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HANKEY’S CLINICAL NEUROLOGY
 HANKEY’S THIRD EDITION

CLINICAL NEUROLOGY

Edited by
Philip B. Gorelick
MD, MPH, FACP, FAHA, FAAN, FANA
Adjunct Professor of Neurology
Davee Department of Neurology
Northwestern University Feinberg School of Medicine
Chicago, Illinois, USA
Emeritus Executive Medical Director, Mercy Health Hauenstein
Neurosciences at Saint Mary’s Hospital
Professor, Translational Neurosciences
Michigan State University College of Human Medicine
Grand Rapids, Michigan, USA
Fernando D. Testai
MD, PhD, FAHA
Associate Professor in Neurology
University of Illinois College of Medicine at Chicago
Department of Neurology and Rehabilitation
University of Illinois–Health
Chicago, Illinois, USA
Graeme J. Hankey
MBBS, MD, FRACP, FRCP, FRCPE, FAHA, FESO, FWSO, FAAHMS
Professor of Neurology
Medical School, Faculty of Health and Medical Sciences
The University of Western Australia
Consultant Neurologist
Department of Neurology, Sir Charles Gairdner Hospital
Perth, Australia
Joanna M. Wardlaw
CBE, MBChB, MRCP, FRCR, MD, FRCPE, FAHA, FESO, FWSO, FMedSci, FRSE
Professor of Applied Neuroimaging
Division of Neuroimaging Sciences
Centre for Clinical Brain Sciences and UK Dementia Research Institute
University of Edinburgh, Chancellor’s Building,
Edinburgh, UK
Third edition published 2021
by CRC Press
6000 Broken Sound Parkway NW, Suite 300, Boca Raton, FL 33487-2742
and by CRC Press
2 Park Square, Milton Park, Abingdon, Oxon, OX14 4RN
© 2021 Taylor & Francis Group, LLC
First edition published by CRC Press 2008
Second edition published by CRC Press 2014
CRC Press is an imprint of Taylor & Francis Group, LLC
This book contains information obtained from authentic and highly regarded sources. While all reasonable efforts
have been made to publish reliable data and information, neither the author[s] nor the publisher can accept any legal
responsibility or liability for any errors or omissions that may be made. The publishers wish to make clear that any
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intended for use by medical, scientific or health-care professionals and is provided strictly as a supplement to the
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Library of Congress Cataloging‑in‑Publication Data

Library of Congress Cataloging-in-Publication Data

Names: Gorelick, Philip B., editor. | Testai, Fernando D., editor. |
Hankey, Graeme J., editor. | Wardlaw, Joanna M., editor.
Title: Hankey’s clinical neurology / edited by Philip B. Gorelick, Fernando
D. Testai, Graeme J. Hankey, Joanna M. Wardlaw.
Other titles: Clinical neurology
Description: Third edition. | Boca Raton ; London : CRC Press, 2020. |
Includes bibliographical references and index. | Summary: “The rapid
expansion of clinical knowledge in the field of neurology warrants a new
edition of this highly regarded textbook of neurology. In addition to
the anchor chapters on major areas such as headache, stroke,
developmental disorders, dementia, epilepsy, acquired metabolic
disorders and others, several new chapters have been added to meet the
clinical demand for those in practice. This edition features new
chapters on neurotoxicology, neuroimaging, and neurogenetics including
basic and more advanced concepts for the practitioner. Finally, as the
health care system continues to evolve, a new chapter on population
health and systems of care reflects current practice in team care,
patient-centric approaches, and value-based care”-- Provided by publisher.
Identifiers: LCCN 2020022754 (print) | LCCN 2020022755 (ebook) | ISBN
9780367280321 (paperback) | ISBN 9780367610876 (hardcover) | ISBN
9780429299476 (ebook)
Subjects: MESH: Nervous System Diseases
Classification: LCC RC346 (print) | LCC RC346 (ebook) | NLM WL 140 | DDC
616.8--dc23
LC record available at https://lccn.loc.gov/2020022754
LC ebook record available at https://lccn.loc.gov/2020022755

ISBN: 978-0-367-28032-1 (pbk)

ISBN: 978-0-367-61087-6 (hbk)
ISBN: 978-0-429-29947-6 (ebk)
Typeset in Warnock Pro
by KnowledgeWorks Global Ltd.
 I dedicate this book in honor of my mother for her high moral standards, intellect, and love of
family for over 90 years, and to my father in his eternal resting place as a continuous inspiration.
Philip B. Gorelick
I dedicate this book to my wife, Flavia, whose support is never-ending. To my daughter, Sofia, who
is an example of dedication and kindness. To my son, Martin, who taught me what it means to be
a true fighter. To my parents, Ruben and Stella, who sacrificed everything to give their children
the privilege of an education they couldn’t access. To my unconditional fans, my sisters Alejandra
and Naiara. And, in particular, to our patients, who are an endless source of inspiration.
Fernando D. Testai
I dedicate this book in honor of my mother for her high moral standards, intellect, and love of
family for over 90 years. And to my father in his eternal resting place as a continuous inspiration.
Graeme J. Hankey
I dedicate this work to my family, in gratitude for their unwavering support, to my
many colleagues, collaborators and patients for their inspiration to me, and to the brain
scientists and medics of the future whom I hope will be inspired by this work.
Joanna M. Wardlaw
 CONTENTS

Preface.....................................................................................................viii 15. Tumors of the nervous system..............................................493

Contributors............................................................................................ix Ugur Sener, Julie E. Hammack
Abbreviations..........................................................................................xi
16. Degenerative diseases of the nervous system..................529
James A. Mastrianni, Elizabeth A. Harris, Brandon R.
1. Neurologic diagnosis................................................................... 1 Barton, Roshni Patel, Vikram G. Shakkottai, Sharan
John Dunne, Robert Edis R. Srinivasan

2. Tools for the diagnosis and management of 17. Nutritional deficiencies.......................................................... 615
nervous system diseases............................................................ 53 Deepa Bhupali, Fernando D. Testai
William McAuliffe, Elizabeth Berry-Kravis, Gian D.
Pal, Deborah A. Hall 18. Acquired encephalopathies...................................................629
Herman Sullivan, Muhammad U. Farooq
3. Population health and systems of neurological care..........81
Philip B. Gorelick, Jong S. Kim, Hee-Joon Bae 19. Neurotoxicology........................................................................641
Sean D. McCann, Trevonne M. Thompson
4. Disorders of consciousness...................................................... 89
Rick Gill, Sean Ruland 20. Disorders of circulation of the
cerebrospinal fluid....................................................................651
5. Epilepsy........................................................................................115 Edward A. Michals
Donald C. Barr, Andres M. Kanner
21. Cranial neuropathies I, V, and VII–XII.............................667
6. Headache..................................................................................... 143 Carlen Yuen, Helene Rubeiz
Peter J. Goadsby
22. Cranial neuropathies II, III, IV, and VI.............................693
7. Vertigo..........................................................................................163 Tanyatuth Padungkiatsagul, Heather E. Moss
Christopher C. Glisson, Jorge C. Kattah
23. Spinal cord disease................................................................... 717
8. Hyperkinetic movement disorders......................................173 Ryan Jacobson, Allison Osen
Morales-Briceno Hugo, Victor S.C. Fung, Annu
Aggarwal, Philip Thompson 24. Autonomic nervous system disorders................................ 749
Robert Henderson, Judy Spies
9. Developmental diseases of the nervous system...............205
James H. Tonsgard, Nikolas Mata-Machado 25. Diseases of the peripheral nerve and
mononeuropathies....................................................................757
10. Hereditary and metabolic diseases of the central Diana Mnatsakanova, Charles K. Abrams
nervous system in adults.........................................................251
Sho T. Yano, Kenneth Silver 26. Neuromuscular junction disorders..................................... 819
Diana Mnatsakanova, Qin Li Jiang
11. Trauma of the brain and spinal cord..................................285
Fernando D. Goldenberg, Ali Mansour 27. Muscle disorders.......................................................................835
Kourosh Rezania, Peter Pytel, Betty Soliven
12. Stroke and transient ischemic attacks
of the brain and eye..................................................................297 28. Sleep–wake disorders..............................................................875
Graeme J. Hankey Margaret Kay-Stacey, Eunice Torres-Rivera,
Phyllis C. Zee
13. Neuroinfectious diseases: Infections
of the central nervous system................................................409 Index.......................................................................................................915
Jeremy D. Young, Jesica A. Herrick, Scott Borgetti,
Michael D. Carrithers

14. Inflammatory disorders of the nervous

system...........................................................................................473
Neil Scolding

vii
 PREFACE

We welcome you to the third edition of Hankey’s Clinical or other neurologic diagnostic results to be utilized in this tome.
Neurology. Some 7 years have been passed since we published Finally, and in advance, we thank those who may utilize the text
the second edition. A substantial expansion of clinical knowl- and we welcome your feedback.
edge in our field has moved us to update the text. The many
successes of the second edition have brought the same editors Philip B. Gorelick
back to the drawing board. Professors Hankey and Wardlaw, the Fernando D. Testai
founding editors of the book have had an active hand in guid- Graeme J. Hankey
ing the third edition forward. Professors Gorelick and Testai of Joanna M. Wardlaw
the second edition have returned as lead editors to clear a path-
way for updates and new chapters. The second edition had 24 A decade has passed since the first edition of Clinical Neurology.
chapters, but the third edition has been expanded to include 28 Those who have embraced it have encouraged us to update it. The
chapters. Whereas a number of anchor chapters remain in place explosion of rigorous scientific evidence for interventions in clin-
such as headache (Goadsby), stroke (Hankey), developmental ical neurology, coupled with astonishing advances in the clinical
disorders (Tonsgard), dementia (Mastrianni), epilepsy (Kanner), neurosciences, have further inspired us to undertake a second
acquired metabolic disorders (Testai), and others that have been edition. As the initial authors (GJH and JMW) are now a decade
updated and expanded, several new chapters have been added to older and have gravitated toward greater subspecialization,
meet the clinical demand for those in practice. The third edition another couple of fellow enthusiasts (PBG and FDT) from Grand
now features a chapter on neurotoxicology as we find ourselves Rapids and Chicago, USA have joined to facilitate a re-energized,
in the midst of possible worldwide pandemics and bioterror- comprehensive, and more global, rather than Anglo-Australian,
ism. Neuroimaging, a mainstay of the neurologist’s tool box for effort. Together we have enlisted the generosity and specialist
diagnosis and management, is a separate chapter based on its expertise of our friends and colleagues throughout the world who
important role in clinical diagnosis. Neurogenetics, a burgeon- are recognized leaders in their field and who have kindly agreed
ing field, is a stand-alone chapter and includes basic and more to enlighten us with a chapter on the subject to which they are
advanced concepts for the practitioner. Finally, as the health dedicated.
care system continues to evolve, we meet a need for a primer The subjects and format of the first edition have been main-
chapter on population health and systems of care as train- tained and are complemented by the addition of a new chapter
ing programs are now emphasizing team care, patient-centric on sleep disorders. The chapter covering degenerative diseases
approaches, and value-based care. of the nervous system has now been subdivided into three main
We have maintained the original style of the first and second sections, dementias, Parkinson’s disease and parkinsonian syn-
editions of the book. The text is a combination of pithy bullet dromes, and hereditary ataxias. The cranial neuropathies chapter
points and standard prose to allow the reader to easily digest now includes an entirely new section on neuro-ophthalmology. In
concepts and key messages. In addition, the chapters are liberally addition, there are over 440 new illustrations.
spiced with summary tables, neuroimages, photomicrographs, The perspective for each chapter is also fresh, as each chap-
neuroanatomic drawings, gross and microscopic neuropatho- ter (with the exception of the chapter on stroke) has been writ-
logic specimen photos, and other graphics. The reader is further ten by one or more of our new contributors, in contrast to the
aided by the presence of summary boxes to capture essential first edition which represented the perspective of GJH and JMW.
clinical messages. Thus, chapters are crafted in a way to appeal to The purpose of the book, nevertheless, continues to focus on the
both the visuospatial and analytic functional centers of the brain, essentials for students of clinical neurology, particularly neurol-
as we stimulate the senses and learn. We hope that you will be ogists-in-training and practicing neurologists, who wish to have
stimulated to read on or use the text as an up-to-date reference ready access to a comprehensive, up-to-date, and evidence-based
source in general clinical neurology. guide to the understanding, diagnosis, and management of com-
We anticipate Hankey’s Clinical Neurology, third edition, will mon and important neurologic disorders.
be of value to medical students, physicians in training, neurol- Many of the illustrations are images taken from our own
ogy fellows, neurologist and neurosurgeon practitioners, and patients, whom we would like to thank for allowing us to photo-
advanced practice professionals (e.g. nurse practitioners and graph them or the outcome of their investigations. Furthermore,
physician assistants) who are faced with neurologic practice we would also like to thank all the current and past contribu-
challenges. The chapters contain recent literature references tors of figures (too many to list individually here) for providing
and some of the more classic ones to help guide the reader who illustrations, as indicated throughout the book. Finally, we would
wishes more information. We wish to thank all the authors who like to thank our families and colleagues for supporting us in this
were tasked to bring together up-to-date chapters on a relatively endeavor. We hope you enjoy it and we welcome any comments
short timeline to allow us to provide current information in a text and criticisms.
book format. Our publisher, CRC Press, responded to the chal-
lenge with a timely turnaround to have the book ready for circu- Philip B. Gorelick
lation while the information was still fresh. It truly took a village Fernando D. Testai
to make this happen, and we thank all who participated includ- Graeme J. Hankey
ing our generous patients who agreed to allow their likenesses Joanna M. Wardlaw

viii
 CONTRIBUTORS

Charles Abrams John Dunne Julie E. Hammack

University of Illinois at Chicago College University of Western Australia Department of Neurology
of Medicine School of Medicine Mayo Clinic in Florida
Chicago, IL, USA Royal Perth Hospital Unit Jacksonville, FL, USA
Perth, WA, Australia
Annu Aggarwal Elizabeth A. Harris
Centre for Brain and Nervous Robert Edis Department of Neurology
System Department of Neurology University of Chicago Medical
Kokilaben Dhirubhai Ambani Hospital Sir Charles Gairdner Hospital Center
and Medical Research Institute Perth, WA, Australia Chicago, IL, USA
Mumbai, India
Muhammad U. Farooq Robert Henderson
Hee-Joon Bae Mercy Health Hauenstein Royal Brisbane & Women’s
Department of Neurology Neurosciences Hospital
Cerebrovascular Disease Center Assistant Professor of Neurology UQ Centre for Clinical Research
Seoul National University College of Michigan State University College of Brisbane, QLD, Australia
Medicine Human Medicine and College of
Seoul National University Bundang Osteopathic Medicine Jesica A. Herrick
Hospital Grand Rapids, MI, USA Division of Infectious Diseases
Seongnam, Republic of Korea University of Illinois at Chicago
Victor S.C. Fung Chicago, IL, USA
Donald C. Barr Movement Disorders Unit
Department of Neurology Department of Neurology Morales-Briceno Hugo
University of Miami, Miller School of Westmead Hospital and Sydney Medical Movement Disorders Unit
Medicine School Department of Neurology
Miami, FL, USA University of Sydney Westmead Hospital and Sydney
Sydney, NSW, Australia Medical School
Brandon R. Barton University of Sydney
Rush University Medical Center Rick Gill Sydney, NSW, Australia
Jesse Brown VA Medical Center Loyola University Chicago
Chicago, IL, USA Stritch School of Medicine Ryan Jacobson
Maywood, IL, USA Department of Neurological Sciences
Elizabeth Berry-Kravis Rush University Medical Center
Department of Pediatrics Christopher C. Glisson Chicago, IL, USA
Department of Neurological Neuro-Ophthalmology Program
Sciences Mercy Health Hauenstein Qin Li Jiang
Department of Integrated Neurosciences Jesse Brown VA Medical Center
Biomedical Sciences Michigan State University Chicago, IL, USA
Rush University Medical Center Grand Rapids, MI, USA
Chicago, IL, USA Andres M. Kanner
Peter J. Goadsby Department of Neurology
Deepa Bhupali King’s College London University of Miami, Miller School of
Advocate Christ Medical Center London UK Medicine
Chicago, IL, USA University of California Miami, FL, USA
Los Angeles, CA, USA
Scott Borgetti Jorge C. Kattah
Division of Infectious Diseases Fernando D. Goldenberg University of Illinois College of
University of Illinois at Chicago Neuroscience Critical Care Section Medicine
Chicago, IL, USA University of Chicago Illinois Neurologic Institute
Chicago, IL, USA Saint Francis Medical Center
Michael D. Carrithers Peoria, IL, USA
Jesse Brown VA Hospital Deborah A. Hall
Department of Neurology and Department of Neurological Sciences Margaret Kay-Stacey
Rehabilitation Department of Integrated Biomedical Northwestern University Feinberg School
Physiology and Biophysics Sciences of Medicine
University of Illinois at Chicago Rush University Medical Center Chicago, IL, USA
Chicago, IL, USA Chicago, IL, USA

ix
x Contributors

Jong S. Kim Gian D. Pal Sharan R. Srinivasan

Department of Neurology Department of Neurological Sciences University of Michigan
Asan Medical Center Rush University Medical Center Ann Arbor, MI, USA
University of Ulsan Chicago, IL, USA
Seoul, South Korea Herman Sullivan
Roshni Patel Mercy Health Hauenstein Neurosciences
Ali Mansour Rush University Medical Center Michigan State University College of
Neuroscience Critical Care Section Chicago, IL, USA Human Medicine and College of
University of Chicago Osteopathic Medicine
Chicago, IL, USA Peter Pytel Grand Rapids, MI, USA
Department of Pathology
James A. Mastrianni University of Chicago Philip Thompson
Center for Comprehensive Care and Chicago, IL, USA The University of Adelaide
Research on Memory Disorders Adelaide, SA, Australia
Department of Neurology Kourosh Rezania
University of Chicago Pritzker School of Department of Neurology Trevonne M. Thompson
Medicine University of Chicago Emergency Medicine and Medical
Chicago, IL, USA Chicago, IL, USA Toxicology
University of Illinois College of Medicine
Nikolas Mata-Machado
University of Illinois at Chicago Helene Rubeiz Chicago, IL, USA
Chicago, IL, USA University of Chicago
Chicago, IL, USA James H. Tonsgard
William McAuliffe Pediatric Neurology
Department of the Neurological Sean Ruland University of Chicago College
Intervention and Imaging Service of Professor of Neurology Chicago, IL, USA
WA (NIISWA) Loyola University Chicago
Perth, WA, Australia Stritch School of Medicine Eunice Torres-Rivera
Maywood, IL, USA Northwestern University McGaw
Sean D. McCann Chicago, IL, USA
Emergency Medicine and Medical Neil Scolding
Toxicology Learning and Research, Southmead Sho T. Yano
University of Illinois College of Medicine Hospital Pediatric Neurology and Medical
Chicago, IL, USA University of Bristol Genetics
Bristol, UK Bethesda, MD, USA
Edward A. Michals
University of Illinois Ugur Sener Jeremy D. Young
Chicago, IL, USA West Virginia University Division of Infectious Diseases
Morgantown, WA, USA The Ohio State University Wexner
Diana Mnatsakanova Medical Center
University of Illinois at Chicago College Vikram G. Shakkottai Columbus, OH, USA
of Medicine University of Michigan
Chicago, IL, USA Ann Arbor, MI, USA Carlen Yuen
University of Chicago
Heather E. Moss
Kenneth Silver Chicago, IL, USA
Department of Ophthalmology
Shriners Hospital for Children Chicago
Department of Neurology and
Department of Pediatrics and Neurology Phyllis C. Zee
Neurological Sciences
University of Chicago Benjamin and Virginia Boshes Professor
Stanford University
Chicago, IL, USA in Neurology
Palo Alto, CA, USA
Northwestern University
Betty Soliven Feinberg School of Medicine
Allison Osen
Department of Neurology Chicago, IL, USA
Department of Neurological Sciences
University of Chicago
Rush University Medical Center
Chicago, IL, USA
Chicago, IL, USA
Judy Spies
Tanyatuth Padungkiatsagul
Royal Prince Alfred Hospital
Department of Ophthalmology
Central Clinical School
Faculty of Medicine Ramathibodi Hospital
University of Sydney School of Medicine
Mahidol University, Bangkok, Thailand
Camperdown, NSW, Australia
Department of Ophthalmology, Stanford
University
Palo Alto, CA, USA
 ABBREVIATIONS

5HT 5-hydroxytryptamine AS Angelman’s syndrome

AA anaplastic astrocytoma ASA atrial septal aneurysm
AAD atlantoaxial dislocation ASD atrial septal defect/autism spectrum disorder
AASM American Association of Sleep Medicine ASO antisense oligonucleotide
Aβ amyloid-β ASP amnestic shellfish poisoning
AC activated charcoal AST aspartate aminotransferase
Aca aceruloplasminemia AT ataxia telangiectasia
ACE angiotensin-converting enzyme ATP adenosine triphosphate
ACE-R Addenbrooke’s Cognitive Examination Revised ATM acute transverse myelitis
AChE acetylcholinesterase AVM arteriovenous malformation
AChR acetylcholine receptors AVS acute vestibular syndrome
ACTH adrenocorticotropic hormone AZA azathioprine
AD Alzheimer’s disease AZP azaspiracid shellfish poisoning
ADAMTS a disintegrin and metalloproteinase with BAEP brainstem auditory evoked potential
thrombospondin motif BAL British anti-Lewisite
ADC apparent diffusion coefficient BBB blood–brain barrier
ADCA autosomal dominant cerebellar ataxia BBS Bardet–Biedl syndrome
ADEM acute disseminated encephalomyelitis BDNGF brain-derived nerve growth factor
ADHD attention deficit hyperactivity disorder BF blood flow
ADL activities of daily living/adrenoleukodystrophy bFGF basic fibroblast growth factor
ADLP adrenoleukodystrophy protein BFP bilateral frontal PMG
ADP adenosine diphosphate BFPP bilateral frontoparietal PMG
AED antiepileptic drug BGP bilateral generalized PMG
AF atrial fibrillation BHC benign hereditary chorea
AFB acid-fast bacilli BMD Becker’s muscular dystrophy
AFP alpha-fetoprotein BMI body mass index
AHI apnea/hypopnea index BNCT boron neutron capture therapy
AICA anterior inferior cerebellar artery BP blood pressure/Bereitschaftspotential
AIDP acute inflammatory demyelinating BPAP bilevel positive airways pressure
polyradiculoneuropathy BPP bilateral perisylvian PMG
AIDS acquired immunodeficiency syndrome BPPP bilateral parasagittal parieto-occipital PMG
AION anterior ischemic optic neuropathy BPPV benign paroxysmal positional vertigo
AIP acute intermittent porphyria BRRS Bannayan–Riley–Ruvalcaba syndrome
aKGD alpha-ketoglutarate dehydrogenase BSE bovine spongiform encephalopathy
ALD adrenoleukodystrophy BSK Barbour–Stoenner–Kelly
ALDP adrenoleukodystrophy protein BTX botulinum toxin
ALS amyotrophic lateral sclerosis BUN blood urea nitrogen
ALT alanine aminotransferase BWSTT body weight–supported treadmill training
AMAN acute motor axonal neuropathy BZDs benzodiazepines
AML angiomyolipoma CADASIL cerebral autosomal dominant arteriopathy with
AMN adrenomyeloneuropathy subcortical infarcts and leukoencephalopathy
(c)AMP (cyclic) adenosine monophosphate CAM computer-assisted myelography
AMSAN acute motor and sensory axonal neuropathy CAS carotid artery stenting
ANC accessory neurenteric canal CBD corticobasal degeneration
ANCL adult neuronal ceroid lipofuscinoses CBF cerebral blood flow
AO anaplastic oligodendroglioma CBS corticobasal syndrome/cystathionine β-synthase
AOA oligoastrocytoma deficiency
ApoE apolipoprotein E CBT cognitive behavioral therapy
APP amyloid precursor protein CBV cerebral blood volume
APS antiphospholipid syndrome CDC Centers for Disease Control and Prevention
aPTT activated partial thromboplastin time CEA carotid endarterectomy/carcinoembryonic antigen
ARAS ascending reticular activating system cEEG continuous electroencephalography
ARDS adult respiratory distress syndrome CE-MRA contrast-enhanced magnetic resonance
ARI absolute risk increase angiography
ARR absolute risk reduction CGRP calcitonin gene-related peptide
ARSA arylsulfatase A CI confidence interval/cholinesterase inhibitor
ARSACS autosomal recessive spastic ataxia of CIDP chronic inflammatory demyelinating
Charlevoix–Saguenay polyneuropathy
ARX Aristaless-related homeobox CIM critical illness myopathy

xi
xii Abbreviations

CIMT constraint-induced movement therapy EBRT external beam radiation therapy

CIP critical illness polyneuropathy EBV Epstein–Barr virus
CIS clinically isolated syndrome ECG electrocardiogram/electrocardiography
CISC clean intermittent self-catheterization ECT electroconvulsive therapy
(f/i/s/v) (familial/iatrogenic/sporadic/variant) EDH extradural hematoma
CJD Creutzfeldt–Jakob disease EEG electroencephalography
CK creatine kinase EGF(R) epidermal growth factor (receptor)
CLAM cholesterol-lowering agent myopathy EIAC enzyme-inducing anticonvulsant
CM congenital myopathy EITB enzyme-linked immunoelectrotransfer blot assay
CMAP compound muscle action potential ELISA enzyme-linked immunosorbent assay
CMD congenital muscular dystrophy EM erythema migrans
CMT Charcot–Marie–Tooth disease EMD Emery–Dreifuss muscular dystrophy
CMV cytomegalovirus EMG electromyography
CNS central nervous system EMS emergency medical services
CNVs copy-number variants EOG electro-oculogram
CO carbon monoxide EPP endplate potential
COACH cerebellar vermis hypo/aplasia, oligophrenia, ataxia EPT enhanced physiologic tremor
congenital, coloboma, and hepatic fibrosis ER extended-release
COMT catechol-O-methyltransferase ERG electroretinography
CORS cerebello-oculo-renal syndrome ESR erythrocyte sedimentation rate
COX cyclo-oxygenase ESRD end-stage renal disease
CPA cerebellopontine angle ET essential tremor
CPAP continuous positive airway pressure EV eustachian valve
CPEO chronic progressive external ophthalmoplegia EVD extraventricular drain
CPK creatine phosphokinase FA Friedreich’s ataxia
CPM central pontine myelinolysis FAST Functional Assessment Staging Test
CPP cerebral perfusion pressure FDA Food and Drug Administration
CPR cardiopulmonary resuscitation FES functional electrical stimulation
Cr creatinine FFI fatal familial insomnia
CRAO central retinal artery occlusion FHM familial hemiplegic migraine
CRP C-reactive protein FIESTA fast imaging employing steady state acquisition
CRVO central retinal vein occlusion sequence
CS Cowden’s syndrome FISH florescent in-situ hybridization
CSA central sleep apnea FLAIR fluid-attenuated inversion recovery
CSF cerebrospinal fluid FMD fibromuscular dysplasia
CT computed tomography FSHD facioscapulohumeral muscular dystrophy
CTA computed tomography angiography FTA fluorescent treponemal antibody
CTV computed tomography venography FTD frontotemporal dementia
CV color vision FTLD frontotemporal lobar degeneration
CVA cerebrovascular accident FVC forced vital capacity
CVT cerebral venous thrombosis FXTAS fragile X-associated tremor/ataxia syndrome
DAI diffuse axonal injury GABA gamma-aminobutyric acid
DALY disability-adjusted life year GAD glutamic acid decarboxylase
DBP diastolic blood pressure GALC galactocerebrosidase
DBS deep brain stimulation GBM glioblastoma multiforme
DD developmental delay GBS group B streptococci/Guillain–Barré syndrome
DFA direct immunofluorescent antibody GCI glial cytoplasmic inclusion
DGC dystrophin glycoprotein complex GCS Glasgow Coma Scale
DIC disseminated intravascular coagulation GCSE generalized convulsive status epilepticus
DLB dementia with Lewy bodies GCT undifferentiated germinoma
DMD Duchenne’s muscular dystrophy GFR glomerular filtration rate
DNA deoxyribonucleic acid GI gastrointestinal
DNET dysembryoplastic neuroepithelial tumor GIST gastrointestinal stromal tumor
DNS delayed neurologic sequelae Glut 1 glucose transporter type 1 (deficiency)
DRPLA dentatorubro–pallidoluysian atrophy GMP guanosine monophosphate
DSA digital subtraction cerebral angiography GPi globus pallidus internus
DSP diarrhetic shellfish poisoning GSS Gerstmann–Straüssler–Scheinker syndrome
DSPN distal symmetric polyneuropathy GTN glyceryl trinitrate
DUB deubiquitinating enzyme GTP guanosine triphosphate
DVT deep vein thrombosis GWAS genome-wide association study
DWI diffusion-weighted imaging H&E hematoxylin and eosin
EACA epsilon-aminocaproic acid HAART highly active antiretroviral therapy
Abbreviations xiii

HAM/TSP HTLV-associated myelopathy/tropical spastic IPC intermittent pneumatic compression

paraparesis IPV inactivated poliovirus vaccine
HANAC hereditary angiopathy, nephropathy, aneurysm, and IRIS immune reconstitution inflammatory syndrome
muscle cramps IVIG intravenous immune globulin
HARP hypoprebetalipoproteinemia, acanthocytes, retinitis JCV John Cunningham virus
pigmentosa, pallidal degeneration JME juvenile myoclonic epilepsy
HBO hyperbaric oxygen KBS Klüver–Bucy syndrome
HCD hepatocerebral degeneration KD Krabbe’s disease
HCG human chorionic gonadotropin KSS Kearns–Sayre syndrome
HCP hereditary coproporphyria LAA left atrial appendage
HD Huntington’s disease LAM lymphangiomyomatosis
HDL Huntington’s disease-like LAST local anesthetic systemic toxicity
HE hepatic encephalopathy LCMV lymphocytic choriomeningitis virus
HELLP hemolysis, elevated liver enzymes, low-platelet LDD Lhermitte–Duclos disease
count syndrome LDL low-density lipoprotein
HHT hereditary hemorrhagic telangiectasia (Osler– LEMS Lambert–Eaton myasthenic syndrome
Rendu–Weber syndrome) LFT liver function testing
HHV human herpesvirus LGG low-grade glioma
HI hypomelanosis of Ito LGMD limb girdle muscular dystrophy
hIBM hereditary inclusion body myopathy LGV lymphogranuloma venereum
HIF hypoxia-inducible factor LHON Leber’s hereditary optic neuropathy
HIS head impulse sign LITAF lipopolysaccharide-induced tumor necrosis factor-α
HIT horizontal head impulse test factor
HIV human immunodeficiency virus LLN lower limit of normal
HLA human leukocyte antigen LMICs low-middle income countries
HMSN hereditary motor sensory neuropathy LMN lower motor neuron
HNPP hereditary neuropathy with liability to pressure LNS Lesch–Nyhan syndrome
palsies LNSS linear nevus sebaceous syndrome
HPE holoprosencephaly LMWH low-molecular-weight heparin
HR hazard ratio/heart rate LOC loss of consciousness
HRIG human rabies immune globulin LP lumbar puncture
HsE Hashimoto’s encephalopathy LS Leigh’s syndrome
HSP hereditary spastic paraparesis LTBI latent tuberculous infection
HSV herpes simplex virus MAO monoamine oxidase
HTIG human tetanus immune globulin MAP mean arterial pressure
HTLV human T-lymphotropic virus MAPT microtubule-associated tau gene
hyperPP hyperkalemic periodic paralysis MBP myelin basic protein
hypoPP hypokalemic periodic paralysis MCA middle cerebral artery
HZV herpes zoster virus MCI mild cognitive impairment
IBM inclusion body myositis MCM L-methylmalonyl-CoA mutase
IBPN immune-mediated brachial plexus neuropathy MCP middle cerebellar peduncle
ICA internal carotid artery MCPH microcephaly
ICCA infantile convulsions and choreoathetosis MCTD mixed connective tissue disease
ICH intracerebral hemorrhage MEG magnetoencephalography
ICP intracranial pressure MELAS mitochondrial encephalomyopathy, lactic acidosis,
ID intellectual disability and stroke-like episodes
ICU intensive care unit MEP motor evoked potential
ICVT intracranial cerebral venous thrombosis MERRF myoclonic epilepsy with ragged red fibers
IF intrinsic factor MFAP muscle fiber action potential
Ig immunoglobulin MFS Miller Fisher syndrome
IGF insulin-like growth factor MHA-TP microhemagglutination for antibodies to
IGRA interferon-γ release assay Treponema pallidum
IIH idiopathic intracranial hypertension MG myasthenia gravis
IL interleukin MGMT methylguanine-DNA methyltransferase
ILAE International League against Epilepsy MIP maximum intensity projection/maximal inspiratory
ILOCA idiopathic late-onset cerebellar ataxia pressure
IMS intermediate syndrome MJD Machado–Joseph disease
INH isoniazid MLD metachromatic leukodystrophy
INO internuclear ophthalmoplegia MLF medial longitudinal fasciculus
INR international normalized ratio MMF mycophenolate mofetil
ION ischemic optic neuropathy MMR mumps, measles, rubella
IOP intraocular pressure MMSE Mini-Mental State Examination
xiv Abbreviations

MND motor neuron disease OPCA olivopontocerebellar atrophy

MoCA Montreal Cognitive Assessment OPIDP organophosphate-induced delayed polyneuropathy
MOTSA multiple overlapping thin-slab acquisition OPV oral poliovirus vaccine
MPR multiplanar reformat OR odds ratio
MRA magnetic resonance angiography OSA obstructive sleep apnea
MRI magnetic resonance imaging OTR ocular tilt reaction
mRS modified Rankin’s score PA pernicious anemia
MRSA methicillin-resistant Staphylococcus aureus PACI partial anterior circulation infarct
MRV magnetic resonance venography PACS partial anterior circulation syndrome
MS multiple sclerosis PAF pure autonomic failure
MSA multiple system atrophy PAM potassium-aggravated myotonia
MSLT Multiple Sleep Latency Test PAS para-aminosalicylic acid/periodic acid–Schiff
MSM men who have sex with men PC phase contrast
MSPNST malignant peripheral nerve sheath tumor PCD paraneoplastic cerebellar degeneration
MTHFR methylenetetrahydrofolate reductase PCNSL primary CNS lymphoma
MTR methionine synthase PCom posterior communicating artery
MUP motor unit action potential PCR polymerase chain reaction
MuSK muscle-specific receptor tyrosine kinase PCV vincristine
MUT methylmalonyl-CoA mutase PD Parkinson’s disease
MWT maintenance of wakefulness test PDD Parkinson’s disease dementia
MZ monozygotic/marginal zone PDGF platelet-derived growth factor
NAAT nucleic acid amplification test PDHC pyruvate dehydrogenase complex
NAc neuroacanthocytosis PDW proton density weighted
NAD nicotinamide adenine dinucleotide PE plasma exchange/pancreatic encephalopathy
NADP nicotinamide adenine dinucleotide phosphate PEG percutaneous endoscopic gastrostomy
NAION nonarteritic anterior ischemic optic neuropathy PEM paraneoplastic encephalomyelitis
NARP neurogenic weakness with ataxia and retinitis PEO progressive external ophthalmoplegia
pigmentosa PET positron emission tomography
NBIA neurodegeneration with brain iron accumulation PFK phosphofructokinase
NCDs noncommunicable diseases PFO patent foramen ovale
NCL neuronal ceroid lipofuscinosis PHTS PTEN hamartoma tumor syndrome
NCS nerve conduction studies PION posterior ischemic optic neuropathy
NCSE nonconvulsive status epilepticus Pi-TON posterior indirect traumatic optic neuropathy
NDT neurodevelopmental therapy PKAN pantothenate kinase–associated neurodegeneration
NF neuroferritinopathy PLED periodic lateralized epileptiform discharge
NF-1 neurofibromatosis 1 PLEX plasmapheresis
NFT neurofibrillary tangle PLM periodic leg movement
NFG nerve growth factor PLMD periodic leg movement disorder
NFLE nocturnal frontal lobe epilepsy PMA progressive myoclonic ataxia
NGGCT nongerminomatous germ cell tumor PME progressive myoclonic epilepsy
NHL non–Hodgkin’s lymphoma PMG polymicrogyria
NIF negative inspiratory pressure PML progressive multifocal leukoencephalopathy
NIH-SS National Institutes of Health Stroke Scale PMN polymorphonuclear
NIID neuronal intranuclear inclusion disease PMzD Pelizaeus–Merzbacher disease
NMDA N-methyl-d-aspartate PNET primitive neuroectodermal tumor
NMJ neuromuscular junction PNFA progressive nonfluent aphasia
NMO neuromyelitis optica PNH-PMG PMG associated with nodular heterotopia
NMS neuroleptic malignant syndrome POCI posterior circulation infarct
NO nitrous oxide POCS posterior circulation syndrome
NPC Niemann–Pick type C POEMS polyneuropathy, organomegaly, endocrinopathies,
NPDS National Poison Data System M-protein, skin changes including thickening and
NPH normal pressure hydrocephalus hyperpigmentation, clubbing of the fingers
NPHP nephronophthisis POST positive occipital sharp transients of sleep
NSAIDs nonsteroidal anti-inflammatory drugs POVL postoperative visual loss
NSE neuron-specific enolase PP preplate zone/perfusion pressure
NSP neurotoxic shellfish poisoning PPA primary progressive aphasia
NTD neural tube defect PPRF paramedian pontine reticular formation
O-AA organic amino aciduria PRES posterior reversible encephalopathy syndrome
OAA oculomotor apraxia PRG pontine respiratory group
OCD obsessive compulsive disorder PrP prion protein
ONH optic nerve head PSC primary stroke center
OP opening pressure/organophosphate PSN paraneoplastic sensory neuropathy
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