a 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 Berkowitz_FM_p00i-xiv.indd 1 02/09/16 6:13 PM Copyright © 2017 by McGraw-Hill Education. 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: 978-1-25-983439-4 MHID: 1-25-983439-5. The material in this eBook also appears in the print version of this title: ISBN: 978-1-25-983440-0, MHID: 1-25-983440-9. 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. 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. Berkowitz_FM_p00i-xiv.indd 3 02/09/16 6:13 PM This page intentionally left blank Berkowitz_FM_p00i-xiv.indd 4 02/09/16 6:14 PM 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 Berkowitz_FM_p00i-xiv.indd 5 02/09/16 6:14 PM 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 Berkowitz_FM_p00i-xiv.indd 6 02/09/16 6:14 PM 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 Berkowitz_FM_p00i-xiv.indd 7 02/09/16 6:14 PM This page intentionally left blank Berkowitz_FM_p00i-xiv.indd 8 02/09/16 6:14 PM 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 Berkowitz_FM_p00i-xiv.indd 9 02/09/16 6:14 PM