Introduction to Clinical Neurology

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Introduction to
Clinical Neurology
Fifth Edition

Douglas J. Gelb, MD, PhD

Department of Neurology
University of Michigan Medical School
Ann Arbor, Michigan

1
1
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Fifth Edition published in 2016
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Library of Congress Cataloguing-​in-​Publication Data
Names: Gelb, Douglas James, 1957- , author.
Title: Introduction to clinical neurology / by Douglas J. Gelb.
Description: Fifth edition. | Oxford ; New York : Oxford University Press, [2016] |
Includes index.
Identifiers: LCCN 2015044837 | ISBN 9780190467197 (alk. paper)
Subjects: | MESH: Nervous System Diseases.
Classification: LCC RC346 | NLM WL 140 | DDC 616.8—dc23
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medical or other professional advice. Treatment for the conditions described in this
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In memory of my father, the best teacher I’ve ever known.
 Preface to the Fifth Edition

With each new edition of this book, it becomes increasingly difficult to

strike a balance between completeness and clarity. Ten medications are
now approved as disease-​modifying therapy for multiple sclerosis. More
than 25 forms of limb-​girdle muscular dystrophy have been identified.
New autoimmune encephalopathies seem to appear every year. Imaging
techniques and interventional technologies keep evolving. Practice guide-
lines are proliferating and accumulating like misfolded proteins. It is hard
enough for a practicing clinician to assimilate all of these developments—​
for someone trying to present an introductory overview, the challenge is
nearly overwhelming.
I’m told that I’m one of the few people still using medical textbooks. So
are you, apparently. I hope you find that this book helps put the field of
clinical neurology in context and provides a coherent summary of major
concepts. As with each of the previous editions, I have focused on the
evaluation and management of the neurologic conditions most clinicians
are likely to encounter. I’ve tried to avoid getting bogged down in details
that would be of interest only to subspecialists. At the same time, I’ve
taken advantage of the expertise of some of my subspecialist colleagues
in an attempt to minimize blatantly inaccurate or obsolete statements.
My sincere thanks go to Jim Burke, Kelvin Chou, Praveen Dayalu, Simon
Glynn, Kevin Kerber, Steve Leber, Mollie McDermott, and Leslie Skolarus
for many helpful suggestions. As usual, I refused to let anyone else have
the last word, so they should not be blamed for the mistakes that remain.
Finally, as with each of the previous editions, thank you to Karen,
Elizabeth, and Molly, for everything.
D.J.G.
 Preface to the First Edition

Is neurology obsolete? Two current trends prompt this question. First,

dramatic biologic and technologic advances have resulted in increasingly
accurate diagnostic tests. It is hard to believe that CT scans have only
been widely available since the 1970s; MRI scans, PET scans, and SPECT
scans are even more recent, and they are constantly being refined. While
chess-​playing computers have not quite reached world champion status
yet, neurodiagnostic imaging studies have long since achieved a degree
of sensitivity that neurologists cannot hope to match. There is more than
a little truth to the joke that “one MRI scan is worth a roomful of neu-
rologists.” Moreover, advances in molecular genetics and immunochem-
istry now permit more accurate diagnosis of many conditions than could
have been imagined 25 years ago. Some conditions can even be diagnosed
before any clinical manifestations are evident. With tests this good, what is
the point of learning the traditional approach to neurologic diagnosis, in
which lesion localization is deduced from patients’ symptoms and signs?
The second major trend challenging the current status of neurology is
health care reform. As of this writing, the first great national legislative
debate concerning health care reform has ended, not with a bang but a
whimper. Yet the problem itself has not disappeared. The already unac-
ceptable costs of health care will continue to escalate if the current sys-
tem remains unchanged. While the various reform plans that have been
proposed differ in many fundamental respects, there appears to be a con-
sensus that there should be more primary care physicians and fewer spe-
cialists. Indeed, several forces are already pushing the medical profession
in that direction even in the absence of a comprehensive national legis-
lative plan. As a concrete example, it is anticipated that the number of
residency training positions in neurology will drop, perhaps to only half
of the current level. With so much nationwide emphasis on primary care,
what is the point of studying neurology?
x Preface to the First Edition

Ironically, these two trends together provide a compelling reason to

study neurology. In coming years, there will be increasing pressures on
primary care physicians to avoid referring patients to specialists (and there
will be fewer specialists in the first place). One response of primary care
physicians might be to order more diagnostic tests. Unfortunately, the only
thing more impressive than the sensitivity of the new tests is their price tag.
Just at the time when diagnostic tests are reaching unprecedented levels
of accuracy, the funds to pay for the tests are disappearing. Rather than
replacing neurologists with MRI scans and genetic testing, primary care
physicians will have to become neurologists (to some degree) themselves.
In short, the role currently played by neurologists may well be obso-
lete, but neurology itself is not. All physicians, regardless of specialty, must
become familiar with the general principles of neurologic diagnosis and
management. That is the rationale for this book.
The purpose of this book is to present a systematic approach to the neu-
rologic problems likely to be encountered in general medical practice. The
focus throughout the book is on practical issues of patient management.
This is a departure from the traditional view of neurologic diseases as fas-
cinating but untreatable. Neurologists are often caricatured as pedants who
will pontificate interminably on the precise localization of a lesion, pro-
duce an obscure diagnosis with an unpronounceable eponym, and smugly
declare the case closed. In years past, this stereotype was not wholly inac-
curate. Even when therapeutic options existed, there were few controlled
studies of efficacy, so it was easy to take a nihilistic approach to therapy.
“First do no harm” could often be legitimately interpreted to mean “Do
nothing.” This was obviously a frustrating position for physicians, and
even more so for patients, but at least it kept things simple. All this has
changed. Controlled trials of both new and traditional therapies are being
conducted with increasing frequency. In the two years since the original
versions of some of the current chapters were first prepared, sumatriptan
has been approved for treatment of headache, beta interferon for use in
multiple sclerosis, ticlopidine for stroke, and tacrine for Alzheimer’s dis-
ease. Felbamate has appeared and (practically) disappeared. Gabapentin
and lamotrigine have been approved for use in epilepsy. The long-​term
results of a large cooperative study of optic neuritis have challenged tra-
ditional practices involving the use of steroids for that condition and for
multiple sclerosis. Preliminary reports have appeared concerning the value
of endarterectomy for asymptomatic carotid stenosis, a new preparation of
beta interferon for MS, and Copolymer I for MS. These are exciting devel-
opments, but many of the studies raise as many questions as they answer.
 Preface to the First Edition xi

They certainly change the way physicians have traditionally approached

many neurologic diseases. This book reflects that change. Esoteric diagnos-
tic distinctions with little practical relevance are avoided. Distinctions that
affect treatment are emphasized. In most chapters, the available treatment
options and general approach to management are presented first, to clarify
which diagnostic distinctions are important and why.
For many physicians and medical students, the most difficult aspect of
neurology is deciding where to start. It is relatively straightforward to man-
age a patient who has had a stroke; it is often harder to determine whether
the patient had a stroke in the first place. When does hand weakness indi-
cate carpal tunnel syndrome, and when is it a manifestation of multiple
sclerosis? When does back pain signify metastatic cancer or a herniated
disk? These general issues are addressed in the three chapters of Part I. In
Part II, common neurologic disease categories are discussed. Part III con-
cerns common symptoms and issues that cross disease categories. Features
that distinguish neurologic problems in the pediatric and geriatric popula-
tions are discussed in Part IV.
This book is not meant to be comprehensive. Certain topics are omitted,
notably specialized management issues that primary care physicians will
probably not need to address and other conditions for which treatment is a
matter of standard medical care. For example, most patients with primary
brain cancer will probably be referred to specialists even in an age of health
care reform, so the different types of brain cancer and their treatment are
not addressed in this book. Diabetes, chronic alcohol abuse, vitamin B12
deficiency, and other metabolic disturbances can affect many parts of the
nervous system. These conditions are mentioned in the relevant sections of
this book, but there is no chapter devoted specifically to metabolic prob-
lems and their management because these topics are covered in stand-
ard medical textbooks. Even for the topics that are included in the book,
much detail has been omitted. Again, detailed discussions are available in
standard reference books. Use of those books requires some sophistication
about neurology, however. A physician trying to figure out why a patient’s
hand is weak may be overwhelmed by a one-​or two-​thousand page text-
book. Even when the patient’s diagnosis is known, the standard references
often fail to distinguish the forest from the trees, making it difficult to glean
the main principles governing patient management. Those principles are
the focus of this book.
Each chapter in this book begins with a set of clinical vignettes and asso-
ciated questions. These are intended to help the reader focus on practical
clinical questions while reading the chapter. Readers should try to answer
xii Preface to the First Edition

the questions before reading the rest of the chapter. After finishing the
chapter (but before reading the discussion of the clinical vignettes) read-
ers should return to the questions and revise their answers as necessary.
Readers can then compare their answers with those given in the discussion
at the end of the chapter.
The vignettes are also intended to convey the message that neurol-
ogy is fun. Many students who used the original version of this book
reported that they enjoyed working through the vignettes, and they even
suggested that more be included. This response is gratifying. Still, the
best “clinical vignettes” come from patients themselves, not from books.
Ideally, readers of this book will be inspired to seek out patients with
neurologic problems, and will approach them not only with confidence,
but with enthusiasm.
 Contents

Contributors xxv

PART I: The Basic Approach

1. Where’s the Lesion? 3
I. Sample Localization Problems 3
II. The Game 4
III. The Rules 6
IV. The Play: The Long Version 7
V. The Play: The Abbreviated Version 14
VI. Rules for Speed Play 38

2. The Neurologic Examination 43

I. More Localization Problems 43
II. General Comments on the Neurologic Examination 43
III. How to Do the Neurologic Examination 48
A. Mental Status Examination 48
B. Cranial Nerve Examination 50
C. Motor Examination 54
D. Reflex Examination 56
E. Sensory Examination 58
IV. Additional Comments on Terminology and
Examination Technique 60
A. Mental Status Examination 60
B. Cranial Nerve Examination 64
C. Motor Examination 66
xiv Contents

D. Reflex Examination 68
E. Sensory Examination 70
V. Interpretation of the Neurologic Examination 70
A. Mental Status Examination 71
B. Cranial Nerve Examination 73
C. Motor Examination 80
D. Reflex Examination 83
E. Sensory Examination 83
VI. Modifications of the Neurologic Examination 87
A. Screening Neurologic Examination 87
B. Examination of Stuporous or Comatose Patients 88
VII. Discussion of Localization Problems 92

3. What’s the Lesion? 93

James W. Albers and Douglas J. Gelb
I. Case Histories 93
II. Beyond Localization 95
A. Localization 96
B. Temporal Profile 97
C. Epidemiology 98
III. Etiology 98
A. Degenerative Diseases 98
B. Neoplastic Diseases 98
C. Vascular Diseases 99
D. Inflammatory Diseases 99
E. Toxic and Metabolic Diseases 99
F. Traumatic Diseases 100
G. Congenital and Developmental Diseases 100
IV. Discussion of Case Histories 102

PART II: Common Diseases

4. Stroke 109
I. Case Histories 109
II. Approach to Stroke 110
 Contents xv

III. Background Information 110

A. Definitions 110
B. Classification of Strokes by Etiology 111
C. Pathophysiology 112
IV. Diagnosis 113
A. Clinical Features 113
B. Imaging 118
V. Management of Acute Stroke 119
A. Restoration of Blood Flow in Ischemic Stroke 119
B. Limitation of Deficits 121
C. Rehabilitation 124
VI. Primary Prevention 124
A. Hypertension 124
B. Smoking 125
C. Diabetes 125
D. Dyslipidemia 125
E. Mechanical Heart Valves 125
F. Atrial Fibrillation 126
G. Carotid Stenosis 127
H. Sickle Cell Disease 129
I. Other Factors 130
VII. Secondary Prevention of Ischemic Stroke 130
A. Cardioembolic Disease 131
B. Carotid Stenosis 134
C. Anti-​Platelet Medications 135
D. Statin Therapy 137
E. Risk Factor Modification 137
F. Stroke Mechanisms Other Than Cardioembolism and
Carotid Stenosis 138
G. Determining the Underlying Mechanism of
Stroke 140
VIII. Secondary Prevention of Cerebral Hemorrhage 142
IX. Discussion of Case Histories 142
xvi Contents

5. Seizures 147
I. Case Histories 147
II. Approach to Seizures 148
III. Background Information 149
A. Definitions 149
B. Clinical Characteristics of Seizures 150
C. Seizures vs. Epilepsy 152
D. Epilepsy Syndromes 153
E. Electroencephalography 154
F. Pathophysiology of Seizures and Epilepsy 155
IV. Diagnosis 156
A. Characterizing the Presenting Spell 157
B. Identifying Prior Spells 158
C. Recognizing Spells That Are Not Seizures 158
V. Determining the Cause of Seizures 160
A. Provoked Seizures 160
B. Epilepsy 161
C. The Diagnostic Evaluation 162
VI. Management of Seizures and Epilepsy 163
A. Patients with Seizures but No Proven Epilepsy 163
B. Patients with Epilepsy 164
C. Patient Education (for Patients with Isolated
Seizures or Epilepsy) 176
D. Restrictions (for Patients with Isolated Seizures
or Epilepsy) 178
VII. Special Clinical Problems 178
A. Status Epilepticus 178
B. Seizures and Pregnancy 181
C. Refractory Seizures 182
VIII. Discussion of Case Histories 183

6. Neuromuscular Disorders 185

Mark B. Bromberg and Douglas J. Gelb
I. Case Histories 185
II. Approach to Neuromuscular Diseases 187
 Contents xvii

III. Background Information 188

A. Functional Divisions of the Peripheral
Nervous System 188
B. Proximal-​to-​Distal Organization of the Peripheral
Nervous System 189
C. Electrodiagnostic and Other Laboratory
Studies 190
IV. Specific Neuromuscular Diseases 192
A. Motor Neuron Diseases 192
B. Nerve Root Disorders (Radiculopathies) 195
C. Plexus Disorders (Plexopathies) 196
D. Peripheral Nerve Disorders (Neuropathies) 196
E. Neuromuscular Junction Disorders 203
F. Muscle Disorders (Myopathies) 206
V. Symptomatic Treatment 209
A. Emergency Measures 209
B. Non-​Urgent Measures: Motor Symptoms 209
C. Non-​Urgent Measures: Sensory Symptoms 210
VI. Discussion of Case Histories 210

7. Dementing Illnesses 213

Linda M. Selwa and Douglas J. Gelb
I. Case Histories 213
II. Approach to Dementing Illnesses 215
A. Is It Abnormal? 216
B. Is It Dementia? 217
C. Is It Progressive? 219
D. Is There a Potentially Reversible Cause? 220
E. Which Diagnosis Is Most Likely? 221
III. Primary Dementing Illnesses 221
A. Alzheimer’s Disease 221
B. Dementia with Lewy Bodies (DLB) 230
C. Frontotemporal Dementia (FTD) 232
D. Vascular Dementia 235
E. Normal Pressure Hydrocephalus (NPH) 236
xviii Contents

F. Creutzfeldt-​Jakob Disease (CJD) 238

G. Other Neurologic Diseases That Produce
Dementia 241
IV. Discussion of Case Histories 241

8. Movement Disorders 245

Linda M. Selwa and Douglas J. Gelb
I. Case Histories 245
II. Approach to Movement Disorders 246
III. Background Information 246
A. Anatomic Definitions 246
B. Clinical Definitions 247
C. Classification of Movement Disorders 248
IV. Specific Movement Disorders 249
A. Essential Tremor 249
B. Parkinson’s Disease 250
C. Other Parkinsonian Syndromes 258
D. Hereditary Ataxias 262
E. Huntington’s Disease 265
F. Tardive Dyskinesia 267
G. Dystonias 268
H. Wilson’s Disease 270
I. Gilles de la Tourette’s Syndrome 271
V. Discussion of Case Histories 272

9. Sleep Disorders 275

I. Case Histories 275
II. Approach to Sleep Disorders 276
III. Background Information 277
A. Definitions 277
B. Sleep Physiology 277
C. Diagnostic Tests 280
D. Classification of Sleep Disorders 281
IV. Trouble Staying Awake 281
A. Insufficient Sleep 281
B. Sleep Apnea 282
 Contents xix

C. Narcolepsy 283
D. Other Causes of Hypersomnolence 285
V. Trouble Sleeping 286
A. Sleep-​Onset Delay 286
B. Early Morning Awakening 288
C. Sleep Fragmentation 289
D. Sleep State Misperception 289
VI. Abnormal Behavior During Sleep 290
A. Nonrapid Eye Movement (NREM)
Sleep Parasomnias 290
B. Rapid Eye Movement (REM) Sleep Parasomnias 293
VII. Discussion of Case Histories 294

10. Multifocal Central Nervous System Disorders 297

I. Case Histories 297
II. Approach to Multifocal Disorders 299
III. Focal Diseases with Multifocal Propagation 299
A. Metastatic Cancer 299
B. Central Nervous System Infections 303
IV. Inherently Multifocal Diseases 317
A. Multiple Sclerosis (MS) 317
B. Connective Tissue Diseases 327
C. Sarcoidosis 329
D. Coagulation Disorders 331
V. Discussion of Case Histories 332

PART III: Common Symptoms

11. Acute Mental Status Changes 337
I. Case Histories 337
II. Background Information 338
A. Definitions 338
B. Focal Mental Status Changes vs. Altered
Level of Consciousness 339
C. Physiology of Normal and Altered
Consciousness 340
xx Contents

III. Approach to Acute Changes in Level

of Consciousness 341
A. ABCs: Airway, Breathing, Circulation 341
B. Oxygen, Glucose, Naloxone 341
C. Pupils, Doll’s Eyes, Motor Asymmetry 343
D. Other Electrolytes, Renal, Hepatic,
Temperature Abnormalities 345
E. Everything Else 346
IV. Special Circumstances 348
A. Head Trauma 348
B. Increased Intracranial Pressure 351
C. Brain Death 352
V. Discussion of Case Histories 353

12. Headache 355

I. Case Histories 355
II. Approach to Headache 356
III. Background Information 356
A. Primary vs. Secondary Headaches 356
B. Pathophysiology of Migraine 356
IV. Headache Emergencies: Subarachnoid Hemorrhage
and Bacterial Meningitis 358
V. Other Secondary Headaches 362
A. Viral Meningitis or Encephalitis 362
B. Fungal or Tuberculous Meningitis 363
C. Mass Lesions 363
D. Giant Cell (Temporal) Arteritis 364
E. Idiopathic Intracranial Hypertension
(IIH; Pseudotumor Cerebri) 365
F. Spontaneous Intracranial Hypotension 365
G. Cerebral Venous Thrombosis 366
H. Arterial Dissection 366
I. Systemic Conditions 366
J. Secondary Headache Syndromes
with Diagnostic Ambiguity 366
 Contents xxi

VI. Primary Headaches 368

A. Migraine and Tension Headaches 368
B. Cluster Headaches 376
C. Trigeminal Neuralgia 377
D. Glossopharyngeal Neuralgia 379
E. Chronic Paroxysmal Hemicrania
and Related Conditions 379
F. Atypical Facial Pain 380
VII. Discussion of Case Histories 380

13. Visual Symptoms 383

I. Case Histories 383
II. Background Information 384
A. Definitions 384
B. Overview of the Visual System 384
III. Approach to Visual Symptoms 384
IV. Monocular Vision Loss 385
A. Acute or Subacute Monocular Vision
Loss in Young Patients 385
B. Acute, Subacute, or Chronic Monocular
Vision Loss in Older Patients 386
V. Transient Vision Loss (Monocular or Binocular) 387
VI. Persistent Binocular Vision Loss 388
VII. Diplopia 389
A. Localization 389
B. Differential Diagnosis and Management 390
VIII. Discussion of Case Histories 392

14. Dizziness and Disequilibrium 393

I. Case Histories 393
II. Approach to Dizziness 395
III. Localization 395
IV. Differential Diagnosis 396
A. Central Vertigo 397
B. Peripheral Vertigo 397
xxii Contents

V. Disequilibrium 403
VI. Discussion of Case Histories 404

15. Back Pain and Neck Pain 407

I. Case Histories 407
II. Approach to Back or Neck Pain 408
A. Emergency Situations 408
B. Non-​Urgent Indications for Surgery 409
III. Specific Conditions Causing Back or Neck Pain 409
A. Musculoskeletal Pain 409
B. Disc Herniation 410
C. Spinal Stenosis 412
IV. Discussion of Case Histories 412

16. Incontinence 415

I. Case Histories 415
II. Background Information 416
III. Approach to Incontinence 416
A. Neurologic vs. Urologic Causes of Incontinence 417
B. Central vs. Peripheral Nervous System Causes
of Incontinence 418
IV. Discussion of Case Histories 419

PART IV: Bookends

17. Pediatric Neurology 423
I. Case Histories 423
II. Developmental Considerations 425
III. Hypotonic Infants 426
IV. Developmental Delay and Developmental
Regression 428
V. Paroxysmal Symptoms 431
A. Headaches 431
B. Seizures 432
C. Breath-​Holding Spells 432
D. Benign Paroxysmal Vertigo 433
 Contents xxiii

VI. Gait Disturbance 433

A. Spasticity 433
B. Weakness 434
C. Ataxia 434
VII. Discussion of Case Histories 435

18. Geriatric Neurology 441

I. Case Histories 441
II. Geriatric Issues 443
III. The Neurologic Examination in Normal Aging 443
A. Mental Status 444
B. Cranial Nerves 445
C. Motor System 445
D. Reflexes 446
E. Sensation 446

IV. Common Neurologic Symptoms in the Elderly 446

A. Dizziness 446
B. Gait Disturbance 447
C. Incontinence 447
D. Dementia 448
E. Pain 448
V. Discussion of Case Histories 448

19. Practice Cases 451

I. Case Histories 451
II. Answers 454

Index 461