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a LANGE medical book Clinical Neurology & Neuroanatomy A Localization-Based Approach Second Edition Aaron L. Berkowitz, MD, PhD New York Chicago San Francisco Athens London Madrid Mexico City Milan New Delhi Singapore Sydney Toronto Berkowitz_FM_pi-xvi.indd 1 14/04/22 4:37 PM Copyright © 2022, 2017 by McGraw Hill LLC. All rights reserved. Except as permitted under the United States Copyright Act of 1976, no part of this publication may be reproduced or distributed in any form or by any means, or stored in a database or retrieval system, without the prior written permission of the publisher. ISBN: 978-1-26-045337-9 MHID: 1-26-045337-5 The material in this eBook also appears in the print version of this title: ISBN: 978-1-26-045336-2, MHID: 1-26-045336-7. eBook conversion by codeMantra Version 1.0 All trademarks are trademarks of their respective owners. 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Under no circumstances shall McGraw-Hill Education and/or its licensors be liable for any indirect, incidental, special, punitive, consequential or similar damages that result from the use of or inability to use the work, even if any of them has been advised of the possibility of such damages. This limitation of liability shall apply to any claim or cause whatsoever whether such claim or cause arises in contract, tort or otherwise. This 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 of Neurology, but in the art of Medicine. The students and residents at Harvard Medical School and the Partners Neurology residency program (Boston); the residents and faculty at Hôpital Universitaire de Mirebalais, Hôpital St. Nicolas de St. Marc, and Hôpital St. Boniface (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. The patients with and through whom I learned the practice of neurology and medicine, and whose courage in the face of suffering inspires us to learn more about their diseases, teach what we learn to others, and serve them and their families to the best of our abilities. My wife Nina, whose boundless support, encouragement, and companionship have been both a sustaining force and a source of great joy. My father (in memoriam), who instilled in me a passion for science, medicine, and service. Berkowitz_FM_pi-xvi.indd 3 14/04/22 4:37 PM Berkowitz_FM_pi-xvi.indd 4 14/04/22 4:37 PM Contents Foreword ix Preface to the First Edition xi Preface to the Second Edition xiii Acknowledgments xv I 4 The Motor and Somatosensory Pathways & P A R T Approach to Weakness and NEUROANATOMY & Sensory Loss 33 NEUROANATOMIC The Corticospinal Tracts 33 The Corticobulbar Tracts 35 LOCALIZATION 1 Somatosensory Pathways for the Body 36 Localization of Motor & Sensory Deficits 37 1 Diagnostic Reasoning in Neurology & the Neurologic History & Examination 1 5 The Spinal Cord & Approach to Localization in Neurologic Diagnosis: Determining Myelopathy 41 Where the Problem is 1 Overview of Spinal Cord Anatomy 41 Time Course in Neurologic Diagnosis: Determining Lamination of the Long Tracts in the What the Problem is 2 Spinal Cord 41 Associated Symptoms & Signs in Neurologic Spinal Cord Syndromes 42 Diagnosis 3 Spinal Cord Pathways for Bowel & Bladder Introduction to the Neurologic Examination 3 Control 47 Causes of Myelopathy 47 2 Introduction to Neuroimaging & Cerebrospinal Fluid Analysis 11 6 The Visual Pathway & Approach to Neuroimaging in Clinical Practice 11 Visual Loss 49 Overview of Neuroimaging Interpretation 12 Anatomy of the Visual Pathway 49 Interpretation of Brain CT 12 Approach to Visual Loss 51 Interpretation of Brain MRI 13 Disorders of Visual Cognition 54 Contrast-Enhanced Neuroimaging 18 7 The Cerebral Hemispheres & Vascular Imaging 19 Vascular Syndromes 55 Nuclear Medicine Studies: Positron Emission Cortical Regions 55 Tomography & Single Photon Emission Subcortical Structures: Thalamus & Computed Tomography 21 Basal Ganglia 57 Neuroimaging of the Spine 21 Arterial Supply of the Cerebral Cerebrospinal Fluid Analysis 21 Hemispheres 63 3 Overview of the Anatomy of the Nervous Clinical Syndromes Associated With Cerebral System 25 Vascular Territories 65 Central Nervous System (CNS) & Peripheral Nervous 8 The Cerebellum & Approach System (PNS) 25 to Ataxia 73 Hemispheres & Lobes of the Brain 25 Anatomy & Function of the Cerebellum 73 Gray Matter & White Matter of the Brain & Arterial Supply of the Cerebellum 77 Spinal Cord 26 Approach to Ataxia 77 The Meninges: Coverings of the Brain & Spinal Cord 26 9 The Brainstem & Cranial Nerves 81 The Cerebral Venous Sinuses 29 Overview of Brainstem Anatomy 81 The Ventricular System & Cerebrospinal Clinical Applications of Brainstem Anatomy 83 Fluid Flow 29 v Berkowitz_FM_pi-xvi.indd 5 14/04/22 4:37 PM vi Contents 10 Pupillary Control & Approach to 17 Radiculopathy, Plexopathy, & Anisocoria Cranial Nerves 2 & 3 91 Mononeuropathies of the Lower Pupillary Constriction: The Parasympathetic Pathway 91 Extremity 169 Pupillary Dilation: The Sympathetic Pathway 93 Neuroanatomy of the Lower Extremity 169 Approach to Anisocoria and Other Pupillary Lumbosacral Radiculopathy & Lumbar Abnormalities 95 Canal Stenosis 171 Lumbosacral Plexopathy 175 11 Extraocular Movements & Approach to Mononeuropathies of the Lower Extremity 176 Diplopia Cranial Nerves 3, 4, & 6 99 Approach to Foot Drop 178 Extraocular Movements I: Muscles & Their Innervation 99 Extraocular Movements II: Cranial II P A R T Nerves 3, 4, & 6 100 Extraocular Movements III: Supranuclear Control of DISEASES OF THE NERVOUS Horizontal & Vertical Gaze 105 SYSTEM 179 Approach to Diplopia 112 12 The Auditory and Vestibular Pathways & 18 Seizures & Epilepsy 179 Approach to Hearing Loss and Definitions & Causes of Seizures & Epilepsy 179 Dizziness/Vertigo Cranial Nerve 8 117 Evaluation of Patients With Seizures 180 The Auditory System 117 Clinical Features of Seizures 181 Approach to Hearing Loss 117 Electroencephalography (EEG) in the Evaluation of The Vestibular System 119 Seizures 182 Approach to Dizziness & Vertigo 122 Evaluation & Management of Patients After a First Seizure 183 13 Facial Sensory and Motor Pathways & Outpatient Management of Epilepsy 185 Approach to Facial Sensory and Special Scenarios in the Management of Seizures & Motor Deficits Cranial Nerves 5 & 7 129 Epilepsy 189 Trigeminal Nerve (Cranial Nerve 5) 129 Status Epilepticus 191 Facial Nerve (Cranial Nerve 7) 132 19 Vascular Diseases of the Brain & 14 Cranial Nerves 1, 9, 10, 11, & 12 137 Spinal Cord 195 Cranial Nerve 1 (Olfactory Nerve) 137 Overview of Ischemic Stroke & Intracerebral Cranial Nerve 9 (Glossopharyngeal) & Hemorrhage 195 Cranial Nerve 10 (Vagus) 137 Ischemic Stroke 196 Cranial Nerve 11 (Spinal Accessory) 138 Intracerebral Hemorrhage 210 Cranial Nerve 12 (Hypoglossal) 138 Central Nervous System Vascular Malformations 213 Subarachnoid Hemorrhage 215 15 The Peripheral Nervous System & Intraventricular Hemorrhage 218 Introduction to Electromyography/ Subdural Hematoma 218 Nerve Conduction Studies 141 Epidural Hematoma 218 Introduction to Anatomy & Diseases of the Peripheral Cerebral Venous Sinus Thrombosis & Cortical Nervous System 141 Vein Thrombosis 219 Anatomy & Diseases of Nerve Roots: Other Cerebrovascular Disorders 221 Radiculopathy 142 Vascular Disease of the Spinal Cord 221 Diseases of Dorsal Root Ganglia: Ganglionopathy (Sensory Neuronopathy) 146 20 Infectious Diseases of the Nervous System 227 Diseases of the Brachial or Lumbosacral Plexus: Meningitis 227 Plexopathy 146 Viral Encephalitis 233 Diseases of Peripheral Nerves 146 Focal Infectious Brain Lesions 235 Introduction to EMG & Nerve Conduction Studies 148 Infections of the Spine 239 Infections of Nerve Roots 241 16 Radiculopathy, Plexopathy, & Infectious Peripheral Neuropathies 241 Mononeuropathies of the Upper Extremity 153 Infection at the Neuromuscular Junction: Neuroanatomy of the Upper Extremity 153 Botulism 241 Cervical Radiculopathy & Cervical Stenosis 157 Infectious Myositis 242 Brachial Plexopathy 159 Neurologic Manifestations of HIV 242 Mononeuropathies of the Upper Extremity 160 Berkowitz_FM_pi-xvi.indd 6 14/04/22 4:37 PM Contents vii 21 Demyelinating Diseases of the Central 26 Headache 307 Nervous System 245 Approach to Headache 307 Multiple Sclerosis 245 Secondary Causes of Headache 307 Neuromyelitis Optica Spectrum Disorder 250 Primary Headache Disorders 308 Myelin Oligodendrocyte Glycoprotein Other Causes of Headache 312 (Mog) Antibody Disease 251 27 Peripheral Neuropathy 315 Acute Disseminated Encephalomyelitis 252 Classification & Differential Diagnosis of Peripheral Optic Neuritis 252 Polyneuropathy 315 Transverse Myelitis 253 Acute Polyneuropathy 317 22 Delirium, Dementia, & Rapidly Chronic Polyneuropathy 319 Progressive Dementia 255 28 Motor Neuron Disease 325 Approach to Altered Cognition 255 Overview of Motor Neuron Disease 325 Delirium 255 Amyotrophic Lateral Sclerosis (ALS) & Dementia 257 Its Variants 325 Rapidly Progressive Dementia 263 Spinal Muscular Atrophy (SMA) & Spinal Bulbar 23 Movement Disorders 267 Muscular Atrophy (Kennedy’s Disease) 327 Introduction to Movement Disorders 267 29 Disorders of the Neuromuscular Junction 329 Tremor 269 Myasthenia Gravis 329 Myoclonus 270 Congenital Myasthenic Syndromes & Neonatal Chorea 271 Myasthenia Gravis 332 Dystonia 273 Lambert-Eaton Myasthenic Syndrome 332 Tics, Tourette’s Syndrome, & Sterotypies 274 Parkinsonism, Parkinson’s Disease, & 30 Diseases of Muscle 335 Parkinson-Plus Syndromes 274 Causes of Muscle Disease 335 Functional Movement Disorders 281 Clinical Features of Muscle Disease 335 Other Movement Disorders 281 Laboratory Testing in Muscle Disease 336 Inflammatory Myopathies 336 24 Neoplastic and Paraneoplastic Muscular Dystrophies 337 Disorders of the Nervous System & Distal Myopathies (Distal Muscular Dystrophies) 339 Neurologic Complications of Congenital Muscular Dystrophies & Congenital Chemotherapy and Radiation Therapy 285 Myopathies 340 Intracranial Tumors 285 Metabolic Myopathies 340 Tumors of the Spine 291 Mitochondrial Myopathies 341 Tumors of the Cranial Nerves 292 Hyperkalemic & Hypokalemic Periodic Paralysis 341 Tumors of the Peripheral Nervous System 292 Muscle Disease Due to Systemic Diseases & Neurocutaneous Syndromes & Associated Nervous Medications 343 System Neoplasms 293 31 Leukodystrophies & Mitochondrial Neurotoxicity of Chemotherapy & Radiation Therapy 293 Diseases 345 Paraneoplastic/Antibody-Mediated Syndromes of Leukodystrophies 345 the Nervous System 296 Mitochondrial Diseases 345 Cerebrovascular Complications of Cancer 299 25 Disorders of Intracranial Pressure 301 Increased Intracranial Pressure (Intracranial Index 349 Hypertension) 301 Decreased Intracranial Pressure (Intracranial Hypotension) 305 Berkowitz_FM_pi-xvi.indd 7 14/04/22 4:37 PM Berkowitz_FM_pi-xvi.indd 8 14/04/22 4:37 PM Foreword So much of neurology exists only “in use”. This is the existing books of its size and scope because of the thoughtful- neurology that is practiced in the clinics, wards, and offices of ness with which the knowledge about diseases and neurological seasoned clinicians and cannot be found in large encyclopedic conditions has been assembled. He gets right down to business, textbooks of neurology or smaller monographs intended for addressing almost every major point that is encountered on the medical students. The accumulated experience of the neurolo- wards and in the clinic. gist can be distilled to a number of action items and thought A book such as this one is more suitable for neurology than processes that are challenging to articulate. for any other branch of medicine. We still depend on the inter- Dr. Aaron Berkowitz has written a book that occupies just face between our own refined clinical skills and our decisions this position. He has taken the transactional daily work of neu- regarding diagnosis and treatment. The pearls contained here rology and produced a wonderfully readable, concise, but by no about the meaning of particulars of the history and examina- means superficial book that fits well in the current pedagogic tion cannot be found elsewhere. The book makes a seamless environment. One might ask whether any book on neurology transit from these data to practical wisdom about their applica- is needed now that disembodied information is so easily avail- tion. The material is clear and avoids the ambiguity that clutters able on the web and algorithms for various signs, symptoms, most other books. In doing so, it also incorporates the latest and diseases abound. But between information that is as often thinking from clinical trials and together, these features provide misleading as it is useful, and the storehouse of wisdom accu- one of the best modern outlooks on the pragmatic practice of mulated over a long career, sits a great body of neurological neurology. knowledge. It is this assembled knowledge that allows us to effi- It takes a certain outlook on pedagogy and practice to pro- ciently move through the workday and can be taught to students duce such a book. Dr. Berkowitz has more than succeeded, and and residents during their rotations. Berkowitz’s book is more I find myself looking at a number of the chapters over and over than a compendium or teaching guide and is far superior to to reorient myself to solid teaching and practice. Allan H. Ropper, MD Professor of Neurology Harvard Medical School Boston, Massachusetts ix Berkowitz_FM_pi-xvi.indd 9 14/04/22 4:37 PM