatment, rehabilitation, and vocational implications, 
are discussed in chapter 30. 

AUDITORY SYSTEM 

External Ear 

The auricle or pinna, the visible portion of the ear, is 
shaped to collect the sound waves, which enter the 
opening of the external ear canal, the external acoustic 
meatus. Sound transmits along the ear canal to the ear 
drum (tympanic membrane). The canal is about 1 X A 
inches (3.18 cm) long and contains modified sweat 
glands that secrete cerumen, a wax-like substance. 
Overproduction of cerumen can block transmission of 



52 DeLisa and Stolov 



sound. Figure 3-38 show the relation of the external 
ear to the middle ear. 



Middle Ear 

The middle ear continues the process of sound 
conduction. The air -filled cavity of the middle ear 
(tympanic cavity), while wholly contained within the 
temporal bone, connects to the throat by the auditory 
(Eustachian) tube. This connection makes the middle 
ear susceptible to infection (otitis media). The auditory 
tube is necessary for normal hearing. It allows the air 
pressure on both sides of the tympanic membrane to be 
equal, and this permits the membrane to vibrate 
maximally when sound waves impinge upon it. 
Normally, the walls of the auditory tube are collapsed. 
Swallowing and chewing actions momentarily open the 
tube to allow air in or out of the tympanic cavity as 
may be necessary for equalization of air pressure. 

The tympanic membrane, which separates the external 
and middle ears and is considered part of the middle 
ear, is made up of three layers. The outer layer is like 
skin and the inner layer like the mucous membrane 
lining the nose and throat. When sound waves impinge 
upon the tympanic membrane, it vibrates. 

A chain of three movable bones {auditory ossicles) 
conducts the vibration of the tympanic membrane to 
the inner ear. The malleus bone is directly attached to 
the inside layer of the tympanic membrane. A 
specialized joint articulates the malleus bone with the 
incus bone, which in turn articulates with the stapes 
bone. The stapes bone has a flat expanse (footplate) 
which Fits into a hole, the oval window, in the wall of the 
inner ear. The three ossicles serve to transmit the 
vibrations of the tympanic membrane into movements 
of the footplate of the stapes. Figure 3 - 38 illustrates the 
relationships of the tympanic membrane, the auditory 
ossicles, and the auditory tube. 

Inner Ear 

The inner ear is an intricate fluid -filled cavity, the 
bony labyrinth, carved out of the temporal bone. The 
cavity has two connected sections, the cochlea and the 
semicircular canals, each with special channels within the 
bone. The oval window opens into the vestibule of the 
inner ear between the two sections. 

Floating within the bony labyrinth is a complex 
membrane -covered sac, the membranous labyrinth. The 
sac fills all the channels. The fluid surrounding the sac 
and cushioning it is the perilymph, the fluid within the 
sac is the endolymph, and the sac also contains three 
sets of special cells. Figure 3-38 shows the cochlea, 
the semicircular canals, and the vestibule between 
them where the oval window is located. 

The hearing function is subserved by the cochlea, 




C f 



FIGURE 3-37. Schematic demonstration of traveling pressure 
(sound) wave using a tuning fork in an air medium. (A) 
Deflection of tuning fork, to right compresses air molecules. 
(B) The pressure wave created further compresses air toward 
the right. The tuning fork returning to original position creates 
reduced pressure. (C) Deviation back to right starts second 
pressure wave. The distance between high pressure points is 
the wavelength of the sound wave. Frequency = velocity of 
sound/wavelength . 

which is coiled to give a snail-like appearance (fig. 
3 - 38). Figure 3-39 shows schematically how the sound 
wave signal is transmitted to the special hearing cells, 
the hair cells of the organ of Corti. Movement of the 
stapes creates waves in the perilymph. These transmit 
through the membrane of the membranous labyrinth 
into the endolymph. Movement of the hair cells leads 
to conversion of the sound vibrations into nerve 
impulses. 

The nerve fibers carrying these impulses collect 
together as the cochlear nerve, entering the skull cavity 
through a hole in the temporal bone, the internal acoustic 
meatus. The nerve enters the medulla. Several synapses 
may occur before nerve impulses reach the auditory 
cortex of the temporal lobe for perception. The 
impulses from either ear have connections to the 
auditory cortices on both sides to the extent that 
removal of one auditory cortex does not noticeably 
impair the hearing of either ear. 

Because of the rigid walls of the bony labyrinth of the 
cochlea, the waves created by the movement of the 



Significant Body Systems 53 




tf^K 



FIGURE 3-38. A section through the temporal bone to show anatomy of outer ear (pinna, external auditory canal), middle ear 
(tympanic membrane, malleus, incus, stapes), and inner ear (semicircular canals, cochlea, and vestibule with oval and round 
windows). The auditory (Eustachian) tube connects the middle ear to the back of the throat. The relative location of these structures 
can be appreciated from the sketched-in nose and eye. 



Stapes 



Muscle 



Endolymph 



Vestibular 
membrane 




Basilar 
membrane 



Hair cells of 
organ of Corti 



FIGURE 3-39. Schematic drawing of sound wave transmission. 
Sound wave vibrates the tympanic membrane, which in 
turn oscillates the malleus, incus, and stapes. Stapes vibrating in 
oval window agitates perilymph, which transmits vibration to 
vestibular membrane and then to endolymph to reach the hair 
cells of the organ of Corti. Wave dissipates at round window. 
Note the two muscles anchoring the malleus and incus. 



54 DeLisa and Stolov 



stapes need to be dissipated to prevent multiple 
reflections. The membrane -covered round window in 
that part of the wall of the inner ear separating it from 
the middle ear dissipates the wave after it has excited 
the organ of Corti. 

VESTIBULAR SYSTEM 

The semicircular canals and central vestibule 
portion of the bony and membranous labyrinths 
contain the special sets of cells for sensing position and 
movement. The three semicircular canals are 
perpendicular to each other to sense movement in the 
three spatial dimensions. At the base of each, the 
membrane cavity is enlarged {ampulla). Within the 
ampulla lie hair cells called collectively the crista 
ampullaris. Depending on the plane of movement, the 
resultant flow of endolymph within the semicircular 
canals excites the appropriate hair cells. In the region 
of the vestibule, enlargements of the membranous 
labyrinth, the utricle and saccule, contain the hair cells 
that sense static head position. Different head positions 
produce different gravity effects on these hair cells. 
Small calcium carbonate particles (otoliths) are the 
ultimate stimulants of the hair cells for position. 

The hair cells for both position and movement create 
nerve impulses. The nerve fibers carrying these 
impulses collect as the vestibular nerve, which enters 
the skull cavity through the internal acoustic meatus 
along with the cochlear nerve. The impulses from any 
one side synapse in various areas of both sides of the 
brain stem, cerebellum, and spinal cord. No definite 
connections to the cerebral cortex exist. The impulses 
therefore predominantly produce reflex actions. A 
sudden loss of balance, for example, creates 
endolymph movement in the semicircular canals and 
triggers the reflex response of leg movement or arm 
movement to restore balance. No interpretation by the 
brain (cortex) is needed to produce the corrective 
response. 



Jacobs SW, Ashworth C: Structure and Function in Man. Ed. 3, Phila- 
delphia, Saunders, 1974. 

Basic anatomy textbooks that cover all of the organ systems. 
Well written and illustrated. Physiology is well presented. 

Atlases 

Clemente C: Anatomy — A Regional Atlas of the Human Body. 

Philadelphia, Lea and Febiger, 1975. 
Grant JCB: Grant's Atlas of Anatomy. Ed. 7, Baltimore, 

Williams and Wilkins, 1978. 

Regional atlas of artist drawings. Most illustrations are 

footnoted with observations and comments that aid in 

understanding. 
McMinn RMH, Hutchings RT: Color Atlas of Human 

Anatomy. Chicago, Year Book Medical Publishers, 1977. 

Camera pictures of dissected cadavers and skeletal bones. 



BIBLIOGRAPHY 

Basic Textbooks 

Basmajian JV: Primary Anatomy. Ed. 7, Baltimore, Williams 

and Wilkins, 1976. 

Basic textbook that covers all organ systems. Concise, easy 

to read, and well illustrated. 
Gardner WD, Osburn WA: Anatomy of the Human Body. Ed. 3, 

Philadelphia, Saunders, 1978. 

Excellent basic anatomy textbook that covers all organ 

systems. Precisely written and well illustrated. 

Combination motions as well as individual muscle actions 

are described. 
Hollingshead WH: Functional Anatomy of the Limbs and Back — A 

Text for Students of the Locomotor Apparatus. Ed. 4, Philadelphia, 

Saunders, 1976. 



4 DISABILITY CONSEQUENCES OF BED REST 



Paul J. Corcoran, M.D. 



INTRODUCTION 

Most people are under the impression that rest is 
beneficial in restoring the health of an ill or injured 
person. Medical and nursing training has emphasized 
bed rest as an essential therapeutic tool. The bed is the 
central focus of hospitals, where policies and tradition 
conspire to keep patients in their beds for most of the day. 
Until the 1940's, strict bed rest was the routine 
treatment for 2 weeks after childbirth, 3 weeks after a 
hernia operation, and 4 weeks or more after 
myocardial infarction. 

The shortages of personnel, resources, and hospital 
beds during World War II led to the startling discovery 
that early mobilization of the sick and injured not only 
was harmless but actually shortened recovery times 
and lessened complications. As postwar medical 
progress led to the survival of more and more persons 
with severe disabilities, rehabilitation practitioners and 
researchers came to realize the dangers of bed rest and 
the benefits of therapeutic activities. Recent research 
in preparation for manned space flight has produced 
still more evidence of the damaging effects of 
prolonged inactivity. Practically no body system 
escapes the progressive deterioration caused by bed 
rest. 

Today it is recognized by many that rest may be 
necessary in the treatment of certain disorders. 
Prolonged continuous immobilization, on the other 
hand, can indeed have a disastrous net effect on the 
body. "Preventive rehabilitation" philosophy limits 
the duration of rest, and confines it only to the diseased 
or injured organ or part, with careful strategies to 
continue the functional use of the remainder of the 
body and mind. Practice, however, lags behind 
knowledge, and far too much rest is still prescribed by 
health practitioners or self-imposed by patients and 
their families. Thus the incidence of the complications 
described below continues to be high, especially in 
severe disabilities with their more lengthy periods of 
treatment. 

As medical progress increases the survival and 
longevity of persons with severe physical disabilities, 
rehabilitation professionals will be asked to deal with 
increasing numbers of clients at risk from these 



complications. Careful preventive rehabilitation 
should minimize the unnecessary additional disability 
caused by inactivity and rest. When the complications 
of bed rest are present, the rehabilitation counselor 
should be knowledgeable about arranging for their 
correction. Functional abilities and work tolerance can 
often be improved significantly, even in clients with 
permanent disabilities, when these complications of 
inactivity are recognized and treated appropriately. 

Ten organs or systems will now be considered in 
more detail. The common impairments which they 
may develop after inactivity will be described, methods 
of evaluation and treatment of these complications will 
be reviewed, and their vocational implications will be 
discussed. 



JOINTS AND CONNECTIVE TISSUES 

The "moving parts" of the body — joints, 
ligaments, tendons, and related muscles and skin — all 
have a normal range of motion which is necessary for 
proper performance of physical tasks. Any decrease in 
the normal range of motion is called a contracture. 
Contractures of muscles, tendons, joints, and skin are 
among the commonest complications of bed rest. 
Sometimes they are the anticipated but unavoidable 
side -effects of necessary treatment. An example would 
be a knee contracture after plaster immobilization to 
treat a fractured tibia. More often, contractures are 
produced unwittingly and unnecessarily: the frozen 
shoulder caused by use of a sling after a wrist fracture, 
or the hip flexion contracture after a period of bed rest 
(figs. 4-1 and 4-2). 

The basic component of connective tissues is collagen 
fibers (see chap. 3, p. 30). Under normal 
circumstances, our ordinary daily activities result in 
frequent excursions of the moving parts through their 
full range of motion. This maintains the collagen fibers 
in a loosely coiled arrangement which permits the 
normal stretching of tissues during activity. 
Immobilization changes these loosely coiled fibers into 
a mass of shortened, straightened, and more densely 
packed fibers. The resulting limitation of the range of 
motion of the part is the contracture. Less than a day of 



55 



56 Corcoran 




FIGURE 4-1. A person lying supine in bed sinks into the 
mattress, which in turn sags into the bedsprings. Pillows under 
the head and knees increase this flexion posture. Side-lying or 
sitting would reproduce the same flexed position, but prone- 
lying or standing would reverse it and promote straightening 
(full extension) of the spine and lower limbs. (Reprinted, by 
permission, from Krusen FH, Kottke FJ, Ellwood PM (eds): 
Handbook of Physical Medicine and Rehabilitation. Ed. 2, 
Philadelphia, Saunders, 1971.) 




FIGURE 4-2. Flexion contractures form after prolonged bed rest 
without prone-lying or range-of- motion exercises. Relaxed 
standing is then impossible because constant muscular contrac- 
tion is required to maintain the semiflexed erect posture. 
(Reprinted, by permission, from Krusen FH, Kottke FJ, Ell- 
wood PM (eds): Handbook of Physical Medicine and Rehab- 
ilitation. Ed 2, Philadelphia, Saunders, 1971.) 

immobilization will initiate these changes. We are all 
familiar with the morning stiffness after 8 hours of 
sleep in a curled -up position. We correct this by 
stretching in the opposite direction as soon as we get 
up. Two or three weeks of immobilization produces a 
much firmer contracture which may require weeks of 
range -of- motion exercises to correct. After 2 or 3 
months of immobilization, a contracture may need 



surgical correction. The rate of contracture formation 
is accelerated by several factors which may often 
coexist in the immobilized parts: edema, bleeding, 
infection, burns, or the healing of traumatic or surgical 
wounds. 



Evaluation and Management 

A physical therapist can measure contractures 
precisely with a protractor and record them on special 
range -of- motion charts. A physiatrist can evaluate the 
contracture, advise on whether surgical correction is 
necessary, and prescribe exercises, activities, or 
orthotic devices to correct them. Heating contractured 
tissues prior to exercise will increase their ability to 
stretch. One of the principal uses of deep heat 
produced by shortwaves, microwaves, or ultrasound 
(medical diathermy) is to heat deep contractures prior 
to range -of- motion exercises or activities. More 
superficial contractures (e.g., fingers, skin) can be 
heated with surface heat modalities such as hot water 
or paraffin prior to exercising. Heat also reduces pain 
and muscle spasm which could interfere with the 
exercises. Massage (manually by a physical therapist 
or mechanically via air -compression machines or 
whirlpool agitators in hot water) may help remove 
edema and facilitate the performance of range- of - 
motion exercises. 

Prevention is obviously the preferred approach to 
contractures. The key to prevention is to carry each 
joint through its full range of motion at least once every 
8 hours. This can be done by the nurse, a family 
member, or the patient himself. Proper positioning in 
bed is also important and can be assisted by sandbags, 
bedboards, and foot cushions. Intermittent lying on 
the abdomen (prone -lying) will help prevent hip 
flexion contractures. Splints can produce any desired 
position, but should be removed every 8 hours for 
range -of- motion exercises. Minimizing bed rest and 
encouraging self- care and diversional activities will 
help. An occupational therapist can position 
therapeutic crafts to encourage joint movements in the 
desired directions. 

When contractures are widespread or cannot be 
corrected, certain vocational adjustments may be 
necessary. Adaptive devices, ambulation aids, or 
special seating may improve function. Equipment or 
controls at a work station may need to be relocated if 
the client's reach is impaired. Hip or knee contractures 
increase the energy cost of walking and standing, 
which should be considered in vocational planning. 

MUSCLES 

The strength of a normal muscle is determined by 
the amount of effort to which it is regularly subjected. 
Each muscle is only as strong as it needs to be for the 
person's life style. Increasing the force of muscular 



Disability Consequences of Bed Rest 57 



contractions will lead to an enlargement of individual 
muscle cells due to an increase in the amount of 
contractile proteins inside the cells (see chap. 3, p. 31). 
The resulting overall increase in muscle bulk is 
the familiar effect of weight lifting or heavy manual 
labor. 

Less familiar is the atrophy which follows disuse of 
muscles. Total disuse of a muscle (as after paralysis, 
tendon tear, plaster casting, or severe pain on motion) 
will lead to the loss of about one -eighth of its strength 
with each passing week. A partially disused muscle 
which never exerts more than 20 percent of its 
maximum force will begin to atrophy, with a decrease 
in strength and size of the muscle cells, and an increase 
in extracellular water, fat, and collagen fibers. Regular 
exertion at 20-30 percent of maximum force will 
preserve a muscle's strength. Exercise to fatigue at 
greater than 30 percent of its maximum force will 
strengthen and hypertrophy a muscle, but work at 70 
percent would be more efficient and more effective. 

Persons who have had prolonged illness or inactivity 
will all have some degree of disuse atrophy of their 
muscles. The upper extremities may escape significant 
atrophy in a bed -bound patient if self- care activities 
are continued, but the weight -bearing muscles of the 
trunk and lower limbs usually atrophy to a greater 
extent. Backache and fatigue during convalescence are 
often due more to disuse atrophy of muscles than to the 
underlying disease. 

Evaluation and Management 

A physical therapist can evaluate muscle strength via 
a manual muscle test which is recorded on a special 
form. An evaluation by a physician skilled in 
rehabilitation medicine can determine the extent to 
which disuse atrophy is reversible. Therapeutic 
exercises and activities can be prescribed to strengthen 
muscles. Even patients in plaster casts can be taught 
muscle -setting, or isometric, exercises to prevent or 
correct disuse atrophy. When health practitioners 
recognize situations which could lead to disuse 
atrophy, preventive measures can often be instituted: 
therapeutic exercise, self- care and recreational 
activities, or mobilization with wheelchairs or 
ambulation aids. 

Atrophy due to nerve damage with complete 
paralysis, unlike disuse atrophy, cannot be corrected 
by exercise. Partially paralyzed muscles, however, can 
be strengthened through exercise assisted by the 
physical therapist or performed with gravity eliminated 
by slings or hydrotherapy. For a patient with complete 
and permanent paralysis, much of therapy is directed 
toward the unaffected or normal muscles (e.g., the 
upper limbs in a paraplegic) to develop even greater 
than normal strength. Functional activities (e.g., 
crutch- walking or wheelchair mobility) can then be 
restored as the strengthened muscles of the unaffected 



limbs begin to compensate for the paralysis. 

The rehabilitation counselor should be alert to the 
possibility that prolonged bed rest, for whatever 
reason, may be causing unnecessary additional 
disability in a client. If this is suspected, the counselor 
should consider seeking informed medical guidance 
from a medical consultant to evaluate the possible need 
for additional treatment and to assist in the proper 
timing of vocational steps. True disuse atrophy should 
respond to therapy. Once an acceptable level of 
strength has been restored, a return to vocational 
activities will often provide the additional exercise 
needed for further recovery of normal strength. 
However, resuming vigorous activities before 
sufficient strength has returned may lead to sprains, 
pains, or accidents. Informed medical guidance should 
help client and counselor with the proper timing of 
vocational steps. 

BONES 

Bones are made of a framework of collagen fibers 
which by itself would be soft and spongy. Salts of 
calcium and phosphorous are deposited in this 
framework to produce the hard, strong characteristics 
of normal bone. Bones are not inert, but undergo 
continual deposition and reabsorption of calcium and 
remodeling of the fibrous framework in response to 
stress. The combined stresses of weight bearing and 
muscle pull are responsible for maintaining the normal 
calcium content of bones. 

Bed rest and inactivity eliminate most of these 
stresses. The rate of calcium removal begins to exceed 
the rate of deposition, and within 3 days there are 
measurable increases in the urinary losses of calcium. 
The bones soften and weaken (osteoporosis), and even 
ordinary forces such as those encountered during 
wheelchair transfers, physical therapy activities, or 
minor falls may cause fractures. A common example of 
such a "pathologic fracture" is the compression 
fracture of a vertebra, most commonly seen in inactive 
elderly persons. The high calcium losses continue for 
several months, but usually reach a new equilibrium 
within a year after the onset of the paralysis or 
inactivity. 

The excessive calcium turnover, especially during 
the first year of a disability, may lead to urinary tract 
stones. Under certain circumstances, calcium may also 
be desposited in soft tissues (heterotopic calcification) 
where it can interfere with joint or muscle function. 
This occurs most commonly in paralyzed limbs, such 
as the lower limbs of a paraplegic, and is also referred 
to as myositis ossificans. 

Evaluation and Management 

Ordinary X-rays do not demonstrate evidence of 
osteoporosis until more than 50 percent of the bone 



58 Corcoran 



Erect 



Supine 




Stagnant area 



FIGURE 4-3. In the supine person, urine must flow uphill for 
about 2 inches to escape from the kidney. Changes to the side- 
lying or erect position help produce normal gravitational 
drainage. (From Browse NL: The Physiology and Pathology of 
Bed Rest, 1965. Courtesy of Charles C. Thomas, Publisher, 
Springfield, IL.) 



mineral has been lost. Heterotopic calcification is 
detectable by X-rays, and may be found in about one 
out of five spinal cord injured persons (although most 
have no resulting limitations of mobility). 

Early resumption of activity in reversible illness will 
minimize osteoporosis. In permanent disabilities, the 
calcium losses cannot be prevented. Standing for 
weight bearing with braces or on a tilt -table may 
diminish the rate of calcium loss, but without the 
added stress of muscle pull, this is not as valuable as 
was once supposed. Eliminating dairy products from 
the diet does not prevent calcification in the urinary 
tract or soft tissues, although excessively large calcium 
intakes should be avoided during the first year. It is 
sometimes useful to maintain a high enough fluid 
intake to ensure at least 1.5-2 liters of urinary output 
per day. This dilutes the urine and helps prevent stone 
formation. Osteoporosis should be assumed to be 
present in paralyzed limbs, and precautions against 
falls should be taken, such as the use of wheelchair lap 
belts. Pathologic fractures usually heal uneventually 
with ordinary management. 

For those with temporary periods of bed rest or 
inactivity, early remobilization is the best protection 
against osteoporosis. Persons with permanent 
disabilities should be educated to take the precautions 
outlined above. Special vocational considerations are 
rarely needed. Successful vocational rehabilitation 
produces increased activity and other benefits which 
outweigh the risks of pathologic fractures. 



position, which produces the additional problems of 
stagnation and loss of gravitational emptying of the 
kidneys (fig. 4-3). Complete emptying of the bladder 
also may fail to occur when voiding in a supine position 
on a bedpan or into a urine bottle. This stagnation 
makes urinary tract infection more likely, especially if 
a catheter is being used. It is impossible to avoid 
introducing bacteria with a bladder catheter, and urine 
containing bacteria (positive urine cultures) can almost 
always be obtained from persons with indwelling 
catheters. In men, the catheter can also produce prostate 
and scrotal infections and erosions from the urethra 
through the skin at the base of the penis (urethral 
fistulae), caused by kinking and pressure from the 
catheter. Without a catheter, any resistance to outflow 
when the bladder contracts can cause "reflux," the 
forcing of urine back up into the kidneys under high 
pressure. Kidney damage from infection (pyelonephritis) 
and back-pressure (hydronephrosis) is still the major 
cause of mortality in spinal cord injured persons, where 
a paralyzed (neurogenic) bladder increases all of the 
above risks still further. 

Stones usually produce severe pain and bloody urine. 
However, large stones can form gradually and grow to fill 
the kidney (staghorn calculi) without symptoms. 
Infections typically cause fever, chills, and burning on 
urination, often with pain and tenderness over the 
kidneys or bladder. However, chronic pyelonephritis 
can also progress silendy until eventually the signs of 
kidney failure (uremia) appear (see chap. 25). 



URINARY TRACT 

Inactivity increases the urinary calcium load, which 
may be three or four times normal within 3 weeks after 
the onset of complete bedrest. The risk of urinary stone 
formation is increased further by the recumbent 



Evaluation and Management 

Periodic urine analysis and cultures for the presence 
of bacteria will detect unsuspected infection. An 
excretory urogram (EUP) and voiding cystogram are 
special X-ray procedures needed once or twice yearly 



Disability Consequences of Bed Rest 59 



by every spinal cord patient to detect silent kidney 
damage from reflux, infection, or stones (see chap. 5, 
p. 69, 70, 74). 

Most of these urinary tract complications can be 
prevented by early mobilization, effective patient 
education, and proper bladder care. Enough fluids 
should be drunk to maintain at least 1.5-2 liters of 
urine output daily, particularly for patients with 
indwelling catheters. Regular use of urine acidifiers 
like cranberry juice or vitamin C (ascorbic acid) will 
make the urine inhospitable to bacterial growth. 
Techniques can usually be taught for adequate bladder 
emptying without using an indwelling catheter. The 
recent introduction of intermittent catheterization 
technique has decreased infection rates significantly. 
Proper use of external collecting equipment will 
usually keep persons dry and free from socially 
objectionable odors which would interfere with 
employment and acceptance. If bladder catheters are 
used for men, they should be taped up onto the 
abdomen to avoid the dangling and kinking that leads 
to urethral fistula. 

Two health professionals are invaluable in the 
prevention and management of urinary tract problems: 
a rehabilitation nursing specialist and a urologist who 
is knowledgeable about the neurogenic bladder. The 
rehabilitation counselor should see that all spinal cord 
injured clients are referred for regular followup and 
training by these two specialists. 

HEART 

The heart is a muscle, and it undergoes disuse 
atrophy like other muscles when a patient remains 
inactive. Being weaker, it can pump less blood per 
contraction, so it must pump more rapidly to move the 
same amount of blood per minute. Studies sponsored 
by the National Aeronautics and Space Administration 
(NASA) in preparation for manned space flight 
revealed that after 3 weeks of bed rest, the resting pulse 
of healthy young men increased by 10 beats per 
minute, and that their pulse after exertion was 40 
beats per minute greater. Exercise tolerance did not 
return to normal again until after 5 to 10 weeks of 
vigorous reconditioning. Older or debilitated persons 
suffer even more cardiac deconditioning after bed rest or 
inactivity. This is manifested by shortness of breath 
(dyspnea) on exertion, palpitations, light-headedness, 
and easy fatigability. 

In the past it was believed that lying flat decreased 
the strain on the heart and was proper after heart 
attacks. Recent evidence, however, has shown that the 
cardiac output and workload are 20-30 percent greater 
when recumbent than when sitting well supported in 
an easy chair. Defecation and urination are also less 
stressful in the sitting position. 



Evaluation and Management 

Rehabilitation counselors should be alert to the 
possibility that a severely physically disabled person 
may have unnecessary additional limitations on his 
physical performance as a result of cardiac 
deconditioning. Referral to a physician who is familiar 
with physical disabilities or to a cardiac work 
evaluation unit, if one is available, can help answer the 
question. 

Exercises and activities of increasing strenuousness, 
prescribed by a physician and supervised by a physical 
therapist, will improve cardiac fitness and work 
tolerance in disabled as in able-bodied persons. 
Graded athletics and wheelchair sports are very useful 
here. Once the work tolerance is adequate for entry 
into employment, the physical demands of the job and 
related travel exertion will produce additional gains in 
cardiac fitness. There is no evidence that hard work 
harms a normal heart. As with the remainder of the 
body, rest may be a necessary evil in certain heart 
diseases, but it does not directly benefit the heart. 

When permanent heart damage sets limits on the 
work tolerance, even with optimal cardiac 
rehabilitation, it may be necessary to reduce the 
vocational demands for exertion. Abundant data is 
available to guide the physician, physical therapist, 
and counselor regarding the energy requirements of 
various occupations, and the extent to which physical 
disabilities increase energy demands. 

CIRCULATION 

A common and potentially lethal complication of 
bed rest is the clotting of the blood in the deep veins of 
the lower limbs and lower abdomen, commonly known 
as thrombophlebitis. Studies have shown a direct 
correlation between the length of bed rest and the 
likelihood of having thrombophlebitis, which will be 
found in the large majority of persons after a few weeks 
of bed rest. Calf pain and tenderness may be present, 
but deep thigh and pelvic vein thrombosis often 
presents no symptoms or signs. The real danger of 
thrombophlebitis results when a blood clot breaks loose 
and flows rapidly to the lungs (pulmonary embolism) 
via the great veins and right heart. A clot will become 
trapped in the lungs, suddenly obstructing all flow 
through the occluded vessel. Symptoms are chest pain, 
cough, and bloody sputum. A significant number of 
pulmonary emboli cause sudden death (often 
attributed incorrectly to a heart attack). 

Another circulatory complication of bed rest is a 
drop in blood pressure upon sitting or standing up 
(orthostatic hypotension), due to the pooling of blood 
in the legs and abdomen. The patient may feel dizzy 
and weak, or may actually lose consciousness. Lying 



60 Corcoran 



down promptly restores the blood pressure and 
consciousness to normal. Orthostatic hypotension is 
common after even a week or two of bed rest, 
especially if paralysis is present. 

Evaluation and Management 

Careful frequent examination of the legs will help 
detect early thrombophlebitis. A chest X-ray or 
radioactive isotope scan of the lungs will detect most 
pulmonary embolism. Prompt use of anticoagulant 
drugs (heparin, Coumadin) usually controls the 
clotting and prevents embolization if begun early 
enough. In high-risk repeat cases, a sieve may have to be 
surgically placed in the inferior vena cava to strain out 
large clots which may break loose from the lower 
extremity. 

Elastic stockings help prevent thrombophlebitis. 
Some centers use anticoagulant drugs prophylactically 
in high-risk groups such as recent spinal cord injuries. 
Early mobilization is, of couse, an important 
preventive measure. 

Orthostatic hypotension is managed by use of elastic 
stockings and a firm corset to prevent pooling of blood 
in the abdomen and legs. Patients can be elevated in 
small stages using a tilt -table, and blood pressure can 
be monitored as they gradually adjust again to the 
vertical posture. 

Clients with a history of thrombophlebitis should be 
taught to move about frequently and exercise their leg 
muscles whenever they have to sit for a prolonged 
period. This is possible in most job situations. Elastic 
stockings may be needed permanently. Garters and 
other constricting clothing should not be used. Obesity 
and pregnancy are also aggravating factors which may 
obstruct venous return from the legs and pelvis. 

LUNGS 

Prolonged bed rest produces several changes in the 
respiratory system which predispose persons to 
pneumonia and other lung complications. The thin 
film of mucus which normally lines the air passages 
(see chap. 3, p. 42) may collect or pool in the 
dependent portions of the lungs. Sedation and 
dehydration increase this tendency. The cough reflex is 
less active and the diameter of the bronchioles 
decreases during recumbency. The work of breathing 
is greater when recumbent or slumped than when 
sitting erect. Fewer deep breaths are taken in. All of 
these factors tend to obstruct air passages, leading to 
collapse of small portions of the lung (atelectasis). This 
favors bacterial growth leading to pneumonia. 

Other complications of bed rest include pulmonary 
embolism (see p. 59) and aspiration of vomitus, 



especially likely in a supine patient who is lethargic or 
sedated. 



Evaluation and Management 

The "vital capacity," the greatest amount of air that 
can be moved in a single breath, is easy to measure (see 
chap. 3, p. 44). This and other more complex 
pulmonary function tests help indicate the likelihood of 
lung complications. The physical examination and the 
chest X-ray help detect atelectasis and pneumonia. 

Prevention of respiratory complications takes 
awareness and teamwork to provide bed activities, 
frequent position changes, and as much time up in a 
chair as possible. Adequate fluid intake prevents 
dehydration. Deep breathing and coughing should be 
encouraged frequently. High-risk patients such as 
postoperative patients benefit from chest physiotherapy 
techniques to mobilize secretions and stimulate 
coughing and deep breathing. When the vital capacity 
is limited by paralysis or other diseases, inhalation 
therapy or regular use of mechanical breathing and 
coughing devices may be appropriate. Such patients 
require more aggressive treatment for minor colds and 
upper respiratory infections. 

Over 20 years ago when polio was widespread, 
respiratory rehabilitation centers possessed the 
equipment and expertise to maintain an active life 
even with total respiratory paralysis. Portable, battery- 
powered respirators mounted on electric wheelchairs 
allowed a few of these persons to attend school, go to 
work, and socialize. Today we are again seeing more 
and more patients with severe respiratory paralysis 
from high spinal cord injuries, muscular dystrophy, 
and certain other diseases. As a result, rehabilitation 
centers are rediscovering the technique for managing 
chronic respiratory insufficiency. With improved 
architectural accessibility and vocational rehabilitation 
services, a productive life can be possible for them. 

GASTROINTESTINAL TRACT 

Bed rest is often associated with a decreased 
appetite. Hospital food may be unfamiliar and the 
surroundings unappealing. The simple mechanics of 
eating or being fed may be quite cumbersome for the 
severely disabled person. Swallowing while reclining is 
more difficult. As a result, malnutrition may develop 
after prolonged bed rest. Some patients may nibble at 
"junk food" and mask their malnutrition behind a 
weight gain. 

Constipation is another common problem in bed- 
bound patients. The lack of gravity assisting 
elimination, the awkwardness of the bedpan, and 
inability to hunch over and bear down all conspire to 



Disability Consequences of Bed Rest 61 



Spinous processes 
vertebral spines 




Greater trochanter 



Base of 

fifth 

metatarsal 



FIGURE 4-4. These are the common sites of decubitus ulcers in a 
recumbent patient. A sitting patient may develop pressure sores 
over the ischial tuberosity, the bones in each buttock upon 
which we sit. 

cause constipation. Hard fecal impactions may form 
and require removal by the gloved finger of a nurse or 
physician. 

Evaluation and Management 

A nutritional history, physical examination, and blood 
tests will point to malnutrition if present. Abdominal and 
rectal examination detect constipation. Evaluation by a 
nutritionist may be extremely helpful. 

Early mobilization and eating in a chair at the bedside 
or group dining in pleasant surroundings are important. 
Adaptive aids and occupational therapy may allow self- 
feeding, always more appetizing than being fed. A 
nutritionist can recommend nutritious foods that are 
simple for disabled persons to prepare and eat. An active 
life is the best stimulus to the appetite. 

Bowel training is facilitated by use of a bedside 



commode chair rather than a bedpan. The guidance of 
a nurse skilled in rehabilitation is invaluable. The use 
of suppositories may help regulate the bowel. 
Dependence on laxatives and enemas can usually be 
avoided, although oral stool softeners are often helpful. 
With regular eating habits and experience, most 
problems with constipation and diarrhea can be 
avoided. 



SKIN 

The bedsore, pressure sore, or decubitus ulcer (plural: 
decubiti), is one of the commonest, most expensive, 
and most misunderstood complications of bed rest. 
There is nothing mysterious about the cause — simple 
pressure is the common factor. If the pressure outside a 
blood vessel exceeds the pressure inside, the vessel will 
collapse and blood flow will cease. (This can easily be 
demonstrated by pressing the lens of one's eyeglasses, 
or any clear glass, against a. knuckle. One is surprised 
at how little pressure causes the skin to blanch. Over a 
soft, fleshy area, much more pressure is required to 
produce tissue blanching.) Tissues that lose their blood 
supply for a few hours will die. This is called infarction. 
A decubitus ulcer, then, is a hole or gap in the skin and 
underlying soft tissues, caused by their infarction due 
to prolonged pressure. 

The earliest sign of a decubitus ulcer is an area of 
redness which does not disappear within 1 5 minutes to 
an hour after removal of the pressure. Next comes a 
blister. The dead, or infarcted, tissues decay, liquefy, 
and separate out, leaving the gap or ulcer. The process 
may be even more extensive deep down, where bony 
prominences compress the soft tissues from the inside. 
Common sites are those places where bony 
prominences are thinly covered with skin and fat (fig. 
4-4). They are totally preventable, yet it is not 
uncommon to see a neglected decubitus ulcer big 
enough to put one's fist inside. 

Normally, prolonged pressure on skin causes pain, 
and we shift our position to allow blood to re-enter 
those tissues. Persons who have lost their protective 
pain sensation due to neurological injury or disease are 
the principal candidates for decubiti. Pure motor 
paralysis without sensory loss (e.g., after polio) rarely 
leads to bedsores because the protective pain sensation 
causes the patient to complain and get someone to 
change his position. Other risk factors are age, anemia, 
malnutrition, diabetes, urinary or fecal incontinence, 
immobilization in plaster or traction, use of sedatives 
or narcotics, habitual alcohol or drug intoxication, 
senility, and any other brain damage which impairs 
alertness or awareness. Wet skin, wrinkled sheets, or 
rough handling during transfers can be additional 
contributing factors. However, none of these will cause 
a bedsore if prolonged pressure is avoided. 



62 Corcoran 



Evaluation and Management 

Physicians and nurses who are experienced in 
rehabilitation can evaluate a pressure sore or a 
decubitus -prone client and recommend treatment or 
preventive measures. Numerous local medications, 
salves, and powders have been promoted to cure or 
prevent decubiti. Most of them work, not so much 
because of any direct effect but because their use 
focuses attention on the trouble spot and leads to 
frequent bandage and position changes. The key is the 
elimination of all pressure once an ulcer has begun to 
form and the limitation of pressure to not more than 2 
hours on intact skin. Large decubiti, like large burns 
and other skin deficits, may require plastic surgery and 
skin grafts to close them (see chap. 32). Small decubiti, 
like any other small skin ulcer or wound, should heal 
promptly if pressure is eliminated and they are kept 
clean and dry. 

Clients with decubitus ulcer problems require a 
team approach by a physiatrist, physical therapist, 
nurse, and equipment dealer. The wheelchair user 
must develop the habit of frequent pushups and 
position changes. Special wheelchair cushions help, 
but none yet developed offers total protection. High 
quadriplegics who cannot change position unaided 
may need an electrically reclining wheelchair or 
arrangements for an able-bodied person to assist them 
with a position change every 2 hours. 

Special mattresses using flotation principles allow 
longer periods without position changes, but the skin 
must be kept dry and watched carefully. Paraplegics 
and quadriplegics should personally inspect their own 
skin every morning and night using a mirror. 

Patient education and motivation are closely related 
to the incidence of pressure sores. The depressed, idle, 
poorly trained paraplegic is much more likely to 
develop decubiti than the optimistic, involved 
paraplegic who has had proper medical rehabilitation 
training and is actively pursuing an educational and 
vocational goal. 

Rehabilitation counselors should not hesitate to 
commit large amounts of client service to prevent 
decubitus ulcers. A $100 cushion or a $2,500 
electrically reclining wheelchair do not seem so 
expensive when compared with the $5,000 or more 
that it may cost to heal a decubitus ulcer. Proper 
equipment and training will permit entry into 
competitive employment with fewer restrictions on 
sitting tolerance. 

MENTAL STATE 

NASA -sponsored studies of eight normal young 
men who were kept in bed for 5 weeks showed 



increases in anxiety, hostility, and depression, and 
altered sleep patterns. Bed rest appears to be a subtle 
form of sensory deprivation. Bed -bound persons lose 
environmental stimuli via the five senses, as well as 
stimuli from their own musculoskeletal systems. It was 
of interest that exercising during the 5 weeks of bed 
rest reduced these deleterious effects significantly. 

Perhaps even more psvchologicallv disabling is the 
learned helplessness and dependency caused by 
medical treatment. The bed -bound patient is expected 
to play a sick role. The "good" patient is the one who 
is docile and compliant, taking medications and 
following professional orders without question. Care- 
providers are likely to positively reinforce this sick 
behavior, and react more negatively to those who 
attempt to manipulate or challenge hospital routine. 
The bed -bound patient loses control over the most 
intimate aspects of his own life. Other forces control his 
diet, sleep hours, money, social contacts, and sexual 
expression. Medications, appointments, and health 
records are managed by others. 

On the day of discharge from the hospital, the 
patient is expected to resume independent 
management and decision making for his own life. If 
it has been a 10 -day stay for appendicitis, he returns to 
the same niche in the community, with his own 
capabilities and self-image unaltered. But if it has been 
a 6 -month stay for treatment of spinal cord injury, his 
self- image may be greatly altered and his niche in the 
environment inaccessible (see chap. 2). Adults who have 
grown up with a congenital or pediatric disability may 
never have learned the skills of social survival. 

Evaluation and Management 

Psychologists and other rehabilitation professionals 
with testing and counseling skills can recognize and 
develop an understanding of the psychological 
disabilities concomitant with a physical handicap. 
Individual and group counseling may be needed. 
Rehabilitation programs should be structured to allow 
gradual resumption of control by the patient for such 
things as medications, appointments, and health 
records. Transitional living programs allow time to 
learn or relearn the social skills necessary for 
independent living. It may be helpful to arrange for 
contacts with older, more experienced disabled persons 
who are successfully rehabilitated and living 
independently. Staff expectations should be realistic to 
allow frequent successes. Every new skill mastered, 
every small hurdle overcome, will help restore a 
positive attitude. 

As with the other complications of bed rest, the best 
prevention is early mobilization and as short a hospital 
stay as possible. 



Disability Consequences of Bed Rest 63 



BIBLIOGRAPHY 

Browse NL: The Physiology and Pathology of Bed Rest. 
Springfield, Thomas, 1965. 

This easily readable monograph reviews the literature on 
the effects of bed rest. An interesting historical review of 
attitudes toward rest introduces the book. There are 
numerous illustrations and references. 

Deitrick JE, Whedon GD, Shorr E: Effects of immobilization 
upon various metabolic and physiologic functions of 
normal men. Am J Med 4:3-6, 1948. 

A classical article describing the altered calcium and 
protein metabolism and other physiologic effects in four 
healthy young men who were immobilized in plaster casts 
in bed for 6 weeks. 

Fisher SV: A literature review: the energy cost of ambulation 
in health and disability. Arch Phys Med Rehabil 
59:124-133, 1978. 

The energy cost of walking and the effects of prosthetics, 
orthotics, and ambulation aids on energy demands are 
reviewed. 

Fordyce WE: Psychological assesment and management. In 
Krusen FJ, Kottke FJ, Ellwood PM (eds): Handbook of 
Physical Medicine and Rehabilitation. Ed 2, Philadelphia, 
Saunders, 1971, pp. 168-195. 

This chapter reviews the psychological reaction to 
disability and dependency. Strategies for psychological 
assessment and management are presented, with a 
discussion of the role of behavior modification techniques 
in rehabilitation. 

Gordon, EE: Energy costs of activities in health and disease. 
Arch Intern Med 101:702-713, 1958. 

This article reviews the energy requirements of normal and 
handicapped persons during many ordinary activities and 
various occupations. 

Kottke FJ: Therapeutic exercise. In Krusen FH, Kottke FJ, 
Ellwood PM (eds): Handbook of Physical Medicine and 
Rehabilitation. Ed 2, Philadelphia, Saunders, 1971, 
pp. 385-428. 

This chapter reviews the pathology of contracture 
formation and the clinical assessment and management of 
contractures. Several helpful diagrams supplement the 
text. 

Muller EA: Influence of training and of inactivity on muscle 
strength. Arch Phys Med Rehabil 51:449-462, 1970. 
A good review of the data on muscle strengthening 
exercises and disuse atrophy. The author has been 
prominent in exercise physiology for many years, and 
pioneered the concept of isometric exercise. 



5 SPINAL CORD INJURY 



William H. Donovan, M.D. 



DISEASE DESCRIPTION 

The vast majority of individuals paralyzed as a 
result of spinal cord injury are young. Recent studies 
have shown that while the mean age of spinal cord in- 
jured patients is about 30 years, the median age is 25 
years, and the age groups most represented lie between 
18 and 21. Therefore, most patients are attending or 
have dropped out of high school, or they have completed 
high school and have embarked on college education or 
employment. Because they generally survive the injury 
with intact cognitive faculties, they still have potential 
vocational careers ahead of them. 

The incidence of spinal cord injury in the United 
States is about 25-30 new injuries per million 
population per year. The data on accidents responsible 
for spinal cord injury show that the automobile has 
accounted for 35 percent of all spinal cord injuries, falls 
15 percent, gunshot wounds 10 percent, and diving 
accidents 6 percent. 

Spinal Cord Anatomy 

The spinal cord lies within the vertebral canal 
formed by the vertebrae of the spinal column (see 
chap. 3). The spinal cord iteself does not occupy the 
entire length of the vertebral canal, but rather ends at 
the level of the LI vertebra (fig. 5-1). Openings 
(intervertebral foramina, see fig. 3-21. p. 35) between 
adjacent vertebrae lie along both sides of the vertebral 
column, through which motor and sensory spinal 
nerve roots exit from the spinal cord and course 
outward to various parts of the body. Nerve roots 
derived from the cervical and thoracic part of the spinal 
cord leave the vertebra] canal through intervertebral 
foramina close to their origin. On the other hand, roots 
exiting the spinal cord around and below LI travel first 
down the vertebral canal to their respective intervertebral 
foramina before exiting. These nerve roots are referred 
to as the cauda equina. 

The neural tissue within the spinal cord consists 
basically of nerve cells and nerve fiber tracts. Fiber 
tracts, in particular the corticospinal tract, carry 
motor, or movement, messages down the spinal cord 
from the brain to the gray matter (anterior horns) 



within the spinal cord. The anterior horn cells within 
the spinal cord then transmit impulses out the anterior 

C1 





2 

3 
4 
5 
6 
7 
8 
T1 

2 

3 

4 

5 

6 

7 

8 

9 

10 

11 

12 

L1 



S1 



Coc 1 



FIGURE 5-1. Relationship of spinal nerve roots to vertebrae. 
Termination of spinal cord is at the level of the LI -L2 vertebrae. 



65 



66 Donovan 



spinal roots to the muscles. Sensory messages from the 
skin and other tissues enter the spinal cord via 
posterior spinal roots and travel up the spinal cord to 
the brain (see chap. 3). When a complete spinal cord 
injury occurs, impulses cannot be transmitted either 
down or up fiber tracts past the point of injury. The 
extent of the resultant paralysis and sensory loss is 
determined by the level of the injury. Table 5-1 lists 
the most important muscles and the functions they 
serve at each of the spinal cord levels. 

The first important nerve roots, cervical 3 and 4 (C3 
and C4), travel via the phrenic nerve to the 
diaphragm. The action of this muscle accounts for 
approximately 75 percent of the lung's breathing 
capacity and the intercostal muscles between the ribs 
account for the remaining 25 percent. Injuries below 
C4, therefore, spare the respiratory function and 
individuals are able to breathe adequately to sustain 
life. 

The remaining cervical nerve roots and the first 
thoracic nerve root (Tl) innervate the muscles of the 
upper extremities. Spinal cord injuries affecting any 
segment between C3 and Tl will produce impairment 
of function in the upper extremities, a condition called 
quadriplegia. However, the arms do not have to be 
completely paralyzed for quadriplegia to exist. Rather, 
quadriplegia is present if any of the segments of the 
spinal cord (C5-T1) which innervate the arms are 
involved. 

The thoracic nerve roots innervate the upper and 
lower intercostal, the abdominal, and the middle back 
muscles. Lumbar nerve roots and the first sacral root 
innervate the muscles of the lower extremities, and the 
remaining sacral nerve roots innervate the bowel, 
bladder, and the muscles controlling continence and 
sexual function. Injuries to the spinal cord below Tl 
will therefore spare the upper extremities, and the 
spinal cord impairment is referred to as paraplegia. 

Sensory impulses carry information from the skin 
and other tissues via peripheral nerves and the spinal 
roots into the spinal cord and then to the brain. 
Sensory innervation also has a segmental distribution 
which corresponds to the sensory nerve roots of the 
spinal cord. The distribution of sensation served by 
each root is shown in figure 5-2. 

Mechanism of Spinal Cord Injury 

Considerable force is necessary to disrupt the bones 
and ligaments of the vertebral column. A bone 
fracture, a dislocation (caused by the disruption of 
ligaments between individual vertebrae), or both may 
result from a severe trauma. When this happens, the 
normal configuration of the vertebrae, one on top of 
the other, is altered, and the spinal cord lying within 
the vertebral canal becomes bruised, crushed, or torn 
(fig. 5-3). The degree of the disruption of the vertebral 
column determines how severe the injury to the spinal 



cord will be, and whether sensation and voluntary 
muscle movement below the level of the lesion will be 
completely or only partially lost. 

Following this initial trauma, several things may 
happen that can increase the severity of the injury. The 
disruption of the vertebral column may lead to 
instability at the injury site. Abnormal movement may 
then be possible in this area, and further damage of the 
spinal cord can occur if care is not taken to immobilize 
the patient adequately when he is being rescued. 
Secondly, the tissues around the spinal cord and within 
the spinal cord itself react to the injury by swelling. 
This swelling (edema) reaches a peak at around 48 to 
72 hours after injury. The swelling at the injury site 
may further compromise function in the spinal cord in 
areas adjacent to the injury. Thus, paralysis may 
worsen 2 or 3 days after the injury if steps are not taken 
to reduce the development of edema. 

Therefore, proper management of the patient 
during the acute phase of spinal cord injury is most 
important, since the spinal cord does not have the 
ability to regenerate. If the cells and fiber tracts within 
the spinal cord are completely destroyed at the time of 
injury, no recovery of function below the level of the 
lesion can be expected. However, if the same tissue is 
only partially damaged, then function may still be 
present below the level of the lesion at the time of 
injury and some further recovery of function may 
occur after the stage of spinal shock has passed. 
Obviously, then, every effort must be made to prevent 
further damage to the spinal cord as a result of 
instability, edema, or hemorrhage during the rescue 
and acute phase of treatment. 

Spina] shock is a condition not only of paralysis and 
sensory loss, but also absence of reflexes below the level 
of injury. It generally lasts from several days to several 
weeks. When it has passed, reflexes will return. If the 
injury is in the region of vertebrae T12, LI, L2, or 
below, reflexes in the legs cannot return because the 
afferent and efferent components within the cauda 
equina are damaged. If reflexes return before voluntary 
function is present, it likely that voluntary function will 
never develop and the lesion is complete. In such an 
instance, the spinal cord injury interrupted all tracts to 
and from the brain. Commands originating in the 
precentral gyrus of the frontal lobe of the cerebrum 
cannot be transmitted across the defect, and total 
paralysis occurs below the level of the lesion. Similarly, 
sensory information originating from those parts of 
the body which transmit impulses through nerve roots 
entering the spinal cord below the level of the lesion 
will be unable to travel past the injury site to the 
thalamus for relay to the parietal lobe. Therefore, total 
loss of sensation (anesthesia) will result. 

Frequently, however, a lesion will be referred to as 
being incomplete. This can mean either of the 
following: 

1 . A localized portion of the spinal cord may be 



Spinal Cord Injury 67 



TABLE 5-1 

MUSCLES SUPPLIED AND FUNCTIONS SERVED BY SPINAL NERVE MOTOR ROOTS 



Root Segment 



Representative Muscles 



Function Served 



CI & C2 
C3 & C4 
C5 & C6 

C6 & C7 
C7 & C8 
C8 & Tl 

T2-T6 
T6-T12 

LI, L2, L3 

L3 & L4 
L4, L5, SI 

L5, SI, S2 
S2, S3, S4 



High Neck Muscles 

Diaphragm 

Deltoid 

Biceps 

Extensor Carpi Radialis 
Pronator Teres 

Triceps 

Extensor Digitorum Communis 

Flexor Digitorum Superficialis 
Opponens Pollicis 

Interossei (intrinsics) 

Intercostals 



Intercostals 
\bdominals 



Iliopsoas 
Adductors 



Quadriceps 
Gluteus Medius 
Tibialis Anterior 

Gluteus Maximus 

Gastrocneumius 

Anal Sphincter 
Urethral Sphincter 



Aid in head control 

Inspiration (breathing in) 

Shoulder flexion, abduction 
(arm foward, out to side) 
Elbow flexion (elbow bent) 

Wrist dorsiflexion (back of hand up) 
Wrist pronation (palm down) 

Elbow extension (elbow straight) 
Finger extension ("knuckles" straight) 

Finger flexion (fist clenched) 
Thumb opposition (thumb brought 
to little finger) 
Spreading and closing the fingers 

Forced inspiration (breathing in) 
Expiration (breathing out, coughing) 

Forced inspiration (breathing in) 
Aid in expiration (coughing) 
Aid in trunk flexion (sitting up) 

Hip flexion (thigh to chest) 

Hip adduction (thigh to midline, legs 

together) 

Knee extension (knee straight) 

Hip abduction (thigh out to side, legs 

apart) 

Foot dorsiflexion (foot up, walk on 

heels) 

Hip extension (thigh in line 
with trunk, hips straight, e.g., standing) 
Foot plantar flexion (foot down, walk 
on toes) 

Bowel function (fecal continence) 
Bladder control (urinary continence) 



totally unable to transmit messages in either direction, 
while the remainder of the cord is near normal. 
Examples of this include (a) the Brown-Sequard syndrome, 
in which one side of the cord is functional while the 
opposite side is not; (b) the anterior spinal artery syndrome, 
in which the anterior two -thirds of the spinal cord is 
nonfunctional while the dorsal one-third is functional; 
(c) the reverse of this, namely, the dorsal column 
syndrome; and (d) the central cord syndrome, which applies 
to lesions, usually in the cervical region, where the 
internal or central portion of the cord is nonfunctional 
but the periphery is functional. Examination of figure 
3-10, p. 26, will reveal which tracts and parts of 
the spinal cord are involved in each type of incomplete 
lesion. In pure examples of any of the four conditions 



listed, tracts in unaffected areas are normal and the 
sensory or motor functions which they serve are 
also normal. 

2. The cord is diffusely injured. Such a lesion is 
considered incomplete because certain nerve tracts are 
still functioning, but in an abnormal way. For 
example, the part of the corticospinal tract to the lower 
extremity might be only partially interrupted, 
producing less than normal strength in the legs. 

Spinal cord injuries are also described as upper 
motor neuron lesions or lower motor neuron lesions. 
Generally, bone injuries above the T12-L1 vertebrae, 
where the spinal cord terminates, leave reflexes intact 
below the lesion. Such lesions are termed upper motor 
neuron lesions. The paralyzed leg muscles still have 



68 Donovan 




FIGURE 5-2. Main areas of skin sensation served by the sensory components of each spina) nerve. Some overlap into neighboring 
segments also exists. (A) Front view. (B) Back view. 

reflexes. On the other hand, when the lesion occurs (the conus medullaris) or the cauda equina, the afferent 
either around the T12, LI, L2 vertebrae or below, and efferent components of reflex arcs are interrupted, 
thereby destroying the lowest portion of the spinal cord Therefore, the paralyzed extremities will be without 



Spinal Cord Injury 69 



Vertebral body 



Crushed spinal cord 



Dislocated neural 
arch joints 




Intervertebral 
disc 



Anterior 

longitudinal 

ligament 



Ruptured 

interspinous 

ligament 



FIGURE 5-3. Vertebral dislocation with rupture of disc and pos- 
terior interspinous ligaments. Neural arch joints are totally 
dislocated. Spinal cord is crushed and angulated by the dis- 
placement. 



reflexes. This is referred to as a lower motor neuron lesion 
since the symptoms result from destruction of anterior 
horn cells or their axons. 



Complications 

Paralysis and anesthesia, the primary results of 
spinal cord injury, whether partial or complete, may 
lead to the development of complications in other areas 
of the body. These complications are listed below, and 
some of them will be discussed in more detail later. 
Acute phase (first month). 

1. Respiratory: (a) infection in the lungs (pneumonia); 
(b) collapse of all or part of a lung (atelectasis); (c) 
occlusion of the pulmonary artery (pulmonary 
embolism) from blood clots arising from veins of the 
legs or pelvis (thrombophlebitis) which break off and 
float to the lungs (pulmonary embolus); (d) need for 
tracheostomy for assisted or improved breathing. 

2. Gastrointestinal: (a) gastrointestinal bleeding, 
usually caused by ulcers in the stomach which can be 
related to stress; (b) absence of intestinal movement 
(paralytic ileus) which accompanies spinal shock and 
interferes with nutrition. 

3. Genitourinary: (a) infection of the kidney 
(pyelonephritis); (b) distention of the bladder, due to 
plugging of a catheter or inadequate frequency of 
catheterization; (c) urethral fistulae due to improper 
positioning of urethral catheter; (d) formation of 
abnormal solidified masses of mineral salts (calculi), 



either in the bladder or in the kidneys, due to the high 
content of calcium in the urine following paralysis. 

4. Cardiovascular: (a) sudden increase of blood 
pressure, with profuse sweating and flushing 
(autonomic dysreflexia), usually caused by 
overdistention of the bowel or bladder; (b) lowered 
blood pressure when changing from a supine to an 
erect position (orthostatic hypotension), due to the 
inability of blood vessels to accommodate rapidly to 
change in posture and maintain an adequate blood 
pressure; (c) inflammation of veins followed by 
formation of blood clots (thrombophlebitis). 

5. Skin: (a) pressure sores (decubitus ulcers), due to 
an inability of the patient to shift his weight because of 
paralysis, and the absence of pain appreciation when 
lying too long without moving. Patients with complete 
lesions are liable to develop skin breakdown over bony 
prominences due to sustained pressure on the skin for 
long periods of time. These ulcers are likely to develop 
over the buttocks, the sides of the hips, or the sacrum 
(low back), depending upon whether the individual is 
spending most of his time sitting, lying on his side, or 
lying on his back. They are also likely to develop over 
the heels, knees, and other bony areas (see fig. 4-4, 
p. 61). 

6. Musculoskeletal: (a) abnormal formation of calcium 
in muscles surrounding joints (myositis ossificans); (b) 
loss of range of motion of joints (contractures), which 
may occur if the joints are not moved by a therapist or 
other person; (c) vertebral column instability. 



70 Donovan 



Chronic phase (usually after 1 month). 

1. Respiratory: (a) pneumonia; (b) lung collapse 
(atelectasis); (c) pulmonary embolus (less likely to 
occur beyond 3 months from the date of injury). 

2. Genitourinary: (a) pyelonephritis, calculi, and 
urethral fistulae must always be guarded against, even 
in the chronic phase; (b) infection of the prostate gland 
(prostatitis) and infection of the epididymis and testis 
are ever-present possibilities in males, particularly 
those who have indwelling catheters in their bladders; 
(c) urine from the bladder flowing back up towards the 
kidneys (urethral reflux) can develop, particularly in 
bladders that are infected. 

3. Cardiovascular: All the complications listed above 
for the acute phase are likely to develop in the chronic 
phase. 

4. Skin: Formation of pressure sores is a persistent 
source of concern at any time in the chronic phase. 

5. Musculo 'skeletal: Fractures of the long bones, 
particularly of the legs, secondary to osteoporosis; 
contractures; myositis ossificans; and vertebral column 
instability with pain are problems that can also occur in 
the late stages. 

6. Neurologic: (a) abnormal painful sensations 
below the level of the lesion (paraesthesia); (b) 
excessive sweating (diaphoresis), together with 
autonomic dysreflexia; (c) spasticity, an exaggerated 
involuntary reflex activity of paralyzed muscles, can be 
a serous, functionally-limiting complication in upper 
motor neuron lesions. 



FUNCTIONAL DISABILITIES 

Physical Disabilities 

As one might infer, the physical limitations in a 
spinal cord injured patient vary gready depending 
upon the level of the lesion. Impairment of some or all 
of the following functions is present: personal hygiene 
(i.e., bathing, grooming, and socially acceptable bowel 
and bladder function); ambulation (bipedal or 
wheelchair); transfers (i.e., movement on and off beds, 
chairs, and toilets, in and out of cars and tubs); 
dressing; eating; writing; and automobile driving. The 
higher the level of the injury, the more extensive the 
disabilities. In appropriate treatment facilities, many 
of the disabilities originally present can be completely 
or partially removed through adaptive techniques and 
training. The level of performance at any time, either 
before or after treatment, is characterized by one of the 
following: 

1 . Independence. The activity can be physically 
performed with consistency and safety by the 
patient alone, without anyone else in the near 
vicinity. 

2. Needs standby assistance. The patient can physically 
perform the activity, but not consistently. 



Therefore, someone needs to be there during the 
activity to ensure safety. 

3. Needs partial physical assistance. The patient can 
perform most of the activity himself consistently 
and safely, but cannot complete the activity 
without some physical assistance. 

4. Total dependence. For the activity to be part of the 
patient's repertoire, someone else needs to 
perform nearly all of the task for him. 

The following discussion considers these functions 
for each injury level and indicates what functions are 
impaired as a result of the injury and to what degree 
they are removed with appropriate treatment. Table 
5-2 summarizes this in more detail. 

At all injury levels genital sexual functions are 
impaired. (Page 78 in Total Treatment details these 
impairments and chapter 21 discusses approaches to 
treatment.) 

Sacral (S2, S3, Si) levels. Only bowel and bladder 
function is initially impaired with injuries at these 
levels. With appropriate treatment, the patient can 
obtain complete independence in the execution of these 
functions. 

Lumbosacral (L5, SI, S2) levels. Initially, 
bowel, bladder, and ambulation functions are 
impaired. Complete independence in bowel and 
bladder function is achieved through appropriate 
treatment. Independence in bipedal ambulation is also 
restored with the assistance of two canes or two 
crutches and sometimes short leg braces (ankle -foot 
orthoses, AFO). Prolonged standing may remain 
impaired. No wheelchair is necessary. 

Lumbar (LI, L2, L3, L4) levels. Initially, 
bowel, bladder, and ambulation functions are 
impaired. With treatment, bowel and bladder function 
can become independent. Bipedal ambulation can 
become independent for short distances. Wheelchairs 
are also used, in which the patient develops complete 
independence. 

Thoracic (T7-T12) levels. Initially, personal 
hvgiene, ambulation, transfers, dressing, and driving 
are impaired. Complete independence in bowel and 
bladder function can be achieved. Independent bipedal 
ambulation is usually achieved only for exercise, and 
complete independence in wheelchair ambulation is 
achieved. With training, transfers, dressing, and 
driving become independent. 

Thoracic (T2-T6) levels. Initially, personal 
hygiene, ambulation, transfers, dressing, and driving 
are impaired. With extensive treatment, the patient 
can become independent in all of these functions. The 
ambulation achieved is by wheelchair. 

Cervical (CI, C8, Tl) levels. Initially, personal 
hvgiene, ambulation, transfers, eating, dressing, 
writing, and driving are impaired. Almost all patients 
in this group become completely independent, with 
extensive treatment, in all functions consistent with 



Spinal Cord Injun' 71 



TABLE 5-2 

FUNCTIONAL EXPECTATIONS AND EQUIPMENT REQUIRED 

FOR SPINAL CORD INJURY PATIENTS 

(Levels indicate lowest functioning nerve root) 



Activities 


C5 


C6 


C7- 
C8 


TI- 
TS 


T6- 
T12 


Ll- 

L3 


L4- 
Sl 


Self care: 
Eating 


D/P, 


P 2 


P 2 /I 










Dressing 


D 


P/I 


P/I 










Toileting 


D 


P 2 /I 


P2/I 










Work with hands: 
Writing 


D 


P2 


P2 










Typing 


p m 


'2.3 


Is 










Operating telephone 


P 


I 


I 










Bed Independence: 

Rolling oven sitting up 


P 4.5 


P5/I 


I 










Wheelchair Independence: 

Transfers to/from wheelchair 


D 


P/I 


I 










Propel wheelchair 


U 


L 


I 










Ambulation 


o 


O 


O 


D 8 


Pe 


I9 


1,0 


Driving with hand controls 


p 


I 


I 










Public transportation: 
Bus 


o 


O 


O 


O 


O 


O 


P/I 


Train 


D 


D/P 


P 










Plane 


P 


I 


I 










Public toilets 


O 


Pn 


Pn 


Pn 


Pn 


Ml, 9 





LEGEND 



Complete independence is possible 

Partial independence is possible with use of special equipment 

Dependence is to be expected 

Not possible 



With electric or C0 2 powered splints 

With nonpowered hand splint 

With electric typewriter 

With loops, bafkan frame— electric bed 

With electric bed 

With electric wheelchair 



living alone. Independent ambulation is by 
wheelchair. For some patients, however, personal 
hygiene may require partial physical assistance, 
transfers may require standby assistance, and dressing 
may require partial physical assistance. 

Cervical (Cb) level. Initially, all functions are 
impaired. Onlv a small number of patients achieve 
complete independence in all functions, and living 
alone is not practical even for these patients. The 
majority require partial physical assistance for personal 
hygiene. Wheelchair ambulation is independent. 
Transfers require standby assistance and dressing 
requires partial physical assistance. Complete 
independence is achieved in eating, writing (although 
slower than usual), and driving. 

Cervical (C>) level. Initially, all functions are 
impaired. With extensive treatment, independence in 
electric wheelchair ambulation is achieved and nearly 
full eating skills are achieved. Total physical 



With adapted hand rims (special lugs or hose wrapped) 

Not practical 

With long leg brace and crutches 

With short leg braces and crutches 

With zipper-back wheelchair 



dependence remains for personal hygiene, transfers, 
dressing, writing, and driving. 

Cervical (above C5) level. Initially, all functions 
are impaired. In addition, the breathing function is 
severely compromised. Training in the use of special 
respiratory equipment is required. The patient 
remains totally physically dependent for all functions, 
although some self- propulsion in an electric 
wheelchair is possible. 

Psychosocial Disabilities 

In addition to their physical disabilities, it is well 
recognized that spinal cord injured patients usually 
suffer psychosocial disabilities as well. Patients and 
their families must make a tremendous adjustment to 
the sudden changes in their lives. Patients must come 
to accept their physical disabilities and learn to 
function as independently as possible as handicapped 



72 Donovan 



persons in a world full of normal people who have very 
little concept of the architectural and attitudinal 
barriers that the disabled must face. 

Both patients and their families are likely to make 
this major adjustment if they receive the appropriate 
guidance and continued counseling from all members 
of the treatme"*. team, beginning as soon as possible 
after the injury. From the beginning, emphasis is 
placed upon the positive, i.e., things that patients can 
still do or learn to do rather than the negative, i.e., 
those activities which are now impossible. If the 
hospital staff, the family, and the counselor focus their 
attention on the positive and offer the patient support 
for every task completed along the rehabilitation 
pathway, constant brooding over residual deficits is 
less likely to persist. 

Generally speaking, the injury heralds the onset of a 
crisis phase and a significant reduction in activity 
caused by the abrupt onset of paralysis. Significant 
emotional problems are common during the first few 
days and weeks thereafter. Frequently, they are 
accompanied by a mixture of disorganization and 
depression. As time goes on, the emotional crisis state 
should abate. If this state seems to continue 
excessively, it usually means that the environment 
either supports the depression or fails to provide a 
viable alternative. 

Depression at this stage is to be expected. If it does 
not appear, one should strongly suspect that the patient 
has not yet accepted the reality of the situation. He 
may be thinking or verbalizing that "this really didn't 
happen to me" or "the doctors overemphasize the 
importance of all this and I'm going to walk out of here 
regardless of what they say." Depression may 
therefore be considered a "good sign" in that it 
generally means that the patient is no longer denying 
the reality of the situation. This reactive depression 
seldom requires pharmacological management unless 
it interferes with treatment. Intervention of the 
rehabilitation team generally consists of encouraging 
the patient to behave in ways that are appropriate to 
his disability — that is, to acquire behaviors necessary 
for successfully facing life as a disabled person. 

The rate or degree with which an individual 
incorporates these behaviors and succeeds in 
increasing his activity during the rehabilitation phase 
depends to a large extent on his preinjury personality. 
If he was prudent, careful, and persistent in the past, 
his chances of success are greater. If he was careless, 
imprudent, and reckless, as many persons who sustain 
spinal cord injuries are, the rehabilitation phase will be 
more difficult. If his personality was primarily motor- 
manipulative centered and he viewed the world of 
physical movement and sports as primarily 
reinforcing, it will be more difficult for him to adapt 
to a life of relative immobility than for a person who 
is either symbol centered or interpersonal centered. 



For some, persistent use of denial mechanisms may 
become a significant psychological problem. Such 
patients are prone to achieve less satisfactory 
rehabilitation results and less than optimum 
continuing good health, and are more prone to medical 
complications through carelessness. 

Finally, social and financial problems are common. 
The costs are high, and the disruption of family and 
social roles is extensive. 



Rehabilitation Potential 

As discussed above, capabilities of spinal cord 
injured patients will vary widely. Their overall 
ultimate functional ability will depend upon the level of 
the lesion and residual muscle function, the extent and 
success of their rehabilitation training, their 
psychological adjustment to disability, their overall 
general medical health, their preinjury vocational or 
educational progress, and their postinjury educational 
and vocational progress. When these factors are 
known, a vocational counselor may plan long-term 
vocational goals for his client. The lesion itself does not 
become progressively worse and long life can be 
anticipated. 

If a counselor follows the patient's course while he is 
still in the rehabilitation center, he will understand 
how these factors are developing. If the client is 
referred at a later stage, the counselor can still 
understand the progress of these factors from a careful 
review of what has occurred. If it appears that mobility 
training is being impeded by the appearance of medical 
complications, or the patient is remaining depressed 
longer than anticipated and is still uninterested in 
pursuing vocational goals, then short-term 
contingencies might best be planned. If these short- 
term goals are met, and a pattern of meeting the short- 
term goals is established, long-term plans can be 
made. 



STANDARDS OF EVALUATION 

Acute Phase 

Ideally, when a patient is admitted to a spinal cord 
injury center with an acute injury he should have the 
services of at least four medical specialists available to 
him: the neurosurgeon, the orthopedist, the urologist, 
and the physiatrist or rehabilitation specialist. 
Additional consultation may also be needed from a 
plastic surgeon and an internist, depending upon the 
patient's initial problems. 

If an acutely injured patient is brought directly to 
the Emergency Room of the spinal cord injury center, 
the initial attending physician is either the 
neurosurgeon or the orthopedist, with the others acting 
as consultants. Either surgeon will determine the 



Spinal Cord Injury 73 



extent of paralysis, initiate protective measures for the 
paralyzed parts of the body, and make decisions 
regarding surgery. 

Attending physician responsibility usually shifts in a 
spinal cord injury center to the rehabilitation 
specialists within 4 weeks after injury, with the other 
physicians assuming consultative roles. During this 
initial period, evaluations are performed by a social 
worker, clinical psychologist, rehabilitation nurse, 
vocational counselor, physical and occupational 
therapists, and frequently by an orthotist and speech 
therapist as well. While acute care is sometimes 
administered away from a rehabilitation center with 
later transfer to the facility, it is considered less 
optimum because collective expertise is more likely to 
exist at the special facility. 

Postacute Phase 

Following the evaluation, and under the leadership 
of the physiatrist or rehabilitation specialist, the spinal 
cord injury team meets collectively and formulates 
initial treatment goals with respect to medical 
treatment needs, ultimate bowel and bladder systems, 
ambulation, transfers, dressing, eating, and personal 
hygiene skills, emotional, social and family support 
systems, and initial vocational directions. An initial 
treatment plan directed toward these goals is 
formulated and instituted with the patient. 

A continuing evaluation of treatment, including goal 
modification, continues until initial discharge, with 
collective review by the team members in concert with 
the patient and his family on a weekly or biweekly 
basis. 

While increasingly less frequent, some spinal cord 
injured patients receive acute treatment and some 
rehabilitation care at a community hospital incompletely 
staffed with the professionals common to a 
rehabilitation or spinal cord injury treatment center 
and present themselves to a vocational counselor 
without the prior benefit of comprehensive 
rehabilitation treatment. To ensure the achievement of 
maximum function, the counselor should receive an 
evaluation at a medical rehabilitation center where a 
collective review can be performed by a medical 
rehabilitation physician and his medical consultants 
and, in addition, at least a clinical psychologist, social 
worker, and physical and occupational therapists, 
together with the patient and the counselor. Such a 
review can be achieved on an outpatient basis or 
during a short inpatient stay. 

Maintenance 

During rehabilitation in a spinal cord injury center, 
the patient should have acquired skills and necessary 
equipment appropriate for the level and degree of 
completeness of his lesion so that he is as independent 



as possible. He should be in excellent general medical 
health. He should have had a chance to interact with 
the staff, his peers, and normal individuals outside the 
hospital in a meaningful way so that he is prepared to 
meet the world as a disabled person. He should have 
developed some appropriate mechanisms to cope with 
the fact that he is different from most people, yet 
possess the confidence and feeling of self- worth 
necessary to carry on a meaningful existence. He 
should have acquired the knowledge and skill necessary 
to maintain his general health. This includes taking 
prescribed medications and carrying out vital 
techniques and routines, such as performing push-ups 
in the wheelchair to relieve pressure on his skin, 
performing a bowel program to maintain bowel 
continence, and following the necessary steps to ensure 
that his bladder will remain as free of infection as 
possible. Finally, he should have had a sampling of 
some work -related experience in order to develop the 
skills necessary to carry on a work or school routine 
once discharged. 

Maintenance of health and achieved goals and 
possible further enhancement of goals requires 
continuing followup evaluation, particularly to abort 
potential medical or psychosocial complications (see 
above). This evaluation includes periodic evaluation 
by knowledgeable rehabilitation physicians and 
necessary allied health staff at least three to four times 
the first year, or more often if required. Urological re- 
evaluation at least twice a year initially and once a year 
thereafter is essential. 

TOTAL TREATMENT 

Acute Phase 

Important factors in the early medical treatment of 
the spinal cord injured patient include: 

1. Support of respiration. Sometimes a trach- 
eostomy must be performed in quadriplegics or 
high paraplegics with chest injuries to prevent 
complications associated with lung collapse 
(atelectasis), such as pneumonia and lung abscess. 

2. Maintenance of blood pressure. 

3. Maintenance of adequate urine output and 
adequate drainage of the urinary bladder, since the 
inability to void can be predicted with virtual certainty. 

4. Protection of the anesthetic skin by adherence to 
a frequent turning schedule so that pressure sores 
(decubiti) do not develop over bony prominences 

5. Maintenance of range of motion of all the joints 
in the paralyzed portions of the extremities so that rigid 
contractures do not develop and interfere with mobility 
training. 

6. Appropriate attention to the gastrointestinal 
tract, including close observation and immediate 
treatment of stomach ulcers and hemorrhages, should 
they develop, and the institution of an appropriate 



74 Donovan 



bowel program to ensure effective and predictable 
elimination once bowel activity has resumed. 

7. Institution of appropriate measures to prevent 
pooling of blood in the veins (venous stasis) due to loss of 
muscle activity in the legs, which may in turn lead to 
the development of thrombophlebitis and, subsequently, 
to pulmonary emboli if a clot in a vein should break 
off, flow through the heart, and lodge in a lung. 

In addition to the precautionary measures just 
given, a physician in charge of the acutely injured 
patient must decide on the preferred method of 
treating the spinal cord itself. This will in large part 
depend upon (a) the nature of the injury and related 
trauma; (b) the mechanism that produced it; (c) 
whether it is a complete or an incomplete lesion; and 
(d) whether the vertebral column is stable or unstable 
and needs surgery. 

An example of an acute treatment regimen for a 
cervical spinal cord injury might include the insertion 
into the skull of tongs to which traction can be applied 
to distract and realign the bony elements of the cervical 
vertebrae. If distraction in this way does not result in 
realignment, the physician may try to realign the 
vertebrae by manipulation under anesthesia. He may 
decide to keep the patient in tongs in bed on a special 
frame for at least 6 weeks, or alternatively, to operate 
and fuse the adjacent injured vertebrae in the area of 
the spinal cord injury with a piece of the patient's own 
bone, generally taken from the pelvis. When this bone 
graft fuses (usually by 6 to 12 weeks), stability of the 
injured vertebral column is usually assured so that 
further trauma to the spinal cord will not ensue. If this 
decision is made, the individual is generally kept in bed 
for another 2 weeks postoperatively, followed by 
immobilization in a protective cervical collar. Whether 
fusion is carried out or the patient is kept in bed on a 
frame with or without tongs, a collar is generally worn 
for a period of 6 weeks once mobilization has begun, 
and usually the patient can gradually be weaned from 
the external protective device. 

Typical management for paraplegia is similar in 
concept to the above and will differ according to the 
same variables. Since an injury in the area of the 
junction of the thoracic and lumbar regions (i.e., at 
T12-L1) is often unstable, a decision to fuse the 
adjacent vertebrae may be made. Realignment of the 
bony fragments is again often attempted either 
preoperatively or operatively. Injuries within the 
thoracic region are often stable without surgery 
because the adjacent vertebrae are attached to each 
other via the ribs. However, if multiple rib fractures 
are also present, this stability is often lost and surgery 
is attempted. Various metal implants such as 
"Harrington rods" and "Weiss springs" are often 
used at surgery to provide internal immobilization of 
the vertebrae of paraplegics. 

Generally speaking, if a lesion is incomplete and the 



patient is improving, surgical intervention is deferred, 
since a stabilization procedure can be done electively at 
a later date, and the patient would have a longer bed 
rest period. If the patient acutely develops a 
progressively higher level of paralysis, immediate 
investigations must be carried out to determine if there 
is increasing pressure developing on the cord that 
should be relieved. This can be accomplished by (a) 
realigning the vertebrae; (b) removing material 
pressing on the cord, such as disc material, bone chips, 
or hematoma; or in some instances, (c) removing the 
roof (lamina) of one or more vertebrae. In addition, 
steroid medication intended to prevent or reduce 
swelling of the spinal cord in response to the injury is 
also given within 72 hours. Change in the neurologic 
status might occur under circumstances where the 
spinal cord is compressed further because an unstable 
lesion developed a progressive bony deformity or a cyst 
developed within the spinal cord. In most instances this 
does not occur and the level of paralysis remains 
unchanged. It should be realized, however, that a 
patient's general medical condition can deteriorate 
from complications that may develop in other areas of 
the body, such as the lungs, kidneys, or skin. 

Other than relieving pressure on the cord, 
controlling edema, and attempting to restore stability, 
one must await the results of time and natural healing 
processes to determine the ultimate residual deficits. 

Rehabilitation Phase 

The postacute treatment or rehabilitation phase is 
concerned with the specific treatments to achieve the 
goals established by the staff with the patient and his 
family in (a) bladder function, (b) bowel function, (c) 
skin care system, (d) psychological adjustment, (e) 
social, family, and home planning, (f) vocational 
planning, (g) ambulation and transportation, (h) 
transfers, (i) eating, (j) dressing, (k) personal hygiene, 
and others as may be necessary. 

Bladder function. The ideal end result in bladder 
training is to achieve a sterile urine free of bacteria; a 
balanced bladder; a system for voiding which is socially 
acceptable and does not soil clothes; the absence of the 
need for a catheter; and maintenance of the anatomical 
integrity of the kidneys, ureters, bladder, and urethra. 

The ideal is not always achieved. While bacteria 
may not be completely eliminated from the urine, 
excellent control and prevention of actual infection 
manifested by real sickness is, in almost all instances, 
possible with appropriate antibiotics. A bladder is said 
to be balanced when the amount of urine voided at any 
one time is much larger than the amount of urine left 
in the bladder (residual urine). The usual requirement 
is a residual urine that is no more than 25 percent of 
total bladder capacity. 

The current preferred technique of working toward 



Spinal Cord Injun' 75 



the goals listed above is the use of intermittent 
catheterization until urine begins to flow and the 
bladder empties by itself or with appropriate 
stimulation by the patient. Electrical and other devices 
for stimulating the bladder for voiding or for control of 
the sphincter are likely to be available in the near 
future. With intermittent catheterization the urine can 
be maintained sterile. A less desirable technique is the 
use of the continuous indwelling Foley catheter to 
drain the urine until such time as the physicians deem 
trials at voiding are feasible. The catheter is then 
removed and voiding trials begin in an effort to work 
toward a balanced bladder. The urine cannot be 
maintained sterile while the indwelling Foley catheter 
is in place. With intermittent catheterization about 
every 4, 6, or 8 hours, the patient can have a nearly 
normal fluid intake. With an indwelling Foley 
catheter, the patient must consume large volumes of 
fluid, up to three liters per day, to dilute the bacteria. 
In addition, he often must use an irrigant to prevent 
crusts from attaching to the catheter and must ensure 
the maintenance of a low urine acidity through 
appropriate medication. Other factors that contribute 
to the achievement of a balanced bladder and a socially 
acceptable voiding system vary at the different levels of 
injury. 

The prevention of the complication of autonomic 
dysreflexia, particularly in lesions above T6, deserves 
special mention. Autonomic dysreflexia is often 
induced by an overly distended bladder which triggers 
off the sympathetic nervous system, causes the blood 
pressure to increase to dangerous levels, and is 
associated with a pounding headache. Educating the 
patient in the control and prevention of this potentially 
dangerous complication is necessary. 

Determination of the best bladder emptying system 
depends upon the neurological exam, the character of 
the bladder reflexes as assessed by cystometrogram 
(CMG) tests, the character of the urethral sphincter, 
and the general health of the urinary tract. The latter is 
assessed by urinanalysis, bacterial culture of the urine, 
and an excretory urinary (intravenous) pyelogram 
(IVP or EUP). An EUP is a special X-ray test to 
visualize the kidneys, ureters, and bladder, and a 
cystogram is another special X-ray test to visualize the 
bladder. Very often a movie -type X-ray film of the 
actual process of the emptying of a dye instilled in the 
bladder is also performed. 

Bowel function. The development of sociallv 
acceptable regular bowel elimination is often easily 
achieved as long as the patient maintains proper diet 
and fluid intake, and avoids foods and infections that 
are productive of diarrhea. If diarrhea occurs, soiling is 
inevitable. Control of the anal sphincter is affected by 
spinal cord injury, but the reflexes for bowel function 
are not particularly disturbed. The patient controls the 
timing of elimination through the use of special 
suppositories (e.g., Dulcolax) and digital distention of 



his anal sphincter, coupled with the ingestion of food 
shortly before the elimination. Stool softening 
medications (e.g., Colace) can also help. 

Skin care system. In areas when- the skin is 
anesthetic, particularlv over bom prominences, the 
patient is unable to appreciate when too much pressure 
has been sustained for too long. Unrelieved pressure 
for a length of time sufficient to squeeze blood out of 
the skin will cause skin breakdown. Ulcers produced in 
this way must be studiously avoided because they 
severely interfere with the patient's independence and 
often confine him to bed for extended periods. They 
may even require surgical closure. Initially, the patient 
must be periodically turned and moved by the staff and 
nurses to relieve pressure on skin areas susceptible to 
breakdown. Later, the patient is trained how to do this 
himself or to have others assist him, so that periodic 
relief over pressure areas is achieved. 

Psychological adjustment. Psychological treatment 
consists of assisting the patient through a depres- 
sion phase and helping him to curtail the use of 
denial mechanisms to handle the stress of the illness, 
particularly if such mechanisms are interfering with his 
ability to learn how to care for himself and move 
actively toward achieving rehabilitation goals. 
Therapeutic tools available are those dealing with 
operant behavior modification techniques, individual 
counseling with patient and family members where 
appropriate, and group therapy techniques. These and 
often other methods, coupled with increasingly intense 
forays into the public, help the patient to make a 
satisfactory social and vocational adjustment. 

Social, family, and home planning. The patient 
and his family are actively involved with a social 
service worker who assists in the maintenance of stable 
interpersonal relationships and the family's relocation. 
(Psychological, social, and sexual issues are further 
discussed in chapters 2, 16, and 21.) 

Vocational planning. Vocational planning builds 
on the patient's prior educational and vocational 
experience, his current interests and aptitudes, his 
ultimate levels of function and mobility after treatment 
of his spinal cord injury, and the nature of the labor 
market. While initially vocational explorations start at 
a low key, they build up to a crescendo as rehabilitation 
and functional goals are achieved. 

Spasticity. Although control of spasticity is often 
not a specific goal in itself, spasticity is an issue that 
greatly interferes with functions such as ambulation, 
transfers, dressing, bladder function, and skin care. If 
the reflexes in the lower extremities below the level of 
the lesion become so hyperactive that they are difficult 
to control and therefore interfere with the above 
functions, specific treatment to reduce the intensity of 
these spasms is necessary. Current medications are 
Valium (diazepam) and Dantrium (dantrolene 
sodium). Other medications will certainly be 
developed in the future. If control by medication is 



76 Donovan 



insufficent, a so-called motor point block is 
performed, in which a dilute solution of phenol is 
injected into the small motor nerves close to the motor 
end plates of muscles. Sometimes it is even necessary 
to actually cut a nerve or a nerve root. In the most 
severe cases, a surgical procedure called a Bischoff 
myelotomy (a longitudinal section of the middle 
portion of the spinal cord on both sides) may be 
performed by a neurosurgeon. This operation is 
performed in the part of the spinal cord where there is 
paralysis, hence no additional weakness results. 
Maintenance of good general health is essential in the 
control of spasticity since it can be aggravated by any 
infection in the lower extremities (such as in the 
toenails), bladder infections, or skin sores of any sort. 

Functional goals. The achievement of functional 
goals in ambulation, transportation, transfers, eating, 
dressing, and personal hygiene have as their basis the 
maximum strengthening of the muscles that are 
unaffected by the paralysis, the prevention of 
contracture in the parts of the body that are paralyzed, 
and the teaching of special techniques to utilize the 
remaining functional parts to assist the achievement of 
these skills. 

Generally speaking, physical therapists assist in the 
development of ambulation and transfer skills, while 
occupational therapists ordinarily deal with the 
development of eating, dressing, personal hygiene, 
and transportation skills. Table 5-2 presents the 
ultimate optimum expectation for independence and 
the necessary aids in these main functional areas. 
Certain additonal comments with relation to the 
different levels of injuries are given below. In table 5-2 
and in all of the following comments, the assumption is 
that the spinal cord injury is complete. If the spinal 
cord injury is incomplete, then achievable goals are 
greater than those listed. The level of spinal cord injury 
given refers to the lowest level that is still functioning 
and under voluntary control. This level, which is the 
more significant factor to consider, is not always 
synonymous with the actual level of the bone injury. 

1 . Levels L5, SJ, S2. Ultimate bladder function is 
usually excellent in these patients. No catheters are 
needed. Elimination is usually accomplished by 
increasing abdominal pressure (bearing down). 
Residual urines close to zero are generally achievable. 
Since at these levels the sphincter might be weak, some 
dribbling may occur. If dribbling is significant, males 
may use an external collecting system consisting of a 
condom, a tube, and a collecting bag, and females 
might wear absorbent pads. In some instances, 
artificial sphincters can be surgically implanted. They 
are still relatively experimental and require the patient 
to inflate and deflate the artificial sphincter by 
squeezing hydraulic fluid through tubes and valves 
implanted under the skin. Bowel function results are 
quite good. Again, since sphincters may be lax, some 



leakage may occur, and hence the patient generally 
might prefer a more constipated stool. 

To circumvent the male patient's inability or 
difficulty in obtaining an erection, plastic devices such 
as dildos can be worn, or a prosthesis can be surgically 
implanted within the penis. Further, the erection 
mechanism can be influenced by surgery performed on 
the arterial and venous supply of the penis. Competent 
sexual counseling should be offered before any such 
procedures or devices are recommended so that the 
patients and their partners can assess their present 
attitudes and feelings toward each other. 

Transfers, eating, dressing, and personal hygiene 
pose no problem. 

The patient's ambulation is quite good, usually with 
two short leg braces (ankle -foot orthoses, AFO) that 
essentially "lock'' the ankle joint. Two canes or 
crutches are usually required, although at the lower 
level one cane may be sufficient. Wheelchairs may be 
totally discarded in many cases. At the lower levels, 
automobile driving without special hand controls can 
be achieved, although hand controls may be preferred 
by some patients. No attendant care is necessary. 

The rehabilitation phase of hospitalized treatment is 
about 1 month, usually no more than 2 months. 

2. Levels LI, L2, L3, L4. At these levels urethral and 
anal sphincters are inclined to be spastic and tight. 
Bladder elimination is still usually accomplished by 
abdominal pressure, perhaps accompanied by 
dilatation of the anal sphincter, which also relaxes the 
urethral sphincter and permits complete emptying. 
External collecting systems for the male and absorbent 
pads for the female are less necessary. Bowel leakage is 
less of an issue, although diarrhea would still be a 
problem. Transfers, eating, dressing, and personal 
hygiene are independent after training. 

Bipedal ambulation may now require above -knee 
orthoses (knee -ankle -foot orthoses, KAFO) and two 
crutches or perhaps even a walker. The gait pattern 
used may be the so-called four-point, swing- through, 
or swing -to gait. Wheelchairs at these levels are 
usually not discarded, particularly not at the higher 
level. Automobile driving requires the use of hand 
controls. No attendant is necessary. 

The rehabilitation phase of hospitalized treatment is 
about 3 months, usually no more than 4 months. 

3. LevelsT7-T12. Bladder and bowel function is as 
above for the higher lumbar levels. However, bladder 
reflexes at these levels might be more active. External 
collecting systems for males and pads for females may 
therefore become necessary. If the sphincters are too 
tight, they may have to be surgically weakened with a 
sphincterotomy, particularly in males. 

The higher the level between T7 and T12, the 
greater the loss of control over the abdominal and back 
musculature. Since these muscles are used for 
coughing, the higher the lesion, the less effective the 



Spinal Cord Injury 77 



cough. Therefore, these patients are at greater risk 
from respiratory infections. 

Transfers, eating, dressing, and personal hygiene 
are easily achieved through training. Bipedal 
ambulation at these levels is less and less functional, 
and may only be used for exercise. Long leg 
orthoses(KAFO) and crutches are definintely required, 
but the wheelchair is the main mode of ambulation. 
Therefore, use of public toilets is restricted to those 
that are wheelchair accessible. Hand controls are 
required for automobile driving. 

The rehabilitation phase of hospitalized treatment is 
about 3 months, usually no more than 4 months. 

4. Levels T2-T6. Bladder elimination involves more 
direct triggering of the bladder reflexes as well as 
efforts at anal dilation. Control of leakage is generally 
more difficult and most patients wear external 
collecting systems all the time. Females may find that 
leakage is not controlled sufficiently by pads, and 
hence use of a continuous indwelling catheter with 
drainage to a leg bag may be required. Bowel function 
control is as above. Transfers, eating, dressing, and 
personal hygiene, while achievable, require much 
training. 

Bipedal ambulation is no longer practical even for 
exercise, though some patients might choose it. The 
wheelchair is the main mode of ambulation. Hand 
controls and sometimes external trunk support are 
required for automobile driving. No attendant is 
necessary, although a roommate could be helpful. 

The rehabilitation phase of hospitalized treatment is 
about 3 months, usually no more than 4 months. 

5. Levels C7, C8, Tl. At these levels, the hands and 
the wrists are beginning to become involved. Training 
now takes much longer because special adaptive 
equipment is needed and techniques need to be 
learned. 

Bladder elimination is accomplished by stimulating 
the bladder reflex. Sphincterotomies are often 
necessary in males. External collecting systems in 
males are mandatory, and almost all females use a 
continuous indwelling Foley catheter. Autonomic 
dysreflexia may be a significant problem. Bowel 
function, particularly the insertion of suppositories and 
the use of digital stimulation of the anus, becomes 
more difficult, but can be achieved with a finger splint. 

Modifications of the wheelchair to make propulsion 
easier may be required, such as applying friction tape, 
tubing, or projections on the hand wheel rims. 
Transfers usually require a sliding board, and double - 
width electric beds are helpful. Clothing modifications 
such as the use of snaps or Velcro will circumvent the 
difficulty these patients have with buttons. Dressing 
and personal hygiene may require partial use of 
an attendant for pants and total body bathing. Special 
adaptations of bathroom systems are required. Hand 
controls and sometimes steering wheel attachments are 
necessary for automobile driving. 



The C7 level is the highest level compatible with no 
attendant whatsoever, although not all achieve this 
goal. 

The rehabilitation phase of hospitalized treatment is 
about 4 months, usually no more than 5 months. 

6. Level C6. The only remaining hand and wrist 
function at this level is extension or dorsiflexion of the 
wrist. The patient cannot forcibly extend his elbow. 
Preservation of full range of elbow motion is essential. 

Bladder and bowel function is the same as for the 
C7, C8, and Tl levels. Eating skills are achieved with 
adaptive equipment applied to the hands, such as a 
wrist -driven flexor hinge splint that creates opposition 
of the second and third fingers by dorsiflexing the 
wrist, or a cuff worn over the hand with a slot for a 
spoon or fork. 

Ambulation is still by manually operated wheelchair 
with modifications of the wheel rims as above, 
although in certain situations electric wheelchairs may 
be appropriate. 

The completion of total dressing and personal 
hygiene usually requires an attendant. Some patients 
can achieve this independently with the aid of loops 
sewn onto the clothes into which they can hook a 
finger, but this may be too time consuming. Pull-over 
garments for the upper torso are usually preferable to 
buttoned shirts because of the difficulty with 
manipulation of buttons, snaps, and the like. If the 
arms are long enough, a sliding board is used for 
transfers. Transfers in and out of bed and on and off a 
toilet can be achieved, and car transfers can also be 
achieved by certain patients without assistance. C6 is 
the highest level compatible with automobile driving 
with hand controls at the current time. The use of 
special vans enhances this function. An attendant or at 
least a helpful roommate is usually required to assist 
with dressing, personal hygiene, and some of the 
bladder and bowel care functions. Such patients are 
not likely to be successful living absolutely alone. 

The rehabilitation phase of hospitalized treatment is 
about 6 months, usually no more than 7 months. 

7. Level C5. At this level the patient has shoulder and 
elbow flexion function but no wrist and hand function. 

Bladder elimination is achievable if automatic reflex 
emptying can be developed. If automatic emptying 
does not occur, an attendant is required because the 
patient cannot assist in stimulating reflexes. For bowel 
function, an attendant must provide a suppository, 
and digital and cleanup function. Eating, except 
perhaps for cutting, can be developed with set up (i.e., 
food already cut, cartons opened and straws inserted), 
but transfers, dressing, and personal hygiene are 
literally totally dependent on an attendant. 

Ambulation is by electric wheelchair which the 
patient controls by placing his hand on a stick mounted 
on the control box of the wheelchair on the same side as 
his stronger arm. Automobile driving is generally 
unlikely, but more specialized vans and operating 



78 Donovan 



techniques might allow this to be achieved. A full-time 
attendant is necessary to assist with the functions but 
need not be at the patient's side at all times. With 
appropriate seat cushions, these patients can sit for 8 to 
10 hours a day with perhaps some pressure relief 
provided by the attendant. 

Other adaptive equipment used by some patients 
with lesions at C5 includes battery - powered and cable - 
operated wrist-driven hinge splints, and ballbearing 
feeders (mobile arm supports) anchored to the 
wheelchair which allow the patient to feed himself and 
do some facial grooming. To operate the mobile arm 
supports, the patient places his arms in special troughs 
attached to movable hinged bars. By using the 
shoulder muscles, the patient can make the troughs 
swivel to bring his arms toward his body and move 
them out again, and also to tip the troughs downward at 
the elbow to raise his hands. 

The rehabilitation phase of hospitalized treatment is 
about 3 months, usually no more than 4 months. 

8. Levels C2, CS, C4. The C4 level is the last 
compatible with life, unless at the time of the accident 
assisted respiration was provided by someone in the 
vicinity. The C2 and C3 patients invariably require 
permanent assisted ventilation, as do some of the C4's. 
A battery-powered portable respirator can, for some, 
be fitted to the wheelchair. The C2 and C3 patients 
usually require a permanent tracheostomy and special 
techniques to control lung and throat secretions. 

C2, C3, and C4 patients may be able to use a chin, 
mouth, or breath control device and perhaps a voice - 
controlled device to operate electric reclining 
wheelchairs. They may have greater control of their 
environment thorough so-called environmental 
control systems. In these systems, the patient can 
control a telephone, radio, television, lights, buzzer, 
and the like from one place. Selection of one of these 
options is made by using breath, mouth, sound, or 
residual muscle power to activate the system. A full- 
time highly skilled attendant is required for the patient, 
and probably should always be available near him. 

The rehabilitation phase of hospitalized treatment is 
as long as 6 to 8 months or longer because of the need 
to develop a rather complex physical and social 
environmental support system. 

Sexual Function 

Sexual functioning is another area that is affected by 
spina] cord injury. While many aspects of sexuality, 
including sexual libido, production of sex hormones, 
feelings of sexual attraction to potential partners, and 
the ability to be sexually attractive to others are 
essentially undisturbed by spinal cord injury, certain 
areas of sexual behavior will be disrupted. As with 
other functions disrupted by a spinal cord injury, 
modifications will be needed in the spinal cord injured 



person's techniques of behaving sexually. Nonetheless, 
the psychic, hormonal, and many of the physical 
aspects of a love relationship are still present and 
available to the spinal cord injured person. 

Physical aspects of the love relationship such as the 
involvement of the senses of vision, smell, and touch 
via oral or manual contact still exist for spinal cord 
injured persons. Areas in which sensation is preserved 
that were previously erogenous to the person will 
remain so. Such areas might, in fact, have heightened 
erotic meaning to the person after his injury. Very 
often, the transition area between normal sensation 
and no sensation acquires a heightened erotic feeling, 
and if the lesion is incomplete, some sensory areas 
below the main level of paralysis may also have 
heightened erotic feeling. Sensation in the genital area 
itself requires an intactness of the second, third, and 
fourth sacral nerves. Thus, almost no spinal cord 
injured person will have direct appreciation of touch or 
contact in these areas. Some of the higher lumbar 
nerves around the upper thigh approach the genital 
area and might convey genital -like sensations. Spinal 
cord injury does alter physical mobility, and therefore 
will alter the "gymnastic" aspects of sexual 
interactions and will necessitate modification of 
technique. 

Genital sexual functions of the male may be broken 
down into erection, seminal emission into the urethra, 
and forceful ejaculation of the semen. Erection is 
controlled by the parasympathetic nerves, emission by 
the sympathetic nerves, and ejaculation by the regular 
motor nerves. The achievement of a full orgasmic 
experience requires a coordination and integration of 
these three nervous system functions. This 
coordination occurs in the spinal cord and is dependent 
upon higher centers. The coordination of these 
nervous systems is thus very much reduced in high 
spinal cord injuries, but becomes more and more 
possible with lower injuries. Therefore, the fertility 
rate for males with lumbar lesions is higher than for 
males with cervical lesions, but in both cases it is well 
under 10 percent. 

Reflex erection is usually possible for all spinal cord 
injured males with lesions at about T12 and above. 
Mechanical stimulation can produce and sustain an 
erection. In some males with lower thoracic and 
upper lumbar level lesions, physically produced weak 
erections can occur. Lumbar lesions, particularly if 
they involve the cauda equina, will interfere with the 
development of a full reflex erection, but may allow for 
a less intense psychic erection which may or may not 
be strong enough to allow for vaginal intromission. 
Since emission depends upon sympathetic nerve 
function, it may appear without intense ejaculation in 
males with lumbar lesions. Ejaculation does not appear 
in males with lesions at any level, although some 
techniques have evolved which, although somewhat 



Spinal Cord Injury 79 



dangerous, have been successful in producing 
ejaculation. Despite the absence of ejaculation, some 
sexually active spinal cord injured males do report an 
orgasmic- like experience associated with a certain 
amount of sexual tension release. 

As with spinal cord injured males, females with 
spinal cord injures may develop sensory areas that 
convey heightened erotic meaning. Lubrication of the 
vaginal area will be inconsistent and mav necessitate the 
use of additional lubrication. While females will 
reflexly exhibit engorgement of the clitoris (equivalent 
to erection in males), they will not exhibit the 
spasmodic contractions of the perineal and vaginal 
musculature (equivalent to ejaculation). The absence 
of the latter orgasmic experience is sometimes 
replaced, as in the male, by an orgasm -like experience 
associated with a release of sexual tension. Female 
fertility is generally undisturbed bv spinal cord injury. 
Immediately after an injury, menstruation may cease 
temporarily, but then return in a regular pattern. 
Thus, spinal cord injured females can conceive and 
carry a fetus to term. A spinal cord injured female who 
chooses to do this needs careful medical attention 
during pregnancy, since there is an increased risk of 
certain complications during pregnancy and delivery. 
For females who do not wish to conceive, birth control 
methods must be considered carefully, as the problems 
associated with some of them may be increased in 
spinal cord injured women. 

It should be obvious that a spinal cord injured 
person can develop a long-term interpersonal 
relationship including love, respect, affection, and a 
mutually satisfying sexual relationship with another 
person. As with able-bodied persons, the development 
of such a relationship requires communication and 
mutual understanding between the partners involved. 
Counseling by informed professionals may be helpful 
to facilitate communication between partners, to help 
them consider exploring alternative sexual behaviors, 
to provide accurate information regarding sexual 
functioning, and to give specific suggestions for 
modifying sexual techniques. 

Post-Rehabilitation Followup 

Regular medical outpatient followup is essential for 
spinal cord injured persons for the remainder of their 
lives in order to ensure that they have a long life-span. 
The purpose of the regular medical followup is to 
maintain good general medical health and, in 
particular, to avoid the complications discussed above, 
especially skin ulceration and kidney disease. 
Maintenance and modification of drugs is also a 
regular part of the followup. A complete evaluation of 
the urological system must be done at least once a year, 
if not more often. Attained rehabilitation goals need to 
be monitored and upgraded and new techniques 



developed for further functional improvement. 
Attention to psychological and social aspects of the 
person's life can anticipate problems or correct those 
that have developed. Furthermore, followup keeps the 
person abreast of the development of new adaptive 
devices that can continually enhance and upgrade his 
vocational potential. 

VOCATIONAL IMPLICATIONS 

Because the effect of spinal cord injury is so 
pervasive, a counselor must look carefully at 
education, interests, and aptitudes, as well as physical 
requirements. 

Education 

Plans for long-term vocational goals requiring 
further education should include consideration of the 
client's general health, his level of functional 
independence, his adjustment to disability, and his 
interest, aptitudes, and intelligence. Since spinal cord 
injury itself in no way affects the cognitive processes, 
the counselor must decide whether to support a 
training or educational program on the basis of factors 
that would be important in vocational planning for any 
individual, handicapped or not. Some of these, such as 
emotional stability and medical conditions, have 
already been discussed. If intellectual and vocational 
testing disclose candidacy for further schooling, and 
other considerations are favorable, every effort should 
be made to encourage the client in this direction, 
because the more education he has, the more 
employable he becomes. However, no rules that would 
apply to all situations can be given. Each case must be 
weighed individually. 

Aptitudes 

Impairments of intellect, learning ability, verbal 
skills, numerical skills, form and space perception, or 
motor coordination, which one would expect 
to encounter in diseases involving the brain, are not 
seen unless the client sustained head injuries in addi- 
tion to the spinal cord injury. 

Factors to be considered regarding a client's ability 
to perform in a given work situation include (a) his 
ability to sit for long periods of time, which in turn 
depends on his skin tolerance, the stability of his 
vertebral column, the fit of his wheelchair, and his 
general strength and endurance; and (b) the 
appropriateness of his equipment. Since quadriplegic 
clients have difficulty using their arms, their ability to 
function at a desk will critically depend upon the 
segmental level of their lesions and the adaptive 
equipment which is available, including splints, special 
handles or holders, tape recording equipment 



80 Donovan 



(dictating equipment), and the like. For example, a 
complete C6 quadriplegic would have difficulty lifting 
a telephone because flexors of the fingers are absent. 
However, if an L- shaped attachment were placed on 
the telephone receiver under which he could slide his 
hand, he could support the attachment between the 
index finger and the thumb and be able to use the 
receiver. Similarly if he had a "dialing stick," held 
with the aid of a wrist -driven flexor hinge splint, he 
would be able to dial the telephone using the muscle 
power remaining at the shoulder. 

Interests 

In addition to factors discussed earlier, the counselor 
should be cognizant of the client's likes and dislikes in 
vocational and avocational areas, his ability to learn 
and perform new tasks, his ability to interact with 
people pleasantly, and his ability to handle stress. 
Ideally, the vocational counselor should have a chance 
to observe the client at a spinal cord injury center 
during a simulated work experience, or at least in 
several occupational therapy sessions, to determine 
how well he functions in carrying out activities which 
are well mastered compared with those just learned or 
still unlearned. 

From his own knowledge of the client acquired 
through testing and interview, as well as first-hand 
information about performance, the counselor will be 
in an ideal position to form plans and construct some 
long- or short-term goals with the client. The 
counselor should also be aware of the fact that interests 
do change. Quite often, for example, a turbulent 
emotional reaction to disability will gradually 
crystallize into acceptance, and new interests in work 
or school will appear. A client may agree to use 
adaptive equipment to permit performance of a work- 
related task although he had previously rejected such 
equipment. If he succeeds in this effort, further interest 
in work experience may follow. Interests incompatible 
with the client's physical function because of the 
paralysis cannot be developed. 

Physical Demands 

In attempting to match a client to the physical 
demands of a job, the counselor must first consider the 
segmental level of the injury and whether the lesion is 
complete or incomplete. Clients with low -level spinal 
cord injuries who require only AFO's may easily be 
able to work in situations that require walking from 
place to place on level ground or negotiating a few 
steps. However, activities that require stooping, 
bending, climbing, or standing for a long time would 
be inadvisable. 

Clients with lesions at higher levels who are able to 
ambulate with KAFO's should generally be considered 



for work that can be carried out from a wheelchair, 
since they will probably not be able to remain standing 
for long periods. Their ability to tolerate the upright 
position will depend upon the stability of the vertebral 
column around the fracture site, the presence or 
absence of deformities in the lower extremities, the 
amount of standing balance, the fit of the braces, and 
the condition of the skin (especially around the feet and 
knees), as well as general strength and endurance. 
Since these factors will vary widely among individuals, 
one should generally plan for the majority of the work 
to be done from the sitting position with standing 
permitted as tolerated. Occasionally, a strap attached 
to the work bench at both sides and placed behind the 
client's buttocks aids standing balance. It should also 
be borne in mind that even though a client with long 
leg braces may be able to walk, he may actually prefer 
not to because of the energy required and the demand 
on the heart and lungs. Further, any walking requires 
crutches and the hands are not free to carry objects. 

Clients with lesion levels from T12 up through T6 
may also be able to walk with long leg braces for short 
distances but will not be able to walk functional 
distances because of cardiovascular demands. 
Therefore, they also must be considered only for 
activities that can be performed primarily from the 
wheelchair. Counselors should be aware of the 
architectural barriers that may exist in many work 
situations and should ascertain the accessibility and 
location of elevators, toilets, cafeteria, and parking lot. 
For males with external urine collection devices and 
females who remain odor free with the use of absorbent 
pads, lack of access to public toilets or toilets in the 
place of employment is not catastrophic, since males 
can empty the collection bags and females can change 
the absorbent pads in any area which offers privacy. 
However, for those individuals who are continent even 
though they may not have control of urination, and 
who therefore are able to get along without catheters or 
collection devices, it is important that at least one toilet 
be wheelchair accessible where they are working or 
attending school. Lacking this, a commode can be 
placed in a room where privacy is afforded so the 
individual can empty his bladder. 

The same considerations apply to clients with lesion 
levels higher than T6, i.e., T6 through C3, who will 
rely solely on a wheelchair. If an attendant or other 
person can provide the C4 quadriplegic with 
transportation, work outside the home certainly is 
possible. All activities must be handled from a 
wheelchair and the amount of activity will be limited. 
Schooling is certainly within the realm of possibility, 
providing a tape recorder is available for preserving 
notes. Since use of the hands is not possible in any 
work- related activity, special adaptive equipment will 
be needed to operate a telephone or electric typewriter 
or any other tool in conjunction with the job. 



Spinal Cord Injury 81 



C4 or C3 quadriplegics can be expected to have 
diminished vital capacity and may require ventilatory 
assistance administered through a mechanical 
respirator mounted on the wheelchair. Clients with 
lesions at C4 and above will not be able to use their 
hands and will require telemetric equipment and 
probably the presence of an attendant in the work or 
school environment. Any work or school situation, 
therefore, must be considered in terms of its wheelchair 
accessibility. 

Clients with lesions below C7 can be considered for 
some outside work if their wheelchairs are equipped 
with air -filled, treaded tires rather than solid rubber 
ones. Paraplegics, in general, can handle a wheelchair 
adequately outdoors, although rough ground, gravel, 
or grass may pose insurmountable problems. Clients 
who ambulate with AFO's and crutches can work even 
longer times outdoors, occasionally even in inclement 
weather. 

Quadriplegic and high paraplegic clients have 
difficulty with body heat regulation, and therefore the 
temperature of the work environment should be 
considered. These clients have difficulty conserving 
heat in a cold climate because the blood vessels of the 
skin do not constrict normally. Similarly, they have 
difficulty losing heat in a hot environment because they 
are unable to perspire normally. 

The circulation and the cleanliness of the air should 
also be considered, since clients with quadriplegia and 
high paraplegia have difficulty with forceful expiration 
and coughing. In a dusty work environment, more 
mucus than normal is secreted in the bronchial 
passages. Because they will have difficulty raising this 
sputum, quadriplegics will be more liable to develop 
respiratory infections if they must work in these 
conditions. 

In most instances, consideration of a sheltered 
workshop will not be necessary except for a client with 
high quadriplegia for whom a job in the competitive 
market commensurate with his physical limitations 
cannot be found. It must be borne in mind that if 
workshop activity is considered, the client should be 
informed and assured in advance that, even though 
many of those about him might be mentally retarded 
or brain damaged, he will not be compared with them 
from an intellectual point of view, he will be 
adequately protected from individuals who may be 
subject to uncontrolled outbursts, and there will be 
adequate supervision and attention to his particular 
needs. Utilization of quadriplegics in some 
administration activities of the sheltered workshop or 
other charitable organizations might prove 
advantageous in some instances. 

BIBLIOGRAPHY 

Fordyce WE: A behavioral perspective on rehabilitation. 
In Albrecht, GL (ed): The Sociology of Physical Disability and 



Rehabilitation. Pittsburgh, University of Pittsburgh Press, 
1976, pp. 73-95. 

The principles of psychologic approaches to the disabled 
patient, including the spinal cord injured patient, are 
explained very clearly and concisely. This is an excellent 
chapter for individuals who have had only limited exposure 
to the rehabilitation of the physically disabled. 

Fordyce WE: Behavioral science and rehabilitation. Rehabil 
Psychol 21:82-85, 1974. 

This reference will provide the reader with an overview of 
psychologic principles and how they can be specifically 
applied to the spinal cord injured. Research horizons in 
psychologic management methods are also explained. 

Glenn, JW, Miller KH: Voice terminal may offer 
opportunities for employment to the disabled. Am J Occup 
Ther 30:309-312, 1976. 

Discussion of the feasibility of employing the severely 
disabled in the information industry. Description of a 
computer terminal that has been developed which is 
operated entirely by voice, obviating the necessity of using 
the hands. 

Goldberg RT, Freed MM: Vocational adjustment, interests, 
work values, and career plans of persons with spinal cord 
injuries. ScandJ Rehabil Med 5:3-11, 1973. 
Twenty-one spinal cord injured patients (ages 18-60) were 
assessed for their vocational development through 
interviews both pre- and postinjury. The interview also 
included diagnosis, severity of disability, age, education, 
level of function, months since injury, and level of 
verbalization. The best predictors of vocational adjustment 
are discussed and ranked. 

Hohmann GW: Psychological aspects of treatment and 
rehabilitation of the spinal cord injured person. Clin 
Orthop 112:81-88, 1975. 

Discusses the stages a spinal cord injured person goes 
through in accepting disability, namely, denial, depression, 
withdrawal and internalized hostilitv, externalized hostility, 
and reaction against dependence. Also discusses pain, 
sexual function, family, and the importance of using Spinal 
Cord Injury Centers for treatment. 

Long C II: Congenital and traumatic lesions of the spinal cord. 
In Krusen FH, Kottke FJ, Ellwood PM (eds): Handbook of 
Physical Medicine and Rehabihtation.Ed 2, Philadelphia, 
Saunders, 1971, pp. 566-578. 

An excellent introduction to the medical problems encoun- 
tered by spina] cord injured patients. It deals further with 
treatment methods discussed in this text and with functional 
expectations. Problems faced by patients with congenital 
spinal cord dysfunction are also discussed. 

Long C II, Lawton EB: Functional significance of spinal cord 
lesion level. Arch Phys Med Rehabil 36:249-255, 1955. 
This reference describes in further detail the functional 
capabilities of spinal cord injured patients and the muscles 
needed to perform various tasks. It is a bit outdated as far as 
the functional performance of quadriplegics is concerned, 
but principles discussed and therapeutic goals given for 
paraplegics are still valid. 

Miller DK, Wolfe M, Spiegel MH: Therapeutic groups for 
patients with spinal cord injuries. Arch Phvs Med Rehabil 
56:130-135, 1975. 

Spinal cord injured patients were given the option of attend- 
ing a short-term therapeutic group designed to help them 
adjust to their disability by receiving information and dis- 



82 Donovan 

cussing experiences and feelings. Knowledge and attitude 
before and after the group experience were measured and 
compared with those of non-group members. The value of 
group therapv as an effective means of disseminating infor- 
mation and aiding patients' adjustment is assessed. 

Romano MD, Lassiter RE: Sexual counseling with the 
spinal cord injured. Arch Phys Med Rehabil 53:568-572, 
1972. 

Description of a sex education and counseling program for 
both male and female spinal cord injured patients. Both 
sensory and motor changes as a result of injury, common 
problems and concerns, and some helpful adaptations are 
discussed. 

Symington DC, Boyd E. Vasa J: Rotating table for the high 
level quadriplegic. Arch Phvs Med Rehabil 55:481-483, 
1974. 

Description of a table with a rotating top on which several 
pieces of eqiupment can be mounted. A high-level 
quadriplegic can use a mouth wand to rotate the table and 
operate the equipment independently. 

Symington DC, McKay DW: A study of functional 
independence in the quadriplegic patient. Arch Phys Med 
Rehabil 47:378-392, 1966. 

This is a more up-to-date representation of the 
performance capabilities of quadriplegics than is provided 
in the article by Long and Lawson cited above. It is well 
written and the tables are quite useful. 

Talbot HS: Management of neurogenic dysfunction of the 
bladder and bowel. In Krusen FH. Kottke FJ, Ellwood 
PM (edsj: Handbook oj Physical Medicine and Rehabilitation. 
Ed 2, Philadelphia, Saunders, 1971, pp. 634-642. 
Problems of bowel and bladder management in the spinal 
cord injured patient and how to overcome them are 
discussed. This chapter will give the reader a clear 
understanding of how elimination techniques are 
performed and what and how equipment is used to make 
these tasks easier or possible. The physiology of 
micturition is also discussed. 



6 NEUROMUSCULAR DISEASES 



Paul J. Corcoran, M.D. 



INTRODUCTION 

Included in this chapter is discussion of a group of 
diseases that affect the motor system at one of several 
points (fig. 6-1). The reader should review the basic 
anatomy and physiology of the nervous system in chapter 
3 for a better understanding of these disease mech- 
anisms. This somewhat arbitrary grouping includes the 
following neuromuscular diseases: 

1. Huntington's disease 

2. Parkinson's disease 

3. Spinocerebellar degeneration 

4. Friedreich's ataxia 

5. Amyotrophic lateral sclerosis 

6. Spinal muscular atrophv 

7. Poliomyelitis 

8. Guillain-Barre syndrome 

9. Myasthenia gravis 
10. Muscular dystrophy 

The impairments produced by this group of 
disorders are limited mainly to the motor system, 
causing weakness or clumsiness on voluntary motion, 
as well as troublesome involuntary movements in some 
cases. Except perhaps in the later stages of the first five 
diseases discussed, communication is generally 
unaffected. In all the diseases, sensation is generally 
not affected and pain is not a major problem. With the 
exception of poliomyelitis and Guillain-Barre 
syndrome, these are progressive diseases whose causes 
are unknown. 

Six other neurological diseases that affect movement 
are of sufficient medical complexity and vocational 
importance that they are treated in separate chapters: 
spinal cord injury (chap. 5), peripheral neuropathy 
(chap. 7), multiple sclerosis (chap. 8), stroke and 
cerebral trauma (chaps. 9 and 10), cerebral palsy 
(chap. 11), and epilepsy (chap. 12). These six disorders 
produce a variety of other neurological limitations in 
addition to the motor system impairments described in 
this chapter. There are, however, a number of areas of 
similarity or overlap. 

It should be kept in mind that many persons with 
neuromuscular diseases do not fit neatly into any of the 
classical disease categories to be described in this 
chapter. Physicians often use the suffix "-opathy" to 



help describe, as best they can, those diseases which 
cannot be more precisely labeled. Thus the 
rehabilitation counselor will meet expressions such as 
"myopathy associated with neoplasm" or 
"encephalomyelopathy related to vasculitis." An 
understanding of the classical patterns, however, will 
provide a framework for dealing with those clients who 
fall into these "gray areas." 

Specific evaluation and treatment measures are 
discussed under each disease category and a general 
philosophy of management is presented in the 
summary at the end of the chapter. 



HUNTINGTON'S DISEASE 

Disease Description 

Huntington's disease is also called Huntington's 
chorea or chronic progressive chorea. It is a hereditary 
disease of the brain with onset usually in adult life, 
characterized by jerky involuntary movements 
(chorea) and mental deterioration. Most cases begin 
between ages 30 and 50, although occasional cases 
have begun in childhood or old age. It is quite rare in 
the general population (3 to 6 cases per 100,000 
population), but it may be more common in certain 
communities where families affected with the disease 
happen to live. It persists in these communities because 
of its strong hereditary tendencies and the fact that it 
does not usually become severely disabling until after 
child-bearing age. For example, over 1 ,000 cases have 
been traced to 2 brothers who emigrated to Long 
Island from England about 200 years ago. 

The direct cause is unknown. Autopsies show 
widespread degeneration throughout the brain, with 
shrinkage of the brain tissue and enlargement of the 
fluid -filled cavities (ventricles) inside the brain. Severe 
degenerative changes are found in the basal ganglia, 
the deeply lying relay stations for nerve tracts that 
control movement (see p. 21). 

The first clinical signs of the disease are usually the 
jerky involuntary "choreiform" movements. These 
abrupt, sudden, jerky movements can occur in any of 
the muscles of the trunk or limbs, as well as the head 



83 



84 Corcoran 




BRAIN 
Motor cortex 
Betz cell 

Upper motor neuron 
Basal ganglia 
Brain stem 

Cerebellum 



SPINAL CORD 
(cross section) 



Lateral column 
Posterior column 
Anterior horn 

Anterior horn cell 



PERIPHERAL NERVE 

Lower motor neuron 

Motor end plate 
(neuromuscular junction) 

Muscle 



FIGURE 6-1. Major components of the motor system. Numbers 
refer to the sites of pathology associated with the 10 diseases 
covered in this chapter: 1. Huntington's disease; 2. Parkinson's 
disease; 3. Spinocerebellar degeneration; 4. Friedreich's ataxia; 
5. Amyotrophic lateral sclerosis; 6. Spinal muscular atrophy; 7. 
Poliomyelitis; 8. Guillain-Barre svndrome; 9. Myasthenia gravis; 
10. Muscular dystrophy. 



ation, usually begins later but sometimes appears 
years before the involuntary movements start. 
There is progressive impairment of memory and 
intellectual functioning, impulsive behavior, and 
personality changes with neglect of business affairs and 
personal hygiene. Fits of violence sometimes occur, 
and suicide is much more common than in the general 
public or in unaffected siblings. This suggests that 



and face muscles. They are increased in intensity by 
emotional stress or concentration on performance of 
physical tasks; they diminish when the person is sitting 
quietly, and disappear during sleep. In the early stages 
of the disease, the patients are often able to mask the 
involuntary movements as mannerisms or "the 
fidgets." 

The other major manifestation, mental deterior- 



Neuromuscular Diseases 85 



suicide is due to the mental deterioration and 
impulsive behavior rather than to a fear of the disease, 
as was once suggested. A small percentage of patients 
will develop seizures. 

The disease usually follows a progressive downhill 
course. The duration between onset and death 
averages 15 years but may be considerably shorter or 
longer. Death seldom results from the disease directly, 
but is more commonly caused by injuries, infections, 
overindulgence in alcohol, suicide, or other results of 
the mental deterioration. 

Functional Disabilities 

Highly skilled functions become difficult after 
several years, and help is needed in dressing and self- 
care activities. Ambulation and transfers are usually 
retained until the late stages of the disease. There is no 
true paralysis, but the involuntary movements plus the 
mental deterioration impair physical performance. 
The ability to go out alone and use public 
transportation is eventually lost. The speech becomes 
thick and hard to understand, but hearing and vision 
are retained intact. The social consequences of the 
bizarre involuntary movements and organic mental 
deterioration result in greater and greater dependence 
on family members. Lacking a social support system, 
patients are commonly institutionalized in the later 
stages of the disease. 

Rehabilitation Potential 

Short-term rehabilitation potential may be good, in 
view of the slow progression of the disease. Skillful 
redirection of the vocational path may allow continued 
productivity within the limitations that develop. 
However, the counselor may assume that the disease 
will get steadily worse with time. Remissions or 
improvement are not a part of the syndrome. 



Standards of Evaluation 

A neurologist should see patients, and can usually 
make the diagnosis without much difficulty from the 
family history and the characteristic involuntary 
movements. Extensive neurological tests are usually 
not necessary. A psychologist should assess the 
presence or degree of organic mental deterioration. A 
social worker can evaluate the family and social 
support system. An occupational therapist is helpful to 
assess whether the performance of activities of daily 
living could be facilitated by training of the patient or 
family members. 

Total Treatment 

There is no known treatment that will halt or slow 



down the steady progression of Huntington's disease. 
Certain tranquilizing drugs sometimes decrease the 
severity of the involuntary movements. Genetic 
counseling of the family is of great importance to allow 
the patient and any as -yet -unaffected children or 
other relatives to make an informed decision about the 
wisdom of having children. An occupational therapist 
can help the patient or family with techniques to 
simplify self- care activities. Counseling support and 
social services are usually needed by the families. 
Psychiatric intervention directly with the patient is not 
usually of much value in organic mental syndromes, 
except perhaps to recommend drugs that might help 
control antisocial behavior. 

Vocational Implications 

Short-term training or education may be 
appropriate in the early stages, where evidence of 
organic mental deterioration is absent. It should be 
remembered that many years may go by before the 
client becomes incapacitated or unable to continue 
work. Clients can be expected to gradually lose their 
aptitude for intellectual work or fine manual dexterity. 
The client's interests should probably be encouraged in 
the direction of routine, structured tasks without 
excessive emotional stress or job tension. Physical 
demands may be medium to heavy, but jobs involving 
climbing, balancing, or handling dangerous materials 
should be avoided. There is no particular reason to 
avoid extremes of temperature, humidity, noise, or air 
pollution. Clients with more advanced stages of the 
disease may have obvious limitations in verbal skills, 
learning ability, memory, or dexterity. Sheltered 
employment may be the highest practical goal at this 
stage. 



PARKINSON'S DISEASE 
Disease Description 

Parkinson's disease ("paralysis agitans," "par- 
kinsonism," or "shaking palsy") is a progressive 
disease of older adults characterized by muscle rigidity, 
slowness of movements, and a unique type of tremor. 
There is no actual paralysis, and the word "paralysis" 
or "palsy" in the names of the disease are misnomers. 
The usual age of onset is 50 to 70; the disease is rare 
prior to age 40. It is a relatively common disease, 
having a prevalence of about 187 cases per 100,000 
population. It is estimated that 1 in 40 persons alive 
today will contract the disease. Since it affects an older 
age group, its prevalence increases as the population 
ages. There is a slight familial tendency. 

Pathological findings in advanced cases include 
extensive degenerative changes in the basal ganglia. 
Degenerative changes are also found in the cerebral 



86 Corcoran 



cortex and often in the brain stem. 

The cause is unknown. Many cases in the past 
occurred in persons who survived the encephalitis 
epidemic of 1918 through 1923. This type of 
"encephalitis lethargica" is rare today, and is not 
likely to be a cause of current cases. Arteriosclerotic 
changes in the cerebral blood vessels are related to the 
disease, but the two conditions do not always coexist. 
Occasional cases follow poisoning with carbon 
monoxide, heavy metals such as manganese, or 
overdoses of certain tranquilizers and drugs. There is 
some experimental evidence of a deficiency in the 
neurotransmitter chemical dopamine in affected areas 
of the brain, a finding which led to the successful trial 
of the drug levodopa in treatment of Parkinson's 
disease. 

The most prominent and disabling physical sign of 
Parkinson's disease is muscle rigidity. On passive 
movements, the muscles resist elongation, alternately 
yielding and tightening up to give a "cogwheel" 
sensation. Voluntary movements are impeded by the 
muscle rigidity, which leads to slowness of movement 
and greater effort, causing fatigue. Even the abilities to 
speak and write are impaired by muscle rigidity. The 
voice volume becomes low and handwriting becomes 
much smaller than normal. 

A second group of physical signs is related to lack 
of spontaneous movements or difficulty initiating 
movements. Patients sit very still in their chairs, with 
few spontaneous changes in position or movements of 
the trunk or limbs. They blink less frequently and tend 
to have a mask- like, expressionless face. Intense 
concentration may be required to perform ordinary 
voluntary activities such as standing up or beginning to 
walk. 

The tremor is the obvous sign that gave rise to the 
common names for Parkinson's disease, but is not as 
disabling as the rigidity and diminished movements. 
There is a characteristic to -and -fro tremor at a 
frequency of about three or four per second, often 
affecting only one extremity and later spreading to 
other extremities or the opposite side. The tremor can 
be suppressed for several seconds by concentration and 
voluntary effort, so that performance of fine activities 
of brief duration remains possible. There is no true 
paralysis, although some disuse atrophy results from 
the impeded movements. Sensation, vision, and 
hearing are unaffected. The speech may become faint 
and hard to understand due to low volume and stiffness 
of the speech muscles. There is no pain directly caused 
by the disease, but patients may complain of muscle 
aching and stiffness secondary to the rigidity. 

The complications of the late stages of Parkinson's 
disease are complications of inactivity. The disease 
itself does not cause death, but patients may become 
immobilized, develop contractures, and become 
bedridden. Flexion contractures of the trunk and limbs 



are a special hazard in patients with Parkinson's 
disease who do not exercise properly. Other 
complications are related to poor nutrition caused by 
the patient's difficulty feeding himself, or slowness of 
chewing and swallowing if being fed by another 
person. Institutionalized patients with Parkinson's 
disease may literally starve to death if overworked staff 
members do not have time to sit long enough to feed 
them. 

The disease generally follows a rather slow course, 
with an interval of 10 to 20 years between the onset and 
severe disability. Remissions or spontaneous 
improvement do not occur. The progression may be 
temporarily slowed by surgical procedures or the new 
drug levodopa, but it is too soon to know whether the 
overall course of the disease will be affected. 

Functional Disabilities 

In the early stages of the disease, most functions can 
be performed independently. After several years, gait 
becomes slow and frequent falls may occur. Patients 
tend to stand and walk with a characteristic flexed, 
stooped posture. The ability to initiate transfer 
activities may be severely impaired. The muscle 
stiffness causes fatigue after minor exertion and limits 
work tolerance. The jerking and swaying of public 
transportation vehicles may make them unsafe for 
patients with poor balance or difficulty making sudden 
movements. Because each physical act requires 
concentration, patients find difficulty performing more 
than one physical task at a time. Self- care activities 
such as dressing and grooming may require a 
prolonged time, making it hard for some patients to 
arrive at work on time in the morning. Chewing and 
swallowing may be quite slow, requiring prolonged 
lunch hours. There is an increased rate of saliva 
formation which, together with the swallowing 
difficulty and the tendency to hold the head forward, 
may lead to drooling of saliva. Communication may be 
difficult due to the slowness of speech and low volume. 
The slow writing with tiny letters may cause 
handwriting to be hard to read. 

Emotional changes, chieflv depression, commonly 
occur as one might expect with a major physical impair- 
ment. Occasionally there is mental deterioration or 
confusion, or personality changes with decreased drive 
and impaired judgment. Patients who become more and 
more dependent on others for self-care create stresses on 
their social support sytems, which may break down, 
leading to institutionalization in the later stage of the 
disease. 

Rehabilitation Potential 

Because of the gradual onset and slow progression of 
the disease, persons with Parkinson's disease have a 



Neuromuscular Diseases 87 



good potential for rehabilitation. The counselor's 
expectation should be for a slow but steady progression 
of symptoms and deficits and he should plan in 
anticipation of further involvement. The future rate of 
progression is likely to be predicted by the past rate of 
progression. 

Standards of Evolution 

The diagnosis can be made by any physician from 
the characteristic history and physical exam. There are 
no specific diagnostic tests that are helpful in 
Parkinson's disease. A neurologist can recommend the 
most appropriate medical treatment. A physiatrist may 
be useful to evaluate functional deficits such as gait and 
self- care, to assist the rehabilitation counselor with 
vocational recommendations, and to prescribe and 
oversee the physical management of the patient. A 
physical therapist can be helpful in evaluating 
limitations of gait and range of motion. An 
occupational therapist can assess self- care limitations. 
A psychologist may be helpful to assess the presence 
and degree of organic mental deficit or personality 
change. A social worker can provide valuable 
information about the family and support system. 

Total Treatment 

Optimal treatment of Parkinson's disease requires 
manipulation of several drugs and is ordinarily best 
done by a neurologist who should monitor the patient 
at intervals of no more than 3 to 6 months. The drugs 
levodopa and carbidopa are the mainstay of treatment 
at the present time. It may take several months to 
achieve the optimum therapeutic effect and to regulate 
the dosage. The symptoms of rigidity and decreased 
movements are benefited the most, while the tremor 
may persist. Many patients experience troublesome 
side effects, the commonest being nausea and 
vomiting, loss of appetite, a drop of blood pressure on 
standing up (postural hypotension), and development 
of involuntary movements of the limbs. These side 
effects usually are controlled by lowering the dosage 
and rarely require that the drugs be discontinued. 

Another useful group of drugs, widely used prior to 
the introduction of levodopa and carbidopa, is the 
belladonna family of drugs. Their therapeutic effect is 
less powerful, and they too cause a number of side 
effects, such as hot flashes, dry mouth, blurred vision, 
and dizziness. A combination of a belladonna- type 
drug and levodopa in reduced dosage is effective for 
many patients. Antidepressant drugs may also be 
useful in despondent patients with a low energy level. 

Surgical intervention was commonly practiced in the 
past, and included various neurosurgical procedures to 
destroy small areas within the basal ganglia to 
interrupt the pathways associated with the rigidity and 



tremor. These have been largely rendered obsolete by the 
success of levodopa. 

The physical management of the patient is no less 
important than the pharmacologic management. A phvs- 
ical therapist should see the patient at regular intervals to 
supervise a daily routine of home exercises. These 
exercises are designed to maintain the range of motion in 
the joints and prevent contractures; to prevent deteriora- 
tion of the muscle strength and gait; and to encourage 
normal speaking, writing, and deep breathing. Exercises 
do not alter the basic course of the disease, but they do 
maintain function and prevent the unnecessary addi- 
tional complications of immobility. If contractures occur, 
particularly at the ankle, surgical correction is sometimes 
helpful in maintaining ambulation. 

An occupational therapist can assist patients and 
family members to use simplified energy -saving 
methods of performing daily activities or employment 
activities, and can also suggest modifications for home 
and work environments. Short-term individual or 
group psychotherapy may be helpful, and psychoactive 
drugs may be needed from time to time. A social 
worker can help support the family and arrange for 
specific services as they are needed. 

Vocational Implications 

White collar jobs may be continued until the disease 
is relatively far advanced unless a large amount of 
speaking is required. Transportation to and from the 
office may be the greatest obstacle. Jobs requiring 
short -duration manual dexterity may also be 
continued. Heavy physical labor with a large amount 
of walking, carrying, and climbing may have to be 
changed earlier in the course of the disease. Since 
mental functioning is not usually affected, it may be 
feasible to retrain a physical worker to do a less 
strenuous and perhaps more skilled occupation. The 
major obstacles to learning are related to the age at 
which the disease usually appears, rather than directly 
due to the disease itself. 

It should be remembered that the client will have 
diminished aptitudes for prolonged fine rapid 
movements of the hands or feet and is likely to develop 
diminished speaking abilities eventually. 

Most interests may be encouraged. Interpersonal 
contacts of a sophisticated nature, such as those of a 
salesman, should perhaps not be promoted, since the 
low voice, mask -like face, and occasional drooling 
may interfere with this. Highly stressful occupations 
should probably be avoided, since emotional stress 
often increases the severity of the tremor. 

The physical demands of an occupation may become 
limited to sedentary or light work levels. The slow 
movements and gait difficulty make it undesirable to 
have jobs involving climbing, kneeling, or working in 
dangerous environments. The muscle rigidity causes 



Corcoran 



fatigue after moderate exertion, but frequent short 
rests will increase the total amount of work that can be 
done in a day. However, prolonged sitting in one 
position is to be avoided because it increases the 
stiffness and may promote contracture. 

Certain environmental factors should be avoided. 
Cold working environments increase the stiffness of 
persons with Parkinson's disease. Heavy metal 
pollution in the air should be avoided, since this may 
be a contributing factor in the disease. Similarly, areas 
of concentrated vehicular traffic should be avoided 
because of the relationship between carbon monoxide 
poisoning and Parkinson's disease. Those clients who 
are troubled by excessive sweating should avoid 
working in a hot or humid environment. 

SPINOCEREBELLAR DEGENERATION 

Disease Description 

The spinocerebellar degenerations are a family of 
hereditary diseases that affect, to varying degrees, the 
cerebellum, the brain stem, and the long tracts of the 
spinal cord. Those that affect predominantly the 
cerebellum and brain stem tend to begin in late middle 
life, progress more slowly, and have less of a familial 
tendency. The commonest are olivopontocerebellar 
degeneration and cerebellar atrophy. Certain other diseases 
in this family tend to affect the spinal cord to a greater 
degree than the cerebellum. These have an earlier age 
of onset (teenagers or young adults), a more rapid rate 
of progression, and a strong familial tendency. 
Common examples are Marie's ataxia, Roussy-Levy 
syndrome, and Friedreich 's ataxia. The latter has sufficient 
individual characteristics and vocational implications 
to be considered separately (see p. 89). 

The spinocerebellar degenerations are relatively 
uncommon. Degenerative changes and shrinkage 
involving the cerebellum, brain stem, and certain 
tracts of the spinal cord occur. Damage to these areas is 
associated with clumsiness and poor coordination. The 
spinal cord tracts most commonly affected are the 
posterior columns, which carry position and 
movement sensation up to the parietal lobe, and the 
lateral corticospinal (pyramidal) tracts, which carry the 
signals for voluntary movement from the frontal lobe 
(see p. 21). 

The cause of the spinocerebellar degenerations is 
unknown. Symptoms usually include unsteady gait, 
clumsiness of the hands, thick slurred speech 
(dysarthria), weakness and easy fatigability of the 
limbs, and sometimes a tremor of the head or limbs. 
The tremor is usually brought on or increased by 
voluntary effort (intention tremor), and is usually 
absent when the individual is at rest. There is steady 
progression at a variable rate, but 10 to 30 years are 
usually required before the patient is severely 



incapacitated. Remissions or spontaneous improve- 
ment are rare. 

Functional Disabilities 

The ability to walk may be progressively impaired 
due to clumsiness and unsteadiness with frequent falls. 
After several years, patients may begin to use canes or 
crutches. In later stages, they use walkers or hold on to 
the walls or furniture. A wheelchair may be required 
for safe mobility in late stages. 

Self- care and other hand functions become slow, 
laborious, and clumsy and may eventually require the 
assistance of another person. Speech may become 
difficult or impossible to understand, especially to 
those not acquainted with the patient. Handwriting 
also becomes less legible with increasing hand 
clumsiness. Vision and hearing are generally 
unaffected. 

There are no specific mental or psychological 
changes. A certain amount of anxiety or depression 
may be seen, usually related to the extent of 
interference with ordinary activities and functional 
abilities. Intelligence and personality are unaffected. 
Family relationships may undergo strain as the patient 
becomes more dependent on others. 



Rehabilitation Potential 

These diseases tend to begin during the productive 
years of employment and have good rehabilitation 
potential because of their slow progression. They can 
be expected to worsen steadily over time, however, and 
the counselor should be aware of the deficits to be 
anticipated. 



Standards of Evaluation 

The diagnosis of spinocerebellar degeneration 
requires a skilled neurologist. It is important to rule 
out other diseases, such as tumors of the cerebellum, 
which may respond to appropriate treatment. The 
diagnosis is made from the characteristic history and 
physical findings, but more extensive neurological and 
laboratory tests may be required to rule out other, 
more easily treatable diseases. 

A physiatrist should evaluate the patient to assess 
residual abilities and complications of inactivity, and to 
prescribe and supervise a physical restoration or 
maintenance program. A physical therapist can help 
evaluate gait, muscle strength, and use of ambulation 
skills, hand function, and the need for adaptive 
equipment. A social worker is important to assess the 
need for family counseling or outside agency 
intervention to maintain satisfactory social function of 
the family unit. 



Neuromuscular Diseases 89 



Total Treatment 

There is no known medical or surgical treatment 
that has any effect on the course of spinocerebellar 
degeneration. A supportive family physician or 
physiatrist may be very helpful. Physical and 
occupational therapy will be needed from time to time 
to maintain function at the optimum level, or to train 
patients in the use of adaptive equipment that may 
become needed. The patient should have access to a 
social worker when specific needs for support arise. A 
genetic counselor may be helpful to inform the family 
about the risks of occurrence in the children of affected 
families. 



Vocational Implications 

Spinocerebellar degenerations do not affect 
intelligence, making these clients good candidates for 
appropriate retraining or education. Aptitudes for 
verbal skills and manual dexterity may be expected to 
deteriorate as the disease progresses. A variety of 
different interests are compatible with these diseases. 
Physical demands for medium to heavy work may be 
tolerated by most clients, as long as coordination is not 
essential. Jobs involving prolonged walking, climbing, 
balancing, and crawling should be avoided, as these 
skills may be expected to deteriorate. Similarly, jobs 
involving sophisticated verbal communication or fine 
hand function may not be appropriate. There are no 
particular environmental factors that need to be 
avoided. 



FRIEDREICH'S ATAXIA 

Disease Description 

Friedreich's ataxia is a hereditary disease of 
unknown cause, usually beginnning in children or 
teenagers with signs of spinal cord and cerebellar 
degeneration. The average age of onset is 13, with a 
range from 5 to 25 years of age. There is steady 
deterioration, and many patients are severely 
incapacitated by the time they reach their middle 
twenties. Although the condition is relatively rare, it 
has important vocational implications because of the 
age group affected. With improved management and 
rehabilitation measures, patients are living longer than 
was previously supposed possible. 

Friedreich's ataxia probably belongs in the family of 
spinocerebellar degenerations. The first signs are 
usually clumsiness and incoordination (ataxia), 
affecting the lower extremities first with unsteady gait 
and frequent falls. There is easy fatigability, and the 
muscles of the limbs may atrophy. A high arch may 
develop in the feet due to muscle imbalance. Hand 
clumsiness and thick, slurred speech (dysarthria) 



develop. Muscle imbalance in the trunk may lead to 
curvature of the spine (scoliosis). 

Some patients with Friedreich's ataxia develop 
progressive heart failure due to degenerative changes 
and scar tissue formation in the heart muscle. About 10 
percent of patients also have diabetes mellitus. 
Occasionally there are disturbances of respiration and 
difficulties in swallowing. These medical complica- 
tions or the affects of inactivity and bed rest are the 
causes of eventual death. The disease itself does not 
ordinarily cause death directly. 

Functional Disabilities 

Ambulation becomes progressively more unsafe and 
laborious. The use of crutches helps for a while, but a 
wheelchair is generally needed for ambulation within 5 
to 10 years after onset. Self- care skills become 
progressively more difficult as hand clumsiness 
increases. The speech may become slurred and hard to 
understand. Psychological and social impairments as 
already discussed above for the other diseases are 
commonly seen. 

Rehabilitation Potential 

Short-term rehabilitation potential is good. Long- 
term potential is hard to predict because of the steady 
progression. The past rate of deterioration is the best 
predictor of the future course. Intelligence may be 
assumed to remain constant. With proper manage- 
ment, many patients can be expected to have 10 to 20 
years of productivity ahead of them. 

Standards of Evaluation 

The diagnosis should be made by a neurologist who 
is skilled in differentiating other, treatable diseases 
which may present similar symptoms. A careful 
medical evaluation is also appropriate to detect 
associated heart disease or diabetes. A physiatrist can 
evaluate residual function and prescribe and oversee a 
program to correct unnecessary additional disabilities 
and maintain function at the optimum level. A 
physical therapist should evaluate muscle strength, 
joint range, and use of ambulation aids. An 
occupational therapist should evaluate self- care skills 
and adaptive equipment. Where pyschological or 
social impairments are prominent, evaluation by a 
psychologist or social worker is important. 

Total Treatment 

There is no specific medical or surgical treatment 
that is effective in altering the course of Friedreich's 
ataxia. Associated medical problems listed above may 
require appropriate treatment. A physiatrist can 



90 Corcoran 



manage the restorative treatment, and physical and 
occupational therapy are important to maintain mobility 
and hand function. A social worker is usually a 
necessary member of the treatment team. 

Vocational Implications 

Intelligence and learning ability are unimpaired, but 
lengthy educational plans should be considered in light 
of the probable rate of progression. Aptitudes for fine 
hand skills, motor coordination, and verbal activities 
may be assumed to deteriorate steadily. A variety of 
interests is compatible with the limitations of the 
disease. Unlike the predominantly cerebellar members 
of this disease family, in Friedreich's ataxia muscle 
strength and exercise tolerance may diminish with 
time, limiting clients to light physical demands. 
Occupations demanding good balance or dangerous 
surroundings are probably inadvisable. The ordinary 
range of temperature, humidity, and air quality should 
be well tolerated. 



AMYOTROPHIC LATERAL SCLEROSIS 
Disease Description 

Amyotrophic lateral sclerosis (ALS) is a progressive 
disease of adults causing degeneration of motor nerve 
cells and their axons, leading to replacement of 
corticospinal tracts by scar tissue (sclerosis). The 
disease attacks both the lower motor neuron — the 
anterior horn cell of the spinal cord and the motor cells 
of the brain stem and cranial nerve nuclei — and the 
upper motor neuron — -the Betz cells of the precentral 
gyrus. For this reason the British call the disease 
"motor neuron disease." The corticospinal 
(pyramidal) tract runs down the lateral columns of the 
spinal cord, where its damage causes the so-called 
"lateral sclerosis" seen in cross sections of the cord. 

The cause of the disease is unknown. Occasionally 
there is a familial tendency, but most cases are 
sporadic. The prevalance is about 5 cases per 100,000 
population. The usual age of onset is between 30 and 
60, and males are affected three times as often as 
females. 

Initial symptoms and signs depend on whether 
upper or lower motor neurons are involved first and 
where the damage first strikes. Lower motor neuron 
involvement leads to weakness and atrophy, frequently 
presenting in one upper or lower extremity and later 
spreading to the other limbs or the facial and tongue 
musculature. If upper motor neuron damage 
predominates, there is spasticity and exaggeration of 
the tendon reflexes. Eventually, the spasticity 
disappears as the lower motor neurons are lost and the 
muscles undergo denervation atrophy. Difficulty 
speaking and swallowing commonly result from 
damage to the motor nuclei in the brain stem which 



control the tongue, face, and throat muscles. Affected 
muscles exhibit a constant fine twitching (fasiculations) 
due to spontaneous contraction of individual motor 
units. Sensation is unaffected by the disease and 
vision, hearing, and intellectual functioning remain 
normal. There is usually no disturbance of bowel or 
bladder function. 

The disease pursues a steady, fairly rapid downhill 
course. The average duration from onset to death is 
about 4 years, with a range of 1 to 10 years. Death 
usually results from the complications of inactivity or 
from paralysis of the muscles of respiration. 

Functional Disabilities 

Functional disabilities depend on which muscles are 
affected at a given point in the disease. The pattern of 
involvement tends to be randomly scattered. Lower 
extremity involvement may cause weakness and easy 
fatigability, leading to the need for ambulation aids 
and eventually a wheelchair for mobility. Transfer 
activities become increasingly difficult. Upper 
extremity involvement leads to weakness of the arms 
and hands, but fine hand coordination for light 
activities remains intact until the late stages. The 
patients may need increasing help with self- care 
activities. Communication skills are unaffected except 
for speech, which may become somewhat thick and low 
in volume if the speech or breathing muscles are 
affected. There are the expected psychological and 
social problems resulting from increasing dependency, 
but there are no specific intellectual or personality 
changes that can be attributed directly to the disease. 

Rehabilitation Potential 

Long-term potential is very poor due to the steady 
progression of the disease. It is uncommon for patients 
to survive more than 6 to 8 years. Short-term 
rehabilitation potential may be considerably more 
favorable if the patient can be redirected into an 
occupation that is consistent with his functional 
limitations and a minimum of time is required for the 
training. 

Standards of Evaluation 

A neurologist should make the diagnosis and rule 
out treatable diseases that may resemble ALS. 
Electromyography and nerve conduction studies are 
very helpful in establishing the diagnosis. 
Myelography and other neurological tests may be 
necessary to distinguish ALS from other directly 
treatable diseases causing similar symptoms. 

A physiatrist can evaluate residual abilities, 
correctable complications of inactivity, and the need 
for therapy or assistive equipment. A physical therapist 
can assess muscle strength and joint range of motion, 



Neuromuscular Diseases 91 



and an occupational therapist can assess hand skills 
and self- care abilities. An assessment of the social 
situation is important, since the patient will become 
increasingly dependent on others for mobility and self- 
care. 

Total Treatment 

There is no medical or surgical treatment that has 
any effect on the course of ALS. A physical therapist 
should monitor the patient at regular intervals to detect 
contracture formation and to supervise a daily home 
range - of- motion exercise routine. The patient may 
also need training in the use of orthotics, ambulation 
aids, or wheelchairs as time goes on. In the later stages, 
as arm strength and endurance wanes, an electric 
wheelchair may substantially improve independent 
mobility, and mobile arm supports can assist upper 
extremity function (see p. 78). 

An occupational therapist can train the patient or his 
family in the use of self-help equipment and energy - 
saving techniques for activities of daily living. Some 
patients require psychological counseling, and most 
patients and their families need the suppo-t of a social 
worker to adapt to the increasing physical deficits. 



and flaccidity of the trunk and limb muscles may 
develop during infancy or even be present at birth, 
causing the so-called "floppy infant" picture. The 
disease is relatively rare, and was originally described 
under several different names, which are now felt to be 
variations of the same disease. When the onset is at 
birth or in the first year of life, it may be called 
Werdnig- Hoffman disease or infantile muscular atrophy. 
If the onset is between ages 1 and 3, it is sometimes 
referred to as Kugelberg-Welander disease or juvenile 
muscular atrophy. 

Infants with the disease usually lie quietly with little 
spontaneous movement. They are slow to develop the 
mobility milestones of rolling over, sitting up, and 
walking. These skills, once present, may be lost if the 
disease appears in the second or third year, leading to 
progressive weakness and increasing limitations of 
muscular function. Wheelchair use becomes more 
difficult as trunk control is progressively lost. Many 
children die within the first decade of life, usually of 
pulmonary complications or other results of inactivity. 
A few affected children, however, progress more slowly 
and may survive into the second or even third decade 
of life and come to the attention of the rehabilitation 
community. 



Vocational Implications 

Short-term retraining into more appropriate 
occupations is often feasible, since intelligence and 
communication skills are unaffected by the disease. 
Adaptation at the current place of employment may be 
more feasible. Even in the early stages of the disease, 
however, no more than 6 to 12 months of retraining 
will ordinarily be appropriate, due to the short life 
expectancy. The clients may retain their aptitudes for 
fine hand and finger dexterity with lightweight objects, 
since sensation and coordination are unaffected. 
However, grip strength and lifting abilities may be 
expected to deteriorate steadily. A variety of interests 
may be encouraged, as long as these physical 
limitations are understood. Physical demands of any 
future occupation should be light and sedentary, 
without the requirement for much, if any, lifting, 
carrying, pulling, or pushing. Even in an ambulatory 
client, a wheelchair- accessible occupation should be 
selected, since the ability to walk will be lost within a 
few years. Ordinary environmental extremes are 
tolerated normally, except in late stages when 
respiratory involvement may make dusty or polluted 
air hazardous. 

SPINAL MUSCULAR ATROPHY 

Disease Description 

Spinal muscular atrophy is a hereditary childhood 
disease of unknown cause with progressive 
degeneration of the anterior horn cells. The weakness 



Functional Disabilities 

The wheelchair is the usual mode of ambulation, 
and older children may use electric wheelchairs if arm 
endurance becomes low. Transfers require the 
assistance of another person. Self- care skills and 
eventually eating require another person's help. Vision 
and hearing are unaffected, but speech may be hard to 
understand due to low voice volume and weak facial or 
tongue muscles. The extensive disability during the 
early formative years usually leads to significant social 
retardation due to the lack of normal learning 
opportunities during childhood. Children who survive 
into the teens are usually quite dependent 
psychologically upon their parents. 

Rehabilitation Potential 

The few children who survive into the teenage years 
and come to the attention of a rehabilitation counselor 
have poor long-term potential due to the low 
longevity. Short-term potential may be somewhat 
more favorable, since intelligence is unimpaired. 
However, social retardation and extreme physical 
dependency sharply limit the potential for gainful 
employment. Rehabilitation goals are generally more 
concerned with developing meaningful interests and 
opportunities for socialization. 



Standards of Evolution 

Until the availability of electromyography 20 years 



92 Corcoran 



ago, children with spinal muscular atrophy were often 
mistakenly diagnosed as having muscular dystrophy. 
Electrical testing of the nerves and muscles, enzyme 
tests of the blood, and muscle biopsy can all help 
establish the diagnosis and differentiate other causes of 
weakness in infants. A neurologist is the appropriate 
specialist for diagnosis. 

A physiatrist can evaluate residual abilities and 
recommend a physical and maintenance program. 
Physical and occupational therapists should assess 
mobility and self- care skills. Clearly, a social worker 
can help delineate the family relationships and the 
likelihood of reduced social skills. Educational 
background also needs careful review for adequacy. 

Total Treatment 

As for any child in a wheelchair, frequent 
monitoring by a physical therapist and changes in the 
size and components of the wheelchair will maximize 
residual abilities of children with spinal muscular 
atrophy. Older children should be given electric 
wheelchairs as soon as they are unable to propel a 
manual chair for significant distances. This will 
provide independent mobility in school settings and 
encourage social growth. The family must be taught a 
simple daily routine of range -of- motion exercises to 
prevent contractures. Self-help devices and training in 
activities of daily living may reduce dependency on 
parents. Corsets or spinal orthotics may be necessary 
to provide trunk support or to control scoliosis. 
Sometimes an adapted wheelchair with a semireclining 
back or trunk and head supports will accomplish the 
same thing with greater comfort. Surgical correction of 
deformities or contractures is hardly ever appropriate, 
since any useful function of the limbs is likely to have 
been lost due to the progressive muscle weakness. 
Family counseling and social services are usually 
helpful. A genetic counselor should be available to 
parents and relatives. 

Vocational Implications 

Education is important, especially for the older child 
with a slow progression, since the mind and the voice 
are the only factors left with which to perform any 
rewarding activities. Educational plans should be 
short-term in nature. It should be understood that 
aptitudes for hand function will be severely limited. 
Interests should be encouraged that lead to emotional 
and social development and counteract the 
dependency. Physical demands of any occupation or 
activity must be very slight. These clients will not be 
able to handle extremes of weather except during brief 
outdoor period for transportation or avocational 
pursuits. 



POLIOMYELITIS 
Disease Description 

Poliomyelitis is an acute virus infection of the spinal 
cord often followed by residual paralysis of muscles. It 
was the major cause of physical disabilities in this 
country until 20 years ago. It is also known as acute 
anterior poliomyelitis , infantile paralysis, or Heine- Medin's 
disease; it is most commonly referred to simply as 
"polio." Unlike the other diseases described in this 
chapter, poliomyelitis has a known cause, is 
preventable, and is not progressive after the. acute 
phase. 

Before the development of effective vaccines, 
poliomyelitis was the most common virus infection of 
the nervous system. The disease occurred most often in 
children under age 10. Because of its widespread 
prevalence, most people after this age had had enough 
random exposures to develop immunity. Major 
epidemics of poliomyelitis occurred in 1946, 1949, 1952, 
and 1954. Until 1954, 25,000 to 50,000 cases per year 
occurred in the United States. The introduction of the 
Salk vaccine in 1955 and of Sabin's vaccine in 1961 has 
practically eliminated poliomyelitis in the United 
States. Thus, it is rare to see residual paralysis from 
poliomyelitis in persons under 20, and the major group 
still bearing residual deficits from poliomyelitis as this 
book is published are adults in the 30 to 50 year age 
group, the prime years for gainful employment. This 
makes polio a disease that is still of major importance 
for rehabilitation counselors. 

The polio virus has a predilection for the anterior 
horn cells of the spinal cord. Other areas of the central 
nervous system may be affected to a lesser extent, but 
usually recover completely. Damage to the anterior 
horn cells leads to flaccid paralysis of the muscles they 
supply. Some anterior horn cells recover, but many do 
not, leading to degeneration of their axons extending 
down the peripheral nerves to the muscles, which in 
turn may undergo atrophy due to denervation. A 
characteristic of poliomyelitis is the scattered 
involvement of anterior horn cells at many different 
locations in the spinal cord. The result is that affected 
muscles may retain a few motor units, which during 
the recovery phase may enlarge to produce weak, but 
functionally useful, contractions of these muscles. For 
the same reason, limb involvement is usually 
asymmetrical. It is not uncommon for one extremity to 
have major paralysis while the opposite one remains 
largely unaffected. In a given limb, muscle groups may 
be affected to differing degrees, leading to muscle 
imbalance and deformities. Uneven involvement of 
trunk muscles may lead to curvature of the spine 
(scoliosis). If an entire limb is extensively paralyzed 
during the growing years, its rate of growth decreases, 



Neuromuscular Diseases 93 



resulting in a small, shortened limb in the adult. 

The cause of poliomyelitis is any of several strains of 
polio virus. The virus enters through the mouth from 
breath droplets of another affected person or through 
contaminated water. The virus spreads via the 
lymphatics or blood stream. Initial symptoms are those 
of an upper respiratory or gastrointestinal infection, 
with flu -like symptoms. There may be an interval of 
recovery lasting a few days, followed by a recurrence of 
fever with headache, stiff neck, and muscle soreness. 
Two to five days later, paralysis begins, and increases 
in severity for another three to five days. Paralysis of 
the respiratory muscles may create the need for 
mechanical respiratory maintenance using devices 
such as the Emerson tank respirator, or "iron lung." 
Tracheostomy is usually needed when there is 
respiratory paralysis. The need for mechanical 
respiratory maintenance may continue for weeks or 
months, and in a few cases where no respiratory 
muscle recovery occurs, this need may be permanent. 

About 5 percent of acute cases are fatal, usually due 
to respiratory paralysis or pulmonary infections. In 
nonfatal cases, control of facial and neck muscles is 
almost always recovered. Improvement of the paralysis 
begins 1 to 2 weeks after onset, and is usually complete 
or well along toward recovery within 2 to 3 months. 
Continued improvement after this time is usually 
related to hypertrophy of surviving muscle cells, and 
sprouting of surviving motor nerves to reinnervate 
other muscles cells that have lost their original nerve 
supply. 

Functional Diasbilities 

A wide variety of functional disabilities can be seen 
after poliomyelitis, depending on the highly variable 
pattern of residual muscle paralysis. Major deficits in 
the lower extremities may impair ambulation and 
transfers and necessitate the use of orthotics, 
ambulation aids, or wheelchairs. If the upper 
extremities are also involved, cane or crutch use may 
not be possible. Occasionally the upper extremities are 
severely or totally paralyzed in a person who remains 
able to walk normally, due to recovery in the lower 
extremities. Self- care skills and hand functions may be 
impaired to varying degrees. Slight remaining function 
in affected muscles allows poliomyelitis patients to 
develop a variety of substitutions or "trick 
movements" to facilitate hand functions. Intellectual 
functioning, personality, and communication skills are 
unaffected. Psychosocial impairments are variable, 
depending mainly on the pre-existing personal and 
social strengths. 

Rehabilitation Potential 

Short- and long-term rehabilitation is excellent in 



poliomyelitis patients. The deficits are stable and 
recurrences are almost nonexistent. 



Standards of Evaluation 

A physiatrist is the medical specialist best suited to 
evaluate functional deficits and residual abilities in a 
person who has had poliomyelitis at an earlier age. The 
physiatrist can advise on the need for the variety of 
therapeutic interventions that may be helpful. Physical 
and occupational therapy evaluations are often useful, 
depending on the location and extent of residual 
paralysis. 

Total Treatment 

During the acute phase of poliomyelitis, treatment is 
supportive in nature, with care to maintain hydration, 
nutrition, and ventilation, and precautions to avoid the 
complications of bed rest. As soon as recovery begins, 
muscle re-education and skill training is started. A 
variety of orthotics, ambulation aids, and self-help 
devices must be provided and the patient trained in 
their use. Occasionally muscle transplantation and 
other orthopedic procedures can help improve hand 
function and ambulation. 

Postpoliomyelitis patients who are entering middle 
age must guard against deterioration due to obesity, 
deconditioning from inactivity, degenerative joint 
disease (see chap. 14), and associated injuries. 
Whenever a period of bed rest is required for some 
unrelated medical problem, it is essential to begin 
vigorous therapy to maintain muscle strength and joint 
range until normal activities can be resumed. 

More specifically, fractures secondary to 
osteoporosis of a paralyzed limb can occur after 
minimal trauma. The treatment of these fractures 
requires careful assessment by the orthopedic surgeon 
of the patient's individual methods of compensating for 
his specific impairments. Degenerative joint disease 
may occur in the large joints of the legs (hip and knee) 
or in the lumbar spine because muscle weakness may 
have left them relatively unprotected over the years. 
Leg length discrepancies, in particular, may result in 
late -onset back pain in need of careful attention. 
Further, as the patient gets older, heavier, and less 
athletic, modification in whatever braces, canes, or 
crutches he is using may be required. Joint ligament 
laxity may also develop, particularly at the knee where 
hyperextension (genu recurvatum) can occur. For a 
reason often not clear, muscles that are known to be 
weak may develop further weakness through use and 
require brace protection that might not have been 
needed earlier. 

Finally, patients with mild respiratory difficulty may 
find greater respiratory difficulty with age as their 
lungs and thoracic cage lose some of their elasticity. 



94 Corcoran 



Patients permanently on respirators should be 
periodically reviewed, for more portable new 
equipment may become available and allow greater 
mobility. In later years, more frequent medical 
evaluation is useful in anticipating, aborting, or 
treating these late -onset problems. 

Vocational Implications 

Education and training may be considered without 
limitations in poliomyelitis clients. Life expectancy for 
these persons should be the same as for nondisabled 
persons of the same age. There may be some 
limitations in aptitudes for hand or leg activities, 
depending on the paralysis. However, the muscles 
which continue to function should have normal 
coordination and skill. Any of a variety of vocational 
interests may be encouraged, depending more on the 
underlying make-up of the individual than on the 
poliomyelitis residuals. The physical demands of the 
job should not tax the client's strength excessively, as 
there is some evidence that muscles weakened by past 
poliomyelitis may deteriorate if the load imposed upon 
them is heavy relative to their residual strength. 
However, ordinary levels of activity within the 
remaining strength of muscles is important to maintain 
their function. No special environmental factors need 
to be considered beyond the obvious limits imposed by 
the residual paralysis. 

GUILLAIN-BARRE SYNDROME 
Disease Description 

A disease with many similarities to poliomyelitis is 
Guillain-Barre syndrome, also known as infectious 
polyneuritis or Landry's ascending paralysis. About two- 
thirds of the cases give a history of a preceding 
infection of the upper respiratory or gastrointestinal 
tracts, but no virus has yet been identified. There is 
some experimental evidence that suggests allergic or 
autoimmune factors, but no definite cause has yet been 
established. The disease causes segments of the myelin 
sheath of peripheral nerve axons to be progressively 
destroyed (demyelination), causing the conduction of 
nerve impulses to slow down. In very severe cases, the 
axon itself degenerates. 

In those cases preceded by a respiratory or 
gastrointestinal infection, there is usually an interval of 
5 to 12 days before weakness begins. The lower limbs 
are often affected first, with weakness ascending within 
a few days to involve the trunk, upper limbs, and the 
muscles supplied by the cranial nerves. Maximum 
paralysis is usually reached within 1 to 3 weeks. Facial 
and respiratory muscle paralysis is rather common, 
and patients may need mechanical respiratory support 
during the acute phase. Like poliomyelitis, flaccid 



paralysis with denervation atrophy may be found in 
affected muscles. The deficits are usually bilateral and 
symmetrical, unlike those caused by poliomyelitis. 
About 85 percent of patients recover completely with 
no neurological residuals. The myelin sheath slowly 
regenerates, and full muscle strength returns after 
many months. 

The remaining 15 percent are those patients with 
axonal degeneration. Although the axons do slowly 
regenerate (1 mm per day; 1 cm per week; 1 inch per 
month), there is no full recovery of muscle function. 
The rehabilitation problems of these patients depend 
on their residual disabilities. 

Functional Disabilities 

Residual weakness or complete flaccid paralysis may 
remain. The hands and feet are most commonly 
affected. Deficits are usually uniform and symmetrical, 
and the deformities common after polio are not usually 
a problem in Guillain-Barre syndrome. Respiratory 
paralysis may persist for months or be permanent, 
requiring mechanical respiratory support. There is 
rarely any sensory loss or cerebral damage. 

Rehabilitation Potential 

Since the deficits are confined to motor neurons, 
without sensory or cerebral damage, rehabilitation 
potential is good. Recurrences are very rare. 

Standards of Evaluation 

A neurologist usually manages the patient during 
the acute phase. The diagnosis is made from the typical 
history and course and from a spinal tap showing a 
characteristic elevation of the protein level of the fluid 
without the associated elevation of white cells found in 
the cerebrospinal fluid of acute poliomyelitis patients. 
An electromyogram may be helpful. 

A physiatrist can assess residual functional abilities 
and the need for equipment and therapy. An 
occupational therapist evaluates hand function and 
self- care skills. A physical therapist can assess muscle 
strength, joint range, and gait. When the severity of 
the paralysis necessitates major changes in occupation 
or life style, assessments by a psychologist, social 
worker, and rehabilitation counselor are important. 

Total Treatment 

Acute management for Guillain-Barre syndrome is 
similar to that for poliomyelitis. Long-term care 
depends on the degree of residual weakness, and may 
include self-help devices, orthotics, ambulation aids, 
or wheelchairs, and training in their use. Extensive 
permanent paralysis may require an electric 



Neuromuscular Diseases 95 



wheelchair and sometimes mechanical respiratory 
support. 

Vocational Implications 

The guidelines for poliomyelitis also apply to 
Guillain-Barre syndrome. Education and retraining 
are usually feasible since intelligence and 
communication are unimpaired by the disease. If 
paralysis is severe, clients may need retraining for 
occupations having fewer physical requirements, 
especially for hand and wrist function and foot and 
anlde function. Occupations requiring fine finger 
dexterity or pushing, pulling, and gripping would be 
contraindicated. Short leg braces or a cane restrict 
walking, stooping, and bending. 

MYASTHENIA GRAVIS 

Disease Description 

Myasthenia gravis is a disease that causes weakness 
and fatigability of the muscles. There may be 
remissions and exacerbations, but the disease is 
generally progressive. It can begin at any age, but is 
commonest in young adults, where female cases 
outnumber males 3 to 1; after age 40, males and 
females are affected equally. The prevalence is about 3 
cases per 100,000 population. 

While the cause is unknown, the symptoms are 
known to be caused by a decrease in the amount of 
acetylcholine at the neuromuscular junction (see p. 
31). There may also be an increased amount of 
acetylcholinesterase, the chemical that inactivates 
acetylcholine.) Acetylcholine is the neurotransmitter 
chemical at the motor end plate which, when triggered 
by a motor nerve impulse, initiates the contraction of 
the muscle. The reasons for its deficiency are 
unknown, but it is probably a form of autoimmune 
disorder. The disease is frequently associated with 
tumors of the thymus gland, and may improve after 
removal of the thymus. It may also occur in association 
with a number of other conditions, including 
pregnancy, increased or decreased thyroid function, 
rheumatoid arthritis, lupus erythematosus, or 
malignant tumors of the lung or pancreas. 

The disease usually begins with weakness of the 
eyelids, throat muscles, or trunk or limb muscles. The 
weakness is greater after exercise or at the end of the 
day and improves with rest. A common sign is 
drooping (ptosis) of the eyelids. There is often fatigue 
after chewing and difficulty swallowing. In late stages 
of the disease, respiratory muscles may be involved, 
leading to pneumonia and other pulmonary 
complications. Except for cardiac deconditioning, the 
complications of inactivity are not usually seen, since 
patients are usually able to continue with ordinary light 
activities of short duration. 



The disease usually worsens progressively, although 
remissions are common. In untreated patients, death 
may result within a few years. Life expectancies are 
somewhat longer in recent years, due primarily to the 
introduction of neostigmine and related drugs of the 
"anticholinesterase" family. These drugs inactivate 
the acetylcholinesterase which breaks down 
acetycholine, thus prolonging and enhancing the effect 
of the diminished supply of acetylcholine at the 
neuromuscular junction. They are ordinarily taken by 
mouth on a long-term basis to maintain exercise 
tolerance, but may be given intramuscularly or 
intravenously in severe attacks. Some patients with 
severe flare-ups become refractory to neostigmine 
treatment (myasthenic crisis) and require trach- 
eostomy and mechanical respiratory assistance. With 
changes in medication, improvement may occur 
and allow resumption of ordinary activities. With 
careful management, many patients may remain 
relatively active for many years with the disease. 

Functional Disabilities 

In general, the disabilities caused by myasthenia 
gravis affect strenuous and repetitive activities of the 
muscles. Light activities of short duration or few 
repetitions can usually be performed satisfactorilv. 
Thus, patients may have difficulty with prolonged 
standing, stair climbing, or long-distance walking, but 
can usually walk for short distances without difficulty. 
Self- care activities are usually performed inde- 
pendently, but they may take longer than normal. 
Even light activities that are prolonged in duration 
may be impaired, such as writing or typing. With 
careful medical treatment, many of the impairments 
can be diminished. 

Social disabilities are usually not as severe as with 
other neuromuscular diseases, since a limited amount 
of normal activity can be continued. The usual 
psychological reactions to disability may be seen (see 
chap. 2), but there are no special personality or 
intellectual changes associated with the disease. Young 
men or women who present the symptoms of 
myasthenia gravis are frequently misdiagnosed as 
"hysterics," which often results in their losing the 
emotional support of family and friends when they 
need it the most. 

Rehabilitation Potential 

Short-term rehabilitation potential is generally 
quite good, since most patients respond well to medical 
management. Long-term potential is also good, except 
in those patients who have rapidly progressive severe 
symptoms that do not respond to treatment. In 
general, the rehabilitation counselor may anticipate 5 
to 20 years of productive life. The somewhat 



96 Corcoran 



unpredictable nature of the disease, with inexplicable 
remissions, should also be kept in mind. Patients may 
be done a serious injustice if vocational rehabilitation 
services are withheld on the assumption that all 
patients necessarily deteriorate. 

Standards of Evaluation 

If myasthenia gravis is suspected, the diagnosis can 
usually be made without difficulty from characteristic 
history and physical findings. A neurologist should 
confirm the diagnosis and rule out related diseases. 
The neostigmine or Tensilon test is usually diagnostic 
of the disease: an injection of one of these drugs should 
be followed within a short time by significant 
improvement of weakness. Characteristic findings on 
electromyography and repetitive nerve stimulation will 
confirm the diagnosis. 

A careful and thorough general medical 
examination is important to identify the several related 
diseases that may coexist with myasthenia gravis (see 
above). There is generally no need for examination by 
a physical or occupational therapist unless associated 
physical disabilities are present. The treating physician 
or a nurse should take the time to explain the nature of 
the disease and the organic cause of the symptoms, 
especially with patients who have been misdiagnosed. 

Total Treatment 

Three related drugs in the anticholinesterase family 
are the mainstays of treatment: neostigmine 
(Prostigmin); pyrostigmine (Mestinon); and amben- 
onium (Mytelase). Patients generally take these 
drugs orally in several daily doses. In severe cases they 
may be given by injection. These drugs may produce 
some troublesome gastrointestinal side effects 
including diarrhea, abdominal cramps, or excessive 
salivation. The side effects can usually be controlled 
with atropine, belladonna, or related drugs. 

In recent years, surgical removal of the thymus 
gland (thymectomy) has been used with success in over 
half of the patients so treated. Benefits may not appear 
for several months or a few years after the surgery. 
Some permanent remissions have been reported after 
thymectomy. 

Psychological or social counseling may be necessary 
in situations where the disease has produced serious 
disruption of the social structure or major 
psychological complications. 

Vocational Implications 

Clients with myasthenia gravis are good candidates 
for re-education to less physically demanding 
occupations. In cases under good medical control, a 2 
to 4 year educational plan may not be inappropriate. 



In unresponsive or rapidly progressive cases, shorter- 
term education might be more suitable. Intellect, 
learning ability, and motor coordination and dexterity 
should all remain intact. A variety of interests may be 
encouraged if they do not involve prolonged or 
repetitive performance of muscular activities. Physical 
demands should be those of sedentary or light activities 
with frequent rest periods possible. Prolonged lifting, 
carrying, or pulling should be avoided in an 
occupational choice. Continuous writing or typing 
may be troublesome, although their intermittent 
performance may present no problems. Speech, 
hearing, and vision will all remain unimpaired. The 
job site should be accessible by motor vehicle or public 
transportation, without long walks or multiple flights 
of stairs. Unusually cold working environments should 
be avoided, but other ordinary environmental or 
weather conditions should be well tolerated. 

MUSCULAR DYSTROPHY 

Disease Description 

Muscular dystrophy refers to a family of hereditary 
diseases that cause degenerative changes in the 
muscles, leading to progressive weakness and 
disability. The cause of muscular dystrophy is 
unknown. It is known that there is abnormal function 
of a number of enzymes found in muscles, but the 
underlying cause for this is not well understood. There 
are about 4 cases per 100,000 population. Since the 
disease affects children and young adults, it has major 
vocational implications. 

There are several major types of muscular dystrophy, 
each affecting different ages with different patterns of 
involvement and rates of progression. The commonest, 
Duchenne, or childhood muscular dystrophy, accounts for 
about 85 percent of all cases. It is carried by a sex-linked 
recessive gene and occurs only in boys. Symptoms and 
signs are usually present before the age of 5, and within 
one decade patients usually require a wheelchair for 
mobility. 

Duchenne dystrophy occurs first in the hip muscles, 
where weakness in young children leads to clumsiness, 
difficulty running and later walking, and weakness, 
particularly when arising from the floor or going up 
curbs or stairs. Later the shoulder muscles become 
weakened, with difficulty reaching over the head or lifting 
heavy objects. The small muscles, such as those of the 
face and hand, usually retain their function until late 
stages of the disease. The respiratory muscles weaken 
progressively, and the vital capacitv falls steadilv. In some 
cases the heart muscle is involved, leading to heart 
failure. About one-third of affected children have some 
degree of mental retardation. Most of the physical 
findings are related to weakness of the affected muscles. 
Shoulder involvement causes difficulty with reaching or 



Neuromuscular Diseases 97 



heavy lifting and a drooping of the shoulder or protru- 
sion ("winging") of the scapula. There may be a waddling 
gait and an increase in lumbar lordosis (see p. 207) due to 
weakness in the abdominal and hip muscles. Contrac- 
tures maybe present in affected joints. Since the disease is 
confined to muscle cells, there are no sensory changes. 

Most of the remaining 15 percent of muscular 
dystrophy cases fall into the categories of limb -girdle 
dystrophy or facioscapulohumeral dystrophy. These forms 
begin in teenagers or young adults, affect mainly the 
shoulder and hip muscles, and progress more slowly, 
usually requiring two decades or more before patients 
are severely incapacitated. They differ from one 
another primarily in the involvement of the facial 
musculature in the latter. 

Two relatively rare forms of dystrophy are myotonic 
dystrophy, and ocular or ophthalmoplegic dystrophy. 
Typically, these forms begin in young adults, progress 
quite slowly, and do not cause severe disability for 
several decades or longer. Myotonic dystrophy, unlike 
the other forms, affects distal muscles first, particularly 
the muscles of the hand. The characteristic myotonia is a 
cramp -like inability to relax muscles for several 
seconds after a forceful contraction. Typically, patients 
with myotonic dystrophy also exhibit cataracts, frontal 
baldness, and testicular atrophy, presumably due to 
associated genetic abnormalities. Facial muscle 
paralysis and drooping of the eyelids are common. 
There is a high degree of associated mental retardation 
and other congenital physical defects. Ocular 
dystrophy is usually limited to the eye and throat 
muscles, causing double vision, drooping of the 
eyelids, and difficulty swallowing. 

The pathological findings in early phases of 
muscular dystrophy may include swelling of the muscle 
cells, leading to enlargement of the weakened muscles 
(pseudohypertrophy). In later stages, there is 
degeneration of muscle cells, which are replaced with 
collagen fibers and fat. 

Complications of muscular dystrophy are related to 
inactivity and bed rest. Injudicious use of the 
wheelchair or prolonged bed rest after routine illnesses 
often leads to premature loss of the ability to walk. 
Contractures of the joints are common, particularly 
when daily home range -of- motion exercises are not 
administered faithfully. Trunk muscle imbalance often 
leads to scoliosis. Pulmonary complications are 
common in late stages when the breathing and 
coughing ability is limited. 

The muscular dystrophies pursue a variable course. 
Most patients with Duchenne dystrophy are severely 
disabled by the middle teens and few survive past their 
twenties. There are exceptions, however; a variation 
called Becker's dystrophy begins like other Duchenne 
cases but progresses more slowly. Limb- girdle, 
facioscapulohumeral, or late-onset Duchenne 
dystrophy patients may progress slowly and remain 



functional well into middle life. In general, the later the 
age of onset, the slower the progression. Many patients 
who die of respiratory failure could continue to live 
with mechanical respiratory assistance, although their 
physical abilities would be severely limited. 
Mechanical respiratory assistance is not usually 
introduced to end -stage muscular dystrophy patients 
unless their intellectual and social skill levels are 
sufficiently high to permit self- directed independent 
living after their parents become unable to care lor 
them. 



Functional Disabilities 

The major functional disabilities result from 
weakness of the proximal muscles of the shoulders and 
hips. Ambulation skills diminish steadily, leading to 
wheelchair mobility. Because of the difficulty in rising 
from a sitting position, wheelchair transfers usually 
require assistance. Eating, dressing, and personal 
hygiene become more and more limited. The ability to 
use public transportation is lost because of the inability 
to mount bus steps or subway stairs. Many of these 
disabilities can be diminished with appropriate 
rehabilitation therapy. Communication skills are 
usually unaffected. Vision and hearing are not 
involved, and speech should be normal except for low 
voice volume in late stages. 

As expected in a severe disability, there are often 
major psychological and social disabilities. In many 
cases, social retardation with dependence on parents 
and lack of normal school and play experience leads to 
a syndrome which may be hard to distinguish from 
mental retardation. Mainstreaming of these children 
and appropriate parental counseling should diminish 
the psychosocial disabilities in these children. 

Rehabilitation Potential 

Short-term rehabilitation potential is usually good 
except in the end stages of muscular dystrophy. This is 
especially true if intelligence is normal. Educational or 
training programs to develop mental or verbal 
vocational skills may be quite appropriate. Hand and 
finger function for light activity is usually preserved 
until the end stages. Some forms of muscular 
dystrophy progress quite slowly, as outlined above. 

Long-term rehabilitation for Duchenne muscular 
dystrophy is quite limited, unless intelligence is normal 
or above normal and social growth is possible. On the 
other hand, even a highly intelligent or verbally skillful 
teenager with muscular dystrophy has very little 
rehabilitation potential if he has been allowed to 
remain totally dependent on his parents and siblings 
and has not had access to the normal social 
opportunities that go with peer contact in school, 
playgrounds, and community settings. The total 



98 Corcoran 



patient, rather than the extensiveness of the muscular 
weakness, is what determines long-term rehabilitation 
potential. 

Standards of Evaluation 

A neurologist or physiatrist who is experienced with 
muscle diseases should make the diagnosis of muscular 
dystrophy. The Muscular Dystrophy Association 
sponsors clinics in most parts of the United States 
where diagnostic services are available. The 
characteristic medical history and physical findings 
point to the proper diagnosis in most cases. Several 
laboratory studies help confirm the diagnosis. The 
level of various muscle enzymes in the blood, such as 
creatine phosphokinase (CPK), may be elevated in the 
early stages of rapidly progressive dystrophy such as 
Duchenne dystrophy, but is normal in the later stages 
or the more slowly progressive forms. Electromyo- 
graphy is very useful in ruling out diseases with 
similar clinical pictures. A muscle biopsy is helpful, 
but must be done with caution to prevent temporary 
bed rest or disuse of the biopsied area, since the 
patient may never regain function after a little 
disuse atrophy or joint contracture has been allowed to 
form. Electrocardiography should be done to detect 
associated cardiac muscle involvement. 

A physical therapist can evaluate muscle strength, 
joint range, and gait skills. The occupational therapist 
can evaluate self- care abilities and the use of adaptive 
equipment and energy -saving techniques by affected 
patients or the care providers in their families. 

A psychological assessment is useful to identify and 
quantitate mental retardation. Evaluation by a skilled 
psychologist is also required to distinguish organic 
mental retardation from the equally common social - 
emotional retardation resulting from severe 
dependency in childhood. A social evaluation of the 
family's strengths is also important. 



muscular dystrophy clinics to see patients who lost the 
ability to walk after "successful" surgical correction of 
a contracture or biopsy of a muscle for diagnostic 
purposes. 

Physical therapy is essential to maintain the range of 
motion of affected joints. However, muscle 
strengthening exercises are usually not helpful, as the 
patient is usually using his weakened muscles to the 
maximum of their ability already. The physical 
therapist also instructs patients in the use of 
ambulation aids and wheelchairs. Occupational 
therapy may be needed for training in self- care skills 
and the use of assistive devices, such as arm slings or 
balanced forearm orthoses, to preserve hand function 
if shoulder weakness is severe. Orthotics may be 
needed to provide ankle stability and prolong the 
period of walking. When scoliosis is present, corsets or 
trunk orthotics, such as body jackets, may provide 
better trunk control or sitting balance. In a patient who 
already uses a wheelchair, special trunk -support 
attachments or a slightly reclining wheelchair back 
may accomplish the same thing without the need for a 
body jacket. 

Several types of counseling are important at various 
stages in the disease. Genetic counseling has obvious 
value to assist unaffected relatives in their family 
planning. Psychological and social service counseling 
helps patients overcome various hurdles and accept 
their limitations. Nutritional counseling and special 
care to avoid obesity will prolong ambulatory or 
transfer independence, and ease the burden of care for 
family members after the patient has become 
dependent in mobility and self- care. 

The medical management of muscular dystrophy, 
then, revolves around the maintenance of good health 
and nutrition, the treatment of routine intercurrent 
illnesses and injuries, and the counseling and guidance 
of the patient and his family so that proper decisions 
are made at the proper time regarding rehabilitation. 



Total Treatment 

There is no drug treatment that is effective against 
the primary symptom, the weakness of muscular 
dystrophy. In the small percentage with myotonia, this 
symptom can be alleviated by oral drugs to relax 
muscular tension, such as quinine, procaine amide, or 
Dilantin. Surgery is only necessary when contractures 
have been allowed to develop through failure to do 
range -of- motion exercises regularly. Contractures, 
once formed, should be surgically corrected only if they 
interfere with some specific function that is important 
enough to justify the surgery. The postoperative 
immobilization and bed rest must be as short as 
possible, and vigorous bedside physical therapy is 
essential to maintain muscle strength and joint range 
during any postoperative period of immobility. The 
same is true after muscle biopsy. It is common in 



Vocational Implications 

Educational planning should take into account the 
anticipated remaining life expectancy. The past rate of 
deterioration is the best predictor of future 
progression. When intellectual and social skills make 
independent self- direction still possible even after 
extensive physical impairment has developed, the 
option of mechanical respiratory maintenance should 
not be ruled out. Connecting the end -stage muscular 
dystrophy patient to a respirator will probably prolong 
his life by 10 to 20 years. Most clinicians feel that this is 
inappropriate for the mentally, socially, or 
educationally retarded teenager or adult who is 
dependent upon aging parents. On the other hand, it 
may be highly appropriate for the intelligent and 
independent -minded teenager or adult with advanced 
muscular dystrophy who is running out of lung 



Neuromuscular Diseases 99 



capacity, but is independent or has the potential for 
continued education leading to independent living. 

Aptitudes for intellectual functioning and learning 
may be limited where mental retardation is associated 
with the muscle weakness. Verbal and communication 
skills are usually intact. Hand and finger dexterity may 
be well preserved, although grip strength is usually 
diminished. If myotonia is present and cannot be 
medically controlled, hand and finger skills may be 
markedly limited. A variety of interests may be 
encouraged as long as the progressive physical 
impairments are kept in mind. 

Physical demands for sedentary or light activity may 
be well tolerated. In the earlier or more slowly 
progressive forms of muscular dystrophy, fairly heavy 
work may remain possible. Shoulder involvement may 
prohibit heavy lifting, pulling, or pushing. Hip 
involvement will limit stooping, kneeling, and 
crawling. However, standing and walking may remain 
possible in the more slowly progressive forms of the 
disease. Eye muscle involvement may interfere with 
reading or fine work due to double vision or drooping 
of the eyelids. Associated cataracts or cardiac 
impairments may add further limitation. The 
advanced -stage client in a wheelchair will have 
difficulty protecting himself from extremes of weather 
outdoors, but otherwise no special environmental 
factors need to be considered. 

SUMMARY 

This chapter has reviewed some rather mysterious 
and frightening diseases which have tended to be 
misunderstood by the general public and sometimes 
mismanaged by the health professions. Studying or 
managing them may produce some strong personal 
reactions in the counselor or student. In order to best 
serve the needs of clients and avoid some common 
pitfalls, several caveats are in order concerning the 
management of neuromuscular diseases. 

Do not be a slave to statistics. One should avoid a 
blind over -reliance on mean survival times and similar 
statistical data. For an individual client, his own 
survival time is what matters, and this may not be easy 
to predict. A great disservice is done to the client who is 
told such things as, "You have 3 years to live," only to 
find 10 to 15 years later that he fell at the long end of 
the range of survival times, and wasted his life waiting 
to die. A more constructive approach is to try to 
maximize the available functional abilities at the point 
in time when the client is encountered. 

Do not overlook the complications of inactivity. Few of 
these diseases are the direct causes of death; clients die 
of the complications of inactivity or bed rest (see chap. 
4). Some of these complications may be present when 
the client is first encountered and may even be the 
major causes of the functional disabilities he presents. 
Common examples are joint contractures, disuse 



atrophy of muscles, cardiovascular deconditioning, 
and psychological depression or social retardation. 
These can all be prevented, detected, and corrected. 
To overlook them is to condemn the client to 
unnecessary additional disability on top of the 
unavoidable burden of his basic neurological deficit. 

Do not overestimate functional limitations. Health 
professionals have a tendency to exaggerate the degree 
to which a physical deficit produces a functional 
impairment. Experience with disabled persons will 
teach the open-minded professional to appreciate the 
sometimes amazing feats of independent performance 
which are possible even with extensive physical 
deficits. The task is not to constrict the horizons of 
clients, but to make available all of the training, 
equipment, and motivational supports that can be 
mustered to maximize their functional potential. 

Do not personalize the emotional response of the client. 
Some health professionals conclude that they would 
feel overwhelmed or devastated if they had the client's 
problem, and then assume that the client shares their 
feelings. Assuming that the client must be terribly 
depressed, they may respond with excessive empathy 
or patronizing pity. It turns out, however, that persons 
disabled by chronic progressive diseases are often less 
depressed than those previously able-bodied persons 
who are suddenly struck down with an acute disability. 
These persons have the psychological advantage of 
having had time to adapt to gradually progressive 
deficits. In addition, those with a family history of the 
same disorder may have had an opportunity to develop 
an appreciation of the residual abilities. 

Hope is a stronger human urge than despair. Even 
those clients who have been given a grimly specific 
prognosis by some well-meaning physician will usually 
choose to hope for recovery or improvement. This is a 
healthy reaction which should be directed into 
constructive, realistic action plans and not 
extinguished by the professional's own personal 
notions of how he might feel under the same 
circumstances. 

Do not use pejorative or negative terms. This is a corollary 
of the preceding precaution. The client is not a polio 
"victim" or a muscular dystrophy "sufferer," nor is 
he "afflicted" with Parkinson's disease. Persons are 
not "confined" to crutches or wheelchair "bound." 
The client is a person who happens to have a disability 
due to polio, muscular dystrophy, or Parkinson's 
disease. His crutches do not confine, but rather free 
him from the chair or bed. The wheelchair does not 
bind, but liberates its user, so that he may go to school 
or work or travel around the world. Our language 
reflects our attitudes, and these pejorative terms have 
no place in the jargon of rehabilitation. 

In progressive neuromuscular diseases, as with other 
categories of disabilities, the counselor should develop 
a positive philosophy of management based on an 
appreciation of the constructive medical and physical 



100 Corcoran 



treatment measures available and the realistic 
rehabilitation potential of this group of disabled 
persons. 

BIBLIOGRAPHY 

Anderson AD, Levine SA, Gellert H: Loss of ambulatory 
ability in patients with old anterior poliomyelitis. Lancet 
2(7786): 1061-1063, 1972. 

A brief report of several cases, this article discusses the 
possible causes and strategies for prevention of late 
deterioration in patients who had poliomyelitis in the 
past. 

Cailliet R: Rehabilitation in parkinsonism. In Licht S (ed): 
Rehabilitation and Medicine . Baltimore, Waverly Press, 1968, 
pp. 435-445. 

This chapter reviews the pathology and clinical picture of 
parkinsonism in nontechnical terms with helpful diagrams. 
The management and physical rehabilitation are well 
covered. 

Chusid JG: Correlative Neuroanatomy and Functional Neurology. 
Ed 16, Los Altos, CA, Lange Medical Publications, 
1976. 

This paperback guide provides simple explanations of the 
anatomy and physiology underlying the disorders of the 
neuromuscular system. 

Downey JA, Low NL (eds): The Child with Disabling Illness: 
Principles of Rehabilitation. Philadelphia, Saunders, 1974. 
This textbook discusses all types of childhood disabilities 
with special emphasis on rehabilitation considerations. 
The section on the neuromuscular system has an excellent 
chapter on muscular dystrophy. 

Greenfield JG: The Spino-Cerebellar Degenerations. Springfield, 111., 
Thomas, 1954. 

This short monograph presents an excellent summary of the 
family of spinocerebellar degenerations, including Friedreich's 
ataxia. An interesting historical review is presented, and the 
range of symptoms, signs, and rates of progression are 
described. 

Martin RD, Flegenheimer VW: Psychiatric aspects of the man- 
agement of the myasthenic patient. Mt Sinai J Med NY 
38:594-601, 1971. 

This article describes the need for psychiatric care in the 
management of myasthenic patients. Because strong emo- 
tions often precede symptoms, and emotional disturbances 
can exacerbate the condition, ways of releasing emotions or 
handling anger need to be taught. Mild depression is also a 
common problem. 

Merritt HH: A Textbook of Neurology. Ed 5, Philadelphia, Lea and 
Febiger, 1973. 

A basic neurology textbook. Good sections on the diseases 
covered in this chapter. 

Singer E: Premature social aging: the social-psychological 
consequences of a chronic illness. Soc Sci Med 8:143-151, 
1974. 

A group of Parkinson patients 65+ years of age and a group of 
younger Parkinson patients were compared with normal 
populations of the same age groups. The younger Parkinson 
patients appeared to be more affected by restriction of social 
and vocational activities than the older patients as compared 
to the normal population. 



Taft LT: The care and management of the child with muscular 
dystrophy. Dev Med Child Neurol 15(4):5 10-51 8, 1973. 
A pediatrician discusses problems likely to occur in the initial 
stages of counseling and treating a child with muscular 
dystrophy and also those likely to occur as the child gets older. 
Written for physicians but general enough for counselors, this 
text provides useful background and evaluation guides. 



7 PERIPHERAL NEUROPATHIES 



Joseph Goodgold, M.D. 



DISEASE DESCRIPTION 

A precise statement concerning the incidence of 
peripheral neuropathy is not readily formulated 
because of the wide variety of causes of this condition. 
According to one report, of a total of 130,922,500 
disabled United States citizens, over 11 percent had 
problems which included lifting, reaching, handling, 
and fingering, and an inability to use the lower 
extremities. About 1.5 million individuals in this 
group suffered from paralysis of various types. While 
not all of the paralyses are peripheral neuropathies, 
their contribution is large and there appears to be little 
dispute that the numbers are increasing. In one 
neuromuscular center (New York University), it is 
estimated that more than 1,500 peripheral neuro- 
pathies are examined annually in the electrodiagnostic 
section, while at least twice that number are identified 
solely by clinical examination. Multiplying these figures 
over the many major medical centers throughout the 
country suggests the magnitude of the problems likely 
to come to the attention of the rehabilitation counselor. 

Peripheral neuropathies are diseases of the 
peripheral nervous system, i.e., those nerves that are 
located outside the brain and the spinal cord (see chap. 
3 for a review of the nervous system). Included, 
therefore, are the cranial nerves, all the spinal nerves 
and their associated plexuses and distal nerve 
divisions, and also the nerves of the autonomic nervous 
system. All three systems carry information between 
the central nervous system and the periphery. 
Involvement of the somatic nervous system is generally 
of greater importance in peripheral neuropathy than 
involvement of the involuntary autonomic nervous 
system. The terms "peripheral neuritis" and 
"polyneuritis" are sometimes used as synonyms of 
peripheral neuropathy and polyneuropathy. The "itis" 
suffix means inflammation. Since inflammation is not 
always present, these terms are best avoided. 

Figure 3-1 (p. 19) depicts the basic unit of the periph- 
eral nervous system and the structures that can be 
diseased in peripheral neuropathies. Since the cell 
body is essentially a central nervous system structure, 
peripheral neuropathies will involve the axon, or the 
myelin, or both. Thus, in a peripheral neuropathy, the 



disease may primarily affect the axon (axonal disease) 
or cause loss of myelin (demyelinating disease), or the 
disease may be axonal and secondarily demyelinating. 
In some situations, axonal disease and demyelinization 
occur simultaneously. In the primary demyelinating 
diseases, the process may be distributed along the 
entire nerve fiber, or it may occur segmentally. 

The speed with which an electrical impulse travels in 
a peripheral nerve depends on several factors: the 
health of the axon membrane; the diameter of the 
axon; and, if the fiber is myelinated, the thickness of 
the myelin and the distance between the nodes of 
Ranvier. 

In the resting state, a nerve axon is polarized, with a 
layer of positive electrical charges on the outside 
surface of the membrane and a layer of negative 
electrical charges on the inside. Thus, the voltage is 
negative inside compared to outside. The amount of 
this polarization depends predominantly on the 
differences in the concentration of potassium (K+) and 
sodium (Na+) ions between the inside and the outside. 

The concentration of sodium is greater on the outside 
and the concentration of potassium is greater on the 
inside. The magnitude of these concentration 
differences is governed by the ability of the membrane 
to allow or prevent the passage of these ions 
(membrane permeability). 

When an impulse begins, either at the cell body or, if 
induced from the outside, by an electrical current 
shock, the amount of the polarization (i.e., membrane 
voltage, membrane potential) is reduced at the point of 
stimulation. When the polarization is reduced to a 
certain level (threshold), the membrane suddenly 
becomes very permeable to Na+ ions, which rapidly 
diffuse into the axon from the outside. The polarization 
then become negative on the outside and positive on 
the inside (fig. 7- 1). 

An unstable situation is created when the polarity of 
one region of the axon membrane is reversed and the 
polarity of adjacent regions is normal. Local electrical 
currents result, which make the neighboring regions 
also less polarized, and therefore more permeable to 
Na-t- ions. In this way, a wave of depolarization travels 
down the length of the axon. The speed of propagation 



10 1 



102 Goodgold 



of this "action potential" (electrical impulse) is termed 
the conduction velocity of the nerve. 

The more rapid the permeability change in the 
membrane, the greater the magnitude of the local 
currents, and the faster the speed of conduction. The 
larger the axon diameter, the faster the speed of 
conduction. If the fiber is also myelinated, the thicker 
the myelin, the faster the propagation speed. In 
addition, for myelinated fibers, the longer the distance 
between the nodes of Ranvier, the faster the conduction 
speed, because depolarization "jumps" from node to 
node. Myelinated fibers therefore conduct impulses 
much more rapidly than unmyelinated fibers do. 

In a demyelinating peripheral neuropathy, the 
myelin thickness diminishes, and the speed of 
conduction is reduced. If the demyelination is diffuse, 
the speed is reduced along the entire length of the fiber. 
It the demvelination is segmental, the speed may be 
reduced only in those demvelinated sections and be 
norma] in other sections. If the demyelination is severe, 
the fiber may not conduct at all, and strength and 
sensation will be reduced. 

In an axonal peripheral neuropathy, axon diameter 
may decrease and cause a decrease in conduction 
velocity. The decrease in conduction velocity in axonal 
disease is much less than in demyelinating disease. The 
more usual effect of axonal disease, however, is a 
cessation of impulse conduction. Axonal disease 
therefore causes weakness and a reduction in 
sensation, usually more than in demyelinating disease. 

Peripheral neuropathies can be mostly sensory, 
mostly motor, or a combination of both. In addition, a 
peripheral neuropathy may only involve one nerve 
(mononeuritis or mononeuropathy); several nerves, 
with complete sparing of the others (mononeuritis or 
mononeuropathy multiplex); or all of the nerves 
usually in a somewhat symmetrical manner 
(polyneuritis or polyneuropathy). The disturbances 
associated with autonomic system neuropathy, often 
referred to as trophic changes, include dryness and 
thinning of skin and subcutaneous tissue, brittleness 
and slowing of growth of nails, loss of hair, and other 
phenomena associated with the peripheral vascular 
system, such as swelling and discoloration. Sweating 
may also be impaired where skin sensation is reduced. 

When an axon dies in axonal disease because of 
death of the cell body, the segment distal to the cell 
body undergoes what is referred to as Wallerian 
degeneration. If an axon is interrupted, as it may be in 
acute trauma, the axon distal to the interruption also 
undergoes Wallerian degeneration. The axon may lose 
its ability to conduct an externally applied electric 
current immediately after interruption or it may take a 
few days before conduction ceases. 

Recovery requires regeneration of the axon down 
the residual tube which previously contained the 
healthy fiber. Growth rates are about a millimeter per 




body 



Axon 
membrane 

/ Axon 

++++- — ■=4== 



+ + • 



(b) 



FIGURE 7-1. Schematic representation of the depolarization 
wave beginning at the cell body of an unmyelinated nerve. The 
region of depolarization, (A), is close to the cell body. The nor- 
mal resting polarization is at (B). The arrows indicate the local 
currents, which cause neighboring regions to become suc- 
cessively depolarized toward the right. After a very short period 
of depolarization, the membrane restores the polarization to its 
normal resting condition. 

day. Therefore, if the location of the axon death is 
known, as is usually the case when it is caused by 
external injury, the recovery time can be estimated. It 
is rare in such regeneration processes for recovery to be 
complete. The longer the path over which regeneration 
must occur, the less complete the recovery will be. 
Regenerating axons are of smaller diameter, have less 
mature membranes, and, if they are myelinated, a 
thinner layer of myelin and perhaps also a reduced 
distance between the nodes of Ranvier. A regenerating 
axon therefore has a much slower conduction velocity 
than a normal axon. Even when the regenerating axon 
is fully mature, its conduction velocity may still be less 
than normal. 

Another interesting form of recovery or partial 
recovery, which results particularly in the case of 
motor nerves, is the ability of unaffected motor nerves 
to begin to "take over" for a dead nerve fiber. A 
residual motor nerve fiber can send out additional 
In inches to make connections with muscle fibers that 
have lost their nerve connections. This branching 
process may take up to 2 years to be fully complete, 
and again, recovery via this technique is never a total 
return to normal. 

There is a broad range of severity in peripheral 
neuropathies. The effects of motor nerve involvement 
may vary from only very mild and barely perceptible 
weakness to profound and total paralysis. Sensory 
involvement may range from mild feelings of 
numbness to complete loss of sensation. Usually in 
peripheral neuropathy, the further away part of an 
extremity is from the central nervous system, the more 
severe the disorder. Thus, sensory changes and 
weakness may be major in the hands, but less marked 
at the elbows and shoulders, and similarly, major in 
the feet and ankles, but mild at the knees and hips. The 
latter is not entirely true in the case of mononeuritis 
and mononeuritis multiplex. 



Peripheral Neuropathies 103 



Various terms are used to describe sensory 
symptoms. Anesthesia refers to complete loss of all 
sensation. Hypalgesia and hypesthesia are reductions in 
the sensations of pain and touch, respectively. 
Hyperesthesia is an abnormally increased sensitivity of 
the skin, which is often annoying. Paresthesia is a feeling 
of tingling, or "pins and needles." Further, 
continuous pain can occur, ranging in intensity from 
very minor to very excruciating pain which greatly 
interferes with the patient's behavior. 

With regard to motor symptoms, weakness is the 
subjective complaint made by the patient. Partial loss 
of strength (paresis) or total loss (paralysis) are the signs 
detected by examination. Atrophy, a reduction in the 
mass of muscle, may also be an associated sign. 

For discussion purposes, the remainder of the 
chapter groups the peripheral neuropathies into two 
classes: polyneuropathy and traumatic neuropathy. 
Under polyneuropathy, the peripheral neuropathies 
associated with hereditary causes and various acquired 
diseases, and those induced by toxins, are included. 
Under traumatic neuropathy, the peripheral 
neuropathies due to nerve injury from external trauma 
or from internal mechanical trauma are included. 
Internal mechanical trauma includes nerve 
constriction by such things as ligaments, bone 
overgrowth, extra ribs, or ruptured discs. 

Polyneuropathy 

Classification. The polyneuropathies may be hered- 
itary, toxic, associated with other known diseases, 
or idiopathic (i.e., primary, but of unknown cause). 

A sampling of the major hereditary neuropathies 
includes: 

1. Charcot-Marie-Tooth disease 

2. Dejerine- Sottas disease 

3. Hereditary sensory neuropathy 



4. Friedreich's ataxia 

5. Spinocerebellar degenerations of adulthood 

6. Familial spastic paraplegia 

7. Refsum's disease 

8. Acute intermittent porphyria 

Since the diagnosis may simply list these names and 
not actually say "peripheral neuropathy," the 
counselor should be aware that when such diagnoses 
are given, possible disabling consequences of 
peripheral neuropathy should be anticipated. 

Table 7-1 lists the toxic causes of peripheral neuropathy: the 
heavy metals, drugs (including, in addition to the 
prescription type, two of the "street drugs"), and 
those organic compounds (some of which are used in 
industry) capable of inducing peripheral neuropathy. 

There are a number of diseases associated with peripheral 
neuropathy. These include: 

1 . Diabetes mellitus 

2. Alcoholism 

3. Chronic renal insufficiency 

4. Cancer 

5. Rheumatoid arthritis 

6. Sjogren's syndrome 

7. Scleroderma 

8. Systemic lupus erythematosus 

9. Hypothyroidism 

10. Polyarteritis nodosa 

1 1 . Sarcoidosis 

12. Chronic liver disease 

13. Thermal burns 

14. Diphtheria 

15. Leprosy 

16. Herpes zoster 

17. Vitamin Bl, B2, B6, and B12 deficiency 

18. Postgastrectomy state 

Several of these diseases, namely, rheumatoid 
arthritis, alcoholism, diabetes, and cancer are 
discussed in chapters 14, 17, 24, and 28, respectively. 



TABLE 7-1 

toxic Causes of peripheral neuropathies 



Heavy Metals 



Organic Compounds 



Drugs 



Lead 

Arsenic 

Thallium 

Mercury 

Antimony 

Gold 



N-Hexane 
Acrylamide 

Tri-ortho-cresyl phosphate 
Methyl-butyl-ketone 
Carbon disulfide 
Carbon monoxide 
Dichlorophenoxyacetic acid 



Nitrofurantoin (Furadantin) 

Diphenylhydantoin (Dilantin) 

Vincristine 

Isoniazide 

Dapsone 

Corticosteroids 

Sodium cyanate 

Halogenated oxyquinoline derivatives 

Hydralazine 

Chloramphenicol 

Disulfiram (Antabuse) 

Sulfonamides 

Heroin 

LSD 



104 Goodgold 



Familiarity with this list of diseases associated with 
peripheral neuropathy will alert the counselor to the 
fact that clients with these diseases may also have 
peripheral neuropathy as one of the complications. 
The four idiopathic neuropathies are: 

1 . Guillain - Barre syndrome 

2. Chronic polyradiculoneuropathy 

3. Fisher's syndrome 

4. Shoulder girdle neuritis 

Of these, Guillain - Barre syndrome (see chap. 6) and 
shoulder girdle neuritis are the most important. 

Peripheral neuropathy of unknown cause may later 
be found to be associated with cancer. Cancer 
apparently may produce derangement in the 
biochemistry of the nerves. The neuropathy may 
appear before the cancer itself becomes obvious. 
Cancer can, however, more directly and on a 
mechanical basis produce a mononeuropathy or 
mononeuropathy multiplex. Tumors growing near 
nerves may compress them directly and thus block the 
conduction of impulses, or the tumors might interefere 
with the blood supply to the nerves, causing nerve 
damage. In some instances, invasion into nerves by 
malignant cells can occur. 

While primary tumors of the axon are rare, tumors 
arising from myelin sheaths do occur and can produce 
neuropathies by compressing the axons. Neurofibro- 
matosis, or von Recklinghausen's disease, is an 
example of a tumor arising from myelin sheaths. It is a 
congenital disorder characterized by the presence of 
multiple tumors, which can appear in almost all nerves 
or segments of nerves and produce neuropathy if the 
tumors are not removed. The disease is associated with 
other signs and symptoms affecting, for example, skin 
and bone. 

Prognosis. The hereditary neuropathies generally get 
progressively worse with time, but the time course of 
these diseases is very slow, and the counselor should be 
aware of the expected progression when planning with 
his client. Acute intermittent porphyria, however, is 
not relentlessly progressive, but is rather inclined to be 
stationary once it occurs, or to improve with time. 
Central nervous system impairment is associated with 
some of the hereditary neuropathies. When a client has 
a hereditary neuropathy, the counselor may need to 
find out if brain damage is part of the syndrome to 
determine whether any intellectual and behavioral 
changes are to be expected. 

The toxic neuropathies usually have very good 
prognoses after the offending agent has been removed. 
As already indicated, however, it is rare to have total 
regeneration of nerves back to normal, and hence some 
disability may remain. 

The prognosis for the peripheral neuropathies 
associated with the diseases listed on page 103 depends 
in great part on the success of the primary disease 
treatment. This is particularly true for diabetes, 



alcoholism, chronic renal disease, and the vitamin 
deficiencies. To assist planning, the counselor should 
obtain advice regarding prognosis of the disease - 
related peripheral neuropathies from the client's 
physician. 

The prognosis of the idiopathic neuropathies varies. 
In Guillain- Barre syndrome, if normalcy does not 
return within 3 to 4 months after onset, patients will 
not fully recover and will have residual disability but 
no progression. In shoulder girdle neuritis, the 
neuropathy will not progress, but at the same time will 
not necessarily improve. The prognosis of chronic 
polyradiculoneuropathy varies greatly and must be 
considered on an individual basis. 



Traumatic Neuropathy 

Classification. Three types of injuries make up the 
traumatic neuropathies: compressions, tractions 
(stretching), and transections, as may occur in a knife 
wound. The symptoms are determined by the site and 
severity of the injury. They may be minor, as in the 
case of a mild compression, or major, as in the case of a 
severe traction or complete transection. 

In the traumatic neuropathies, the condition of the 
nerve is described in any one of three ways depending 
upon the severity of the injury. The least serious lesion 
is neurapraxia. The nerve axon is still anatomically 
intact but there is a block to the conduction of 
impulses. Removal of the pressure causing the 
neurapraxia will result in a return of conduction in 1 to 
2 months with complete recovery. Axonotmesis refers to 
the condition of a nerve which, while still anatomically 
intact, has profound axonal degeneration. Removal of 
the compression inducing the degeneration will result 
in partial recovery as regeneration of the axon occurs. 
A traumatized nerve may initially show a neurapraxia, 
but if the lesion is not treated soon enough, progression 
to axonotmesis may occur. In neurotmesis, a complete 
severance of the nerve has occurred. Repair and 
recovery requires first suturing the nerve ends 
together. Regeneration past the suture down the distal 
portion of the nerve then follows. 

Compression or entrapment neuropathies may 
occur in any region where nerves pass through 
apertures formed by bone, tendon, muscle, and other 
soft tissue. For example, entrapment of a portion of the 
brachial plexus (see p. 27) may take place as the 
nerves forming the brachial plexus descend at the base 
of the neck into the upper extremity. The nerves here 
pass through a narrow outlet (the thoracic outlet) in the 
region of the first rib and the clavicle where they may 
be compressed. Accessory congenital ribs or 
malformed cervical vertebrae may reduce the volume 
of this outlet and also result in compression. 

The most well-known compression or entrapment 
neuropathy involves the median nerve at the wrist 



Peripheral Neuropathies 105 



where it passes through a tight tunnel to serve the 
muscles of the thumb, and skin of the thumb, index, 
and middle fingers. This tunnel is referred to as the 
carpal tunnel, and when the median nerve is 
compressed, the term "carpal tunnel syndrome" is 
used. The incidence of carpal tunnel syndrome with 
minimal obstruction in the tunnel is associated with 
other diseases which apparently make the nerves more 
susceptible to damage. For example, roughly 20 
percent of the patients with hypothyroidism are prone 
to developing carpal tunnel syndrome. 

An exhaustive review of each of the signs and 
symptoms associated with the various different nerve 
damages that can occur in compression, traction, or 
transection is beyond the scope of this discussion. The 
reader may refer to tables 3-1 (p. 19), 3-2 (p. 20), 
3-3 (p. 20), and 5-1 (p. 65) for additional 
information regarding specific nerves and the sensory 
and motor consequences of their injury. A brief 
overview of the sites of damage (spinal nerves, 
plexuses, and distal nerve trunks) is useful, however. 

If a nerve lesion involves the spinal nerve roots, a 
radiculopathy is said to be present. A herniated 
intervertebral disc, spondylolisthesis, and some forms 
of degenerative disc disease (see chap. 15) may give 
rise to characteristic symptoms, depending upon the 
spinal nerve involved. 

Plexuses are formed by combinations of spinal nerve 
roots in the upper and lower extremity. The brachial 
plexus in the upper extremity and the lumbosacral 
plexus in the lower extremity are the two major 
combinations. Traumatic neuropathy involving a 
plexus causes multiple muscle and sensory symptoms 
corresponding to the part of the plexus damaged. The 
plexuses redivide to the final distal nerve trunks, the 
major ones being the median, ulnar, and radial nerves 
in the upper extremity, and the sciatic, femoral, 
peroneal, and tibial nerves in the lower extremity. The 
specific disabilities that result depend upon which of 
the distal nerves are involved. 

Prognosis. Compression injuries have the best 
prognosis, for if the compression is recognized and 
removed early, recovery may be practically complete. 
It is rare for compressions to produce total paralysis or 
total loss of sensation because the patient will usually 
consult his physician before the problem has gone that 
far. 

Traction injuries do not have a good prognosis. 
Severe stretching can disrupt the nerve and cause it to 
become quite scarred and greatly inhibit the likelihood 
of regeneration. Further, traction injuries are 
associated with chronic pain more than the others. 

Cleanly sectioned nerves have a better chance for 
recovery if the nerve is sutured together, although 
recovery is never complete. The longer the segments 
through which the regenerating nerve must grow after 
suture, the less complete the recovery. 



Complications 

The major potential complications of peripheral 
neuropathy are contractures, causalgia (reflex 
sympathetic dystrophy), and osteoporosis. 

Contractures. In peripheral neuropathy, contractures 
are secondary to profound weakness and paralysis. 
If the joints associated with the involved muscles 
do not receive appropriate range - of- motion exercises, 
muscle and ligament shortening may occur, leading to a 
limitation of joint motion and added disability. Pre- 
vention of contractures and measures for treating 
them when they have occurred are discussed in 
chapter 4. 

Causalgia. The disabling condition in causalgia is 
pain, which is generally intense, severe, burning, and 
paroxysmal. It is precipitated by minor stimuli, 
including even the pressure of clothing or a slight 
breeze. The intensity may range from extremely 
disturbing to frankly intolerable and can precipitate 
chug abuse. The process is generally confined to the 
extremities, most commonly only one. Causalgia is 
more likely to occur in the traumatic neuropathies, 
particularly in those caused by traction. The limb is 
usually swollen, cold, and sweaty. The skin may be 
shiny, the joints contracted, and the bones 
osteoporotic. Exquisite tenderness of the limb may be 
present, causing the patient to avoid minimal contact. 
Severe fear and emotional anxiety are generally 
associated. 

The cause of causalgia is frequently a severe intial 
trauma to a major nerve. This event triggers a massive 
outflow of impulses from the sympathetic nervous 
system to produce most of the signs and symptoms. 
Treatment is difficult and consists of interruption of 
the sympathetic outflow with local anesthesia, certain 
drugs, and sometimes surgical excision of the 
sympathetic ganglia associated with the limb. In 
addition, the original lesion is treated, and the limb is 
remobilized through physical therapy. 

Osteoporosis . In peripheral neuropathy, osteoporosis 
may develop if there has been a profound paralysis of 
the muscles of the extremity. The paralysis results in 
reduced tension on the bones, which in turn causes 
calcium to dissolve out of the bone. Chapter 4 deals 
with some of the secondary problems that can result 
from osteoporosis. 



FUNCTIONAL DISABILITIES 

Physical Disabilities 

A peripheral neuropathy involving the nerves of the 
lower extremities, with the production of weakness and 
sensory loss, will cause ambulation impairments. Level 
ambulation, stair climbing, and rough -ground 
negotiation may be affected. Sensory losses will affect 



106 Goodgold 



balance, for the patient will not receive adequate 
messages concerning the location and position of his 
limbs. Negotiation is particularly difficult in the dark, 
since the patient has no visual input to guide him. 

Peripheral neuropathic involvement in the upper 
extremities, with weakness and sensory loss in the 
hands, may interfere with dressing and eating skills, 
and, if sufficiently severe, with personal hygiene and 
writing skills as well. Fortunately, rehabilitation 
techniques can reverse these physical disabilities, even 
if the peripheral neuropathy is unchanged, through 
appropriate training, technique instruction, and 
adaptive equipment. 

Psychosocial Disabilities 

Patients with peripheral neuropathies are relatively 
free of psychosocial disabilities unless the neuropathies 
are associated with some of the diseases listed on page 
103. Chapters 14, 17, 24, and 28 discuss the 
psychosocial impairments that may be associated with 
four of these primary diseases. 

Pellagra (vitamin B2 deficiency), porphyria, and 
some of the hereditary peripheral neuropathies also 
affect brain function. Variable cognitive and 
personality problems may therefore appear and be in 
need of evaluation. Further, in severe peripheral 
neuropathy with extensive nonreversible sensory and 
muscle function loss, problems of dependency and 
depression, and family, social, and financial upheaval 
may also be present, similar to those associated with 
spinal cord injury (see chap. 5). 

Rehabilitation Potential 

As a general rule, rehabilitation potential is very 
good for persons with peripheral neuropathies, and 
long-term planning in most instances can be 
considered. Except for those associated with the major 
diseases, peripheral neuropathies are inclined to be 
stable or to improve, rather than progressively worsen. 
Hereditary neuropathies may progress, but slowly 
enough to allow for planning programs of 2 or more 
years in duration. 

STANDARDS OF EVALUATION 

Peripheral neuropathies are best evaluated by a 
neurologist and/or a physiatrist, although internists 
with special interest in nerve disease may also perform 
the evaluation. If any of the diseases listed on page 103 
are present, evaluation by an internist would be 
essential. 

The evaluation includes a search for hereditary 
factors, a search for exposure to toxic substances, and 
specific examination of the nervous system. Careful 
testing of muscle strength, sensory function, and 
reflexes is included. Special instruments are sometimes 



used to quantify the sensations of touch and pain. Von 
Frey hairs, of various thicknesses, can record the 
pressures required for touch to be perceived. An 
algesimeter measures pain sensation by recording the 
force, when applied to a fine steel needle directed at the 
skin, that results in the perception of pain. 

Since peripheral neuropathy may be the first 
manifestation of some of the diseases listed on page 
103, certain basic tests may also be done to search for 
the primary disease entity. Muscle and nerve biopsies 
may be required if the diagnosis is unclear. In the 
traumatic neuropathies, consultation by a 
neurosurgeon or orthopedic surgeon is usual. 

In the detailed diagnosis of the nerve involvement in 
peripheral neuropathy, electromyography and nerve - 
conduction studies, usually performed by a physiatrist 
or a neurologist, are important. In electromyography, 
a needle electrode is placed in the various muscles 
whose nerves are suspected of involvement and the 
electromyographer looks at the nature of the electrical 
activity when the muscle is at rest and when it is 
actively contracting. Axonal disease is suspected if 
special electrical potentials are present when the 
muscles are at rest and there is abnormal electrical 
activity during motion. Nerve-conduction studies 
examine the speed with which messages travel in 
nerves. The examiner electrically stimulates the nerve 
and records how fast the electrical impulse travels 
down the length of the nerve. For the long nerves, the 
examiner can determine the speed of conduction in 
different segments. If the speed of conduction is 
markedly reduced, demyelinating disease is suspected. 
If it is reduced only in segments, segmental 
demyelinization is suspected. Electrical stimulation of 
nerves can also help evaluate the reflexes and hence the 
character of the spinal nerve roots (see p. 26). So- 
called H- reflex and F-wave studies are helpful in this 
regard. Electromyographic and nerve -conduction 
studies are not only helpful in establishing the 
definitive diagnosis with regard to the location, nature, 
and site of the disease, but they are also useful in 
following the course of treatment and in determining 
prognosis. 

In addition to these medical evaluations, evaluations 
by a physical therapist and an occupational therapist 
are helpful. The physical therapist can assess 
ambulation and transfer skills and the occupational 
therapist can assess whether eating, dressing, and 
personal hygiene difficulties are present and, if so, how 
they might be reversed. Where weakness, particularly 
in the legs and the hands, interferes with certain skills, 
an orthotist may assist by fabricating braces to restore 
function. 

Since most peripheral neuropathies are likely to be 
self- limiting and not very progressive, psychological 
evaluation and family and social service review may 
not be necessary. However, in conditions that might 
affect central nervous system function, review of 



Peripheral Neuropathies 107 



intellect and personality by a psychologist may help the 
counselor with planning. Certainly, severe disability, 
with its potential family, financial, and social 
disturbances, may warrant social service review. 
Finally, patients with disease -related peripheral 
neuropathies may require psychosocial review for 
problems associated with the disease, rather than with 
the peripheral neuropathy itself. 

TOTAL TREATMENT 

Polyneuropathy 

There is no specific treatment that reverses the 
neuropathy in the hereditary disorders. Exposure to 
toxic agents and trauma must be avoided, and 
nutrition and vitamin B intake must be adequate, since 
patients with hereditary neuropathies may be more 
susceptible to insults from toxins, trauma, and vitamin 
deficiencies. 

In addition to removing the patient from exposure to 
the agent, specific therapy for the toxic neuropathies 
includes the administration of detoxifying substances 
designed to remove the poison from the system. 
Examples are penicillamine for lead and mercury, and 
the chemical British anti - lewisite (BAL) for arsenic. In 
addition, specific antidotes also exist for the organic 
compounds. 

Specific therapy for the peripheral neuropathies 
associated with the diseases listed on page 103 is 
basically the primary treatment of the disease itself. 
Thus, the best treatment for diabetic neuropathy is 
good diabetic control; for chronic renal insufficiency, 
appropriate dialysis or kidney transplantation; and for 
hypothyroidism, treatment with thyroid medication. 

For the idiopathic neuropathies, in particular 
Guillain-Barre syndrome and chronic polyradiculo- 
neuropathy, corticosteroid treatment has been 
introduced and is considered helpful. Hospitalization, 
bed rest, and analgesics for pain control usually are 
required for the severe acute -onset neuropathies. 



Traumatic Neuropathy 

Surgery is the specific treatment for the traumatic 
neuropathies, particularly those associated with 
compression. Neurolysis refers to the surgical freeing of 
a nerve from encapsulating and constricting 
neighboring ligaments or adhesive bands. Suturing cut 
nerve ends together is called neurorrhaphy. If a nerve is 
moved to a less constricting location, it is referred to as 
a translocation. 

Rehabilitation Treatment 

Rehabilitation measures include prevention of 
unnecessary additional disabilities. Contractures are 
prevented by appropriate range -of- motion exercises 



performed for and by the patient. Exercise activities to 
strengthen both weak and uninvolved muscles, and 
training in the appropriate use of canes and crutches 
help restore ambulation and transfer functions. In 
severe lower extremity paralysis, short or long leg 
braces might be prescribed to improve standing 
endurance and walking. Treatment by an occupational 
therapist usually eliminates any impairments in eating, 
dressing, and personal hygiene skills through 
appropriate techniques and adaptive equipment. In 
most instances, writing and driving difficulties can also 
be overcome. Orthoses and splints may also be used 
when there is profound paralysis to maintain a joint in 
its functional position in order to prevent deformity. If 
joint deformity has already occurred, certain orthoses 
may need to be prescribed to correct it. In addition, 
there are orthoses that can assist function when 
weakness is present. They are designed to be used 
while the function is being performed, and are referred 
to as dynamic or kinetic devices. 

Electrical stimulation of paralyzed muscles on a 
regular basis can retard atrophy, although it cannot 
accelerate recovery. If recovery is expected in a 
reasonably short time (e.g., within 4 to 6 months), an 
electrical stimulation program to be carried out by the 
patient at home may be prescribed. Trancutaneous 
electrical stimulation is also sometimes helpful in the 
control and alleviation of pain, especially in herpes 
zoster and in some cases of causalgia. 

The need for psychosocial treatment, such as 
ongoing care by a psychologist or social worker, is 
usually only required for patients with the more 
profound paralyses, as discussed in chapter 5. 

Followup Treatment 

Medical followup after the initial treatment is 
generally dictated by the condition involved, and 
usually includes monitoring changes in status, and 
perhaps modifying treatment. The hereditary 
neuropathies might not need followup more than once 
or twice a year. The followup required for disease - 
related peripherial neuropathies is dictated by the 
primary disease, rather than the peripheral 
neuropathy. In the toxic neuropathies, once the 
offending agent is removed and specific treatment is 
instituted, followup is usually not required after 1 year 
unless there is persistent paralysis and observation is 
required to avoid complications. The idiopathic 
neuropathies require closer followup during the period 
of active treatment. Some patients may be seen once a 
month, or more often, until full stabilization occurs, 
after which followup can be less frequent. 

VOCATIONAL IMPLICATIONS 

Since residual physical impairments in muscle and 
sensory function may vary so widely in location. 



108 Goodgold 



quality, and degree, the rehabilitation counselor needs 
to obtain rather detailed and accurate information 
from the client's physician, and perhaps from the 
counselor's medical consultant, regarding etiology, the 
extent of functional disabilities, the efficacy of various 
treatments, and the prognosis. Further, the coun- 
selor should verify that his client has had access 
to all possibilities for improving function, such as 
surgery and the various treatment modalities of physical 
rehabilitation. 

Although the extent of disease in peripheral 
neuropathies varies greatly, the following general 
statements may serve as a useful baseline. 

Education 

Peripheral neuropathy does not generally limit long- 
term planning, such as extended education or specific 
vocational training for 2 or more years. Education 
planning may have to be modified if the client has a 
peripheral neuropathy secondary to one of the diseases 
listed on page 103. In this case, the primary disease 
will determine whether short-term goals will be more 
appropriate. 

Aptitudes 

Intellect, learning ability, and verbal and numerical 
skills may be partially impoverished in those conditions 
with central nervous system effects, such as pellagra, 
alcoholism, and porphyria. In these situations, careful 
aptitude and intellectual testing may be necessary. 
Finger and hand dexterity, eye-hand coordination, 
and eye-hand-foot coordination difficulties may result 
from a sensory impairment in the hands and feet or 
from weakness in the hands and lower extremities. A 
client with sensory impairment may need to have his 
hands in constant view during manipulation. This may 
prevent activities that require direct observation of the 
object being manipulated, as well as hand activities 
that are out of sight. Jobs that may involve hand 
dexterity and eye -hand -foot coordination should be 
carefully reviewed to ensure that performance is 
possible for the client. 

Interests 

In peripheral neuropathy, a clash between the 
client's interests and his level of physical function is 
unlikely, except when interests are in activities within 
the medium and heavy work categories. Such tasks 
may exceed the strength and endurance of the client 
who may, however, handle light and sedentary 
activities quite well. 

Physical Demands 

Peripheral neuropathy in the legs, with or without 



obvious weakness, may mean that the client will have 
difficulties with jobs requiring extensive ambulation, 
standing, lifting, and carrying. Balancing, stooping, 
and kneeling may also have to be avoided. For some, 
sensory impairments in the legs may require sedentary 
and indoor work. Indoor work may also be required if 
the client's ambulation is impaired on uneven, rough, 
or hilly ground. Even if leg function is normal and 
neuropathy is confined to the upper extremities, the 
client may be unable to handle more than light work 
because pushing, pulling, and carrying requirements 
may exceed his upper extremity limits. Motor or 
sensory leg and arm impairments will interefere with 
climbing. For some clients, relatively minor sensory 
deficits in the upper extremities, even with good 
muscle function, may impair fine manipulation 
sufficiently to require occupational adjustment. 

Environment 

A client with reduced sensation may be unable to 
appreciate excessive heat, with the result that 
accidental burns may be possible if he works near 
flames or hot objects. Employment environments 
containing any of the toxic agents that can contribute 
to neuropathy, such as lead, arsenic, or organic 
compounds, should be avoided. 

Hot and cold climatic extremes may aggravate the 
pain of causalgia, and should be avoided by clients 
with this complication. 

In general, the rehabilitation counselor should find 
relatively easy success and rewarding outcomes when 
working with the client with peripheral neuropathy. 
The nature of the disability is rather clear and distinct, 
the condition is relatively stable, physical restoration 
possibilities are good, and complicating psychological 
and social problems are generally minimal. These 
factors, coupled with careful communication with the 
treating physicians involved, should allow the 
counselor to effectively plan with his client. 

BIBLIOGRAPHY 

Brain, Lord: Brain's Diseases of the Nervous System. Ed. 8, revised bv 
Walton JN. Oxford, Oxford University Press, 1977. 
Complete text on the nervous system, with a well -written 
section on peripheral nerve diseases. 

Dyck PJ, Thomas PK, Lambert EH (eds): Peripheral 
Neuropathy. Philadelphia, Saunders, 1975. 
Most recent exhaustive two- volume consideration of 
almost every aspect of peripheral nerves, e.g., anatomy, 
physiology, pathology, and therapy. 

Haymaker W, Woodhall B: Peripheral Nerve Injury. Ed. 2, 
Philadelphia, Saunders, 1953. 

Relatively old but excellent book based on extensive 
experience with traumatic lesions. Easily understood, with 
simple review of clinical diagnostic features. 



Peripheral Neuropathies 109 



Kopell HP, Thompson VVAL: Peripheral Entrapment 
Neuropathies. Ed. 2, Huntington, NY, Krieger, 1976. 
Brief but concise review of peripheral neuropathies due to 
compressive lesions. 



8 MULTIPLE SCLEROSIS 



George H. Kraft, M.D. 



DISEASE DESCRIPTION 

Multiple sclerosis (MS) is one of the most common 
neurological diseases in North America and Europe, 
affecting as many as 500,000 persons in the United 
States alone. The disease is two to three times more 
common in the northern half of the United States than 
in the southern half. MS can affect any age, but most 
commonly has its onset between the ages of 20 and 40, 
with a peak incidence occurring around age 30, and is 
more common in females (F:M = 60:40). Because MS 
occurs during the most significant periods of one's 
vocational life, rehabilitation and vocational services 
are particularly necessary. 

Etiology 

The cause of MS is unknown and there are many 
theories as to its cause. A series of new discoveries 
during the past few years suggests that MS may be the 
result of a complex interaction of infection, immune, 
and genetic factors. One such theory suggests that a 
viral infection may precipitate an attack by the body's 
antibody (immune) system on the myelin, the fatty 
substance that sheaths nerve axons (see p. 19). In the 
last few years, many scientists have been studying 
measles (rubeola) and other viruses as triggering 
agents. Other investigators feel that the attack on 
myelin is mediated by different mechanisms. Some 
evidence suggests that there are certain genetic 
predispositions to the disease. 



Pathology 

The lesions of MS may occur in various places in the 
central nervous system (brain and spinal cord), and 
can therefore cause almost any type of central nervous 
system symptom, depending on the location of the 
lesions. It only minimally affects the nerves in the arms 
and legs (peripheral nervous system). The myelin 
sheaths are attacked and destroyed in localized areas 
within the brain and spinal cord. Patches of destroyed 
myelin are later replaced by scar tissue, producing 
lesions known as plaques. These plaques interrupt or 
distort the flow of nerve impulses in much the same 



way that breaks in the insulation on electrical wires can 
cause a malfunction of an electrical system. There is 
evidence that alterations of the nerve impulses may 
also occur before the myelin has been destroyed. 

The symptoms that any one patient with MS may 
develop depend on the location of his specific lesions. 
The lesions may be distributed widely over the 
cerebrum, cerebellum, brain stem, or fiber tracts of the 
spinal cord. When located in the frontal or parietal 
lobes, symptoms of mental and psychological 
deterioration may occur. If corticospinal tracts in the 
cerebrum, brain stem, or spinal cord are involved, a 
spastic paralysis of the extremities may result. 
Similarly, involvement of sensory axons anywhere in 
the spinal cord, brain stem, or cerebrum along their 
path to the parietal lobe may produce numbness. 
Visual disturbances may result from direct 
involvement of the optic nerves or in the optic 
radiations in the occipital lobe. Plaques in the 
cerebellum may produce loss of coordination (ataxia), 
loss of balance, and tremors, particularly in the upper 
extremity when a person moves to grasp a specific 
object. Lesions in the cerebellum and the cerebrum can 
also cause speech and language disturbances. Bladder 
dysfunction may result from lesions distributed 
anywhere within the central nervous system. Not all of 
these symptoms are seen in each MS patient. 

Transiently impaired vision is an early sign in about 
20 percent of patients, and the most common problems 
are double vision (diplopia) or blurring. When these 
symptoms first appear, the physical and 
ophthalmologic examinations may still be normal. 

As the disease progresses, many patients show a 
combination of weakness, spasticity, ataxia, and 
tremor. An inability to normally control bladder 
function is one of the most common, frustrating, and 
difficult problems encountered. Both a difficulty in 
initiating urination and incomplete emptying of the 
bladder (urinary retention) and dribbling 
(incontinence) can occur. Many patients with MS also 
need to urinate often (frequency), are unable to hold 
urine once the sensation to urinate has occurred 
(urgency), and have frequent urinary tract infections. 
Speech problems often occur, with dysarthria much 



11 1 



112 Kraft 



more common that aphasia (see p. 129). Confusion, 
because it also can affect language performance, is 
often misjudged as aphasia. 

Late in the disease, the mental abilities of some 
patients with MS may be affected. Of the various 
mental changes which can occur, euphoria is the most 
common and occurs in 31 percent of patients. 
Intellectual deterioration occurs in 26 percent, lability 
of mood (see p. 133) in 16 percent, depression in 7 
percent, and psychotic episodes in 4 percent. 

One of the characteristics of MS patients in general 
is that they are intolerant of heat and generally feel 
weaker in hot weather or in warm environments. 
Fatigability is another common symptom; patients 
may have good strength to begin a task but fatigue 
rapidly. 

The classical pattern of MS is one of general overall 
progression of symptoms in a pattern of exacerbations 
and remissions. Symptoms become worse 
(exacerbation), then partially resolve (remission), only 
to become worse again in a continuing cycle. An 
exacerbation may be an exaggeration of prior 
symptoms or the appearance of new symptoms due to 
additional lesions in previously unaffected neurologic 
areas. An exacerbation typically lasts 2 to 3 weeks, 
although it can last longer. The average interval 
between exacerbations early in the disease is about 5 
years. Some patients exhibit a continuous progression 
without remission. 

Complications 

The major complications that can occur include (a) 
contractures, which result in limitations of joint range 
of motion; (b) skin breakdown (decubitus ulcers) over 
bony prominences, particularly over back, hips, and 
buttocks; (c) chronic urinary tract infections, resulting 
in compromised renal function; and (d) seizures in a 
small percentage of patients. Although more of an 
exaggerated common MS symptom, excessive 
spasticity may also be considered a complication, for if 
it exists, it makes the prevention of contractures, skin 
breakdown, and urinary tract infection extremely 
difficult. 

The development of contractures may be due to 
weakness or spasticity. Weakened muscles result in a 
joint not receiving its normal range of motion. Muscles 
with spasticity actually prevent the joint from being 
put through a normal range of motion. Contractures 
aggravate the physical disability beyond that caused by 
the lesions themselves. 

Decubitus ulcers develop when numbness or reduced 
sensation is part of a patient's symptom complex. The 
patient is unable to tell when he has been lying or 
sitting for too long without shifting his weight. 
Weakness also contributes to the development of 
decubitus ulcers because it reduces the ability to shift 



weight off the pressure area. With sufficient time, 
continuous pressure on the skin over bony 
prominences reduces the blood supply to the skin. The 
skin then dies and a localized ulcer results. The bony 
prominences over which these ulcers are likely to occur 
are the low back (sacrum), the hips (trochanters), and 
the sitting bones (ischial tuberosities). Ulcers left 
unattended can progress and involve the underlying 
bone to produce osteomyelitis. The infection can also 
enter the bloodstream and create a widespread acute 
infection. 

Urinary tract infections are basically secondary to 
bladder dysfunction, particularly chronic retention of 
urine and incomplete emptying of the bladder. 
Difficulty with perineal hygiene contributes to 
infection due to contamination from the anal area. 

A complete discussion of seizures may be found in 
chapter 12. 

Severe spasticity, particularly in the lower 
extremities, can be devastating. The slightest 
stimulation may cause the legs to involuntarily move 
into postures of flexion of the hip and knee. Severe 
adduction forces can also develop so that separation of 
the legs is difficult to accomplish. The limbs may 
actually cross over (scissoring). Flexion can be severe 
enough to maintain the heels drawn up against the 
buttocks. The resultant inability to sit or lie contributes 
to the possibility of decubitus ulcer development and 
aggravation of bladder, bowel, and perineal hygiene 
problems. 

Prognosis 

MS is an unstable and unpredictable disease. Early 
prognostic indicators that can determine which 
patients are likely to show rapid disease progression 
and which will show only slow progression have yet to 
be determined. However, it should be noted that many 
more patients have a mildly progressive form of MS 
than previously recognized. The vocational counselor 
will see persons who can function relatively normally 
for many years. Severe, rapidly progressive disease 
occurs in fewer than 1 percent of patients and 5- and 1 0- 
year periods between exacerbations are common. A 
significant number of patients are never seriously inca- 
pacitated. 

MS itself does not produce mortality. Mortality is 
due rather to pneumonia or widespread infection 
secondary to decubitus ulcers in advanced disease 
where general debilitation is present. 

FUNCTIONAL DISABILITIES 

Physical Disabilities 

The most common symptom preventing patients 
with MS from working is spasticity. This was 
demonstrated by a comprehensive vocational study of 



Multiple Sclerosis 113 



over 1,200 patients with MS in Germany. Two-thirds 
of the patients who were not working considered 
spasticity of the legs or arms to be the main reason they 
had to drop out of the employment market. 

The combination of weakness, spasticity, ataxia, and 
tremor may interfere with walking and ultimately neces- 
sitate a wheelchair for mobility. 

The use of a cane illustrates the functional problem 
encountered in the rehabilitation of a patient with both 
weakness and tremor. A cane might be very helpful to a 
patient with generalized weakness alone. However, a 
patient with MS might have great difficulty in placing a 
cane properly because of tremor, and consequendy the 
cane might not be an effective ambulation aid. It might 
actually impair walking. A patient with coordination 
problems might need more time to ambulate a given 
distance than a patient with weakness alone. Curbs, 
stairs, and other barriers may present severe problems 
for a patient with both weakness and ataxia. Ambulation 
ouside the home will become increasingly difficult and 
time consuming as the disease progresses. Transfer skills 
are impaired because of incoordination. With ataxia, a 
patient may not have the balance required to get from a 
wheelchair to a bed or a toilet, or in and out of a car. 
With ataxia, a patient may not have the balance 
required to get from a wheelchair to a bed or a toilet, or 
in and out of a car. 

With severe intention tremor, eating skills may 
diminish considerably and the patient may have 
difficulty in getting his hand to his mouth. Similarly, 
persona] hygiene, bathing, and grooming may be very 
difficult for a patient with severe incoordination. 

Speech slurring may reduce the patient's ability to 
communicate effectively. If the patient needs to 
communicate well in his job, this becomes a functional 
impairment as well as a "cosmetic" impairment. 
Because of tremor, handwriting also may be very 
difficult. Visual symptoms may have a deleterious effect 
on reading and driving. 

In some patients, the loss of normal bladder control 
is the main reason for not being able to function 
vocationally, socially, or as a homemaker. Symptoms 
such as urgency and incontinence produce much embar- 
rassment and perceived loss of social acceptability. It is 
important to specifically ask a client whether bladder 
problems occur, since the client may simply withdraw 
from the vocational environment. Withdrawal from work 
because of this is unfortunate, since most bladder 
dysfunction can be improved sufficiendy with appro- 
priate treatment to achieve a socially acceptable system. 

Psychosocial Disabilities 

The specific configuration of intellectual or cognitive 
deficits associated with MS varies with the individual 
case. As in other forms of cerebral insult, the single 
most likely interference will be in the ability to learn 



and retain new information, skills, or procedures; i.e., 
learning rate is impaired, though not necessarily the 
ability to learn. The contours of the intellectual 
impairments are likely to be scattered and diffuse, 
rather than discrete and focal, as often happens with 
stroke (see chap. 10). 

Because of the pattern of exacerbations and 
remissions, the psychological stresses associated with 
MS are variable and unpredictable, rather than 
occurring in a single episode as in a stable disability. 
The MS patient needs to adjust to each new disability 
associated with an exacerbation, and must also adjust 
to the uncertainty inherent in his disease. This 
uncertainty, together with the psychological and 
intellectual effects of the cerebral plaques (emotional 
lability, intellectual deterioration, euphoria, 
depression, and, occasionally, frank psychosis), may 
produce disruption of the patient's social and family 
life and his vocational capabilities. Therefore, the 
disease can have an impact not only on the patient, but 
on family members and job colleagues as well. 

Rehabilitation Potential 

The major problem in determining the rehabilitation 
potential of patients with MS is the uncertainty of the 
future course of the disease. Some patients may have a 
very slowly progressive disease, while others may have a 
very rapidly progressive course. Fortunately, in the ma- 
jority of MS patients, the disease progresses slowly and 
the patient has only a slighdy shortened life expectancy. 
Yet, the combination of weakness, spasticity, and ataxia 
may significantly shorten the period of employability. 

Studies are currently underway to determine early 
vocational prognostic indicators, but they are not yet 
available. At the present time, probably the most 
useful indicator is the past course of the disease. A 
patient who has had only very mild and infrequent 
exacerbations may be expected to continue this pattern 
for a number of years in the future. On the other hand, a 
patient with a rapidly progressive disease course could be 
expected to continue this pattern. Some clinicians feel 
that patients with primarily sensory symptoms (such as 
numbness or tingling) have a better future course than 
those with weakness or ataxia. Consequently, it would 
not be appropriate to plan a program requiring pro- 
longed education and training for a young client with 
rapidly progressive MS. The appropriate plan would be a 
short training course to prepare the client for a vocation 
with minimal physical demands. 

A certain degree of optimism must be retained by 
the vocational counselor when working with a client 
with MS. In a recent vocational study of MS in Israel 
based upon a country -wide survey of patients, only 24 
percent of patients under 50 years of age were 
considered to be completely handicapped with no 
rehabilitation potential. Fifty -five percent were still 



114 Kraft 



working, without the aid of vocational rehabilitation 
services, and 21 percent were in need of these services. 
As might be expected, patients who were working in 
occupations that did not require much physical effort 
were better able to keep their jobs than those in 
occupations requiring physical effort. 

Other studies have shown that the percentage of 
patients still able to work 10 years after the onset of MS 
ranges from 12 to 60 percent. In the comprehensive 
German study mentioned earlier, the overall 
percentage of employment dropped from 71 percent 
after 5 years of disease, to 50 percent after 10 years, 
and 31 percent after 15 years, with no further 
subsequent drop. 

STANDARDS OF EVALUATION 

Medical Evaluation 

The diagnosis of multiple sclerosis is difficult. 
Several different experimental chemical laboratory 
tests have been reported from various medical centers 
as able to diagnose MS, but as yet none is conclusive. 
Currently, therefore, there is no diagnostic laboratory 
test specific for MS, although in the future there may 
be. 

The diagnosis of MS must therefore be made from 
the history. A history of diffuse neurologic symptoms 
(multiple lesions) occurring over a period of time with 
physical examination signs of disease in more than one 
part of the central nervous system are required. 
Therefore, a long interval, usually 5 years, is common 
between the first symptoms and a diagnosis. The 
diagnosis can rarely be made with any degree of 
assurance at the time of the first attack. Even when the 
symptoms have been present for many months or 
years, considerable diagnostic skill is necessary to 
exclude the many conditions that may be simulated by 
MS. 

Diagnostic errors are not uncommon, and it has 
been estimated that approximately 10 percent of the 
patients studied in a major epidemiologic survey were 
misdiagnosed and actually had other neurologic 
diseases. The extensive hospital and laboratory testing 
that may be performed are therefore not done to 
diagnose MS, but to eliminate similar-appearing and 
perhaps treatable diseases. It should be emphasized that 
this is an important undertaking since, at the present 
time, there is no cure for MS. Therefore, treatable 
conditions that mimic MS should be sought. 

The only significant, but nondiagnostic, 
abnormalities among the usual laboratory tests are 
changes in the cerebrospinal fluid. Examination of the 
fluid usually shows an elevation of a certain type of 
protein (gamma globulin) and an absence of an 
increase in blood cells, as well as an abnormality of the 
colloidal gold curve test. In this test, cerebrospinal 
fluid dissolved in varying concentrations of salt 



solutions is mixed with a colloidal suspension. When 
the fluid has increased protein, generally in the gamma 
globulin fraction, there is a change in color of the 
suspension. The dilutions in which this change is noted 
define a positive test. This test is often positive in MS. 
Other types of neurologic conditions may produce 
similar findings, and some MS patients show no spinal 
fluid abnormalities. Therefore, spinal fluid tests are 
helpful but not infallible in confirming the diagnosis of 
MS. 

MS is so variable that it is not possible to set forth in 
detail tests which should be authorized to rule out the 
possible presence of treatable disease. As a minimum, 
a 2- to 3-day hospitalization is appropriate for the spinal 
fluid examination and other tests, such as X-rays of the 
spine, a myelogram, and an electromyogram. In some 
cases, depending on the types of symptoms, other tests 
must also be done. For some patients, such an evaluation 
may be conducted in a number of outpatient clinic visits. 
These decisions should be based on the judgement and 
experience of a competent neurologist. 

Very recently, several new electrophysiologic 
techniques, such as visually evoked cerebral potential, 
auditory evoked cerebral potential, the blink reflex, 
and electrospinogram, have been developed. They 
appear to be positive in the 75-95 percent range when 
used to test portions of the central nervous system 
affected by MS. These tests can be obtained in only a few 
research institutions and are not available in most 
hospitals at the present time. At their current stage of 
development, they would not be appropriate for making 
a diagnosis of MS. In the future, however, these tests may 
be developed sufficiently to be very useful. 

If a visual disturbance is present, evaluation by an 
ophthalmologist may be necessary. Speech 
disturbances should be evaluated by a speech 
pathologist who will determine the type of dysarthria 
and recommend suitable therapy. A urologist may be 
necessary to evaluate bladder dysfunction. 
Consultation by a neurosurgeon may be suggested to 
determine whether mechanical pressure on the spinal 
cord exists rather than MS. 

When impairment in ambulation and perhaps other 
physical disabilities occur, evaluation by a physiatrist 
and a comprehensive rehabilitation team is 
appropriate. Such an evaluation is particularly 
appropriate when speech, vision, and the urinary 
system are also involved and psychosocial problems are 
present or impending. In a comprehensive 
rehabilitation setting, physical and occupational 
therapy assessment and multidisciplinary review can 
produce the best evaluation for total treatment and 
vocational planning. 

Psychosocial Evaluation 

Administration of the Reitan test battery by an 



Multiple Sclerosis 115 



appropriately trained psychologist to determine the 
patient's intellectual abilities, learning abilities, verbal 
skills, numerical skills, form and space perception, 
eye -hand coordination, and color discrimination may 
be useful to the counselor, both to identify subtle 
higher level intellectual impairments otherwise 
overlooked and to help him and others working with 
the patient to direct their rehabilitation efforts in light 
of cognitive/intellectual impairment. It should be 
remembered, however, that these tests will determine 
present dysfunction and, in a progressive disease such 
as MS, future performance must be couched in 
probabilities. 

A psychiatrist or qualified psychologist should also 
evaluate the patient to detect the presence of emotional 
lability, euphoria, depression, or the possibility of 
psychosis. In addition, the patient's adjustment to his 
disabilities and to the uncertainty inherent in MS 
should be assessed. 

Evaluation of the family unit by a social worker is 
important to detect problems caused by the patient's 
disability and by the recurring stress associated with 
the pattern of exacerbations and remissions. 

TOTAL TREATMENT 
Medical Treatment 

There is no treatment to prevent MS, to prevent 
progression of the central nervous system lesions, or to 
prevent new lesions from developing. Furthermore, 
there is no conclusive proof that any medicine can alter 
the plaques. However, some physicians believe that 
ACTH (adrenocort icotrophic hormone) or 
corticosteroids (e.g., prednisone) can reduce the 
severity of exacerbations. Many patients receive such 
treatment. ACTH must be given by injection into a 
muscle at intervals up to twice daily or by continuous 
infusion into the veins. Long- acting preparations are 
also available which can be injected every few days. 
Intramuscular injection of ACTH must be given by a 
nurse or physician, and consequently require many 
clinic visits. Medicine given into the veins requires 
hospitalization. Other treatments, such as fat -free 
diets, sunflower seed oil, and vitamin supplements are 
not considered to be efficacious. 

Some of the disabling symptoms can be reduced in 
intensity. For example, an intention tremor that 
seriously complicates the rehabilitation of a patient 
with MS may be managed by a neurological 
procedure. Surgical removal of an area of the brain 
(the ventrolateral thalamus) has been effective in some 
MS patients in reducing this tremor. This is a major 
operation carrying serious potential risks (e.g., 
hemiplegia) of its own. The risks must therefore be 
carefully weighed against the potential benefit before it 
is performed. To date, success has been variable. 

Profound fatigue may be especially marked in the 



early afternoon. Mild central nervous system stimulants 
such as caffeine, Ritalin, or dextroamphetamine may 
be tried with occasional beneficial results. 

Double vision can often be managed by patching 
one eye. 

Treatment of Complications 

Contractures . The joints associated with weakened 
and spastic muscles need to be carried through a 
passive range of motion daily to prevent or delay the 
development of contractures. In the early stages of the 
disease, the patient may be able to do this himself, but 
in later stages he may require the assistance of a family 
member. If the weakness or spasticity is severe, the 
assistance of a physical therapist will be needed. 

The best physical therapy technique for treating 
mild contractures is deep heat combined with 
prolonged static stretch. When contractures are severe, 
they are disabling and cannot be controlled by 
conservative physical therapy treatment. In such cases, 
surgical correction by an orthopedic surgeon may be 
indicated. 

Decubitus ulcers. Patients should shift their weight at 
least every hour when sitting and every 2 to 4 hours 
when lying to help prevent the development of 
decubitus ulcers. For wheelchair patients, special 
cushions, particularly those of a gel consistency, are 
helpful. They may not, however, prevent ulcer 
development if weight shifting does not occur. When 
ulcers are impending or have developed, removal of all 
pressure over the site and meticulous cleaning of the 
wound until it is healed are required. If the ulcer is 
extensive, surgical repair is necessary (see p. 417). 
When infection is present in the wound, antibiotics 
may be needed. 

Bladder dysfunction and urinary tract infection. 
Management of bladder dysfunction and urinary tract 
infection requires the careful assistance of a urologist. 
Bladder dysfunction, particularly retention, is the 
precipitating event that allows infection to develop in 
the urinary tract. 

Urinary frequency and urinary urgency are often 
effectively treated with the drug Probanthine or other 
agents blocking the parasympathetic nervous system to 
the bladder. For retention, agents such a Urecholine 
that increase the force of the bladder contraction and 
agents or techniques that reduce the resistance at the 
sphincter can be helpful. Medication that reduces 
spasticity (see below) may also affect and help the 
urinary sphincter if it too is spastic. Urological surgical 
procedures such as sphincterotomy, which cuts the 
sphincter muscle, or transurethral bladder neck 
resection (TUR) in male patients may be effective to 
provide a successful bladder. Where urgency is severe 
but retention is minimal in the male, a condom 
collecting system (see p. 76) may help produce a 



116 Kraft 



socially acceptable system. When severe urgency and 
minimal retention occur in females, there may be no 
option but a permanent indwelling catheter. 

When urinary tract infections occur, the offending 
organism is identified and an appropriate antibiotic 
given. Simultaneous treatment of the bladder 
dysfunction is necessary to prevent recurrence. 

Seizures. The drug management of seizures is 
discussed in chapter 12. 

Spasticity. Oral medications, in particular Valium, 
Dantrium, and, more recently, Lioresal, are useful in 
the management of spasticity. These must be taken on 
a regular basis to control the spasms. Possible liver 
toxicity with Dantrium requires ongoing monitoring 
with liver function tests. Where one or a few offending 
muscles can be identified, spasms in these muscles may 
be managed by phenol motor point blocks (see p. 75), 
usually performed by a physiatrist or anesthesiologist. 

If such measures are not effective, more major 
surgical procedures, such as injecting chemicals like 
alcohol or phenol directly into the spinal cord, cutting 
of spinal cord roots, or cutting the muscles from their 
points of attachment or insertion, can be dramatically 
effective. Recently, surgical implantation of electrical 
stimulators in the posterior columns of the spinal cord 
has been advocated as effective in the control of spas- 
ticity, but as yet the experience with this technique is 
limited. These procedures are only considered after the 
more conservative, less destructive procedures have been 
tried, and are usually performed by a neurosurgeon or an 
orthopedic surgeon. 

Rehabilitation Treatment 

A rehabilitation program must maximize 
ambulation and transfer skills in particular. As a 
general guideline, any patient falling more than twice a 
month needs an ambulation aid. In a patient who has 
severe ankle spasticity unrelieved by treatment with 
medications or motor point block, immobilization of 
the ankle with a brace may be the treatment of choice 
to maintain walking. This can be accomplished with 
either a metal or plastic brace, and may also require a 
special type of shoe incorporating a lateral "T- strap" 
to prevent the ankle from turning in. Knee instability 
can be treated by the use of two canes or, if hand 
function is poor, forearm crutches. Long leg braces are 
less desirable since they lock the knee and make 
walking difficult. 

In patients with weakness, ataxia, and tremor, a 
weighted walker may be the only device that will 
provide sufficient stability for walking. A wheelchair 
might also be needed to provide functional 
ambulation. Often a patient will need a wheelchair for 
prolonged periods of mobilization and a cane for short 
periods. Skill in the use of a cane in a patient who can 



only ambulate over limited distances without a 
wheelchair improves his ability to be mobile in areas 
that are not wheelchair accessible. 

In late stages of the disease when weakness is more 
pronounced, the patient may need devices to assist in 
eating and dressing. An occupational therapist may be 
needed to help the patient learn techniques for 
dressing, reading, writing, and personal hygiene. In 
general, the patient can be taught strategies to carry 
out these activities, although they may take 
considerably longer than normal to perform. 

Management by a speech pathologist may be 
necessary for patients with swallowing difficulty and 
problems of articulation, including the common 
slurred speech. The speech pathologist will assist the 
patient in the use of compensatory systems for 
communication. 

Psychological management may be required to help 
the patient deal with depression or denial. In addition, 
there may be a need for social case work with the 
patient and his family during various periods in 
the course of the disease to deal with the continued or 
recurring psychological stress associated with the 
severe problems occurring over a long period of time. 

Patients with a number of physical impairments and 
associated psychosocial or speech problems are best 
managed in a comprehensive rehabilitation setting 
where various professionals work together to identify 
treatment goals. In such a setting, vocational planning 
is likely to be more appropriate because the counselor 
may be able to gain insight into what further 
progression may occur and be able to plan accordingly. 

If a specific physical function has been lost within the 
prior 6 months and no active rehabilitation treatment 
for it has occurred, rehabilitation techniques and 
adaptive aids and equipment might allow the function 
to be regained. While some patients may require 
inpatient treatment for 3 to 6 weeks, others may be 
able to attain their goals in an outpatient program. 
Strenuous exercise, per se, is not useful because of the 
easy fatigability associated with MS. 

Followup Management 

Because of the remissions and exacerbations that 
occur in MS, continued medical followup is necessary 
even though such visits are not likely to influence the 
central nervous system lesions. Visits to a neurologist, 
a physiatrist, or a rehabilitation center every 1 to 2 
months can help abort a potential loss of functional 
skills, or restore a functional skill recently lost through 
an exacerbation. Adaptive equipment, environmental 
manipulation, and utilization of residual function plus 
technique training can help restore independence or at 
least minimize dependency. In addition, regular 
followup is important to detect and/or treat any 



Multiple Sclerosis 117 



complications that may occur. A counselor may 
therefore wish to incorporate such a followup system in 
his vocational planning. 

VOCATIONAL IMPLICATIONS 

As shown in the Israeli study discussed earlier, more 
than one in five MS patients are in need of vocational 
rehabilitation services. Because the disease is so 
variable, the length of time spent in a retraining 
process should be based on the physician's judgment of 
the expected future course of the disease. A patient 
with a rapidly progressive form of MS should not be 
enrolled in a long-term training program, but a short 
training course to allow such a client to learn a less 
physically demanding task might be highly 
appropriate. On the other hand, if retraining is needed 
to teach the client with a slowly progressive form of MS 
a less physically demanding skill, it might be 
reasonable to enroll the client in a 1 - to 2 -year, but 
probably not a 4 -year, program. 

The vocational counselor should give major 
consideration to the physical demands of the client's 
occupation. Vocational interests of the client with MS 
which require heavy physical work should not be 
encouraged because of the fatigue factor in particular. 
The ultimate goal of vocational rehabilitation of a 
client with MS should be to provide services that lead 
to light rather than heavy work. All things being equal, 
the client should be trained and directed toward 
sedentary work, since the ability to work from a 
wheelchair, should it later become necessary, would 
prolong the period during which a client can be 
vocationally productive. 

Even in the late stages, the client might be quite 
mentally capable of a vocational pursuit that does not 
demand physical strength. The Israeli study cited 
earlier found that fewer persons with sedentary jobs 
had to change their occupation as a result of their 
disease than persons with nonsedentary jobs. The 
sedentary work chosen, though, should not require 
fine hand movements, since tremor and ataxia are 
likely to develop. With tremor and ataxia, even writing 
might become difficult. 

Fatigability is a major component of MS, and 
consequently occupations requiring repetitive physical 
tasks may need to be avoided. Some patients may 
actually need to lie down and rest for a short period of 
time in the afternoon, and vocational considerations 
may need to center around this. 

Persons with MS tend to do less well at high 
temperatures, and therefore the temperature of the 
environment in which the client works is very 
important, and should not be excessively high. 

Finally, persons with MS are thought to function 
less well outdoors and, if possible, should be placed on 
inside jobs protected from the extremes of 



temperature. Also, because ambulation may be a 
problem in late stages, a minimum of physical 
movement from place to place within a vocational area 
is desirable. 

A vocational plan should also take into consideration 
the bladder needs of a client. Clients with catheters 
need to be able to empty the collection bags in an area 
affording privacy. Toilets need to be close to the work 
area for clients with frequency and urgency, and they 
must be wheelchair accessible if the client is in a 
wheelchair. 

MS should have no effect on whether the client 
functions better dealing with things and objects rather 
than people and ideas, except that the client with 
advanced MS who has slurred speech and bladder 
incontinence might function less well with people than 
with objects. It has been the feeling of many physicians 
that stress should be avoided by clients with MS and, 
in the absence of contrary findings, it is appropriate 
that the less emotionally stressful the occupation, the 
better. 

In the late stages of MS, the mental abilities of some 
clients may be affected. These disturbances may be the 
major reason why the client must finally stop working. 

In view of the unstable nature of MS, a vocational 
counselor should provide continuous intermittent con- 
tact with the client. This may salvage the vocational 
situation when an exacerbation develops. The 
counselor also needs to be alert to new functional losses 
associated with an exacerbation which may interfere 
with a client's vocational activity. When an exacerba- 
tion occurs, it is important to consider rehabilitation 
treatment or environmental adaptations in the job to 
re-establish a function that the exacerbation may have 
caused to be lost or impaired. 

In conclusion, an indoors, sedentary, physically 
nondemanding job requiring minimal hand dexterity 
may be optimal for a client with MS. Trained in such 
an occupation by vocational rehabilitation services, a 
client with MS may have many more productive years 
than if vocational rehabilitation services had not been 
provided. 

BIBLIOGRAPHY 

Bauer, HJ, Firnhaber W, Winkler W: Prognostic criteria in 
multiple sclerosis. Annals NY Acad Sciences 122:543-551, 
1965. 

This is an excellent review of the working capacity of pa- 
tients with multiple sclerosis. The study was done in Ger- 
many for the German Ministry of Labor to determine the 
feasibility of rehabilitating MS patients who were receiving 
compensation. The study sample is large, involving over 
1,200 patients with MS. 

BrownJR: Recent studies in multiple sclerosis: Inferences on 
rehabilitation and employability. Proc Mayo Clinic 
44:758-765, 1969. 
This article discusses the rehabilitation potential of MS pa- 



1 1 8 Kraft 



tients with reference to four major areas: the neurological 
systems involved; the course of the disease; the severity of 
the disease; and the patient's personal and vocational ad- 
justment. 

Kelly R: Management of MS. Nurs Mirror 143(6):48-59, 1976. 
This is an up-to-date, very brief, and easy-to-read review of the 
management of MS. 

Matson RR, Brooks NA: Adjusting to multiple sclerosis: An 
exploratory study. Soc Sci & Med 11:245-250, 1977. 
In this study of 174 MS patients, a measure of self-concept 
was used as the key indicator of adjustment to the disease, 
and a four-stage process of adjustment to MS is proposed. 
The authors contend that a successful adjustment to MS is 
often misinterpreted as euphoria. 

Maugh TH II: Multiple sclerosis: Genetic link, viruses 
suspected. Science 195:667-669, 1977. 

: Multiple sclerosis: Two or more viruses may be 

involved. Science 195:768-771, 1977. 
: The EAE model: A tentative connection to multi- 
ple sclerosis. Science 195:969-971, 1977. 
This is a comprehensive and thoughtful analysis of the 
multiple sclerosis problem, with an emphasis on basic 
scientific investigation. It is not written for physicians but 
for the intelligent layman with an in-depth interest in 
multiple sclerosis. 

Mei-Tal V, Meyerowitz S, Engle GL: The role of 
psychological process in a somatic disorder: Multiple 
sclerosis. Psychosom Med 32:67-80, 1970. 
Twenty-eight of thirty-two patients studied were found to 
have had psychological stress prior to the onset of MS 
and/or exacerbations. The kinds of stress found and case 
studies illustrating the stress situations are included. 

Poser CM: Diseases of the myelin sheath. In Merritt 
HH (ed): A Textbook of Neurology . Ed 5, Philadelphia, Lea & 
Febiger, 1973. p. 566-727. 

Thi» is a comprehensive, medically oriented review of ac- 
cepted medical information on multiple sclerosis and 
related neurologic diseases. Although most of the informa- 
tion in the chapter is beyond the scope of interest of the 
vocational counselor, it will be useful for two purposes: (a) 
to suggest as a guide to the counselor's medical consultant, 
and (b) to look up the accepted relevance of a variety of 
diagnostic tests. 

Pulton TW: Multiple sclerosis: A social psychological 
perspective. Phys Ther 57:170-173, 1977. 
This article includes a brief description of MS, mention of 
the relation between stress and the onset of MS and/or ex- 
acerbations, and the personal, familial, and social pro- 
blems the MS patient may have. 

Rozin R, Schiff Y, Kahana E, Soffer D: Vocational status of 
multiple sclerosis patients in Israel. Arch Phys Med Rehabil, 
56:300-304, 1975. 

This highly recommended article for vocational counselors 
surveys the problem of multiple sclerosis and its vocational 
implications in Israel. Although the study was not done in 
the United States, the data can be extrapolated to patients 
with MS in the United States. The article is very readable. 

Tourtellotte WW: Multiple sclerosis. In Conn HF (ed): 
Current Therapy. Philadelphia, Saunders, 1968, p. 664-673. 
This is an easy-to-read review of a variety of common 
treatments for the common complications of MS. It is well 
written and concise. 



STROKE AND CEREBRAL TRAUMA: 
MEDICAL ASPECTS 

Thomas P. Anderson, M.D. 



DISEASE DESCRIPTION 

Stroke is the term given to the condition often 
referred to by the medical profession as cerebral 
vascular accident (CVA). Stroke can be defined as a 
sudden onset of weakness or other neurologic 
symptoms as a result of injury to a blood vessel in the 
brain (cerebrum, cerebellum, or brain stem). 
Approximately 50 percent of strokes are transient 
ischemic attacks (TIA's). These produce only 
temporary or transient neurologic deficits, from which 
a patient recovers within hours. The remaining strokes 
leave some permanent neurological damage and are 
termed completed strokes. In this chapter, the term 
"stroke" refers to completed stroke. 

Cerebral trauma, or injury, which can occur 
anywhere in the brain, may differ from stroke in the 
mechanism of insult to the brain tissue, but the results 
and effects on the person are sufficiently similar that 
they can be discussed together. To avoid confusion in 
the discussion of cerebral damage, one should always 
keep in mind that damage to one side of the brain 
generally affects the opposite side of the body. A 
patient, therefore, who receives an insult to the right 
hemisphere will have a left hemiparesis and vice 
versa. Strokes and head injuries can also occur which 
are largely confined to the brain stem and cerebellum. 
Such insults, even if largely one-sided, are likely to 
influence the action of both sides of the body because of 
the smaller size of the brain stem and cerebellum 
compared to the cerebrum. 

Because our culture is deeply involved with the 
automobile and motorcycle, the prevalence of cerebral 
trauma is steadily increasing. In some rehabilitation 
centers, brain trauma is more common than stroke. 
While the average age of patients with cerebral trauma 
is younger than the average age of stroke patients (45 
to 55 years), stroke can also occur in children, 
adolescents, and young adults. Stroke has received 
much attention because it ranks third as a fatal disease 
in the U.S. Of greater significance to the vocational 
counselor, who deals with patients who survive 
completed stroke or cerebral trauma, is that stroke is 
second only to arthritis as a crippling disease and, as 



indicated, many patients fall within the working age 
group. 

Stroke is often accompanied by one or more other 
medical problems associated with diseases of blood 
vessels, such as hypertension, heart disease, diabetes 
mellitus, atherosclerosis, elevated blood cholesterol, 
and peripheral vascular disease. Similarly, patients 
who have suffered an injury severe enough to result in 
cerebral trauma have often sustained injuries to other 
parts of the body as well, and these injuries may cause 
symptoms unrelated to the area of the brain damaged. 
Hence, when dealing with patients with cerebral 
trauma and /or stroke, it is important to remember that 
other related conditions often must be taken into 
consideration (see chaps. 22 and 24). 

Pathology 

The response of brain tissue to injury is similar 
whether the injury results from direct trauma or from a 
vascular accident. In either case, function in the area of 
the brain affected either stops altogether or is 
impaired. There are three main causes of stroke: 

1. Thrombosis is the most common cause. A blood 
clot (thrombus) forms in a blood vessel and reduces or 
blocks the blood flow past that point. The part of the 
brain served by the blood vessel dies within minutes 
through lack of nourishment. The size of the area of 
brain death partly depends on how much of the area 
might also receive a partial blood supply from a blood 
vessel not involved in the stroke. In the older patient, 
the area will be larger because blood vessels are 
generally smaller and the collateral circulation is poor. 
In the younger patient, the area is apt to be smaller 
because of a good collateral circulation. 

2. Hemorrhage is caused by a rupture of a blood vessel 
resulting in bleeding into the brain tissue. Damage 
from hemorrhage is likely to be twofold. In addition to 
brain death associated with lost nourishment from the 
vessel, damage to involved brain tissue can also occur 
from the pressure produced by the blood. 
Hemorrhagic stroke is often associated with high blood 
pressure, a factor in causing the blood vessel to 



119 



120 Anderson 



rupture. In the younger patient, hemorrhage can occur 
from rupture of an aneurysm. An aneurysm is a 
ballooning out of a vessel because of weaknesss in the 
vessel wall. Aneurysms are usually on the surface of the 
brain; hence when bleeding occurs, the blood fills the 
subarachnoid space, and the term subarachnoid 
hemorrhage is used to describe the situation. 

3. Embolism occurs when a blood clot that has formed 
elsewhere (thrombus), such as in the heart or in the 
great vessels leading off from the heart, breaks off 
(embolus) and travels up the blood stream until it 
reaches an artery that is too small for it to pass and 
thereby blocks the artery. 

The way brain tissue responds to interference with 
its blood supply is called an infarct. The infarcted 
area has two components: the tissues that have died 
as a result of blood supply loss, and the peripheral 
area in which there may be temporary dysfunction 
due to swelling (edema) or ischemia. If a part of the 
brain does not function for a period of time after 
cerebral trauma or stroke, but function gradually 
begins to reappear within 4 to 5 months, it is assumed 
that the period of nonfunctioning or altered function- 
ing was due to swelling that was slow and gradual 
in subsiding. 

Different portions of the brain are highly specialized 
in their functions, and therefore the effects on the 
whole person as a result of a vascular accident in the 
brain depend on several factors: (a) the side of the 
brain damaged (particularly for cerebral lesions); 
(b) the location of the infarct (cerebrum, cerebellum, 
or brain stem); (c) the size of the infarct; (d) the 
general character of the blood vessels and collateral 
circulation; and (e) the recovery of the involved tissue. 

It is often said that no two patients are ever alike 
who have suffered brain damage. Although this is quite 
true, some generalizations can be made. The 
stereotypical stroke that is referred to in most of this 
chapter is the cerebral lesion produced when the 
middle cerebral artery (MCA) or one of its branches 
(see p. 25) is involved, because this is the most 
common artery involved in cerebrovascular accidents. 
Unless otherwise specified, "stroke" refers to this 
stereotype. 

Anterior and posterior cerebral arteries and arteries 
in the brain stem may also suffer stroke syndromes, 
and areas served by these arteries (see p. 25) can be 
damaged by brain trauma. Brief summaries of the 
particular characteristics of these lesions as they may 
differ from the middle cerebral artery lesion will be 
considered later. 



Signs and Symptoms 

The outward signs and symptoms of stroke or 
cerebral trauma depend on whether the right or left 
cerebral hemisphere is affected. Patients with lesions in 



the dominant left cerebral hemisphere may have 
impairment in communication (speaking, under- 
standing, reading, writing, and judgment), along 
with paralysis of the right arm and leg. Patients 
with lesions in the right cerebral hemisphere generally 
have perceptual (visuospatial) and judgment deficits, 
along with paralysis of the left arm and leg. The left 
hemisphere is dominant not only in right-handed 
persons, but in most left-handed persons as well. The 
right hemisphere controls communication functions in 
only 15 percent of left-handed individuals. 

The most obvious finding in completed MCA stroke 
or one-sided cerebral trauma is paralysis or weakness 
of the opposite side. Soon after the onset of completed 
stroke, the paralysis of the involved side may appear to 
be complete. As edema resolves within a few days or 
weeks, some muscles may begin to show return of 
functioning and are classified as weak, or paretic. 

More disabling are the sensory deficits produced by 
the brain damage. All sensations may be involved 
(light touch, pain, temperature, deep pressure, 
vibratory, visual, and position sense) or only one or 
two may be affected. Impairment of the sense of 
verticality and loss of the ability to maintain sitting and 
standing balance are also seen early after the onset of 
brain damage. When all modalities of sensation are 
affected but not totally paralyzed, there appears to be a 
dominance of pain sensation, such that any kind of 
stimulus to the patient's involved side is recognized as 
pain, even though it might have been only light touch. 
Sometimes lesions in the parietal lobe of the cerebrum 
( see p. 20) produce a total neglect by the patient of all senso- 
ry signals from his impaired side (anasognosia; seep. 130). 

Visual deficits include a failure to recognize and 
respond to input from the visual field (see p. 48) on 
the side opposite the lesion because fibers to the 
occipital cortex crossing over the midline are inter- 
rupted. Most commonly, one-half of the visual field in 
both eyes is involved. Persons with left cerebral 
damage tend to have right visual field involvement and 
persons with right cerebral damage tend to have left 
visual field involvement. The effect on vision is rather 
like wearing goggles with either the right or left half of 
both lenses taped over, making it difficult to see objects 
positioned on the taped side. This condition is referred 
to by a variety of terms, such as visual field cut, visual 
field defect, visual field impairment, or homonymous 
hemianopsia. The most common manifestation of this 
symptom is the patient's failure to see the food on his 
tray on his involved side. 

In anterior cerebral artery strokes, sensory deficits 
are usually absent and motor deficits usually involve 
the leg more than the arm. In posterior cerebral artery 
strokes, both sensation and motor impairment may be 
minimal, but visual impairments and balance and 
coordination problems may be significant. In brain 
stem strokes, weakness and sensory deficits are usually 



Stroke: Medical Aspects 121 



bilateral, although one side may dominate. 
Furthermore, brain stem strokes may produce 
paralysis of the cranial nerves, in particular those that 
control eve movement (cranial nerves III, IV, and VI; 
see p. 24) and those dealing with swallowing and 
tongue movement (cranial nerves IX, X, and XII; see 
p. 24). One of the more disabling symptoms in brain 
stem strokes is a swallowing difficulty severe enough 
that feeding may initially require utilization of a tube 
through the nose into the stomach. Brain stem strokes 
are often also associated with an initial period of coma 
because of the damage to the areas in the brain stem 
associated with alertness and wakefulness. 

Brain trauma, more often than stroke, will produce 
bilateral effects even though one side may dominate. 
Thus, brain trauma affecting the cerebrum may 
produce communication as well as perceptual 
difficulties. Cerebral trauma and even brain stem 
trauma are also often associated with initial periods of 
coma. Pure brain stem strokes and pure trauma episodes 
affecting the brain stem alone, by sparing the cerebrum, are not 
associated with intellectual, communication, or perceptual 
dysfunction . 

Complications 

In addition to the communication or perceptual 
dysfunction and the motor and sensory deficits 
discussed above, which are the primary consequence of 
stroke or cerebral trauma, there are several secondary 
conditions which may occur and affect the patient's 
recovery and rehabilitation. 

Depression. By far the most common complication is 
depression. However, it should not be considered a 
part of the natural history of these disorders. The 
depression can often be avoided or at least minimized. 

Contractures. If a joint is not moved through its full 
range of motion several times a day, the surrounding 
muscles, tendons, ligaments, and joint capsule tighten 
and shorten, causing joint motion to become limited. 
The most common sites of these contractures are in the 
shoulders, the muscles that flex the hips, and the calf 
muscles that point the foot downward. 

Contractures develop much more readily in the 
presence of spasticity. In fact, spasticity and 
contracture have a special relationship which can 
become a vicious cycle. When the contracture becomes 
severe enough to cause pain, the pain causes increased 
spasticity, and this in turn enhances the development 
of more contracture. 

Now that contractures are better understood and 
preventive measures are more commonly practiced, 
they are not nearly as frequent a complication of brain 
damage as they were in the past. 

Spasticity. As much (or more) disability can be caused 
by spasticity as by paralysis or weakness. An involved 
extremity is usually flaccid in its paralysis right after 



the onset of a stroke. Loss of moderating influence 
from higher brain centers then causes reflexes to 
become hyperactive. Spasticity is a state of very 
hyperactive reflexes. The muscles may contract 
rhythmically, particularly at the ankle (ankle clonus). 
Spasticity can also appear in flexors of the fingers, 
extensors of the knee, and other muscle groups. It is 
often enhanced by any condition that causes pain, such 
as corns and bunions, or by tension, emotional upset, 
or trying to hurry. In some cases, spasticity may 
enhance the patient's functional abilities. This is 
particularly true if it occurs in the lower extremity 
where the spastic contractions of muscles may help 
stabilize the hip or knee for standing or walking. 

Bowel and bladder dysfunction. Soiling or 
incontinence may occur and is usually due to a 
temporary loss of control of the sphincters. In the male, 
the problem can be further complicated by an enlarged 
prostate which was no problem prior to the stroke. 
Bowel and bladder dysfunction is usually temporary 
and can be corrected except in patients with the most 
severe strokes and head injuries. 

Urinary tract infections. Chronic or recurring 
urinary tract infections may appear in patients who 
have had an indwelling urinary catheter during the 
acute phase of treatment. These infections, while 
usually treatable, can continue to be a problem long 
after the catheter has been removed and urinary 
function has apparently returned to normal. 

Pneumonia. Pneumonia may occur as a 
complication of brain stem stroke or trauma because of 
the interference with the swallowing mechanism. Food 
particles may pass into the lungs and cause 
pneumonia. 

Seizures. Seizures may also occur after brain 
damage, particularly after cerebral trauma. The 
damage and resultant scar may irritate the cortex and 
cause a spontaneous discharage of nerve impulses 
which may generalize to a full grand mal convulsion 
(see chap. 12). 

Shoulder problems. Two shoulder problems may 
occur in stroke patients, particularly when the shoulder 
is not completely paralyzed. The shoulder joint 
(glenohumeral joint) may nearly dislocate (sublux). 
The weight of the arm simply draws the head of the 
humerus from the socket of the scapula (see p. 
35). This condition may be painful, but can be 
controlled. Further, a chronically painful shoulder can 
lead to a shoulder -hand syndrome, a complex 
condition producing severe pain, swelling, 
contracture, and edema of the hand and shoulder on 
the involved side. 

Others. Complications of the primary diseases 
associated with and partially contributory to the stroke 
may also exist. Thus, complications associated with 
hypertension, myocardial infarction, generalized 
atherosclerosis, peripheral vascular disease (see chap. 



122 Anderson 



22), diabetes (see chap. 24), and sickle cell disease (see 
chap. 26) may also be present in patients with stroke. 

Course and Prognosis 

There are generally two types of functional 
improvement that occur after the onset of cerebral 
trauma or stroke. The first type is spontaneous recovery 
from the neurologic deficits that have occurred. The 
second type of functional improvement is due to 
adaptation and/or training. 

Spontaneous recovery usually occurs as edema in 
the brain tissues subsides or the impaired circulation 
begins to improve. At present, usually very little can be 
done to influence this with medication, although 
agents may be developed in the future. Spontaneous 
recovery usually takes place within the first 3 to 6 
months, although some smaller changes may be seen 
during the rest of the first year. 

In general, a person with the typical middle cerebral 
artery stroke shows spontaneous recovery in the lower 
extremity before the upper extremity, and in muscles 
in the proximal part of an extremity before those at the 
distal end of the extremity. Therefore, the hand is 
often the last part to show return of function, if it 
occurs at all. Only 10 percent of stroke patients develop 
good hand function. Recovery of hand function 
beginning before the third week usually means useful 
function in the hand will develop. A person who has 
gone over 3 weeks with no evidence of returning hand 
function is not likely to have useful function of the 
hand. In contrast, the prognosis for lower extremity 
function is quite different; approximately 90 percent of 
stroke patients can learn to walk again. 

While most spontaneous recovery has finished by 
the end of 3 months after onset, patients and their 
families do not like to give up hope at that time. 
Maintaining hope should be encouraged if it motivates 
a person's efforts for rehabilitation. However, if hope 
is associated with denial and postponement of 
rehabilitation efforts, progress is impeded (see p. 15). 

Improvement in functioning through training and 
adaptation is a result of the person making use of his 
remaining abilities, coupled with technique 
development. This may consist of his learning to do 
things with one hand and even changing hand 
dominance. A few individuals may be able to devise 
their own adaptations, but often persons with brain 
damage are sufficiently depressed to have poor 
motivation to try new ways of carrying on former 
activities without guidance and direction. Hence, 
rehabilitation training helps the patient adapt and 
adjust to decreased neurologic function in the limbs on 
the involved side. The loss of the use of one hand does 
not necessarily mean loss of independence in self- care 
and most activities of daily living. 

Once a person has survived the first week or two 



of brain damage from cerebral trauma or stroke, it 
is more often not the brain damage that limits his 
survival, but rather the accompanying conditions such 
as diabetes, hypertension, or heart disease. However, 
it is true that elderly persons who have once had 
a stroke are more likely to have a second stroke than 
the rest of the population. Young patients who have 
suffered strokes from obscure causes and without 
underlying disease can expect a long life. Even in 
older persons, survival after stroke may be as long as 
7 or 8 years after onset. 



FUNCTIONAL DISABILITIES 

Physical Disabilities 

Almost all patients with cerebral or brain stem 
strokes or trauma initially develop physical disability 
affecting skills in eating, dressing, personal hygiene, 
transfers, and ambulation. In left cerebral lesions, 
verbal communication is impaired but nonverbal 
communication may remain unimpaired. In right 
cerebral lesions, verbal communication is unimpaired 
but nonverbal (perceptual) interpretation is impaired. 

Visual field defects may impair reading even in the 
absence of language dysfunction. Reading may also be 
impaired by right cerebral lesions because the 
visuospatial deficits result in poor tracking across the 
printed page. 

Finally, driving skills may also become impaired, 
not only because of arm and leg paralysis, but also 
because of the communication and reading deficits and 
possible perceptual deficits. 



Psychosocial Disabilities 

The cognitive functions of learning, memory, and 
judgment are usually involved. Emotional lability may 
also appear mostly through a release of inhibition, 
although a true reactive depression may occur as a 
result of the insult. Denial of disability can also occur. 
These disabilities are discussed in more detail in 
chapter 10. 

Stroke or brain trauma can also have a significant 
effect on the family unit. Removal of homemaker, 
breadwinner, or young adult can seriously disrupt the 
entire family. 

Rehabilitation Potential 

When planning for the vocational rehabilitation of a 
person with stroke or cerebral trauma, several factors 
should be considered. 

The damage produced by a stroke or head injury is 
not progressive. Improvement occurs after the acute 
period. Furthermore, the patient's adaptation to the 
various disability components appears to improve over 



Stroke: Medical Aspects 123 



the long term. 

Particularly for insults occurring in the under-40 
age group, a long life can be expected because there are 
usually no underlying diseases. Even for the older 
age group, a certain amount of long-term planning 
can be considered, since the average life expectancy 
after stroke is 7-12 years. 

Most of the impairments listed above can be 
removed through effective treatment, instruction, and 
training. 

Planning may have to be monitored if any 
underlying diseases are present which produce 
complications or other impairments that need to be 
considered. The limits of the ultimate vocational 
rehabilitation may be dictated not so much by the 
degree of paralysis but rather by the total residual 
verbal and nonverbal intellectual function with regard 
to learning, memory retention, and judgment. 

Although no single factor in the early stage of stroke 
or cerebral trauma can positively or negatively predict 
the ultimate functional outcome, some generalizations 
can be made. 

1. The shorter the coma (or absence oi coma), the 
better the person's chances for functional 
recovery. 

2. The earlier the beginning of return of function in 
the involved limbs, the greater the overall return 
of function. 

3. If some return of function shows up only 2 
months after the onset, the likelihood of 
ultimate functional use of this return is low. 

4. Persons with higher socioeconomic and 
educational backgrounds tend to make greater 
use of their potentials. 

5. Persons with significant family members who are 
capable of providing emotional support and 
meeting some of the person's minimal residual 
dependency needs not only avoid institu- 
tionalization, but are more apt to succeed 
vocationally. 



STANDARDS OF EVALUATION 
Diagnosis 

In many cases, the diagnosis of stroke or cerebral 
trauma is not difficult to make. However, there are 
some conditions which can easily be mistaken for 
stroke initially. These are tumors (neoplasms), either 
primary in the brain or spread to the brain from other 
areas of the body; subdural hematomas; multiple 
sclerosis; and toxic conditions due to drugs, such as 
barbiturates, alcohol, or bromides, or to metabolic 
conditions, such as myxedema and diabetes. Infections 
such as meningitis, encephalitis, or brain abscess may 
occasionally have an abrupt onset that simulates 
stroke. Sometimes persons with epilepsy can have a 
stroke -like paralysis for a short period after a severe 



seizure. Suspicion that the diagnosis of stroke or 
cerebral trauma may be incorrect should be aroused 
whenever it is observed that the neurologic deficits are 
progressively becoming worse. 

The essentials of medical diagnosis of stroke and 
cerebral trauma include history and general physical 
examination, neurologic examination, skull X-rays, 
and lumbar puncture, if indicated. Sometimes cerebral 
arteriography or angiography and other procedures 
such as brain scan, computerized axial tomography 
(CAT scan), echoencephalography, and ophthalmo- 
dynanometry are indicated. This last group of 
procedures are not standard evaluations but are done 
in some special cases, particularly when there is a 
question about the diagnosis. If the counselor 
encounters a patient who has had some or all of these 
special procedures performed, he can feel more certain 
that the diagnosis is unquestionable. 

Patients with conditions related to stroke (e.g., 
hypertension) should have appropriate evaluation and 
treatment to keep these conditions under good control, 
particularly during and after rehabilitation treatment. 

Evaluation of Function 

Neurologists, neurosurgeons, and some internists 
are the specialists most involved with the diagnosis and 
immediate acute treatment of cerebral trauma and 
stroke. Evaluation of the severely involved brain- 
damaged person's ability to function is optimally 
handled by a physiatrist. The physiatrist is familiar 
with all the various impairments of function in the 
brain -damaged person, and most of them work with a 
full interdisciplinary allied health team to carry out 
such evaluations and subsequent treatment. 

Often, but not always, the impairments of 
ambulation and transfers are evaluated by the physical 
therapist working with the physiatrist. A rehabilitation 
nurse or an occupational therapist assists in evaluation 
of eating, dressing, and personal hygiene skills, as well 
as homemaking. 

A master's level speech pathologist experienced in 
working with brain -damaged persons is needed to 
evaluate communication deficits in patients with 
aphasia or dysphasia. He can describe the level of 
verbal communication the patient can handle and 
properly interpret to the family and staff the patient's 
level of verbal communication. A speech pathologist 
can be helpful to patients with brain stem strokes or 
trauma in which involvement of cranial nerves inter- 
feres with the peripheral speech mechanism and 
swallowing. Evaluation by a speech pathologist can 
also assist in developing techniques likely to allow 
successful reading for patients with right cerebral 
lesions who have dysfunctions in visual perception that 
interfere with tracking across the printed page. 

Clinical psychological assessment, particularly of 
patients with strokes or brain damage involving the 



124 Anderson 



cerebrum, allows for a more precise understanding of 
memory functions, learning potential, and personality 
patterns. The psychologist can determine whether the 
patient is best approached through pantomime, verbal 
communication, or both. 

Early in the course of stroke or brain damage, 
evaluation of the patient and the family unit by a social 
worker can help detect problems brought on by the 
patient's deficits that can possibly be alleviated by 
various traditional social case work interventions. 

Simultaneously, review by the vocational counselor 
of prior education and work experience, specific 
requirements of the patient's pre-onset job, and 
current job possibilities can encourage positive efforts 
not only toward physical restoration, but toward 
vocational readjustment as well. 

TOTAL TREATMENT 

Acute Phase 

Diagnosis determination is the first part of acute 
treatment. This is followed by control of hypertension 
if present, maintenance of adequate nutrition, and 
maintenance of respiration. Direct treatment of any 
underlying heart disease or heart rhythm irregularities, 
such as atrial fibrillation, may be necessary if 
embolism is suspected. Anticoagulants, such as 
Coumadin for TIA's and sometimes for slowly 
progressive thrombotic strokes, may be used. 
Anticoagulants are not used for completed strokes, 
although some patients may be put on aspirin and 
Persantine. These medications may prevent or delay 
future additional thrombotic strokes by reducing the 
speed with which arterial walls narrow. Anticoagulants 
are never used acutely in cerebral trauma or in strokes 
in which hemorrhage has occurred. 

Bed location and position are part of acute 
treatment. The bed should be positioned in such a way 
that when the patient is supine, personnel and family 
approach toward his strongest side, the side not likely 
to show a defect in visual field. 

Neurosurgical procedures are sometimes indicated. 
Surgery on the carotid arteries in the neck (see p. 25) 
may be indicated for thrombosis which occurs in these 
arteries or if such arteries are a source of emboli. As a 
preventive measure, such surgery may be performed 
on patients who suffer TIA's and who demonstrate 
partial obstruction in the carotid arteries. 
Neurosurgical procedures may be performed on 
patients who suffer hemorrhages from rupture of 
aneurysm walls. The surgery removes the aneurysm 
and prevents additional bleeds in the future. Similarly, 
surgery may be performed on malformed arteries or 
veins if bleeding from them can be demonstrated. 
Hemorrhage into or around the brain which appears 
to aggravate the situation might also lead to neuro- 
surgical procedures to decrease pressure. Finally, 



paralytic episodes that might be induced by tumors 
mimicking strokes usually also require surgical 
removal. 

Acute treatment also includes measures to prevent 
pneumonia, standard methods to ensure adequate 
bladder function, the prevention and treatment of 
urinary tract infection, and the initiation of bowel 
regularity, sometimes assisted by suppositories and 
oral medications. 

From the start, contractures are prevented by 
regular daily performance of passive range -of- motion 
exercises of paralytic parts by a nurse or physical 
therapist. 

Early participation by the patient in self- care 
activities, even if only eating skills and face hygiene are 
attempted initially, will usually prevent a reactive 
depression. Further, depression may be aborted if the 
patient and his family learn the nature of the functional 
improvement to be expected from a rehabilitation 
program. 

Rehabilitation Phase 

Specialists in internal medicine and neurology 
usually attend the patient during the acute stage and 
are able to manage the rehabilitation of patients with 
less complicated and less severe strokes. However, 
physicians specializing in physical medicine and 
rehabilitation and the team associated with them in 
rehabilitation centers are generally more experienced 
and better prepared to deal with the more severe 
strokes. 

The rehabilitation phase usually begins within the 
first week of the acute episode and is best not delayed, 
for delay is inclined to aggravate complications. 
During the evaluation phase, the rehabilitation team 
identifies the problems of dependency in ambulation, 
transfers, eating, dressing, personal hygiene, cognitive 
functions, communication, social and family function, 
homemaking skills, and vocational function. They 
then simultaneously approach these problems in a 
systematic way and communicate with one another 
throughout the rehabilitation program. Using 
techniques that work around whatever residual 
sensory, paralytic, and communication deficits the 
patient may have, they help him achieve maximum 
independence in all of these activities. 

Under the direction of the physiatrist, the 
rehabilitation phase includes activity by several 
professionals. The speech pathologist works daily with the 
patient with communication deficits, particularly 
aphasia. Verbal reading and writing skills, as 
appropriate, are developed, as well as additional 
means of communication through gesture, alphabet 
(word) boards, and other techniques. As skill training 
progresses, the speech pathologist analyses the 
patient's communication strengths and instructs the 



Stroke: Medical Aspects 125 



family and staff in the best techniques of 
communicating with the patient. 

The occupational therapist develops the patient's 
independence in personal hygiene, dressing, writing, 
eating skills, and, as necessary and appropriate, 
homemaking and possibly driving skills as well. The 
occupational therapist assists in the utilization of 
whatever adaptive equipment may be necessary to 
allow even a one-handed individual to be independent 
and suggests home modifications to encourage 
independence. 

The physical therapist initially provides range-of- 
motion exercises to paralytic parts and attempts to 
stimulate function in the paralyzed extremities. 
Strength development in the uninvolved extremities so 
they can begin to "take over" for lost functions also 
proceeds. These activities are associated with training 
in sitting balance, wheelchair ambulation, transfers, 
standing balance, and ultimately ambulation. Walking 
then follows and includes, as necessary, stairs, ramps, 
rough ground, and public transportation. Ambulation 
may require the use of a cane in the uninvolved hand 
and a short leg brace (ankle -foot orthosis) on the 
involved leg. 

The clinical psychologist may be able to recommend 
approaches by staff and family that may be useful if 
depression or denial mechanisms are troublesome and 
how best to work around certain intellectual deficits. 
Intervention may be indicated if there are severe emo- 
tional reactions by any family members to the patient's 
disability. 

The vocational rehabilitation counselor begins early to 
assess prior educational and employment background 
and to receive information on current function and 
ultimate goals. The counselor begins to plan with the 
patient, attempting to draw on prior background and 
current strengths which, coupled with goals of 
treatment, suggest vocational rehabilitation plans. 

The length of hospitalization is usually no more than 
4 to 6 weeks, although for severely and multiple 
handicapped patients and in certain cerebral trauma 
situations, hospital treatment may be as long as 3 
months. 

Treatment of Complications 

Spasticity. When severe spasticity exists, partic- 
ularly in the shoulder, forearm, hand, and ankle, 
to the extent that gains in function are impeded, 
specific treatment may be necessary. Medications, 
such as Valium and Dantrium, and certain special 
physical therapy techniques may help. Rigorous 
attention to ensuring full range of motion of involved 
parts is usually the mainstay of treatment. Injections of 
phenol into the muscle often lessen spasticity. In severe 
cases, cutting the tendons of the involved muscles may 
be necessary. 

Shoulder subluxation. The use of an arm sling is 



indicated when shoulder subluxation occurs in a 
patient with no voluntary motor function in the 
involved shoulder. In order to inhibit the weight of the 
arm simply pulling the humerus away from the 
scapula, a sling fashioned to support the weight of the 
arm is usually helpful. 

Shoulder - hand syndrome. The shoulder-hand 
syndrome usually requires special injections in the 
sympathetic nerves of the neck to block sympathetic 
nerve innervation in the involved extremity, coupled 
with specific physical therapy treatment to the hand 
and shoulder, and sometimes shoulder joint injections. 

Seizures. Usually all patients who have had 
subarachnoid hemorrhages or cerebral trauma are 
placed on seizure medication as a preventive measure. 
Patients with vascular strokes who develop seizures 
several months after their strokes are placed on seizure 
medication after the first episode and this usually 
prevents any further seizures. The likelihood of 
seizures is small and need not be considered when 
making vocational plans. 

Followup 

Following discharge from the hospital, the patient 
with stroke or cerebral trauma should initially be seen 
monthly, progressing to perhaps no less than two to 
four times per year. Initially after discharge, physical 
therapy as often as three to five times a week might be 
part of the treatment program to develop further 
ambulatory skills. 

The patient needs regular followup by his physician 
to ensure that he has an adequate system of maintain- 
ing range of motion in his involved side and that he 
maintains skills achieved during his rehabilitation. 
Further, management of any underlying disease 
(e.g., hypertension, seizures, heart rhythm irregular- 
ities) is necessary. The counselor should encourage the 
patient to protect himself against future problems by 
maintaining his outpatient visits. During the followup 
phase, it is appropriate for the counselor working 
actively with the patient on vocational objectives 
to maintain some measure of contact with the patient's 
physician to ensure that vocational plans are 
compatible with health status. 

BIBLIOGRAPHY 

American Heart Association: Strokes: A Guide for the Family. 
Dallas, TX, 1969. 

This booklet is written for easy understanding of the 
complexities of stroke. It defines stroke and covers the 
blood supply of the brain, the various mechanisms of 
stroke, and how the affected area repairs itself. It also 
briefly but accurately deals with prevention, treatment, 
and rehabilitation for stroke, and includes three keys to 
successful rehabilitation. 

Anderson E, Anderson TP, Kottke FJ: Maintenance 



126 Anderson 



of gains achieved during stroke rehabilitation. Arch Phys 
Med Rehabil 58:245-352, 1977. 

Patients with completed stroke maintained the gains in 
function achieved during stroke rehabilitation throughout 
their long survival time, averaging 7 to 8 years. This article 
describes the factors that influence the maintenance of 
functional gains. 

Dresser AC, Meirowsky AM, Weiss GH, McNeel 
ML, Simon GA, Caveness WF: Gainful employment 
following head injury: Prognostic factors. Arch Neurol 
29(2): 111-1 16, 1973. 

Over eight hundred Korean conflict veterans who received 
head injuries were evaluated 15 years later to determine 
employment status. The factors contributing to 
unemployment or lower employment are discussed. 

Espmark S: Stroke before 50: A follow-up study of 
vocational and psychological adjustment. Scand J Rehabil 
Med Suppl 2: 1973. 

This study evaluated the readjustment to work and the 
psychological adjustment of the younger age stroke 
patients. Some patients were followed for several months 
to obtain an indepth psychiatric evaluation to learn more 
about the factors influencing the adjustment to stroke. 

Gresham GE, Fitzpatrick TE, Wolf PA, McNamara 
PM, Kannel WB, Dawber TR: Residual disability in 
survivors of stroke — the Framingham study. N Engl J 
Med 293(19):954-956, 1975. 

Of the 123 survivors of the original 5,209 persons in the 
1952 Framingham study, 119 were successfully evaluated. 
This article discusses the mean age of onset and the percent 
of persons who were living at home, who were 
institutionalized, who were independent in mobility, who 
were independent in activities of daily living, who resumed 
normal vocational function, and who showed no decrease 
in social activity outside of the home. 

Lehmann JF, DeLateur BJ, Fowler RS, et al: Stroke: 
Does rehabilitation affect outcome? Arch Phys Med 
Rehabil 56:375-382, 1975. 

A sample of 114 consecutive stroke admissions to a 
rehabilitation center was studied statistically to determine 
functional gains achieved and retained after rehabilitation. 
A functional profile was developed that evaluates seven 
activities, each according to a five-point scale. 

McHenry LC, Anderson TP, Raskind R, et al: Essentials of 
Stroke Diagnosis and Management. Philadelphia, Smith, Kline 
and French Laboratories, 1974. 

This brief booklet, designed for use by the medical 
profession, covers all important aspects of stroke. After 
classifying strokes according to both temporal and causal 
aspects, it lists symptoms of various types of stroke. The 
schematic drawings are excellent. The diagnostic work-up 
is outlined and treatment is described in three parts. 

Sarno JE, Sarno MT: Stroke: The Condition and the 
Patient. New York, McGraw-Hill, 1969. 
This book on completed stroke for those ouside the health 
profession is written in the form of pertinent questions and 
answers covering such aspects of stroke as cause, physical 



consequences, associated speech disorders, intellectual and 
emotional aspects, rehabilitation, and prognosis. The 
index facilitates the book's use as a quick reference for 
specific questions about stroke. 

Waite NS: Social problems and the social work role. In 
Licht S (ed): Stroke and its Rehabilitation. New Haven, Licht, 
1975. 

This chapter deals with the adjustments the stroke patient 
and his family must make, including changes in image and 
role, communication, work, and living arrangements. 
Social work roles are briefly explained. 

Weisbroth S, Esibill N, Zuger RR: Factors in the vocational 
success in hemiplegic patients. Arch Phys Med Rehabil 
52: 441-446 and 486, 1971. 

This article describes a study of patients with hemiplegia 
resulting from CVA, tumor, or trauma. The results indicated 
that one third of the patients returned to work, with more 
women returning than men. The factors influencing return to 
work for both left and right hemiplegics are delineated. 



10 STROKE AND CEREBRAL TRAUMA: 

PSYCHOSOCIAL AND VOCATIONAL ASPECTS 

Roy S. Fowler, Jr., Ph.D. 



INTRODUCTION 

There is a continuing tendency to regard human 
behavior and brain function with prejudice, awe, and 
misunderstanding. The brain-damaged person may 
receive inadequate or ineffective services because his 
behavioral deficits are misdiagnosed as psychiatric 
illness. Because it is commonly known that the central 
nervous system does not repair itself, the brain- 
damaged person may be regarded as permanently and 
hopelessly disabled. At best, he may be viewed as 
unpredictable and as a continuing source of 
puzzlement. 

This chapter is designed to help clarify some of the 
intellectual, behavioral, and emotional changes that 
occur as consequences of stroke and cerebral trauma. 
It is hoped that the chapter will help the counselor 
understand why the brain-damaged person behaves 
the way he does, as an understanding of a person's 
abilities in these areas is required for effective services. 
Vocational considerations and some guidelines for 
evaluation are also included. 

Cerebral vascular accident (CVA), stroke, and 
cerebral trauma are all synonymous with brain 
damage. These terms imply that an individual has had 
a normally functioning brain, consistent with his age, 
which was acutely damaged in some fashion, either by 
a disruption in blood flow or by tissue damage caused 
by a blow or a lesion of any sort (see chap. 9). Such 
damage will almost always result in behavioral deficits, 
which may include decreases in physical abilities, 
intellectual performance, social functioning, emotional 
control, or any combination of the four. Even very 
mild injuries, which may not give observable evidence 
of damage, can show up as behavioral deficits later. 

Prognosis 

Almost without exception, a person who has 
experienced brain damage has lost some cognitive 
ability and behavioral function. Rarely, if ever, is a 
patient behaviorally better off following brain damage 
than he was when his brain was functioning normally. 
The extent of deficits, the course of recovery of 



function, and the individual's level of maximum 
performance will all depend upon a variety of issues. 

Age. The effect of brain damage is closely related to 
the age of the individual at the time of the injury. The 
younger the person, the better the prognosis. Given 
the same magnitude of injury, a child will have fewer 
long-term deficits than the adolescent. The adolescent, 
in turn, will do much better in the long run than the 
person over age 30. The aging process seems to be 
associated with a decrease in plasticity and a tendency 
to be less able to compensate for deficits. These 
tendencies are expected in all of us but are especially 
noticeable in the brain -damaged person. 

Severity and magnitude of the lesion. There is a 
positive relationship between the severity of deficit and 
the amount of brain tissue damaged: the more tissue 
lost, the higher the probability of significant deficits. 
There is also a relationship between the length of coma 
and prognosis: the longer the period of coma, the 
greater the probability of permanent deficits and the 
greater the severity of impairment. A possible 
exception is coma secondary to pure brain stem 
injuries that spare the cerebrum. If even a relatively 
young person is in a coma longer than 13 weeks, there 
is a significantly reduced probability that he will 
achieve independent living, whereas a person who is in 
a coma less than 13 weeks has a much higher 
probability of being able to function independently, 
although it is very unlikely that he will be totally deficit 
free. 

Premorbid Junction. Prognosis following a brain 
injury is largely affected by the patient's previous 
abilities, personality, milieu, and life style. The 
brighter, better educated, productive person with 
excellent family support will tend to perform at a 
higher functional level, given the same injuries, than 
his duller, less well educated, less productive, more 
lonely counterpart. This is, however, a potentially 
dangerous generalization, because there are many 
factors that influence ultimate level of function. 
Premorbid personality patterns will also play a role. 
Generally, personality characteristics are not so much 
changed by cerebral damage as they are exaggerated. 



127 



128 Fowler 



High 



UJ 



m 
< 



Low 




Injury 



12 



18 



24 



MONTHS SINCE ONSET 

FIGURE lO-l. Time course of spontaneous recovery of intel- 
lectual function following brain injury. Most of the functional 
improvement occurs in the first 3 months, with smaller gains in 
the next 3 months. Thereafter recovery tends to stabilize. 



The so-called "well -adjusted" person who suffers 
brain damage has a much greater probability of 
maintaining an "appropriate" repertoire of social 
skills, and also is more likely to have supportive friends 
and family who are of much value in helping achieve 
maximum performance levels, than a less "well- 
adjusted" person. 

The demands of the environment in which the 
person has lived or will live are also very important. 
The bright person living in a low-demand environment 
may be able to resume his roles even after fairly 
significant deficits have occurred. The less bright 
"over achiever," functioning in a high-demand 
setting, cannot afford to have even minor impairments 
if he is still to perform adequately. Persons whose 
premorbid behaviors were characterized by 
impulsivity, poor judgment, and carelessness may 
demonstrate more of the same characteristics after 
brain damage. 

Course of Recovery 

The typical course of recovery is illustrated in figure 
10-1. An individual may be characterized by a 
particular level of function prior to injury. After stroke 
or brain injury, an immediate precipitous decrease in 
his total breadth of function occurs. With time, there is 
a spontaneous recovery of many abilities, the extent of 
which will depend upon the variables described above 
and the treatment the patient receives. Most 
spontaneous recovery will have occurred, however, by 
the sixth month following the insult (fig. 10-1). 
Recovery of function and improvement in abilities may, 
for some, occur for several years after injury, but the 
rate of recovery slows considerably after the initial, 
more rapid gains and is usually well stabilized at 12 
months. 

The following generalizations are helpful: (a) 
the more closely an individual's abilities immediately 
following an injury resemble his premorbid abilities, 



the greater the probability that his future function will 
approximate his premorbid function; (b) the greater 
the loss immediately after injury, the lower the 
probability that, even with time, the individual will 
come close to approximating his premorbid level; (c) 
the longer the period of time that the deficits persist, 
the greater the probability that they will be permanent. 

Data on the level of performance in stroke patients 
following discharge from a rehabilitation program 
suggests that more than 60 percent and as many as 90 
percent of all patients, including the most severely 
damaged, will walk, dress themselves independently, 
feed themselves, and have bowel and bladder control. 
They may need some adaptive devices, such as short 
leg braces, crutches, or special fasteners on their 
clothing, but the vast majority of patients will be 
independent in most activities of normal daily living. 

Between 50 and 70 percent of all stroke patients with 
language problems will be able to communicate at a 
functional level sufficient for the mechanics of living. 
This does not mean that they will not have deficits, but 
it does mean they will have useful communication 
skills. 



Area of Cerebrum Affected 

The area of the cerebrum damaged, namely, right or 
left, largely determines the nature of the deficits the 
individual will experience, although variations are 
seen. Thus, while a person may appear at times as 
capable as ever when confronted with some intellectual 
tasks, he may at another time be totally unable to 
perform seemingly comparable or less taxing 
intellectual tasks. In spite of these apparent 
inconsistencies, there are patterns in the behavior of 
brain-damaged persons which can be identified, and 
which tend to be related to the side of the cerebrum 
damaged. The following sections describe these 
behavior clusters and are useful as general guidelines. 
They are usually true but not always correct, as there 



Stroke: Psychosocial Aspects 129 



are inevitably exceptions. Patients with strokes 
affecting the cerebrum usually have one or the other 
side affected (unless, of course, they had more than one 
stroke). Cerebral trauma may well affect both sides 
and produce mixed behavior patterns. This may hold 
true for patients with multiple emboli that lodge in 
both sides. Stroke or trauma patients with pure brain 
stem or cerebellar damage may not show either of the 
following behavior patterns. 

LEFT CEREBRAL DAMAGE 

Deficits 

Typically, the most visible sign of left cerebral 
damage will be a motor and sensory paralysis of the 
right side of the body (right hemiplegia). Because the left 
cerebral hemisphere is associated with language 
function (the processing and coding of data into verbal 
symbols), individuals with left hemisphere damage 
may be unable to understand spoken or written 
language or even produce it. Thus, when teaching 
such a patient a task like dressing, spoken instructions 
may need to be supplemented by pantomime. 

Communication deficits may involve any or all of 
the four language modalities (auditory comprehension, 
speaking, reading, and writing). The term aphasia is 
most commonly used in the United States and Europe 
to refer to disorders of the language processing centers 
in the brain. The term dysphasia is sometimes used 
interchangeably with aphasia, although literally 
aphasia means totally affected and dysphasia means 
partially affected. Usually aphasic symptoms are 
present in all of the language modalities; however, all 
of the modalities usually are not equally involved. 

The counselor will encounter numerous terms 
associated with various patterns of aphasic disorder. 
The terms expressive, nonfluent, or Broca's aphasia refer to 
a language disorder characterized by effortful, halting 
speech with reduced grammar and relatively good 
comprehension. Reading ability is usually better than 
writing ability. Lesions producing such language 
disorders are usually located in Broca's area (see fig. 
3-4, p. 21). The terms receptive, fluent, or Wernicke's 
aphasia refer to a language disorder characterized by 
fluent speech with reduced information content, 
impaired auditory comprehension, and impaired 
reading and writing. Lesions associated with this type 
of aphasia are usually located in Wernicke's area (see 
fig 3-4, p. 21). Care must be taken not to assume that 
receptive functions are normal if the label is 
"expressive aphasia," and vice versa. The impairment 
of sound production accuracy often accompanying a 
lesion in Broca's area is known as apraxia of speech. 

Other communication deficits may be caused by 
brain damage. Dysarthria is an impairment of the 
speed, accuracy and coordination of movement of the 
vocal cords, lips, tongue, and palate (speech 
mechanism), which results in reduced speech 



intelligibility. Lesions causing dysarthria can occur in 
several areas of the brain. For example, lesions of the 
cerebellum result in incoordination of speech - 
mechanism movement, while lesions of the cranial 
nerve nuclei in the medulla result in weakness and thus 
reduced speed of speech -mechanism movement. 

Alexia or dyslexia, almost exclusively a reading 
disturbance, and agraphia or dysgraphia, almost 
exclusively a writing disturbance, are considered by 
some to be specific forms of aphasia. 

Resulting Behavior 

When confronted with a new problem, persons with 
damage to the left cerebral hemisphere tend to respond 
in a slow, scattered, disorganized, anxious fashion, 
regardless of whether the problem is presented verbally 
or by demonstration. They may exhibit an extreme 
and disruptive transient emotional disturbance. This 
may appear as an acute, often disorganizing agitation 
or anxiety that may completely disrupt ongoing 
activity, and very frequently appears if the new 
problem highlights the person's limitations. As the 
person becomes familiar with the new problem and 
begins to handle it, anxiety disappears. 

Depression can be a very common overall emotional 
response because the person with left cerebral damage 
tends to be overly sensitive to his disabilities. However, 
with time and experience, these persons frequently are 
capable of compensating rather well and making a 
satisfactory adjustment to their disability and living 
situation, which in turn resolves the depression. 



Therapeutic Techniques 

When dealing with persons with left cerebral 
damage, it is essential to have a clear understanding of 
the nature of their language problems. For this reason, 
it is advisable to obtain an accurate and current speech 
assessment from a certified speech pathologist. The 
speech pathologist should be able to provide an 
accurate estimate of the person's current level of 
language functioning, an estimate of his future 
language potential, and an operational appraisal of 
what level of function can be achieved and in what 
period of time. In addition, the speech pathologist can 
recommend the most appropriate techniques for 
maximizing communication with the person, and ways 
of structuring the environment to increase 
communication effectiveness and minimize those 
deficits in performance which are due to 
communication problems. 

While the specifics for each person with left cerebral 
damage will differ, some generalizations can be made 
for helping them deal with their communication 
problems. It is important to understand that speech 
and language are different. Language refers to the 
symbols (words, sounds, signs, noises, movements, 



130 Fowler 



gestures, and expressions) which people use to 
communicate with each other. Speech, on the other 
hand, refers only to the sounds made with the mouth. 
A deaf person, for example, may have no speech but 
have excellent language function with hand symbols. A 
great deal of normal day-to-day communication is 
carried on without speech through the use of a variety 
of nonverbal techniques. Therefore, a person who 
cannot speak and be understood or listen and 
understand may still be able to use and understand 
considerable "body" language. Often the use of 
demonstration or pantomime, rather than words, is 
very effective in teaching right hemiplegics a task. 
Demonstrating how to put on a shirt may achieve 
immediate learning, while verbally describing the 
process may produce no learning. 

Persons with language problems may be able to 
understand a short and simple message, but miss a 
long or complicated one. For example, asking the 
person, "Open the door, let the cat out, pick up the 
newspaper, and bring in the mail," may yield only one 
of these actions, or perhaps none of them, because the 
stream of words is overwhelming. Delivering the 
request one stage at a time and awaiting the response 
before presenting the next stage may yield full 
performance. 

Aphasic persons should be addressed with normal 
voice volume and inflection. Since shouted messages 
tend to be short and concise and are usually associated 
with a body posture whose meaning is quite clear, it 
appears that shouting to an aphasic person is more 
effective than speaking at normal volume. However, it 
is the brevity and the gesture, not the volume, that is 
effective. 

Because right hemiplegics tend to be very cautious 
and anxious about performing any new tasks, they are 
likely to need reassurance and frequent indications that 
they are performing correctly. Smiles, nods, or pats on 
the back after successful completion of each stage of a 
task will reassure the person and encourage him to 
proceed to the next step. 

Cautions 

1 . Do not overestimate the ability of a patient 
with left cerebral damage to understand spoken 
speech. Sometimes a person becomes so adept 
at understanding nonverbal communication 
that it is possible to mistake it for verbal 
understanding. A right hemiplegic may seem 
uncooperative, cross, senile, or irrational if his 
understanding is overestimated. Verify his level 
of comprehension. 

2. The fewer words the better. Keep messages 
simple and concise. 

3. Norma] voice volume is preferred. 

4. Use your ordinary voice. Avoid slipping into 
the affected voice one might use with a child. 



RIGHT CEREBRAL DAMAGE 

Deficits 

The most visible sign of right cerebral damage will 
usually be a motor and sensory paralysis of the left side 
of the body (left hemiplegia). Of often greater 
importance is the loss of the intellectual functions 
governed by the right cerebral hemisphere. The 
intellectual functions concerned are depth perception, 
appreciation of the concept of wholes from incomplete 
or fragmentary data, intuition, critical imagination, 
and nonverbal perception. The primary deficit of right 
cerebral hemisphere damage is the inability to 
accurately interpret visual information and properly 
orient oneself with respect to the environment. The 
terms visuospatial deficits or perceptual deficits are often 
used to describe this condition. Minor visuospatial 
failures (e.g., attempting to put down a coffee cup and 
missing the table while reading) are experienced by 
many normal persons when they are very tired or 
concentrating on something else. Persons with right 
cerebral lesions may have deficits that are more major 
and pervasive. 

Visuospatial deficits, although equally as important 
as speech and language deficits, are easily overlooked. 
Consequently, the abilities of the left hemiplegic may 
be overestimated. These deficits may cause the person 
to be mislabeled by disappointed observers as 
uncooperative, unmotivated, overly dependent, or 
confused when he unexpectedly encounters difficulties 
with what appear to be simple daily routine activities. 

Persons with severe visuospatial deficits have more 
difficulty learning to care for themselves than persons 
with speech deficits. Even with concentration, they 
might be unable to steer a wheelchair through a large 
doorway without bumping the frame. They might 
confuse the inside and the outside of their clothes, or 
they may miss buttons and not get their clothes on 
correctly. They may have difficulty knowing whether 
they are sitting upright or tilting, and they may have 
difficulty estimating their distance from objects. They 
may be unable to read a newspaper or add a column of 
figures, not because they lack the ability to read or add, 
but because they lose their place on the paper. A 
person with even minor visuospatial deficits will 
probably not be safe driving a car. 

In addition, persons with right cerebral damage 
have more problems with visual field deficits (see p. 
48) and with one-sided neglect (anosognosia) than 
those with left cerebral damage. The problem of 
neglect cannot be explained solely by sensory loss, nor 
is it due to a visual field deficit. A person with a serious 
neglect problem behaves as if he were selectively 
ignoring everything that happens on his involved side, 
even if he might be able to see it. He may ignore a 
person approaching from his left side, or he may not 
recognize his left arm or leg as parts of his own body. 



Stroke: Psychosocial Aspects 131 




Sample (examiner) 



Copy (patient) 



FIGURE 10-2. Attempt at copying a cross by a left hemiplegic 
patient with a left neglect problem. While the patient's repro- 
duction of the right portion of the cross is not complete, the 
larger error is in his reproduction of the left portion. 

A person with a neglect problem can easily become 
confused when traveling. If he is wheeled down an 
unfamiliar corridor and back again, he may think he 
has been in two different corridors because he saw only 
one side of the corridor going one way and only the 
opposite side when returning. 

Some persons with severe neglect problems divide 
the things they see at the midline (e.g., read the word 
"woman" as "man"). A useful test for neglect is to ask 
a person to reproduce a drawing of a Greek cross. If he 
has a neglect problem, he will often only draw the right 
half of the cross (fig. 10-2). 

Resulting Behavior 

Persons with right cerebral hemisphere damage tend 
to behave in ways that promote overestimation of their 
abilities. They are often completely unaware of their 
deficits or may deny or ignore them. They tend to be 
quite satisfied with even a poor performance, are often 
unable to profit from experience, and sometimes seem 
to be complacent or euphoric. They tend to set 
unrealistic goals for themselves which they frequently 
fail to achieve because they do not take their deficits 
into consideration. This tendency toward diminished 
self-awareness often makes them insensitive to the 
needs and desires of others and may result in their 
being rejected by family and friends. 

The left hemiplegic will frequently attempt to do 
things that are not within his limits or abilities and 
thereby endanger himself. He may fall when he tries to 
walk across the room without putting on his brace, or 
he may attempt to drive his automobile and injure 
himself or others. 

Unlike persons with left cerebral damage, persons 
with right hemispheric lesions do not usually become 
depressed following stroke or cerebral trauma. If 
depression does occur, it frequently develops over a 
long period of time and is associated with repeated 
inability to achieve unrealistic goals. It tends to be 
more chronic and debilitating than the depression of 
persons with left cerebral damage and is often fairly 
resistant to intervention techniques. 



Therapeutic Techniques 

It is important to remember that the verbal abilities 
of persons with right cerebral damage remain intact. 
Because of this, and because they are so often poor 
judges of their own abilities, they can often describe in 
detail a task they are about to attempt but which they 
cannot, in fact, perform safely. Their verbal skills, 
however, can be used to supplement faltering 
visuospatial skills. In teaching these persons to perform 
a task, it is often helpful to talk them through the steps 
to be mastered or for them to talk their own way 
through the task. Demonstration is frequently quite 
ineffective because of faulty interpretation of visual 
information. Persons with right cerebral damage need 
a great deal of verbal feedback when they are 
attempting to learn a new task, and they need to be 
encouraged to slow down and check each step carefully 
as it is completed. 

Since left hemiplegics have difficulty processing 
visual cues, it is important to consider the clutter of the 
environment. A poorly lit or cluttered room with 
vividly patterned wallpaper or rapid movements of 
people and objects around the person may be very 
distracting and hinder his performance. A well -lighted 
room and clearly marked reference points, such as 
door frames or mirrors, are helpful. Removal of 
unnecessary equipment and furniture is advisable. 

In general, it is helpful to give the person with a 
severe neglect problem as much information as 
possible about his neglected environment. Important 
items that have been neglected can be pointed out in a 
calm, informative manner. When traveling with the 
person, it is helpful to point out landmarks to the right 
and left along the way and to give frequent reminders 
of destination. 

If neglect is particularly persistent and disabling, the 
left hemiplegic may perform better when the 
environment is modified to compensate for his deficit. 
Furniture or equipment can be moved to the 
nonneglected (i.e., right) side of his bed, wheelchair, 
or desk. His clothes can be hung on the right side of his 
closet, and commonly used items can be arranged on 
the right side of his dresser drawers. His place at the 
table can be arranged so that he receives food from the 
right and passes to the left. This same model may be 
used for arranging work -related items. 

Cautions 

1 . A person with right cerebral damage is likely to 
overestimate his abilities. Ask him to demonstrate 
skills he attributes to himself. 

2. Words are more effective than demonstration 
with left hemiplegics. Do not use gestures or other 
nonverbal instruction techniques. 

3. Simplify the environment to minimize visual 
distractions. 



132 Fowler 



BRAIN DAMAGE IN GENERAL 

We have discussed intellectual problems which are 
typically associated with damage to the right or left 
cerebral hemisphere. Some patients will give no 
evidence of paralysis and yet they may show many of 
the signs of intellectual deficits discussed so far. Others 
will show paralysis but little or no evidence of the 
problems mentioned. If, however, the patient has 
experienced brain damage, it is very likely that his 
behavior will give some evidence of decreased ability. 
Some functions of intelligence are so complicated that 
they need virtually a totally functioning brain. Even 
minor damage may interfere with these complicated 
functions. 



Quality Control 

Quality control refers to the ability to guide and 
check one's own behavior. This means doing the 
"right" thing at the "right" time. Frequently, a 
quality control problem is first identified by those 
closest to the patient. They see changes in behavior 
that are missed by someone less familiar with the 
patient's premorbid abilities. The once fastidious 
person may become sloppy and seem to care little 
about his appearance. He may say the wrong thing at 
the wrong time. He may perform inconsistently, 
making errors today on a task he performed quite well 
yesterday. A person formerly described as shy may 
become immodest and aggressive, or the quiet person, 
noisy. 

Because quality control problems annoy others and 
are not well understood, it is easy to incorrectly infer 
an emotional or psychological problem. While it is 
possible that a brain-damaged person may have 
significant emotional problems which may or may not 
need professional help, it is important that his 
problems not be made more severe by failure to 
recognize intellectual deficits. 

The person with quality control problems due to 
brain damage needs special assistance from his 
environment. He needs to learn to analyze his 
behavior and respond appropriately to the situation. It 
is more likely that his need is for more cues, feedback, 
and information than for psychotherapy. 



Memory 

Almost any injury to the brain, however minimal, 
produces memory problems. Even a healthy person 
will develop memory problems as he grows older. 
Memory has both visuospatial and language 
components, and memory problems can therefore be 
related to the side of the brain that has been injured. 
The person with left cerebral damage tends to have 
more memory deficits associated with language, and 



the person with right cerebral damage will have more 
memory problems associated with visuospatial 
information. It is also possible to have neither obvious 
language nor visuospatial deficits and still have 
significant memory problems. The effect of brain 
damage on memory has many components. Three of 
the most important components will be considered 
here. 

Old versus new learning. There is a tendency for 
information to be remembered selectively according to 
whether it involves new information or old learning. 
Old learning refers to information acquired prior to the 
brain injury, and new learning refers to information 
acquired since the injury. Brain -damaged persons 
tend to have difficulty with new learning even when 
they have very little difficulty recalling previously 
learned material. For example, a person may be able to 
describe in great detail friends, events, or situations 
that occurred many years in the past but may be 
unable to remember what he had for breakfast, the 
name of a new person recently introduced, or the task 
to be performed at this time. 

Sometimes the forgetful patient can improve his 
level of function if he uses a "prosthetic memory." 
Typically, this involves the systematic use of such 
memory aids as lists, appointment calendars, 
schedules, or written notes. The patient records the 
activity he plans to accomplish over a given interval 
(minutes, hours, days, or weeks, depending upon the 
severity of the memory problem). When in doubt as to 
appropriate action, the patient learns to consult his list. 
He then carries out the recorded activity for that 
interval, checks the task off as it is completed, and goes 
on to the next step on the list. More elaborate systems 
involving tape-recorded messages and timers have 
been devised for patients who cannot read. Many 
patients spontaneously discover that a small weekly 
appointment book carried in a pocket can help them 
avoid embarrassment. It is typically used to record 
names, appointments, addresses, and other bits of data 
that can be retrieved later to bolster a faulty memory. 

Retention span. Retention span refers to the number of 
pieces of information in a given message which can be 
retained and used. A brain -damaged person 
frequently has a limited retention span; that is, he can 
remember only a few parts of a complicated message. 
For example, if he were asked to stand up, take off his 
coat, put it on a chair, pick up a magazine, and hand it 
to another person, a person with a short retention span 
might assimilate only the part concerning the 
magazine. He might also simply look puzzled and not 
act at all, being overwhelmed by the complexity of the 
instruction. 

A person with a short retention span may perform 
very well if he is given only brief, simple messages. 
Instructions should be broken into small components 
and the components presented one at a time. 



Stroke: Psychosocial Aspects 133 



Generalization. Persons with memory problems also 
frequently have a decrease in their ability to 
generalize. In its simplest form, generalization means 
applying what has been learned in one setting to 
another setting. A person with a generalization 
problem will therefore have difficulty carrying over 
learning from one setting to another. It has been found 
that in a rehabilitation setting, a patient may perform 
less well on a task he has done day after day if he is 
confronted with a new physical therapist rather than 
his usual therapist. The patient who learns to perform 
a task in a hospital or workshop setting may or may not 
be able to perform that identical task when he goes 
home or into a competitive employment situation. 
Transfer of learning from one setting to another is by 
no means automatic when the brain has been 
damaged. 

Many rehabilitation programs include home visits 
by therapy personnel to facilitate the transfer of 
learning and the carry-over of skills. It is also 
reasonable to attempt to mock up, in a sheltered work 
setting, those kinds of activities that are to be 
performed in a competitive setting, and to give the 
person an opportunity to participate in a work -station 
program. 

A reliable and consistent daily routine is usually 
helpful for persons with generalization deficits. As they 
are very sensitive to any minor changes in their 
environment, they will frequently respond to change 
by becoming irritable and confused because change is 
frightening. When there is a need for change, a 
deterioration in the person's abilities should be 
expected. It is helpful to warn him in advance and 
discuss the events that are likely to occur so he can 
anticipate and rehearse behaviors in his mind. If a new 
routine is quickly and effectively established, the 
person is likely to improve rapidly. 

Some patients, particularly in the early periods 
following the onset of brain injury, will demonstrate a 
problem called perseveration. The patient becomes 
"stuck" in a pattern of repeated responses. For 
example, he may say the same word over and over or, 
when asked to draw a circle on a piece of paper, he may 
continue making circles until someone stops him. As 
an analogy, it is very much like a faulty record player, 
which fails to track properly and plays a record groove 
over and over until it is nudged into continuing to play 
the remainder of the record. Many perseverating 
patients will have to be "nudged" in order to break up 
their pattern of perseveration. Generally, asking the 
patient a new question, calling him by name, or 
touching him lightly will be sufficient to distract him 
and break up the perseveration. 

Emotional Lability 

Brain -damaged persons very frequently show 
partial loss of emotional control. They may switch 



from laughing to crying without apparent reason. 
Feelings and the overt expression of emotion are not 
always linked as cause and effect. Excessive crying is 
the most common problem and is frequently due to the 
insult to brain tissue (organic emotional lability), 
rather than to depression or sadness over perceived 
losses. Organic emotional lability is characterized by 
little or no obvious relation between the start of 
emotional expression and what is happening around 
the person. The emotional behavior (crying, 
unexpected laughter, flares of anger, or moaning) is 
typically easily interrupted by diverting the person's 
attention by saying his name or asking him a question. 
Crying caused by depression is not so easily 
interrupted. 

An effective therapeutic approach is to interrupt the 
emotional behavior of a person with organic lability, it 
possible. If the emotional expression is due to brain 
damage, the person himself has little control over it. 
Interrupting the behavior may help him to avoid 
embarrassing and fatiguing activity. 

STANDARDS OF EVALUATION 

It is highly advisable to have an adequate assessment 
of a patient's deficits and abilities performed by the 
appropriate professionals when stroke or head trauma 
has occurred. In the case of emotional, intellectual, 
and behavioral problems, the minimum workup 
should include an evaluation by a clinical psychologist 
with a Ph.D. or equivalent who is well trained in 
evaluation of brain damage and behavior. 

Evaluations are equally necessary for patients with 
minimal problems and those with severe deficits. 
The patient with severe deficits needs to be evaluated 
not so much from the point of view of what he 
cannot do, but for what he can do. Much too 
frequently, the patient with obvious deficits is simply 
deemed hopeless. An analysis of his abilities as 
opposed to his disabilities can be highly enlightening. 

Significant deficits which can influence behavior and 
emotion often exist without noticeable physical 
evidence. The patient may only be aware of decreased 
energy and efficiency, accompanied by a prevailing 
depression. Often such a patient is experiencing 
emotional difficulties because he is working very hard 
to compensate for unrecognized minor deficits. These 
deficits may only show up on standardized tests of 
intellectual function when evaluated by a 
knowledgeable and skilled professional. An accurate 
assessment may allow the patient to adjust his goals or 
develop compensating approaches to the problem and 
thereby reduce his energy expenditure. 

The following guidelines may be useful in selecting a 
consultant. 

Select a psychologist with experience in rehabilitation 
medicine. Ideally, this psychologist's frame of reference 
will be the restoration of function. He is likely to be 



134 Fowler 



working within the context of a rehabilitation 
treatment team whose members will be able to 
contribute significant observations of the client's 
behavior and abilities from several points of view. 

If a psychologist working within a rehabilitation program is 
not available, select a consultant who identifies himself as a 
neuropsychologist . An increasing number of psychologists 
are trained in the assessment and evaluation of the 
neurological basis of behavior. Typically, these 
professionals work in medical contexts, such as 
departments of neurology or neurosurgery, and 
specialize in the diagnosis of brain damage and its 
behavioral consequences. 

If neither of the above is available, select a clinical 
psychologist who completed his training after 1960. Most of 
the advances in neuropsychological assessment have 
occurred since 1960. It is expected that at least some 
training in neuropsychological assessment procedures 
will be included in any current, competent graduate 
training program for clinical psychologists. Persons 
trained before 1960 may be fully competent if they 
have remained knowledgeable of the neuropsycho- 
logical literature. 

Finally, a counselor is much more likely to receive the kind of 
information he desires if he learns how to use his consultants. 
Simply requesting "an evaluation" of a client will in 
no way guarantee that the consultant will provide the 
kinds of information the counselor needs. If the 
referral is vague, the consultant may simply perform a 
routine evaluation, which may or may not include 
useful, vocationally oriented information. Typically, 
the counselor will be more concerned about the 
prognosis than the diagnosis. The consultant should be 
asked the following questions: (a) What are the client's 
current deficits and strengths? (b) What is the 
projected course of recovery and estimate of maximum 
abilities? (c) When will the client be ready for 
rehabilitation and vocational endeavors? (d) What 
kind of remedial or modification techniques are needed 
and how can they be implemented most effectively? 

It may well be advisable and necessary to obtain 
evaluations of a client several times throughout his 
course of recovery. An initial evaluation should 
probably be performed within 6 to 8 weeks after the 
stroke or injury. Followup testing should be 
accomplished between the fourth and sixth month. It 
may be desirable to evaluate the client again following 
the first year or year and a half. This repeated 
evaluation will allow for the development of a curve of 
recovery and estimation of function. 

VOCATIONAL IMPLICATIONS 

Returning a person to work following brain injury 
will demand a very careful evaluation of (a) the 
individual's job or school setting; (b) the nature of his 
deficits; (c) the severity and kind of brain damage; and 
(d) the type of job and its location, physical demands, 



complexity, interpersonal requirements, language or 
visual perceptual requirements, and degree of new 
learning required. Each person's situation will be 
different, but the following general guidelines may be 
helpful. 

Brain -damaged clients may have the use of only one 
hand and are thereby limited in their ability to perform 
lifting, carrying, pushing, and pulling, especially if 
they use a cane. The majority will need to be confined 
to sedentary or light work, though a few may be able 
to perform medium work. Generally, they should be 
limited in the amount of walking they are required to 
do, but may tolerate standing for a long period. Climb- 
ing, balancing, stooping, and kneeling also generally 
have to be limited for these clients. 

Lack of speed is a critical vocational implication. 
The majority of these clients do not function as rapidly 
as they did prior to their stroke or brain injury. Some 
of them work so slowly that it is preferable for them to 
be employed in a sheltered setting. Others can produce 
high quality work as long as they do not feel they need 
to hurry. The pressure of trying to hurry may itself 
decrease quality. Hence, the competitive workshop is 
often less than ideal for some of these clients. 

Some brain -damaged persons have good tolerance 
for outside work, such as farmwork, but the majority 
probably do better on inside work where they are 
protected from the weather. There are no general 
limitations with regard to climate, noise, vibration, 
gases, fumes, dust, and ventilation. Clients with right 
cerebral damage will perform best in an uncluttered 
environment with good light and a minimum of 
distracting movement around them. 

Special consideration needs to be given to 
impairments in speaking, hearing, writing, and seeing, 
all of which are often seen in these clients. 
Vocationally, these impairments are often more 
limiting than physical impairments. 

The ideal place setting is one in which it is possible to 
arrange job or academic challenges that will vary 
directly with demonstrated ability. Such an ideal 
program will have a job sampling possibility ranging 
from a fully sheltered workshop setting, through 
semisheltered job settings, on to full competitive 
employment. Where such facilities exist, it is possible 
to return the brain -damaged client to some type of 
vocational activity quite early in the course of recovery 
and then adjust his activity to his needs as he improves. 
This type of setting will maximize the probability of 
appropriate vocational disposition. 

If such an ideal setting is not available and the 
rehabilitation plan is to return the client directly to the 
competitive labor market or to an academic program 
where remedial education is not possible, it is 
important to wait until maximum recovery has been 
reached or there is a very clear leveling off in return of 
function. This usually means waiting at least 6 months 
following brain injury or stroke. It is very important to 



Stroke: Psychosocial Aspects 135 



maximize the probabilities of success for a client 
entering a competitive setting. If the client returns to 
such a setting too early, the probability that he will fail 
increases. 

Early failure holds many risks. The client and his 
family may become so discouraged that they may give 
up. The client's employer may be so upset by his errors 
that he may prematurely terminate employment. An 
even greater problem is that the reputation the client 
may gain through such failure may prejudice future 
opportunties in similar settings. 

The young student returning too early to school may 
find himself placed in a special education program 
which may not be flexible enough to adjust to his 
changing status. In many school settings it is not easy 
to make the transition from special educational 
programs into normal competitive academic 
programs. The student who waits until his condition 
has stabilized usually only runs the risk of falling 
behind his peers in the chronology of the educational 
process. While delay may be very distressing to 
adolescents, the long-term potential problems of 
returning to school too early must be considered and 
weighed very carefully. 

Statistics gathered here and abroad suggest that in 
the under -50 age group a rather high percentage of 
stroke patients will be able to return to work. The data 
from Scandinavia and England indicate that between 
32 and 70 percent of all "young" stroke patients will 
be able to return to employment. Some of this 
employment may be part-time or sheltered workshop 
activities. In the United States, the estimates run as 
low as 17 percent to a high of 40 percent. 

In this country, males are more likely to return to 
work than females. Persons with a high school 
education or greater are more likely to return to work 
than blue collar workers. It would appear, however, 
that we do not return as many brain -injured persons 
to work as we might. The knowledge and treatment 
techniques exist and we should be doing a better job. 
Brain -damaged individuals in our society should be 
more active and productive. The counselor may not be 
able to "cure" the client of his deficits, but he can be a 
major agent for change in the quality of services 
received. 

BIBLIOGRAPHY 

Bond MR: Assessment of the psychosocial outcome of severe 
head injury. Acta Neurochir (Wien) 34:57-70, 1976. 
Fifty-six patients with severe head injury and post- 
traumatic amnesia exceeding 24 hours were studied to 
evaluate the neurophysical, mental, and social results of 
their brain injuries, and the relation of duration of amnesia 
to degree of disability. 

Darley FL: A retrospective view: Aphasia. J Speech Hear 
Disord 42:161-169, 1977. 
This article presents the current status of research on the 



nature of aphasia and related disorders, the location of brain 
lesions causing aphasia, language dissolution in aphasia, 
and aphasia therapy. 

Diller L: Cognitive and motor aspects of handicapping 
conditions in the neurologically impaired. In Neff WS (ed): 
Rehabilitation Psychology. Washington, D.C., American 
Psychological Association, 1971, p. 1-32. 
This chapter is an overview of what is known about the 
sensory, cognitive, and motor functions of neurologically 
impaired adults and children, with special attention 
devoted to the assessment and treatment techniques of 
aphasics, hemiplegics, children with minimal brain injury, 
and children with learning deficits. 

Fugl-Meyer AR, Jaasko L, Norlin V: The post-stroke 
hemiplegic patient II. Incidence, mortality, and vocational 
return in Goteborg, Sweden, with a review of the 
literature. Scand J Rehabil Med 7:73-83, 1975. 
A thorough review of the literature on incidence, 
mortality, and prevalence of stroke worldwide is presented 
in this article. It contains a review of the probabilities of 
return to work following a stroke. 

Geschwind N: Language and the brain. Sci Am 226:76-83, 
1972. 

This article describes some of the research on language 
disorders and brain function that has led to a model of how 
the language areas of the brain are interconnected and 
what each area does. It provides useful background for 
understanding language dysfunction. 

Lezak MD: Neuropsychological Assessment. New 

York, Oxford University Press, 1976. 
This excellent textbook covering adult neuropsychological 
assessment provides a detailed overview of the current 
state of knowledge regarding the relationship between 
brain and behavior. It also contains a comprehensive 
description of current neuropsychological testing 
techniques. 

Sheridan J: Restoring speech and language skills. Geriatrics 
31:83-86, 1976. 

This article describes some common speech problems in 
geriatric patients and the role of the speech pathologist in 
treating them. The common speech disorders are described 
in nonmedical terms. 

Smolkin C, Cohen BS: Socioeconmic factors affecting the 
vocational success of stroke patients. Arch Phys Med 
Rehabil 55:269-271, 1974. 

Seventy-four stroke patients were evaluated for vocational 
outcome after rehabilitation services. The major factors 
determining successful return to work after stroke were 
found to be: (a) level of education; (b) prior occupation; 
and (c) sex. 

Vignolo LA: Evolution of aphasia and language 
rehabilitation: A retrospective exploratory study. In Sarno 
MT (ed): Aphasia: Selected Readings. Englewood Cliffs, NJ, 
1972, P. 370-384. 

An evaluation of the effects of speech therapy on the course 
and outcome of aphasia following stroke. 



1 1 CEREBRAL PALSY 



Jessie K.M. Easton, M.D. 
Daniel Halpern, M.D. 



DISEASE DESCRIPTION 

The term "cerebral palsy" refers to disorders of 
movement resulting from damage to the brain. By 
convention, the term is used to describe individuals 
who have suffered damage to the brain at some time 
during its period of growth and development. This 
period is generally considered to continue to 8 years of 
age, though some authorities extend it to 12 years. The 
neurological condition itself is not progressive, but 
manifestations vary in expression with the maturity of 
the brain and body, the activity and experiences of the 
individual, and the quality of management. By the 
time the person has reached vocational counseling age, 
however, the condition is stable. 

The incidence of cerebral palsy is difficult to 
determine, since many cases of congenital origin are 
not diagnosed until after 6 to 12 months of age or 
older. Also, many secondary complications associated 
with cerebral palsy have their onset somewhat later in 
life. While some statistics indicate the incidence of 
cerebral palsy is declining with improved neonatal and 
perinatal care, other considerations indicate that as 
pediatric or neonatal intensive care units save the lives 
of more seriously ill and involved infants, the incidence 
of cerebral palsy is increasing. Undoubtedly both 
trends are present. 

The prevalance of cerebral palsy, at a conservative 
estimate, is about 900,000 clinical cases in the United 
States, or 4.5 cases per 1,000 in the general 
population. The prevalence is increasing because more 
children with cerebral palsy are surviving into 
adulthood, including the more severely involved. 
Thus, the total number of individuals with cerebral 
palsy is gradually increasing. They will come to the 
attention of vocational counselors in greater numbers 
than heretofore. 

A diagnosis of cerebral palsy requires the presence of 
a motor disorder. It is based on the clincial finding of 
abnormal neurological function regardless of the 
ability to identify anatomic or histologic pathology. 
The array of problems is not confined to those 
associated with control of movement. Other deficits in 
function due to damage to parts of the brain other than 
the motor cortex also occur. (The reader will find 



a review of the nervous system and musculoskeletal 
system sections of chapter 3 helpful in understanding 
the following discussion.) 

Since areas within the brain in addition to the 
cerebral cortex may be involved, the diagnosis of 
cerebral palsy does not have the precision or accuracy 
of most other medical diseases. For this reason, some 
authorities feel uncomfortable with the term, since it 
does not truly describe a discrete entity, its etiology, or 
its anatomical location. It is useful as an administrative 
term, however, since all the conditions included 
require a comprehensive understanding, continuous 
management, and similar facilities for proper care. 

The manifestations of cerebral palsy depend upon 
the location and severity of the brain damage. 
Although the locations are not precisely' known, thev can 
be inferred from the symptom complex seen. Typical 
patterns of dysfunction are produced when the damage 
is limited to one area of the brain, when it is scattered 
throughout the brain, when it is unilateral, and when it 
is bilateral. The manifestations also depend upon the 
cause of the damage, the relative sensitivity of different 
structures to these causal factors, and the state of 
development of the brain at the time of the injury. 

Basically, the motor or movement problem in 
cerebral palsy is lack of control of the muscles rather 
than muscle weakness. This lack of control is often 
expressed by a failure of inhibition of some specific 
central nervous system reflexes. These reflexes 
involving muscle movement, in increasing order of 
complexity, are: 

1 . Stretch 

2. Crossed extension 

3. Long spinal 

4. Symmetrical tonic neck 

5. Asymmetrical tonic neck 

6. Vestibular 

7. Startle 

The stretch reflex (see p. 27) is the simplest, and its 
center is located at the level of the spinal cord 
appropriate to the muscle being considered. The startle 
reflex, which is much more complex, is centered at the 
midbrain (see fig. 3-6, p. 22). In table 11-1, the 
muscle movements associated with each reflex, in 



137 



138 Eastern and Halpern 



TABLE 11-1 
REFLEXES LIKELY TO BE UNINHIBITED IN CEREBRAL PALSY 



Reflex 


Definition 


Usual Site of 


Functional Ability 






Pathology' 


Likely To Be 

Disturbed When 

Uninhibited 


Stretch 


Stretch of a 


Central nervous 


Ambulation when lower 




muscle resulting 


system above the 


extremities involved; 




in contraction 


level in the spinal 


dexteritv, fine 




of the muscle. 


cord of the 


manipulation, complex 




Hyperactivity of 


innervation of the 


motor skills when upper 




these reflexes 


muscle being 


extremities involved; 




is referred to 


considered 


speed and strength 




as spasticity. 






Crossed 


Extension of one 


Same as stretch 


Transfers, dressing. 


extension 


arm or leg 


reflex 


writing, standing with 




produces flexion 




both legs straight; 




of the opposite 
arm or leg. 




activities requiring 






bimanual control 


Long spinal 


Flexion of one 


Central nervous 


Sitting balance, 
standing with both legs 




leg results in 


system above the 




extension of the 


level of 


straight, ambulation, 




arm on the same 


innervation of the 


transfers, standing 




side. Extension 


muscles involved 


balance, eating, dressing 




of the leg 








results in 








flexion of the 








arm on the 








same side. 






Symmetrical 


Flexion of the 


In or above the 


Sitting balance, 
ambulation, transfers, 


tonic neck 


neck results in 


midbrain or 




flexion of the 


upper medulla 


eating, dressing 




arms and extension 








of the legs. 








Extension of the 








neck results in 








extension of the arms 








and flexion 








of the legs. 






Asymmetrical 


Turning or 
tilting the head 


Central nervous 


Writing, sitting balance. 


tonic neck 


system above 


transfers, standing 




to one side 


level of midbrain; 


balance, eating, dressing 




results in 


usually considered 






extension of the 


to be in basal 






arm and flexion 


ganglia, cerebral 






of the leg on 


cortex, or both 






that and the 








opposite side. 







essence their definitions, are described. The usual 
location of the damage (pathology) and the functional 
skills likely to be disturbed when these reflexes are 
uninhibited are given. 

These reflexes exist in all people and are normally 
well controlled (inhibited) and effectively utilized. In 
cerebral palsy , control is incomplete, and the reflexes 
are said to be uninhibited and hence exaggerated. For 
example, uninhibited long spinal and vestibular 
reflexes are shown in figures 11-1 and 11-2, 
respectively. Voluntary motion therefore becomes 
impaired or impossible. Persons with cerebral palsy 



may be able to inhibit the reflexes when they are 
relaxed, but if they are excited or anxious, they can no 
longer exert this voluntary inhibition and the reflexes 
override any attempt they may make to perform 
voluntary or controlled actions. 



Etiology 

Most cases of cerebral palsy are acquired, either 
congenitally or postnatally, although a few show 
evidence of being hereditary (hereditary spastic 
diplegia and hereditary tremors). In some cases, no 



Cerebral Palsy 139 



TABLE 11-1 — Continued- 



Reflex 


Definition 


Usual Siu' of 
Pathology 


Functional Ability 
Likel) To Fir 

Disturbed When 
Uninhibited 


Vestibular 


A series of complex 
responses to the 
position and movement 
of the head. Tipping 
the head back or 
upward results in 
flexion or abduction 
of the arms and 
extension of the 
legs. Tipping head 
downward may result 
in extension and 
adduction of the arms 
and flexion of the legs. 
Tilting head to 
the left may result 
in extension of the 
left arm and flexion 
of the left leg with 
opposite actions on 
the right side. 


Above the vestibular 
nuclei and midbrain 


Writing, ambulation, 
standing balance, 
tranfers 


Startle 


A quick generalized 
flexion, usually of 
all four extremities. 
Head and back often 
extend at the same 
time. Head extension 
occurring together 
with upper extremity 
flexion often resembles 
a vestibular reflex, 
but differs in that it 
is elicited by loud 
noises, frightening or 
sudden occurrences. 
Even nonthreatening 
and mild stimuli mav 
be sufficient to trigger 
the startle reflex. 


Midbrain or above 


Sitting balance, 
ambulation, transfers, 
standing balance, 
dressing, eating 



etiology can be established. Any agent or event that 
causes damage to the brain during its development can 
cause the syndrome of cerebral palsy. Table 11-2 
summarizes the causes seemingly responsible during 
pregnancy (prenatal), at labor and delivery (natal), 
and after birth (postnatal). The type of damage (e.g., 
infections, toxins, or trauma) is also summarized. 

Prenatal causes. Among the more common prenatal 
causes are bacterial, protozoan, or viral infections in 
the mother. Different clinical syndromes result, 
depending on whether the infection occurs early, when 
the basic structure of the brain is initially developing, 
or later, when more specific functional interactions are 
maturing. Infectious agents definitely identified are 
rubella, toxoplasmosis, and congenital syphilis. 
Occasionally, other infections are implicated, such as 
influenza or kidney infections, but the cause and effect 
relationship is not as well documented. 

Toxic causes of brain damage in the developing fetus 



usually relate to certain pathologic conditions in the 
mother, such as eclampsia, toxemia of pregnancy, and 
maternal "poisoning" by drugs and heavy metals. 
There are no clear-cut relations between narcotic 
ingestion by the mother and brain damage. Some 
infants born to chemically dependent mothers, 
however, do have cerebral palsy and many have overt 
evidence of addiction at birth and suffer from 
unrecognized withdrawal. Rh factor incompatibility is 
also technically a toxic cause, the toxin being the 
antibodies developed by the Rh negative mother to the 
child's Rh positive blood. 

Trauma is not a very common prenatal cause of 
cerebral palsy. A premature birth, either spontaneous 
or associated with trauma to the mother, can result in 
poor fetal nutrition and oxygenation, with possible 
damage to the fetal brain. Other events in the mother, 
such as asphyxia, severe anemia, or hypotension, may 
interfere with the oxygen delivery to the fetus. Further, 



140 Eastern and Halpern 




FIGURE ll-l. This patient's long spinal reflexes are poorly 
inhibited. An attempt to perform an activity with the right upper 
extremity results in purposeless patterned positioning of all 
other limbs, and is characteristic of athetosis. 




FIGURE 1 1-2. Uninhibited vestibular reflexes cause this man's 
arms to lift up as he raises his head, making it difficult for him to 
use his hands at table level. The severe hyperextension of the 
fingers, coupled with wrist and elbow flexion, is a characteristic 
dystonic posture elicited when reaching is attempted. This 
patient's particular condition is called tension athetosis with 
spasticity. 

a bleeding tendency in the mother (hemophilia) may 
induce bleeding in the fetus. If the bleeding occurs in 
the brain, damage will result, which can give rise to 
cerebral palsy. 

Natal causes. Most cases of cerebral palsy occurring 
around the time of birth are associated with difficulty 
during labor and delivery, resulting in hemorrhage or 
anoxia and subsequent loss of functioning brain tissue. 
Premature infants are particularly susceptible, but 
prematurity alone is not felt to cause cerebral palsy 
unless these other factors are present. 

Postnatal causes. In the period immediately after 
birth, infections such as meningitis or encephalitis may 
affect the brain directly, pneumonia may cause anoxia, 



and any kind of trauma to the head in the neonatal 
period may cause brain damage. When the blood sugar 
is temporarily very low (hypoglycemia), the child may 
suffer convulsions. Either direct brain damage from 
the hypoglycemia or anoxic damage during 
convulsions may occur. 

Rarely, a child may have a congenital aneurysm, 
which will cause brain damage if it bleeds. 
Occasionally, an X-ray will show an empty space 
within the brain substance (porencephalic cyst). Most 
often, this is due to severe damage, with disappearance 
of the brain tissue. Cerebrospinal fluid fills the empty 
space, which gives it the appearance of a cyst. 

Classification 

The movement disorders in cerebral palsy are 
classified both by the area of the body affected 
(topography) and by the symptom complex exhibited. 

Topography. The topographic classification of 
cerebral palsy describes six areas of the body or limbs 
affected by the brain damage. 

1. Qiiadnplegia describes the involvement of all four 
extremities relatively equally. 

2. Diplegia describes involvement of the lower 
extremities predominantly, with only mildly 
affected upper extremities. 

3. Paraplegia refers to involvement of the lower 
extremities alone, with apparently normal 
upper extremities. 

4. Monoplegia describes involvement of only one 
extremity, either upper or lower. 

5. Hemiplegia describes involvement of both limbs 
on one side. 

6. Triplegia describes involvement of three extremi- 
ties, with only one extremity unimpaired. 

Symptom complex. The motor problems associated 
with brain damage in cerebral palsy may occur alone 
or in conjunction with others and may affect one or 
more body parts. Nine symptoms are included in the 
concurrent symptomatolgic classification accepted by 
the American Academy for Cerebral Palsy and 
Developmental Medicine. These include: (a) 
spasticity, (b) athetosis (nontension and tension), (c) 
dystonia, (d) atonia, (e) rigidity, (f) ataxia, (g) tremor, 
(h) mixed, (i) undetermined. Of the above, spasticity, 
ataxia, and athetosis are the main movement disorders 
and are discussed first. 

Spasticity occurs when the stretch reflex (see table 11-1) 
is not inhibited, and is manifested in three ways: (a) 
an excessive response to rapid stretch of the involved 
muscles when the tendons are tapped by a reflex 
hammer during a clinical examination; (b) one or 
more "bouncing" contractions (clonus) occurring 
spontaneously when the muscle is stretched and held 
by a force; and (c) stereotyped postures assumed as a 
result of persistent excessive muscle contraction (tone) 



Cerebral Palsy 14] 



TABLE 11-2 
ETIOLOGY OF CEREBRAL PALSY 



Prenatal Factors 


Natal Factors 


Pi istnatal Fa< tors 


(Conception to onset of labor) 


(Onset of labor to birth) 


I - birth oj . table fetus 


Hereditary 


Anoxia 


Traumatic 


Genetically transmitted, often 


Mechanical respiratory 


Contusions "I the brain 


sex-linked, symptoms present at 
birth or soon after, does not 


obstruction 






Skull fractures 


progress 1 e.g.. hereditary athetosis, 
familial tremors) 


Atelectasis 






Infections 




Narcotism 


Acute (e.g., meningitis. 


Acquired in utero 




encephalitis 


Prenatal infection (e.g., toxo- 


Placenta previa or abruptio 




plasmosis, syphilis, rubella, or 




Chronic (e.g.. syphilis, brain 


other maternal infection) 


Maternal anoxia or hypotension 
(e.g., spinal anesthesia) 


abscess, granulomas) 


Prenatal anoxia 




Vascular 


Maternal anoxia, anemia, or 
hypotension 


Breech deliyeries, with delay 
of aftercoming head 


Hemorrhage 


Placental pathology (e.g.. abruptio, 


Prolapse of cord 


Embolus 


infarcts, premature atrophy) 


( erebral hemorrhage & contusion 


Thrombosis 


Prenatal cerebral hemmorrhage 


Trauma 


Anoxia 


Toxemia 
Trauma 


Physiologic (e.g., disproportion, 


Carbon monoxide poisoning 


Maternal bleeding diathesis 


dystocia) 


High altitude & low 


Rh factor 


Obstetrical (e.g., inaccurate 
forceps application) 


pressure anoxias 


Respiratory distress syndrome 


Metabolic disturbances (e.g., 
maternal diabetes) 




Constitutional factors 


Respiratory obstruction (e.g., 




Prematurity, congenital 
anemias of the newborn 


croup, foreign bodies. 




drowning) 




Hvpoprothrombinemia 






Metabolic abnormalities 






Insulin reaction 






Postneoplastic 



in certain muscles, associated with persistent reduced 
tone in their opposites (antagonists). Typical postures 
are flexion of the wrists, elbows, and knees; flexion, 
adduction, and internal rotation of the hips, and 
plantar flexion of the feet. 

Together with these abnormalities of tone, spastic 
patients also demonstrate a very reduced ability to 
perform complex varied movements. Movements 
available may be quite stereotyped, regardless of what 
movement may be attempted. A loss of dexterity and 
reduced ability to learn motor skills results. Spasticity 
may occur in conjunction with athetosis or, less often, 
with ataxia. 

Ataxia is defined as difficulty in controlling the 
accuracy of a needed muscle length or limb position. It 
may result from a loss or reduction of sensory input 
from joints or, more frequently, from an inability to 
appropriately activate or inhibit muscles with sufficient 
rapidity and accuracy, usually associated with 
cerebellar dysfunction. The patient exhibits an 
uncertain aim in reaching, grasping, or transferring 
objects, manifested as a "hunting" movement, or 
dysmetria. There is repetitive overshooting and 



overcorrection of each motion, giving rise to a 
continuous tremor during each attempted motion 
(intention tremor). In walking, the gait is staggering, 
with uncertain and inaccurate foot placement, poor 
balance, and constant readjustment of position that has 
the appearance of alcohol intoxication. 

Athetosis refers to the involuntary occurrence of 
purposeless movement when the patient attempts 
purposeful motion. The abnormal movements may 
occur not only in the limb that is being purposefully 
moved, but may also involve an "overflow" of activity 
to all the other limbs (fig. 11-1). Most often, the motor 
activity can be identified as an exaggeration of one or 
more of the reflexes described in table 11-1. Athetosis 
varies from patient to patient and from time to time, 
but is usually characterized either by flailing or writhing 
movements of the trunk or extremities that are not 
voluntarily initiated. The patient may be able to learn, 
with training, to inhibit athetotic movements so that 
purposeful movements can be performed with 
improved accuracy and diminished overflow to the 
other limbs. Emotional excitation above a certain level 
can cause the patient's voluntary control or ability to 



142 Eastern and Halpern 



inhibit the movements to weaken, with resultant return 
of the involuntary (overflow) motor activity and loss of 
control. 

Dystonia, an expression of abnormal increased 
muscle tone occurring simultaneously in both muscles 
and their antagonists, may be a component of 
athetosis. The symptom complex is then described as 
dystonic or tension athetosis (fig. 11-2). Impairment of 
function of the extrapyramidal system is associated 
with athetosis or certain forms of dystonia. Dystonia is 
exaggerated by central nervous system excitation or 
stimulation, and it may be induced even by the simple 
activation required for an attempt at voluntary 
movement. Characteristic dystonic postures are often 
elicited during certain motor activities. In figure 11-2, 
the dystonic component of tension athetosis is evident 
in the posture assumed by the patient's hands. 
Dystonia may also occur in association with 
uninhibited stretch reflexes, when it is recognized as a 
component of spasticity. It also often occurs alone. 

Rigidity exists when severe excessive muscle tone, 
such that no passive motion is possible, appears 
simultaneously in both flexors and extensors. The 
rigidity may mask underlying spasticity and/or 
athetosis. 

Atonia or hypotonia, a condition where muscle tone is 
decreased or practically absent, occurs only in a small 
number of patients with cerebral palsy. 

Although not part of the list of symptoms used for 
classification by the American Academy for Cerebral 
Palsy and Developmental Medicine, other symptoms 
of brain damage occur in cerebral palsy and affect the 
patient's ability to function. One such motor symptom 
is apraxia, the inability to organize movement of body 
parts consistently in appropriate sequence and 
coordination to perform a useful activity. The key 
factor here is sequencing the muscles in appropriate 
order. When arm movements are involved, the term 
"constructional apraxia" is used, and when the 
muscles of speech are involved, the term "verbal 
apraxia" is used. 

Other symptoms are not related to movement, such 
as certain behavioral deficits, which can also be directly 
attributed to specific brain damage. A prototype of a 
behavioral abnormality is the hyperkinetic, distractible 
individual with a short attention span. A behavior 
pattern of this type is primarily due to a defect in the 
ability to regulate a response to a stimulus on the basis 
of its relevance or significance to ongoing situations or 
problems. Thus, a characteristic of this behavior may 
be a response made to stimuli on the basis of loudness, 
brightness, or suddenness, rather than social 
importance. The person cannot inhibit either the 
influence of the stimuli on the central nervous system 
or his responses to them. 

Another form of behavior deficit almost the opposite 
of the above is perseveration of an ongoing activity. The 



individual is unable to identify the need to stop one 
activity and attend to other stimuli clearly more 
important. 

Complications 

In describing the symptom complexes, only those 
concerned with the basic movement disorder were 
elaborated. Others, such as visual and auditory 
deficits, are also primarily due to brain damage. They 
are discussed in this section, however, because they 
compound the vocational rehabilitation problem, as do 
the true complications resulting from a primary 
symptom. 

Contractures . Limitation of passive and active joint 
range of motion (contracture) arises from a shortening 
of the muscles or the capsules around the joints (see fig. 
3-13, p. 30). The cause of contractures in cerebral 
palsy is the hyperactive stretch reflexes in spasticity. 
Activation of the stretch reflex at an increasingly 
earlier point in the range of motion makes full motion 
progressively more difficult to achieve. Capsular 
shortening usually follows muscle shortening. After a 
prolonged period of time, relief of the contracture may 
require an attack on the capsule as well as the muscle. 
The joints most commonly affected by contractures 
are the ankles, feet, hips, knees, elbows, wrists, 
thumbs, and sometimes shoulders. Contractures can 
interfere with walking, self- care, bed position, sitting 
posture, and perineal hygiene. 

Bowel and bladder incontinence . In cerebral palsy, 
bowel and bladder incontinence arises most frequently 
from inability to attend or respond to sensory signals 
indicating a need to void or evacuate. This is especially 
difficult when the response requires interruption of an 
ongoing activity. The person may ignore sensory 
signals but not on any deliberate or conscious basis. 
Rather, appreciation of the stimulus is inhibited by the 
ongoing activity. Perseveration in the ongoing task, 
even when the stimulus is perceived, may also be 
present. Discrimination amongst stimuli is a function 
of the limbic system of the brain. 

Constipation. Insufficient fluid or bulk in the diet 
and/or failure to establish and maintain a regular 
schedule for bowel movements may result in 
constipation. If the patient requires assistance, 
problems may arise from inability to attract the 
attention of the attendant or to communicate the need 
to evacuate. 

Dental problems. The same factors that caused the 
brain damage may also have affected developing tooth 
enamel. If the noxious event occurred at the time of 
tooth formation, the teeth will be less resistant to 
decay. Further, the patient's motor problems may 
make it very difficult to brush the teeth adequately and 
the diet may be insufficient to keep the teeth clean 
through chewing. Chewing and the ability to 



Cerebral Palsv 143 



manipulate food with the tongue may also be impaired. 
In addition to problems of tooth decay, cerebral palsy 
patients often have overbites as a result of persistent 
tongue thrust against the upper incisors. Patients who 
receive phenylhydantoin for seizure control commonly 
have hypertrophic gums that are prone to infection and 
inflammation. Preventive gum care requires careful 
frequent brushing, which may be very difficult if the 
patient has poor hand function. Regular, frequent 
dental prophylaxis is important. 

Osteoporosis. The formation of insufficiently 
mineralized bone sometimes results from inadequate 
diet, but more often from deficient muscle activity, 
especially after a patient has been immobilized in 
plaster for surgical procedures. Osteoporosis may 
cause pain in the feet and legs when weight bearing is 
attempted, or the bones may become fragile and 
fracture on attempts to stretch the joints with excessive 
force. Sometimes simply transferring the patient may 
break a bone. 

Degenerative joint disease. Abnormal wear and tear 
on poorly aligned joints may cause dejenerative joint 
disease (see chap. 14). It occurs frequently in the 
cervical spine in athetoid patients with poor head 
control and dystonic neck muscles. It may also affect 
hips, knees, and ankles in the ambulatory spastic 
athetoid patient. 

Scoliosis. Usually an abnormally curved spine is the 
result of poorly supported sitting posture with 
asymmetrical muscle pull on some portion of the spine. 
It may also occur in bedridden patients who are 
allowed to lie in one position consistently. Severe 
scoliosis that is associated with limited respiratory 
function may be a life -shortening factor. Pneumonia 
aggravated by inefficient cough is generally the cause 
of death. 

Malnutrition. Several factors may cause mal- 
nutrition: the patient's inability to chew or swallow 
effectively; the patient's inability to feed himself within 
the time available; "food fads;" and restricted diets. 
Further, the patient with athetosis or spasticity who 
expends relatively more than average energy to perform 
activities may receive inadequate amounts of food 
for his energy needs. 

Respiratory infections. Many patients with cerebral 
palsy, particularly those more severely involved, have 
frequent and severe respiratory infections. These 
infections are due to poor coughing ability and 
inefficient swallowing. Because of the inability to 
coordinate the larynx and muscles of respiration and 
poor muscle control generally, the patient cannot 
obtain the explosive movement of air which is 
necessary to move mucus or foreign matter up the 
bronchial tree. Aspiration of food or fluids may be 
caused by inefficient swallowing. Food may enter the 
trachea instead of the esophagus, and the patient may 
be unable to cough it up before it has acted as a foreign 



body and produced infection. Patients with cerebral 
palsy may take longer than normal to recover from an 
infection. 

Visual impairment. Severe specific damage to the 
occipital cortex results in the total loss of the ability to 
process visual information (cortical blindness). Partial 
or unilateral lesions of the occipital cortex give rise to 
visual field defects in which one side of the visual field 
is not seen and is therefore ignored (sec p. 48). 

Less severe involvement results in impaired visual 
perception or attention. Visual perceptual difficulties 
may affect appreciation of objects in space, the ability 
to sort figures from background, the ability to attend to 
one part of a page preferentially, and the ability to 
establish directionality. All of these difficulties may 
interfere with performing the activities of daily living. 
Occasionally, a patient with good physical ability will 
have such severe perceptual or perceptual -motor 
problems that he is much more handicapped than a 
severely physically involved patient. In all of these 
deficits, the problem is not in the eye itself but in the 
processing of the impulses leaving the retina. 

Auditory impairment. Hearing losses, as described 
in chapter 30, may occur incidental to the cerebral 
palsy. In addition, brain damage may also produce a 
central deafness. In this disorder, brain damage blocks 
the auditory pathways from the brain stem to the 
temporal lobe. 

Deafness of some type has been reported in about 25 
percent of cerebral palsy patients and severe loss in 
6 to 16 percent. Hearing loss may be especially important 
if the person also has some visual deficit. Even if he can 
hear well, the cerebral palsied person may have 
difficulty in interpreting auditory input due to central 
deafness. Poor head control also interferes with 
localization of sounds. 

Seizures. Seizure disorders occur in perhaps 50 to 60 
percent of patients with cerebral palsy. Seizures may 
be typical grand mal, Jacksonian, psychomotor, or 
petit mal (see chap. 12). They may be more disabling 
than the physical aspects of cerebral palsy if control by 
medication is incomplete or impossible. Control of 
seizures to a reasonable degree is therefore extremely 
necessary before other management can be successful. 

Petit mal and minor seizures may present a serious 
diagnostic problem. Seizures may be seen by the 
teacher or other professionals as inattentiveness and 
may be interpreted as willful behavior. Seizures 
causing strange behavior are especially prone to 
misinterpretation. Only good teamwork and 
interaction among medical, rehabilitation, and 
educational personnel can succeed in identifying the 
behavior correctly and referring the patient for 
appropriate treatment. 

Fatigue. Due to the motor control and coordination 
deficits, performance that is normally relatively 
automatic requires conscious effort by the cerebral 



144 Easton and Halpern 



palsy patient. This effort, combined with involuntary 
movement or excessive tension, results in the 
expenditure of an enormous amount of physical and 
mental energy to perform relatively ordinary tasks. 
Cerebral palsy patients are therefore subject to fatigue, 
which occurs sooner and is more profound than in 
normal persons performing the same task. Fatigue also 
tends to accumulate during the week, and if there is not 
adequate rest at night or on the weekends, the person 
may lose efficiency in school or on a job. 

FUNCTIONAL DISABILITIES 

Functional ability is dependent upon the interaction 
of joint movement and muscle strength; perceptual 
function; cognitive and intellectual function; memory 
function; attention and response abilities; and personal 
and social attitudes and skills. Any one of these areas 
can be so deficient or aberrant that satisfactory physical 
independence and vocational function may be 
impossible. Training and provision of assistance by 
aides and/or mechanical and electronic devices can, 
however, improve the cerebral palsy patient's 
functional level. 

Physical Disabilities 

Ambulation. Walking depends upon an ability to 
appreciate position in space; to balance the body 
segments above the feet and support them; and to shift 
weight and to move one foot in front of the other in 
sequence. Deficits in motor control and/or visuospatial 
deficits will therefore impair walking ability. Figures 
11-3, 11-4, 11-5, and 11-6 demonstrate various types 
of ambulation. 

Most hemiplegics learn to walk, as do diplegics if 
their spasticity and impairment of control are not too 
severe. Both may require one of the various canes (figs. 
11-3 and 11-4), crutches (fig. 11-5), and/or long or 
short leg braces. Quadriplegics, depending upon 
severity and type, more rarely learn to walk. They may 
be able to walk in sheltered conditions, but often a 
startle reflex, vestibular reflexes, or an insufficient 
voluntary motor repertoire interfere with ability to 
perform. 

Wheelchair ambulation requires the ability to sit 
with fair stability in the chair and to either reach and 
move the wheels with the hands, or to reach the floor 
and move the feet to push the chair. A poorly fitted 
wheelchair can even exaggerate abnormal movements, 
as shown in figure 11-7. Wheelchair adaptations, such 
as the specially fabricated contour seat in figure 11-8, 
can improve sitting posture and the ability to move the 
wheelchair. Figure 11-9 illustrates a more formidable 
approach to achieving truncal balance and wheelchair 
control. Hemiplegics may be able to propel the chair 
using one hand and one foot, or they may use a 
wheelchair with both hand rails on one side. The very 



severely involved athetoid or spastic quadriplegic 
patient is unlikely to be able to use a wheelchair 
independently. Electric wheelchairs have proven 
practical for many of these patients, though the chairs 
and the controls may have to be adapted (fig. 11-10). 
Patients with perceptual deficits will have difficulty 
steering a wheelchair properly, regardless of their 
motor ability. 

The presence of ataxia alone or, more frequently, in 
combination with athetosis or spasticity, severely limits 
the functional possibilities and prospects for walking or 
wheelchair travel. 

Transfers. For patients who can walk, transfers may 
be totally independent except transfers in and out of 
the bathtub; in situations where balance depends on 
specific muscle groups that are under poor control; or 
where contractures prevent clearing of a leg or arm 




FIGURE 1 1-3. This man with left hemiparesis and some moder- 
ate spasticity is able to walk with a cane in his right hand and no 
leg braces. 




FIGURE 1 1-4. Even with a broad-base cane, this man with right 
hemiparesis, spasticity, and apraxia has poor balance because 
he has trouble learning the appropriate motions to maintain 
equilibrium. He requires a wheelchair for more than a few steps 
across the room. 



Cerebral Palsv 145 




FIGURE 1 1-5. This man with spastic diplegia can walk indepen- 
dently, using a lour-point gait and Kennv crutches. His gait 
deteriorates to swing-through when he hurries. 




FIGURE 1 1-7. In a poorly fitting wheelchair.this man has poor 
trunk control. Note his exaggerated facial grimace as he 
attempts to use his arm to right himself. 




FIGURE 1 1-6. With a left short leg brace, this man with athetosis 
and abnormal appearance has good standing balance and is 
able to walk long distances independently. 

across an obstacle. Patients with visuomotor problems 
will have difficulty orienting supports properly to aid in 
transfers. Generally, the spastic diplegic patient who is 
unable to walk has sufficient upper extremity function 
to allow independent sliding-board or pivot transfers. 
Moderately involved individuals who are able to 
support some weight on the lower extremities and to 
balance a little can be transferred by a pivot transfer 
and can be managed by one assistant with ease unless 
ataxia is extreme. The most severely involved patients 
will require two -person lift transfers, being unable to 
assist at all. 

Eating. Eating independence is achieved by most 
mildly and moderately affected individuals. The more 
severely involved may develop some abilities to feed 
themselves but require adapted equipment. Those 
patients who have difficulty with the manual 
components of motor control in self- feeding activities 



A 


IIJ 


**"t^S 


^^gV"-— — — ■"! 




t F* 


5^ 




FIGURE 1 1-8. (Left) Without adequate seat support, this man is unable to maintain erect posture in his wheelchair because of poor 
truncal balance. (Right) A specially fabricated contour seat, which is shown on the regular chair, ( Left), provides adequate support and 
improves the man's posture, function, and appearance. 



146 Eastern and Halpern 



will not be able to feed themselves. 

Some eating difficulties are due to poor lip, tongue, 
and throat control, resulting in slow eating and poor 
chewing and swallowing ability. Careful technique in 
assisted feeding is required to prevent the patient from 
either aspirating the food or pushing it back out of the 
mouth by tongue thrust. Visuomotor problems often 
interfere with self- feeding by making the purposefully 
coordinated sequence of motions for scooping and 
carrying the food without spilling extremely difficult 
to achieve, even though the specific hand motions are 
within the capability of the person. Self- feeding 
problems due to visual acuity deficits can often be 
overcome with training. 

Dressing. Independent dressing will be possible for 
mildly and moderately involved patients, regardless of 
disability type. Moderately involved patients will 
require more time and more adapted clothing and will 
function less well with tension or hurrying. A patient 
may be able to dress himself in most circumstances but 
prefer assistance, rather than expending the necessary 
time and effort in dressing himself, if he must get to a 
job at a certain time. 

Contractures, particularly at the shoulder, knee, 
and hip, can make dressing more difficult. Also, an 
impairment of visual perception might make it difficult 
for the person to orient a shirt properly, front to back 
and right side out, even though the physical ability is 
adequate. 

Personal hygiene. Most hemiplegics without severe 
sensory disturbance will be independent in personal 
hygiene activities. Some patients may be independent 
except for certain transfers, such as in and out of the 
bathtub, but may become completely independent 
with minor modifications or adapted equipment. 

Impaired upper extremity dexterity and poor sitting 
balance interfere with the ability to maintain 
satisfactory perineal hygiene, and hip, shoulder, or 
elbow contractures may make this quite impossible. 
The more severely involved patients will be totally 
dependent in this area. 

Language and Communication Disabilities 

The development of language and communication 
skills from infancy depends upon an intact hearing 
mechanism, an intact brain, an environment of peers 
and adults in a series of interactions of ever - increasing 
breadth and depth, and sufficient motor skills to allow 
the person to be present in many different settings. 
The person with cerebral palsy may be deficient in all 
four areas from birth. In cerebral palsy, the speech 
output may range from normal or nearly normal to 
complete lack of speech. The average incidence of oral 
communication disorders among cerebral palsied 
individuals has been estimated at 70-80 percent. 




FIGURE 1 1-9. With a molded plastic body jacket, this patient is 
comfortable, able to run her wheelchair, and use her com- 
munication board (she is nonverbal). 




FIGURE 11-10. This patient with spastic triplegia has reasonably 
good function only in his right arm. In an electric wheelchair, he 
is fullv mobile. 

Identification of the causes of the disabilities is not 
an easy task. The first level is determining whether the 
person has a hearing impairment (see chap. 30). Then 
it must be determined whether the brain damage may 
be so severe that the person does not recognize the 
social need to communicate. Assuming some 
recognition of this need exists, the next determination 
is whether there is capacity for symbolic language, 
either nonverbal (e.g. , pantomime) or verbal. In some, 
the retardation is such that no symbolic 
communication is possible and only more concrete 
responses are present (e.g., pointing at a desired 
object). Of course, both concrete and symbolic 
communication require some motor ability to point, 
look, or vocalize in order to attract sufficient attention. 

Verbal communication requires the capacity for 
symbolic language plus the complex motor skills 



Cerebral Palsy 147 



necessary to vocalize sounds and words with 
appropriate clarity. Nonverbal communication 
similarly requires the capacity for symbolic language, 
but an alternate means of expression is used. Verbal 
and nonverbal communication in cerebral palsy are 
not necessarily equally impaired. When verbal skills 
are impaired but nonverbal skills are not, manual or 
visual language systems may be used. Communication 
substitutes, such as the Bliss and Rebus systems, 
require intact visual perceptual skills, and the manual 
sign language used by the deaf requires motor skills. 

When a verbal person who is not trained in nonverbal 
methods communicates with those who have only 
nonverbal skills, there is a tendency to take shortcuts, 
to restrict communication to concrete immediacies, 
and hence to limit the verbal exposure of the 
developing child. Thus, basic language, social, and 
intellectual development is more limited than it could 
be under more optimum management. 

The breadth of the potential communication 
problems in the adult with cerebral palsy seem, 
therefore, to include: 

1. Hearing disorders 

2. Auditory comprehension problems 

3. Visual comprehension problems for verbal and 
nonverbal input 

4. Insufficient experiences 

5. Distractability 

6. Limited intelligence 

7. Weakness, slowness, and/or incoordination of 
speech mechanism movements 

Psychosocial Disabilities 

Brain damage may result in deficiencies in one or 
more of the four basic cognitive abilities: verbal 
receptive, verbal expressive, visual perceptual, and 
visual motor. Visual motor skills are not identical with 
the movement disorder but refer rather to creative 
construction. Visual perceptual functions include such 
activities as figure -background discrimination and 
spatial orientation. The verbal abilities and associated 
communication impairments have already been 
discussed. 

Behavior deficits, such as the stimulus -bound 
response and inadequate discrimination among 
stimuli, are reviewed above. Task preservation and 
rigidity in approach to problem solving may also mark 
the behavior of a person with cerebral palsy. Easy 
distractibility, dependency, and manipulative behavior 
can further compound the disability. Short attention 
span and short-and long-term memory impairments 
may also be present. 

The combination of cognitive and behavioral deficits 
may be viewed as components of the learning 
disabilities present in cerebral palsy. Deficits in 



acquisition, retention, interpretation, and application 
of information, coupled with inadequate training, lead 
to relatively low levels of achievement. 

By the time they reach adulthood, cerebral palsied 
persons generally still have inadequate social skills. 
Reduced experiences and fewer interpersonal contacts 
are consequences of diminished mobility and social 
isolation. Contact with nondisabled peers will have 
been very restricted, especially if the person attended a 
school for handicapped children. 

Another general consequence of an individual 
growing up with a disability is the qualitative difference 
in the kind of social relationships experienced. Other 
persons, in relating to the cerebral palsy patient, are 
inclined to present a fairly consistent pattern of 
support, assistance, and reduced expectations. The 
patient may actually develop the attitude that he is the 
"center of the universe." Consequently, basic 
principles of interaction, such as giving as well as 
receiving, are not within his developmental 
experience. He may not recognize the need to 
take some responsibilitv personally in social 
relationships, but rather expect the world to come to 
him regardless of how he responds. Relationships are 
often perceived in only two dimensions, as directed 
either toward or away from himself. Persons who 
direct themselves toward him are perceived as helpful, 
and those who direct themselves away from him are 
perceived as hostile. This egocentricity is often 
manifested as excessive dependency and self-centered 
behavior. These behaviors, often acceptable in a child, 
are objectionable in an adult. Because these social 
behaviors have developed and have been reinforced for 
15 to 20 years for many handicapped adults, they are 
strongly entrenched and resistant to change. 

In addition to these general deficiencies in social 
relations, cerebral palsied persons also have specific 
difficulties in developing sexual relationships. They 
experience normal desires, but lack the skills or 
training to satisfy those desires. They also have limited 
access to and opportunity for sexual experience. The 
person with cerebral palsy has many barriers to 
overcome in the development of a sexual relationship: 
physical barriers due to physical disabilities; social 
barriers due to society's mores and conventions of 
sexual preferences (e.g., physique and cosmesis); and 
personal barriers due to the inadequate knowledge of 
both biological facts relating to sexuality and the 
accepted social concepts of relationships to peers of the 
opposite sex. 

Rehabilitation Potential 

The person with cerebral palsy has a stable lesion. 
Brain damage does not increase with age, and 
therefore long-term planning is appropriate. As the 



148 Easton and Halpem 



cerebral palsied person reaches adulthood, his total 
physical, psychosocial, and language abilities will be 
maximal only if prior total treatment was exemplary. 
The counselor may well find this not to be the case for 
his client, and full realization of a client's rehabilitation 
potential may not be present. Treatment intervention 
may be necessary. 

Preservation of skills and abilities is dependent upon 
repeated regular practice, avoidance of enumerated 
complications, and effective followup. In middle age, 
the client may not have as much endurance as his 
nondisabled peers for the performance of the 
demanding kinds of work that were performed at a 
younger age because of a relatively greater decrease in 
total available energy. 

A realistic appreciation of the status of the disability, 
while important for all diseases, is particularly' 
important in rehabilitation planning with cerebral 
palsy clients. The inexperienced background and 
learning disabilities of individuals with cerebral palsy 
may have interfered with their own appraisal and 
recognition of their abilities. Expectations may not 
match abilities. Further, the client may not appreciate 
the breadth of skills needed for a particular occupation. 
It may be necessary to assist the client's comparison of 
his special repertory of skills with those skills required 
by a potential occupation. 

Rehabilitation potential for the person with cerebral 
palsy is affected by a number of factors in addition to 
the physical, communication, and psychosocial 
problems present. These factors include maintenance 
therapy required, living arrangements required, and 
the client's desires and attitude toward employment. 
Rehabilitation potential may be further hindered by an 
unattractive appearance or the physical need for 
attendants to assist with personal care. These problems 
must be taken into account in preparing a 
rehabilitation plan. 

In addition to treatment of physical problems, 
mature social skills may need to be learned from the 
beginning. The sexual frustrations of the young person 
may often need to be addressed before it is possible to 
deal with other emotional problems and begin training 
for a job. Negative attitudes toward employment and 
dependent attitudes toward the world need to be 
overcome before employers can be satisfied with an 
applicant with cerebral palsy. 

An important element in the successful realization of 
potential is the availability of an adequate range of 
placement and support opportunities. The greater the 
spectrum of possibilities within the community, the 
better the potential. The existence of sheltered 
workshops, modified work opportunities, selective job 
placement, day activity centers, supported residential 
facilities, group homes with community recreational 



and educational facilities, and foster homes all enhance 
the client's potential. 

Despite the many obstacles to successful 
rehabilitation of the client with cerebral palsy, it is 
worthwhile working with even the most severely 
involved persons to try to establish some kind of 
vocational or avocational activity leading toward a goal 
of maximal functioning and life satisfaction. 

STANDARDS OF EVALUATION 

Depending on the nature and severity of his 
problems, evaluation of the person with cerebral palsy 
may require more disciplines working at a more 
sophisticated level than is required in most other 
disabilities. It can also take more time than average to 
get an accurate evaluation of the individual's abilities. 
A complete assessment of functional status requires 
medical evaluation and evaluation of physical 
function, communication, and psychosocial 
development. 

Medical Evaluation 

A neurologist should assess neurological status 
including, where appropriate, the identification of 
seizure activity. The latter may require brain wave 
analysis (EEG). Nutritional requirements and 
adequacy of diet should be reviewed by a nutritionist. 
Examination by a dentist is essential to identify dental 
problems. Not every dentist will have the capacity, 
interest, and physical facilities to provide evaluation 
and treatment. A search may have to be made. 
Orthodontic evaluation for overbite should also be 
performed. Visual impairments should be evaluated by 
an ophthalmologist (see chap. 29), and hearing 
impairments should be evaluated by an otologist and 
an audiologist (see chap. 30). Evaluation of other 
organ systems should be done by an internist, with 
special attention given to problems of recurrent 
infections. The achievement of the above, while 
perhaps possible sequentially, may best be performed 
in a central setting where interest in cerebral palsy 
exists. 

Functional Evaluation 

The physiatrist, physical therapist, and occupational 
therapist working together can evaluate the patient's 
motor repertory and identify the voluntary, 
involuntary, and accuracy control aspects of his 
movement characteristics. They will also assess 
contractures and skeletal deformities and, where 
appropriate, refer the patient to an orthopedic surgeon 
for further evaluation and possible recommendations 



Cerebral Palsy 149 



for surgery. Language and swallowing ability should 
be evaluated by a speech pathologist, who can identify 
problems and recommend therapeutic approaches. 



Psychosocial Evaluation 

Cognition. Intellectual evaluation is a particularly 
difficult area in cerebral paJsy. Ideally, intelligence 
testing should provide as specific a profile as possible of 
strengths and deficiencies in the four major areas of 
intellectual functioning: visual perception, visual - 
motor performance, verbal reception, and verbal 
expression. For this purpose, the standard intelligence 
tests require analysis, modification, and adaptation in 
their application and interpretation by a psychologist 
particularly experienced in working with cerebral palsy 
clients. Standard IQ testing may not be particularly 
rewarding or useful. 

To separate cognitive function from motor function, 
time -dependent criteria may have to be ignored or 
other means of ascertaining a response may be 
advisable. Multiple tests may be required to answer 
specific questions, since tests differ in the relative 
emphasis on certain cognitive functional abilities. It is 
most important to make a distinction between the 
ability to process visually mediated information and 
verbally mediated information. Further, if 
performance is impaired, it is important to make a 
distinction between difficulty in understanding or 
carrying out the perceptual and conceptual elements of 
a task and difficulty in executing the response. Finally, 
a distinction should be made between the creative 
constructional aspects of the response and the strictly 
motor abilities required. 

Behavior. Certain generalized difficulties in 
attentive skills exist in cerebral palsy which contribute 
strongly to learning disabilities. Therefore, careful 
observation and analysis of performance should be 
carried out to identify attention duration, short 
auditory memory, distractibility, perseveration, or 
inability to select and respond to stimuli on the basis of 
their relevance rather than their physical charac- 
teristics. Impairment of these functions is often 
responsible for failures otherwise attributed to 
cognitive, intellectual deficit. Appropriate analysis will 
identify a remediation strategy likely to produce 
improvement. 

Personality assessment to identify the patient's 
behavior patterns, personal attitudes, problem -solving 
strategies, and level of self-esteem may be critical. The 
patient's interactions with peers, authority figures, 
family members, groups, and individuals should also 
be evaluated. The psychologist and/or social worker 
can identify problem areas and recommend appro- 
priate remediation approaches. 

Evaluation of a condition with all the complexities of 



cerebral palsy can be carried out effectively only in a 
context of total management. Since evaluation is an 
intrinsic component of management in cerebral palsy, 
it will be appreciated best by the discussion in the 
following section. 

TOTAL TREATMENT 

Medical Treatment 

Drugs. A number of medications are in use for the 
control of excessive muscle tone and to induce muscle 
relaxation. The most common are diazepam (Valium) 
and dantrolene sodium (Dantrium). Occasionally, 
some of the phenothiazines (see p. 255) or other 
tranquilizers are used to assist in management of the 
excessive muscle tone that may be related to 
generalized central nervous system hyperactivity. 
Levodopa (see p. 87) is also in use for the control of 
excessive muscle tone with some suggestive evidence 
that it may be of value in cerebral palsy in certain 
instances. Other medications are very likely to become 
available. Baclofen, for example, is a new drug for 
excess muscle tone that has not yet been fully evaluated 
for use in cerebral palsy. 

These drugs have a number of side effects. An 
unfortunate side effect of diazepam and the 
tranquilizers, in some cases, is not only lethargy but a 
true affective depression which will limit their 
usefulness. Affective depression is occasionally a 
disturbing side effect of levodopa as well. Dantrolene 
sodium is often associated with a feeling of lassitude 
and other subjective signs of discomfort, such as 
nausea and fatigability. In addition, there are unusual 
but serious occurrences of liver damage, which require 
careful medical followup as long as the patient receives 
the medication. Drugs likely to be prescribed are for 
use indefinitely. Regular medical attention to monitor 
side effects, ensure compliance, adjust dosages, and 
consider newer preparations must be assured. 

Neurolytic procedures. For spacticity, in particular, 
interruption of reflex pathways by blocking nerve 
impulses is helpful to achieve improved ambulation, 
upper extremity function, and posture. They are 
usually done at the point where nerves enter the 
muscles (intramuscular neurolysis). Phenol or alcohol 
injections may be used and repeated if necessary. 

Surgery. Orthopedic surgery is relatively effective in 
reducing muscle spasticity. Usually a muscle or tendon 
lengthening procedure is performed, which effectively 
decreases stimulation of the stretch reflexes. This form 
of surgery is not as consistently effective for the 
excessive tone of athetoid dystonic patients. Various 
other orthopedic procedures are done to correct 
deformities in the feet, hips, and wrists. 

Recently, the implantation of an electrical stimu- 
lator in the cerebellum has been introduced to achieve 



150 Easton and Halpern 



muscle relaxation. While some dramatic results have 
been claimed and demonstrated, the evidence is insuf- 
ficient to define the specific types of problems for which 
successful results can be consistently expected. 
Neurosurgical procedures to produce destructive 
lesions in precisely determined locations in the basal 
ganglia or thalamus regions of the brain (see chap. 3) 
have also been advocated to control undesired 
movements or rigidity. Results vary, and dependable 
criteria have not yet been established which will predict 
a reasonable rate of success for any special 
neurosurgical procedure. With drugs, neurolysis, or 
surgery, motor skill and balance training are often 
required to develop improved function with the altered 
neurological mechanism. 

Treatment of Complications 

Contractures. Management of contractures is best 
applied preventively, rather than after deformities 
have developed. The techniques available for control of 
contractures are passive stretching, orthopedic surgical 
procedures, intramuscular neurolysis, and bracing. 
Where passive stretching cannot be done or is 
ineffective because tone is too persistent and too 
strong, surgical tendon or muscle lengthening 
procedures at an early stage should be performed. 
This is particularly important around the hips to 
prevent dislocation and to facilitate perineal hygiene 
and toileting. 

Bowel and bladder incontinence. Bowel incontinence 
may be managed by a regular evacuation schedule, 
dietary control, and, on occasion, medications. 
Training the individual to become more aware of 
toileting needs must proceed concurrently. Bladder 
incontinence caused by the inability to pay attention to 
bladder stimuli may respond to training in attention to 
significant stimuli. Incontinence may also often be 
controlled through training of attendants and 
development of a communication system between 
them and the patient. 

Constipation. Adequate bulk and liquid in the diet 
and a regular evacuation schedule are essential to 
prevent constipation. Given the problems in respon- 
siveness and physical difficulties in accomplishing 
self- care tasks, sufficient time needs to be allowed 
to evacuate the bowel completely. Often a morning 
time is not really practical, and an evening before - 
bed routine will allow adequate time and attention for 
successful management. Harsh, irritant laxatives are 
not advisable since they tend to make the bowel more 
spastic and add to the problem by causing dehydra- 
tion. Further, they ultimately diminish the sensitivity 
of the bowel to stimulation. 

Dental problems. Careful training in oral hygiene 
measures is required. If the patient is unable to assume 
full responsibility for oral care, assistance must be 



provided. Inaccessibility of dental care is often a 
serious problem. This may be the result of the patient's 
inability to cooperate, his inability to get to an upstairs 
dentist's office, or the inability of the dental office to 
master specific techniques of managing the 
hyperresponsiveness of patients with cerebral palsy. 
For some situations, it may be necessary to admit the 
patient to the hospital and do the dental work under 
anesthesia in order to have time and ideal conditions to 
perform needed work. 

Osteoporosis. Osteoporosis may be minimized by 
maintaining weight bearing and encouraging muscle 
activity, and maintaining a normal dietary intake of 
calcium, vitamin D, and protein. Complications of 
osteoporosis, such as fractures, may be avoided by 
awareness of its presence, care in handling, and careful 
graduated restoration of muscular activity to the 
osteoporotic bone. 

Degenerative joint disease. Cervical degenerative 
joint disease can be minimized by posture control 
lessening the amount of neck movement and tension. 
This can sometimes be accomplished by head support 
and training, assisted by a physical therapist. 
Otherwise, traction or sometimes a supportive or 
restrictive neck collar is used. Aspirin and other pain 
relievers may be indicated, but should be used with 
care so they do not lead to more stress on the joints as 
pain is relieved. Obesity may contribute to degenera- 
tive joint disease, and a reducing diet successfully 
lessens pain and improves function. 

Scoliosis. Bracing and splinting are used to prevent 
persistent abnormal postures tending to deformities. 
Spine deformities can be delayed by appropriate 
bracing, use of a body jacket, or wheelchair 
modifications. Corrective orthopedic surgery may be 
necessary to stabilize the posture of these patients to 
improve both their function and appearance. 

Respiratory infections. Respiratory infections may 
be prevented to some degree by having the patient 
sleep on his face so that saliva drains outward at night 
rather than pooling in his throat. Careful feeding 
technique will avoid choking or leakage of food into the 
lungs (aspiration). This may require the combined 
skills of the rehabilitation nurse, speech pathologist, 
and occupational therapist, working with the help of 
the physiatrist. In extreme cases, the use of a feeding 
tube directly inserted into the stomach (gastrostomy) to 
bypass swallowing may dramatically lessen repeated 
respiratory infections. Occasionally, tonsils become 
inflamed and chronically infected and need to be 
removed, even in the adult, to reduce a source of 
continuing infection and a hindrance to swallowing. A 
speech pathologist or a physical or respiratory therapist 
can assist in the development of improved breathing 
patterns, coughing, and lung expansion. Where a poor 
cough is a problem, respiratory assistance and suction 
may be needed to avoid the pneumonia resulting from 



Cerebral Palsv 151 



otherwise simple upper respiratory infections. 

Seizures. Where seizures have been identified, a 
neurologist will prescribe seizure-control medications 
appropriate to their type. Chapter 12 reviews the 
medication approaches used for the different seizure 
types. 

Fatigue. Management of fatigue will include 
adequate nutrition, minimized emotional components 
on the job, and assessment of the total demands on the 
client in order to set priorities with the client for the use 
of the total available energy. The client's physician 
can assist in setting up a disciplined program of activity 
which includes a period of rest during the day. 
Avocational as well as vocational activities should be 
monitored so that the client gets enough rest on the 
weekends and in the evenings. Part-time rather than 
full-time work may be necessary to achieve a balanced 
total life. 

Where attentiveness is a problem in controlling 
fatigue, additional medication, such as dextro- 
amphetamine, Methylphenidate, or the tricyclic 
antidepressants, may improve the client's total energy 
level and make it possible for him to attend more 
readily to his work. Factors in fatigue, such as the need 
to concentrate, the need to pay attention, the need to 
do things the hard way (i.e., unassisted) where an easy 
way might be possible, must all be considered when 
managing the client's total energy output in a training 
or work situation. 



Rehabilitation Treatment 

Rehabilitation in cerebral palsy covers a wide range 
of activities. Treatment and training are required to 
improve motor control and physical function, 
language, communication, learning abilities, and 
social skills. This total rehabilitation program requires 
interaction among many rehabilitation specialists and 
is best performed in a comprehensive rehabilitation 
setting. Depending on the breadth or complexity of 
disabilities, an initial inpatient treatment phase may be 
required. 

Physical Junction. Close collaboration is required 
among the physiatrist, physical therapist, and 
occupational therapist in establishing and executing a 
motor training program. In addition to their analysis 
of the patient's motor repertory, information gathered 
from other disciplines is incorporated into planning the 
program. Information from the psychologist regarding 
the patient's sensory, perceptual, and cognitive 
processing abilities is required to plan the most 
appropriate and useful feedback modalities by which 
the patient can be made aware of the consequences of 
his performance efforts. Behavioral information from 
the psychologist, school teacher, rehabilitation nurse, 
and others on the patient's attention span, 
distractibility, memory characteristics, and 

communication abilities is also required to plan the 



details of the management and training techniques. On 
the basis of this information, decisions can be made 
regarding the extent to which situational structure can 
be used to enhance attentiveness and learning, and to 
what extent medication is required to improve 
attentiveness, diminish stimulus-bound behavior and 
perseveration, and increase the patient's ability to 
respond to stimuli from within. 

In practice, training of motor skills is carried out by 
breaking up a desired voluntary motion into small 
component kinesiological segments, according to the 
individual's ability to attend, observe, and control the 
activity. At the same time, undesirable involuntary or 
reflex activities are brought to the attention of the 
patient, and he is trained to inhibit these motions. 
Criteria are set to determine success within the 
observed ability of the patient. Verbal, visual, tactile, 
and proprioceptive feedback, as appropriate, is given 
to identify clearlv to the patient the character or quality 
of his performance. 

Improvement of ambulation may have several 
components. Prescription of diazepam or dantrolene 
sodium may help relieve dystonia. The surgeon may 
help in improving mechanics, the physiatrist may help 
in performing intramuscular neurolysis, and adequate 
training by the physical therapist in walking techniques 
may make walking practical. 

Where walking is not feasible and wheelchair ambu- 
lation must be considered, ajoint decision to obtain a 
wheelchair must be made by the physiatrist, physical 
therapist, occupational therapist, and rehabilitation 
counselor working together. Each may have recom- 
mendations for special adaptations or equipment for 
optimum usefulness. Often the social case worker and 
the rehabilitation nurse who observes the patient's 
daily self-care functions may have important contribu- 
tions to the decision. Once the chair is obtained, it 
must fit, provide the patient with adequate lateral sup- 
port, have a seat belt for safety, and be suited to the 
patient's abilities to propel the chair and transfer him- 
self into and out of it (see fig. 1 1-5). A period of training 
by the physical therapist, occupational therapist, and 
nurse in use of the wheelchair is indicated to be certain 
of optimum utilization and to verify its appropriate- 
ness in practice. 

Improvement of the ability to accomplish transfers 
may begin with the surgeon correcting contractures or 
the physiatrist prescribing braces to allow accurate foot 
placement and ankle stability. The physical therapist 
and nurse then cooperate in working out the most 
useful method for the patient's special needs and 
assisting the patient in learning the method. They may 
also recommend adapted equipment, such as a steady 
seat in the bathtub and handrail or a commode in the 
shower. Nonslip surfaces are extremely important in 
reducing danger and anxiety. If the abilities of the 
patient necessitate a goal that is less than independent, 
this goal needs to be defined and clearly established 



152 Easton and Halpern 



and stated, with all disciplines working along parallel 
lines. 

For improving function in eating, a whole -team 
approach is required. The speech pathologist can 
provide oral training, the occupational therapist can 
provide adaptive motor- skill training and adaptive 
devices (such as a special spoon with a built-up handle 
or holding strap, or a splint to stabilize the wrist to 
facilitate scooping of food and bringing it to the 
mouth), the physical therapist can provide training in 
balance and coordination, and the nurse can assist with 
practice in practical application of techniques. 

For independence in dressing, team cooperation 
may again be required. Assistance from the orthopedic 
surgeon may be necessary to achieve optimum motor 
function by eliminating contractures. The occupational 
therapist can provide adaptive devices to assist where 
hand function is deficient and can recommend clothing 
modifications to facilitate independence. A physical 
therapist may be required to assist with the development 
of sitting balance and moving ability. The nurse may 
be involved in the teaching and practice of dressing 
techniques. 

Training in personal hygiene is usually carried out 
by the nurse, although it may be necessary to enlist the 
aid of the occupational therapist for assistance with 
manual skills, adaptive techniques, and assistive 
devices, and the physical therapist for such needed 
skills as balance, transfers, hand and arm control, and 
standing. Further, it may be necessary to treat hip 
adduction contractures to allow adequate perineal 
hygiene. 

It should be emphasized that the patient with 
cerebral palsy may need to adapt a personal hygiene 
system to his own particular needs. For those who 
perspire heavily during the night, it may be advisable 
to schedule the bath time in the morning so that 
offensive body odors are not a problem in a work 
situation; for others, a morning schedule may be too 
brief to enable them to arrive at work on time. In 
general, the more time-consuming elements of 
personal care are best scheduled in the evenings. 

Language, communication, and learning. Again, a 
combined approach is needed to help the person with 
communication disabilities to function optimally. 
Where language concepts are inadequate, remedial 
work with a teacher and language therapist is 
necessary. The basic concepts may need to be taught 
and later elaborated with emphasis on grammar, 
syntax, and accurate expression. It may be necessary, 
for purposes of communication with fellow workers, to 
teach the cerebral palsied individual some "street 
language" and the meaning of certain colloquial 
expressions, since these have often not been part of his 
past experiences. 

Nonvocal patients may have difficulty utilizing 
adaptive equipment in order to communicate. The 



assistance of an engineer familiar with communications 
technology is often invaluable to adapt available equip- 
ment for special needs, to suggest new techniques in a 
rapidly changing field, or to help in maintenance of the 
specialized equipment. A teacher, speech therapist, and 
occupational therapist working together may be needed 
to determine the best means of communication, to train 
the person in its use, and to help him practice it until, 
with improved communication, his language skills also 
improve up to a practical level. Because these are such 
skilled activities, daily practice is necessary if they are to 
be maintained. All members of the team, both 
professional and nonprofessional, including the family, 
need to be fully informed of the current level of 
language being employed, so that daily application of 
communication skills in a meaningful context is 
assured. 

Some individuals have a severe verbal language 
problem rather than, or in addition to, a physical 
inability to vocalize or speak. These persons will not be 
able to use either manual or electronic spelling boards, 
word boards, or devices that utilize the written or 
printed word. Some can develop a visually mediated 
language, and a pictorial system like the Rebus or a 
symbolic ideographic system like Bliss symbolics may 
be most useful and rewarding. 

Medication may help alleviate difficulties in 
attentiveness which contribute to learning disabilities, 
at least on a temporary basis until skills in attending 
are learned or the necessary structured environment is 
established. 

The teacher, occupational therapist, psychologist, 
and behavior analyst will cooperate in working on a 
patient's perception of spatial orientation. Ways of 
compensating for visual -perceptual problems may be 
worked out, or auditory methods of learning may be 
substituted. If the patient's auditory memory is short, 
instructions and information must be given in small 
units, thus allowing digestion and filing before 
proceeding further to the next unit. 

Most of these problems should have been dealt with 
fairly adequately by the school system prior to the 
person's entering vocational training. If they have not, 
it is still not too late to give them some attention and at 
least try to improve function in these areas, since they 
are necessary skills if the person is to be successful 
vocationally. In any case, an accurate knowledge of the 
specific cognitive abilities and weaknesses is important 
for appropriate vocational training and placement. 

Social Junction. The management of problems in 
social functioning involves assessment of what the 
patient is doing that is unacceptable; determination, if 
possible, of the reason for the behavior; making the 
patient aware of what he is doing and perhaps why he 
does it; removing rewards for unacceptable behavior; 
and finally, training in ways of interacting that are 
acceptable and desirable. For a time, this process may 



Cerebral Palsv 153 



involve the whole environment in the treatment 
process. There is need for a social therapist, who may 
be a recreational therapist, a social worker, a 
psychologist, or simply a lay person who is able to 
accept the handicapped person's disabilities, to be 
honest with him in informing him when he is being 
unacceptable, and to be willing to help him learn 
appropriate social skills. Teaching the person to take 
responsibility for his own actions and for his own social 
and emotional life within his capability level is an 
essential part of developing social adaptability. 

When social problems result from the patient's short 
attention span and /or problems of perseveration and 
rigidity in approach to problem solving, it is necessary 
to recognize the organic component of the problem. 
Medications may be necessary for management in 
many instances. In all cases, strong, consistent social 
structuring is required to maintain circumstances 
within the capability of the individual to respond, to 
provide the requirement to respond appropriately, and 
to guarantee appropriate reinforcement to modify the 
behavior. Simple verbal counseling or instruction is 
not adequate to cope with these strong behavioral 
characteristics. 

It is necessary for the patient to learn social skills 
before sexual information can be effectively utilized. 
Simple training in conventional manners often may be 
needed. The patient may lack rapid, easy 
communication and he may not use the conventions 
like "please" and "thank you" which make the world 
a little easier for people to live in. Certain individuals 
may need adapted concrete formulas for such social 
routines in order to function satisfactorily in the 
community. These attitudes and skills are learned 
through experience and effort. The experience 
necessary to acquire appropriate social skills must be 
introduced by careful structuring into the daily life of 
the individual, with adequate, appropriate, and timely 
reinforcement. Supportive counseling for the 
individual can then be useful. The significant other 
individuals in his life must be included as well. 

Careful structured experience and counseling are 
required to alter the egocentric outlook common to 
cerebral palsy patients. This outlook may not be 
amenable to treatment if approached too late in life, or 
if family and peer relations cannot be modified. With 
careful situational management and teaching, even the 
mature patient may still be able to acquire a veneer of 
sociability that allows him to function satisfactorily, in 
spite of still considering himself the center of the 
universe. 

VOCATIONAL IMPLICATIONS 

Some clients with cerebral palsy will be able to enter 
competitive employment with very little special help. 
Others, in spite of maximum help, will not really be 
vocational candidates, but will need assistance to 



achieve avocational interests and adequate living 
arrangements. Where seizures are present, the 
vocational factors discussed in chapter 12 apply. 
Attention to the detailed requirements for optimum 
vocational function can suggest those physical 
adaptations, equipment modifications, and assistance 
required for a balance between client abilities and 
performance requirements. 

Vocational considerations should begin with a 
realistic appraisal by the client and the vocational 
counselor of the client's actual physical, mental, social, 
and psychological abilities, as developed by appro- 
priate evaluation and total treatment. It is, of course, 
axiomatic that each individual's personal interests and 
aspirations need to be taken into account. It is the task 
of the counseling psychologist to match the client's 
interests and abilities. Because of the limitations and 
distortions in life experiences of cerebral palsy 
clients, their interests may not be mature and are 
likely to be inappropriate. An organized experiential 
plan may be required for certain individuals to 
arrive at an appropriate articulation of their abilities 
and interests. To accomplish this goal, the fullest 
resources of the entire rehabilitation team, including 
the educational system, vocational training centers, or 
other available community resources, may be 
required. 

Where lack of motivation makes constant 
supervision necessary, occupations where the client 
must provide most of his own discipline or initiative 
will not be appropriate, regardless of physical ability. 
A person with a hearing disability, receptive language 
deficit, or short auditorv memory will not succeed in an 
occupation where he must take messages from other 
people or where long, complicated instructions must be 
absorbed quickly. On the other hand, if visual - 
perceptual and visual -motor skills are intact, the client 
may be successful in work utilizing graphic 
representational skills, such as preparing and 
maintaining blueprints. The client with a good deal of 
spasticity or who requires intense concentration to 
perform a carefully trained activity may not be able to 
work full time, may need to take a rest in the middle of 
the day, or may not be able to continue with a very 
demanding full-time occupation as long as a normal 
person and may have to consider a midlife career 
change. 

Work environment may be very important. For 
example, a distractible person may be able to perform 
a task quite well if provided with equipment set in a 
corner rather than out in the middle of a busy office. In 
general, the cerebral palsy client is most likely to be 
successful in indoor settings with a minimum of 
architectural barriers. Temperature extremes may 
aggravate spasticity. Jobs involving interactions 
primarily with regular employees, rather than always 
with new people, will be less stressful. 

A period of extended work evaluation may be the 



154 Eastern and Halpern 



best initial "job placement" after a study of the client's 
abilities has been achieved in treatment and initial 
counseling. Extended work evaluation can assist the 
client in developing work habits and a realistic 
appreciation of skills prior to actual job placement. 
Following the initial placement, care must be taken to 
ensure that the client has the opportunity to progress 
up the employment ladder as job experiences may 
allow. Employment below developing abilities should 
not be allowed to continue indefinitely. Even though 
the brain damage is present, growth in vocational 
potential with increasing success should be expected. 

Clients with cerebral palsy present the rehabilitation 
counselor with some of his most difficult challenges. 
However, once the myriad of problems have been dealt 
with and adequate support given during the first 
months or even years of vocational experience, the 
effort will usually have been very worthwhile. 

BIBLIOGRAPHY 

Allen RM, Jefferson TW: Psychological Evaluation of the Cerebral 
Palsied Person: Intellectual, Personality, and Vocational 
Applications . Springfield, IL, Thomas, 1962. 
This is a list of tests suited to the assessment of intellectual 
and perceptual function of individuals with cerebral palsy. 
Includes some suggestions for adaptation of the tests for 
individual handicaps and modifications of interpretations. 

DenhoffE, Robinault IP: Cerebral Palsy and Related Disorders: A 
Developmental Approach to Dysfunction. New York, McGraw- 
Hill, 1960. 

A survey of cerebral palsy, with emphasis on the 
intellectual and perceptual handicaps. Also includes 
evaluations of physical functioning and a review of the 
basic literature relating to cerebral palsy. 

Floor L, Rosen M: New criteria of adjustment for the 
cerebral palsied. Rehab Lit 37:268-274, 1976. 
Report of a study conducted to determine the effectiveness 
of traditional rehabilitation services for cerebral palsied 
persons. The results suggest that the traditional vocational 
criteria of successful rehabilitation should be revised to 
include improvements in "quality of life." 

Guthrie JT: Educational assessment of the handicapped 
child. Pediatr Clin North Am 20:89-103, 1973. 
A discussion of adult expectations for trainable and 
educable mentally retarded children, cerebral palsied 
children, and children with learning disabilities; general 
learning characteristics of each group; a review of available 
educational tests; and a short review of findings on 
placement in regular vs. special classrooms, and home vs. 
institution. 

Heisler V: Dynamic group psychotherapy with parents of 
cerebral palsied children. Rehab Lit 35:329-330, 1974. 
The author advocates group therapy for parents of cerebral 
palsied children on the grounds that parents involved in a 
process of inner growth themselves will be better able to 
respond to the inner life of their child. Because 
handicapped children's greatest resources are in their 



inner worlds, they need parental support and 
understanding to develop these resources. 

Lekemo U: Technical educational aids for motor 
handicapped schoolchildren with severe speech disorders. 
Scan J Rehabil Med 8:67-81, 1976. 

A short report on various technical aids to permit 
schoolchildren with cerebral palsy and allied disorders, 
combined with lack of speech or severe speech disorders, to 
communicate with individuals and groups. 

Richardson SA: People with cerebral palsy talk for 
themselves. Dev med Child Neurol 14:524-535, 1972. 
This is a transcript of statements by individuals with 
cerebral palsy. Their personal needs, desires, and 
perspectives are verbalized. In particular, social and sexual 
problems faced by individuals with cerebral palsy are 
highlighted in a frank and open personal discussion. 

Samilson RL (ed): Orthopedic aspects of cerebral palsy. In 
Clinics in Dev Med 52/53, Spastics International Medical 
Publications. Philadelphia, Lippincott, 1975. 
A review of the orthopedic procedures and their indications 
and contraindications, advantages and disadvantages, is 
presented concisely from the vantage point of expertise and 
experience in cerebral palsy management. The discussion 
is largely confined to the operative and postoperative 
management of the surgical procedure, with little emphasis 
on any necessary training for improvement of function 
after the surgery has been accomplished. 

Wooldridge CP, Russell G: Head position training with the 
cerebral palsied child: An application of biofeedback 
techniques. Arch Phys Med Rehabil 57:407-414, 1976. 
Discussion of an effective biofeedback training program 
which employs a mercury switch device called a head 
position trainer to provide cerebral palsied children with 
visual and auditory information regarding their head 
positions. Equipment, training methods, and data 
collection and analysis are described. 



1 2 EPILEPSY 



Arthur A. Ward, Jr., M.D. 
Robert T. Fraser, Ph.D. 
Allan S. Troupin, M.D. 



DISEASE DESCRIPTION 

Epilepsy is the most common of the chronic 
neurological disorders. A meaningful simple definition 
of epilepsy is difficult because of its wide variability. A 
simple epileptic attack may consist of only a brief 
suspension of activity, as in a brief petit mal absence 
seizure. In other persons, the attack may consist of 
automatic motor activity or complex alterations of 
behavior, as in temporal lobe or psychomotor epilepsy. 
In still other cases, a full-blown generalized tonic - 
clonic (grand mal) motor seizure may occur. 

The incidence of epilepsy is difficult to determine 
with accuracy. In practical terms, probably 1 person in 
15 has a seizure of some sort during his life, and 
between 0.5 and 1.5 percent of the general population 
have chronic, recurring seizures. 

As Hughlings Jackson described it some 100 years 
ago, the seizure consists of "an occasional, an 
excessive and a disorderly discharge of nervous 
tissue." This explosive discharge often starts in one 
area of the brain, the focus. It then spreads through the 
circuits of the brain "like an electrical storm." The 
pattern of the seizure will be determined by the 
location of the focus and the pathways of spread. If the 
focus is located in the motor cortex on the surface of the 
brain, the first evidence of the seizure would be 
twitching of the appropriate muscles on the opposite 
side of the body. If it starts in the visual cortex of the 
occipital lobe, the first event would be flashing lights, 
which appear to the patient to come from his opposite 
visual field. (See chap. 3 for a review of the nervous 
system.) 

Thus, the seizure discharge activates the circuits 
in which it is involved and the function of these 
circuits will determine the clinical pattern of the 
seizure. Since the brain is a very complex 
communication network, in which many functions are 
localized in different circuits, it should come as no 
surprise that the clinical patterns of epilepsy can vary 
widely from person to person. 

Except at those times when this electrical storm is 
sweeping through it, the brain is working perfectly well 
in the person with epilepsy. Thus, there is no alteration 



of brain function except for those few seconds which 
may come only once a day, once a month, or once a 
year. At other times, there is no physical disability due 
directly to the epilepsy itself. 

Some persons with epilepsy have a headache, feel 
nauseous, or experience some other predictable 
symptom on the day a seizure will occur. About half 
experience premonitory symptoms just before a seizure 
occurs. These symptoms are various, consisting 
perhaps of a sensation of dizziness, discomfort in the 
abdomen, numbness, or spasm. These auras 
correspond to the first gust before the storm. The time 
interval between aura and seizure onset may or may 
not be too brief to allow the patient to sit or lie down. 
Each person's aura, if he has one, is different. 

The International League Against Epilepsy has 
published a detailed classification of the many types of 
seizures. Discussion of all seizure types is beyond the 
scope of this chapter, but five representative seizure 
types will be briefly described here. 

Focal Seizures 

The nature of a focal seizure depends on the area of 
the cortex in which it begins. For example, a seizure 
beginning in the parietal lobe cortex will cause sensory 
symptoms, such as numbness or tingling, in the 
affected extremity. Difficulty in speaking (dysphasia) 
results from a seizure originating in the speech area of 
the left cerebral hemisphere. 

In focal seizures, the electrical discharge may remain 
localized and its effects will thus be limited to the areas 
of the body controlled by the focus. The discharge 
may, however, spread to other areas of the brain and 
cause a generalized (grand mal) seizure secondary to 
the focal seizure. 



Jacksonian Seizures 

A Jacksonian seizure is a focal seizure originating in 
the motor cortex. One part of the face, arm, or leg on 
the opposite side of the body begins to jerk. These 
convulsive movements then spread in an orderly 



155 



156 Ward, Fraser, and Troupin 



fashion (Jacksonian march) and may involve the entire 
side of the body. The person usually remains conscious 
during the seizure. 

Grand Mal Seizures 

If the discharge spreads widely through the brain 
circuits, a seizure will result which has few local- 
izing features and is usually characterized as a 
generalized tonic -clonic grand mal seizure. An 
individual first endures a tonic state, characterized by 
body rigidity, and then undergoes a clonic state, 
characterized by convulsive, jerking movements. The 
person may also salivate excessively, vomit, or lose 
control of bladder and bowel function. Even when the 
spread of the seizure discharge is more restricted, the 
patient is usually amnesic for the seizure because the 
discharge commonly will spread to at least some deeper 
relay circuits. Since these deeper circuits are concerned 
with consciousness, the person loses consciousness 
during the seizure. Thus, the seizure discharge evokes 
a crude parody of the function of the circuits involved. 
For this reason, a careful description of the initial 
events in a seizure can often serve to localize the part of 
the brain in which the seizure originates with great 
accuracy. 

After the seizure is over, the nerve cells involved in 
the "excessive discharge" are metabolically fatigued. 
It is therefore common, particularly after major 
seizures, for the person to feel tired and want to sleep 
for some minutes or even several hours. This fatigue is 
obviously most marked at the very end of the seizure, 
and at this time not all the circuits are going to recover 
equally fast. Thus, for a few minutes, the person may 
be confused, may have difficulty speaking clearly, or 
may have some weakness of one arm or leg. These 
symptoms almost always clear in a matter of minutes, 
although the person may continue to feel tired. 

Individuals may fear a grand mal seizure because of 
the practical risk of falling or incurring bodily injury, 
such as burns, chipped teeth, or broken bones. 
However, it should be recognized that the actual 
seizure does not cause the person any direct 
discomfort, regardless of the pattern of the seizure. 
Nevertheless, the seizure does interfere with 
performance to varying degrees. 

Psychomotor Seizures 

If the electrical discharge starts in one temporal lobe, 
the clinical manifestation would be automatic motor 
activity and alterations of behavior which may 
occasionally be rather bizarre. This type of seizure has 
been called psychomotor epilepsy and represents 
approximately one-half of all adult epilepsy. The 
pattern of the seizure varies rather widely from patient 
to patient. In some, it consists only of a blank stare, 



rhythmic picking at the clothes, and often smacking of 
the lips. In other instances, the psychomotor seizure 
may be more complex and the person may wander 
aimlessly, continue to wash the same dish over and 
over, or perform other semipurposeful but irrelevant 
behavior. These episodes may last from a short period 
of time to many minutes. There is always complete loss 
of memory for the events during the seizure. Attempts 
to restrain or interfere with the automatic behavior of a 
patient during a seizure may evoke resentment and 
strong reaction, but the violence or aggression is poorly 
directed toward a goal and is usually carried out in a 
purposeless fashion. A structured violent attack is 
almost unknown in epilepsy. 

Psychomotor epilepsy is sometimes confused with 
psychiatric illness. This is not surprising, for many of 
the symptoms of a temporal lobe seizure are those of 
panic or emotional behavior, both of which occur as 
primary events in psychiatrically disturbed patients. 

Petit Mal Seizures 

Petit mal epilepsy occurs almost exclusively in 
children and they often "outgrow" it as they go 
through puberty. It is the most common type of 
epilepsy in children and appears to be set apart from 
the rest of the epilepsies. It occurs in the young, 
maturing brain. Petit mal or absence seizures do not 
arise from a focus on the surface of the brain, but 
rather arise deep within the core of the brain. They are 
characterized by a rather typical EEG, and respond to 
a special category of anticonvulsant drugs. 

The seizures themselves consist of very brief lapses 
of consciousness or "staring spells." There may be a 
slow rhythmic blinking of the eyes or occasionally other 
very subtle motor manifestations, but no significant 
motor component. There may be no loss of motor 
tone, although in "drop attacks" there may be a 
sudden slump to the floor with prompt return of 
consciousness within a very few seconds. 

If the petit mal seizures occur very frequently, the 
disruptions of the stream of consciousness may be 
sufficient to significantly affect performance. Very 
often it is in the school classroom where frequent 
absence seizures are first noticed. 

In approximately 40 percent of patients with petit 
mal, major motor seizures (grand mal) develop. It is 
important to realize that anticonvulsant medication 
that may benefit petit mal seizures usually has no effect 
on the grand mal seizures and vice versa. 

The most glaring diagnostic errors result from 
confusing petit mal epilepsy with other types of 
epilepsy. It must be emphasized that true petit mal 
seizures occur in children and that not all relatively 
short seizures characterized by a loss of contact are 
petit mal seizures. When short absence seizures occur 
in adults, they almost invariably are of focal origin and 



Epilepsy 157 



respond to a different group of drugs than the petit mal 
seizures. Furthermore, many other types of relatively 
short seizures are erroneously called petit mal in order 
to distinguish them from grand mal seizures, which are 
more dramatic displays of the phenomena of epilepsy. 
For example, since there are often no twitching 
movements in psychomotor epilepsy, small 
psychomotor seizures are often confused with petit mal 
epilepsy. However, these are very different kinds of 
seizures and should not be confused because the 
treatment and prognosis are very different. 

Complications 

The only cause for major concern is when another 
seizure follows the first within the next half hour or so, 
or when a seizure state persists for a prolonged period 
of time ( Vi hour for grand mal seizures, 3 A hour for 
other types). This condition is called status epilepticus 
and requires prompt and aggressive medical 
treatment. Status epilepticus can occur in petit mal 
seizures but is relatively uncommon. The major 
problem occurs in adults with grand mal seizures or, 
less commonly, with psychomotor epilepsy. When 
status epilepticus occurs, the patient should be 
promptly transported (preferably by ambulance) to the 
emergency room of the nearest hospital. However, a 
single seizure, lasting a few minutes, is no reason to 
seek immediate medical care. 



Etiology 

The causes of some types of epilepsy are well known, 
but in other instances the causes are poorly 
understood. One of the least understood and most 
controversial areas of epilepsy is the question of 
whether genetic predisposition plays a part. In general, 
those who study epilepsy in children find a high genetic 
causality, and those who study epilepsy in adults find 
the opposite. This is related to the fact that the most 
common type of epilepsy in children is petit mal and it 
has been proposed that this type of epilepsy is an 
expression of an autosomal dominant gene with 
unusual characteristics. Expression of this gene is very 
low at birth but it is nearly completely expressed 
between ages 4'/£ and I6V2 years. Expressivity 
gradually declines to almost zero after the age of 40. 

The practical problem lies with genetic counseling. 
On the basis of present knowledge, if one parent has 
epilepsy, the overall risk to a child of developing 
epilepsy is between 2 and 3 percent. This is compa- 
rable to the random risk of having a mentally handi- 
capped child or one with a congenital abnormality. 
This risk is certainly not great enough to advise 
limitation of family. However, if both parents have 
epilepsy, the risk may rise to as high as 25 percent, and 
in such instances medical advice might be helpful. 

For types of epilepsy other than petit mal, the causes 



are better understood. In the majority of temporal lobe 
epilepsy cases, the cause of the seizures is scarring of 
the medial side of the temporal lobe of the brain. In 
most instances, such scarring appears to be the result of 
the birth process, a normal part of which is a molding 
of the skull so it can fit through the birth canal. This 
molding of the skull compresses the brain and is 
thought to result in the observed scarring of portions of 
the brain in a small number of normal births. It 
appears that, in some instances, this scarring irritates 
the nerve cells around the scar, resulting in the 
abnormal electrical discharges. 

Scar-induced epilepsy can be the result of trauma, 
stroke (see chaps. 9 and 10), or even the presence of a 
tumor. Penetrating wounds of the central regions of 
the cortex (such as some depressed fractures or gunshot 
wounds) will result in the development of seizures in 
about 50 percent of patients, while penetrating wounds 
of the occipital region are followed by seizures in less 
than 20 percent of cases. The most common trauma is, 
of course, closed head injury, and this results in 
epilepsy in about 5 percent of cases. 

Precipitation of Seizures 

One of the mysteries of epilepsy is why the brain 
may function perfectly normally for several months 
and then produce a seizure, which is again followed by 
weeks or months of normal brain function before the 
next seizure. Although the occurrence of a seizure can 
rarely be predicted, certain factors have been identified 
which increase the probability of seizure occurrence in 
a person with epilepsy. 

Seizures can be precipitated by excessive alcohol 
intake and tend to occur during the withdrawal phase. 
Emotional stress makes it easier for a seizure to occur. 
Some women tend to have seizures that cluster around 
the time of menstrual periods. In some patients, 
seizures may be rare during waking periods, 
particularly when they are busy, but occur when the 
brain is "idling." It has been repeatedly demonstrated 
that patients with frequent seizures who are leading 
protected, nonproductive lives (such as in an 
institution) will undergo a dramatic reduction in 
seizure frequency when they are put into a sheltered 
workshop where they are kept busy, active, and 
productive. Thus, many of the factors which are 
known to influence seizure frequency are amenable to 
modification, and the therapeutic results may be as 
useful as with medical treatment. 



Prognosis 

The outcome of epilepsy depends on many factors: 
type of seizures, age, underlying cause, and finally, 
quality of treatment. An overall picture which emerges 
from the literature is that complete seizure control is 
achieved for 2 years in 30 to 37 percent of patients, but 



158 Ward, Fraser, and Troupin 



this statistic reduces to approximately 20 percent at 5 
years and 10 percent at 10 years. It is clear that the 
outlook for complete seizure control on a long-term 
basis is significantly improved if the seizures can be 
completely suppressed with anticonvulsant 
medication. The following factors are relevant to the 
probability of achieving complete seizure control. 

1. The longer the illness lasts, the less likely control 
is to be complete. 

2. The more seizures the patient experiences before 
initial treatment, the less likely control is to be 
complete. 

3. The more different types of seizures a patient 
has, the less likely he is to be free of them. 

4. The more abnormal the neurological and mental 
status examinations and the lower the IQ. the 
more difficult it is to control the seizures. 

5. The younger the patient is at the time of onset 
of major seizures, the less likely control is to 
be complete. 

Approximately 50 percent of those patients with 
major motor seizures without associated minor attacks 
will be free of seizures for at least 2 years. However, 
this figure drops to 20 to 30 percent if one deals with 
patients who have temporal lobe epilepsy. 

The situation is appreciably different for children 
with petit mal absence or minor seizures. In children 
with absence seizures but no other type of seizures, 80 
percent will be seizure free by adulthood. However, for 
those children with petit mal seizures who also develop 
grand mal seizures, only one- third will be seizure free. 
There is some evidence to indicate that, in the petit 
mal group, appropriate therapy will decrease the risk 
of developing grand mal. 

FUNCTIONAL DISABILITIES 

Physical Disabilities 

Except for those persons whose epilepsy is secondary 
to stroke or cerebral trauma (see chaps. 9 and 10), no 
physical disabilities are present. Thus, persons with 
epilepsy are fully independent in all eating, dressing, 
transfer, ambulation, personal hygiene, and 
communication skills. While mechanical driving skills 
are also unimpaired, local laws dictate the seizure-free 
status required for issuance of driving licenses. Even 
immediately after a seizure, independence of action is 
preserved, although level of consciousness may be 
impaired. 

Anticonvulsant medication may, however, influence 
physical function. Low-grade toxicity may produce 
some clumsiness of gait, hand coordination problems, 
and eye-focusing difficulty without loss of 
independence. Higher levels of toxicity (table 12-2, 
p. 163) can create dependency in physical functions. 
This dependency, however, is reversible with medical 
attention and alteration of drug regimens. 



Psychosocial Disabilities 

For many years, a number of psychologists discussed 
what they termed the "epileptoid personality," 
characterized by a flatness of affect, various distinctive 
physical characteristics, and other traits which they 
believed to be associated with a personality typology. 
Much of this thinking seems to have evolved in 
institutional settings where the psychologists were 
working with seizure patients who were often heavily 
medicated, had associated mental retardation, and 
were otherwise nonrepresentative. Today, this 
personality concept is refuted in psychological circles. 
It is generally agreed, however, that individuals with 
psychomotor, temporal lobe seizures can be prone to a 
number of characteristics which differentiate them 
from the normal population. These characteristics can 
include dependency, lack of humor, obsessionality, 
overconcern with religious and philosophic issues, and 
an emotional lability which often includes anger. 
Although the relationships are not clearly definable, 
the temporal lobe is involved with behavior and 
emotion. It is also agreed that there is a higher 
incidence of psychosocial problem areas among those 
with epilepsy when contrasted with a normal 
population. The relationships between neurological 
deficits and personality aberrations are difficult to 
establish. 

The etiology of much psychosocial symptomatology 
seems to be related to the nature of an individual's 
seizures (type, severity, and age of onset) and the 
reaction of the person's environment (parents, 
siblings, teachers, employers, and peers) to the specific 
type of seizure disorder. 

The major psychosocial disability of the grand mal 
seizure stems from the reaction of others in the vicinity at 
the time of occurrence. The observer is usuallv frightened, 
tends to overreact, and thereafter tends to treat the 
patient as someone verv "different" from other human 
beings. In many cases, neither the individual nor society 
have adequate time to adapt, for unlike a paralysis 
problem, seizures are not present all the time. Other than 
the occasional seizure, many persons with epilepsy look 
and function like everyone else in society. The seizure 
problem, because of its occasional, unpredictable nature, 
creates a state of expectation and uncertainty. With most 
other disabilities, stability allows for the possibility of a 
fuller understanding of the nature of the disability. It is 
this lack of experience-based understanding and a ten- 
dency to categorize epilepsy as a psvehiatric disturbance 
which has impaired rehabilitation efforts in this field. 
The manner in which the family reacts to the child 
with epilepsv is a major determinant of later 
adjustment. Problems can arise from feelings of guilt 
and parental conflict regarding approach to 
childrearing (e.g., involvement in competitive sports, 
amount of independence to be fostered, whether or not 
to disclose the child's epilepsy, or the manner of 



Epilepsy 159 



discussing the disability with other siblings). Fear and 
concern based on ignorance may cause relatives, in a 
misplaced effort to be helpful and protective, to place 
restrictions on the activities of patients with seizures. 
The restrictions foster dependency and further isolate 
and alienate these individuals from the rest of society. 
The person with epilepsy who has been overly 
sheltered by the family is usually very naive regarding 
the world of work. 

It is these social consequences of the seizures which 
form the significant disabling component of epilepsy. 
Adjusting to the change from the dependency status 
which was "licensed" by the seizures is often a 
problem for those who have become completely seizure 
free following surgery. 

In the area of intellectual functioning, the randomly 
selected client with epilepsy will tend to function within 
the normal range, barring organic brain damage. Data 
suggests, however, that there tends to be a skew in 
functioning toward the lower end of the normal range. 
This tendency toward the lower end in both intellectual 
functioning and neuropsychological adaptive skills 
(e.g., problem solving) is due to the negative effect of a 
number of factors: the severity, duration, and type of 
seizures; early age of onset; and frequency of major 
seizures. 

In addition to seizure activity, high (toxic) levels of 
anticonvulsants in the blood will impair motor 
functioning, attention, and memory, with subsequent 
decrements in intellectual and neuropsychological 
performance. Other factors, such as excessive fatigue 
or abnormal electrical brain discharging on the day of 
testing can also detract from performance on these 
measures. 



Rehabilitation Potential 

The rehabilitation potential for persons with 
epilepsy and no other chronic neurological impairment 
is governed by psychosocial factors. For those persons 
who do have chronic neurological impairment in 
addition to epilepsy, rehabilitation potential is 
governed by the nature of the neurological 
impairment. 

Without question, most individuals with epilepsy 
are employable. Rehabilitation potential must be 
considered very good and not diminished from the 
perspective of the epilepsy itself, particularly if seizures 
are under good control. Hence, long-term planning 
and goals are appropriate. 

STANDARDS OF EVAULATION 

In considering the diagnosis and etiology of seizures 
in new patients, the age-related patterns of occurrence 
of different types of epilepsy are taken into account. 
The appearance during adolescence of seizures of focal 
origin, whether or not associated with major 



generalized seizures, generally implies a minor injury 
to the cerebral cortex at birth, without additional 
associated neurologic disease. 

Seizures following major head trauma can occur at 
any age, but major head trauma is more common 
during adolescence and early adulthood. Again, there 
are no implications for major neurologic disease other 
than the obvious results of the trauma. 

In middle to late adulthood, the possibility of a brain 
tumor assumes more importance when the history does 
not reveal obvious trauma. In the older age group, the 
possibility of tumor persists, but cerebral vascular 
disease assumes a progressively greater importance. 

While all of the above statements are generally true 
for the population at large, any individual must receive 
a specific evaluation aimed at defining the background 
of his own seizure problem. Certain clinical 
manifestations of some kinds of seizures may be most 
difficult to distinguish from some ordinarv 
nonepileptic behavior. Simple fainting episodes 
(syncope), for example, usually are due to vascular 
changes with a transient drop in blood pressure and are 
not epilepsy. Careful history- taking will usually 
indicate that such episodes are provoked by syncope 
and that the patient is not suffering from epilepsy. 
Certain cardiac problems or small strokes may also 
mimic epileptic phenomena. 

Temporal lobe seizures may mimic psychiatric 
symptoms, such as anxiety attacks. However, unlike 
temporal lobe seizures, anxiety attacks are prolonged, 
are not associated with disturbances of consciousness, 
and are usually precipitated by environmental events. 
Similarly, outbursts of rage and violence associated with 
a seizure may be construed as a psychiatric disturbance. 
Such misinterpretations and inadequate diagnoses have 
repeatedly resulted in involuntary commitment of 
seizure patients to psychiatric hospitals. When episodic 
unusual behavior caused by temporal lobe epilepsy is 
throught to be due to mental illness, patients and their 
families may be subjected to untold anguish. The 
combination of a skilled history, neurological 
examination, and electroencephalogram (EEC) are 
essential and should differentiate between epilepsy and 
psychiatric conditions with similar manifestations. 

Medical Evaluation 

The clinical evaluation of seizure patients is 
appropriately carried out by a neurologist, although in 
some communities a neurosurgeon may also have an 
interest in epilepsy. In view of the potentially 
progressive nature of epilepsy, the new seizure patient 
should be seen promptly for an evaluation, 
appropriately scheduled as an outpatient visit rather 
than as an emergency. 

There are two principal aims in the initial evaluation 
of the patient with epilepsy. The first aim is to define 
the etiology of the seizure disorder to separate those 



160 Ward, Fraser, and Troupin 



patients who are in need of therapy directed at an 
underlying disease (e.g., brain tumor) from those who 
have stable epilepsy amenable to drug suppression of 
seizure activity. The second aim is to classify the 
seizure type so that appropriate medical therapy can be 
chosen. Both etiology and classification are most 
readily determined by the neurologic examination, 
which consists of the neurologic history, the physical 
examination, and the EEC The history and physical 
are generally carried out by the neurologist in his office 
and require approximately 1 hour. The EEG must be 
performed in a laboratory with the necessary 
equipment and also requires about an hour. 

The major aim of the neurologic history is an 
accurate description of the seizure itself, since it is 
generally possible to determine the site of origin of the 
seizure discharge from the initial display of abnormal 
behavior. A description of the events following the 
active element of the seizure (the postictal phenomena) 
also has bearing on the location of the seizure focus. 
Another major objective of the neurologic history is a 
description of events which tend to precipitate seizures, 
either acutely or after a period of time. 

The history also provides significant clues to the 
etiology of the seizure disorder. Seizures could 
represent a symptom of some other disease or a 
mechanical process within the brain requiring a 
surgical solution. The recent onset and progressive 
worsening of a focal neurologic deficit, such as a 
hemiparesis, tends to point to a localized intracranial 
mass, such as a tumor or subdural hematoma. The 
presence of additional symptoms related to other body 
systems might suggest a general medical disease 
requiring specific attention. In many patients, the 
history reveals an episode of brain injury which may be 
an adequate explanation for the subsequent 
appearance of seizures. Sometimes the history is 
obvious, such as the previous presence of encephalitis, 
meningitis, or a major head injury. Sometimes the 
history is inferential only, such as a story of a difficult 
labor at birth, coupled with slow development of motor 
skills and subsequent normal performance, all of which 
point to birth injuries as the cause of seizures. 

The second element in the neurologic evaluation is a 
specialized physical examination directed at the 
nervous system. The activities of the motor system, the 
cranial nerves, the sensory system, the coordination 
system, the intactness of reflex arcs, and an overall 
estimate of mental status are reviewed in a physical 
examination requiring 20 to 30 minutes. The 
information gleaned from this examination is 
integrated with the patient's history to confirm or 
refute the initial diagnostic impression. The 
demonstration of localized neurological dysfunction of 
recent appearance without obvious recent trauma 
might suggest the presence of a progressive 



neurological disease, while a normal neurologic 
examination suggests the presence of an old or static 
process. 

The electroencephalogram, a dynamic display of the 
brain's spontaneous electrical activity, has particulai 
bearing on the evaluation of the patient with epilepsy. 
"Reading" the EEG consists of examining the 
recording of the brain's activity from several points on 
the skull simultaneously, in an effort to identify specific 
patterns. The information gleaned from the EEG has 
bearing on the location within the brain of the 
discharging focus. Less frequently, some inferences 
can be made concerning etiology and classification 
which might have bearing on the therapeutic agents 
chosen. 

For the vast majority of patients, the above sequence 
of neurological history, examination, and EEG 
represents all the investigation that is necessary. In 
those instances, however, where there is a suggestion 
that generalized medical disease is present, certain 
laboratory blood tests might appropriately be chosen to 
evaluate the specific disease in question. II the tests 
suggest that further investigation is necessary, the 
patients are usually sent to an internist. 

If the initial evaluation has suggested the possibility 
of a progressive neurological disease, further radiologic 
investigation may be undertaken. Skull films have only 
a small yield in this respect, but the new technique of 
computerized axial tomography (CAT scan) rapidly 
yields excellent information concerning structural 
abnormalities within the brain without danger, 
discomfort, or hospitalization. Depending on the 
findings, it may indeed be appropriate to hospitalize 
the patient for specific studies of the arteries leading to 
and supplying the brain (arteriogram) or the spaces 
within and over the brain as visualized by the injection 
of air into the spinal canal (pneumoencephalogram). 
These latter two studies are currently rarely done 
unless surgery is seriously contemplated for a given 
problem. 

Psychosocial Evaluation 

In a recent study of 369 outpatients, fewer than 25 
percent were categorized as having "epilepsy only"' 
without an associated problem, such as intellectual 
disturbance, neurological impairment, or behavior 
problems. Those in the "epilepsy only" category 
seemed to function very competently at work or in the 
school setting, while the other groups, especially those 
with brain damage, seemed to have difficulties in 
various aspects of living. It is those clients with epilepsy 
plus associated problems who will usually be seen in 
the rehabilitation setting. Therefore, a counselor having 
a client with epilepsy should procure as much 
information as possible from the person's family, the 



Epilepsy 161 



neurologist, and in many cases, a psychologist and 
social worker. 

Neuropsychological evaluation, although not always 
readily available in all communities, can be invaluable. 
A rehabilitation agency's psychological consultant 
should be contacted regarding the appropriateness of 
this type of evaluation. The measures developed by 
Drs. Halstead and Reitan comprise the core 
neuropsychological battery. It is used to discriminate 
between normal performance and performance of 
brain -damaged populations. The neuropsychological 
assessment can provide the information regarding 
specific brain -behavior relationships (e.g., a 
determination of brain damage and the type of 
adaptive skills which the individual lacks, the efficiency 
of functioning of one side of the body versus the other, 
or particular patterns of ability). Decreased 
neuropsychological and intellectual functioning may 
result from seizure activity, fatigue, or toxic levels of 
anticonvulsants on the day of testing. The examiner 
should therefore check for these problems before 
administering the tests. 

When the rehabilitation counselor is dealing with an 
epilepsy client, it becomes extremely important to 
identify the arena in which a behavioral problem 
occurs. For example, the problem may be related to 
the reactions of the client's family to his seizure 
disorder. Family difficulties in adjusting to the client's 
epilepsy can be a fertile area for counseling and 
involvement by the rehabilitation counselor. 
Adolescents with epilepsy can have their prospects for 
employment greatly improved through the active 
involvement of the rehabilitation counselor at a 
prevocational stage. 

The behavior problem may be related to side effects 
of anticonvulsants, such as irritability, lethargy, and 
depression. Since these side effects may be subtle, the 
counselor should always be alert for their appearance 
and refer the client for evaluation of his anticonvulsant 
drug program if side effects are suspected. 

In some cases, the behavioral characteristics are 
related to the focus of the seizure (e.g., with temporal 
lobe seizures). Abnormal behavior is sometimes 
actually part of a seizure or related to anticipation of a 
seizure. If the counselor is familiar with the client's 
seizure pattern, he will be able to recognize those 
behaviors which are clearly seizure related. 

Finally, the maladaptive behavior may simply be 
related to a person's attempt to "cloak" the fact that he 
has epilepsy. In many cases, however, behavioral 
problems have no identifiable relationship to the 
disability, and one simply observes some of the 
personality aberrations noticeable in the general 
population. In any case, the more specific the 
information gathered, the better prepared the 
counselor is for a positive counseling intervention. 



TOTAL TREATMENT 

The absence seizure of childhood is so brief (a few 
seconds) that it may go unrecognized and, when 
identified, requires no attention. When a person has a 
generalized tonic-clonic seizure, there is little required 
of those around him. The goal is to let the seizure run 
its course without letting the individual hurt himself. 
For unknown reasons, the average onlooker often tries 
to insert some firm object between the teeth, 
presumably to prevent biting of the tongue. It is true 
that occasional biting of the tongue may occur, but this 
is a relatively minor problem and far less dangerous 
than the significant damage that has been produced by 
injudicious attempts to insert a spoon handle or other 
object between the jaws, which can easily result in 
broken teeth and other damage. If it is easily possible, 
insertion of a padded wooden tongue blade or its 
equivalent can be undertaken, but no attempt should 
be made to force the jaws open. The patient may be 
moved into a comfortable position, preferably on his 
side or semiprone, so that any excess salivation will 
drain to the outside. Something soft may be placed 
under the head. Beyond such simple measures, 
nothing else is necessary. The seizure will be over in a 
few minutes, and it is important not to overreact while 
reorienting the person. 



Anticonvulsant Drugs 

Repeated seizures are anatomically and 
physiologically bad for the brain, can produce 
irremedial neuropsychological deficits, and tend to 
facilitate the subsequent appearance of additional 
seizures. The therapeutic goal in the management of 
epilepsy, therefore, is suppression of all seizures before 
they can recur. This requires that appropriate 
anticonvulsant drugs be chosen for any given seizure 
type and administered on a steady basis. With an 
appropriately chosen and managed program and with 
cooperation on the part of the patient, it is possible for 
most patients to be entirely free of seizures on 
medication, with the remainder having only rare 
seizures under major precipitating circumstances. 

Scientific drug therapy for epilepsy is only now 
developing, but certain guiding principles can be 
described. It must first be appreciated that not all 
anticonvulsant drugs operate equally well for all 
seizure types. It is therefore necessary to make an 
appropriate choice based on an accurate diagnosis. 

The drugs appropriate to the several categories of 
seizures are listed in table 12-1. As the table indicates, 
most of the seizures of adulthood, all those of focal 
origin, and all the major convulsive seizures can be 
considered as one group for the purposes of therapy. 
Having chosen the proper drug, it is necessary to 



162 Ward, Fraser, and Troupin 



TABLE 12-1 
MOST COMMON ANTICONVULSANT DRUGS 



Seizure Tvpe 



Pnmarv 
Anticonvulsants 



Secondai j 

Anticonvulsants 



Focal, Jacksonian, 
grand mal, and 
psychomotor 



Phenvtoin (Dilantin) 
Carbamazepine 

(Tegretol) 
Mephenvtoin 

(Mesantoin) 



Phenobarbital 
Primidone 

(Mvsoline) 
Clorzepate 

(Tranxene 
Methsuximide 

(Celontin) 



Petit mal 



Ethosuximide 
(Zarontin) 

Valproate 
(Depakene) 

Clonazepam 
(Clonipini 



Trimethadoine 
Tridione) 



ensure that an adequate amount of the drug will be 
delivered to the circulation so that the blood level is 
sufficient to have the desired therapeutic effect. Dose 
alone is not a sufficient guide, since different patients 
have different body sizes, varied fluid -fat relationships 
within the body, and different capabilities of their 
livers (where most drugs are metabolized). Differences 
in rate of metabolic destruction of the drug can 
account for variation in serum levels between 
individuals. Furthermore, with the continued exposure 
of the patient to any drug, a progressive ability of that 
patient's liver to dispose of the drug will develop, 
resulting in slowly declining serum drug levels on 
stable doses. It is not uncommon that a dose initially 
providing adequate protection against seizures is 
subsequently no longer adequate. With various dose 
increase, and after some period of time, a long-term 
stable state is reachieved. 

A problem which has immediate impact on the 
serum level and usually almost as rapid an impact on 
seizure control is the matter of compliance. Simply 
stated, if patients do not take their medicine, they are 
not protected, and the seizures will quite likely recur. 

In order to assess whether lack of control of the 
seizure in a patient taking a specific drug is due to 
relative ineffectiveness of that drug for that patient or 
merely due to inadequate amounts of the drug being 
present, it is necessary to measure the level of the drug 
in the patient's serum. This gives an accurate guide to 
the amount of drug delivered to the brain, which is the 
effector site for anticonvulsant activity. The blood - 
level information must be interpreted with an 
understanding that the so-called therapeutic ranges 
are a rough guide only. 

The most effective way to deliver stable 
anticonvulsant protection is by the use of a single drug 
delivered in maximum tolerable doses. Those drugs 



which are likely to control the appropriate types of 
seizures when given alone are primary anticonvulsants 
and are so indicated on table 12-1. The 
anticonvulsants which offer some measure of 
protection, but in most patients do not confer full 
protection when given alone, are called secondary 
anticonvulsants . 

There are two problems associated with using more 
than one drug for the treatment of epilepsy, although it 
is necessary to do this on some ocassions. First, the use 
of more than one drug tends to promote more rapid 
destruction of both drugs than would tend to occur if 
either were given alone. Use of the second drug may, 
therefore, decrease the therapeutic effectiveness of a 
given program, rather than increase it. This can, to 
some extent, be overcome by increasing the doses of 
either or both of the drugs to the point at which the 
body's ability to dispose of the drugs is nearly 
saturated. 

Secondly, drug side effects tend to be additive, 
presumably because all the drugs interfere with the 
coordination system and depress alertness. The 
therapeutic effects do not seem to add 
pharmacologically because they apparently operate via 
different mechanisms. It is therefore hard to achieve 
adequate therapeutic levels of any of the multiple drugs 
because of the addition of side effects. 

It is now becoming more common practice to switch 
from one primary anticonvulsant to another, rather 
than merely to add more drugs to the first drug. When 
it is necessary to use secondary anticonvulsants, the 
trend has been away from more sedating ones, such as 
the barbiturates, and toward drugs with fewer side 
effects, which can then be added in sufficient quantity 
to achieve some degree of therapeutic effectiveness. 
Continued frequent followup and accurate controlling 
of prescribed drugs are critical for good control. It is 



Epilepsy 163 



TABLE 12-2 
CLASSIFICATION CRITERIA FOR SIDE EFFECTS AND INTOXICATION 



Category Title 



Findings 



I None 
II Side effects 

III Mild intoxication 



IV Definite intoxication 



V Encephalopathy 



I None 

II Nystagmus without diplopia 

Minimal (barely perceptible) 
coordination disturbance 

III Symptomatic nystagmus 

Diplopia 

Mild to moderate coordination 
disturbances 

IV Mental status change 

Severe/marked coordination 
disturbance 

Vomiting 

V Brain-stem and cerebellar signs 

Changes in level of consciousness 

Focal neurological signs 

Increase in seizure frequency 



essential that every seizure patient have a 
knowledgeable physician who is seen regularly at least 
every 3 to 6 months. 

The anticonvulsant side effects, which are listed in 
table 12-2, represent a major barrier to the easy 
control of seizures. All the drugs have essentially the 
same side effects which limit the progressive raising of 
drug doses and levels. Frequently, the side effects are 
represented by impairment of coordination, which can 
range from difficulty focusing the eyes, through mild 
clumsiness of the hands, to gait disturbances ranging 
from slight stumbling to a major difficulty in walking. 
These side effects are objectively apparent, and when 
drug dose is reduced, side effects diminish. 

Much more subtle are the mental and emotional side 
effects related to many of the anticonvulsants. Some of 
the drugs are sedating, and it is only a complaint from 
the patient that will bring this to anyone's attention, 
since when the patient is sitting in the physician's 
office, he is sufficiently involved to remain alert. The 
same patient with the same drug level, however, might 
be incapable of remaining alert while performing a 
routine task. Additionally, noticeable deteriorations in 
intellectual performance are found on neuro- 
psychological evaluation which are related specifically 
to certain of the anticonvulsants. Although these are, 
as previously mentioned, to some degree dose -related 
as well, they may also occur even at small doses in 
some patients and not affect other patients at all. Sensi- 



tivity to the complaints of the patient is again the 
best clue to the presence of such side effects. 

Another rather common side effect is depression. This 
can be subtle in onset and may well be confused 
initially with depression related to the occupational and 
social limitations imposed on patients with seizures. 
Nevertheless, certain of the drugs, particularly the 
barbiturates, tend to promote emotional depression, 
which will disappear with the removal of those agents. 
Since many of these more subtle side effects will persist 
even at blood levels under those needed to achieve 
seizure control, it is now considered appropriate to 
shift drugs and attempt to achieve adequate 
therapeutic levels with an agent that does not produce 
side effects in the particular individual in question. 

Another group of side effects are idiosyncratic , 
meaning that they are simply due to the mere presence 
of the drug. Most of these, such as rashes, are benign, 
but infrequently they may be dangerous, such as a 
failure of the bone marrow to produce blood cells 
(aplastic anemia). Idiosyncratic side effects require 
prompt termination of the offending drug and the 
substitution of another. 

Problems of side effects tend to make seizure 
control in some patients difficult to achieve until a 
logical process of trial and error, aided by serum -level 
measurements, is pursued. Throughout, it must be 
recognized that unless the patient's report of side 
effects is taken into account, the patient himself may 



164 Ward, Fraser, and Troupin 



very well alter the dose of medicine and thereby 
interject an unknown and random factor into the 
attempt to control the seizures. 

Surgery 

Surgical treatment of epilepsy is now well 
established, the criteria for consideration are clear, and 
the results of treatment well documented. 

The most important criterion for consideration of 
surgery is ineffective control of seizures by competent 
and aggressive treatment with anticonvulsant drugs. A 
trial on one or two of the common antiepilcptic drugs 
does not satisfy this criterion. It is necessary that the 
patient receive a thorough trial on tht best drugs 
effective for his type of seizure and that he receive 
adequate dosage as judged by quantitative tests of the 
amount of drug in his blood serum. 

If the seizures cannot be controlled with medical 
treatment, the necessary diagnostic studies must be 
undertaken to determine the exact location of the 
seizure focus. This focus must be located on the surface 
of the brain, in an area of cortex which can be 
surgically removed without producing significant 
neurological deficit. Thus, a patient whose focus is 
located in the speech cortex is not a candidate for 
surgery because, while surgery might control the 
seizures, the patient would be left with an aphasia far 
more disabling than the seizures. The diagnostic 
studies to acquire the information to make these 
judgments require a short period of hospitalization. Of 
all patients with epilepsy, about 5 percent will qualify 
for surgery. 

The surgery itself can almost be described as plastic 
surgery. Irritating scar is removed in a very delicate 
fashion so that the postoperative scar is as small as 
possible. The operation is lengthy and requires a 
complex team who have had special training and 
experience in the surgery of epilepsy. It also requires 
rather elaborate electronic instrumentation. For these 
reasons, there are relatively few centers in the United 
States that have the personnel and facilities to 
undertake surgery of this kind. 

The success of the surgery depends to a large degree 
on the location of the focus and the type and degree of 
scarring causing the seizures. The overall results are 
that about 60 percent of all patients undergoing the 
surgery become seizure free. Patients with temporal 
lobe epilepsy represent more than one-half of all adult 
epileptics and also represent the largest group receiving 
surgery. In this group, 71 percent become seizure free 
after surgery. The value is 77 percent for temporal lobe 
epilepsy due to birth trauma and 61 percent when the 
cause is unknown. In general, slightly more than 50 
percent of patients are seizure free and taking no 
anticonvulsant medication more than 2 years after 
surgery. An additional group (about 20 percent) are 



seizure free, but still require medication. When one 
considers that the candidates for surgery are those with 
the most intractable seizures, such figures are 
impressive. Furthermore, surgery is the only treatment 
modality which can result in permanent eradication 
seizures. 

If the surgery is to be undertaken, it is most effective 
if carried out fairly early in the course of the disease. 
Not only does early surgery improve the results in 
terms of seizure control, but it also inhibits the 
development of the social consequences of epilepsy, 
and hence a more optimal rehabilitation result 
becomes possible. On the other hand, patients who are 
seizure free following successful surgery may need 
psychological counseling to help them overcome their 
previous dependencv. 

VOCATIONAL IMPLICATIONS 

A recent nationwide survey of persons with epilepsy, 
performed by the National Epilepsy League, indicated 
vocational counseling as the highest priority of direct 
services requested, substantially above the stated need 
even for medical services. Rehabilitation professionals 
must channel more energy and skill into providing the 
specific assessment and counseling services which these 
clients require. 

The maturation of a person with epilepsy is often 
hindered, and he tends to enter the job market several 
years later than a normal peer. This delay must not be 
prolonged by waiting for the ideal situation of a 
seizure -free state before initiating rehabilitation 
planning efforts. Remaining seizure free is not certain, 
even if a client's seizures do cease for several months or 
even several years. In developing a rehabilitation plan, 
success is more likely if the counselor fully understands 
his client's seizure disorder, indentifies the client's 
adaptive skills and job goals, coaches the client toward 
social competence, and, if necessary, assists in locating 
a "seizure tolerant" work environment. Enhancing 
the client's social skills is especially important, because 
the more socially acceptable he is, the less likely 
repeated seizures will result in a quickly contrived 
"lay-off" and social isolation from other employees. 

The importance of seizure -related information 
cannot be overstressed. Understanding how the seizure 
disorder may specifically affect functioning on a job is critical. 
The counselor must be concerned with type and 
frequency of seizures; the existence, if any, of a 
warning on the day of a seizure; the time lapse between 
an aura and the onset of the seizure, and whether the 
client is conscious and capable of taking safety 
precautions during this period; any evidence of a 
relationship of seizure onset to other factors; and the 
amount of recovery time necessary following a seizure. 
Information regarding the possible precipitants of 
seizures (e.g., fatigue, flickering lights, alcohol intake, 



Epilepsy 165 



stress) should be carefully noted for discussion with a 
medical advisor and for analyzing the total work 
environment. Other factors, such as the direction an 
individual falls during a generalized grand mal seizure 
or whether this person has ever been injured during a 
seizure, should be considered when selecting a work 
station or job. 

The more seizure -related information counselors 
have in regard to their clients, the better prepared they 
are to engage an employer for placement purposes. If a 
client has both grand mal and petit mal seizures, but it 
can be determined that the grand mal seizures occur 
late in the evening and the petit mal seizures are barely 
discernable (lasting only several seconds) during the 
day, client employability is significantly improved. 
The existence of a dependable aura and warning 
sensations is also important to an employer, as they 
provide time for the individual to withdraw from 
potentially unsafe vvork situations. 

With regard to vocational evaluation techniques, 
traditional vocational interest inventories, various 
aptitude tests, and traditional education and training 
approaches can easily be utilized with this group. 
Further, a series of volunteer experiences in hospitals, 
nonprofit corporations, or workshops, for example, 
can provide both vocational exploration and work 
adjustment. Such experiences provide more precise 
seizure -related and social skills feedback through job 
supervisors. Job experiences can also serve to identify 
precise positive or negative information on 
performance which may be at variance with 
information provided by clients or their overly 
supportive families. While neuropsychological 
measures can identify organic deficits and provide the 
general directions with regard to adaptive strengths 
and weaknesses, they cannot predict performance on 
specific jobs. When using sheltered work settings for 
job experiences, care must be taken to avoid holding a 
client in a sheltered workshop longer than necessary 
due to workshop staff misconceptions about the 
disability. 

Rehabilitation planning should have this solid 
assessment as a base, complemented by energetic 
counselor- client involvement. Since dependency is a 
significant problem of many clients with epilepsy, the 
assumption of personal responsibility for life and work 
planning by the client should be a consistent 
counseling focus. 

There are a number of medical concerns to which 
the counselor should be especially attentive in the field 
of epilepsy rehabilitation. The counselor must 
understand the anticonvulsants used by his client. 
Deterioration in work performance may be due to side 
effects, especially those in category III on table 12-2. 
The counselor should not hesitate to refer the client 
promptly for medical re -evaluation if side effects are 
even in the least suspected. 



Should seizure activity either recur or increase in 
frequency, it is attributable in many cases to self- 
medication by the client as opposed to the considered 
advice of a physician. Anticonvulsant side effects, costs 
of medication, and feelings of social discomfort may 
lead to the client's abandonment of his treatment 
regimen. These issues can often be addressed by the 
counselor. The counselor should also be aware of 
medically prescribed changes in primary or secondary 
anticonvulsants, for their influence on the client may 
affect vocational planning and performance. 

Since neurological surgery for seizure reduction in 
some persons with severe and frequent seizures can 
radically improve their vocational potential, the 
counselor should ensure that this possibility is 
explored. The counselor should be aware, however, 
that although surgery often improves seizure control, 
the individual may undergo substantial psychological 
adjustment postoperatively that may require attention 
and time. 

A decided majority of those with epilepsy prefer 
disclosure of their disability to an employer. If this is 
chosen, it is very helpful for clients to be able to explain 
the impact of seizures on their work performance 
clearly. Specificity of information is an extremely 
important tool for effective job placement. The 
employer often wants very complete information 
regarding a client's seizure disorder in relation to 
functioning on the job. The counselor and client may 
therefore wish to rehearse together the clear 
communication of this pertinent information. 

In following up a client's job interview with an 
employer, seizure -related questions may often arise, 
and the counselor must be prepared to respond 
appropriately. In discussing seizure activity with an 
employer, it may be helpful to avoid some of the 
medical terminology and concentrate on a clear and 
specific description of the seizure, its aftermath, and 
what, if anything, those in the environment ought to 
do should it occur. Employers seem to appreciate this 
type of approach. If a client with active seizures never 
falls forward during a seizure (most persons with grand 
mal seizures fall to the side or backwards), has never 
injured himself while working, or has never used all 
available sick leave in previous jobs, this information 
should be carefully presented to the potential 
employer. A client's personal methods of coping with 
seizures in job settings should also be described to 
employers. Some will simply compensate for any 
"time out" due to a seizure by working overtime on 
the same day. The fact that approximately 50 percent 
of those with grand mal epilepsy consistently have a 
seizure warning aura greatly improves prospects for 
employment, if explained carefully to potential 
employers. 

Many employers fear that their employee insurance 
rates will be raised if thev hire an individual with 



166 Ward, Fraser, and Troupin 



epilepsy. This is not true, even if the company is 
privately insured, as opposed to having coverage 
through state Workers' Compensation. Insurance rate 
hikes are related chiefly to the frequency and severity 
of accidents which actually occur on a job site. Hiring 
an individual with epilepsy does not, in itself, increase 
insurance rates. A discussion of insurance coverage 
should always be preceded by statements affirming that 
employees with epilepsy have no more accidents on the 
job than their peers and that, in some instances, firms 
employing only individuals with epilepsy have actually 
been awarded reductions in insurance costs by private 
agents due to excellent safety records. 

In general, although there is some variance among 
states, the Workers' Compensation Second Injury 
Fund will cover an employee with epilepsy who has an 
injury on the job resulting in total disability. This 
coverage is specifically for persons who already have a 
disability and are further disabled by an injury on the 
job. Therefore, if an employee is to be eligible to utilize 
this Second Injury Fund, an employer should have 
prior knowledge of the first disability (in this case, 
epilepsy), preferably documented in the firm's medical 
file. 

Most persons with seizure disorders are especially 
attuned to potential dangers in a work environment 
and make extra efforts to avoid physically hazardous 
situations. The rehabilitation counselor, however, 
should be selective in job placement efforts with these 
clients, especially with those having more severe types 
of seizure activity or no seizure -related warnings. 
Working around heights, potentially dangerous 
machinery, or other situations in which these 
individuals can endanger themselves or others should 
be avoided. If an individual who is no longer having 
seizures desires to return to a potentially hazardous 
job, a medical consultant should review EEG results 
and the individual's natural propensity for seizures. 
Possible precipitants of seizures should also be taken 
into account in the placement process. 

Although avoiding stress -inducing jobs is a concern 
for many clients, these same clients may also have 
frequent seizures when bored or tired sitting at home. 
It is often a better "trade off" for a person to have 
occasional seizures in a mildly stressful work 
environment than to sit alone at home with no 
appreciable lessening of seizure activity. Acclimation 
to the job and concentration on the work itself can 
result in a tapering off of seizure activity initially 
exacerbated by the stress of a new job. 

There is no question that the counselor will be 
confronted with numerous additional problems in 
working in the field of epilepsy rehabilitation. Many 
individuals with epilepsy are not eligible to drive due to 
seizure activity. State laws vary regarding the length of 
time a person must be medically certified seizure free 



for license eligibility. If they are eligible to drive, 
epilepsy rehabilitants are often subject to more 
expensive assigned risk insurance rates. This group 
also has difficulty securing adequate health and life 
insurance. Local epilepsy associations can be helpful in 
the identification of insurance companies with more 
equitable rates. 

Greater cohesiveness among those with epilepsy in 
lobbying for remediation of particular needs could 
definitely bring about improvement of services. There 
is a lack of consumer involvement, probably because of 
the "invisible" nature of the disability. The 
rehabilitation counselor should be aware that agencies 
serving the developmentallv disabled (e.g., the cerebral 
palsied or mentally retarded) often are prepared or 
required by sponsors to make their services available to 
those with epilepsy as well. 

Another problem area in employment is the 
existence of negative employer attitudes toward hiring 
those with epilepsy. A recent study suggests that 
employer education alone does not dispel this attitude. 
It appears that less traditional educational approaches 
are more effective and that the employer really has to 
be reached at an emotional level. Vocationally 
successful citizens with epilepsy, popular TV 
personalities, and established members of the business 
community might be used in more innovative 
programming focused on changing these negative 
employer attitudes. 

The counselor has a number of tools which should 
help in placement of the client with epilepsy. New 
affirmative action legislation demands "accommoda- 
tion" on the part of the employer. No longer can a 
seizure in a person with a physically hazardous job 
constitute sufficient grounds for dismissal, when 
altering the job or transferring the employee to other 
positions within a firm can result in safe and competent 
job performance. 

Epilepsy rehabilitants who encounter negative attitudes 
from employers and others in the community and, 
additionally, must work through problem areas 
associated with their primary disability, can often 
profit from group involvement. Job -finding clubs 
allow clients to share job leads and confront or 
reinforce each other regarding vocational goals. Job- 
seeking- skills groups can also be useful to epilepsy 
rehabilitants, for they are often very naive regarding 
the world of work and can profit from coaching in job - 
seeking procedures. Finally, group social- skills 
training in areas such as assertiveness and 
conversational skills can have substantial impact on 
greater life satisfaction for the individual with epilepsy. 
Above all, the group setting fosters the development of 
emotional strength. When the person with epilepsy 
learns that he is not alone in encountering life and 
work barriers, his confidence improves. 



Epileps 



167 



BIBLIOGRAPHY 

Barmore A (ed): Epilepsy. . Major Aspects: A Counseling Guide. 
Wisconsin Epilepsy Association, 1954 E. Washington 
Avenue, Madison, WI 53704, 1977. 

This counselor handbook offers a succinct overview of 
rehabilitation concerns for the client with epilepsy. Of 
particular interest are discussions of insurance, Workers' 
Compensation, Second Injun,- Fund, and driver's 
licensing. 

Epilepsy Foundation of America: Basic Statistics on the 
Epilepsies. Philadelphia, Davis, 1975. 

The Epilepsy Foundation of America has compiled an 
excellent overview of statistics related to this disability, 
framed within topical reviews of the literature. Chapters on 
psychological considerations, education, employment, and 
rehabilitation are particularly pertinent to the 
rehabilitation counselor. This text is an invaluable desk aid 
for grant writing or the development of project rationales 

Goldin GJ, Perry SL, Margolin RJ, Strotsky BA, Foster JC: 
Rehabilitation of the Young Epileptic Dimensions and Dynamics. 
Lexington, MA, Lexington Books, 1971. 
This book presents the results of an extensive survey study 
focused on identifying needs of the young person with 
epilepsy. Basic and psychosocial needs are reviewed within 
the context of educational, vocational, social, and family 
settings. The book concludes with specific 
recommendations for prevocational involvement by the 
rehabilitationist on behalf of the young person with 
epilepsy. 

Laidlaw J, Richens A (eds): Textbook on Epilepsy. Edinburgh, 
Churchill Livingstone, 1976. 

A comprehensive and current text on the medical 
treatment of epilepsy. The editors have succeeded in their 
purpose of making the present state of the art equally 
understandable to those in other than the medical 
profession. 

Rodin EA: The Prognosis of Patients with Epilepsy. Springfield, 
IL, Thomas, 1968. 

Dr. Rodin uses extensive data from the Michigan Epilepsy 
Center and thorough reviews of the literature to provide an 
excellent exposition of epilepsy and its associated problem 
areas. Chapters related to intelligence and prognosis for 
seizure control, behavior, and employment provide 
extremely useful and interesting information for the 
rehabilitation counselor. 

Rodin EA, Shapiro HL, Lennox K: Epilepsy and life 
performance. Rehabil Lit 38:34-39, 1977. 
In this article, staff of the Michigan Epilepsy Center 
summarize data collected on 369 patients with a diagnosis 
of epilepsy during the years 1970-74. Patients were initially 
classified into an "epilepsy only" group or into another 
category, depending on an identified problem in addition 
to the primary disability. Statistical analysis of the data on 
various outcome measures compares the function of those 
with "epilepsy only" to the function of those with an 
additional problem (e.g., brain damage). 



Schlesinger LE, Frank DS: Epilepsy: On the Way to Work. 
Washington, D.C., Artists and Writers Syndicate, 1976. 
This book, sponsered by the Epilepsy Foundation oi 
America, is written in a dramatic script format as a guide 
for the rehabilitation counselor. Dialogue is between 
experienced and inexperienced counselor or counselor and 
physician, and coverage includes: medical aspects of the 
disorder, epilepsy and the community, preparing the client 
for the job, reaching employers, and group vocational 
counseling. An "epilepsy quiz" with answers is included. 

Schlesinger LE, Frank D: From demonstration to 
dissemination: Gateways to employment for epileptics 
Rehabil Lit 35:98-106 and 109, 1974. 

This article describes the three -cities project, in which 
comprehensive vocational demonstration projects were 
established for those with epilepsy in Adanta, Chicago, and 
San Antonio (1967-68). The article is particularly valuable 
because of the authors' description ol the various 
vocational program components important in meeting the 
needs of this population. 

Wright GN (ed): Epilepsy Rehabilitation. Boston, 
Little, Brown, 1975. 

This is the most comprehensive reference text that 
currently exists in the field of epilepsy rehabilitation. Dr. 
Wright has judiciously utilized the talents of 16 well-known 
authorities in the medical and rehabilitation aspects of the 
disability. Within the chapters on vocational counseling 
and rehabilitation planning are various data protocols 
which can be valuable for use in intake, assessment, 
planning, or program-evaluation procedures. 



1 3 AMPUTATION 



Lawrence W. Friedmann, M.D. 



INTRODUCTION 

The term "amputation" means the complete loss of 
all limb elements below a certain point. In this chapter, 
however, the term also includes congenital limb 
deficiency. Congenital deficiencies may consist of an 
absence of all limb elements below a particular level, 
complete absence of only an intermediate section (e.g., 
upper arm), or the partial absence of a limb element 
(e.g., not all fingers present). The reader is referred to 
the section of chapter 3 on the musculoskeletal system 
for a review of the extremities. 

The exact incidence and prevalence of limb 
amputation in the United States is difficult to 
ascertain. The National Center for Health Statistics of 
the U.S. Public Health Service estimated a prevalence 
of 311,000 amputees in 1970. An incidence of 
approximately 43,000 new amputations per year is 
estimated, of which 77 percent occur in males, and 90 
percent involve the legs. In lower extremity 
amputations, 40 percent are above the knee, 50 
percent are below the knee, and 10 percent are at the 
hip. Of particular interest is the fact that 58 percent of 
amputations occur in those between 21 and 65 years of 
age. If we include the late teenage population, two- 
thirds of all amputees are potential candidates for 
vocational rehabilitation. 

Amputations may be traumatic or surgical. The 
traumatic amputations result from a sudden severe 
injury. Explosions, mangling in a machine, severance 
by a sharp object, or burns, for example, cause 
traumatic amputations. Surgical amputations are 
usually associated with cancer (see chap. 28) or 
peripheral arterial disease (see chap. 22). Surgical 
amputation for cancer is usually major, occurring at 
either the shoulder or hip and usually in young adults. 
Osteogenic sarcoma of bone is the most frequent cause. 
Vascular amputees comprise 89 percent of the amputee 
population over 50 years of age. Half of these patients 
have diabetes mellitus (see chap. 24). Progression of 
peripheral arterial disease is generally slow. 
Amputation becomes necessary when occlusion of the 
vessel either by progressive encroachment on its 
interior or by a sudden clotting (embolus) causes death 



(gangrene) of that part of the limb ordinarily nourished 
by the vessel. 

Congenital limb deficiencies evolve largely from 
insults during pregnancy. Drugs (e.g., Thalidomide) 
and X- radiation have been implicated. Genetically 
induced limb deficiencies are not transmitted from 
generation to generation, as they are recessive traits or 
one-time changes in the genes. 

In general, the disabilities, treatment, prosthetic 
prescription, and vocational implications of 
amputation are similar regardless of cause. Where 
special considerations for a specific cause exist, they 
are noted throughout the chapter. Otherwise, this 
chapter deals with amputations generally, regardless of 
cause. Psychosocial factors are considered first. Upper 
and lower extremity amputations, prosthetics, and 
vocational considerations are then discussed, followed 
by consideration of the complications of amputation 
and prosthesis use. 

PSYCHOSOCIAL FACTORS 

Amputation as punishment, common in ancient 
times, still occurs in isolated areas. The concept of 
amputation as punishment for sin persists, and a 
feeling of guilt and shame often accompanies an 
amputation. Further, the disfigurement and 
destruction of a previous self- image contribute to the 
possible development of introversion, self-pity, 
feelings of inferiority, and social isolation. Anger and 
frustration are also common responses. Not all of these 
psychological characteristics, of course, are seen in 
every patient. 

A reactive depression, however, is natural after an 
amputation. Proper preoperative preparation and 
good guidance through the amputation, prosthesis 
fitting, and training phases helps prevent the 
depression from becoming chronic. The total 
treatment principles outlined in this chapter help avoid 
unnecessary delays in the development of skill in use of 
a prosthesis. This in turn also helps prevent chronic 
depression and social isolation due to lack of contact 
with family, friends, and employment opportunities. 



169 



170 Friedmann 



The traumatic amputee has no psychological 
preparation for the loss of a limb and therefore 
experiences a sudden shock. Adjustment to the 
amputation may be more difficult for him than for 
those who have had some preparation. Additional 
concerns also exist for patients whose amputations 
were required by cancer or arterial disease. The 
counselor should be aware of the whole complex of 
psychological responses associated with cancer (see 
chap. 28) and the anxiety and fear of possible loss of a 
second limb from arterial disease. 

Individuals with congenital limb deficiency have 
diverse problems quite different from those of 
individuals who lose limbs later in life. Sudden loss is 
not their concern. During their childhood, parental 
rejection or parental overprotectiveness may have been 
experienced if the parents were unable to successfully 
adjust to having a "deformed child." Both of these 
reactions inhibit social maturation of the child with 
congenital limb deficiency. Reduced mobility, if the 
lower extremities are involved, and reduced function, 
if the upper extremities are involved, limit the 
experiences of the growing child and contribute to 
relative immaturity as adulthood is reached. 

Some methods of functioning which are acceptable 
in children with congenital limb deficiency are not 
acceptable in the adult. For example, the development 
of leg, foot, and toe dexterity to substitute for upper 
extremity deficiencies may be considered "cute and 
brave" in the child, but not in the adult. The adult 
may well be unprepared to handle the rejection by 
peers, parents, and friends of activities that were 
acceptable when he was younger. Withdrawal, 
depression, and feelings of inferiority may result. 

In addition to restricted experiences, persons with 
congenital limb deficiency may also have missed a 
considerable amount of time in school due to 
hospitalization for surgery. They may therefore have 
educational deficiencies. 

The counselor must be alert to the possible existence 
of the above psychosocial disability factors. If 
suspected, they require careful evaluation and 
treatment and incorporation into the vocational plan. 



UPPER EXTREMITY AMPUTATION 

Levels 

An amputation in the upper limb may be at any 
level along its length. Figure 13-1 illustrates the 
varius amputation levels and the terminology used to 
describe them. The most severe level, the forequarter 
or interscapular - thoracic amputation (not shown in 
fig. 13-1), removes the entire arm, scapula, and 
clavicle, leaving the patient with only the rib cage. The 
shoulder is therefore eliminated. In the shoulder 
disarticulation (S/D) amputation, the arm is removed at 
the shoulder (glenohumeral) joint. The scapula and 



Shoulder 
* disarticulation (S/D) 



Short 
above-elbow (A/E) 



Standard 
above-elbow (A/E) 



Elbow 
disarticulation (E/D) 

Very short 
below-elbow (B/E) 

Short 
below-elbow (B/E) 

Medium 
below-elbow (B/E) 

\ I Long 
A f below-elbow (B/E) 

J^X Wrist 
fff\)S disarticulation (W/D) 

FIGURE 13-1. Classification of upper extremity amputation 
levels. 

clavicle remain and the shoulder is preserved. The 
above - elbow (A/E) amputation refers to any level below 
the glenohumeral joint. The very short above -elbow 
amputation is functionally the same as the shoulder 
disarticulation because the short humerus is literally 
buried in the axilla. An above -elbow amputation 
below the anterior axillary fold leaves a bony and soft 
tissue stump which can be contained by an artificial 
limb socket. 

The elbow disarticulation (E/D) amputation removes 
the arm through the elbow joint. This leaves an 
irregular bony end covered mainly by skin alone. The 
below-elbow (B/E) amputation passes through both 
bones of the forearm (radius and ulna). The more 
proximal the amputation, the greater the muscular 
covering of these two bones. The more distal the 
amputation, the more irregular the shape, for the 
radius and ulna are relatively more exposed. The wrist 
disarticulation (W/D) leaves an irregularly shaped distal 
forearm, with almost no soft tissue under the skin over 
the bone ends. Finally, the partial hand amputation may 
vary from loss of one or more digits to a loss of 
complete fingers or a loss of part of the palm and 
associated fingers. 

Functional Disabilities 

The functional disabilities associated with unilateral 
upper limb amputation at all levels are not as great as 
might be expected. Immediately after the amputation, 



Amputation 171 



impairments in eating, dressing, bathing, writing, and 
driving are apparent. Usually less than a week of 
training in new techniques with the use of adaptive 
devices is sufficient to restore independence in most 
functions, even without a prosthesis. Performance of 
these tasks is easier with a prosthesis, however. If the 
dominant hand has been amputated, redevelopment of 
writing skills takes longer. Activities that absolutely 
require bilateral skills cannot be performed until the 
prosthesis has been fitted, and if the dominant hand 
has been amputated, more extensive training is 
required for bilateral skills because dominance must be 
developed on the other side. The more proximal the 
amputation, the more difficult it is to restore bilateral 
skills. 

With bilateral upper limb amputation, the problems 
multiply because the patient now has no hand to do 
fine manipulation. Nonetheless, the bilateral amputee 
can usually develop independence in all of the above- 
mentioned functions relatively easily. Even without 
prostheses, these skills can be achieved through more 
extensive use of modifications at home and adaptive 
clothing and equipment. Writing with a prosthesis is 
possible, but typing is not very practical, and a tape 
recorder is usually needed for notes at lectures or 
meetings. The bilateral amputee can be trained to 
drive, some even without prostheses. 

The bilateral shoulder disarticulation amputee has 
the most serious problems with self- care and may 
never develop independence, even with prostheses. 
Special clothing adaptations are obviously required for 
this severe disability. Some can learn to independently 
dress, eat, bathe, write, take care of personal hygiene, 
and even drive by training their feet to act as hands. 
The hips, knees, and toes must be very limber, and 
such activities are therefore much more difficult for the 
adult who loses both arms at the shoulder level than for 
the child who is born with such a loss or had an early 
accident. 

Principles of Management 

Preoperative preparation. Before a surgical 
amputation, except in cases of acute emergency, the 
patient usually receives an outline of the immediate 
postoperative care, initial dependency to be expected, 
training required to achieve independence, a brief 
description of the prosthesis and of prosthesis training, 
and expected outcome. This presentation should 
incorporate knowledge of the patient's intellectual 
capacity, skills, current life and family responsibilities, 
and vocational and avocational requirements. When 
feasible, the patient is introduced to the treatment 
team to be involved in his recovery: physician, physical 
therapist, occupational therapist, psychologist, social 
worker, rehabilitation counselor, and prosthetist. 
Introducing him to another patient of similar age, level 
of amputation, and medical condition is also advisable, 



if possible. 

An amputation, even a therapeutic one, is often 
viewed as a treatment failure. This is stressful to both 
the patient and the physician. Total team treatment 
helps prevent severe reactions to this stress, and helps 
the patient achieve a positive but realistic attitude 
toward his future. 

Maintenance of hope for the future is vital to the 
psychological well-being of the amputee. It enlists his 
cooperation and that of his family in therapeutic efforts 
on his behalf. Instilling an unrealistic concept of 
potential future function, however, creates a basis for 
later disillusionment that is sure to lead to depression, 
resentment, and even legal redress. Honest appraisal 
at all stages of the amputee's treatment is essential to 
retain respect and obtain cooperation. 

The following items should be included in both 
preoperative and postoperative discussions with the 
patient and his family. This information cannot and 
should not be presented in too brief a form and perhaps 
not all at once. Patients cannot assimilate more than a 
few of the items at one time and the information must 
be repeated often after amputation. 

1. The sensation that the amputated limb is still 
present ("phantom limb") is normal in all 
amputees and is not evidence of an emotional 
disturbance. 

2. A functioning and happy life can be achieved 
and maintained after amputation. 

3. Adjustments will have to be made in order to 
cope with the various problems engendered by 
amputation. These problems can be psycho- 
logical, physical, social, vocational, and 
recreational. 

4. Becoming accustomed to wearing the prosthesis 
takes several weeks, since it does not feel 
like a normal limb. It lacks sensation and, at 
the beginning, seems heavy. 

5. There will be a training period to achieve skill 
with the prosthesis. Without training, the 
patient will be unable to use the prosthesis 
adequately. 

6. The stump shrinks as a result of reduced use 
of residual muscles, bandaging, elastic "stump 
shrinkers," and pressure from the prothesis. 
Prosthetic adjustments will therefore be required 
over an indefinite period in the future. 

7. The prosthesis is basically cosmetic and partially 
functional. It will not wholly substitute for 
the amputated limb, and some frustration and 
disappointment with it should be expected. 

8. Independence in most functions can be achieved 
in a few weeks with the help of the treatment 
team. 

9. No cosmetic prosthesis is ever entirely natural 
looking, especially at close range. 

The prosthesis must be viewed by the medical team 
and by the patient as a tool to perform certain tasks 



172 Fried man n 



and not as a replacement for the human limb. Hope 
must be tempered with realism, so that this useful 
force is not converted into disappointment and 
resentment. 

Surgery. Surgical amputations and stump revisions 
are usually performed by an orthopedic surgeon, but 
may also be performed by general or vascular surgeons. 
Wounds are bandaged with soft dressings either with or 
without elastic wraps to control postoperative edema. 
Some centers use plaster of Paris for final wrapping 
(rigid dressing). Others shape plaster of Paris into a 
socket of a temporary, detachable prosthesis. This 
temporary prosthesis, while different in nature from 
the later permanent prosthesis, allows the patient to 
begin rehabilitation efforts early. 

Partial hand amputations are often revised to 
achieve pinch (prehension) or grasp without a 
prosthesis, thereby preserving sensation. Reconstruction 
after loss or congenital absence of a thumb is an 
example. In this procedure, the index finger and its 
metacarpal are rotated and displaced to perform as a 
thumb able to oppose the middle and other fingers 
(see fig. 32-7, p. 422). 

Within a week or less after surgery, patients with 
uncomplicated unilateral upper limb amputations and 
revisions can be discharged from the hospital to 
continue their rehabilitation as outpatients. 

Postoperative care. Within a day or two after 
surgery, the patient will begin physical therapy for 
posture training and an exercise program to maintain 
and improve the range of motion and muscle strength 
of the uninvolved joints of the limb. The patient will 
also receive retraining by an occupational therapist in 
activities such as personal hygiene, dressing, and 
eating, and in using the opposite limb if a shift in 
handedness is required. The daily training program is 
generally at least 2 hours in physical therapy and 2 
hours in occupational therapy for perhaps 2 to 4 weeks. 

A period of about a month after surgery may be 
necessary for the stump size to stabilize sufficiently to 
receive the final prosthetic socket. Elastic bandaging, 
elastic ' 'stump shrinkers, ' ' and serial castings are often 
used to facilitate this maturation. 

Prosthesis Description 

The functions of the upper extremity which the 
prosthesis attempts to replace are very complex. The 
proximal portions of a normal upper extremity serve to 
position and stabilize the hand for action. The muscles, 
largely those at the shoulder and elbow but also those at 
the wrist, are used to move these joints so that the hand 
can be positioned for performance. Hooking, 
grasping, and pinching are the main modes by which 
objects are held. Manipulation is then performed by 
the many muscles controlling finger and wrist action. 

In addition, adequate function requires sensory 
feedback not only from the skin of the hand, but also 



from the muscles themselves. Ordinarily, one of the 
upper extremities develops greater dexterity than the 
other. This is what is meant by "handedness." No 
prosthesis applied to the dominant extremity can 
achieve dexterity equivalent to prior function. 
Handedness must therefore shift after dominant 
extremity amputation. 

The prosthesis, which attempts to duplicate lost 
function, has the following components: (a) 
suspension, a method to hold the prosthesis on the 
body; (b) a socket to interface with the stump skin; (c) 
mechanical joints to replace lost joints; (d) a terminal 
device to substitute for hand loss; and (e) a source of 
power both to pre -position the terminal device and to 
activate it. Feedback, although partially received from 
pressure of the socket, suspension apparatus, and 
power activation, is primarily received by the amputee 
through visual inspection. 

Suspension. Methods of suspending the prosthesis to 
the body include straps, suction between the socket and 
the stump, or shaping of the socket over bony 
prominences to achieve a clamping effect. A common 
type of strap suspension is the so-called figure -8 
harness, which anchors the prosthesis to the opposite 
shoulder via straps that cross over the back (fig. 13-2). 
Another variation is the "shoulder saddle chest strap 
harness." The latter may be less uncomfortable for 
carrying heavy loads. 

Socket. The socket is always custom made of special 
plastic, and is shaped to the stump and underlying 
muscle and bone. Total contact is required, i.e., the 
socket must be in contact with the entire stump. This 
total contact may be hard, soft, or semisoft. Some 
sockets are made with a distal air chamber for special 
purposes. Total -contact sockets support the distal soft 
tissues, prevent edema, and provide additional sensory 
feedback. The socket usually has two walls, the inner 
wall or stump wall which is in intimate contact, and an 
outer cosmetic or structural wall designed to resemble 
the limb (fig. 13-3). To avoid additional weight, the 
space between the walls is not filled. Stump socks, of 
varying thicknesses, are often required. They absorb 
perspiration and protect the skin from friction. 

Joint replacement. The joints of a prosthesis are 
relatively simple. They do not in any way approximate 
the complexity of the joints they replace and usually 
move in only one plane. A passive joint is one which is 
not provided with power that controls movement. 
Movement is achieved by gravity or positioning by the 
opposite arm. An active joint is provided with power. 
Joint stabilization is achieved, when desired, via 
mechanical locks or friction devices. Shoulder joints 
are usually passive but may be coupled with friction. 
Wrist joints are passive and contain friction devices for 
stabilization and positioning. 

Terminal device. The most important part of the 
upper limb prosthesis is the terminal device, either a hook 
or a hand. Terminal devices mostly pinch, hold, or grasp. 



Amputation 173 





FIGURE 1 3-2. Figure-8 suspension harness on patient with right 
A/E prosthesis. Note cable attached to harness and passing into 
cable housing anchored to socket of prosthesis. 



FIGURE 1 3-3. Above-elbow amputation socket cut open to show 
double-wall structure. Inner wall is in total contact with the 
stump and was custom made from a plaster cast of stump. Outer 
wall "fills out" the upper arm. The flat base serves as a place of 
attachment for prosthetic elbow joint. 








FIGURE 13-4. (Left) Aluminum hook lined with latex rubber (neoprene) in the open position. The cable is attached to the "thumb" ot 
the hook which, when pulled, causes the "finger" on the left to move awav from the "finger" on the right, which is always stationary. 
The patient's voluntary action creates the force in the cable to open the hook (V.O.|. As the "fingers" separate, the wide rubber band at 
the base of the "fingers" stretches. When the cable force is relaxed, the rubber band causes the hook to close. The hook is screwed into 
the wrist unit and held in position bv a rubber disc in the unit (friction wrist). (Right) Heavy-dutv steel hook "utility," "farmer's," or 
"carpenter's." hook) in the closed position. The surfaces of the "fingers" are serrated (not shown). The shape of the hook [compare 
with fig. at left) allows it to hold things such as nails, files, and hammers. The bolt at the base of hook screws into a wrist unit 



They cannot sense shape, texture, or temperature and 
they do not duplicate in any way the wide range of 
manipulation and dexterity of a normal hand. 

Hooks are made of aluminum for lightness or steel 
for heavy use and come in a variety of sizes and shapes. 



All are characterized by two opposing surfaces, one of 
which is movable. Steel hooks are used for heavy work, 
while the aluminum hooks are used for everyday life 
and sedentary, desk-type jobs. Aluminum hooks 
generally come with a latex rubber inner surface to add 



174 Friedmann 



friction to enhance contact with such objects as 
doorknobs, paper, and pencils (fig. 13-4, left). Steel 
hooks may come with or without the rubber gripping 
surface. They may also be in different shapes for 
specific functions and have earned titles such as 
"utility hook" or "farmer's hook" (fig. 13-4, right). 

A source of power is necessary for active terminal 
devices. Power may be used to open the device 
(voluntary opening, V.O.), with closure achieved 
passively via springs or rubber bands, or power can be 
used to close the device (voluntary closing, V.C.), with 
opening achieved passively. Some terminal devices can 
voluntarily open and close and also can be locked at 
various open positions. 

The prosthetic hand resembles a normal hand only 
in appearance. It has a palm and five fingers but no 
real dexterity. The cosmetic hand is fully passive and 
does nothing. The functional hand has only one plane 
of motion, opposition of the thumb against the tips of 
index and middle fingers in a three -finger pinch (fig. 
13-5). Encirclement of the three fingers around an 
object also adds grasp function. 

Both cosmetic and functional hands are covered with 
cosmetic gloves designed to match a person's skin 
color. These gloves can be cut easily by sharp objects 
and are easily stained by newsprint, mustard, ink, and 
other dyes. They therefore can only be used in "clean" 
situations, as replacements are expensive. 

The fine tips and carefully engineered flat opposing 
surfaces of hooks give them much greater utility than 
functional hands in a performance setting. Patients are 
usually fitted with hooks first and hands second. Many 
end up using both, hooks for "work" and hands for 
"play." 

Power. Power to move active joints, activate locks, 
or open or close the terminal device may be obtained 
by harnessing movement produced by unamputated 
muscles. This is achieved by a cable running from the 
normal joint supplying the power to the part of the 
prosthesis to be moved. Power can also be supplied 
externally from compressed gas or from a battery 
source built into the prosthesis. Whether the power is 
wholly internal or external, control of its activation is 
achieved through movement of an uninvolved body 
part or from action potentials induced by muscle 
contraction (see p. 19). 

Prosthesis Prescription 

The specific prosthesis prescription will depend 
upon the patient's anticipated requirements for his 
activities of daily living, and his vocational, 
avocational, and cosmetic needs. The prosthesis will 
generally be prescribed by a physiatrist or orthopedic 
surgeon. Other physicians generally are not trained in 
prosthetics or knowledgeable on what is available. 
Input from the treatment team can help ensure that 




FIGURE13-5. Northrup-Sierra voluntary opening functional 
hand without cosmetic cover. Thumb has two possible posi- 
tions. The thumb position depicted allows for three-finger 
pinch. The second position, a more open one, allows for 
grasping of larger objects. Direct external pressure on the 
thumb controls the position. Using power harnessed from 
proximal normal joints and muscles, the amputee can volun- 
tarily pull on the cable to move the index and middle fingers 
together away from the thumb. The ring and little fingers are 
passive and do not move. 

prescriptions will be appropriate. 

Fabrication of prostheses is usually performed by 
certified prosthetists or supervised technicians. 
Fabrication facilities may be found in most large cities 
and in some smaller ones. Artificial limbs can still be 
obtained in communities without such facilities if a 
certified prosthetist is available to send the 
prescription, measurements, and appropriate casts of 
the stump to a central fabricating facility. An adequate 
prosthesis may be obtained in this manner. 
Adjustments are then made locally for final fitting. 
Actual fabrication of a prosthesis takes no more than 1 
to 2 weeks. Longer delays are generally due to 
administrative problems, lack of authorization for 
payment, or inefficiency in the prosthetic facility. This 
may include a lack of an adequate number of people to 
make the prosthesis more rapidly. 

Fitting must be done carefully. A patient will 
abandon an upper limb prosthesis if it is not 
comfortable because he can manage so well with one 
arm. The longer the delay, the harder it is to replace 
inefficient one-handed techniques with bilateral 
techniques. This principle is incorporated in the care of 
congenital limb deficiency, where application of 
prostheses during infancy is advocated. While each 
prescription is specific to an individual patient, certain 
concepts apply to all prostheses for each of the 



Amputation 175 





FIGURE 1 3-6. (Left) Front view of the same patient as in figure 13-2. Note how figure-8 harness anchors around normal shoulder. The 
cable extending to the hook is the same cable described in figure 13-2. The second cable, which enters the prosthesis at the elbow, 
controls the elbow lock. Forward movement of 'he stump (shoulder flexion i will bend the elbow into flexion. In this figure, the patient 
has flexed and locked the elbow. Shoulder flexion, if executed now, will open the hook. The turntable, shown just above the elbow 
lock cable, has been positioned in external rotation. (Right) The turntable is now in internal rotation, which places the hook in front of 
the bodv. The patient has now flexed the shoulder, causing the hook to open. 



amputation levels with regard to suspension, socket, 
joint replacement, terminal devices, power, and 
control. 

Shoulder disarticulation and interscapular - thoracic. 
Both S/D and interscapular- thoracic amputations are 
extremely difficult to fit with a prosthesis for effective 
function. The prosthesis is often discarded unless it 
provides a clear advantage to the patient's work or 
avocation. The socket ("shoulder cap") covers a large 
area of what is left of the shoulder and trunk and is 
suspended via chest straps. At this level, harnessing of 
power from other joints is most difficult. To activate 
and move a cable attached to the limb, use of chest 
expansion or opposite arm movement is required. Use 
of a good normal arm to operate a prosthesis, however, 
is not particularly recommended. The shoulder joint 
replacement is passive and has limited motion in 
flexion and abducation. Positioning prior to use is 
usually done with the good arm, and friction to hold a 
specific position may be used for the shoulder joint. 
Use of the opposite hand for positioning elbow flexion 
or for operation of an elbow lock may be used. Power 
harnessed from other joints may only be used for the 
terminal device. External power may also be 
prescribed. The terminal devices that can be used are 
hands for cosmesis and lightweight aluminum hooks if 
an active device is required. The amount of body 
power that can be obtained is not sufficient to operate a 
functional hand. The prosthesis is heavy and difficult 
to put on. Cosmesis may be the only goal for most. 

Above-elbow. Double-wall sockets are used for A/E 



prostheses. Figure-8 suspension is used and stump 
socks are preferred. Elbow joint replacement is a 
prefabricated item. Its mechanism is internal, 
occupying the space distal to the stump. Since shoulder 
rotation cannot be transmitted to the terminal device 
because of the soft tissue in the stump, a turntable is 
added to the elbow mechanism. The turntable can be 
pre -positioned by the amputee to a desired position of 
rotation (fig. 13-6). A friction wrist or a mechanical 
wrist which can be locked into one of several positions 
of pronation and supination, using the opposite 
extremity, is preferred. The choice of a hook or a hand 
for the terminal device is governed by needs. 

Power is required for three functions: elbow flexion, 
elbow locking, and terminal device operation. This 
power is obtained exclusively from harnessing shoulder 
motion via cable attachments to the forearm section, 
the elbow lock, and the terminal device. At least two 
motions (power sources) are required. The sequence of 
action is usually shoulder flexion to achieve elbow 
flexion, immediate shoulder depression and abduction 
to lock the elbow, and then shoulder flexion again to 
operate the terminal device. Cables are so attached 
that once the elbow is locked, the shoulder flexion 
action force can be transmitted to the terminal device. 
External power triggered by shoulder motion is 
increasingly being used for elbow flexion and 
automatic elbow locking. The devices are still 
experimental, expensive, and fragile. 

Elbow disarticulation. Suspension of E/D prosthesis 
is again achieved by a figure-8 type shoulder harness 



176 Friedmann 



and by socket shaping. The socket is shaped to the 
upper arm and is usually double -walled. The forearm 
section is usually prefabricated, and wrist units and 
terminal devices as described above are attached to it. 
Generally, voluntary opening terminal devices are 
preferred. An external elbow joint which can be locked 
in several positions connects the socket to the forearm 
section. Harnessing of power from proximal joints for 
operation of elbow flexion, elbow locking, and 
terminal device operation is the same as for A/E 
prostheses. 

Below - elbow . Figure -8 suspension and a double - 
walled total -contact socket with a stump sock are usual 
for B/E prostheses. Unless the stump is quite long, a 
wrist unit as above is required for pronation and 
supination. A voluntary opening device can be 
prescribed. An elbow hinge connecting the forearm 
socket to an upper arm cuff anchors the cable. Rigid 
metal hinges are used if the stump is too short to 
achieve active pronation and supination. The hinge is 
made of flexible material when pronation and 
supination can still be actively achieved. Cable control 
is again by shoulder flexion. 

A myoelectric prosthesis (fig. 13-7) should be 
carefully considered for B/E amputations. The 
prosthesis motor is activated by the myoelectric 
potentials produced by the muscles and picked up by 
electrodes situated over the skin covering the muscles to 
be used. It is suspended by shaping of the socket 
around bony prominences at the elbow. No shoulder 
straps are necessary. The electrical circuits, battery, 
and motor are contained within the prosthesis, making 
it heavy. Currently, hands are used which can be both 
V.O. and V.C., with locking at any position. The 
terminal device is activated by using the same muscles 
normally associated with opening and closing of the 
hand. Myoelectrically controlled hooks may also soon 
be available. 

Wrist disarticulation. Suspension of W/D pros- 
theses is mosdy via socket shaping, although the 
figure -8 harness can assist. The socket is double 
walled, the inner wall shaped snugly over the forearm 
ends. A thin stump sock may also be used. The socket 
shape allows forearm pronation and supination motion 
to be actively achieved, and a friction wrist joint 
can be kept in any position. Interchangeable hooks 
and hands are used. Power is achieved by harnessing 
energy from proximal joints through a cable attached 
to the terminal device as above. A myoelectric prosthesis 
can also be considered for wrist disarticulation, but 
it is long and the functional gains are minimal. 

Fhrtial hand. Suspension of prostheses for partial 
hand amputations is achieved via straps and socket 
shaping about the wrist. The socket is usually applied 
without a stump sock. No joints are replaced. The 
terminal device may simply be a passive cosmetic 
completion of the hand or a passive, specifically 
designed post or surface against which parts of the 




FIGURE 1 3-7. Bilateral below-elbow amputee, with two VA- 
Northwestern University Below-Elbow Myoelectric Prostheses. 
Harnesses are not necessary. No cables are required, and 
suspension is achieved by shaping the socket around the bony 
prominences at the elbow. The electrodes registering the 
electric signals from the muscles in the stump are buried in the 
socket walls and cannot be seen. The cosmetic cover extends to 
the mid-forearm level. The thumb and the index and middle 
fingers voluntarily open and close when the amputee contracts 
the muscles of the back and front sides of the stump, respec- 
tively. (Photograph courtesy of Bulletin of Prosthetics Research, 
Veterans Administration 

remaining hand are applied for grasp. No power is 
necessary. 

Bilateral amputee prescription. In the bilateral 
amputee, all the above principles apply. In addition, it 
is rare that a bilateral amputee regularly uses two 
prosthetic hands because hands do not provide 
sufficient function. Hooks are therefore preferred. 
Further, at least one wrist flexion unit may be used. 
This is a passive device pre -positioned and locked in 
flexion before use to bring at least one terminal device 
in close to the body. Suspension of bilateral prostheses 
may be achieved by separately harnessing and 
suspending each prosthesis or by tying them together. 
The latter makes donning easier, but the former 
provides greater versatility and preserves at least one 
functioning arm should one of the prostheses be in 
need of repair. 

Congenital limb deficiency. Prosthesis prescription 
for congenital limb deficiency becomes much more 
individualized to utilize all parts of the extremity, 
particualrly when the loss is of an intermediate part 
(e.g., elbow and wrist gone with hand residuals at the 
shoulder). Efforts are made to take advantage of even 
vestigial elements. Prosthesis prescription for such 
patients requires a center with professionals 
experienced with limb deficiency. 



Amputation 177 



General. Each prosthesis must be custom made for 
the particular patient's needs. Many patients need 
multiple prostheses for different uses. Any prosthesis is 
a tool with which the patient performs one or more 
jobs, including not only work, but also play and 
personal care. Other things being equal, the prosthesis 
must be as good looking as possible and, in unilateral 
amputees, create a balanced appearance. However, 
the patient, his family, and his employers need to 
recognize that no prosthesis is truly cosmetic. There is 
always a balance between the appearance of a 
prosthesis and its function. Unfortunately, especially 
in upper limb prostheses, cosmesis decreases as 
•function improves. The amount of attention that must 
be paid to appearance depends upon the individual 
patient, the society in which he lives, his ethnic group, 
and his job. 

Each person's standards of what is appropriate will 
vary. Some are so disturbed by the fact of the 
amputation that they strive for perfection in 
appearance. This is not attainable since all pigments 
used for hand covers appear a different color under 
day, incandescent, and fluorescent light. Striving for 
less noticeability, rather than true cosmesis, is more 
appropriate. 

The noticeability of an artificial hand may be 
decreased by the use of a wrist watch or a conservative 
ring to draw attention away from the abnormal 
appearance. The noticeability of the lack of dynamic 
movement of the hand may be diminished if the 
patient uses an adhesive bandage around one of the 
fingers. This creates the illusion of injury as a cause for 
lack of movement. Clothing is another way to draw 
attention elsewhere. While some patients may use such 
"tricks" initially to help them become accustomed to 
the prosthesis, they may soon find them pointless. 

Prosthesis Checkout and Training 

The prosthesis is best not given directly to the 
patient upon completion of fabrication, but should 
rather be delivered to the treatment facility. This 
avoids early failures and disappointment. The 
checkout and training for an upper limb prosthesis are 
usually done by an occupational therapist. Checkout 
proceeds according to a standard format, beginning 
with a check to see if all components are as prescribed. 
Systematic examination of suspension, socket fit, 
power operation and control, and joint and terminal 
device operation follows. Certain minimum standards 
have been established. The physician and treatment 
team then review for corrections. If the prosthesis is 
satisfactory, training can begin. As training proceeds, 
the occupational therapist may make suggestions for 
further improvements as the patient utilizes the device. 

For a B/E prosthesis, the training program is a 
matter of about 2 weeks, when done on a daily basis. 
For the A/E amputee, up to 4 weeks may be necessary. 



Training of bilateral amputees and patients with more 
proximal amputations may take longer. 

The training will include the care of the skin of the 
stump, maintenance of stump socks, an explanation 
of what the prosthetic components are and how they 
operate, and prosthesis maintenance. The patient first 
learns controls operation alone so that he understands 
how the power is transmitted from his own muscles or 
from external sources to the parts of the artificial limb. 
After achieving some skill in controls operation, the 
patient will be taught how to use the prosthesis for the 
activities of daily living, such as toileting, personal 
hygiene, eating, dressing, general housework, and 
driving. Subsequently, the patient is trained in more 
complex tasks related to actual vocational and 
recreational needs. The patient also spends 
progressively more time in public settings to establish 
confidence, with the knowledge that people will stare 
and may be inclined to turn away or ask questions. 
Guidance in these public forays, first with family 
members or with the occupational therapist and then 
alone, establishes confidence. 

Followup Management 

Physician followup will be required weekly at first 
and then at progressively longer intervals to ensure 
that there is no stump breakdown and that the 
prosthesis functions adequately for the patient's needs. 
In the first 1 to 2 years, followup visits will be required 
every 3 to 6 months to provide stump socks, to ensure 
the prosthesis still fits and is functioning properly, for 
routine professional cleaning and maintenance of the 
prosthetic components, and to guard against 
complications. The patient is also instructed to come in 
sooner if any problem or emergency arises. 

A new socket may be needed twice in the first 2 years 
after provision of the prosthesis, depending upon use, 
due to a change in stump size caused by skin and 
muscle atrophy. The first new socket is usually 
required at 4 to 6 months, and the second at 24 
months. Even without a need to change because of 
stump alteration, a prosthesis may last only 2 or more 
years, depending upon the severity of use and the care 
it is given. Whether the patient needs just a socket or a 
whole prosthesis, and whether any components can be 
salvaged, varies from patient to patient. Like any 
mechanical device, if the prosthesis is cared for and 
used judiciously, it lasts longer than if it is abused. 

Vocational Implications 

The majority of clients who were engaged in 
professional, managerial, or executive careers prior to 
a unilateral arm amputation return to their former 
employment, especially if they are under 50 years of 
age. Thus, those whose work is intellectual do well 
vocationally. The vocational disability of the loss of 



178 Friedmann 



one or both upper limbs is much greater for those 
whose former occupation required manual labor. 

The lower the educational level, the greater the 
problem. Careful vocational evaluation and guidance, 
job training, and education to prepare for white collar 
employment or more intellectual pursuits are usually 
required. The counselor must, however, first analyze 
the client's previous occupation to determine whether 
the amputation is a real, or only an apparent, 
vocational impediment. Frequently, modifications of 
the job task or of specific responsibilities can be made. 

Clearly, the bilateral amputee will require an 
extensive review of his interests, aptitudes, educational 
level, and skills with prostheses, and perhaps also with 
his feet, if such dexterity has developed. 

The client with congenital limb deficiency may need 
more extensive psychological review and educational 
assessment. Education may have been impaired 
because of multiple medical visits or prior 
hospitalizations for surgery. Social maturation may be 
inadequate due to a protective upbringing. These 
psychosocial factors, rather than physical inability to 
do a job, may well be the primary vocational problem. 
More extensive vocational preparation may therefore 
be required for these clients than for amputees in 
general. 

No prosthesis provides real sensation, though 
some amputees do get some sense of what is happening 
at the terminal device from strap and harness pressure. 
All amputees, however, must use vision to get sensory 
feedback. Eye-hand coordination and finger-hand 
dexterity skills are impaired or absent. The client's 
interests in employment areas that require such skills 
cannot be developed, and exploration and 
development of other interests is necessary. 

A client who works in an office will generally be 
advised to have an aluminum hook or a functional 
hand, while a person who works in a factory or who 
does heavy manual labor will generally be given a 
voluntary opening steel hook. A person in contact with 
the public will generally want a cosmetic hand, but it 
should be remembered that the cosmetic gloves cannot 
withstand heavy use. It is quite likely that the client 
will need different terminal devices for different 
vocational, recreational, and social activities. The 
concept of the prosthesis as a tool to perform a certain 
job is important in this context. Multiple terminal 
device prescription is therefore generally warranted. 

The prosthesis is used for holding, while the normal 
hand is used for manipulation. If the client's dominant 
limb has been amputated, job performance will require 
training to shift dominance to the other side. 

The climate in the client's place of residence and the 
nature of the work environment are very important. 
Hot, humid environments may induce skin 
maceration, due to excessive sweating and friction 
between stump and socket. Loss of heat by the body 
may be impaired, due both to the socket and to loss of 



surface area. The macerated skin is easily scraped by 
repetitive tasks. Working in wet locations may cause 
prosthesis deterioration. Dust or grit in the 
environment may be abrasive to the skin and grind 
the moving parts of the prosthesis. Provided wool 
stump socks are utilized, cold is less of a problem. The 
prosthesis must, however, be made of a material that 
does not transmit cold to the stump readily. The 
environment may determine the frequency of 
prosthetic repairs, especially if these factors were not 
taken into consideration in the initial prescription. 

The physical demands of a job are particularly 
important for the amputee client. The general physical 
capabilities of clients with amputations at each level are 
discussed below. 

Interscapular - thoracic and shoulder disarticulation. 
The prostheses for these high-level amputations are 
essentially cosmetic. The terminal device cannot be 
operated higher than desk level. Locks can be placed 
on the elbow to carry objects, but an object placed in 
the terminal device cannot be lifted by movement of 
the prosthetic joints (live lift). Lifting can only be 
accomplished by locking the elbow at an angle, 
bending down and securing an object in the terminal 
device, and then straightening up again. The 
maximum pinch force of the hook is usually only two 
pounds. 

Above-elbow. The A/E amputee can position the 
terminal device somewhat below shoulder level. Lifting 
force actively increases to about two pounds greater 
than the weight of the forearm, but lifting is generally 
not done. Carrying capacity is considerable, however. 
Up to 50 or 60 pounds can be carried if the elbow is 
straight and locked. Pinch force can increase up to four 
pounds in exceptional cases. 

Externally powered elbow joints now coming into 
use can add more live life at the elbow, up to about 
four to six pounds, but the rate of lift is slow. 

Below -elbow and wrist disarticulation. The terminal 
device of a B/E prosthesis can be raised to shoulder 
height. If active motion above the shoulder is required, 
the shoulder saddle chest strap harness may be used. 
Lifting force approaches normal if the client has a long 
stump. The shorter the stump, the less the lifting 
force. With very short stumps, the lifting force may be 
no more than one or two pounds in addition to the 
weight of the prosthetic forearm and hand or hook. 
Maximum pinch force can be six pounds. 

The myoelectric prostheses for B/E amputation with 
either hands or hooks are generally more suitable for 
office ("white collar") than for factory ("blue collar") 
activity. They are not suitable for heavy work. 

Partial hand. The partial hand amputee treated 
with surgical revision or a custom prosthesis for pinch 
has the advantage of sensation. Special surgery may be 
needed to ensure that the part of the residual hand that 
is used for grasping has sensate skin. Lifting force and 
carrying force are normal. 



Amputation 179 



Hip 

disarticulation (H/D) 

Very short above-knee (A/K) 



Short 
above-knee (A/K) 



Medium 

above- knee (A/K) 



Long above-knee (A/K) 

Very long above-knee (A/K) and 
Knee disarticulation (K/D) 



> Short below-knee (B/K) 



► Standard below-knee (B/K) 



Long below-knee (B/K) 
Syme 



FIGURE 13-8. Classification of lower extremity amputation 
levels. 



T 



of the femur bone. The below-knee (B/K) amputation 
preserves the patella and leaves a functioning knee 
joint. The Syme amputation is a section through the 
ankle. The sharp projecting ends of the tibia and fibula 
are removed. Usually a firm soft tissue pad from the 
heel allows the amputee to walk indoors on the stump 
end without a prosthesis. Transmetatarsal or partial fool 
amputations are usually managed without a prosthesis. 
A firm shoe reinforced with a steel shank through the 
sole and simply filled with lamb's wool or other 
atraumatic material to take up the space is generally all 
that is required. Partial foot amputations, therefore, 
will not be further discussed. 

The Syme amputation is done mainly for injury. 
Half of the Syme amputations done for vascular 
disease (see chap. 22) fail, and a reamputation below 
the knee, where blood supply is more adequate, is 
required. 

The below-knee amputation is usually the procedure 
of choice for peripheral arterial disease and in most 
cases of foot injuries where salvage of the foot is not 
possible. 

The knee disarticulation is generally done for 
trauma or malignancy in young individuals. If the 
patient is still growing, a K/D becomes equivalent to 
an A/K in adulthood due to reduced growth in bone 
length of the femur. 

In the past, above-knee amputations were 
traditional for severe vascular disease. Improved 
surgical techniques for repair of clogged arteries and 
for the amputation itself have decreased the number of 
A/K procedures. Amputations for bone cancer are 
usually quite high. An H/D or at least a short A/K is 
usual. 



LOWER EXTREMITY AMPUTATION 
Levels 

The lower extremity amputation levels shown on 
figure 13-8 are analogous to the upper extremity 
levels. Congenital lower limb deficiencies, as in the 
upper limb, can include losses of intermediate 
segments or partial losses of the separate sections. 

The most severe level of amputation is the 
hemipelvectomy (H/P), or hindquarter amputation, which 
removes the limb through half of the pelvis. The soft 
tissues of the buttock and sides of the trunk become the 
stump. The sitting bone (ischial tuberosity) is usually 
absent. The hip disarticulation (H/D) preserves the 
ischial tuberosity and pelvis. The stump remains 
mostly the soft tissue of the buttock. The above -knee 
(A/K) amputation is the first level to provide a 
movable stump. If the stump is long enough, the 
patient has good hip control. The knee disarticulation 
(K/D), a rare amputation, involves provision of 
healthy skin for weight bearing, and sometimes 
includes removal of the patella and shaping of the end 



Functional Disabilities 

In lower extremity amputations, the basic function 
compromised is ambulation. In some patients, transfer 
skills are also impaired. Personal hygiene, dressing, 
and eating skills are unimpaired. Hemipelvectomy 
patients may have toileting problems due to a poor 
sitting surface. While unilateral amputees quickly 
achieve transfer skills, bilateral amputees need some 
time to learn transfers in and out of a wheelchair. 
Within a few days, unilateral amputees with healthy 
normal limbs can balance well on one leg and ambulate 
with underarm and forearm curtches. Bilateral 
amputees, of course, ambulate via wheelchairs prior to 
prostheses fitting. 

Unilateral amputees, following prosthesis fitting and 
training, achieve full independence in level-ground 
ambulation, most stairs and ramps, and driving. The 
more proximal the amputation, the more difficult 
ambulation will be on rough, uneven terrain, and the 
greater the potential need for a cane. Unilateral hip 
disarticulation and hemipelvectomy patients 
frequently abandon their prostheses during work 



180 Friedmann 



because crutch ambulation is faster. They may only 
use their prostheses for recreation and in social settings 
for better cosmesis. 

Bilateral amputees do not all ambulate satisfactorily 
with prostheses. If ambulation is achieved, two canes 
are usually required, and uneven terrain can only 
rarely be handled. Fitness of the cardiovascular and 
pulmonary systems is necessary for ambulation 
because of high energy requirements to walk with two 
above-knee artificial limbs. All bilateral amputees will 
use a wheelchair some of the time, and those with 
limited ambulation will use wheelchairs a major part of 
the day. 

Principles of Management 

Preoperative preparation. Patient preparation for a 
lower extremity amputation is the same as for an upper 
extremity amputation. Since, however, practically all 
amputations for vascular disease occur in the lower 
extremities, the potential amputee with peripheral 
vascular disease is usually made aware of the following: 

1 . The physicians and surgeons did their best to 
save the leg, and at surgery every effort will be 
made to leave as long a stump as possible 
consistent with good healing and better function 
with a prosthesis. 

2. The care of the other leg is vital, for while 
walking may be achieved with one leg 
amputated, it is difficult or impossible with two, 
particularly if amputated above the knee. 

3. Continuous and indefinite medical supervision 
will be required, not only for care of the stump, 
but also for protection of the other leg and 
management of the arterial disease that 
necessitated the amputation. 

Preoperative preparation includes information 
concerning the need to maintain range of motion and 
strength of the residual joints of the amputated as well 
as the normal leg after surgery. Instruction in 
necessary exercises can even start before amputation so 
that the patient understands what will be required. 

Surgery. An orthopedic surgeon, a vascular surgeon, 
or a general surgeon will generally do the surgery. 
Dressings may include fashioning of a plaster of Paris 
"socket" over the stump with inclusion of a device to 
which a temporary prosthetic leg and foot can be 
attached (fig. 13-9). This early or immediate 
postoperative fitting technique allows for some 
standing, weight bearing, and crutch walking with 
minimal weight bearing within a week. 

Postoperative care. The physical therapist works to 
improve walking function by concentrating not only on 
the amputated side, but also on general body 
conditioning. Exercises to strengthen and maintain 
range of motion of the leg are necessary. Less obvious, 
but just as important, are exercises to strengthen the 
abdominal and back muscles. Strengthening of the 




FIGURE 13-9. Below-knee amputation patient with an immedi- 
ate postoperative prosthesis with plaster socket, leg pvlon, and 
prosthetic foot. The hole in the plaster allows room for the knee 
cap (patella). Bv incorporating the knee joint, the plaster 
prevents knee motion during wound healing. The strap at the 
top of the plaster socket is tied to a waist belt for suspension. 
(Photograph courtesy of Prosthetics Research Study, Seattle, 
WA, Veterans Administration Contract No. V663P-937.) 

arms is also required for successful use of the crutches, 
canes, or walker often required, at least on a 
temporary basis, after amputation. The physical 
therapist also works with the patient on transfer skills 
and crutch ambulation, and provides wheelchair 
training, if required. Occupational therapy assistance 
in dressing and personal hygiene skills may be 
necessary. 

The patient is hospitalized at least 2 weeks, but 
usually longer, before outpatient care is possible. If the 
immediate- fit technique is used, usually two to three 
recastings are required. These are made every 1 to 2 
weeks until stump shape matures and the patient is 
ready for a temporary or permanent prosthesis. If rigid 
plaster cast dressings are not used, a longer period of 
elastic wrappings is usually necessary, and a 
temporary prosthesis is used to condition the stump 
and train the patient. 

Prosthesis Description 

Walking, the basic lower extremity function, is 
much less complex than the multiple functions 
performed by the upper extremities. Prosthetic 
replacement is therefore easier. The components of the 
lower limb prosthesis fall into the same general 
categories as upper limb components and include 
suspension, socket, joint replacement, and, instead of a 
terminal device, an ankle-foot assembly. 

Suspension. A lower limb prosthesis is heavier than 



Amputation 181 




FIGURE 13-10. Patient in an above-knee prosthesis with suction 
socket suspension. The one-wav valve shown at mid-thigh level 
is located close to the bottom of the total contact socket. 

an upper limb prosthesis and therefore suspension is 
more difficult. Adequate suspension is critical to 
ensure that the prosthetic leg does not slip off during 
walking. The many types of suspension available are 
generally the same in principle as in upper limb 
prostheses, employing straps, suction, or socket 
shaping around bony prominences. 

Socket. All sockets are made of special plastics. First, 
a plaster of Paris cast is made of the stump and then a 
mold of the stump is made from the cast. The socket is 
made over this mold, and is shaped to support the soft 
tissues and to transmit body weight to the floor. A 
special valve may be included in the socket for suction 
suspension (fig. 13-10). 

For most prostheses, except those employing suction 
suspension, stump socks are required to protect the 
skin from friction and to absorb perspiration from the 
skin. They vary in thickness and are added as the 
stump shrinks. As a general rule, if more than two 
thick socks are necessary for total contact, a socket 
adjustment is required. 

Joint replacement . Depending on the level of the 
amputation, the prosthesis replaces the ankle, knee, 
and hip joints. The ankle replacement may be 
immovable or movable in a single axis. More elaborate 
ankle joints are available, but are rarely useful. The 
many options for knee joint replacement will be 
discussed below. Hip joint replacements allows single- 
axis movement only. 

Power. In lower extremity prostheses, external 
sources of power are not required. Energy for advance 
of the prosthesis during walking is obtained mainly 
from residual parts. Patients with H/D and H/P 
amputations advance the thigh portion of the 
prosthesis by thrusting the lower part of the body 



forward. The lower portions of the prosthesis follow 
automatically. The A/K and B/K prostheses follow 
the movement of the stump. 

Alignment . An additional factor essential in lower 
extremity prostheses is the vertical alignment of the 
different components. Balance and support of the body 
weight while standing on a prosthesis depends on 
proper alignment of the joints with relation to the 
patient's center of gravity. In particular, the knee joint 
axis must be at or behind a vertical line through the 
center of gravity. Most complaints of knee buckling 
and balance difficulties can be traced to a faulty 
alignment. This may be due to an error in fabrication 
or a reduction in range of motion of the joints 
remaining after amputation and even of the joints on 
the normal leg. Flexion contractures of the hip and 
knee, in particular, may thwart the achievement of a 
successful alignment. Alignment varies with heel 
heights of shoes. Therefore, once alignment is 
established, all shoes worn by the amputee must have 
identical heel heights. If both low heels and high heels 
are regularly worn by women, two removable 
prosthetic feet are required. 

Prosthesis Prescription 

The prescription of a lower extremity prosthesis 
takes into consideration the patient's vocational, 
avocational, and cosmetic needs, whether ambulation 
will include outdoors as well as indoors, the nature of 
the terrain expected, and whether canes will be 
required. Cosmetic factors relate more to how well the 
patient walks than to the appearance of the prosthesis. 
While the shape of the prosthesis attempts to 
reasonably match the normal limb, appearance is not 
an important issue except for women wearing dresses. 

The options available in the various components are 
discussed below for each amputation level. Prosthesis 
prescription for congenital lower limb deficiency is 
consistent with the same principles, but the prostheses 
are further customized depending upon the character 
of the residual appendages. 

Hemipelvectomy and hip disarticulation. Suspension 
of the H/P and H/D prosthesis is achieved by shaping 
the total-contact socket around bony prominences at 
the pelvis and above. The socket is also secured around 
the waist with a strap or an extension of the plastic 
socket. Rarely, straps over the shoulder are required 
for suspension. 

In H/P amputation, the entire mass of soft tissue 
distributes weight to the prosthesis. In H/D 
amputation, weight is transmitted to the prosthesis 
predominantly at the ischial tuberosity. All H/P and 
H/D sockets include a stump sock. 

Prostheses for these high-level amputations replace 
the hip, knee, and ankle joints. A special prosthesis 
called the "Canadian- type Hip Disarticulation 
Prosthesis" is used. It includes a single-axis hip and 



182 Fried mann 



a knee joint. The stability of these joints in the upright 
posture is assisted by an elastic strap. The ankle -foot 
mechanism can be either the single -axis type or an 
immovable assembly called the Solid Ankle Cushion 
Heel, or SACH, foot. 

A cane is required in most situations. Only one 
speed of ambulation (very slow) on level ground is 
possible. Many patients use the prosthesis only for 
special occasions, preferring to go without the 
prosthesis and use crutches or a wheelchair for work 
and more rapid ambulation. 

Above-knee. Suction suspension is preferred for the 
above-knee prostheses (fig. 13-10). For suction 
suspension, the socket is shaped intimately to the stump 
in total contact to conform to underlying muscles, and 
a one-way valve is incorporated near the end of the 
socket. Air is expelled through the valve when the 
patient puts his body weight in the socket. When the 
leg is raised and swung, the force of gravity pulls the 
artificial leg down, but it does not fall off because the 
one-way valve does not permit air to rush back into the 
socket. Air also cannot enter through the top of the 
socket because of total contact. A relative negative 
pressure is thus created to hold the stump in the socket. 
No stump sock is worn with a suction socket. The 
donning of a prosthesis with suction suspension is 
difficult and requires good balance and arm strength. 
It is not advisable for elderly, infirm, or neurologically 
impaired patients. 

Short A/K stumps and stumps that vary in volume 
do not handle suction suspension well. For these 
patients, a webbing Silesian band suspension may be 
added to the prosthesis. If more stability is needed, a 
metal or plastic joint (hip joint) that lines up with the 
patient's hip joint is tied to a belt that goes around the 
patient just below his waist (pelvic band, fig. 13-11). 
No valve is needed, a stump sock is worn, total contact 
is still preferred, and the prosthesis is put on more 
easily than when suction suspension is used. 
Sometimes a hip joint and pelvic band suspension is 
used together with suction and a valve. 

The most common and preferred A/K socket is the 
total contact quadrilateral socket, so-called because of 
its four-sided shape. The older type socket, which was 
totally round, is rarely prescribed. A depression in the 
anterior wall of the quadrilateral socket supports the 
soft tissues and holds the stump so that the patient's 
ischial tuberosity can "sit" on the top of the posterior 
wall of the socket (ischial seat). The shape is modified 
depending upon the amount and nature of the soft 
tissue, the age of the patient, and the specific medical 
problem. 

There are many possibilities for knee joint 
replacement to achieve a stable knee during standing 
and walking. Perhaps more critical than the type of 
joint selected is the alignment and the amputee's ability 
to extend his hip and drive the stump against the back 




FIGURE 13-11. Patient in an above-knee prosthesis with pelvic 
band and hip joint suspension. The pelvic (waist) belt connects 
to the prosthesis via the hip joint on the side next to the patient's 
left hand. The top of the posterior wall of the socket is flat to 
provide a platform for the ischial tuberosity. The overhang of 
buttock skin blocks the view of this platform. 

wall of the socket for knee stability. In addition to 
preventing the knee from buckling, knee joints control 
the amount of bend that occurs during leg swing. 

The "conventional" joint has a single axis. The 
friction between the moving parts of the joint is set 
during training to control leg swing. Only one speed of 
walking is possible with such a constant-friction knee 
without adverse effects occurring. 

Knee joints fitted with any of several types of 
hydraulic mechanisms have variable friction. The 
friction automatically increases as speed of walking 
increases, and decreases as the patient slows down. 
Walking is therefore more natural. A hydraulic 
mechanism which couples knee motion with ankle 
motion is also available and is sometimes preferred 
over the hydraulic knee alone with a SACH foot. 
Hydraulic mechanisms are best suited to active, 
healthy, usually younger A/K amputees who are quite 
able to maneuver without a cane and can handle 
suction suspension well. Occasionally, a knee joint will 
be prescribed with a lock that can be operated 
manually, not so much for walking, except for the 
infirm, but rather for standing activities associated 
with some hand and arm lifting requirements. A 
"safety knee" is like a constant-friction knee that has 
an additional component which inhibits buckling. It is 
sometimes also prescribed, especially for workers who 
must carry weights. 

Ankle and foot replacement is usually with a SACH 
foot. A single-axis ankle joint may be prescribed for 
less agile amputees or for use where the terrain may 



Amputation 183 




FIGURE 13-12. Below-knee prosthesis with thigh lacer, waist 
belt suspension, and single-axis ankle and foot. 




FIGURE 13-13. Below-knee prosthesis with cuff above the 
patella for suspension. Side-to-side (mediolateral) stability is 
achieved by extension of the sides of the socket across the knee 
joint rather than by the lacer and side joints as in figure 13-12. 

not be quite flat. More complex ankle joints are 
available and should be carefully considered. They 
provide flexibility, but less stability. 

Knee disarticulation. Suspension of the K/D 
prosthesis is achieved by waist belt and, in part, by 
socket shaping. The socket is shaped around the thigh 
if the end of the stump is bulbous, and may extend to 
include an ischial seat as in the A/K socket. More 
likely, weight transmits to the prosthesis from the end 
of the stump. The socket has a trap door or expandable 
walls to allow the femoral condyles to reach the bottom 



of the socket. 

The knee joint replacement is usually a hinge placed 
on the side of the socket above the end of the stump 
rather than at the end of the stump. This joint allows 
the knee to flex, but does not allow the prosthesis to 
extend beyond a straight position. Restraining straps 
are sometimes required to reduce the speed of the 
lower leg swing during ambulation. Polycentric 
hydraulic knee units are now available. Ankle-foot 
assemblies are usually SACH. 

Below-knee. For suspension of the B/K prosthesis, 
the socket may be formed around the bony 
prominences about the knee joint. There are two types 
of such suspensions: the supracondylar, which shapes 
over the bony end of the femur, and the suprapatellar, 
which shapes over the patella. More commonly, 
suspension is achieved by a strap anchored above the 
patella. Waist belt suspension is also sometimes 
required, and when used, a leather thigh lacer may be 
added (fig. 13-12). The thigh lacer is tied to the 
prosthesis via metal joints that coincide with the knee 
joint. 

The socket for B/K prostheses is designed so that the 
weight is borne almost entirely on the patellar tendon 
and the medial part of the tibia. Some weight is borne 
on the thigh if a thigh lacer is used. This is most 
important for obese patients and workers carrying 
heavy loads or walking a great deal. The socket must 
also be designed to relieve certain parts of the leg below 
the knee of pressure. The most common prosthetic 
socket design is the patellar tendon bearing, or PTB, 
socket (fig. 13-13). Variants of this design may assist 
slightly with weight bearing, but generally are 
variations in methods of suspension. The socket types, 
by name, include PTB with suprapetellar strap 
suspension, PTS (supracondylar-suprapatellar 
suspension), and PTB with thigh lacer and knee joints. 
Total contact, either soft, moderately firm, or hard, is 
used in most, but not all, below-knee sockets. An air 
cushion at the end of a socket is a variant of total 
contact. 

In some B/K sockets, soft padding material is 
required. This may be cotton or wool prosthetic socks, 
a soft insert liner of sponge rubber and leather 
(Kemblo liner), or a sponge or foam plastic material. 
An insert liner also helps the prosthetist adjust the 
socket for stump changes. Its use to compensate for 
errors in fabrication is very undesirable. 

A SACH foot is the ankle-foot mechanism of choice. 
A single-axis ankle is sometimes used for a short B/K 
or when a thigh lacer is used. 

Syme amputation. Suspension of the prosthesis for 
Syme amputation is achieved by shaping the socket to 
the lower leg over the bulbous end of the stump (fig. 
13-14). No straps are necessary. 

Generally, the equivalent of a B/K prosthesis with a 
trap door or expandable walls to accommodate the 
bulbous distal end of the stump at the ankle is 



184 Friedmann 




FIGURE 1 3- 1 4. Syme amputation stump alongside the Syme 
prosthesis. Trap door allows the bulbous end of the stump to be 
inserted into the socket. The trap door is secured by Velcro 
straps after the stump is inserted. 

required. The Syme socket widens the ankle and has a 
poor appearance. Body weight transmits to the 
prosthesis directly from the end of the stump, with 
some weight bearing at the patellar tendon. The 
SACH foot is used. 



Prosthesis Checkout and Training 

Prosthetic checkout is usually done by the physical 
therapist and reviewed by the physician, often with the 
prosthetist and the other treatment team members. 
Determining whether the prosthesis was made as 
prescribed is followed by review of socket fit, presence 
of total contact, adequacy of suspension, proper 
distribution of weight bearing to the prosthesis, and 
careful attention to the alignment. The prosthesis is 
best delivered to the patient at the center, so that initial 
wear proceeds under supervision of the physician 
and/or physical therapist. Often it is delivered 
unfinished to allow for changes in alignment based on 
what is seen during walking (dynamic alignment). 

Ambulation training begins with simple standing 
balance training in the parallel bars. Training then 
progresses to walking, first in the bars with hand 
support, then out of the bars with cane support, and 
then without support. Training proceeds under 
supervision until good habits, stability, safety, and 
independence develop. Gradual increase in wear time 
helps in stump conditioning. Daily training for 3 to 6 
weeks or longer may be required, particularly for A/K 
and higher amputations, before skills are sufficient for 
independent walking. 

Techniques for climbing stairs and ramps are taught 



next. A/K and higher level amputees usually climb 
stairs one at a time, going up with the normal leg first 
and going down with the prosthetic leg first. 
Generalization of skills to outdoors, public 
transportation, automobiles, and work-related 
activities may then follow. The training period for 
bilateral amputees is longer than that required for 
unilateral amputees. 

Throughout the training period, the stump is 
carefully examined for unusual pressure points 
requiring socket correction. Care of the prosthesis and 
the stump is taught simultaneously. The need to wash 
the stump daily, change and wash stump socks daily, 
and wash the inside of the socket every night is 
emphasized. The components of the prosthesis are 
explained to the patient, and danger signs that might 
appear on the stump or with the prosthesis are also 
explained. 

Followup Management 

Monthly followup visits are required intitially, and 
less often later, to monitor the patient's general 
condition, and for stump inspection, component 
checkout, and shoe wear. The physician will also 
examine the cancer patient for evidence of metastases 
and recommend any necessary additional treatment. 
For patients with peripheral arterial disease, followup 
must include inspection of the other limb. Further, 
underlying diabetes, if present, must be managed. 

Stump shrinkage will require modifying the size of 
the socket. This can be accomplished initially by filling 
the socket in or by adding additional stump socks if 
they are worn. Eventually, a new socket will be 
required, usually after 6 months in the first year and 
every 1 to 2 years thereafter. A whole new prosthesis 
may be required in 3 to 5 years due to component 
breakdown. 



Vocational Implications 

Long-term planning can and should be considered 
for the amputee. However, clients whose amputations 
were associated with cancer and peripheral vascular 
disease may require short-term vocational training. 
Also, the eventual vocational evaluation and training 
of child amputees must not be neglected. For children 
over the age of 12, evaluation of interests and 
vocational potential by a vocational counselor, if one is 
available, is recommended. Such evaluation can help 
direct the child's education toward areas consistent 
with the amputation or limb deficiency. The earlier 
such planning begins, the less difficulty is encountered 
in the adult years. 

The vocational plan for the amputee must take into 
consideration the environment in which the client will 
work. Unusually warm environments cause sweating 
of the stump and may cause skin problems. Stump 



Amputation 185 



volume may also change. Cold environments can be 
harmful, and excessively dusty or sandy areas may 
injure the prosthetic components. 

The vocational implications of lower extremity 
amputation are primarily concerned with the physical 
demands of a job, particularly walking, climbing, 
standing, pushing, pulling, and balancing. These 
functions will be impaired to varying degrees, 
depending on the level of the amputation and whether 
the amputation is unilateral or bilateral. 

Walking with a prosthesis is more difficult on 
uneven ground and on sand or mud. Climbing stairs, 
walking up and down ramps, and walking up hills 
usually present difficulties, and therefore jobs 
involving these activities to a considerable degree are 
best avoided. 

Carrying objects is impossible if the client uses two 
canes, crutches, or a walker because his hands are 
occupied. Even with no cane, carrying capacity is 
limited because the weight of the carried object throws 
off the person's alignment. 

Clients with unilateral amputations or bilateral 
below-knee amputations can drive a vehicle with 
automatic transmission with no difficulty. Clients with 
bilateral above-knee amputations will require a special 
vehicle with hand controls. Some jobs requiring 
driving may therefore be considered. 

The following discussion deals with the more specific 
vocational implications of lower extremity amputations 
at the various levels. 

Hemipelvectomy and hip disarticulation. With a 
prosthesis, clients with amputations at these high levels 
will expend 75 percent more energy than nonamputees 
to walk the same distance on level ground. Jobs in 
which the client will mainly be seated, with very little 
walking required, are appropriate. Such jobs will 
generally be indoors. Clients with bilateral ampu- 
tations at these levels will require a wheelchair for work. 

Above-knee. A client with bilateral above-knee 
amputations would expend so much energy to walk 
that he would frequently have no energy left for a job. 
A wheelchair is therefore required for employment, 
especially for clients middle aged or older. The 
unilateral above -knee amputee expends about 60 
percent more energy walking with a prosthesis than a 
nonamputee walking the same distance. The higher 
the level of the unilateral amputation, the greater the 
need for a cane or a crutch, and consequently the 
greater the likelihood of carrying limitations. More 
sedentary occupations with slight walking or carrying 
requirements are therefore best. 

Below-knee . With a prosthesis, the unilateral below- 
knee amputee will expend only 10 percent more energy 
than a nonamputee to walk on level ground. The only 
function definitely impaired is running, although 
walking on rough ground, especially if it is hilly, may 
also present some difficulties. Walking in sand may 
also cause some problems, but special prostheses can be 



made for this activity. Stairs do not present a 
significant problem, and all-day standing and heavy 
work are possible. 

Syme and partial foot . The vocational implications of 
these amputations are essentially the same as for the 
below-knee amputation. 

COMPLICATIONS AND THEIR TREATMENT 

The complications of amputation are the same for 
upper and lower extremity amputations. They include 
edema, skin ulceration, contractures, infections, pain, 
bone overgrowth, and scoliosis. 

Edema 

Stump edema in the immediate period after 
amputation needs to be controlled and resolved before 
a permanent prosthetic socket is fitted. Of greater 
concern is stump edema that develops in the course of 
prosthesis wear, which usually means that there is 
some constriction of the stump or greater pressures 
higher up on the stump than at the more terminal end. 
The higher pressures block the return of venous blood 
to the heart, causing a backup of blood in the veins of 
the stump and extrusion of fluid through the capillary 
walls into the tissues. Assuming the prosthesis socket 
was originally fitted correctly, the usual cause is 
shrinkage of the stump with time and wear. 
Constriction can occur when the shrinkage is uneven 
and the distal end of the stump has more room than the 
proximal end. In particular, proximal constriction can 
develop when the amputee increases the number of 
stump socks he wears to make up for shrinkage at the 
end. 

Edema can occur with stump shrinkage as a direct 
result of trauma at the end of the stump, particularly in 
the lower extremity if the stump bears weight 
improperly. 

Edema must be recognized early before it proceeds 
to ulceration. Chronic edema can be recognized by 
discoloration of the stump caused by rupture of the 
capillaries. Treatment requires re-establishment of a 
proper socket-stump fit and usually means a new 
socket, although sometimes modification of the old 
socket can suffice. Before the patient again wears the 
prosthesis with the new or modified socket, the edema 
must be resolved. 

Ulceration 

Ulceration in the skin of the stump occurring during 
prosthesis wear may be secondary either to excess 
pressure or to chronic edema. In the upper extremity, 
excess pressure may occur with chronic lifting or heavv 
use if stump shrinkage has destroyed total contact. 
Ulceration due to pressure also occurs in the lower 
extremity if socket fit is lost and parts of the stump take 



Fried i 



more pressure on weight bearing than they can 
withstand. 

In both instances, the treatment required is usually a 
new socket. Some control of the ulceration may be 
necessary before the new socket can be fabricated, 
although in some cases the new socket can be 
fabricated and the prosthesis again worn even though 
the ulceration is still present. With a good fit, the 
ulceration can usually heal during prosthesis wear. 

Contractures 

Joint contractures are not a complication of 
prosthesis use, but rather a consequence of events prior 
to the amputation. In particular, contractures may 
occur as a result of efforts to save the limb over an 
extended perod of time during which the extremity is 
inactive and treatment needs prevent full joint range of 
motion. Failure to recognize that the uninvolved joints 
must have range-of-motion exercises to preserve their 
mobility can also result in contractures. In the upper 
extremity, contractures at the shoulder or elbow will 
interfere with operation of the prosthesis, and in the 
lower extremity, contractures at the hip or knee will 
reduce stability in standing and limit the patient's 
walking capability. 

Physical therapeutic exercises can reverse 
contractures in most cases, and surgery to manipulate 
the joint or actually cut the tight structures may help. 
Contractures are always easier to prevent than to 
correct. 

Limitations of joint motion may also occur 
secondary to scarring of the skin across the joint from 
healed burns or from major injury. Correction by 
plastic surgery (see chap. 32) may help improve the 
skin problem and subsequently the joint range of 
motion. 



Infection 

Stump infection may occur if an ulceration becomes 
contaminated, or simply through neglect of stump 
cleanliness leading to an infected hair follicle or sweat 
gland. Careful daily cleansing of the stump, stump 
socks, and the socket prevents such infections from 
developing. Treatment of stump infections may 
require surgical incision to drain the pus, and 
associated antibiotic treatment may be indicated. 



Pain 

The pain problems associated with amputation and 
prosthesis wear are many and varied. Relatively 
circumscribed local pain may be due to a neuroma. 
When an amputation is performed, the nerves, of 
course, are also cut. The ends of the cut nerves heal 
with the formation of a scar containing a bundle of 
nerve fibers called a neuroma. Under excess pressure, 



these neuromas can produce pain, which the patient 
perceives as existing not only in the stump but also in 
the amputated limb (phantom limb) from which the 
cut nerve formerly carried impulses. 

The first treatment approach to a painful neuroma is 
a correct socket fit. If this is not sufficient, efforts to 
desensitize the neuroma through injections and 
ultrasound therapy may help. Sometimes increasing 
the sensory stimulation coming from the limb by 
periodically rubbing, tapping, and massaging it can 
reduce the sensitivity of a neuroma. If these 
approaches are not successful, surgery may be 
necessary to excise the neuroma and place the residual 
nerve in a more protected location in the stump. Such 
a procedure may change the shape of the stump and 
therefore necessitate a new socket. 

Pain localized in the stump but more diffuse than 
pain from a neuroma may also occur. In the absence of 
any underlying bone spur (see below), this type of pain 
is caused by improper socket fit. 

Another type of pain associated with amputation is 
phantom pain. This must be distinguised from phantom 
sensation, which is the sensation that the amputated 
bodv part is still present and which is experienced by 
all adult amputees. Phantom sensation diminishes with 
time, but it may last a lifetime. Patients with congenital 
limb deficiency or who have had surgical or traumatic 
amputations before the age of 4 do not usually experi- 
ence phantom sensation or phantom pain. 

Unlike phantom sensation, phantom pain can be 
disabling. There are three types of phantom pain. The 
least serious type is a vaguelv distributed discomfort in 
the phantom limb associated with muscle tenderness or 
spasm in the stump. This is commonly caused by 
improper socket fit, and correction of this problem 
usually eliminates the phantom pain. Exercise of the 
phantom is helpful. 

The second type of phantom pain is an electric- 
shock-like discomfort in the phantom limb which lasts 
for a few moments and then disappears. It is generally 
associated with a neuroma under excess pressure and 
resolves with treatment of the neuroma itself. It may 
also be due to pressure on the nerve caused by an ill- 
fitting prosthesis. 

The third and most severe type of phantom pain is a 
burning, agonizing discomfort throughout the stump 
and in the phantom limb. The stump may feel warm or 
cold or appear mottled, but it may appear quite 
normal. Clothing or a breath of air suddenly touching 
the stump can trigger the pain. The patient may find it 
necessary to wrap his stump in protective towels to 
avoid the sudden touch. 

A gradual increase in the amount of sensation from 
the stump by more sustained prosthesis wear, or by 
rubbing, tapping, or heating the stump may block this 
agonizing discomfort. Surgical excision of the 
sympathetic nerves is sometimes helpful. Intractable 
phantom pain may require neurosurgical procedures 



Amputation 187 



on the spinal cord. 

Back pain, particularly low back pain, can develop 
secondary to walking on a prosthesis for A/K amputa- 
tion. This type of pain is caused by excessive motion of 
the pelvis and lower spine produced by hip flexion 
contractures or simply by improper prosthetic 
alignment. Careful examination by physicians skilled 
in both prosthesis use and back pain problems may be 
necessary to identify and correct the problem. 

Finally, pain due to factors totally unrelated to the 
stump and the prosthesis may be perceived by the 
patient as coming from the stump or the phantom 
limb. When, for example, a herniated intervertebral 
disc (see chap. 15) impinges on a spinal nerve, referred 
pain is perceived in those areas where nerve fibers 
ordinarily contained in the spinal nerve originated. 
Again, a careful evaluation by a physician 
knowledgeable in prosthetics and vertebral column 
problems can help resolve and correct referred pain of 
this type. 

Bone Overgrowth 

There are two types of bone overgrowth, one 
associated with surgical amputation and one associated 
with congenital limb deficiency or amputation in the 
growing child. Surgical amputations require cutting of 
the bone and therefore cutting the periosteum, the 
covering of the bone which has the capacity of 
producing new bone elements. If shreds of periosteum 
are left in the stump at the time of surgery, they 
develop into bone spurs which change the effective 
shape of the stump and produce local pain. Correction 
requires surgery to remove the spur and the shreds of 
periosteum. 

In the young amputee or the patient with congenital 
limb deficiency, bone overgrowth means that the 
stump bone increases in length faster than the skin of 
the stump grows. The bone end begins to push through 
the skin. When feasible, skin traction is the preferred 
treatment because, if successful, no stump shortening 
results. If stump shortening is necessary, surgery can 
be performed to put a bend in the bone and effectively 
reduce its length, or the end of the bone is cut off. 



surgical spinal fusion to prevent progression may be 
required if special bracing does not arrest or retard 
progression. 

Other Complications 

Some patients may have significant complications 
associated with their underlying diseases, which may in 
part have contributed to the amputation, rather than 
with the amputation or prosthesis wear itself. Patients 
with peripheral vascular disease, such as that 
associated with diabetes, may have visual problems, 
cardiac problems, or cerebral problems due to arterial 
disease elsewhere (see chaps. 22 and 24). Patients with 
heart and pulmonary disease in association with, or 
incidental to, amputation may become limited be- 
cause of the extra energy requirements of walking with 
an artificial limb, particularly if the amputation is 
above the knee. The heart and lungs may not have suf- 
ficient reserve to handle the extra energy require- 
ments and walking may therefore be more limited. 
Patients whose amputations are associated with arthritis 
(see chap. 14) may find themselves limited because the 
residual joints are not sufficiendy normal to handle 
the extra demands placed on them. Patients with cancer 
(see chap. 28) may have additional difficulties. 

CONCLUSION 

The above review of amputations and prosthetics is 
simply the current picture. Perhaps more than with 
other disabilities, this picture is ever changing. Research 
by the Veterans Administration and in university and 
industrial laboratories has been productive in the past 
and promises to continue to be so in the future. 
Advances in the fields of prosthetics and in 
reconstructive surgical techniques is to be expected. 

The counselor should be alert to new developments 
to ensure that his client receives those services likely to 
be of added benefit. The Bulletin of Prosthetics Research, a 
semiannual publication of the Veterans Administration 
which is available through the U.S. Government 
Printing Office, is currendy the best source for keeping 
abreast of new developments. 



Scoliosis 

In patients with high upper extremity amputations, 
spinal curvature (scoliosis) may occur secondary to 
imbalance of the trunk. In lower extremity amputees, 
scoliosis may occur secondary to unequal leg lengths or 
insufficient use and wear of the prosthesis. Trunk 
exercises as a regular routine for high upper extremity 
amputees and careful attention to length at the time of 
prosthesis prescription for lower extremity amputees 
helps prevent the development of scoliosis. Once 
scoliosis develops and becomes fixed, it is hard to 
reverse. If severe, particularly in the growing child, 



BIBLIOGRAPHY 

Barnett AJ, Twist E, Balfe A: Lower limb amputation in a 
general hospital: A comparative review. Med J Aust 
2:14-18, 1976. 

Report of a study of lower limb amputees from 1970-1973 
compared with a similar 1964 study. Mobility, 
independence, use of prostheses, medical condition, and 
mortality are discussed. 

Caine D: Psychological considerations affecting rehabili- 
tation after amputation. Med J Aust 2:818-821, 1973. 
Short article describing reactions to amputations in 
children, adolescents, adults, and the aged. Useful because 
it highlights the attitude and techniques most likely to aid 



188 Friedmann 



in the adjustment of amputee clients to their disability. 

Friedmann LW: Rehabilitation of amputees. In Licht HM: 
Rehabilitation and Medicine, Licht, New Haven, 1968. 
An extensive review of upper and lower extremity 
amputation and prosthetics. Useful for those needing 
greater elaboration of the material covered in this 
presentation. In particular, more training detail is given 
and a section on the juvenile amputee is included. 

Friedmann LW: The Psychological Rehabilitation of the Amputee. 
Springfield, IL, Thomas, 1978. 

Covers phantom limb sensation, phantom pain, and 
common emotional reactions to amputation and prosthesis 
wear. 

Klopsteg PE, Wilson PD (eds): Human Limbs and Their 
Substitutes. New York, McGraw-Hill, 1954. 
Presents results of engineering and medical studies of the 
human extremities and application of the data to the design 
and fitting of artificial limbs and to the care and training of 
amputees. 

Miller LS, Naso F: Conditioning program for amputees with 
significant heart disease. Arch Phys Med Rehabil 
57:238-240, 1976. 

Lower extremity amputees with heart disease were first 
stress tested and were then placed in a conditioning 
program. Reports the effect of the program on ambulation 
ability. 

Murdoch G (ed): Prosthetic and Orthotic Practice. London, 
Arnold, 1970. 

Detailed discussion of prosthetics and orthotics. 
Helpful for the counselor who requires additional 
information. 

Parks CM: Psychosocial transitions: Comparison between 
reactions to loss of a limb and loss of a spouse. Brit J 
Psychiatry 127:204-210, 1975. 

A study of the similarities and differences between the 
experience of losing a limb and losing a spouse. Discusses 
the process of "realizing the loss," which includes phases 
of numbness, pining, disorganization, and reorganization. 

Parkes MC, Napier MM: Psychiatric sequelae of 
amputation. Br J Psychiatry Spec No 9:440-446, 1975. 
An excellent review of the psychological responses to 
amputations, psychosocial factors affecting employment, 
psychological factors associated with phantom limb pain, 
and methods of prevention and alleviation of these 
problems. 



1 4 RHEUMATIC DISEASES 



John J. Nicholas, M.D. 



INTRODUCTION 

The rheumatic diseases include all those diseases 
and syndromes that involve joints, chiefly the synovial 
joints, and/or the soft tissue structures around them 
(para-articular structures). In some of these 
conditions, the joint (rheumatic) complaints occur 
irregularly or constitute only a minor problem. In 
others, the joint disease may play an important or a 
dominant role in the patient's illness. The classification 
of the rheumatic diseases established by the American 
Rheumatism Association, the professional medical 
organization devoted to arthritis and rheumatism, 
actually lists 113 diseases and syndromes distributed 
over 13 general classes of disorders. With new 
knowledge, this classification undergoes periodic 
review. 

The prevalence of the rheumatic diseases in the 
United States is quite large. A recent Arthritis 
Foundation survey indicated that 31.6 million persons 
in the United States suffer from rheumatic disease. 
The prevalence in persons under 45 years old was 48 
per thousand, while in individuals over 45 and under 
65 it was 333 per thousand. The prevalence rates for 
women were higher than those for men at every level, 
and two -thirds of those afflicted were women. The 
incidence of all forms of arthritis is now estimated at 
900,000 new cases per year. 

The three diseases discussed in this chapter, 
rheumatoid arthritis, ankylosing spondylitis, and 
degenerative joint disease (DJD), are particularly 
important because of their frequency, their disabling 
character, and their occurrence in the working age 
group. Rheumatoid arthritis and ankylosing spondylitis 
are both in the category "polyarthritis of unknown 
etiology," and degenerative joint disease occupies a 
category all its own. The latter appears both in primary 
and secondary forms (secondary meaning an 
occurrence as a result of some other problems). 

The incidence of rheumatoid arthritis is estimated at 
500 per 1 million population per year. It may be found 
in about 3.2 percent of the general population, with 
women having the disease three times as often as men. 
DJD is a ubiquitous condition which steadily increases 



in frequency with age and affects men and women 
equally. 

The section on the musculoskeletal system in chapter 
3 should be studied by the reader. An appreciation of 
the normal character of this system allows for a better 
understanding of the disturbances that occur in 
rheumatoid arthritis, ankylosing spondylitis, and 
degenerative joint disease. 

RHEUMATOID ARTHRITIS 

Disease Description 

Rheumatoid arthritis is a total body (systemic) 
disease characterized chiefly by inflammation of the 
synovial joints. Since these joints are designed both to 
allow motion and to bear weight, motion restrictions 
and weight -bearing problems can be an early 
consequence of the disease. In particular, the shoulder, 
elbow, wrist, hip, knee, ankle, and the small joints of 
the hands and feet are affected by rheumatoid arthritis. 
In addition, many tendons and ligaments that are lined 
with synovial tissue are also inflamed in rheumatoid 
arthritis. 

Rheumatoid arthritis may occur in persons of any 
age, but children aged 2 to 4, women in the fourth and 
fifth decade, and men and women over the age of 50 
are the most susceptible. 

Figure 3- 1 7 and page 32 describe the anatomy of a 
synovial joint and the location of the thin synovial 
tissue layer. This tissue produces the fluid (synovial 
fluid) which lubricates the joints and provides cells that 
"eat up" the debris from normal wear. When a 
synovial joint develops rheumatoid arthritis, huge 
numbers of small round cells (lymphocytes) which 
manufacture antibodies invade the synovial tissue, 
accompanied by new blood vessels and often a 
collection of fibrin (a protein essential for blood 
clotting). As a result, the synovial tissue increases in 
size (hypertrophies) and bulges out under the skin 
covering the joint. Substances elaborated from the cells 
cause the joint cartilage to wear away and the joint 
capsule to stiffen and become thin. The cellular activity 
also causes the adjacent bone to thin and lose strength. 



189 



190 Nicholas 



Further, the hypertrophied synovial tissue invades the 
bone and "eats it away," forming defects in the bone 
called erosions, which are noted on X-rays and are 
characteristic of rheumatoid arthritis. The synovial 
tissue around tendons and ligaments similarly thins 
and weakens these structures. 

The disease may start suddenly with rapid onset of 
heat, swelling, pain, and tenderness in multiple joints. 
In other patients, a decrease in range of joint motion 
may be noted for many months or years before the 
heat, redness, tenderness, and swelling appear. 
Corresponding joints on both sides of the body are 
usually involved, and the inflammation is persistent. 

The amount of synovial fluid increases in many of 
the involved joints and it is filled with white cells. 
Subcutaneous nodules characteristically develop at the 
elbow, but may also occur at other points where 
pressure is applied to the skin, e.g., over the bridge of 
the nose, on the sacrum or ischial tuberosities, or over 
the knuckles. The patient will usually report that use of 
the affected joint and weight bearing cause an increase 
in the inflammation and that he can predict a change in 
the weather by noting increased stiffness and pain. 

The cause of rheumatoid arthritis is not known, but 
the bulk of the current evidence points to an immune 
or allergic reaction by the body to an unknown invader 
(antigen). Currently, it is thought that this antigen is 
probably a virus that persists in the body, causing an 
immunity to develop which is transferred to some of 
the body's own structures. In other words, the body's 
usual defenders against allergy attack the joints, 
tendons, and bones as if they were the invaders. This 
theory is supported by the existence of an antibody 
called "the rheumatoid factor," which is found in the 
blood of about 80 percent of patients who have had 
arthritis for several months. This antibody is usually a 
large protein molecule that interacts with the patient's 
normal gamma globulins (antibodies). The rheu- 
matoid factor is manufactured by inflammatory 
cells in the synovial tissue. Experimental animals 
develop a persistent arthritis similar to rheumatoid 
arthritis after being inoculated with various protein 
substances similar to these antibodies, verifying this 
hypothesis in part. 

Muscle weakness and reduction in muscle size 
(atrophy) appears in many patients soon after the onset 
of arthritis. The muscles within the hand, for instance, 
weaken early and can rarely be strengthened. When 
the shoulder joint becomes inflamed, the shoulder 
muscles weaken and also remain weak. The exact 
cause of the muscle wasting and weakness in patients 
with rheumatoid arthritis is not clear. The avoidance 
of motion to prevent pain, the invasion of lymphocytes 
into the muscle, and perhaps a lack of nerve impulses 
all contribute to muscle weakness. 

Loss of joint movement is the impairment in 
rheumatoid arthritis. Some patients report they are 
"paralyzed," but they are not. There is simply so 



much pain on motion that they cannot make 
themselves move and the lack of movement allows their 
muscles to become weak and atrophic. The 
combination of unwillingness to move and muscle 
weakness diminishes the patient's ability to perform 
activities requiring both strength and dexterity. The 
functional and vocational skills of the patient may be 
impaired early by the inflammation, pain, lack of 
movement, and weakness. 

If the inflammation clears after several weeks, there 
may be no residual impairment. If, however, the 
inflammation persists, the patient will find he has 
developed a permanent loss of joint motion. If the 
inflammation in a particular joint cannot be 
satisfactorily treated, the early loss of joint motion will 
persist and lead to the gross and readily observable 
deformities which are characteristic of patients with 
rheumatoid arthritis. These permanent deformities 
change the mechanics of the joint so that it cannot 
function well, even though pain and inflammation may 
have subsided. 

The early and late deformities have been well 
described for many years and should be recognized by 
those involved in the care of patients with rheumatoid 
arthritis. In the hand, a common initial deformity is 
the inability to bend (flex) the knuckles fully. 
Subsequently, an exaggerated flexion of the middle 
knuckle (the boutonniere deformity) may appear (fig. 
14-1). In other patients, the "swan's neck" deformity, 
with exaggerated straightening of the middle knuckle, 
develops (fig. 14-2). The knuckle (metacarpo- 
phalangeal) joint of the hand may fail and the finger 
bones slip underneath it (metacarpophalangeal sub- 
luxation, fig. 14-3). Also at this same joint, the fingers 
may bend over toward the little finger side of the hand 
(ulnar deviation, fig. 14-4). Surprisingly, many 
patients with these deformities are able to continue 
performing their various activities of daily living as 
well as nondexterous vocational tasks. 

At the wrist, the deformity is an inability to bend 
(flex) or straighten (extend). With severe disease, an 
exaggerated flexion occurs and the joint may be nearly 
dislocated (subluxed). 

Deformities also occur at other joints. At the neck, 
they may limit rotation, and at the hip, they may limit 
rotation or extension. Other deformities cause the knee 
to become permanently flexed, the ankle to bend 
outward, and the foot to become flat. 

These deformities occur singly or at many joints. 
While it is impossible to predict which patient will get 
which deformity, it is possible to predict which 
deformities will occur at which joint. The efforts of 
physicians and allied health professionals are required 
to perhaps prevent, but more likely to delay and 
possibly correct, these deformities. Their efforts should 
be supported throughout by the counselor. 

Patients frequently act on two misconceptions with 
which the counselor should be thoroughly familiar. 



Rheumatic Diseases 191 




FIGURE 14-1. Ring and little fingers ofleft hand ofa patient with 
rheumatoid arthritis. So-called "boutonniere" deformity is 
present in the ring finger (top). Proximal interphalangeal joint 
I see p. 35 1 is fixed in flexion, and distal interphalangeal joint is 
fixed in extension. This hand, therefore, does not fullv open. 




FIGURE 14-3. Left hand ofa patient with rheumatoid arthrius 
showing near dislocation (subluxation) of knuckle (metacarpo- 
phalangeal) joints and distal joint of the thumb. This hand will 
not fully open and will have weak pinch force. 

The first is that it is mandatory for them to "keep 
going" or they will stiffen up and become deformed. 
At the heart of this misconception is the subjective 
feeling of stiffness that rheumatoid patients notice on 
arising in the morning. However, there is no evidence 
that this morning stiffness leads to permanent 
deformity, especially if the patient performs his 
prescribed exercises conscientiously. The patient may 
be motivated by morning stiffness to pace up and down 
or perform activities that may lessen his feeling of 
stiffness, but may actually increase the inflammation 
and joint destruction. 

The second misconception patients frequently have 
is that their ordinary work and play activities are as 
beneficial as any special therapeutic exercise. This is 
not true. Therapeutic exercise allows the patient to 
move a joint in a direction that he has neglected 
because of pain. During work or play, this motion is 
usually ignored or avoided because of pain. A 




FIGURE 14-2. Left hand ofa patient with rheumatoid arthritis. 
So-called "swan's neck" deformity is present in all four fingers. 
The proximal interphalangeal joint is fixed in extension. The 
distal interphalangeal joint is fixed in flexion. This hand, 
therefore, does not fullv close. 




FIGURE 14-4. Hands of a patient with rheumatoid arthritis. Left 
hand demonstrates subluxation of knuckle (metacarpopha- 
langeal) joints with deviation of fingers awav from the thumb 
(ulnar deviation). This hand will have weak pinch and grasp and 
will open incompletely. 

therapeutic exercise may be directed at strengthening a 
muscle by graded maximum contractions that fall 
short of exhaustion and are designed to cause 
minimum joint motion. During work or play, the 
patient must follow whatever motions are required by 
the activity and may contract his muscles to the point 
of fatigue. A therapeutic exercise is not designed to 
force the patient to move his joint so frequently that the 
inflammation is increased, whereas a particular 
activity may require that the patient repeat the same 
maneuver over and over until his joint "cries out" in 
pain. This "work equals exercise" misconception is as 
common among housewives as among laborers. The 
counselor may reinforce the suggestions of the 
therapist by recommending that his client avoid work 
which leaves him inflamed and fatigued and perhaps 
unable to perform the exercises that could strengthen 
and correct deformities and weakness of his joints. 
Complications. There are a number of complications 



192 Nicholas 



of rheumatoid arthritis. These include the carpal 
tunnel syndrome, Sjogren's syndrome, peripheral 
neuropathy, pleural effusion, Baker's cyst, and 
anemia. 

The carpal tunnel syndrome is characterized by 
numbness and tingling and eventual loss of feeling in 
the thumb, index finger, and middle finger. The small 
muscles of the thumb may fail to function. This 
svndrome occurs when the hypertrophied synovial 
tissue fills the narrow tunnel at the wrist through 
which the median nerve (see p. 104) travels from the 
forearm to the hand. Failure of the function of the 
compressed median nerve causes the tingling and 
loss of strength. 

In Sjogren's (show-grens) syndrome, multitudes of 
lymphocytes invade the glands of the mouth, nose, 
eyes, throat, and lungs and cause the patient to suffer 
dry eyes (keratoconjuntivitis sicca) and dry mouth 
(xerostomia). The loss of the glandular function may 
cause ulcers of the eye tissue, serious dental caries, and 
an inability to chew food normally. When dry eyes and 
dry mouth occur alone, the "sicca syndrome" is said 
to be present. When the sicca syndrome is 
accompanied by rheumatoid arthritis, the condition is 
termed Sjogrens syndrome. The lymphocytes also 
invade the kidney, liver, and lungs, causing these 
organs to function poorly. 

Many rheumatoid arthritis patients with peripheral 
neuropathy complain of mild numbness and tingling in 
their fingers and toes (see chap. 7). Rarely, however, 
do they lose complete function of a major nerve. This 
loss of nerve function is due to inflammation of the 
blood vessels that supply these nerves. When one nerve 
is involved, it is termed a mononeuritis, and when more 
than one are involved, it is termed a mononeuritis 
multiplex. This condition may remit spontaneously or 
require drastic medical treatment. Vasculitis, the blood 
vessel inflammation, it also thought to be the cause of 
both the subcutaneous nodules and the shallow, 
painful ulcers which may occur on the patient's ankles. 
These ulcers probably occur following minor trauma. 
They then may become infected and fail to heal. Many 
days of disability may result, and prolonged 
hospitalization may be required. 

Pleural effusion may occur in rheumatoid arthritis 
patients. Commonly, patients will have X-rays 
showing some mild accumulations of scars in the lungs, 
but these findings are rarely of significance. However, 
on occasion the patient may develop a collection of 
fluid (pleural effusion) in the pleural space (see p. 44). 
This accumulation causes little harm but must be 
carefully distinguished from cancer, tuberculosis, 
bacterial infection, and heart failure. In addition, 
nodules may also develop in the lungs and these must 
be distinguished from the more serious but similar- 
looking nodules of cancer, tuberculosis, fungus 
infection, and pneumonia. 



Baker's cyst occurs when inflamed synovial fluid 
escapes from the knee and collects in the space behind 
the knee, with extension into the calf. Patients who 
have only recently developed rheumatoid arthritis may 
complain of sudden pain and swelling in the calf of the 
leg caused by this escape of fluid. Baker's cyst must be 
distinguished from thrombophlebitis of the calf veins 
(see chap. 22). Patients who have had rheumatoid 
arthritis for a longer period of time may develop a 
chronic escape of fluid in the calf. This may respond to 
treatment of the knee disease. Surgical removal of the 
cyst is occasionally required. 

The anemia of rheumatoid arthritis has two sources. 
Chronic inflammation can affect the production of the 
red cell elements of the bone marrow. Further, the 
various medications used in the treatment of this 
disease may contribute to chronic, very low-grade 
blood leakage from the stomach walls. In some 
patients, actual ulceration of the stomach walls can 
occur. Anemia may contribute to the fatigue 
experienced by the patient with rheumatoid arthritis. 
If anemia is severe, it can weaken the patient 
sufficiently to cause him to become more susceptible to 
other diseases. 

Prognosis. The general course of rheumatoid 
arthritis is characterized by gradual diminution of 
inflammation and progression of deformity and 
crippling. The speed with which the deformities occur 
varies from patient to patient, but their progression 
can be anticipated, observed, and measured. On 
occasion, patients with rheumatoid arthritis appear to 
lose all signs of inflammation and are said to have 
undergone a remission. The usual course, however, is 
for the deformities to progress and persist. 

A physician who has observed a patient with active 
rheumatoid arthritis for several months is in a position 
to evaluate the prognosis and to discuss this cautiously, 
yet realistically, with the patient and the rehabilitation 
counselor. Factors associated with a poor prognosis 
include persistent disease of more than 1 year's 
duration and an age below 30, coupled with the 
presence of subcutaneous nodules and high titers of 
rheumatoid factor. On the whole, however, about 50 
percent of the patients are in the so-called stationary or 
unimproved category after 10 years of the disease. If 
there is no improvement by that time, there is little 
likelihood that it will occur. The counselor can assume 
that the course of the client's disease will be no better 
and that deformities probably will be worse in 
subsequent years. 

Functional Disabilities 

Physical Disabilities. The interference in motion 
induced by pain, the loss of proper mechanical joint 
motion due to deformities, and the loss of strength all 
may markedly limit the patient's activities. The 



Rheumatic Diseases 193 



particular activity impaired depends upon which joints 
are involved. If the shoulders and hands are involved, 
activities such as dressing, combing hair, using 
wrenches, and peeling vegetables may be limited or 
impossible. If the knees, hips, or feet are involved, 
walking may be limited, as will bending, stooping, and 
lifting. While the patient may have no difficulty getting 
around within limited enclosures, for example, 
walking longer distances may be impossible. In severe 
cases, even transferring from bed to chair, chair to 
toilet, and chair to car will become impaired, and daily 
bathing, feeding, and grooming activities will be 
limited. Certainly persistent joint pain or deformity 
of the hips, feet, or knees will limit the patient's 
ability to use public transportation. 

These disabilities are usually caused by inability to 
continue the customary way of doing things. As new 
techniques are learned, many of these disabilities can 
be overcome. 

Psychosocial disabilities. Psychologists frequently 
characterize patients with rheumatoid arthritis as 
hostile, repressed, angry, and frustrated. Generally, it 
is believed that these characteristics are caused by 
constant pain with movement, rather than that a 
particular person with a propensity for hostile or 
repressed feelings gets rheumatoid arthritis. Patients 
who are chronically limited by pain have difficulties 
with their personal feelings and social interactions. 
Marital, parent -child, and other relationships may be 
seriously affected. 

Some patients adapt to their disease and disability 
and function well with limited abilities, while other 
patients seem incapacitated by fairly minimal 
involvement. Adaptation to rheumatoid arthritis 
requires the patient to give up a former sell-image and 
life style, without giving up those efforts required to 
achieve and sustain maximum independence. 
Maintenance of effort requires a tolerance for pain. 
This adaptation is difficult, and may be thwarted by 
denial, depression, or dependence. 

Many patients who are in almost constant pain may 
never entirely give up the hope that one morning the 
pain will all vanish. This type of denial may lead to 
unrealistic expectations and resistance to altering 
behavior patterns. The patients may presume that 
once their "pain goes away" and their strength 
returns, their vocational problems will disappear. 

Another reaction to the limitations imposed on the 
patient by his disease is increased dependency. Faced 
with the prospect of progressive deformities and pain 
on movement, the patient may decide to avoid pain 
and give up trying to remain active by increasingly 
depending on others for care. 

However, for some patients, increased dependency 
may conflict with their previous active, independent 
self-image. When such a patient resists a desire to 
become excessively dependent and is willing to tolerate 
pain in order to continue with activities, he may 



become frustrated and depressed. The frustration 
occurs because, despite the effort, it becomes 
increasingly difficult or impossible to maintain 
previous activities, due to the accumulation of 
deformities. Patients become particularly frustrated 
when they discover that certain physical outlets and 
exercise activities are no longer feasible. The patient's 
inability to accept diminished activity and continued 
pain often leads to depression. 

Rehabilitation potential. Longevity of rheumatoid 
arthritis patients is somewhat less than that of the 
normal population because of an apparent increased 
susceptibility to bacterial infection. Longevity is 
sufficient, however, so that the counselor can plan in 
terms of 2 or 4 years, rather than 6 or fewer months, 
for possible education and/or retraining. 

After the counselor is assured that appropriate 
consultation and evaluation have been obtained from 
physicians and allied health personnel and is satisfied 
that the standards of evaluation have been met, he 
must consider several factors. The problem is not how 
long the patient will live but how he will function. 
Many patients seem to function well with severe 
deformities, while others with lesser deformities are 
more impaired in their functioning. 

The prognosis for work is of course better for 
patients who possess highly trained skills which do not 
depend upon manual dexterity or strength. Those who 
possess more intellectual skills before the onset of their 
disease will likely have more skills afterwards. 

Standards of Evaluation 

A patient with rheumatoid arthritis should have 
certain evaluations performed to aid in diagnosis and 
treatment and to assess the extent of disease. An 
internist specializing in rheumatology (rheumatologist) 
is likely to be best equipped to do this. The patient 
requires a complete medical history and a physical 
examination, and descriptions of inflamed joints, of 
any joints which have less than normal range of 
motion, and of any weakness present. Deformities in 
the most seriously involved joints should be noted so 
that excessive use can be avoided. Complications 
should be sought. 

A history should include all drug treatment, past and 
present. The duration of morning stiffness should be 
recorded. A complete blood count will disclose anemia, 
if present. A hematocrit of less than 30 percent suggests 
that at least part of the anemia is due to blood loss, and 
the source of the loss must therefore be sought. Also, 
this degree of anemia should be further investigated by 
study of the bone marrow and life span of red blood 
cells. 

The urinalysis is usually normal. If blood is present 
in the urine (hematuria), it may mean that the patient 
is having an adverse reaction to a drug or perhaps is 
ingesting too much of a drug. For example, ingestion 



194 Nicholas 



of compounds containing both salicylate (aspirin) and 
acetaminophen (e.g., Tylenol) may cause destruction 
of the renal collecting system (see p. 329). Also, if 
protein is present in the urine, the cause must be 
sought. 

The erythrocyte sedimentation rate (ESR) is a very 
useful measure regularly obtained to help assess the 
degree of inflammation and the effect of any particular 
medication regime. This test is a nonspecific general 
measure of inflammation and is quite simple. A blood 
sample is mixed with an anticoagulant to prevent 
clotting and a column of blood is then set in a rack to 
keep it perfectly vertical. The red blood cells 
(erythrocytes) gradually settle to the bottom of the 
container. The sedimentation rate is expressed in 
millimeters of plasma remaining (i.e., the clear area 
above the settling blood cells) per unit of time, usually 
1 hour. In normal persons, sedimentation occurs 
slowly. The normal sedimentation rate for males is less 
than 10 mm per hour and for females, less than 20 mm 
per hour. The ESR will be elevated proportionately to 
the degree of inflammation in rheumatoid arthritis and 
may decrease with effective treatment. The 
sedimentation rate for rheumatoid arthritis patients 
can be as high as 150 mm per hour. 

Other tests assist in analysis of the extent and 
severity of the disease. The rheumatoid factor test 
(latex fixation) becomes positive during the first year in 
approximately 80 percent of patients with rheumatoid 
arthritis. The sheep cell agglutination titer (SCAT), 
while positive less frequently, is thought to be more 
specific for rheumatoid arthritis. 

The lupus erythematosus preparation (LE prep) and 
antinuclear antibody tests are also performed in all 
patients thought to have rheumatoid arthritis. The 
connective tissue disease, systemic lupus 
erythematosus, frequently begins with an arthritis 
identical to rheumatoid arthritis, but the presence of a 
positive LE prep or antinuclear antibody test and the 
absence of the bom erosions characteristic of 
rheumatoid arthritis serve to separate these two 
diseases. Because the course, treatment, and prognosis 
of systemic lupus erythematosus and rheumatoid 
arthritis are quite different, diagnosis must be 
accurate. 

A uric acid test should be obtained. Results are 
usually normal in patients with rheumatoid arthritis 
(or low in those taking large doses of aspirin). The test 
is elevated in patients with gout, renal disease, and 
other problems that are themselves associated with 
joint disease. 

X-rays should be taken of all involved joints to 
determine if erosions or cartilage destruction are 
present. Subsequent X-rays should be obtained at 
yearly intervals to assess the degree of joint destruction 
and to be used as a guide to treatment. 

The patient should be referred by his physician to a 
physical therapist to assist in evaluation of the joint 



range -of- motion limitations and muscle strength. A 
physical therapist should continue to periodically assess 
the patient's progress or lack of it under prescribed 
treatment for range -of- motion and strength 
limitations. 

Rheumatoid arthritis patients should also be 
referred to an occupational therapist, who can evaluate 
activities of daily living, such as dressing, eating, 
personal hygiene, and housekeeping, and recommend 
training and techniques to achieve improved 
independence and to minimize joint damage. The 
therapist will be able to provide information regarding 
the likelihood that the patient will benefit from assistive 
devices, whether splints can be fitted, and what 
changes in the home or work place may be useful. 

A clinical psychologist can assess the patient's 
adjustment to his disease, and search for the existence 
of the psychosocial disabilities discussed above. In 
most cases, patients and their families will benefit from 
referral to a social worker who can identify problems in 
family relationships and/or financial difficulties due to 
the patient's disabilities. 

When a patient has multiple problems, a thorough 
evaluation may be best carried out in a comprehensive 
rehabilitation setting under the direction of a 
physiatrist. In such a setting, all professionals, 
physicians and the allied health group, simultaneously 
and in concert evolve a comprehensive treatment plan 
that incorporates an attack on all the problems that the 
evaluations uncover. 

Total Treatment 

Drugs. Drugs used in rheumatoid arthritis primarily 
function to control the inflammation. Many drugs are 
available because no single drug is completely effective 
for all patients. The patient very likely will be given 
several drugs simulataneously, as the effect of several 
seems additive. 

Aspirin or another form of salicylate is most 
commonly prescribed first. Many preparations are 
available: plain aspirin, buffered aspirin, liquid 
preparations, and rectally administered aspirin. Each 
relieves pain, lowers the body temperature, and may 
reduce inflammation. All may cause side -effects in 
sufficiently high doses. Buffered, liquid, and rectally 
administered preparations cause less stomach upset 
and gastrointestinal bleeding. Excessive doses which 
may have been taken with suicidal intent cause marked 
derangements of the body's mineral and salt 
metabolism and require hospital treatment. 

The appropriate dose of a salicylate preparation is 
that which almost causes ringing in the ears (tinnitus). 
At the level of tinnitus, the amount of aspirin has 
exceeded the liver's ability to metabolize and eliminate 
it and hence more is available to exert a therapeutic 
affect. Up to 15 or more aspirin tablets a day may be 
prescribed. Concentration levels of aspirin in the blood 



Rheumatic Diseases 195 



are periodically measured to determine whether they 
are high enough for anti- inflammatory effects. 

Recently, several new anti -inflammatory drugs 
(Motrin, Nalfon, Indocin, Clinoril, Tolectin, and 
Naprosyn) have become available. As a group, they 
are called the nonsteroidal anti - inflammatory drugs. They 
relieve pain and decrease inflammation as well as 
salicylates, but no better. Their side effects are similar 
but perphaps less common. They are, however, 
considerably more expensive. Butazolidine, an older 
nonsteroidal anti -inflammatory drug, has been 
available for many years. It is usually used for only a 
few days, as it has caused skin rashes and bone marrow 
depression, a serious complication that can result in 
death. 

Adrenocorticosteroid preparations (cortisone, prednisone, 
methylprednisone, triamcinolone, dexamethasone) are 
the most effective anti- inflammatory drugs available. 
In high doses, they may practically eliminate the 
clinical symptoms of rheumatoid arthritis. They do 
not, unfortunately, stop the formation of erosions, the 
loss of cartilage, or the weakening of ligaments or joint 
capsules. In spite of their good anti -inflammatory 
action, their use is avoided if possible because of their 
major side -effects. These side -effects include cataracts 
(see chap. 29), thinning of the bones (osteoporosis), 
peptic ulcers with bleeding, augmentation or initiation 
of high blood pressure and diabetes, thinning of the 
skin and delayed wound healing, muscle weakness, 
unsightly weight gain of the face and the trunk, and 
poor resistance to bacterial and tuberculous infection. 
Doses higher than that equivalent to roughly 7.5 mg of 
prednisone consistently result in undesirable side- 
effects and require cessation of the drug whenever 
possible. In an attempt to avoid these side-effects, 
steroids may be given in a double dose every second 
day. When inflammation is brought under control, the 
physician usually attempts to taper steroids and 
substitute the nonsteroidal medications for 
maintenance. 

Special steroid preparations may also be injected 
locally into particularly involved joints. The injections 
may be repeated three or four times a year without fear 
of damaging the joint. 

Chloroquine, an antimalarial drug, may also be 
prescribed. Its beneficial effect occurs only after several 
months of use. Because of the frequent occurrence of 
permanent retinal damage when used regularly, 
ophthalmological examination is necessary. 

A more commonly used drug whose effect is noticed 
only after several months is gold. The rwo most 
commonly used compounds are Myochrysine and 
Solganol. They are given intramuscularly, usually 50 
mg weekly for about 20 weeks. If there is no response, 
it is stopped. If the patient has improved, the drug is 
continued, although often in smaller doses and at 
wider intervals. 

Side -effects of gold are serious, and weekly 



examination of the urine for protein, and of the blood 
hematocrit, white blood count, and platelet count are 
required to avoid serious complications. If the urine 
contains abnormal amounts of protein, gold must be 
stopped before severe kidney damage occurs. If the red 
or white blood cell or platelet count falls, the drug must 
be stopped. The most common side -effect is skin rash. 
Since it is difficult to tell whether or not a rash is due to 
gold, the gold is stopped and then readministered. If 
the rash recurs upon the readministration of gold and 
is extensive, gold must no longer be given. 

Penicillamine is a new antirheumatic drug whose 
long-term benefit has not been fully established. 
Anticancer drugs (Cytoxin, Immuran, see chap. 28) are 
also given to patients with rheumatoid arthritis. 
These drugs seem to decrease the signs and symptoms 
of rheumatoid arthritis but have many distressing 
side -effects, which include liver poisoning, hair loss, 
bloody urine, and bone -marrow poisoning. These 
drugs may increase the risk of infection, predispose 
to malignancy, and increase mutations in offspring. 

While aspirin is the mainstay of drug treatment, 
patients during the course of their disease usually are 
exposed to one or more of the others. Frequent 
followup visits for drug management are necessary, 
since the disease has a variable course for each patient 
and drug prescription needs to be revised. At each visit 
(anywhere from weekly to three or four times per 
year), the physician assesses the degree of 
inflammation present by examining the patient's joints 
and obtaining blood samples. The sedimentation rate 
(ESR) is a useful measure to assess degree of 
inflammation and to assist in adjusting medications. 

Surgery. Surgical treatment in rheumatoid arthritis 
is of two types, one to control or remove inflamma- 
tion and the other to correct deformity. 

To control inflammation, removal of the synovial 
tissue to as great a degree as possible (synovectomy) 
prior to the occurrence of joint destruction and the 
formation of major erosions is indicated when 
conservative measures fail. The two most common 
joints for which synovectomies are done by orthopedic 
surgeons are the knee joint and the knuckle 
(metacarpophalangeal) joints of the hand. While 
results are often rewarding, synovial tissue regenerates 
and therefore the procedure is not a curative 

When surgery is performed to correct deformities, 
the goals are decreased pain and greater functional 
use. Some of these surgical procedures include: 

1 . Resection and removal of the distal ends of the 
metatarsal heads ("balls") of the feet to reduce 
foot pain and improve comfort and walking. This 
is a consistently successful procedure. 

2. Fusion of the metacarpophalangeal and/or 
interphalangeal joint of the thumb to convert an 
unstable thumb into one quite able to sustain 
fingertip pressure from the other fingers as 
objects are grasped. 



196 Nicholas 



3. Replacement of the metacarpophalangeal joints 
with plastic "joints" to allow realignment and a 
better ability to grasp larger objects. 

4. Fusion of the proximal interphalangeal joints of 
the fingers to relieve pain and increase the angle 
of flexion so the patient can function securely. 

5. Sophisticated surgery to correct boutonniere and 
swan's neck deformities of the proximal 
interphalangeal joints which, if performed early, 
have given quite good results. 

6. Surgical fusion of the wrist to provide a stable, 
painless wrist improves function in many cases. 

7. Shoulder and elbow surgery are also performed, 
although less frequently. 

Prostheses. The recent development of the total hip 
prosthesis (hip arthroplasty) holds great advantages for 
patients with rheumatoid arthritis. The procedure has 
few postoperative complications and causes 
improvement in motion and diminution in pain. The 
total hip prosthesis has been implanted bilaterally in 
many patients. The long-term durability of this 
prosthesis has not yet been determined, but the results 
are so good to date that the counselor should support 
the patient for hip arthroplasty, as it likely will add 5 to 
15 more years of useful hip function. 

The total knee prosthesis (knee arthroplasty) is 
constantly improving, and several successful models 
have been devised. These knee operations result in 
joints with less than optimum stability in many cases 
and have more complications than the total hip 
operations. Nonetheless, in a patient with a largely 
destroyed knee who cannot ambulate, surgery is 
clearly indicated. A failure will result only in knee 
fusion, which in itself is not a catastrophe and will also 
allow ambulation to a greater extent than is possible 
with a painful, destroyed, inflamed rheumatoid knee. 

Total wrist prostheses have been recently devised but 
are still in an experimental state. In time, other joint 
replacements are likely to become successful, such as 
those for the elbow, shoulder, ankle, and hand. 

Rehabilitation treatment. Rehabilitation medicine 
treatment may be classified into five categories: (a) 
measures to reduce pain and inflammation; (b) 
measures to prevent deformities; (c) measures to 
correct deformities; (d) measures to increase strength; 
and (e) measures to improve functional skills. 

Measures to reduce pain and inflammation, such as moist 
heat, paraffin baths, contrast baths, and cold packs, 
decrease the heat and swelling around a joint and allow 
motion and exercise to be performed more extensively 
with less discomfort. The choice of which treatment to 
use depends upon patient preference, as there is no 
substantial body of data upon which to base the choice. 
The pain relief from these treatments is temporary and 
may last only 2 to 4 hours. The patient should be 
taught to perform these treatments in his home so he 



will not be limited to visiting the office of a physician or 
therapist unless, of course, the inflammation is too 
severe. 

Placing a joint in plastic, fiberglass, or plaster splints 
or casts will also diminish the inflammation. The 
devices may need to be worn for several weeks to 
achieve maximum benefit, however, and therefore are 
cumbersome and unacceptable to many patients. For 
some, night use only may be sufficient. Splints or casts 
are most conveniently placed on the wrists, hands, and 
knees. Such splints also can control the severity of 
deformity progression. Design is critical, as is repeated 
checking of splint fit. If splints interfere with function 
to the degree that greater disability results, the patient 
is likely to discard them. Close followup can allow for a 
balance between function and joint protection. 

Hydrotherapy in Hubbard tanks, walking tanks, 
pools, and spas has given relief to patients for 
centuries. While beneficial effect has been attributed to 
the mineral content or radioactivity of the spring, 
probably it is only the hot water which causes the 
soothing effect. Hydrotherapy can be expensive and 
inconvenient and is usually reserved for hospitalized 
patients. 

Measures to prevent deformities can be taken, since an 
observer can predict which kind of deformities are 
going to occur. The patient who does not balance rest 
and activity well may actually increase inflammation 
and joint destruction. Exercises taught by the physical 
therapist will prevent loss of motion at the joints and 
help maintain strength. Various joint protection 
techniques are also taught. For example, the patient 
will be advised not to sleep with his knees bent or with 
a pillow beneath them, but rather to sleep on his 
abdomen. He will then be less likely to develop hip and 
knee flexion deformities. 

The effect of exercises and splints or casts is 
dependent upon their use and repetition. The 
counselor may therefore wish to review his client's 
treatment program and urge him to comply with it. 

Measures to correct deformities include the use of serial 
casts or splints which place the joints in more and more 
extension to correct flexion deformities at the wrist and 
knee. Casts and splints are awkward at the shoulder, 
and if this joint has lost motion and has become 
deformed, stretching exercises by the physical therapist 
are the only available treatment. The application of 
heat or cold prior to corrective exercises increases the 
motion which may be gained and decreases the pain. 
Shoe inserts may be used to correct flat feet. 

Measures to increase strength in some muscles can be 
performed by the physical therapist. Although 
weakness in the hand muscles and shoulder muscles is 
difficult to improve, exercise can decrease the 
weakness that occurs in the knee muscles and in the 
muscles which extend the hip. Efforts should be 



Rheumatic Diseases 197 



concentrated upon increasing strength in these 
muscles. Exercise while moving the joints as 
little as possible (isometric exercises) will allow for an 
increase in strength without joint inflammation. This 
type of exercise requires diligence on the part of the 
patient and skilled coaching on the part of the 
therapist. The therapist can also advise the patient on 
which sorts of work or other activity increase his 
inflammation and should therefore be avoided or 
minimized, as well as those specific therapeutic 
exercises to strengthen muscles without joint stress. 

The counselor can reinforce the suggestions of the 
therapist by recommending his client avoid work that 
leaves him inflamed and fatigued and perhaps unable 
to perform the exercises that could strengthen his 
joints. Further, if the client reports to the counselor 
that he is pacing or performing other activities in the 
morning to alleviate his feeling of stiffness, the 
counselor should advise the client to consult his 
physician. Such morning activities may be harmful. 

Measures to improve function can be recommended by 
both the physical and occupational therapists, the 
former dealing with ambulation and transfer tech- 
niques and the latter concentrating on activities of 
daily living. When the problems are carefully outlined, 
devices such as reachers, hooks, and built-up utensil 
handles can be provided. 

The occupational therapist may also visit the 
homemaker in the home to determine in what ways 
labor-saving and energy-saving reorganization may be 
accomplished. Recommendations such as the raising 
or lowering of table tops, the lowering of storage 
cabinets, and provisions of more easily activated faucet 
handles may be appropriate. Similarly, a visit by an 
experienced occupational therapist to a client's place of 
employment may yield recommendations for the 
worker's station that will yield increased productivity 
with less joint strain. 

Followup treatment. Good total care of the patient 
with rheumatoid arthritis unfortunately rarely results 
in a complete absence of pain and inflammation. This 
fact, coupled with the unpredictable nature of the 
course of the disease, requires regular followup for 
increases in pain, inflammation, deformity, and 
reduced functional skills. Monthly visits to the 
physician are quite usual for evaluation of results of 
prior treatment and recommendations for additions 
and changes. The sedimentation rate is usually 
repeated frequently to assess degree of inflammation. 
Low values mean good control. The hematocrit or 
hemoglobin test adequately checks for anemia. If there 
is a drop in either of these, bleeding from the 
gastrointestinal tract secondary to medication or bone 
marrow depression should be sought and treated. The 
occurrence of proteins in the urine (proteinuria) 
suggests the complication of gold or penicillamine 



therapy. Review of joint range of motion status, 
strength and functional skills, and of splints the patient 
may be using are also performed at regular followup 
visits. 

Psychosocial treatment. The rheumatoid arthritis 
client needs understanding while he is grieving for his 
previous self-image and life style, and he needs help 
and support during the transition to a more limited 
range of activities. It is not sufficient for the counselor 
simply to tell the client to go out and do things 
differently. The counselor needs to be understanding 
and listen to the exact nature of the particular problems 
the client may have. He must also attempt to help the 
client achieve a resolution of the problems and an 
alteration in the type and degree of physical activity 
that realistically conforms to the client's limitations. 
He should encourage the client to avoid those activities 
believed to increase his deformities and disability and 
help him find activities that allow him to continue as 
active a life as his arthritis will allow. If a client has 
unrealistic expectations due to denial, the counselor may 
need to be forthright in a supportive way in explaining 
to the client that it is unlikely his arthritis will vanish to 
such an extent that he will not have to alter his life 
style. 

Clients who are obsessed with performing their daily 
jobs, tasks, or housework, and by so doing ignore all 
advice about protecting their joints, mav be helped by 
psvchotherapv with a clinical psychologist or 
psychiatrist. Also, psychotherapy may be useful in 
helping clients adjust to their disability. A skilled social 
worker experienced in counseling clients with 
rheumatoid arthritis will let the client ventilate his 
feelings and advise him and his significant "others" of 
ways to attempt to work out the marital, parent-child, 
and/or other family problems associated with the 
client's disability. 

Vocational Implications 

The signs and symptoms of rheumatoid arthritis are 
not static. Unlike the client with an amputation, 
paraplegia, or a stroke, the client with rheumatoid 
arthritis has a condition that varies. The counselor 
must therefore observe and understand his client's 
changing condition and provide vocational goals and 
support appropriate to the more difficult periods so 
that employment can be sustained. 

Not all clients with rheumatoid arthritis progress 
unremittingly to disability. The counselor should be 
alert for the client with only a few involved joints who 
seems to undergo a remission. Such a client is more 
easily provided with vocational goals and supports. 
This discussion of specific vocational implications will 
assume, however, that the client has a persistent, 
generalized involvement with a slow, steady, but 



198 Nicholas 



inexorably progressive course. 

Education. Advanced or additional educational 
goals should be a strong consideration both for clients 
with recent-onset rheumatoid arthritis and those with 
long-standing disease. The longevity of the client with 
rheumatoid arthritis is diminished, but not sufficiently 
to make long-term educational goals unreasonable. 
Vocational training and/or college courses of 2 to 4 
years should clearly be considered. If the client is in the 
third or fourth decade of life, the common age of onset 
of rheumatoid arthritis, educational goals should be 
guided by his past intellectual skills and experience, 
rather than limited by the fear of death or total 
disability. 

A further consideration is that manual labor and 
many vocations requiring chiefly physical skills may 
cause exacerbations of rheumatoid arthritis. A client 
who has this experience would appear to be a likely 
candidate for rehabilitation to a vocation where mental 
rather than manual skills are paramount. 

Aptitudes. There is nothing inherent in rheumatoid 
arthritis that affects the client's intellect, learning 
ability, verbal skills, numerical skills, color 
discrimination, or form and space perception, except 
the constant frustration of pain with nearly every 
movement. 

Motor coordination, finger and hand dexterity, and 
eye-hand-foot coordination are adversely affected by 
rheumatoid arthritis. Loss of motion and pain on 
motion certainly slow the client's movements and 
diminish coordination. 

Later in the course of the disease, ligament, tendon, 
and muscle weakness will affect coordination. 
Deformities subsequent to destruction decrease 
mechanical advantages (e.g., weakening of the grip 
when the wrist is subluxed in a palmar direction and 
when the fingers are distorted or stiff). Clients lose the 
ability to raise the arms and hands above the shoulder 
level due to contractures of the shoulder joint. The loss 
of stability of the thumb decreases the force of pinch. 
Vocational goals dependent upon fine, dexterous, or 
coordinated movement of the hand are therefore not 
good choices for clients with rheumatoid arthritis. The 
operation of machines requiring dexterous, rapid, 
repeated movements probably also is a bad choice. 
Nevertheless, if the force required is quite low, 
dexterous tasks, including the use of an electric 
typewriter, may be accomplished. 

Interests. The counselor must remember that the 
client with rheumatoid arthritis is likely to be in his 
third or fourth decade. By the fifth or sixth decade, the 
client will have progressed from the inflammatory 
phase to the stage of persistent deformity where 
movement will be limited by mechanical failures of the 
joints to support weight and allow motion. The 
counselor thus will have to guide the interests of his 
younger clients into areas where frequent use of joints 
will not aggravate the disease and increase the clients' 



rage and frustration. Some younger clients more 
inclined to anger may succeed best when dealing with 
objects or with scientific or technical activities rather 
than with people. 

It is likely that the client with rheumatoid arthritis 
would fare better with activities of an abstract, creative 
nature, rather than with routine organzied activity, if 
such interests and aptitudes are present. Routine 
activities are frequendy associated with repetitive 
mechanical motions, which may induce overuse of an 
inflamed joint. Certainly, the client with rheumatoid 
arthritis will have difficulty succeeding when 
productivity is the chief criterion for success. In all 
likelihood, he will be frustrated even if his interests and 
personality traits suit him to high performance, piece 
work, or production -oriented tasks. The counselor may 
well try to seek situations where prestige and esteem 
are the rewards. Clients with rheumatoid arthritis may 
have diminished self- images, which would be enhanced 
by rewards of esteem. 

Jobs with continuous stresses, both to the joints and 
to the person, are not recommended. They may cause 
exacerbations of both disease and frustration. The 
management of rheumatoid arthritis usually requires 
rest periods, and a combination of rest and exercise 
often must be available on the job as well as at home. 
Some clients may even require a nap at noon or in the 
afternoon. In addition, they also benefit from frequent 
changes of position. 

The solo or group nature of a job minimally affects 
the client with rheumatoid arthritis. Some clients will 
be buoyed up by companions and others plunged into 
despair, self-pity, and hostile antagonism. If the client 
responds to a fellow sufferer or a well person with 
increased self-pity, he should likely work alone. The 
client who obtains support and inspiration from others 
should be placed with a group. 

Physical demands. Most clients with rheumatoid 
arthritis will be better employed in vocations with 
sedentary or, at most, light work. While most of the 
clients who respond to treatment will be able to 
perform medium or heavy work, the counselor must 
keep in mind that the progressive nature of this disease 
is toward further crippling and disability. Thus a client 
who initially mav do medium work (i.e., lift 25 to 50 lb, 
or 1 1.3 to 22.5 kg) or even heavy work (i.e., lift 100 lb, 
or 45 kg) will not be able to do so throughout his 
working career. Many clients may be able to continue 
sedentary or light work until retirement, and should 
not be encouraged to continue medium or heavy work 
which will perhaps further inflame their joints and 
result in total disability before the usual age of 
retirement. 

Climbing skills, balancing skills, stooping, and 
kneeling are all hampered by pain on weight bearing or 
with motion. All these activities may be accomplished 
by most clients with rheumatoid arthritis, but activities 
that repeatedly overuse joints without periods of rest 



Rheumatic Diseases 199 



and recovery should be avoided. Reaching, handling, 
and fingering will be clumsy, painful, weak, and 
awkward in clients whose hand joints are inflamed. 

A further consideration concerns which joints are 
the most seriously involved. Unfortunately, at onset 
this is somewhat difficult to predict or determine. 
However, if the arthritis seems to attack particular 
joints and spare others, the counselor may consider 
recommending vocations that utilize the less affected 
joints. Thus, if the fingers are spared but the hips and 
knees are seriously involved, a sedentary clerical job 
might be appropriate. 

Unless the rare occurrence of peripheral 
neuropathy, mononeuritis, or the carpal tunnel 
syndrome decreases finger sensation, no loss of 
sensation should interfere with the rheumatoid arthritis 
client's ability to work. Speech and hearing are also not 
impaired. Vision is unaffected unless steroid-induced 
cataracts occur, or if a toxic response to chloroquine 
appears. 

Environment. It is usual for clients with rheumatoid 
arthritis to detect changes in humidity, temperature, 
or barometric pressure. An indoor climate where the 
environment is relatively controlled is therefore well 
advised. Extremes of weather or temperature with 
abrupt variation probably should be avoided. Whether 
the climate is wet, humid, dry, warm, or cold is 
apparently less important to a client's comfort than the 
frequency of abrupt changes. The occurrence of 
excessive noise, vibration, fumes, gases, dust, and 
poor ventilation have no specific effects on clients with 
rheumatoid arthritis. 

Many clients with rheumatoid arthritis may not be 
industrially competitive but are able to work in a 
sheltered workshop. This experience may enable them 
to work in an environment consistent with their level of 
productivity and may also support them until they 
acquire the skills that will enable them to gain access to 
the competitive commercial world. 

Above all, the counselor must realize that the client 
with rheumatoid arthritis does not have a permanent 
disability with clearly defined limits, but rather a 
changing disability with frequent pain, varying from 
day to day, but generally resulting in an unfavorable, 
disabled outcome. Such clients require a combination 
of analysis, sympathy, encouragement, and adequate 
evaluation and treatment. The vocational counselor 
who skillfully coordinates these issues will be amply 
rewarded. 



ANKYLOSING SPONDYLITIS 

Disease Description 

Ankylosing spondylitis is a form of inflammatory 
joint disease which most affects the joints and 
ligaments of the spine. Ankylosis refers to bony or 
fibrous union, and spondylitis means inflammation of 



the joints of the spine. The disease usually occurs in 
young men in the first through third decades and 
frequently affects members of the same family. 

The cause of ankylosing spondylitis is unknown, but' 
it is thought that an allergic reaction occurs, causing 
the body's immune cells (lymphocytes) to invade joints 
and tendons. The presence of immune cells in the 
joints and tendons is accompanied by the elaboration 
of various substances which start an inflammatory 
reaction. The inflammatory reaction is followed by 
repair and healing with new bone formation. The 
disease is thus characterized by erosion and destruction 
of tendons and joints, particularly in the spine, with 
the formation of bony bridges and reduced motion. 

The sacroiliac joints, or posterior pelvic joints, are the 
most frequently involved at first, manifested by low 
back pain. Since these joints have little motion 
normally, there is little motion impairment when they 
become fused. Subsequently, the lumbar spine, 
thoracic spine, and eventually the cervical spine are 
involved in ascending order, although the process may 
skip over parts. At first the spine is painful and tender, 
but later, when it is completely fused and the patient 
has lost his ability to rotate or bend it, pain may 
disappear. As the patient develops a stiff or "poker" 
spine, the joints where the ribs attach to the vertebrae 
also are inflamed and subsequently heal with bone 
formation and loss of motion. The patient therefore 
cannot expand his chest well and takes in less air with 
each breath. 

Less frequently, other joints, including the hips, 
shoulders, and, more rarely, the peripheral joints, such 
as elbows, hands, feet, and ankles, are involved. This 
inflammation is usually not as destructive as in 
rheumatoid arthritis, but on occasion hip joints are 
solidly fused and other joints are damaged and lose 
motion. 

Some patients complain little of pain, and physical 
examination and X-rays are necessary to diagnose 
ankylosing spondylitis. Some of these patients progress 
to relatively marked loss of motion quite insidiously. 

Two features of this disease as it affects the vertebral 
column contribute to the degree of disability produced: 
(a) pain is less when the vertebral column is flexed, 
and (b) the disease progresses continuously to fusion of 
the vertebral column. Since fusion will occur in the 
usual position of the back, a severe flexion deformity of 
the trunk may result. A patient might progress to the 
point where fusion occurs with his head in so much 
flexion that he cannot look around and can focus only 
on the floor. 

Ankylosing spondylitis is not onlv the name of a 
particular inflammation of the spine which occurs in 
young men, but is also a descriptive term which is 
applied to other diseases in which inflammatory 
stiffening of the spine occurs. Among these are 
regional enteritis, ulcerative colitis, Reiter's 
syndrome, and psoriasis. They are briefly discussed 



200 Nicholas 



here, since spondylitis may be the predominant finding 
for which the patient is referred to the vocational 
counselor. 

Regional enteritis and ulcerative colitis are both 
inflammatory diseases of the bowel. They are 
distinguished by X-ray evidence of bowel 
inflammation, persistent bloody diarrhea, and 
characteristic findings on direct visual examination of 
the sigmoid colon. Reiter's syndrome, like anklyosing 
spondylitis, is a disease mostly of young men. It is 
characterized by inflammation of the eye (uveitis), 
inflammation of the lower urinary passage (urethritis), 
and inflammation of peripheral joints (arthritis). Skin 
eruptions on the sole of the foot or the penis are often 
found. Psoriasis is a very common skin disease. The 
spondylitis of Reiter's syndrome and psoriasis differs 
from that of inflammatory bowel disease and primary 
ankylosing spondylitis in having less complete 
involvement of the spine with more "skip" areas. 
Motion is therefore less restricted. 

All forms of ankylosing spondylitis demonstrate a 
gradually worsening course in which the pain lessens 
but the stiffness and lack of motion increase. 
Currently, no form of treatment protects the patient 
from ultimately developing a stiff spine. 

Patients with ankylosing spondylitis may also have 
inflammation of the aortic semilunar valves (see chap. 
3). If this is sufficiently extensive, blood will leak from 
the aorta back into the heart through the damaged 
valve (aortic insufficiency), and the patient may 
develop heart failure. The electrical conduction system 
for activating the heart may also become partially 
blocked, causing various forms of irregular heartbeat 
which will require medical treatment. On occasion, 
patients will have inflammation of the tissues of the eye 
and develop pain on viewing and, if untreated, may 
develop blindness. Pain in the heel may occur from 
inflammation of the attachment of the tough tissues of 
the sole of the foot to the heel bone (calcaneous), and 
painful bone spurs may develop. 

Functional Disabilities 

Loss of motion of the spine or pain in the spine 
with motion may greatly affect a patient's mobility. 
Walking, however, will be unimpaired unless the hips 
and knees become inflamed and stiffen. A stiff spine 
may make certain chores difficult, and certainly 
frequent stooping and bending will be impossible. 
Toilet activities and dressing may be difficult, but 
rarely does the patient become less than self-sufficient. 
In fact, a characteristic of patients with ankylosing 
spondylitis is that, in spite of progressive stiffness, they 
are able to continue their chosen vocational activity 
unless it requires significant back mobility. 

There are no specific psychological or social 



impairments ascribed to patients with ankylosing 
spondylitis except those that may develop in any 
disability where there are major functional losses. As 
the spine stiffens, the patient's posture may become 
unsightly. Patients with ankylosing spondylitis and 
psoriasis may become socially isolated or resentful. 

The rehabilitation potential of the patient with 
ankylosing spondylitis is relatively good. Patients may 
have to change their vocations if their jobs require 
flexed postures or much back movement. The 
prognosis for life approaches that of normal 
individuals, so that if the patient can no longer perform 
his duties because of back pain or stiffness, he may be 
supported in training programs of any length. No goal 
which is appropriate to the patient's resources, skills, 
and aspirations should be denied because of the time 
required for its attainment. 

Standards of Evaluation 

The patient with ankylosing spondylitis must have 
X-rays of the sacroiliac joints, the entire spine, and any 
other joints that demonstrate pain and swelling. 
Besides a complete history and physical examination, it 
is imperative that the patient with suspected 
ankylosing spondylitis have precise measurements 
made and recorded of his ability to expand his chest 
and of the distance between the back of his skull 
(occiput) and a wall against which he is standing as 
erect as possible. His ability to flex his spine should be 
measured at frequent intervals. These three 
measurements characterize the degree of physical 
impairment and measure its progress. In addition, 
respiratory function and involvement of other joints 
need to be evaluated. An internist with special interest 
in rheumatology is usually best able to assess the 
disease. 

A recently described association between patients 
with ankylosing spondylitis and the tissue typing 
antigens has revealed that the antigen HLA-B27 
occurs in 95 percent of patients with ankylosing 
spondylitis, 90 percent of patients with Reiter's 
syndrome, and about 50 percent of patients with 
bowel inflammation and psoriasis who develop 
anklyosing spondylitis. It is not essential for the HLA- 
B27 test to be ordered on all patients with obvious 
ankylosing spondylitis. However, for the young patient 
with early arthritis in whom the diagnosis is unclear, the 
test may provide evidence that the arthritis will eventually 
turn out to be ankylosing spondylitis. 

Cardiac and eye examinations should be performed 
routinely. All patients should be referred to a physical 
therapist for posture evaluation. Patients with psoriasis 
must be referred to a dermatologist for care of the skin 
problem, and patients with an inflammatory bowel 
disease should have gastroenterological consultation. 



Rheumatic Diseases 201 



Total Treatment 

The patient is usually treated with the appropriate 
medicines to control pain. Aspirin is still the first 
choice and is used in the highest tolerable doses to 
achieve the best effect. If aspirin is inadequate, the 
addition or substitution of the nonsteroidal anti- 
inflammatory drugs should be tried (see above). 
Indocin and phenylbutazone appear to work especially 
well in patients with spondylitis. Phenylbutazone 
(Butazolidine), however, has considerably more dan- 
gerous side -effects and its choice is therefore less 
frequent. Adrenocorticosteroids (e.g., prednisone) are 
also quite effective, but because of the more frequent 
and more serious side -effects, they are considered less 
desirable than Indocin, but preferable to phenylbutazone. 
The side -effects of these drugs, their doses, and the 
responses are discussed in greater detail on page 195. 

If the spine pain of ankylosing spondylitis does not 
respond to anti-inflammatory medication, radiation 
treatment may be considered. Radiating the spine 
certainly eliminates pain and inflammation in many 
resistant cases, but an increased incidence of leukemia 
at a later date in these patients has been reported. 

Physical therapy treatment includes instruction in 
exercises which will enable the patient to maintain as 
erect a posture as possible, to expand his chest and fill 
his lungs to as great a degree and for as long as 
possible, and also to vigorously and deeply exhale in 
order to expand the thorax. It is felt by rheumatologists 
that if the exercises are performed diligently and daily, 
the patient will have better posture and lung expansion 
than a patient who does not exercise. Braces have been 
found ineffective in passively keeping the spine 
extended. 

When a marked flexion posture has developed (a 
forward slump), surgical correction may be 
considered. On occasion, removal of a piece of the 
bony spine has allowed a bent spine to be straightened, 
but this operation must be performed only by the most 
experienced surgeon. In addition, it must be 
determined if the patient is properly educated, 
motivated, and has had adequate conservative 
treatment in order to benefit from this operation. 

The treatment of Reiter's syndrome is similar to 
that of ankylosing spondylitis, but more attention to 
peripheral joint arthritis is required because these joints 
are more frequently inflamed. Treatment of psoriasis 
includes many skin medications, ultraviolet light, and 
anticancer drugs. 

Inflammatory bowel disease requires more vigorous 
treatment than the accompanying spondylitis. On 
occasion, surgical removal of the inflamed and 
bleeding bowel is required. Since there is an increased 
incidence of cancer in inflammatory bowel diseases, 
repeated X-ray observations must be performed. 

Rarely, patients with ankylosing spondylitis develop 



limited motion in the hip and shoulder joints secondary 
to synovial inflammation similar to that of rheumatoid 
arthritis, with bony bridging across the joint. When 
this occurs at the hip, surgical implantation of a total 
hip prosthesis has been used successfully. Sometimes 
the bone re-forms, undoing the surgeon's work. 

Vocational Implications 

The client with spondylitis may be considered for 
vocational, college, or professional training as his 
resources indicate. Spondylitis will not affect the 
client's mental aptitudes, learning ability, verbal skills, 
numerical skills, or form and space perception. 
However, although motor coordination, eye-hand 
coordination, and eye-hand-foot coordination will not 
be impaired, a stiff back will limit the client's twisting, 
turning, and perhaps reaching in such a way that 
dexterity may be limited. The client will be quite 
dexterous with tasks placed directly in front of him but 
may have difficulty with tasks requiring reaching or 
bending. Color discrimination should be unimpaired, 
although the complication of inflammation of the eye 
(uveitis) may diminish vision. 

Severe deformity may cause embarrassment in 
interpersonal relations, but this is hard to predict. The 
client's preference for technical versus personal work 
would depend on his particular personality charac- 
teristics. In addition personal characteristics will 
determine whether or not the client will do better 
with tasks of a routine or a more creative nature. 
Tasks which require piecework or are production 
oriented often require a person to maintain a fixed 
position while working rapidly with the hands. The 
client with spondylitis mav not do well with such tasks 
because he often requires frequent changes of position. 

A client with a persistently painful back probably 
will do less well in a situation of chronic emotional 
stress. Associated muscle tension, for example, can 
increase pain and further reduce motion. In such a 
situation, the client may become too preoccupied to 
attend to the need to pull himself erect and practice his 
exercises during the course of the day. 

Clients with spondylitis should not be offered tasks 
requiring lifting over 10 to 15 pounds (4.5 to 6.8 kg). 
Even lifting 20 pounds (9 kg) with significant walking 
or standing (light work) may cause intolerable back 
pain. Some clients, however, have little pain. Although 
their spines become quite stiff, they must pay careful 
attention to posture. Lifting and bending will not be 
well tolerated and may even enhance the spine flexion. 
Clients with arthritis of the hips and knees certainly 
should not be required to perform consistent or 
prolonged walking. 

Climbing skills, balancing skills, stooping, and 
kneeling may be tolerated by some clients but not by 
others. The counselor should avoid suggesting 



202 Nicholas 



vocational opportunities which require these activities. 
Reaching, handling, fingering, and feeling will not be 
impaired, but as spinal motion is often an integral part 
of these maneuvers, they may be well tolerated. Tasks 
requiring dexterous activity without reaching, 
standing, stooping, or bending are certainly within the 
vocational reach of clients with ankylosing spondylitis. 
However, even with sedentary tasks, the client must be 
allowed the opportunity to stretch his spine frequently. 

While many arthritic clients describe joint pain in 
relation to weather changes, clients with spondylitis 
should not necessarily require an indoor environment. 
Some noise, vibration, fumes, gas, dust, and poor 
ventilation should be no more troublesome than they 
would be to an ordinary worker. 

A sheltered workshop will be required only by the 
clients with severe spinal deformity who cannot 
perform at industrial levels. Clients with advanced 
education or clerical skills will frequently be able to 
continue employment, while those with only manual 
skills will become disabled. 



DEGENERATIVE JOINT DISEASE 
Disease Description 

Degenerative joint disease (DJD; osteoarthritis) is 
usually a disease of patients in their sixth or seventh 
decade. It affects joints singly or in pairs, but rarely 
involves more than one to three joints in an individual. 
Commonly, the patient notes the gradual onset of pain 
on use or weight bearing in a hip, knee, carpometa- 
carpal joint at the base of the thumb, or distal 
or proximal interphalangeal joints of the fingers. 
The shoulder is rarely involved. In the spine, the term 
degenerative disc disease is preferred, and this disease 
is discussed in chapter 15. 

When swelling occurs around the joint, it is usually 
due to bony overgrowth rather than to increased 
synovial tissue or joint fluid accumulation as in 
rheumatoid arthritis. DJD begins as a roughening of 
the surface cartilage of the joint. There follows an 
increase in water content of the cartilage, a 
proliferation of cartilage cells, and an increase in 
cellular metabolism. Subsequently, the bone under 
the cartilage proliferates, with the development of 
"spurs" of bone growing out from the margin of the 
joints, an apparent attempt of the body to repair or 
replace the damaged joint. The cartilage does not 
regenerate or repair itself and does not become 
stronger, despite the increased number of cells and 
increased metabolism. It progressively frays, thins, 
weakens, and becomes disarrayed until the 
cartilaginous cushion which normally protects the 
bones of the body from shock becomes ineffective. The 
bony surfaces then rest and rub painfully on each 
other. There often is a little fluid in the joint and a few 



inflammatory cells, and hence anti-inflammatory 
arthritis medication is often beneficial. 

The cause of DJD is entirely unclear. It is thought to 
occur as a consequence of some earlier, perhaps 
unnoticed, damage to the joint, which then fails to 
withstand wear and use with time. Overweight is 
often associated with DJD of the hip and knee, and 
DJD of the proximal interphalangeal and distal inter- 
phalangeal joints seems to be hereditary. 

Some diseases predispose an individual to DJD. 
Ochronosis, a hereditary defect in the metabolism of 
the amino acids phenylalanine and tyrosine, results in 
a deposition of a dark pigment in connective tissue and 
joint cartilage, leading to cartilage breakdown and the 
development of DJD. In addition, diabetes, syphilis, 
syringomyelia, and other neurological conditions 
which affect the nerve supply and sensation of a joint 
lead to rapid and extreme DJD. This appears to be due 
to lack of protective nerve impulses from the joint. 
Thus use, loss of innervation, hereditary cartilage 
defects, joint trauma, and probably other as yet 
unknown factors lead to this condition. 

The occurrence of DJD in multiple finger joints is 
common and should not be alarming. Rarely are these 
joints a site of intense inflammation. When it does 
occur, the condition is known as erosive osteoarthritis 
(EOA). This condition should not be confused with 
rheumatoid arthritis because the treatment, prognosis, 
and vocational implications are radically different. 
Even inflammatory DJD of the fingers with bony 
proliferation is an isolated condition for which local 
treatment is indicated, and unless marked finger 
dexterity is required by the patient, he should not 
otherwise be limited in his vocational capabilities. 

The course of DJD is generally progressive and 
unremitting. The patient will have good days and bad 
days. Weather, overuse, and unknown factors will 
cause exacerbations from time to time, and rest and 
proper treatment will diminish symptoms. DJD does 
not shorten life expectancy. 

Functional Disabilities 

DJD affects the patient's performance by impeding 
use of the involved joint. Since the hips and knees are 
common sites of DJD, walking and transfer activities 
may be impaired. At first the patient will be able to 
function well in a limited area, but as joint damage 
increases, he will be less and less able to walk to the 
parking lot, the bus, or the shopping center. Dressing, 
eating, and other activities of daily living, however, 
will be little impaired. Although DJD of the jaw joint 
(temporomandibular joint) may occur, talking and 
even eating are rarely impaired. DJD of the hip may 
interfere with perineal hygiene due to limited motion 
of the hip, and sexual activities may be difficult due to 
hip pain. 



Rheumatic Diseases 203 



Decreased ability to walk or perform related 
activities and the associated pain can certainly lead to 
despair, frustration, anger, and depression. Treatment 
of the primary condition can considerably alter both 
function and these psychological responses. Unlike 
rheumatoid arthritis, acute attacks do not occur. The 
course of the disease is slow, with gradually increasing 
impairment as pain increases and joint motion 
decreases. 

The rehabilitation potential of a patient with DJD is 
generally limited by his age and the results of medical 
and surgical treatment. Since walking is the function 
most commonly impaired in patients with DJD, 
treatment that improves ambulation may allow 
continued employment. If treatment cannot eliminate 
the pain, the long- and short-term goals and vocational 
aspirations should be guided by the patient's age and 
interest. The presence of DJD will not decrease the 
number of a patient's working years. 

Standards of Evaluation 

The evaluation of the patient with DJD includes 
X-rays of each involved joint and its mate on the 
opposite side for comparison. Repeat films at annual 
intervals help assess the progress of the disease. A 
medical history should elicit any history of trauma, 
diabetes, syphilis, or sensory abnormalities. The 
history will point out which joints are involved, the 
duration of pain, and factors, such as use or increased 
weight, which influence symptoms. Laboratory data 
obtained for evaluation should include tests for syphilis 
and diabetes. No tests other than X-rays are needed to 
confirm the presence and extent of DJD in ajoint. The 
physical examination includes a detailed examination 
of joint range of motion and strength and an 
assessment of walking limitations. 

While an internist or family practitioner usually can 
easily make the diagnosis, evaluation by physicians 
with special interest in the musculoskeletal system, 
such as orthopedic surgeons and physiatrists, should be 
obtained prior to vocational rehabilitation planning. 

The patient might also be referred to the physical 
therapist for evaluation and instruction in hip and knee 
exercises, and for gait training if gait is impaired. The 
occupational therapist may be useful for evaluation of 
self-care skills and provision of splints, such as a thumb 
splint for those with carpometacarpal DJD of the 
thumb. Dieticians may be helpful if the patient is 
overweight. If functional losses are severe, 
psychological and social evaluations may be 
appropriate. 

Total Treatment 

Total treatment begins with the administration of 
antiarthritic drugs to decrease the pain. The same 
drugs described for rheumatoid arthritis are utilized, 



and aspirin again is the mainstay. The injection of 
steroids directly into the involved joint is also an 
effective treatment, but should be limited to about four 
times annually so as not to advance joint destruction. 
Weight reduction and some curtailment of weight 
bearing is essential. Judicious use of canes and 
crutches, for example, protects against progression. 
The patient should be taught by the physical therapist 
to exercise the muscles around the knee and hip to 
maintain strength and range of motion. 

When nonsurgical treatment fails to decrease the 
pain sufficiently, referral to an orthopedic surgeon for 
consideration of joint replacement arthroplasty is 
appropriate (see p. 196). Complications of surgerv 
include dislocation, infection, or failure to achieve the 
pain relief, motion, or function hoped for. 

Vocational Implications 

Since DJD is not a systemic disease, successful 
treatment of a single involved joint may result in 
continued employment in the client's current job or a 
resumption of the same or related activity. The client is 
therefore likely to be able to perform those tasks which 
fit his education, aptitudes, and interests unless they 
require dexterous or heavy use of an involved joint. 
Even if surgical or medical treatment of the affected 
joint results in an increased range of motion, 
diminished pain, and ability to walk further, the use of 
that joint should be limited. Heavy lifting, which 
repeatedly places unusual stresses on the hips and 
knees, is best avoided. Light to medium work, 
however, should be possible. 

Return of the client to work after undergoing 
successful surgery requires careful consideration. The 
pain and the need to limit activity will be less and his 
ability to walk will probably be increased. Nonetheless, 
the client's activity should be limited, as the durability 
of the prosthetic implants has not yet been measured. 
While further surgery might be attempted, it is not yet 
clear that a second, replacement prosthesis will be as 
successful as the initial prosthesis. 

Climbing, balancing skills, stooping, and kneeling 
will be impaired in many patients with DJD unless 
treatment removes the pain and consequent 
unsteadiness of hip and knee. Stooping and kneeling 
may be accomplished without pain following surgery 
but should be limited. Certain motions involved with 
stooping and kneeling may cause an implanted hip 
prosthesis to become dislocated. Climbing and 
balancing can be accomplished, but are hazardous and 
should not be allowed to make up the bulk of the 
client's vocational activity. 

Dexterity will be impaired or limited so slighdy by 
surgical fusion of the carpometacarpal joint of the thumb 
that only occupations requiring unusual finger dexterity 
need be eliminated from consideration. 

The environment has no significant effect on 



204 Nicholas 

patients with DJD. Changes in relative humidity, 
barometric pressure, and temperature may cause joint 
discomfort as they do in other form of arthritis, but 
these are not influential enough to lead to a change of 
residence in most cases. 

If the client cannot have surgical replacement of the 
hip and knee joint and medication does not suppress 
the pain sufficiently, sedentary or sheltered workshop 
activity may be advisable. Unless there is severe 
involvement, however, the client should be able to 
pursue competitive industrial tasks. 

BIBLIOGRAPHY 

Harris R: Rehabilitation. In Licht S (ed): Arthritis and 
Physical Medicine. Baltimore, Waverly Press, 1969, pp. 
458-481. 

A general review of rehabilitation medicine techniques 
applied to the patient with rheumatoid arthritis. The section 
describing vocational modifications and restrictions is brief. 

Hill AGS: General management of rheumatoid arthritis. 
In Scott JT (ed): Copeman 's Textbook of the Rheumatic Diseases. Ed 
5, Edinburgh, London, New York, Livingstone, 1978, pp. 
391-403. 

A discussion of the identification of problems and questions of 
communication. It introduces methods of treatment and a 
general guide to overall management. 

Nichols PJR: Rehabilitation. In Scott JT (ed): 
Copeman's Textbook of the Rheumatic Diseases. Ed 5, Edinburgh, 
London, New York, Livingstone, 1978, pp. 491-508. 
A detailed description of many aids and devices and a brief 
statement about feasibility of particular jobs. 

Robinson HS: Prognosis: Return to work — arthritis. In 
Erlich, GE (ed): Total Management of the Arthritic Patient, 
Philadelphia, Lippincott, 1973, pp. 183-192. 
This brief chapter gives citations of available employment 
statistics for arthritic patients. 

Vignos PJ Jr, Thompson HM, Katz S, Moskowitz RW, 
Fink S, Svec KH: Comprehensive care and psychosocial 
factors in rehabilitation in chronic rheumatoid arthritis: A 
controlled study. J Chronic Dis 25:457-467, 1972. 
Forty rheumatoid arthritis patients were divided into two 
groups. One group received additional intensive treatment, 
including visiting nurse services, and the control group did 
not. For both groups, the interrelationships among social 
adjustment, personal adjustment, intelligence, and 
motivation were studied. The influence of these factors and 
intensive treatment on medical condition and maintenance or 
improvement of function in activities of daily living was also 
studied. 



15 ORGANIC MUSCULOSKELETAL 
BACK DISORDERS 

Rene Cailliet, M.D. 
Loren A. Helberg, Ph.D. 



INTRODUCTION 

Pain in the low back, with or without radiation into 
the lower extremity, is a frequent symptom that affects 
approximately 80 percent of all persons sometime 
during their lifetime. Back problems constitute a large 
percentage of all disabilities. They are perhaps the 
most nebulous of all musculoskeletal disorders, and 
affect persons of both sexes at any age. 

The location of the discomfort is usually in the 
lumbosacral area. It may be at the midline, on either 
or both sides of the midline, or across the entire low 
back. Radiation (spread) of the discomfort, if present, 
may extend into the buttocks or the groin, down the 
posterior or posterolateral thigh, or into the calf, ankle, 
and foot. In many cases, there are not objective 
findings on physical examination and the various 
specific tests of the neurological, musculoskeletal, and 
other related systems are normal. Diagnosis in such 
situations is usually based on the details of the patient's 
description of his discomfort and its history. The 
absence of positive pathology on examinations or tests 
is, however, not entirely without value, for it assists in 
eliminating some potential causes of back pain. 

Many of the labels applied to problems of low back 
pain are not always accurate or meaningful from the 
point of view of management. Patients and even 
physicians might interpret the condition as a slipped 
disc, bulging disc, ruptured disc, disintegrating or 
degenerated disc, lumbago, and/or sciatica, often 
without true documentation. Others might more 
rightfully simply apply the symptom itself as the 
diagnosis. Hence, "chronic low back pain" may 
appear on the records as the diagnosis. 

In this chapter, the normal spine will be discussed 
first to provide a basis for understanding the pathology 
of the major specific back disorders described later. 
The sections of chapter 3 on the musculoskeletal and 
nervous systems and pages 65 to 68 in chapter 5 also 
provide additional helpful background information. 

THE NORMAL SPINE 

The vertebral column has 7 cervical vertebrae, 12 



thoracic vertebrae, 5 lumbar vertebrae, the sacrum, 
and the coccyx. It is useful to consider as a functional 
unit any two neighboring vertebrae and their 
connections (fig. 15-1). Each of these two vertebrae 
consists of a body, two pedicles, two laminae, two 
transverse processes, one spinous process, and two 
articular processes with their facets. The two vertebrae 
are connected by the anterior longitudinal ligament, 
the intervertebral disc interposed between the two 
bodies, the ligaments attached to the transverse and 
spinous processes, and the posterior, or neural arch, 
joints. These posterior joints are formed by the 
articulation of the facets of one vertebra on the facets of 
its neighbor. The facets are covered by articular 
cartilage and are surrounded by a joint capsule much 
like any synovial joint (see p. 32). 

The intervertebral discs are remarkable structures. 
They are 88 percent water retained in a matrix of 
protein and complex sugar compounds. This matrix is 
reinforced by concentric layers of tough collagen fibers 
(annulus fibrosus). The central portion of the disc, the 
nucleus pulposus, is well contained within the annulus. 
The nucleus is an amorphous gel under pressure and it 
keeps the bodies of the vertebrae apart and the 
connecting ligaments taut. 

The spinal cord lies within the vertebral canal 
formed by the vertebral bodies, pedicles, and laminae. 
As discussed on page 65 and as illustrated in figure 
15-2, each spinal nerve leaves the vertebral canal 
through an opening (intervertebral foramen) between 
the pedicles of the two vertebrae of a functional unit. 

A number of tissues in and around each functional 
unit can cause localized or radiating pain if they are 
irritated, injured, or inflamed. These tissues are the 
posterior longitudinal ligament, the outer layers of the 
annulus fibrosis, the spinal nerve passing through the 
intervertebral foramen, the posterior synovial joints 
and capsules, the interspinous ligaments, and the back 
muscles themselves. The skin and subcutaneous fat 
overlying the back muscles can also be a source of 
discomfort. Figure 15-2 illustrates how these tissues 
receive their nerve supply and hence how they are able 
to "communicate" discomfort perceived as coming 



205 



206 Cailliet and Helberg 



Vertebral body 



Pedicle 



Transverse 
process 



Disc 



Anterior 

longitudinal 

ligament 




Spinous 
process 

Intervertebral 
foramen 

Interspinous 
igament 

Facet 



Posterior 

longitudinal 

ligament 



Articular processes 
with opposing facets 



FIGURE 15-1. Lateral (side) view of the functional unit of the 
vertebral column. Two vertebrae and one disc make up the unit. 
The spinal cord (not shown) is located behind the posterior 
longitudinal ligament. The spinal nerve (also not shown) exits 
through the intervertebral foramen. 



Spinous process 



To skin and muscle 




Posterior 

longitudinal 

ligament 

Nucleus pulposus 



Annulus fibrosus 

FIGURE 15-2. Top view of functional unit showing nerve 
connections to the soft tissues of the functional unit. The spinal 
nerve leaving the vertebral canal branches into an anterior and a 
posterior division. The posterior division serves the facet joints, 
the interspinous ligaments, and the skin and muscle of the back. 
The anterior division sends a nerve branch back into the 
vertebral canal to serve the posterior longitudinal ligament, 
annulus fibrosis, and meninges (coverings) of spinal cord. The 
anterior division goes on to connect to the sympathetic nervous 
system (see fig. 3-12, p. 29) and to innervate muscle function 
and sensation in the limbs. 



from the local area of damage or as radiating pain. 
Radiating pain seemingly comes from areas away from 
the local damage which receive their nerve supply from 
the same spinal nerve associated with the damaged 
tissues. 



The Erect Spine 

The erect vertebral column is composed of four 
curves. The curves created by the cervical and lumbar 
vertebrae are convex toward the front of the body and 



Back Disorders 207 




FIGURE 15-3. Lateral view of the erect normal spine, pelvis, and 
lower extremity. The normal curves (cervical lordosis, thoracic 
kyphosis, lumbar lordosis, and sacral kyphosis) are seen in 
relation to a gravity force line. 

concave toward the back and are termed lordotic curves. 
The curves created by the thoracic vertebrae and the 
sacrum are in the opposite direction and are termed 
kyphotic curves. Figure 15-3 shows the relationship of 
these curves to a vertical line passing through the 
center of gravity of the body. This vertical line passes 
from the ear down through the entire spine behind the 
hip joint and in front of the knee joint. Any excessive 



curvature in any one of the four curves must produce a 
compensatory increase in the curvature of the 
neighboring curve in order for the head to remain 
oriented over the feet. 

The cervical, thoracic, and lumbar vertebrae are 
supported at their base by the sacrum. The basic angle 
or orientation of the sacrum (lumbrosacral angle) is one 
factor that determines the degree of curvature of the 
lumbar lordotic curve, the thoracic kyphotic curve, 
and the cervical lordotic curve. The lumbosacral angle 
is formed by a horizontal line and a line parallel to the 
top surface of the sacrum (fig. 15-4). Since the sacrum, 
for all practical purposes, is rigidly attached to the 
pelvis, all muscles and ligaments attached from the 
thigh to the pelvis can influence the degree and the size 
of the lumbosacral angle. These muscles are the hip 
extensors, namely, the muscles of the buttock (glutei) 
and the posterior thigh (hamstrings), and the hip 
flexors (iliopsoas). The dominant ligament influencing 
this angle is the iliofemoral ligament in front of the hip 
joint extending from the pelvis to the femur. 

The influence of the size of the lumbosacral angle 
and the orientation of the sacrum to the curvature of 
the lumbar spine in the low back is illustrated in figure 
15-4. An increased lumbosacral angle results in an 
exaggeration of the lumbar lordosis ("swayback"), 
and a smaller lumbosacral angle reduces the curvature 
of the lumbar lordosis. As already mentioned, 
exaggerations or reductions of this type will also 
influence the magnitude of the curves in the thoracic 
and cervical areas. 

The body weight above any particular functional 
unit is supported by the vertebral body and the 
surfaces (facets) of the posterior synovial joints. The 
vertebral body, with its large surface area, is better 
designed to support the superincumbent body weight 
than is the smaller surface area of the posterior joint. 
As figure 15-5 shows, the greater the lordosis, the 
greater the amount of the body weight supported by 
the joints, and the smaller the amount supported by the 



(A) Normal 



(B) Increased lordosis 



(C) Reduced lordosis 




\ 

Lumbosacral 
angle 





Sacrum 



FIGURE 1 5-4. Effect of lumbosacral angle on lumbar lordosis. (A) Normal, (B) Increased, (C) 
Decreased. 




208 Cailliet and Helberg 




FIGURE 15-5. Effect of an increased lumbar lordosis on the 
forces acting on the posterior neural arch joints. The super- 
incumbent body weight, W, can be viewed as made up of two 
components: B, the force perpendicular to the vertebral bodv, 
and F, the force parallel to the surface of the vertebral body 
which is absorbed at the facets. The greater the lordosis, the 
greater the force F. 



vertebral body. An excessive lumbar lordosis, in 
addition to putting greater weight-bearing demands 
upon the facets, also induces more pressure on the 
posterior portions of the disc and tends to narrow the 
intervertebral foramen through which the spinal nerve 
emerges. For all these reasons, an excessively lordotic 
posture can induce back pain. 

Low back pain noted in the erect nonmoving spine is 
termed "static" or postural pain. Occupations that 
require relatively erect nonmoving postures would 
therefore be less than desirable for persons with static 
pain. 

The Moving Spine 

The lumbar spine allows movement in three 
directions; sideways, forward, and backward. The 



forward movement, such as occurs during bending and 
lifting, and the backward movement, such as occurs on 
the resumption of the erect posture, are the most 
important. Figure 15-6 indicates what happens to the 
lumbar spine when forward bending occurs. 
Normally, the lordosis of the erect posture fully 
reverses and becomes kyphotic at the end of a full 
forward movement. For this curvature change to be 
achieved, the discs must be flexible and the posterior 
longitudinal ligament, interspinous ligaments, and 
back muscles must be able to lengthen sufficiently. 

Full forward flexion is also associated with rotation 
of the pelvis about the hip joint axes and hence also 
requires the full length of the hamstrings. Resumption 
of the erect posture from a forward flexed position is 
ordinarily a smooth action as the pelvis rotates and 
the lumbar spine simultaneously resumes its lordosis. 



Back Disorders 209 




Ligament 
of hip joint 



FIGURE 15-6. Schematic display of lumbar spine, pelvis, and lower extremities during forward 
flexion. (A) Erect spine showing normal lumbar lordosis and lumbosacral angle. The iliofemoral 
ligament is fully stretched and the hamstrings are relatively slack. (B) As flexion begins, the lumbar 
lordosis disappears. The lumbosacral angle increases as the pelvis begins to rotate around the hips. 
The iliofemoral ligament becomes redundant and the hamstring muscles increase in length. (C) In 
full forward flexion, the lumbar curve is kyphotic, the lumbosacral angle is maximum, and the 
hamstring muscles are at maximum length. 



Low back pain that occurs during movement, either 
during forward bending or during resumption of the 
erect posture, is termed "kinetic" or functional back 
pain. Occupations associated with such movement 
therefore would be considered less desirable for clients 
with kinetic pain. 

SPECIFIC SYNDROMES 

Introduction 

A normal individual will be free of static and/or 
kinetic pain under the following conditions: 

1. No abnormality in the structures of the 
functional unit 

2. Adequate but not excessive length in the 
ligaments 

3. Proper strength and endurance of the 
musculature 

4. Body weight consistent with the individual's size 

5. Posture that, over the years, has developed 
without excessive curvatures 

6. Acute and chronic stresses and demands well with- 
in the capacity of the various tissues to respond. 



Clearly, then back pain may result: (a) from stresses 
exceeding the capacity of the tissues to respond, even if 
the individual's action was properly performed; (b) 
from less severe stresses on tissues with prior 
degeneration or injury; or (c) from improper 
performance of an activity, such as a bend or a lift, 
even with fully normal structures and stresses 
otherwise well tolerated. 

Fatigue, anxiety, anger, depression, and other 
distractions may induce improper performance and 
cause an injury. The likelihood of back pain is greater 
with even small stresses if individual muscles and 
ligaments are in poor condition due to a prior history 
of insufficient exercise and activity, or if the tissue ;s 
were previously damaged. Injury may produce actual 
ligament tears (sprains), overuse without actual tears 
(strains), intervertebral disc rupture, and joint 
inflammation. 

It must always be kept in mind that back pain may 
be secondary to other conditions, such as cancers or 
infections originating in bone or seeding into bone 
from other areas; kidney disease; bowel and gall 
bladder conditions; and diseases of the bladder and 



210 Cailliet and Helberg 



uterus. Ankylosing spondylitis (see chap. 14) should 
also be considered when back pain complaints appear. 

Osteoporosis is another condition that may cause 
low back pain. In osteoporosis, the amount of calcium 
in the bone is reduced and the bone is weakened. The 
manner in which osteoporotic vertebrae might cause 
back pain is not always clear. It is felt by some that 
osteoporosis may produce pain through tiny 
microfractures not apparent on X-ray examination. 
Frank obvious fractures can also occur if the bone is 
sufficiently weakened through loss of calcium to be 
unable to withstand the stress to which it is subject. 
Such stresses need not be great. In relatively severe 
osteoporosis, the simple action of getting out of bed or 
bending forward, even in a normal controlled manner, 
may be sufficient to produce fractures visible on X-ray. 
Osteoporosis is likely to produce both static and kinetic 
back pain. If a client has this condition, the counselor 
must deal directly with the physician involved with 
regard to any vocational planning. More often than 
not, osteoporosis requires relatively sedentary 
activities until treatment restores bone strength, if 
possible. 

To understand a major dimension of chronic low 
back pain from a behavioral point of view, chapter 16 
should be read. The following sections are limited to 
several specific syndromes that can cause low back pain 
with or without radiation into the buttock and the 
lower extremities. The salient features of acute sprains 
without disc rupture, chronic strains, herniated 
nucleus pulposus, degenerative disc disease, and 
spondylolisthesis are discussed. 

Acute Sprain Without Disc Rupture 

The counselor will find that the concepts of strain 
and sprain are not always explained or defined in the 
same way. For purposes of this discussion, a sprain, 
usually the result of an acute or sudden stress, 
invariably means that tissue has torn. A strain on the 
other hand, is a discomfort originating out of a 
chronic, repeating stress not excessive but occurring 
over a sufficiently long period of time to exceed the 
endurance capacity of the tissues involved. A strain, 
therefore, is not viewed as a frank tear or rupture. The 
following relatively common statements can help 
clarify these definitions. 

"I tripped and fell and sprained my ankle." 

"I jogged for six miles, and the doctor said I strained 
my ankle." 

"I lifted the motor out of my car and sprained my 
back." 

"I stood in line for 6 hours waiting to buy football 
tickets, and the doctor said it strained my back." 

The terms "acute sprain" and "chronic strain" are 
redundant, given these definitions for strain and 
sprain. It is useful, however, to use these terms to help 
avoid confusion. 



Acute low back sprain without disc rupture therefore 
implies the occurrence of an excessive stress on a 
normal well -conditioned back, a minor stress on a 
normal but unprepared back, or a relatively minor 
stress on an unconditioned or diseased back. The tears 
referred to in this condition are localized to the 
muscles, the ligaments, and the capsules of the neural 
arch joints. 

The pain associated with such tears is usually 
appreciated at the instant of the stress, although the 
initial pain may not be severe and might abate, only to 
become more intense within about 2 hours. 
Hemorrhage, edema, and inflammation following 
the tear cause increased pressure in the tissue and 
intense pain shortly thereafter. The muscles around 
the site will usually go into a spasm as a reflex response 
to splint the area and reduce movement. Chronic 
contractions of even the relatively healthy muscles may 
add additional secondary pain. The discomfort usually 
causes activity to cease and will cause any but the most 
stoic individual to seek medical attention, if not the 
same day, then certainly a day later. The pain, while 
initially located at the site of the tear, may also be 
experienced as radiating. Both static and kinetic pain 
are present. 

The key issue with acute sprains is adequate 
treatment and reduced use to allow healing to occur 
and hopefully to ultimately restore the tissue to a 
normal state. Lack of adequate treatment can result in 
unhealed tissue and predispose the back to further 
injury from less severe stresses and to chronic strain 
problems. 

A counselor's client is not likely still to be in the 
acute phase. However, he may well have an initial 
history of an acute sprain which did not fully heal, 
resulting in a chronic pain problem. 

Chronic Strain 

With static pain. A chronic strain problem exists 
when the onset and persistence of static low back pain 
is associated with a history of prolonged standing and 
sitting, superimposed on tissues no longer able to 
sustain such stresses. Prior sprains, poor posture, or 
general deconditioning may be the cause. The 
discomfort is described as aching or throbbing, 
possibly even sharp or excruciating. It is aggravated by 
prolonged activity and is eased by frequent changes in 
position. The pain may be referred into the buttock 
area, but rarely into the legs. 

Patients with this condition often have an increased 
lumbar lordosis, and the pain is aggravated by further 
arching or extension of the back, high heels, or a 
protuberant abdomen (fig. 15-5). Persistence of the 
swayback posture without a program of exercises to 
reverse it may cause the back to lose flexibility on 
forward bending and introduce kinetic pain as well. 

With kinetic pain. Chronic kinetic low back strain is 



Back Disorders 211 



Spinal nerve 



Posterior 

longitudinal 

ligament 



Pedicle 




Nucleus 
pulposus 

Annulus 



Anterior 

longitudinal 

ligament 



FIGURE 15-7. Top view of an intervertebral disc whose annulus 
fibrosis has ruptured in two places. On the left, extruding 
nucleus pulposus material abuts against the posterior longi- 
tudinal ligament. On the right, the extruding material bulges 
against the spinal nerve. 

associated with, and develops as a result of, repeated 
activities requiring forward bending. The pain may 
centralize in the low back, but may also refer into the 
buttocks and the legs. The variation depends upon 
which structures involved with the chronic activity are 
less able to handle the continued stresses. The pain, 
once it develops, is aggravated by forward flexion and 
resumption of the erect posture, and may also be 
present in the static sitting and standing postures. 

Patients with chronic kinetic low back pain usually 
demonstrate limited low back flexibility. The normal 
lumbar lordosis does not reverse on forward bending 
(fig. 15-6). If the hamstring muscles behind the thigh 
are tight, an added burden is placed on the lumbar 
spine during forward bending, which aggravates the 
flexibility limitation. 

The condition may develop after acute sprains or in 
deconditioned persons subjected to continuing stresses 
which, for them, are excessive. Poor techniques when 
executing forward bending and extension can aggravate 
the condition. Patients with increasing kinetic low 
back pain may develop secondary muscle spasm to 
inhibit further forward bending, creating back rigidity. If 
rigidity occurs only on one side, it will induce a 
scoliosis. 



Herniated Nucleus Pulposus (HNP) 

Herniation of the nucleus pulposus of the 
intervertebral disc refers to its extrusion through a tear 
in the annulus fibrosis (fig. 15-7). The tear may be 
secondary to an acute high-level stress (acute sprain) or 
to a long-standing chronic strain problem. Trunk 
rotation stresses are more likely to induce a tear or 
rupture in the annulus fibrosis than forward and 



backward forces. As the nucleus pulposus herniates 
through the tear, it may bulge against the posterior 
longitudinal ligament in the midline or rupture 
somewhat laterally to cause pressure on the spinal 
nerve exiting through the intervertebral foramen. 

The pain of a herniated nucleus pulposus (ruptured 
disc) is usually more severe than that of the other 
conditions discussed above. Spasm of the back muscles 
may be particularly marked and the patient may stand 
with a tilt and be unable to flex forward. The location 
of the pain in herniated or bulging discs depends upon 
which tissues are being pressed upon. Most commonly, 
herniated discs occur between the L4 and L5 vertebrae 
and between the L5 vertebra and the sacrum. 

In addition to the signs of kinetic low back pain, 
particularly if nerve pressure exists, pain will radiate 
down the extremity along the usual course of the 
particular nerve involved. Pain may be aggravated by 
straight leg raising, coughing, sneezing, and straining. 
In addition, weakness of the leg muscles served by the 
involved nerve, and numbness, tingling, and loss of 
sensation over the skin of the area ordinarily served by 
the nerve may also be present. Reflexes can also be 
affected and, in extreme situations, problems with 
bowel and bladder function may result. 

Degenerative Disc Disease 

Degenerative disc disease (DDD) is a condition of 
aging, and occurs in all individuals. A history of acute 
sprains and chronic strains can aggravate the 
progression of the degeneration. With aging, the 
intervertebral disc gradually dehydrates and the 
collagen fibers of the annulus fibrosis may gradually 
fray. Pressure within the nucleus pulposus causes it to 
gradually encroach outward into the annulus. This 
dissipates the piessure in the nucleus, the disc therefore 
begins to collapse, and the vertebrae begin to 
approximate each other (fig. 15-8). The anterior and 
posterior longitudinal ligaments become slack and the 
facets of the neural arch joint compress together, 
inducing degenerative joint disease (see chap. 14). 
Bone overgrowth occurs at the facet joints and at the 
vertebral bodies on either side of the degenerating disc. 
These bony overgrowths (osteophytes) appear on the 
anterior and posterior surfaces of the intervertebral 
foramen and may trap the emerging spinal nerve roots. 
The collapse of the vertebrae on each other restricts 
their mobility, resulting in somewhat more rigidity in 
the back and development of both static and kinetic 
pain. 

Movement of the lumbar spine is limited, 
particularly in extension. In the majority of situations, 
the degenerative process is greater on one side, and 
scoliosis may therefore occur. If the nerve roots are 
trapped, pain will radiate into the involved extremity, 
with numbness, tingling, loss of sensation, and muscle 
weakness, as in HNP. 



212 Cailliet and Helberg 



(A) Norma 



Anterior 

longitudinal 

ligament 

Vertebral 
body 

Intervertebral 
disc 




(B) Degenerative disc disease 



Articular 
processes with 
opposing facets 



Osteophytes 



Spinal nerve 




Compressed 

ntervertebral 

foramen 



Nesting 
facets 



/ 
Posterior 
longitudinal 
ligament 

FIGURE 1 5-8. Effect of degenerative disc disease. (A) Normal lateral view of functional unit showing 
spinal nerve exiting through intervertebral foramen. (B) Degenerative disc disease showing narrowed 
disc, bulging anterior and posterior longitudinal ligaments, nesting of facets, osteophyte production, 
narrowing of intervertebral foramen, and distorted spinal nerve. 



Unlike acute sprains, chronic strains, and HNP, 
which are exclusively diseases of the soft tissues, 
degenerative disc disease represents actual changes in 
the bone and joint structure. Hence, the condition is 
always there and is neither reversible nor correctable. 
Fortunately, degenerative, disc disease, although 
present in practically everyone as a function of aging, 
is not necessarily symptomatic and painful for 
everyone. While it is not always completely clear why 
some patients with degenerative disc disease have pain 
and others do not, one of the factors is the degree of 
static or kinetic stress on the more rigid back. 
Hopefully, proper conditioning and proper control of 
the static and kinetic stresses can allow the patient to 
function without pain. 

Spondylolisthesis 

Spondylolisthesis is a condition that may have its 
onset in the late teens and be present at the beginning 
of the working years. It is a unique mechanical 
abnormality of the lower lumbar spine. 

If the lumbosacral angle were zero, the lumbar spine 
would be perfectly vertical and all the superincumbent 
body weight would be directed straight through the 
center of the vertebral bodies. Since the lumbosacral 
angel is rarely ever zero and a lumbar lordotic curve 
almost invariably exists, the superincumbent body 
weight creates a stress that is chronically attempting to 
cause the L4 vertebra to slide forward on the L5 
vertebra, or the L5 vertebra to slide forward on the 
sacrum. Forward slipping is prevented by the locking 
of the articular facets (fig. 15-9A). The inferior facet of 
the uppermost vertebra of the functional unit locks 
behind the superior facet of the lower vertebra of the 
functional unit, preventing the forward slippage. 



If forward slippage does occur, spondylolisthesis is 
said to be present. Forward slippage can occur if there 
are defects (spondylolysis) in the locking mechanism. 
Figure 15-9B demonstrates one of these sypondolysis 
defects which can lead to spondylolisthesis. In this 
defect, the facet and the articular processes are 
separated from the bodies. Thus, while the facets may 
remain locked, the bodies still can slide forward. 

The sliding forward of one body on the other 
stretches the anterior and posterior longitudinal 
ligaments and the annulus fibrosis, producing pain. 
The interspinous ligaments can also be stretched and 
cause pain. Further, if the spinal nerve roots are 
trapped by the forward sliding, the same symptoms 
that occur in HNP or in DDD can be present, namely, 
pain referred into the leg, thigh, and hip, numbness, 
tingling, loss of sensation, and weakness. An increased 
lumbar lordosis, reduced lumbar spine mobility, and a 
depression in the back above the sacrum where the slip 
is occurring may be palpable. 

The various conditions associated with spondylolysis 
and the presence of forward slippage can be seen by 
X-ray. Spondylolisthesis, like degenerative disc 
disease, is a bony abnormality and cannot be reversed. 
The pain of spondylolisthesis, however, can often be 
corrected. 

Chronic Low Back Pain 

The presence of chronic low back pain after surgery 
may mean an earlier incorrect preoperative diagnosis, 
a failure to completely remove an extruded disc, 
or a failure to explore both sides of an offending 
disc or more than one level, all of which require proper 
diagnosis and correction. Persistent chronic low back 
pain which is not associated with any of the specific 



Back Disorders 213 



(A) Normal 



(B) Defect 



Pedicle 



Intervertebral 
foramen 

Anterio 

longitudina 

ligamen 




Defect in 
articular process 



Articular 
processes 
with opposing 
facets 



Interspinous 
ligament 



L5 vertebral 
body 




FIGURE 15-9. Spondylolysis and spondylolisthesis. (A) Normal lateral view of L5 vertebra and 
sacrum. (B) Defect in articular process of L5 (spondylolysis) with forward slippage of body of L5 on 
the sacrum (spondylolisthesis). 



syndromes discussed in this chapter can also occur. 
Such chronic pain may well be considered a disease in 
itself, and one that is particularly disabling, even 
though objective signs do not seem to "justify" the 
degree of disability. Chapter 16 deals with the nature 
of this condition, its causal factors, and the techniques 
available for treatment and rehabilitation. 

FUNCTIONAL DISABILITIES 

Physical Disabilities 

All of the specific syndromes listed above produce, to 
a varying degree, reduced levels of function in 
standing and walking, and possibly also in dressing, 
bathing, and other tasks that require general trunk 
mobility. Negotiation on uneven ground, slopes, and 
stairs may also be impaired. In the majority of cases, 
the ordinary requirements of dressing, walking, and 
standing can be met. Those activities associated with 
the patient's particular employment activity are likely 
to be impaired, since they are often the activity 
requirements contributing to the back problem 
initially. 

Pyschosocial Disabilities 

Back pain, psychological disturbances, and social 
upheaval frequently go together. Anxiety, depression, 
anger, and fear influence how a person uses his 
musculoskeletal system. All these emotional factors 
may increase the person's estimation of his disability 
and may in themselves aggravate the discomfort. Legal 
factors, compensation factors, and fear of supervisors 
all contribute to the psychological response and may 
interfere with an effective rehabilitation. Attitudes of 
spouse and family, co-workers, and others may 



influence how a patient responds to his discomfort. In 
the male, it is not unusual to find psychological 
impotency as part of the picture. 



STANDARDS OF EVALUATION 
Medical Evaluation 

Orthopedic surgeons and physiatrists are usually the 
physicians most able to evaluate the back and lower 
extremities. Since there are other than mechanical 
causes of back pain, a complete physical examination 
also needs to be included in addition to the localized 
examination of the back and the legs. When other 
causes might be suspected, an internist, urologist, or 
gynecologist might well be asked to see the patient if 
the diagnosis is unclear. 

The evaluation includes a careful history which 
characterizes the back pain, indicates factors that make 
it better or worse, analyzes the relationship of the pain 
to activities at work, play, or in the home, and a review 
of the details of an accident, if one has occurred. 
Examinations of posture, leg length, back mobility, hip 
range of motion, knee range of motion, and strength of 
the back, abdomen, and legs are generally included. A 
neurological examination of the lower extremities is 
mandatory for the presence or absence of spinal nerve 
disease. The search for causes might also include a 
number of blood and urine tests. 

X-ray examination of the back and sometimes the 
hips is standard. Part of the X-ray examination 
searches for congenital defects, such as: spina bifida 
occulta, a failure of the posterior elements of the 
vertebrae to fuse; sacralization, a condition in which 
the L5 vertebra is fused to the sacrum rather than 
existing alone; lumbarization, a condition in which the 



214 Cailliet and Helberg 



SI vertebra, rather than being part of the sacrum, is 
separated out as a vertebra on its own; degenerative 
disc disease; spondylolysis; and spondylolisthesis. 

Additional special tests may include electro- 
myography, an examination of electrical activity 
in the muscles of the lower extremity which can be 
altered due to spinal nerve involvement. A special 
X-ray examination, during which dye is injected into 
the vertebral canal (myelography), may be considered 
and is usually done after the decision to operate has 
already been made. The outline of the column of dye 
after injection is viewed by X-rays and can suggest 
injury to spinal nerves by herniated discs, narrow 
intervertebral foramina, spondylolisthesis, and degen- 
erative disc disease. 

II posture abnormalities or significant obesity 
problems are superimposed on the back pain problem, 
the primary physician might ask for assessment by a 
physical therapist to review posture and lifting 
techniques, and by a dietician to recommend 
modification of eating habits. 

Psychosocial Evaluation 

There is a clear interrelationship between emotional 
and social factors and back pain and disability. 
Therefore, all back pain patients with discomfort of a 
chronic nature and all who respond poorly to 
management and are still uncomfortable within 2 to 4 
months of an initial episode should have psychological 
or psychiatric consultation for review of contributing 
factors. It has been suggested by many that no patient 
should be operated upon who has not yet had careful 
psychological and personality review. Too often, 
psychological and psychiatric consultation is 
considered too late. 

Review by a medical social worker of social 
adjustment, the health of spouse and family, financial 
status, and any litigation factors can be valuable in 
understanding the entire milieu in which the back 
problem is occurring. Careful review of vocational 
history and performance also contributes to the 
understanding of the overall adjustment of the patient 
prior to the back discomfort. 



TOTAL TREATMENT 

Acute Sprain Without Disc Rupture 

Acute sprains require 6 to 8 weeks of immobilization 
for proper healing. The first 2 weeks might well 
include total bed rest, and hospitalization may be 
required for some patients. The remaining period of 
immobilization may not. require full bed rest, but will 
require reduced activity and the support of a corset. 
Medications to ensure relaxation and to control 
inflammation and pain are usually included during the 
acute phase. Judicious medication prescriptions are 



required and are best limited in duration to guard 
against drug abuse. Mild superficial heat and light 
massage are also useful. 

As the initial acute phase subsides, exercises are 
introduced to restore appropriate length to the 
muscles and ligaments involved in the area of the 
sprain or to restore mobility to the back and hips, and 
to restore strength and endurance to the back, 
abdominal, and leg muscles. For many patients, the 
remobilization and strength restoration activities may 
not begin until after 8 weeks of relative 
immobilization. 

The physician may instruct the patient in a home 
exercise program, ask a physical therapist to do so, or 
suggest that the patient regularly receive such 
treatment by a physical therapist until full range of 
motion and strength are restored. Instructions must 
also include proper posture, lifting techniques, and 
stooping techniques. Lifting techniques for back pain 
patients involve holding the object close in front of the 
body, with the pelvis tucked under the trunk and the 
lifting done largely with the legs rather than with the 
back. The amount of lifting allowed is often difficult to 
determine and is best assessed by starting with smaller 
weights and building up gradually to larger ones. The 
lifting of excessively bulky objects and those weighing 
over 25 pounds (11.3 kg) is usually discouraged. 

During the remobilization and strengthening 
program, the patient is gradually weaned off his corset. 
Depending upon the severity of the acute sprain, full 
resumption of all activities might not be achieved until 
4 months after the initial episode. Static and kinetic 
stresses associated with work, play, or home activities 
need to be carefully reviewed for modification, if 
suggested by the severity of the patient's sprain. 
Patients who have experienced acute sprains may well 
be advised to maintain a regular exercise program 
indefinitely to ensure future protection of their backs. 



Chronic Strain 

The back pain associated with chronic strain 
requires a shorter immobilization period than acute 
sprains, and rarely requires hospitalization. Cessation 
of the repetitive activity is important initially. 
Medication for pain relief might consist simply of 
aspirin or other mild analgesics. More important in the 
treatment of the chronic strain is usually a 2-month 
period of full reconditioning. This includes a physical 
exercise program to increase flexibility and strength, 
posture training, and training in lifting and bending 
techniques. A careful review of the activity productive 
of the strain can suggest alternative approaches to 
reduce the likelihood of a repetition. 

Herniated Nucleus Pulposus 

Herniated discs with nerve root irritation require 



Back Disorders 215 



complete bed rest with the hips and knees slightly 
flexed. At least a 2-week period should be considered to 
determine whether rest will prevent damage. While 
complete bed rest may be possible at home for some 
patients, others may require hospitalization to ensure 
complete rest. Pain medications and relaxants assist 
control of discomfort and anxiety during this period. If 
relief results, the patient might be allowed up with a 
corset and be managed subsequently as in acute 
sprain. A successful nonsurgical treatment program 
may require a term of 3 months for healing and 
restoration of back health. During this period, various 
anti-inflammatory medications of the type discussed in 
chapter 14 might be considered. 

If nonsurgical treatment is failing, surgery is con- 
sidered. A lamintomy (laminectomy), an opening in 
the lamina (see fig. 15-2), exposes the vertebral canal 
to the surgeon. This exposes the disc and the region of 
the compressed nerve. The herniated fragment of the 
disc is removed. 

Convalescence after successful surgery is usually a 
1 - to 3 -month period, depending upon the severity of 
the findings and the extent of nerve damage. After the 
first month, the convalescence reconditioning exercises 
may begin. These exercises are prescribed by the 
surgeon and outlined and performed with the 
assistance of the physical therapist. As in the prior 
conditions, these exercises are aimed at regaining low 
back flexibility and muscle strength. Proper bending 
and lifting techniques are also taught. 

Degenerative Disc Disease 

If degenerative disc disease is associated with spinal 
nerve root irritation, the treatment program outlined 
for herniated nucleus pulposus is followed. If 
nonsurgical conservative management does not 
succeed, surgical intervention is required to relieve the 
nerve of the pressure. Postoperative recovery may take 
longer. The various anti- inflammatory agents 
discussed in chapter 14 may also be regularly 
prescribed. Indefinite corset wear might be prescribed, 
and the reduced mobility produced by the corset must 
be considered in later activity and work planning. 

Spondylolisthesis 

When mild, spondylolisthesis may be manged with a 
period of bed rest followed by exercises to promote 
reduced lumbar lordosis and to prevent further 
slippage. However, it may be necessary to fuse the 
involved vertebrae surgically. If nerve root pressure is 
present and causing pain, surgery to relieve the 
pressure by widening the intervertebral foramen 
(foraminotomy) may also be required. When fusion is 
performed, as much as a 6-month period may be 
required for healing. Conditioning exercises for the 
back are also required after surgical fusion. 



General 

In addition to the specifics for each of the above 
conditions, some techniques may be applied to all of 
them. Local injections into painful back areas are 
sometimes useful. More recently, relatively less major 
surgical techniques have been introduced. In 
chemonucleolysis, a chemical is injected directly into 
the disc and is a potentially useful substitute for open 
surgery for a relatively minor herniated nucleus 
pulposus. An anesthetic agent, steriod medication, or a 
destructive chemical can be directly injected into the 
neural arch joint. Other techniques that destroy the 
nerve to the facet and reduce pain are also sometimes 
used. The procedures may be helpful simultaneously 
as a diagnosis and as a treatment. 

Special medication regimes for chronic pain have 
recently been found to be helpful where a source of 
pain is reasonably clearly documented but cannot be 
removed surgically. These medications include 
Dilantin, Tegretol, Elavil, and Prolixin, all of which 
require careful monitoring by a physician familiar with 
their use. Treatment techniques based on behavior 
modification are discussed in chapter 16. 

Psychosocial Treatment 

Depending upon psychological, psychiatric, and 
medical social work evaluations, simultaneous 
psychotherapy and family and marital counseling may 
assist patient recovery. 

VOCATIONAL IMPLICATIONS 

Evaluation of the suitability of potential employment 
for a client with current or prior back pain requires a 
particularly careful analysis of the sitting, standing, 
moving, lifting, pulling, pushing, and climbing 
requirements of the job. These specific requirements 
should be reviewed with the client's physician to 
determine whether they are compatible with the 
client's condition. If not, the counselor must then 
determine whether job environment modifications or 
work simplification can make a particular employment 
suitable. The counselor may also wish to consult the 
physician about prognosis, for any known anticipated 
change in the client's condition over time should be 
incorporated into the vocational plan. 

During the recovery phase of any one of the back 
syndromes, it may be possible to return the client to 
work either part time, or full time with temporary 
modifications in his job. This may ensure the 
continuation of the work habit and make it easier to 
resume full activities later. Some employers may prefer 
this approach to a longer layoff period. 

An industrial engineer or perhaps the counselor 
himself, equipped with knowledge of the client's limits 
and the job requirements, can suggest modifications in 



216 Cailliet and Helberg 



the work environment. Such things as proper elevation 
of tables, desks, and chairs, and adequate space for the 
client to utilize the movement techniques he has 
learned, can be considered. It may also be possible to 
modify the frequency and duration of any bending, 
lifting, carrying, pulling, shoving, or other weight- 
bearing activities and the size of objects to be handled 
by the worker. The amount of weight to be lifted is 
often not as important as the bulk of the object, the 
lifting technique, the height and direction of the lift, 
and the frequency and rapidity of the lifting process. 
For example, lifting an object measuring 2 feet by 4 
feet by 2 feet weighing only 12 pounds may be more 
detrimental than lifting a small 25 -pound object. The 
heavier object can be held close in to the body to 
minimize back stress. Experience has suggested that 
lifting weights greater than 25 pounds requires caution 
and careful assessment of whether this is within the 
client's limits. 

If psychosocial assessment suggests that psycho- 
logical stress is an aggravating factor in the client's 
back problem, it is important to identify potential 
sources of stress in the client's job, and to reduce or 
eliminate them. Productivity requirements, personality 
clashes, and less esthetic physical environments are 
examples of stress -provoking issues to consider. For 
some clients, it may well be that these stress factors, 
rather than the physical requirements of the job, may 
govern the choice amongst various employment 
possibilities. 

The following additional guidelines should help the 
counselor in vocational planning with back pain 
clients: 

1. For clients with back disorders, the physical 
demands of employment will usually have to be 
reduced, and more sedentary employment sought. 

2. The counselor should assume that the client will 
be less able to handle acute and chronic physical 
stresses as well as he did prior to the onset of his back 
problem, even after optimal treatment. 

3. Educational or vocational retraining into less 
physically strenuous occupations is indicated, 
particularly for clients in the younger age groups, if 
such retraining is consistent with the client's interests, 
aptitudes, intellect, and personality. 

4. After a prolonged period of unemployment, a 
gradual reintroduction of employment through 
successive experiences in job stations, sheltered 
workshops, and on-the-job training is more likely to 
lead to successful job placement. Through this process, 
the client is able to build up his confidence and his 
physical endurance before re-entering the competitive 
job market. 

5. The client will attain his maximum strength, 
endurance, flexibility, and skill in body mechanic 
techniques from a regular physical therapy program 
within 3 months. The counselor should recognize that 
additional physical therapy is not likely to increase the 



client's function after the completion of such a 
remobilization program. Improvement in functional 
level can, however, be anticipated if a physical therapy 
program is established with a client who has not had 
adequate physical therapy previously. Further, the 
counselor should ascertain whether the client requires a 
continued home exercise program to maintain his 
functional level and whether the exercises are being 
performed. Clients who are not regularly performing 
their prescribed home program can also be expected to 
improve with the resumption of exercises. 

6. Any medical treatment plan which includes 
surgery and is expected to improve the client's work 
potential should be delayed until psychological, 
psychiatric, and social work evaluations have been 
performed. Surgery is not likely to produce the 
expected improvement if psychosocial issues 
contributing significantly to the client's disability are 
not first resolved or concurrently managed. 

7 . The counselor should pay particular attention to 
whether the client's back pain is primarily related to 
static or kinetic activity, and consider employment 
possibilities accordingly. Further, jobs requiring 
frequent changes of position are generally beneficial, 
while those requiring maintenance of the same 
position for long time periods are generally 
detrimental. 

CONCLUSION 

The client with chronic back pain poses a unique 
and difficult challenge to the counselor. The disease 
component is often nebulous, the visibility of the 
handicap is relatively small when compared to such 
conditions as spinal cord injury and amputation, and 
yet the disability is quite profound. The counselor able 
to successfully return his back pain client to work and 
sustain such employment has indeed achieved a 
success. 



BIBLIOGRAPHY 

Beals RR, Hickman NW: Industrial injuries of the back and 
extremities. J Bone Joint Surg 54A:1593-161 1 , 1972. 
This study of 180 industrially injured men compares those 
with extremity injuries to those with back injuries. All 
received comprehensive evaluation. The relation to return 
to work of such factors as duration of unemployment, 
number of operations, and psychosocial problems, and the 
predictive value of the MMPI are discussed. Documents 
the importance of psychological evaluation for optimum 
results. 

Calliet R: Low Back Pain Syndrome. Ed 2. Phila- 
delphia, Davis, 1968. 

Covers causes, evaluation, and treatment of low back pain 
in greater detail than this chapter. Includes an illustrated 
chapter on therapeutic exercises which the counselor may 
find helpful 



Back Disorders 21 7 



Kokan PJ, Wing PC, Thompson WJ: Factors associated with 
failure of lumbar spine fusion. Can J Surg 17:294-298, 
1974. 

Reviews medical, vocational, psychological, and economic 
factors associated with success and failure of spinal fusion. 
Success is defined relative to postoperative disability, 
rather than to stability of the fusion. 

McNab I: Backache. Baltimore, Williams & Wilkins, 
1977. 

Although written for physicians, this book is not too 
technical for the counselor. Clearly presents some of the 
causes of low back pain and discusses the management of 
several syndromes. Useful for counselors desiring more 
detail than this chapter. 

Rosenthal AM: Rehabilitation of the patient with chronic 
low back pain. Ill Med J 146:189-190 and 223, 1974. 
Short, concise article describing a treatment program for 
chronic low back pain. Emphasizes the need for evaluation 
and treatment of psychological, social, and vocational 
problems in addition to the medical problem. 

Westrin CG, Hirsch C, Lindegard B: The personality of the 
back patient. Clin Orthop 87:209-216, 1972. 
Description of a study of patients who were absent from 
work for at least 8 days during the year 1964 due to low 
back pain. Using matched controls, factors which might be 
causes of absenteeism were identified. 

Wilfling FJ, Klonoff H, Kokan P: Psychological, 
demographic and orthopaedic factors associated with 
prediction of outcome of spinal fusion. Clin Orthop 
90:153-160, 1973. 

A study of 26 male chronic back pain patients. One group 
had a single previous spinal fusion and a second group had 
multiple previous fusions. Identifies factors differentiating 
patients with good, fair, and poor surgical outcome. 
Supports previous research indicating the value of the 
MMPI in predicting surgical outcome. 



16 CHRONIC PAIN 



Wilbert E. Fordyce, Ph.D. 



DISEASE DESCRIPTION 

Chronic pain cannot be approached in quite the 
same way as other disabilities described in this text 
because it does not respect the usual body boundaries 
evolved by medical practitioners to support 
specialization. The site of the pain and the 
circumstances which first caused it are often of 
secondary importance and have diminishing relevance 
after the problem has persisted for months or years 
(i.e., has become chronic). 

The distinction between acute and chronic pain 
must be made clear. Acute pain, also called time- 
limited or recent -onset pain, represents the pain 
prototype most often written about and studied, in 
which a known or approximately known noxious 
stimulus is applied for a time- limited period. In 
experimental pain studies, the stimulus can be 
measured precisely and is applied for limited and 
clearly defined time intervals. Pain from a wound, a 
bruise, a fracture, or an infected tooth are examples of 
typical acute pain. 

One of the differences between chronic pain and 
acute pain is that, in the case of chronic pain, the 
noxious stimulus is typically not observed or precisely 
known, but may only be inferred from previous medical 
examinations, without direct confirmation that those 
findings are currently valid. Also, the individual has 
little basis for estimating when, or even whether, the 
pain problem and associated disruption of his life will 
end. Finally, chronicity ensures that learning or 
conditioning effects will have had an opportunity to 
become established and to complicate an 
understanding of the problem. In this chapter, the 
term "chronic pain" represents a set of problems 
involving a complex interaction of organic or physical 
factors, environmental influences, and psychological 
factors, principally learning or conditioning. 

Another way of describing pain problems is to make 
a functional distinction between respondent pain and 
operant pain. Respondents are actions of an organism 
which are reflexive in nature. If an adequate stimulus 
occurs, the response will automatically follow. 
Respondents are controlled by antecedent stimuli, and 
usually involve glandular or smooth muscle functions 



of the body. Operants, on the other hand, are responses 
that are under voluntary control. Operants may also be 
elicited by antecedent stimuli, but they are influenced 
by the consequences of the response. Therefore, 
operants can come under the control of those 
consequences. Operants mainly involve striated or 
voluntary muscles (e.g. , speech, body movements, and 
facial expressions). 

Neurophysiologically, the distinction between 
operants and respondents is becoming less important. 
Work in recent years on automatic or glandular 
conditioning has demonstrated that many actions that 
were previously classified as respondent can be 
conditioned, and therefore have the functional 
characteristics of operants. However, making a 
distinction between problems of respondent pain and 
operant pain is useful in determining the appropriate 
approach to the problem. When the expression of pain 
is found to be influenced or controlled to a significant 
degree by environmental consequences, the problem is 
one of operant pain and the solution to the problem must 
necessarily consider the role of these environmental 
consequences. In contrast, expressions of pain found to 
be influenced or controlled by antecedent 
environmental stimuli reflect respondent pain. 

Through careful evaluation and analysis, a patient's 
pain can generally be classified as either predominantly 
respondent or predominantly operant in nature. The 
distinction can only be made after carefully assessing 
information relating to both body damage factors and 
the environmental consequences of the patient's 
expressions of pain. Using the terms "respondent" 
and "operant" pain avoids the issue of whether a 
patient's pain is "reaT'or "imaginary." Pain is what 
is felt and reported by the patient. The proper question 
is not whether the pain is real, but rather what factors 
influence it. 



Respondent Pain Model 

Traditionally, chronic pain has been viewed from 
the perspective of a disease or medical model. During 
the diagnostic process, it is usually assumed that the 
indicators of the pain problem, the symptoms, occur 



219 



220 Fordvce 



because of some underlying body damage process. In 
other words, it is assumed that the problem is one of 
respondent pain. Further, it is assumed that if the body 
damage factors were eliminated, the pain would cease. 
This conceptual model works well with most problems 
of acute pain, but its effectiveness diminishes as time 
passes and the pain becomes chronic. 

There are, however, some chronic pain problems 
that may respond to medical treatment and a 
traditional approach to pain problems. These include 
headaches, limb or extremity pain, phantom limb 
pain, and pain associated with spinal cord injury. 

Headaches . When headaches are sufficiently severe 
to immobilize a person and occur frequently enough to 
make a major difference in routine activities, such as 
employment, a significant functional impairment 
results. Headaches tend to fall into four subgroups. 
The first is associated with the growth of tumors in the 
cranial cavity. Individuals with this type of headache 
are rarely candidates for rehabilitation, either because 
the tumors are untreatable and have a dire prognosis 
or because they have been successfully treated and 
leave no residual pain problems. 

The second subgroup is called cluster headaches 
because the headaches occur episodically but in groups 
(e.g., several over consecutive days, followed by weeks 
with no occurrence). If medication can alleviate the 
problem, there is no functional impairment. Patients 
with cluster headaches rarely appear as candidates for 
vocational rehabilitation. 

Migraine or vascular headaches make up the third 
subgroup. They may occur at any time, often 
awakening the person from a sound sleep, but they are 
more likely to occur following an emotional crisis. 
These headaches are often preceded by a signal, or 
"aura," such as visual blurring or spots before the 
eyes. They tend to be asymmetrical in pain 
distribution, and the individual's hands are usually 
cold. The present treatment for migraine headaches is 
generally medication, biofeedback, or a combination 
of the two. The combination has a better than 50 
percent chance of reducing the problem to manageable 
levels. In many cases, migraine headaches are so 
infrequent that they do not cause a vocational 
problem. There may, however, be a momentary 
problem if an individual with infrequent headaches 
does have one while at work. 

The final and most common subgroup is tension- 
headaches. They tend to be symmetrical, involving 
both sides of the head and/or neck. Tension headaches 
tend to occur during the day, particularly in the later 
afternoon after tensions have accumulated for a 
number of hours. Muscle relaxants are often helpful. 
In recent years, with the advent of biofeedback and 
other forms of relaxation training, the symptoms often 
can be brought under effective control. When they 
cannot, the person usually is highly vulnerable to 
emotional upset, with very limited capability for 



coping with stress. Often when these treatment 
methods fail to resolve or control the problem, the 
person is also found to have a psychiatric problem. 

A brief mention should be made of facial pain. Aside 
from dental problems, facial pain tends to take the 
form of either tic douloureux or so-called atypical 
facial pain. Neurosurgical treatment methods for tic 
douloureux are effective in about 80 percent of cases. 
For the remaining 20 percent of patients, there is little 
likelihood of their being able to work at all. The 
atypical facial pain problems can be considered with 
chronic pain in general. 

Limb pain. Chronic pain problems which stem from 
injuries to the upper and lower extremities are usually 
found to be respondent in nature. Even late in the 
problem's history, the pain is substantially more 
influenced by body damage factors than conditioning 
or environmental factors. Elbow, wrist, hand, knee, 
ankle, or foot pain problems which interfere 
significantly with activity "cost" the individual a great 
deal, for nearly every self- care act can be hindered. 
Most persons push to restore function and activity once 
the injured limb begins to heal. Therefore, those who 
come to vocational rehabilitation programs or pain 
evaluation centers are likely to have impairments that 
are respondent, rather than operant, in nature. 

The major exceptions to this are (a) cases in which 
careful evaluation of the functional impairment 
reported by the patient reveals inconsistencies (e.g., 
the patient cannot grasp a pair of pliers but can hold a 
full stein of beer); and (b) cases in which the 
"impairment" is primarily a visual display of pain 
other than inactivity (e.g., the patient walks with a 
pronounced limp, signaling to his environment that he 
has pain, but nonetheless moves around and does 
things). In either of these instances, an operant 
component is likely and a detailed behavioral analysis 
will be necessary. 

It is more difficult to generalize about shoulder pain. 
Many shoulder pain problems originate with body 
trauma and persist because of associated muscle 
tension. The individual's tendency to guard against 
movement by tensing the involved shoulder may make 
the problem worse. In such cases, biofeedback 
relaxation training may significantly reduce or resolve 
the problem. 

Phantom pain. Following amputation of a limb or 
part of a limb, phantom sensations of the missing part 
are very common. These sensations may persist 
indefinitely to some degree, although intensity is likely 
to diminish with time. Not all phantom sensations are 
painful; phantom sensations and phantom limb pain 
should not be confused (see chap. 13, p. 186). 

Fitting an amputee with a prosthetic limb very soon 
after surgery markedly reduces the frequency, 
intensity, and duration of phantom pain. If persistent 
and interfering phantom limb pain does occur, the 
treatment of choice is chemotherapy, such as Dilantin 



Chronic Pain 



221 



or Tegretol. Phantom limb pain is rarely a problem of 
lasting significance in vocational rehabilitation. 

Pain associated with spinal cord injury. Two 
different forms of pain are often reported by spinal 
cord injured persons. The first is a sharp, burning pain 
which occurs episodically and often lasts only a few 
seconds. These episodes usually occur several times a 
day, although several days may pass without any 
occurrence. This form of post -injury pain seems 
similar to an epileptic seizure. Again Dilantin or 
Tegretol is the medication of choice and often 
adequately controls the problem. 

The second type of pain is a deep, dull aching pain 
which tends to persist for hours, days, weeks, or even 
months, and generally increases with activity. This 
type appears to function more like operant pain and 
should be treated in the same way as other operant 
pain problems (see below). 

It should be emphasized here that these respondent 
pain problems should not be confused with those 
associated with chronic systemic illness, such as 
rheumatoid arthritis or degenerative joint disease. 
•However, the extent to which the functional 
impairment displayed seems warranted by the current 
state of the disease should be considered. 

Treatment of chronic pain based on an analysis of 
the patient's problem by the medical/disease model 
often fails. When this happens, the physician 
committed to that model is in a dilemma, for he has 
few options. In most situations, he will simply refer the 
patient to another physician. However, the second 
physician is likely to apply the same conceptual model, 
and unless the first physician made an error or the 
second has some special skill or method unknown to 
the first, it is unlikely that the referral will lead to a 
successful "cure." 

The physician's second option is to repeat the 
therapy which originally failed. Often a second surgery 
or a second course of such conservative management 
techniques as heat, massage, traction, or an alternation 
of these treatment modalities is attempted. Although 
sometimes successful, these techniques will likely have 
been tried repeatedly on most chronic pain patients 
without major changes in either self- reports of pain or 
functional ability. 

A third option, not generally used until other 
therapies have been tried and found unsuccessful, is to 
infer that the pain problem is not real. This is often 
communicated to the patient by telling him the 
problem is "all in his head." This option may lead to 
one or more responses. 

One response is to label the pain problem 
"psychogenic," which implies that the pain symptoms 
are influenced by some personality problem. The 
patient may then be referred for psychotherapy. 
Patients often reject this recommendation. If they 
accept, it is generally with reluctance and their 
participation in the therapy process is unenthusiastic. 



Even if psychotherapy is accepted and followed 
diligently, it is indeed unusual to find a chronic pain 
patient whose problem is resolved by this treatment. 

The most common response of physicians who have 
inferred that the patient's pain is imaginary when 
traditional treatment methods have failed to solve the 
problem is to tell the patient there is nothing more he 
can do. The patient is then given a medication regimen 
of renewable prescriptions, some recommendations 
regarding rest and exercise, and told he is going to 
have to go home and learn to live with the pain. The 
intent is to reduce fruitless pursuit of new diagnostic 
and treatment interventions, but it tends to have the 
opposite effect. Challenging the authenticity of the 
patient's pain in this way often serves to increase, 
rather than decrease, his use of health care providers 
because he is placed in the position of having to prove 
that he really hurts. 

Persisting with traditional approaches to chronic 
pain occurs, therefore, primarily because physicians 
find themselves with few alternatives. There is, 
however, another way of considering a chronic pain 
problem, which is to deal with those behaviors of the 
patient which indicate to others that he is in pain. This 
approach to the problem of chronic pain may be called 
an operant pain model, in contrast to the respondent 
pain model traditionally used. 

Operant Pain Model 

There are persistent and repeated signals from the 
chronic pain patient to the environment that he has a 
pain problem. Such signals include grimacing, 
moaning, gasping, limping, giving verbal descriptions 
of pain, moving in a guarded manner, or spending 
much of the day reclining. Such signals, or pain 
behaviors, are operants and subject to the same 
influences as any other behavior. 

Pain behaviors rarely are controlled exclusively by 
body damage factors (respondent pain), with no 
influence from environmental consequences (operant 
pain). There is now considerable evidence to indicate 
that pain behaviors which began as a response to a 
body damage stimulus often persist because of the 
influence of environmental consequences. 

A positive consequence which systematically follows 
a particular behavior and which is contingent on that 
behavior is a reinjorcer. Behaviors tend to recur more 
frequently or consistently if there is some prospect of 
again achieving a reinforcer. Conversely, a behavior 
which has been occurring but is no longer reinforced 
will begin to fade and ultimately will disappear. This 
process is known as extinction. 

This is not to suggest that simple, or even complex, 
changes in environmental consequences automatically 
have immediate and unlimited impact on behavior. 
The relationships between the human organism, the 
learning process, and the residual effects of prior 



222 Fordvce 



learning are too complex for this to be the case. 
However, persons are sensitive to consequences and 
their behavior does show change under the impact of 
systematic and persisting modification of environ- 
mental consequences. 

Conditioning plays a role in the establishment of 
pain behaviors in three basic ways. First, pain 
behaviors may be directly reinforced. For example, a pain 
patient displaying distress may receive special 
attention which would not occur otherwise. The special 
attention is contingent upon display of pain behaviors. 

A second and probably more important source of 
conditioning involves avoidance conditioning. When an 
individual is faced with an aversive or noxious event, 
he takes action to avoid it, if possible. Consider what 
may happen to a pain patient with a sore back. He 
reclines to rest it, thereby avoiding the pain which 
would occur if he went to work. Suppose that this 
person had a poor job or had recently been promoted 
beyond his competence and was anxious about keeping 
his new job. Staying home from work is reinforced not 
only by avoiding pain but also by avoiding the stress or 
anxiety caused by being at work. Either source of 
reinforcement is capable of effectively strengthening 
the pain behaviors. 

For the majority of chronic pain patients, their pain 
behaviors not only guard against or reduce pain but 
also avoid other noxious events. When the body 
damage factor which originally triggered the pain heals 
or is effectively treated, the avoidance behaviors are no 
longer being reinforced by avoiding pain. Most pain 
behaviors are awkward and cumbersome and thus are 
themselves somewhat aversive to the patient. 
However, if the alternatives to being sick are more 
aversive, the pain behaviors would continue to be 
effectively reinforced by the avoidance of these 
aversive alternatives. If being sick or impaired with 
pain does not result in other hardships for the person, 
the risk of the avoidance pattern persisting is even 
higher. For example, if disability compensation 
benefits maintain an adequate level of income, the pain 
problem will probably be less aversive. If, in addition, 
the patient avoids returning to an aversive job by 
continuing to demonstrate evidence of persisting pain, 
these behaviors would be further strengthened. 

There is a third and less important way in which 
conditioning may play a role. Some patients find that 
being more active is directly punished. Family members 
may insist that the patient take it easy to avoid making 
the pain worse, and physicians may prescribe rest for 
longer than is actually necessary. 

Two additional observations may substantiate the 
importance of conditioning and learning factors in 
chronic pain. One almost never finds young persons 
under age 22 with chronic pain, except those affected 
by a disease process such as juvenile arthritis. They are 
too active, energetic, and involved in things requiring 
action and mobility to be immobilized by pain for 



more than a few days. Unless complications set in, a 
sprained back or ankle usually heals quickly in this age 
group, after which normal activity levels are resumed. 
The probability of rapid recovery and resumption of 
normal activity begins to diminish as age advances. 

One might also consider the professional football 
player's response to pain. During the course of a game, 
there are stimuli far exceeding those sufficient to 
produce severe pain under other circumstances. But 
the player gets up, shakes it off, and continues to play. 
There appear to be three reasons why this occurs. 
Neurological and biochemical mechanisms 
undoubtedly play a short-term role. An ingenious 
neurological theory, the Gate Control Theory, 
contends that the nervous system is capable of 
generating nerve impulses from the brain 
"downstream" on the spinal cord. These impulses 
have the effect of partially or completely closing the 
"gate" of the brain, so that pain impulses arising from 
the site of body damage cannot get through to the brain 
or get through only in a reduced amount. More recent 
biochemical research indicates that the brain is capable 
of releasing substances which act much like morphine 
to reduce the pain experience. These mechanisms, 
however, are unlikely to persist through a full game, 
much less over a season. A second factor is that the 
player has had years of experience and prior 
conditioning against the display of pain behaviors. 
Third, pain behaviors would "cost" too much — he 
would not be able to play if he repeatedly signalled that 
he was experiencing severe pain. 

Examination of some of the failures of the traditional 
medical (respondent pain) model for approaching 
problems of chronic pain will further highlight the 
principles of conditioning underlying the behavioral 
approach to this problem. One such failure involves 
medication. Medical students learn that pain 
medications carry the hazard of addiction or 
habituation. The traditional safeguard is to require 
patients to limit their use of pain medications to only 
those times when they are needed; that is, the patient 
must hurt before getting the medication. This is 
written on a prescription as p.r.n., "take only as 
needed." The p.r.n. regimen seems sensible, but it 
often fails. Experience in clinics specializing in chronic 
pain problems suggests that nearly 50 percent of clinic 
patients give evidence of addiction or habituation to 
pain medications. Nearly all of them have been on 
p.r.n. regimens. 

Examining the p.r.n. plan from a behavioral or 
conditioning viewpoint suggests a possible explanation 
for the high addiction/habituation rate. The p.r.n. plan 
makes medication pain contingent. The patient does 
something, hurts, and then requests medication. The 
activity -hurting sequence is systematically followed by 
a particular consequence: medication. If the 
medication makes the patient feel better, it could be 
considered a reinforcement for pain. This is likely to 



Chronic Pain 223 



make the pain behaviors, which signal the presence of 
pain and the need for medication, to become 
conditioned. Clinical experience indicates that this is 
often the case. 

Traditional methods for handling rest and exercise 
also present problems. Whether by formal prescription 
or their own devices, pain patients for whom a specific 
exercise or physical activity makes the pain worse tend 
to fall into a "work -to -tolerance" pattern. They will 
engage in an activity or exercise until pain begins or 
reaches an intensity they no longer wish to tolerate, 
then stop to rest. Activity or exercise is systematically 
followed by expressions of pain, which are in turn 
systematically and contingently followed by rest, 
avoidance of further work, and the associated 
beneficial results. As with pain - contingent 
medications, pain -contingent rest is likely to 
strengthen or increase (i.e., condition) the pain 
behavior which leads to the reinforcer. 

FUNCTIONAL DISABILITIES 

The functional disabilities associated with chronic 
pain vary considerably from patient to patient. They 
may be physical and/or psychosocial, and are caused 
by the pain behaviors which limit activity or lead 
others to encourage activity restriction. 

Physical Disabilities 

Many pain behaviors interfere with physical 
movement, vigor, and speed. For example, 
ambulation may be impaired by a limp, or the distance 
a patient can walk may be limited by the need to rest 
frequently. Pain behaviors may limit both the extent 
and duration of body motions, such as lifting, bending, 
twisting, and reaching. In addition, actions guarding 
against pain or the residual effects of surgeries to 
alleviate the problem may result in selective 
limitations. A person with a back fusion, for example, 
may not be impaired by pain but may be mechanically 
restricted in twisting or bending. 

Pain medications significantly interfere with 
alertness or body control, and may cause dizziness, 
changes in intellectual and cognitive function, or 
lethargy. Judgment and memory may also be affected. 
These in turn may affect ambulation and coordination, 
and may impair reading, writing, driving, and fine 
motor skills. 

Psychosocial Disabilities 

The communication of pain, either verbally or 
nonverbally by movements or facial expression, is 
likely to elicit oversolicitous responses from family 
members, co-workers, and others. This reaction may 
reinforce the pain behavior and cause activity to be 
limited more than necessary. The patient's spouse or 



other family members may provide much pain- 
contingent support or attention for pain behaviors, 
encourage the use of medication, or discourage the 
patient's attempts to become more active. Spouses 
may also be strongly supportive of efforts to maintain 
compensation income in lieu of wages. 

Chronic pain patients may be unnecessarily inactive 
because of fear of incurring additional body damage or 
pain. If continued activity increased the patient's pain 
early in the history of his pain problem, he may 
continue to fear activity as a source of pain even when 
this is no longer necessarily true. 

Chronic pain patients are often depressed. 
Sometimes the patient's problem is really depression 
rather than pain, but it is often difficult for the patient 
or those who observe and examine him to differentiate 
between the two. For example, one often finds a 
chronic pain patient with markedly lowered activity 
level, allegedly because physical movement results in 
increased pain, who displays greatly increased activity 
level and tolerance when the only treatment has been 
antidepressant medications. Thus, sometimes a 
problem is mislabeled pain when it actually is 
depression. 

Many chronic pain patients have other problems 
which would give them much distress if they were not 
limited by pain. The first -ranking functional 
disabilities are the pain behaviors and the immediate 
impairments associated with them, but the functional 
impairments characterized by limited ability to be 
effectively well are not less important. This relates to 
avoidance learning and corresponds to what is often 
termed "secondary gain." For example, some workers 
disabled by pain and receiving wage -replacement 
compensation find those funds sufficient reinforce- 
ment to maintain their pain behaviors, particularly 
when return to work might be aversive because of poor 
job skills, conflict with their employers, or aversive 
change in job responsibilities. In effect, being sick does 
not "cost" enough to offset the reinforcement from 
avoiding the aversive consequences of work. 

Another source of aversive stimuli which may 
reinforce pain behaviors is the existence of another 
disability more aversive to the patient than pain. For 
example, some older pain patients are found to have 
memory deficits associated with minor strokes or 
arteriosclerosis. Were they not disabled by "pain," 
they would risk revealing intellectual deterioration. 
Similarly, patients with multiple sclerosis or spinal 
cord injury often will attribute their limited 
performance ability to pain when the real problem is 
that they find being seen in public in a wheelchair 
aversive. "Pain" rationalizes their immobilization. 

Rehabilitation Potential 

The presence of pain or a pain problem by itself 
often has little direct influence on rehabilitation 



224 Fordyce 



potential, which is determined primarily by what each 
person does about the pain. The kind of action taken 
when pain arises varies among individuals and affects 
the level of physical disability. Some persons get up, 
move around, and change body position when they feel 
pain. They tend to have high activity levels and are 
therefore called "pacers." Usually they have either 
tension headaches or pain problems in the high back, 
neck, shoulder, and arms. Most pacers maintain their 
usual activity patterns, and do not have functional 
disabilities. These patients are not likely to require 
vocational rehabilitation services. 

Persons who rest when pain arises are called 
"recliners." They tend to have low activity levels and 
more functional impairments. Their pain is more 
likely to be located in the low back or abdomen. There 
is usually a marked difference in the kinds and 
amounts of physical work recliners can and will do 
compared with pacers. Recliners will require treatment 
to increase their activity level. They are also more 
likely to require vocational rehabilitation services. 

Assuming that treatment has restored an optimal 
level and range of activity, or that evaluation indicates 
that such a restoration is feasible, the potential for 
rehabilitation is likely to be influenced mainly by 
avoidance learning factors. Restoration of activity level 
with treatment and reduction of the various pain 
behaviors will not suffice if personality functioning is 
not also brought to a level compatible with job 
demands. One must ask what the person would be 
required to do if he were not limited by pain and then 
assess the extent to which he could cope with the 
situation. 

Not all pain patients, even though effectively 
treated, can return to their previous level of 
employment. This is generally due to inconsistencies 
between the basic characteristics and strengths of the 
individual and the specific demands and requirements 
of the job. Once pain behaviors have been minimized, 
care should be taken not to reinstate the individual into 
the environment which may partially have been 
responsible for the initial maintenance of those 
behaviors. 



STANDARDS OF EVALUATION 

The proper evaluation of chronic pain can rarely be 
adequately accomplished solely by a physician's 
examination. By the time a pain problem has become 
chronic, it is rarely merely a disease or respondent pain 
problem which requires only the identification of the 
body -damage factor stimulating the pain behaviors. 
Both medical and nonmedical factors need to be 
evaluated. Therefore, evaluation of long-standing 
pain problems usually is best accomplished in a facility 
using a multidisciplinary approach. Further, it is 
essential that an evaluation unit be organized to ensure 



there is adequate communication among those 
contributing to the evaluation process. If they do not 
communicate with each other, the evaluation is likely 
to reflect only the potentially narrow perspective of the 
dominant figure in the evaluation team. 

Some chronic pain patients are essentially 
intoxicated from pain medications. Effective 
evaluation of the pain problem cannot be carried out 
under these conditions. The pain evaluation unit 
should implement a rapid detoxifying regimen for such 
patients so that communications are clear and 
evaluation can proceed. Methods for accomplishing 
this can be found in the professional literature. Pain 
evaluation units which do not have access to inpatient 
facilities will be unable to deal adequately with 
evaluation of the toxic patient. 

Medical evaluation will require a specialist trained 
to assess the body system or parts involved. For 
example, an orthopedic surgeon or physiatrist should 
evaluate back or limb pain and an internist should 
evaluate abdominal pain. 

The frequency with which tension plays a role in 
pain indicates that a comprehensive evaluation unit 
should also have a biofeedback component to assess 
this factor. Tension headaches and the muscle tension 
pain problems often associated with neck, shoulder, 
and high back pain should be examined via 
biofeedback trials, using the EMG mode. 

Some pain evaluation programs attempt to evaluate 
nonmedical factors solely by use of the Minnesota 
Multiphasic Personality Inventory (MMPI). Studies 
have been published alleging that the MMPI 
adequately identifies who will or will not profit from 
surgery for pain, but other studies fail to support this. 
Extensive clinical experience with hundreds of chronic 
pain patients makes it apparent that there are too many 
contributing factors for any single device to be 
considered as an adequate appraisal tool. The MMPI 
should be used, as it is very helpful in gaining an 
understanding of the personality factors which 
influence the individual and will also identify the 
severity of a patient's depression. However, the 
MMPI needs to be analyzed by persons expert in its 
use. There are computer-based memory banks from 
which MMPI interpretations can be obtained, 
although most of these systems are based upon, and 
address themselves to, psychiatric problems. 
Unfortunately these are only sometimes relevant in 
evaluation of chronic pain. Therefore, availability of 
staff experienced in using the MMPI with chronic pain 
patients is helpful. 

Adequate evaluation of nonmedical aspects requires 
careful consideration of the patient's total situation. 
This can be broken down into two parts: (a) What 
good things happen if there is pain behavior? This 
refers to the direct positive reinforcement of pain 
behaviors, (b) What bad things do not happen if there 



Chronic Pain 225 



is pain behavior? This refers to the avoidance learning 
issue. 

Examination of the direct reinforcers for pain 
behavior requires assessment of previously prescribed 
medication and rest/exercise regimens to determine 
whether they have been producing physician -induced 
problems. Further, the family's response to the 
patient's pain behaviors and the compensation/ 
litigation issues should be assessed. This evaluation 
should be performed by a person skilled in interview- 
ing and sensitive to the special problems of chronic 
pain, such as a social worker, psychiatrist, or 
psychologist. 

Examination of avoidance learning factors is more 
complex and usually will require the expertise of an 
experienced psychiatrist or psychologist. The examiner 
needs to be aware that when a pain problem (i.e., the 
expression of pain behaviors) cannot be adequately 
accounted for on the basis of clearly identified and 
currently active physical findings, the alternative is not 
simply some form of mental illness, personality 
problem, or motivation problem. Each of those may be 
playing a role, perhaps even a dominant one but they 
also may not. 

Evaluation of chronic pain should include gathering 
information about actual activity levels: what activities 
are engaged in, how often, for how long, and at what 
rate or speed. Although it is generally not practical to 
obtain direct observations, careful interviewing of the 
patient and spouse can often help to pinpoint how 
much activity is possible and how much impairment 
exists. A pain evaluation which uses only a brief 
statement by the patient regarding what he can and 
cannot do is not likely to be accurate or effective 
because there is often a discrepancy between what the 
patient says and what he does. A "diary" to record 
when the person sits, stands, walks, or reclines, 
completed by patients prior to evaluation, is much 
more useful. 

TOTAL TREATMENT 

In keeping with the conceptual model used thus far, 
the approach to describing treatment of chronic pain 
will consider the behaviors that need to be changed. 
Although not all of the treatment methods are 
necessarily behavioral in nature, treatment objectives 
can only be discussed in terms of the effects on 
behavior they are intended to produce. 

If the pain problem is largely respondent in nature, 
with the pain behaviors predominantly under the 
control of currently active body damage factors, 
traditional therapies will presumably have been tried 
and found inadequate. It is possible, but not likely, 
that the problem may still be responsive to medical 
intervention and that the medical system failed to 
properly diagnose the problem. If, after compre- 



hensive evaluation, a physician is prepared to 
assert confidently that there is a definitive treatment 
available with a high likelihood of a positive outcome, 
then that course should receive serious consideration 
and be followed if it has not been tried previously with 
the patient and found inadequate. 

In most cases, however, additional traditional 
medical intervention is not likely to be fruitful. 
Furthermore, pain behaviors which were originally 
elicited by chronic systemic disease, such as 
rheumatoid arthritis or degenerative joint disease, are 
still pain behaviors and, as such, may be influenced by 
partial or substantial control of the environmental 
consequences of the behaviors. Thus, even when the 
problem is largely respondent in nature, treatment 
should address itself to the same set of issues discussed 
below that must be faced when the pain problem has 
significant operant elements. 

Activity Restriction 

Restriction in body movement, position, or range of 
motion of a limb should be treated with physical 
therapy and/or occupational therapy. Guided, 
selectively prescribed exercise has several objectives. 
First, it is generally important that reduced activity 
levels be increased to a point where re -employment is 
feasible. Reduced strength and stamina due to disuse 
of a limb or body parts will need to be restored. 
Secondly, as discussed before, many pain patients 
continue to regard the pain they experience upon 
moving a limb or body part as a warning signal which 
should be responded to with rest to prevent increased 
pain and further body damage. The patient now needs 
to be taught that it is safe to move about and increase 
activity without incurring future pain and body 
damage. Graduated exercise, starting at low levels and 
systematically increasing, will accomplish this. A third 
function of exercise is to help the patient's family 
realize that activity will not prove harmful so that they 
can provide support and additional encouragement for 
increased activity. 

Procedures for programming exercise plans for 
chronic pain patients are now well established. First, 
current exercise tolerance is established through a 
series of observed exercise trials (baseline tolerance). 
This information is then used by the patient and 
therapist together to set goals or quotas. The patient 
initially does less work than that observed in the 
baseline tolerance sessions and is rewarded by rest, 
therapist attention, and effective avoidance of pain. 
The quotas are then gradually and systematically 
raised by, for example, an increment of one repetition 
of each exercise for each treatment session. By working 
to quota, rest, attention, and the avoidance of pain 
become work contingent rather than pain contingent. 
The patient learns to stop when a certain amount of 



226 Fordyce 



activity is completed, independently of how he feels. 

Medication Toxicity 

Toxicity or intoxication from narcotics and other 
pain medications must be eliminated in order to 
proceed with evaluation and/or treatment. This is most 
easily accomplished by the so-called "pain -cocktail" 
method, in which all medications are shifted to oral 
form. Narcotics, if used, are changed from rapid- to 
slow -acting types. Methadone is usually the preferred 
substitute. All other analgesics are shifted to 
phenobarbiturate equivalents and tranquilizers are 
eliminated. The ensuing mix is incorporated into a 
color- and taste-masking vehicle and taken everv six 
hours. Active ingredients may then be systematically 
reduced at a rate of approximately 10 percent each day 
until medication is low enough to allow precise patient 
evaluation or treatment. 

This detoxifying method is usually too rapid to 
permit a lasting deconditioning of long-established 
drug habits. If there is evidence of addiction or 
habituation, a more gradual form of withdrawal, 
continuing with the pain cocktail format, is indicated. 
When the patient enters treatment following the 
evaluation phase, the active ingredients are restored to 
a level providing comfortable coverage for the pain. 
Still delivered in the color- and taste -masking vehicle 
(usually cherry syrup), the active ingredients are now 
reduced approximately 10 percent every 7 to 10 days. 
The slower rate provides sufficient time for activity 
level to build up and for the patient to learn he can 
indeed handle movement without dire consequences. 
The process should always be carefully explained to 
patients at the outset of the program. 

Overt Pain Behaviors 

Patients need to be helped to eliminate endless 
complaints of pain by word, gesture, or facial 
expression. This usually can be best handled by (a) 
ensuring that treatment staff are nonresponsive to 
signals of pain by avoiding reassuring or comforting 
comments; and (b) by being responsive to effective 
effort and activity. This part of the treatment is 
explained to the patient and family. It is usually simple 
for treatment personnel to adopt a posture of 
nonresponsiveness to pain behavior while providing 
positive attention to activity accomplishments. 

Family members of some pain patients are militantly 
protective and supportive of pain behaviors and ready 
to discourage or limit activity for fear more pain and 
body damage will be produced. It is usually essential to 
the success of treatment that they be helped to learn 
new ways of responding to the patient's pain 
behaviors. 

In addition, where there is a severe limp or a history 



of "knee buckling" which has been established as 
operant in character, it is important to help the patient 
eliminate such behaviors by systematic gait retraining. 
If these behaviors are not minimized, the display of 
such pain behaviors on a job is likely to elicit excessive 
concern or overprotection from fellow workers, 
thereby reinstituting social reinforcement of pain 
behaviors and making the pain problem again worsen. 

Depression 

In the context of operant pain, depression should not 
be seen as a form of mental illness for which 
psychotherapy is indicated, but simply as the natural 
result of a deprivation of reinforcers resulting from 
reduced activity level. Therefore, treatment of 
depression is fairly simple. If the depression is mild, 
re-establishing activity levels, resulting in increased 
access to work and play, may well alleviate it. If the 
depression is more severe, chemotherapy is also used. 
Antidepressants (usually Elavil and Sinequan) are 
added to the pain cocktail at levels sufficient to help 
"energize" the patient, and are reduced at a much 
slower rate than other elements. If there is real concern 
about the depression, reduction of the antidepressants 
should not start until the patient is actually re-engaged 
in the vocational and avocational activities which are 
the aim of treatment. 

"Avoidance" Problems 

Where it has been established that pain behaviors 
were being significantly supported by the "time out" 
they yielded from aversive events or activities, it is 
essential to reduce either those events or activities or 
their aversiveness to the patient. For example, a 
worker may be promoted or shifted to new job 
responsibilities he cannot handle or which produce 
much anxiety. The emergence of a pain problem has 
the side effect of helping to avoid the now- aversive job. 
Treatment for pain which restores activity level and 
reduces medication intake must also help the person 
find work he can handle effectively. The treatment 
plan is more likely to be successful and probably more 
cost-effective if the vocational counselor focuses on 
elements of the job (i.e., either altering the job 
demands or finding the client a more suitable job), 
rather than trying to alter the worker to fit the aversive 
job. 

Some chronic pain patients are found to be highly 
vulnerable to confusion and emotional upset, 
functioning in ways often termed "inadequate 
personality" or "borderline schizophrenic." Both 
terms are used to describe persons who are functioning 
marginally in their environments. Their pre -onset work 
histories are likely to be marked by marginal 
performance and frequent job failures. Restoration of 



Chronic Pain 227 



activity level and reduction of pain behaviors and 
medication intake will not likely yield enduring gains 
unless these marginally functioning persons are helped 
to find work with limited demands. 

"Avoidance" problems need to be identified, 
coordinated, and integrated with the overall pain 
treatment. If it is to be accomplished by referral to 
another facility or individual, planning for this and 
obtaining the client's approval must be done in the 
earliest stages of pain treatment, rather than at the 
end. The transfer must also be closely monitored to 
ensure that the new therapist is well aware of the main 
features of the just -completed pain treatment 
program. 



treatment with opportunities to practice the new 
activity level in work -related activities while still 
within the treatment program. This may occur in 
specially arranged situations in the hospital; in 
community facilities, such as sheltered workshops, 
where work -related activity levels can be practiced and 
strengthened; or by arrangement with the employer to 
provide graduated return to full work demands under 
the guidance of the treatment team. 

Effective pain treatment programs also work at 
generalization of activity gains through programmed 
weekend passes and recreational activities. These are 
important, but are not likely to be adequate substitutes 
for work -related rehearsal. 



Muscle Tension 

Muscle tension components of pain problems 
require specialized treatment. There are a number of 
techniques for training persons to relax, such as direct 
relaxation instruction, hypnosis, training in self- 
hypnosis, yoga, or transcendental meditation. The 
easiest and most effective technique for a wide variety 
of patients is EMG-mode biofeedback. Surface 
electrodes are placed on the skin above the hyperactive 
muscles. The amount of electrical activity in those 
muscles is then communicated back to the patient by a 
tone which varies in pitch with the intensity of muscle 
activity, by a needle moving on a dial, or by both. 
Using the feedback information, the patient then 
experiments with ways of reducing the tension or level 
of muscle activity until one is found that works for him. 

Biofeedback treatment for muscle tension is fairly 
effective with high back, neck, and shoulder pain, 
tension headaches, and the jaw pain associated with 
teeth clenching. For tension headaches, electrodes are 
usually attached to the trapezius muscles supporting 
the head or on the frontalis muscle on the forehead. 
For jaw pain, the electrodes are attached to the 
masseter muscle of the jaw, and for other muscle 
tension pain sites that can be clearly identified, the 
electrodes are positioned as appropriate. Biofeedback 
is rarely effective with low back pain. 

Generalization of Performance 

The objective of pain treatment is the restoration of 
performance. Although reduction of pain is usually 
one step along the way, it does not always lead to 
restored and maintained performance or activity. 
Reliance solely on physical therapy and occupational 
therapy exercise programs often is not sufficient. The 
increased activity level generated in an exercise 
program needs to be generalized from the treatment 
center to the world of work. 

Effective treatment of chronic pain usually requires 
linking increasing exercise level and tolerance in 



Other Treatment Modalities 

While chronic pain problems are only occasionally' 
alleviated by additional surgery or nerve blocks, there 
are three other treatment approaches which have 
gained a fair amount of attention and should be 
mentioned here. 

Transcutaneous stimulation (TCS). The basic 
concept of TCS is to provide electrical stimulation to 
nerve fibers. Although this is usually accomplished 
through the use of surface electrodes, the transmitter 
can be introduced into a nerve trunk by surgical 
means. Numerous studies have been carried out in 
what appears to be a fruitless effort to identify which 
pain problems and which patients will benefit. As 
many as 25 percent may report significant benefit from 
TCS or some variation, but the findings of various 
review studies are scattered and variable. Certain 
generalizations about this approach can be made: 

1. TCS, a noninvasive technique as distinguished 
from surgical implantation of transmitters, is not 
likely to be harmful. 

2. TCS is relatively inexpensive and therefore 
warrants at least an exploratory trial. 

3. Many patients report an initial benefit which 
subsequently declines, often to no benefit at all, 
after 2 to 6 weeks. A TCS device, therefore, 
probably should not be purchased until at least 6 
weeks of tryout have elapsed and there is some 
indication that it produces a discernible effect on 
a previously limited activity or on medication 
consumption. Verbal reports of significant relief 
of pain, if unaccompanied by alleviation of 
at tivity constraints, are of questionable merit. 

4. Significant benefits from TCS do not necessarily 
preclude proceeding with further treatment 
aimed at producing additional gains. 

Acupuncture. There are not as vet reliable and 
scientifically sound data which indicate lasting and 
significant benefit on functional performance and/or 
medication consumption from acupuncture treatment 
applied to problems of chronic pain. Some patients 



228 Fordyce 



report subjective benefit from periodic acupuncture 
treatments but those few have not yet been shown to 
perform significantly differently in work or daily living 
activities. 

Hypnosis. Hypnosis is a potentially powerful, but as 
yet not well- understood, tool. Only a handful of 
clinicians have been able to demonstrate lasting 
benefits from the use of hypnosis for persisting 
problems of chronic pain. Most hypnosis achievements 
in relation to pain have been with such time -limited 
problems as toothaches or childbirth. The major 
contribution of hypnosis to a spectrum of chronic pain 
patients has been in assisting reduction of muscle 
tension. As knowledge expands and skill progresses, 
perhaps more success will be achieved with this 
method. 

VOCATIONAL IMPLICATIONS 

Education 

The chronic pain patient will likely have been 
unemployed for several years. Taking additional 
"time out" for further education is risky, particularly 
immediately following a treatment program which has 
restored activity level. Unless that increased activity 
level is related to such positive environmental 
consequences as wages, and the desirable things those 
wages make accessible, treatment gains can quickly 
fade. Further education is indicated only where 
acceptable vocational alternatives are not found and 
there is compelling evidence to indicate that the newly 
restored activity level will be sustained during the 
educational program. If brief training (e.g., 3 months) 
is undertaken, efforts should be made to continue 
exercise treatment during this period in order to keep 
the activity level up. 

Aptitudes 

Chronic pain, per se, is unlikely to influence 
aptitudes. The exception would be permanent loss of 
some body movement or skill due to the effect of 
surgery. For example, a fused neck will limit head 
turning. 

Interests 

The basic question to address here concerns the 
client for whom pre -onset work proved to be aversive. 
The counselor must determine the characteristics of 
that type of work which made it aversive and also 
identify transferable skills and other interests which 
would lead to a more satisfactory vocational 
placement. The methods the counselor will use in 
dealing with these problems are not different than 
those used with any other type of client. 



Physical Demands 

Body changes resulting from surgery or medically 
prescribed limitation of motion or activity must be 
considered. A comprehensive treatment program will 
have addressed itself to restoring physical function to 
some practical maximum, which can then be translated 
to specific job demands. There may be some 
constraints on physical activity even with successful 
treatment, such as those imposed by surgery. If the 
chronic pain patient continues to have some visible 
residual of the pain problem (e.g., the consistently 
rigidly postured fused back or the limited head turning 
associated with neck fusion), co-workers may have a 
tendency to overprotect. Once the work limits have 
been defined, all involved should be made aware of 
them and no special accommodation should be made 
beyond those prescribed. 

Chronic pain patients are likely to be more 
vulnerable to recurrence of pain problems than other 
persons, and some prudence in regard to physical 
activity should be prescribed. The translation of those 
prescriptions into categories ranging from 
"sed