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This page intentionally left blank
Fundamentals of
Neurologic Disease

Larry E. Davis, M.D.

With
Molly K. King, M.D.
Jessica L. Schultz, M.D.

Neurology Service
New Mexico VA Health Care System
Albuquerque, New Mexico
and
Departments of Neurology and Neuroscience
School of Medicine
University of New Mexico
Albuquerque, New Mexico
Demos Medical Publishing, Inc., 386 Park Avenue South, New York, New York 10016

© 2005 by Demos Medical Publishing, Inc. All rights reserved. This book is protected by copyright. No
part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means,
electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of
the Publisher.

Illustrations by Yvonne Wylie Walston, CMI © Creative Imagery, Inc.

Library of Congress Cataloging-in-Publication Data

Davis, Larry E. (Larry Ernest)

Fundamentals of neurologic disease : an introductory text / Larry E.
Davis, with Molly K. King, Jessica L. Schultz ; illustrations by Yvonne
Wylie Walston.
p. cm.
Includes bibliographical references and index.
ISBN 1-888799-84-6 (pbk. : alk. paper)
1. Neurology. 2. Nervous system—Diseases.
[DNLM: 1. Nervous System Diseases. WL 300 D262f 2005] I. King, Molly
K., 1962- II. Schultz, Jessica L., 1974- III. Title.
RC346.D385 2005
616.8—dc22 2004030159
Contents

1 Approach to the patient with a neurologic problem: Key steps in

neurological diagnosis and treatment 1

2 Neurologic examination 9
3 Common neurologic tests 23
4 Disorders of muscle 39
Duchenne muscular dystrophy (Muscular dystrophies) 40
Dermatomyositis (Inflammatory myopathy) 44
Primary hyperkalemic periodic paralysis (channelopathies) 45

5 Disorders of the neuromuscular junction 49

Myasthenia gravis 49
Botulism 53

6 Disorders of peripheral nerves 57

Diabetic distal symmetrical polyneuropathy 58
Carpal tunnel syndrome 61
Bell’s palsy 63

7 Disorders of the spinal cord and vertebral bodies 67

Amyotrophic lateral sclerosis 68
Transverse myelitis and myelopathy 73
Low back pain with radiculopathy 74
iii
iv CONTENTS

8 Disorders of the brainstem and cerebellum 79

Lateral medullary infarction (Wallenberg syndrome) 81
Spinocerebellar ataxia (SCA 1) 84

9 Disorders of the cerebrovascular system 87

Ischemic strokes (embolic and lacunar) 87
Transient ischemic attacks 93
Hemorrhagic strokes 94
Spontaneous intracranial hemorrhage 94
Saccular aneurysms 96

10 Disorders of myelin 101

Myelin 101
Multiple sclerosis 102
Guillain-Barré syndrome 105

11 Disorders of higher cortical function 109

Prefrontal lobe 110
Limbic system 110
Parietal lobe 111
Aphasias 113
Intelligence 115
Neurologic changes of normal aging 115
Dementia 116
Alzheimer’s disease 117
Mental retardation 120

12 Disorders of the extrapyramidal system 123

Essential tremor 124
Parkinson’s disease 126
Huntington’s disease 130

13 Central nervous system infections 133

Bacterial meningitis 134
Brain abscess 137
Herpes simplex virus encephalitis 139
Prion diseases 141
 CONTENTS v

14 Brain tumors 145

Brain herniation syndromes 145
Cerebral edema 146
Glioblastoma multiforme — malignant astrocytoma 147
Meningioma 149
Pituitary adenoma 150
Cerebral metastases 152

15 Seizures and status epilepticus 155

Primarily generalized tonic-clonic seizures and secondarily generalized
partial seizures (Grand mal seizure) 156
Absence seizure (Petit mal seizure) 158
Infantile spasms (West’s syndrome) 159
Complex partial seizure (Localization-related, temporal lobe, or
psychomotor seizure) 160
Status epilepticus 161

16 Coma and cerebral death 165

Coma 165
Cerebral death 171

17 Disorders of the developing nervous system 173

Anencephaly 174
Chiari type I and II malformations 176
Phenylketonuria (Phenylalanine hydroxylase deficiency) 178
Tay-Sach’s disease (Hexosaminidase A deficiency) 179
Down syndrome 181

18 Traumatic brain injury and subdural hematoma 185

Traumatic brain injury 185
Chronic subdural hematoma 190

19 Neurologic complications of alcoholism 193

Drunkenness and alcoholic coma 194
Alcoholic tremulousness and hallucinosis 194
Alcohol-withdrawal seizures 195
Delirium tremens 195
Wernicke’s encephalopathy and Korsakoff ’s psychosis syndrome 195
vi CONTENTS

Alcoholic cerebellar degeneration 196

Alcoholic polyneuropathy 198
Fetal alcohol syndrome 198

20 Disorders of pain and headache 201

Pain 201
Headache pain 202
Tension-type headache 203
Migraine headache 204

21 Disorders of the vestibular system 209

Principles of vertigo management 212
Benign paroxysmal positional vertigo 212
Meniere’s disease 215

Glossary of common neurologic terms 219

Index 227
 Preface

This textbook is intended for students who wish to The later chapters are divided into chapters that
learn the basic principles of neurology and to review common diseases present at different neu-
understand common neurologic diseases. We roanatomic sites along the neuroaxis from muscle
selected 58 neurologic diseases based on their fre- to the cerebral cortex and chapters on diseases that
quency, ability to represent that category of neuro- have a similar pathophysiology. Each chapter
logic disease, value in teaching neuroscience begins with an overview to understand the com-
concepts, and diagnostic importance. mon features of this group of diseases. Selected
We recognized that most introductory courses diseases are then discussed with an emphasis on
on neurologic diseases are short and lack suffi- the pathophysiology, major clinical features, major
cient time for a student to extensively read a com- laboratory findings, and the principles of disease
prehensive neurology textbook. In addition, while management. Our book covers both adult and
abbreviated versions of neurology textbooks cover pediatric neurologic diseases.
a myriad of neurologic diseases in telegraphic This book does not cover detailed aspects of dis-
style, they are difficult to comprehend unless one ease variants, all possible laboratory tests, drug
already knows about the disease. Thus, many stu- dosages, or many related neurologic diseases as it is
dents finish the course with a spotty understand- not designed for the specific treatment of neuro-
ing of neurology. We designed our book to be read logic patients. Our goal is to provide broad and
from cover to cover, giving the reader a more integrated coverage of the fundamentals of com-
thorough understanding of the fundamentals of mon neurologic diseases in the context of a
neurology. competent examination strategy, the disease patho-
The first chapters cover the basic approach a physiology, and the principles of disease manage-
neurologist takes when encountering a patient with ment. We hope that students will find the book
a neurologic problem, the key elements of the neu- structure and context useful in their studies and that
rologic exam, and an overview of common neuro- it will contribute to the instructors’ efforts to sup-
logic tests. We discuss how to use the history and port students in their learning.
neurologic exam to localize the patient’s problem to
specific neuroanatomic site(s) and to use the neu- Larry E. Davis, M.D.
roanatomic information along with results of Molly K. King, M.D.
appropriate laboratory tests to establish a diagnosis. Jessica L. Schultz, M.D.

vii
This page intentionally left blank
 Acknowledgments

Dr. Davis is indebted to his late father, Lloyd, for Finally, we thank everyone who helped in the
his years of encouragement in his pursuit of schol- preparation of this book. In particular, we are
arly endeavors; to his wife, Ruth Luckasson, for her grateful to Yvonne Walston, CMI, of Creative
support, ideas, patience, and encouragement dur- Imagery, Inc. whose artwork improved the clarity
ing the many hours of writing this book; and to his of the chapters, to Dr. Blaine Hart, who con-
children Meredith, Colin, and Charles. tributed neuroimaging illustrations, to Drs. Mark
Dr. King thanks Dr. Joe Bicknell for his enthu- Becher and Mario Kornfeld who contributed neu-
siastic mentoring and Dr. Kurt Fiedler for his ropathology illustrations, and to Diana Schneider,
inspiring, never-ending pursuit of knowledge. Dr. PhD, President of Demos Medical Publishing, who
Schultz would like to thank her husband, Brandon, supported the creation of this book.
for his support and unfailing patience during the
creation of this book.

ix
This page intentionally left blank
 Foreword

A detailed patient history and physical examina- anatomical and pathological correlation and pro-
tion remain the underpinnings of neurologic diag- vides many tables and line drawings that help in
nosis. Imaging and laboratory testing are understanding the anatomy and physiological
important, but the ability to piece together clues in basis of disease and the differential diagnosis of
the patient’s story and to localize lesions by the neurologic illnesses. Too often introductory vol-
findings on the neurologic examination still sepa- umes on neurology are overwhelming in bulk and
rate the good neurologist from the main body of complexity. The authors of this volume have suc-
physicians. Medical students who choose neurol- ceeded in presenting a gentler and concise intro-
ogy for a career often cite two factors that have duction to this fascinating subject.
influenced them—first, the spectacular basic and
clinical advances in the understanding and treat- Richard T. Johnson, MD, FRCP
ment of neurologic illnesses and, second, the con- Distinguished Service Professor of Neurology,
tinued reliance on clinical skills and the Microbiology and Neuroscience
formulation of diagnoses at the beside or in the The Johns Hopkins University School of
clinic. Medicine & Bloomberg School of Public Health
This condensed volume introduces the reader Baltimore, Maryland
to neurologic diseases; it emphasizes the clinical

xi
This page intentionally left blank
1 APPROACH TO THE PATIENT
WITH A NEUROLOGIC PROBLEM
Key Steps in Neurologic Diagnosis and Treatment

When helping a patient with a neurologic disorder, ment. The expert clinician often is able to estab-
a clinician uses clinical skills (taking the history, lish the correct diagnosis by listening to the his-
conducting the physical and neurologic examina- tory, forming a hypothesis, and confirming that
tion, and ordering of appropriate tests), knowl- hypothesis based on the neurologic exam. How-
edge of neuroanatomy, and an understanding of ever, the medical student seldom possesses these
the pathogenesis of the neurologic disease process skills, as they come with experience. Fortunately,
(Figure 1-1). The goals are to alleviate signs and following eight logical steps can help one arrive at
symptoms and to restore and keep the patient in the same diagnosis. This avoids costly mistakes
the best possible health. due to ordering inappropriate laboratory tests,
To achieve these goals, one must establish the establishing an incorrect diagnosis, and prescrib-
correct diagnosis and initiate appropriate treat- ing the wrong treatment.

Figure 1-1 Approach to the neurologic patient.

1
2 FUNDAMENTALS OF NEUROLOGIC DISEASE

Steps in Diagnosing in which damage to any lobe produces similar

symptoms.
Neurologic Conditions The nervous system can be divided into discrete
1. Determine whether the condition involves anatomic compartments that give rise to a specific
the nervous system constellation of signs and symptoms. This book
2. Determine an anatomic localization follows the neuroanatomic outline below:
3. Establish the time course of symptoms Muscle
4. Determine the most likely disease cate- ↓
gory(s) Neuromuscular junction
5. Make a clinical diagnosis or differential ↓
diagnoses Peripheral nerve
6. Order appropriate laboratory or neuroimag- ↓
ing tests Nerve root
7. Establish definite diagnosis ↓
8. Begin appropriate etiologic and sympto- Spinal cord
matic treatment ↓
Brainstem
↓
1. Determine Whether the Cerebellum
Condition Involves the ↓
Basal ganglia and thalamus
Nervous System ↓
Cerebral cortex
The first step is to determine whether the patient’s
↓
signs and symptoms are due to an illness involving
Meninges and cerebrospinal fluid
the nervous system. This decision is based on the
history and physical exam coupled with knowl-
In defining the neuroanatomic site, the clini-
edge of general medical diseases. For example, syn-
cian should establish the highest and lowest points
cope causes loss of consciousness, but the etiology
of the nervous system that can give rise to the
can be from cardiovascular disease.
patient’s signs and symptoms. Helpful keys in
determining the most likely neuroanatomic local-
ized site include:
2. Determine an Anatomic
Localization 1. Finding the earliest signs and symptoms of
the illness, which usually denote anatomi-
Another important step based on the history and cally where the disease began.
physical examination is to establish the most likely 2. Determining the anatomic site where weak-
neuroanatomic site that could cause the patient’s ness and/or sensory changes likely are pro-
problem. While experts may bypass this step, it is duced. Motor and sensory systems are
helpful to the beginning clinician. Knowledge of multisynaptic long-tract systems commonly
the site enables the clinician to narrow the list of involved in many diseases. Weakness pro-
differential diagnoses and to determine which lab- duced by dysfunction of the motor system at
oratory and neuroimaging tests will yield the most the motor cortex, brainstem, spinal cord,
useful information. peripheral nerves, neuromuscular junctions,
Neurologic localization is possible because the and muscle has unique characteristics that
nervous system is organized such that each major help localize the site of the problem.
neuroanatomic location gives rise to specific 3. Identifying accompanying nonneurologic
signs and symptoms. As such, the nervous system signs and symptoms that may help localize
differs from many other organs such as the liver, the site.
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