Table Of Contenta LANGE medical book
Clin ical Neu r ology
an d Neu r oan at om y
A Localization -Based Appr oach
Aaron L. Berkowitz, MD, PhD
Assistant Professor of Neurology
Harvard Medical School
Brigham and Women’s Hospital
Boston, Massachusetts
New York Chicago San Francisco Athens London Madrid Mexico City
Milan New Delhi Singapore Sydney oronto
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T is book is dedicated to:
My mentors Dr. Martin A. Samuels, Dr. Allan H. Ropper,
and Dr. Steven K. Feske, who through their extraordinary
mentorship and teaching trained me not only in the science o
Neurology, but in the art o Medicine.
T e students and residents at Harvard Medical School and the
Partners Neurology residency program (Boston); the residents
and aculty at Hôpital Universitaire de Mirebalais, Hôpital St.
Nicolas de St. Marc, and Hôpital St. Boni ace (Haiti); and the
students and residents at Queen Elizabeth Central Hospital
and Kamuzu Central Hospital (Malawi), who through their
brilliant questions and insatiable desire to learn taught me
how to teach neurology.
T e patients with and through whom I learned the practice
o neurology and medicine, and whose courage in the ace o
suf ering inspires us to learn more about their diseases, teach
what we learn to others, and serve them and their amilies to
the best o our abilities.
My wi e Nina, whose boundless support, encouragement,
and companionship have been both a sustaining orce and a
source o great joy.
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Contents
Foreword ix
Pre ace xi
Acknowledgments xiii
I 4 T e Motor and Somatosensory Pathways
P A R
and Approach to Weakness and
NEUROANA OMY AND
Sensory Loss 33
NEUROANA OMIC T e Corticospinal racts 33
T e Corticobulbar racts 36
LOCALIZA ION 1
Somatosensory Pathways or the Body 36
Localization o Motor and Sensory De cits 38
1 Diagnostic Reasoning in Neurology and the
Neurologic History and Examination 1 5 T e Spinal Cord and Approach to
Localization in Neurologic Diagnosis: Determining Myelopathy 41
Where T e Problem Is 1 Overview o Spinal Cord Anatomy 41
ime Course in Neurologic Diagnosis: Determining Lamination o the Long racts in the
What T e Problem Is 2 Spinal Cord 41
Associated Symptoms and Signs in Neurologic Spinal Cord Syndromes 42
Diagnosis 3 Spinal Cord Pathways For Bowel and
Introduction to the Neurologic Examination 3 Bladder Control 44
Causes o Myelopathy 45
2 Introduction to Neuroimaging and
Cerebrospinal Fluid Analysis 11 6 T e Visual Pathway and Approach to
Neuroimaging in Clinical Practice 11 Visual Loss 47
Overview o Neuroimaging Interpretation 12 Anatomy o the Visual Pathway 47
Interpretation o Brain C 12 Approach to Visual Loss 48
Interpretation o Brain MRI 13 Disorders o Visual Cognition 50
Contrast-Enhanced Neuroimaging 17
7 T e Cerebral Hemispheres and Vascular
Vascular Imaging 19
Syndromes 53
Nuclear Medicine Studies: Positron Emission
Cortical Regions 53
omography And Single Photon Emission
Subcortical Structures: T alamus and
Computed omography 21
Basal Ganglia 55
Neuroimaging o the Spine 21
Arterial Supply o the Cerebral Hemispheres 57
Cerebrospinal Fluid Analysis 21
Clinical Syndromes Associated With Cerebral
3 Overview o the Anatomy o the Nervous
Vascular erritories 59
System 25
8 T e Cerebellum and Approach
Central Nervous System (CNS) and Peripheral
to Ataxia 67
Nervous System (PNS) 25
Anatomy and Function o T e Cerebellum 67
Hemispheres and Lobes o the Brain 25
Arterial Supply o the Cerebellum 70
Gray Matter and White Matter o the Brain and
Approach to Ataxia 70
Spinal Cord 26
T e Meninges: Coverings o the Brain and
9 T e Brainstem and Cranial Nerves 75
Spinal Cord 26
Overview o Brainstem Anatomy 75
T e Cerebral Venous Sinuses 29
Clinical Applications o Basic Brainstem
T e Ventricular System and Cerebrospinal Fluid
Anatomy 77
Flow 29
v
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vi Contents
10 Pupillary Control and Approach to 16 Radiculopathy, Plexopathy, and
Anisocoria 83 Mononeuropathies o the Upper
Cranial Nerves 2 and 3
Pupillary Constriction: the Parasympathetic Extremity 141
Pathway 83 Neuroanatomy o the Upper Extremity 141
Pupillary Dilation: the Sympathetic Cervical Radiculopathy 145
Pathway 84 Brachial Plexopathy 147
Approach to Anisocoria and Other Pupillary Mononeuropathies o the Upper Extremity 147
Abnormalities 87
17 Radiculopathy, Plexopathy, and
11 Extraocular Movements and Approach to
Mononeuropathies o the Lower
Diplopia 91
Cranial nerves 3, 4, and 6 Extremity 157
Extraocular Movements I: Muscles and their
Neuroanatomy o the Lower Extremity 157
Innervation 91
Lumbosacral Radiculopathy and Lumbar Canal
Extraocular Movements II: Cranial Nerves
Stenosis 159
3, 4, and 6 93
Lumbosacral Plexopathy 163
Extraocular Movements III: Supranuclear Control o
Mononeuropathies o the Lower Extremity 163
Horizontal and Vertical Gaze 96
Approach to Foot Drop 165
Approach to Diplopia 101
12 T e Auditory and Vestibular Pathways
II
P A R
and Approach to Hearing Loss and
Dizziness/Vertigo 105 DISEASES OF HE NERVOUS
Cranial Nerve 8
T e Auditory System 105
SYS EM 167
Approach to Hearing Loss 105
T e Vestibular System 107
Approach to Dizziness and Vertigo 110 18 Seizures and Epilepsy 167
De nitions and Causes o Seizures
13 Facial Sensation and Movement and Approach
and Epilepsy 167
to Facial Sensory and Motor Def cits
Cranial
Evaluation o Patients With Seizures 168
117
nerves 5 and 7
Clinical Features o Seizures 169
rigeminal Nerve (Cranial Nerve 5) 117
Electroencephalography (EEG) in the Evaluation o
Facial Nerve (Cranial Nerve 7) 120
Seizures 170
Evaluation and Management o Patients A er a First
14 Cranial Nerves 1, 9, 10, 11, and 12 125
Seizure 171
Cranial Nerve 1 (Ol actory Nerve) 125
Outpatient Management o Epilepsy 173
Cranial Nerve 9 (Glossopharyngeal) and
Special Scenarios in the Management o Seizures and
Cranial Nerve 10 (Vagus) 125
Epilepsy 175
Cranial Nerve 11 (Spinal Accessory) 126
Status Epilepticus 178
Cranial Nerve 12 (Hypoglossal) 128
19 Vascular Diseases o the Brain and Spinal
15 T e Peripheral Nervous System and
Cord 179
Introduction to Electromyography/Nerve
Overview o Ischemic Stroke and Intracerebral
Conduction Studies 129
Hemorrhage 179
Introduction to Anatomy and Diseases o the
Ischemic Stroke 180
Peripheral Nervous System 129
Intracerebral Hemorrhage 192
Anatomy and Diseases o Nerve Roots:
Central Nervous System Vascular Mal ormations 195
Radiculopathy 130
Subarachnoid Hemorrhage 196
Diseases o Dorsal Root Ganglia: Ganglionopathy
Intraventricular Hemorrhage 198
(Sensory Neuronopathy) 134
Subdural Hematoma 199
Diseases o the Brachial or Lumbosacral Plexus:
Epidural Hematoma 200
Plexopathy 134
Cerebral Venous Sinus T rombosis and
Diseases o Peripheral Nerves 135
Cortical Vein T rombosis 200
Introduction to EMG and Nerve Conduction
Other Cerebrovascular Disorders 201
Studies 136
Vascular Disease o the Spinal Cord 203
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Contents vii
20 In ectious Diseases o the Nervous System 207 26 Headache 275
Meningitis 207 Approach to Headache 275
Viral Encephalitis 212 Secondary Causes o Headache 275
Focal In ectious Brain Lesions 214 Primary Headache Disorders 276
In ections o the Spine 218 Other Causes o Headache 279
In ections o Nerve Roots 219
27 Peripheral Neuropathy 281
In ectious Peripheral Neuropathies 219
Classi cation and Di erential Diagnosis o Peripheral
In ection at the Neuromuscular Junction:
Polyneuropathy 281
Botulism 219
Acute Polyneuropathy 283
In ectious Myositis 220
Chronic Polyneuropathy 284
Neurologic Mani estations o HIV 220
28 Motor Neuron Disease 289
21 Demyelinating Diseases o the Central Nervous
Overview o Motor Neuron Disease 289
System 223
Amyotrophic Lateral Sclerosis (ALS) and its
Multiple Sclerosis 223
Variants 289
Neuromyelitis Optica 227
Spinal Muscular Atrophy (SMA) and Spinal Bulbar
Acute Disseminated Encephalomyelitis 228
Muscular Atrophy (Kennedy’s Disease) 291
Optic Neuritis 229
ransverse Myelitis 229 29 Disorders o the Neuromuscular Junction 293
Myasthenia Gravis 293
22 Delirium, Dementia, and Rapidly Progressive
Congenital Myasthenic Syndromes and Neonatal
Dementia 231
Myasthenia Gravis 296
Approach to Altered Cognition 231
Lambert-Eaton Myasthenic Syndrome 296
Delirium 231
Dementia 233 30 Diseases o Muscle 299
Rapidly Progressive Dementia 238 Causes o Muscle Disease 299
Clinical Features o Muscle Disease 299
23 Movement Disorders 241
Laboratory esting in Muscle Disease 300
Introduction to Movement Disorders 241
In ammatory Myopathies 300
remor 241
Muscular Dystrophies 301
Myoclonus 243
Distal Myopathies (Distal Muscular Dystrophies) 303
Chorea 244
Congenital Muscular Dystrophies and Congenital
Dystonia 246
Myopathies 304
ics, ourette’s Syndrome, and Sterotypies 246
Metabolic Myopathies 304
Parkinsonism, Parkinson’s Disease, and Parkinson-
Mitochondrial Myopathies 306
Plus Syndromes 247
Hyperkalemic and Hypokalemic Periodic
Other Movement Disorders 253
Paralysis 306
24 Neoplastic and Paraneoplastic Disorders o the Muscle Disease Due to Systemic Diseases and
Medications 307
Nervous System and Neurologic Complications
o Chemotherapy and Radiation T erapy 255
31 Leukodystrophies and Mitochondrial
Intracranial umors 255
Disorders 309
umors o the Spine 258
Leukodystrophies 309
umors o the Cranial Nerves 260
Mitochondrial Diseases 309
umors o the Peripheral Nervous System 261
Neurocutaneous Syndromes and Associated Nervous
System Neoplasms 262
Index 313
Neurotoxicity o Chemotherapy and Radiation
T erapy 262
Paraneoplastic Syndromes o the Nervous System 264
25 Disorders o Intracranial Pressure 269
Increased Intracranial Pressure (Intracranial
Hypertension) 269
Decreased Intracranial Pressure (Intracranial
Hypotension) 273
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For ewor d
So much o neurology exists only “in use”. T is is the neu- ar superior to existing books o its size and scope because o
rology that is practiced in the clinics, wards, and of ces o the thought ulness with which the knowledge about diseases
seasoned clinicians and cannot be ound in large encyclopedic and neurological conditions has been assembled. He gets right
textbooks o neurology or smaller monographs intended or down to business, addressing almost every major point that is
medical students. T e accumulated experience o the neurolo- encountered on the wards and in the clinic.
gist can be distilled to a number o action items and thought A book such as this one is more suitable or neurology
processes that are challenging to articulate. than or any other branch o medicine. We still depend on
Dr. Aaron Berkowitz has written a book that occupies the inter ace between our own re ned clinical skills and our
just this position. He has taken the transactional daily work decisions regarding diagnosis and treatment. T e pearls con-
o neurology and produced a wonder ully readable, concise, tained here about the meaning o particulars o the history
but by no means super cial book that ts well in the current and examination cannot be ound elsewhere. T e book makes
pedagogic environment. One might ask whether any book on a seamless transit rom these data to practical wisdom about
neurology is needed now that disembodied in ormation is so their application. T e material is clear and avoids the ambigu-
easily available on the web and algorithms or various signs, ity that clutters most other books. In doing so, it also incor-
symptoms, and diseases abound. But between in ormation that porates the latest thinking rom clinical trials and together,
is as o en misleading as it is use ul, and the storehouse o wis- these eatures provide one o the best modern outlooks on the
dom accumulated over a long career, sits a great body o neu- pragmatic practice o neurology.
rological knowledge. It is this assembled knowledge that allows It takes a certain outlook on pedagogy and practice to
us to ef ciently move through the workday and can be taught produce such a book. Dr. Berkowitz has more than succeeded,
to students and residents during their rotations. Berkowitz’s and I nd mysel looking at a number o the chapters over and
book is more than a compendium or teaching guide and is over to reorient mysel to solid teaching and practice.
Allan H. Ropper, MD
Executive Vice Chair o Neurology
Brigham and Women’s Hospital
Pro essor o Neurology
Harvard Medical School
Boston, Massachusetts
ix
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