Merritt’s Neurology

Contents
SECTION I
GLOBAL BURDEN OF NEUROLOGIC DISEASE 1
Section Editor: Mitchell S. V. Elkind
1 Worldwide Epidemiology of Neurologic Disease 1
Jennifer Sevush-Garcy and Mitchell S. V. Elkind
2 Global Delivery of Neurologic Care 9
Hiral Shah, Serena Spudich, and Kiran T. Thakur
SECTION II
APPROACH TO THE NEUROLOGIC PATIENT 15
Section Editor: James M. Noble
3 Neurologic History Taking, Localization, and Differential
Diagnosis 15
James M. Noble
4 The Neurologic Examination 25
James M. Noble
SECTION III
COMMON PROBLEMS IN NEUROLOGY 45
Section Editor: J. Kirk Roberts
5 Dizziness, Vertigo, and Hearing Loss 45
J. Kirk Roberts
6 Syncope 51
Tina T. Shih
7 Seizures 57
Michelle Bell, David J. Roh, and Jan Claassen
8 Pain, Numbness, and Paresthesias 64
Comana M. Cioroiu
9 Headache and Facial Pain 70
Ashhar S. Ali and Julio R. Vieira
10 Visual Disturbances 77
Heather E. Moss
11 Delirium 88
Merrick E. Miles, Christina J. Hayhurst, and
Christopher G. Hughes
12 Dementia and Memory Loss 95
Lawrence S. Honig and James M. Noble
13 Involuntary Movements 103
Sara M. Schaefer and Elan D. Louis
14 Muscle Weakness, Cramps, and Stiffness 110
Comana M. Cioroiu
15 Gait Disorders 122
Ashwini K. Rao
16 Acute Stroke: The First Hour 132
Barry M. Czeisler and Stephan A. Mayer
17 Acute Spinal Cord Syndromes 145
Natalie Weathered and Noam Y. Harel
18 Focal Mass Lesions 152
Michelle Bell, Alexander G. Khandji, and Fabio M. Iwamoto
19 Stupor and Coma 168
Jan Claassen and John C. M. Brust
20 Brain Death 179
Eelco F. M. Wijdicks
SECTION IV
DIAGNOSTIC TESTS 184
Section Editor: James M. Noble
21 Computed Tomography 184
Priya Shoor, Daniel S. Chow, and Angela Lignelli
22 Magnetic Resonance Imaging 189
Eric Newman and Angela Lignelli
23 Positron Emission Tomography and Single-Photon Emission
Computed Tomography 203
William Charles Kreisl
24 Neurovascular Ultrasound 208
Tatjana Rundek
25 Angiography and Neuroendovascular Surgery 217
Katarina Dakay, Scott M. Woolf, Gurmeen Kaur,
Daniel H. Sahlein, and Fawaz Al-Mufti
26 Electroencephalography and Evoked Potentials 230
Nicolas Gaspard and Emily J. Gilmore
27 Electromyography, Nerve Conduction Studies, and
Magnetic Stimulation 243
Louis H. Weimer
28 Autonomic Testing 255
Louis H. Weimer
29 Evaluation of the Special Senses (Vision, Hearing,
Smell, Vestibular System) 259
J. Kirk Roberts
30 Sleep Studies 264
Andrew J. Westwood and Carl W. Bazil
31 Neuropsychological Evaluation 271
Yaakov Sternxxvi Contents
32 Lumbar Puncture and Cerebrospinal Fluid Analysis 277
Kiwon Lee
33 Brain, Muscle, Nerve, and Skin Biopsy 283
John F. Crary, Thomas H. Brannagan III, and Kurenai Tanji
34 Intracranial Pressure and Neurocritical Care Monitoring 288
Charles L. Francoeur and Stephan A. Mayer
35 Genetic Testing and DNA Diagnosis 297
Jill S. Goldman and Jacinda B. Sampson
SECTION V
CEREBROVASCULAR DISEASES 301
Section Editor: Stephan A. Mayer
36 Acute Ischemic Stroke 301
Charles C. Esenwa and Stephan A. Mayer
37 Transient Ischemic Attack 320
Setareh Salehi Omran and Jose Gutierrez
38 Hypoxic-Ischemic Encephalopathy 324
Sachin Agarwal and Alexandra S. Reynolds
39 Intracerebral Hemorrhage 332
Stephan A. Mayer and Fred Rincon
40 Subarachnoid Hemorrhage 342
Stephan A. Mayer, Gary L. Bernardini, and
Robert A. Solomon
41 Cerebral Venous and Sinus Thrombosis 353
Jennifer A. Frontera and Natalie Organek
42 Vascular Malformations 361
Fawaz Al-Mufti and Stephan A. Mayer
43 Central Nervous System Vasculitis 370
Kavneet Kaur, Hussein Alshammari, Ramandeep Sahni,
Steven Marks, Stephan A. Mayer, and Fawaz Al-Mufti
44 Posterior Reversible Encephalopathy Syndrome and
Other Cerebrovascular Syndromes 384
Claire S. Riley, Sara K. Rostanski, and Joshua Z. Willey
45 Primary and Secondary Stroke Prevention 401
Charles C. Esenwa and Mitchell S. V. Elkind
SECTION VI
NEUROTRAUMA 413
Section Editor: Neeraj Badjatia
46 Concussion and Chronic Traumatic Encephalopathy 413
James M. Noble and John F. Crary
47 Traumatic Brain Injury 420
Gunjan Y. Parikh, Neeraj Badjatia, and
Stephan A. Mayer
48 Traumatic Spinal Cord Injury 441
Christopher E. Mandigo, Michael G. Kaiser, and
Peter D. Angevine
49 Traumatic Cranial and Peripheral Nerve Injury 451
Dominique M. O. Higgins, Lillian Liao,
and Christopher J. Winfree
SECTION VII
DEMENTIA 469
Section Editor: Karen S. Marder
50 Mild Cognitive Impairment 469
Lawrence S. Honig, Arash Salardini, William Charles Kreisl,
and James M. Noble
51 Alzheimer Disease 476
Lawrence S. Honig and James M. Noble
52 Frontotemporal Dementia 486
Edward D. Huey, Megan S. Barker, and Stephanie Cosentino
53 Lewy Body Dementias 492
Oren Levy, Richard A. Hickman, and Karen S. Marder
54 Vascular Dementia and Cognitive Impairment 500
Nikolaos Scarmeas and Adam M. Brickman
55 Prion Diseases 506
Boon Lead Tee and Michael Geschwind
SECTION VIII
HEADACHE AND PAIN SYNDROMES 521
Section Editor: Richard B. Lipton
56 Primary and Secondary Headache Disorders 521
Peter J. Goadsby and Richard B. Lipton
57 Facial Pain Disorders and Painful Cranial Neuralgias 538
Paul G. Mathew and Zahid H. Bajwa
58 Complex Regional Pain Syndrome 549
Steven P. Cohen, Michael L. Weinberger, and
Thomas H. Brannagan III
59 Neuropathic Pain 557
Jeffrey Shije and Thomas H. Brannagan III
SECTION IX
EPILEPSY AND PAROXYSMAL DISORDERS 567
Section Editor: Carl W. Bazil
60 Classi? cation of Seizures and Epilepsy 567
Shraddha Srinivasan and Carl W. Bazil
61 Management of Epilepsy 578
Carl W. Bazil and Shraddha Srinivasan
62 Ménière Syndrome, Benign Paroxysmal Positional Vertigo, and
Vestibular Neuritis 597
Ian S. Storper
63 Transient Global Amnesia 602
John C. M. Brust
SECTION X
CENTRAL NERVOUS SYSTEM INFECTIONS 604
Section Editor: Karen L. Roos
64 Acute Bacterial Meningitis 604
Karen L. Roos Contents xxvii
65 Brain, Spinal, and Epidural Abscess and Other Parameningeal
Infections 612
Tracey A. Cho
66 Other Bacterial Central Nervous System Infections
and Toxins 624
Kyle J. Coleman
67 Chronic Meningitis 640
Prashanth S. Ramachandran, Joseph R. Zunt,
Kelly J. Baldwin, and Michael R. Wilson
68 Parasitic Infections 653
Gustavo C. Román
69 Viral Infections 661
Shibani S. Mukerji, Maria Martinez-Lage, and
Kiran T. Thakur
70 Human Immunode? ciency Virus (HIV) and Acquired
Immunode? ciency Syndrome (AIDS) 690
Anne Damian Yacoub, Deanna Saylor, Ned Sacktor,
Kiran T. Thakur, Carolyn Barley Britton, and
Barbara S. Koppel
SECTION XI
DEMYELINATING AND INFLAMMATORY
DISEASES 711
Section Editor: David A. Ha? er
71 Multiple Sclerosis and Related CNS
Neuroimmune Diseases 711
Erin E. Longbrake and Sarah F. Wesley
72 Transverse Myelitis 736
Eoin P. Flanagan and Brian G. Weinshenker
73 Neuromyelitis Optica Spectrum Disorder 744
Iris Vanessa Marin Collazo and Brian G. Weinshenker
74 Autoimmune Encephalitis and Meningitis 755
Andrew McKeon, Divyanshu Dubey, Eoin P. Flanagan,
Anastasia Zekeridou, and Sean J. Pittock
75 Neurosarcoidosis 773
Siddharama Pawate and Barney J. Stern
SECTION XII
MOVEMENT DISORDERS 778
Section Editor: Elan D. Louis
76 Essential Tremor 778
Elan D. Louis
77 Tics and Tourette Syndrome 784
Harvey S. Singer
78 Restless Legs Syndrome 793
Brian B. Koo
79 The Dystonias 800
Hyder A. Jinnah
80 Hemifacial Spasm 820
Paul Greene
81 Myoclonus 824
Pichet Termsarasab and Steven J. Frucht
82 Hereditary and Acquired Ataxias 831
Sheng-Han Kuo and Vikram G. Shakkottai
83 Tardive Dyskinesia and Other Neuroleptic-Induced
Syndromes 849
Un Jung Kang and Stanley Fahn
84 Choreas 854
Ruth H. Walker and Joseph Jankovic
85 Huntington Disease 861
Karen S. Marder and Richard A. Hickman
86 Parkinson Disease 871
Peter LeWitt
87 Parkinson-Plus Syndromes 893
Paul Greene
SECTION XIII
NEUROMUSCULAR DISEASES 904
Section Editor: Thomas H. Brannagan III
88 Amyotrophic Lateral Sclerosis and Motor
Neuron Diseases 904
Neil A. Shneider
89 Bell’s Palsy and Cranial Neuropathies 915
Comana M. Cioroiu and Thomas H. Brannagan III
90 Mononeuropathies and Compression Neuropathies 927
Thomas H. Brannagan III
91 Acquired Peripheral Neuropathies 936
Thomas H. Brannagan III and Kurenai Tanji
92 Inherited Peripheral Neuropathies 955
Chiara Pisciotta and Michael E. Shy
93 Myasthenia Gravis and Other Disorders of the
Neuromuscular Junction 963
Christina M. Ulane
94 In? ammatory and Autoimmune Myopathies 979
Rebecca Traub, Kurenai Tanji, and Christina M. Ulane
95 Critical Illness Myopathy and Neuropathy 989
Ahmad Riad Ramadan, Michio Hirano, and
Louis H. Weimer
96 Endocrine and Toxic Myopathies 992
Christina M. Ulane
97 Periodic Paralysis and Other Channelopathies 998
Comana M. Cioroiu
98 Stiff Person Syndrome and Peripheral Nerve and Muscle
Hyperexcitability 1004
Jonathan Perk and Christina M. Ulane
99 Metabolic and Mitochondrial Myopathies in Adults 1011
Michio Hirano, H. Orhan Akman, and
Salvatore DiMauroxxviii Contents
SECTION XIV
NEUROONCOLOGY 1019
Section Editor: Tobias Walbert
100 Gliomas 1019
J. Ricardo McFaline-Figueroa and Patrick Y. Wen
101 Metastatic Tumors 1034
Rupesh Kotecha and Minesh P. Mehta
102 Meningiomas 1050
Thomas J. Kaley
103 Primary Central Nervous System Lymphoma 1056
Lakshmi Nayak and Tracy T. Batchelor
104 Tumors of the Pituitary Gland 1064
Pamela U. Freda, John Ausiello, and Jeffrey N. Bruce
105 Pineal Region Tumors 1072
Jeffrey N. Bruce
106 Acoustic Neuroma and Other Skull Base Tumors 1080
Randy S. D’Amico and Michael B. Sisti
107 Spinal Tumors 1089
David Cachia, Claudio E. Tatsui, and Mark R. Gilbert
108 Paraneoplastic Syndromes 1100
Erika Santos Horta and Tobias Walbert
109 Complications of Cancer Therapy 1106
Jasmin Jo and David Schiff
SECTION XV
HYDROCEPHALUS AND CEREBRAL
EDEMA 1114
Section Editor: Fred Rincon
110 Hydrocephalus 1114
Kumud Sharma and Fred Rincon
111 Brain Edema and Increased Intracranial Pressure 1127
Stephan A. Mayer
112 Spontaneous Intracranial Hypotension 1139
Kevin E. Immanuel, Tiffany R. Chang,
and Kiwon Lee
SECTION XVI
SPINAL CORD DISORDERS 1145
Section Editor: Paul C. McCormick
113 Intervertebral Disk Disease and Radiculopathy 1145
Hani Malone and Peter D. Angevine
114 Cervical and Lumbar Spinal Stenosis 1153
Brian J.A. Gill and Paul C. McCormick
115 Acquired and Hereditary Myelopathies 1159
Natalie Weathered and Noam Y. Harel
SECTION XVII
AUTONOMIC AND SLEEP DISTURBANCES 1170
Section Editor: Louis H. Weimer
116 Autonomic Failure, Autonomic Neuropathy, and
Orthostatic Intolerance 1170
Louis H. Weimer
117 Paroxysmal Sympathetic Hyperactivity After Acute
Brain Injury 1178
Sophie Samuel and Huimahn Alex Choi
118 Sleep Disorders 1183
Andrew J. Westwood and Carl W. Bazil
SECTION XVIII
SYSTEMIC ORGAN FUNCTION AND THE
NERVOUS SYSTEM 1195
Section Editor: Kiwon Lee
119 Heart-Brain Interactions 1195
Shouri Lahiri and Stephan A. Mayer
120 Sepsis-Associated Encephalopathy 1201
Aurélien Mazeraud, Cássia Righy, Stephan A. Mayer,
and Tarek Sharshar
121 Respiratory Support for Neurologic Diseases 1207
David B. Seder and Stephan A. Mayer
122 Endocrine Diseases and the Brain 1215
Spyridoula Tsetsou and Alexandra S. Reynolds
123 Hematologic Diseases and the Brain 1227
Andreas H. Kramer
124 Hepatic Disease and the Brain 1245
Charles L. Francoeur and Stephan A. Mayer
125 Renal Disease, Electrolyte Disorders, and the
Nervous System 1253
J. Kirk Roberts and Stephan A. Mayer
126 Gastric and Genitourinary Function and the Brain 1262
Alden Doerner Rinaldi and Charles C. Esenwa
127 Bone Diseases and the Central Nervous System 1270
Roger N. Rosenberg and Alison M. Pack
128 Malnutrition, Malabsorption, and Vitamin De? ciencies 1277
Rebecca Traub and Inna Kleyman
129 Neurologic Disease During Pregnancy 1286
Alison M. Pack
130 Neurologic Complications of Organ Transplantation 1298
Eelco F. M. Wijdicks Contents xxix
SECTION XIX
NEUROLOGIC TOXIDROMES 1303
Section Editor: John C. M. Brust
131 Alcoholism 1303
John C. M. Brust
132 Drug Intoxication and Withdrawal 1313
John C. M. Brust
133 Neurotoxicology 1319
Christopher Zammit and Stephan A. Mayer
134 Radiation Injury 1335
Matthew R. Leach and Christopher Zammit
135 Electrical and Lightning Injury 1347
Imad Khan and Christopher Zammit
136 Decompression Illness 1350
Matthew R. Leach and Christopher Zammit
SECTION XX
PEDIATRIC NEUROLOGY 1355
Section Editor: Arthur M. Mandel
137 Neonatal Neurology 1355
Arthur M. Mandel
138 Nervous System Formation and Malformation 1366
Gary D. Clark
139 Inherited Metabolic Diseases 1385
Marc C. Patterson
140 Chromosomal Disorders and Disorders of DNA 1410
Marc C. Patterson
141 The Floppy Infant 1417
Maryam Oskoui, Laurent Servais, and
Darryl C. De Vivo
142 Disorders of Motor and Cognitive Development 1424
Jason B. Carmel, Toni S. Pearson, and Reet Sidhu
143 Autism Spectrum Disorders 1433
Sylvie Goldman and Jennifer M. Bain
144 Mitochondrial Encephalomyopathies 1441
Salvatore DiMauro, Emanuele Barca, and
Michio Hirano
145 Neurocutaneous Syndromes 1456
Marc C. Patterson
146 Spinal Muscular Atrophies of Childhood 1467
Basil T. Darras, Richard S. Finkel, and Darryl C. De Vivo
147 Muscular Dystrophies and Congenital Myopathies 1474
James J. Dowling, Hernan D. Gonorazky, Dimah N. Saade,
Michael W. Lawlor, and Darryl C. De Vivo
148 Seizures and Epilepsies in Neonates and Children 1502
Tristan T. Sands and M. Roberta Cilio
149 Pediatric Stroke 1515
Sally M. Sultan and Robert H. Fryer
150 Brain Tumors in Children 1528
James H. Garvin, Jr
151 Pediatric Brain Injury and Shaken Baby Syndrome 1572
Joshua Cappell and Steven G. Kernie
152 Human Immunode? ciency Virus, Fetal Alcohol Syndrome,
and Drug Effects 1579
Claudia A. Chiriboga
SECTION XXI
PSYCHIATRY AND NEUROLOGY 1588
Section Editor: David H. Strauss
153 Psychosis 1588
Jacob S. Ballon and T. Scott Stroup
154 Mood Disorders 1596
Andrés Barrera, Licínia Ganança, Alejandro S. Cazzulino,
and Maria A. Oquendo
155 Anxiety Disorders, Posttraumatic Stress Disorder, and
Obsessive-Compulsive Disorder 1606
Franklin R. Schneier
156 Somatic Symptom Disorders 1615
Anna L. Dickerman, Philip R. Muskin, and Vivian Liu
SECTION XXII
RECOVERY AND END-OF-LIFE CARE 1629
Section Editor: Joel Stein
157 Neurologic Rehabilitation 1629
Joel Stein and Heidi Schambra
158 End-of-Life and Palliative Care in Neurology 1636
Tobias Walbert, Fred Rincon, and James M. Noble
Index 1645