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KAUFMAN’S CLINICAL NEUROLOGY FOR PSYCHIATRISTS KAUFMAN’S CLINICAL NEUROLOGY FOR PSYCHIATRISTS Eighth Edition David Myland Kaufman, MD Departments of Neurology and Psychiatry Montefiore Medical Center Albert Einstein College of Medicine Bronx, New York Howard L. Geyer, MD, PhD Department of Neurology Montefiore Medical Center Albert Einstein College of Medicine Bronx, New York Mark J. Milstein, MD Department of Neurology Montefiore Medical Center Albert Einstein College of Medicine Bronx, New York © 2017, Elsevier Inc. All rights reserved. First edition 1981 Second edition 1985 Third edition 1990 Fourth edition 1995 Fifth edition 2001 Sixth edition 2007 Seventh edition 2013 No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein). Notices Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility. With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. ISBN: 978-0-323-41559-0; E-ISBN: 978-0-323-46131-3 Senior Content Strategist: Charlotta Kryhl Senior Content Development Specialist: Ailsa Laing/Sharon Nash Senior Content Coordinator: John Leonard Senior Project Manager: Beula Christopher Senior Designer: Christian Bilbow Illustration Manager: Amy Faith Heyden Illustrator: Marie Dean Marketing Manager: Michele Milano Printed in the United States of America Last digit is the print number: 9 8 7 6 5 4 3 2 1 D EDICATION I dedicate Kaufman’s Clinical Neurology for Psychiatrists to Rita, my wife of more than 45 years, whose love has made everything possible, and our grandchildren – Lila, Owen, Aaron, Penelope, Eliana, and Benjamin. David Myland Kaufman To my parents, my wife, and my daughters, with gratitude, admiration, and love. Howard L. Geyer I dedicate this book to my husband, Chris, who always makes me laugh, and my parents, David and Nancy, who will always inspire me to make the most of my life and career. Mark J. Milstein vii A CKNOWLEDGMENTS My wife and best friend, Rita, acted as my muse by origi- framework and encouragement to pursue the writing of nally suggesting writing this book by expanding the syl- this book and undertake other academic work in the labus for my course, “Clinical Neurology for Psychiatrists,” midst of our clinical responsibilities. At the same time, and then giving me numerous ideas for each future they have grown Montefiore into a vibrant, world- edition. renowned, patient-centered, urban medical center that is David Myland Kaufman, MD the teaching hospital of the Albert Einstein College of Medicine. I am privileged to have worked with and learned from Our housestaff and faculty-colleagues at Montefiore many wonderful mentors, colleagues, and patients over Medical Center/Albert Einstein College of Medicine and the years, and am thankful for all they have taught me. I other academic medical centers have reviewed chapters am especially grateful to my wife Laurence for her love, and in other ways offered invaluable help with this edition: encouragement, and patience, during my work on this Susan Duberstein, Jelena Pavlović, Gail Solomon, Renee project and always. Monderer, Jack Farinhas, Jacqueline Bello, Judah Burns, Howard L. Geyer, MD, PhD Lisa Ferber, and Michael Kaufman. Ms. Meryl Ranzer, Mr. Barry Morden, and Ms. Ann Mannato captured the sense I would like to thank Dr. David Kaufman who not only of neurology in wonderful illustrations. taught me invaluable lessons during my residency train- The library staffs of Montefiore Medical Center Cher- ing but also brought me into this project and Dr. Steven kasky Library and the D. Samuel Gottesman Library of Herskovitz (Director of the Neuromuscular Division) the Albert Einstein College of Medicine have graciously who always pushes me to examine all angles of a problem provided us with modern-day technology information. and answer medical questions as precisely as possible. Our attorneys, Mr. Jeffrey A. Lowin, of Morris Cohen Additionally, I owe tremendous gratitude to Dr. Sheryl LLP, and Mr. H. Joseph Mello, of Winston & Strawn Haut, whose mentorship and support has helped me LLP, provided excellent council. achieve success in student and resident teaching and per- We also thank our editors at Elsevier, Charlotta Kryhl, sonal academic endeavors. Ailsa Laing, John Leonard, and Sharon Nash, who have Mark J. Milstein, MD opened their doors and provided many improvements for this edition. Finally, our thanks go to the Elsevier pro- Drs. Steven M. Safyer (President of the Medical Center), duction team for their hard work and dedication includ- Allen M. Spiegel (Dean of the Albert Einstein College of ing Beula Christopher, Christian Bilbow, and Amy Faith Medicine), Byram Karasu (Chair of Psychiatry), Herbert Heyden. Schaumburg (Chair Emeritus of Neurology), Mark David Myland Kaufman, MD Mehler (Chair of Neurology), and Michael Swerdlow Howard L. Geyer, MD, PhD (partner at Neurologic Associates) have provided the Mark J. Milstein, MD viii N A R OTES BOUT EFERENCES Most chapters provide specific references from the neu- Rosenberg, R. N., & Pascual, J. M. (2015). Rosenberg’s Molecular and rologic and general medical literature. In addition, several Genetic Basis of Neurological and Psychiatric Disease (5th ed.). New York: Elsevier. standard, well-written textbooks contain relevant infor- Schapira, A. H. V. (Ed.), (2007). Neurology and Clinical Neuroscience. mation about many topics: Philadelphia: Mosby Elsevier. Scheiber, S. C., Kramer, T. A. M., & Adamowski, S. E. (Eds.), (2003). Aminoff, M. J., & Faulkner, L. P. (Eds.), (2012). The American Board of Core Competencies for Psychiatric Practice: What Clinicians Need to Know. Psychiatry and Neurology: Looking Back and Moving Ahead. Washing- A Report of the American Board of Psychiatry and Neurology, Inc. Wash- ton: American Psychiatric Press. ington, D.C.: American Psychiatric Publishing, Inc. Biller, J. (2013). Practical Neurology DVD Review (2nd ed.). Philadelphia: Walker, A., Kaufman, D. M., Pfeffer, C., et al. (2008). Child and Ado- Wolters Kluwer. lescent Neurology for Psychiatrists. Philadelphia: Lippincott Williams & Blumenfeld, H. (2010). Neuroanatomy Through Clinical Cases (2nd ed.). Wilkins. Sunderland, MA: Sinauer. Ellison, D., Love, S., Chimelli, L., et al. (2013). Neuropathology: A Refer- Web Sites That Offer Information About Several Areas ence Text of CNS Pathology (3rd ed.). Philadelphia: Elsevier. (Sites relevant to single areas are listed in each chapter’s references and Goetz, C. G. (2011). Textbook of Clinical Neurology (3rd ed.). Philadel- in Appendix 1.) phia: WB Saunders. American Academy of Neurology’s Practice Guidelines: http://www Heilman, K. M., & Valenstein, E. (2003). Clinical Neuropsychology (5th .aan.com/professionals/practice/guideline ed.). New York: Oxford University Press. Gene Clinics: http://geneclinics.org/ Howard, J. (2013). Neurology Video Textbook. New York: DemosMedical. Medlink Neurology (a commercial neurology textbook): http://www Jones, K. L., Jones, M. C., & del Campo, M. (2013). Recognizable Pat- .medlink.com terns of Human Malformations (7th ed.). Philadelphia: Elsevier. National Institute of Health: http://health.nih.gov/category/Brainand Kanner, A. M. (2012). Depression in Neurologic Disorders. Oxford, UK: NervousSystem Wiley-Blackwell. Online Mendelian Inheritance in Man: http://www.ncbi.nlm.nih.gov/ Lyketos, C. G., Rabins, P. V., Lipsey, J. R., et al. (Eds.), (2008). Psychi- omim atric Aspects of Neurologic Diseases: Practical Approaches to Patient Care. Pubmed: http://www.ncbi.nlm.nih.gov/pubmed New York: Oxford University Press. Registry and results database of publicly and privately supported clinical Posner, J. B., Saper, C. B., Schiff, N., et al. (2007). Plum and Posner’s studies of human participants conducted around the world: http:// Diagnosis of Stupor and Coma (Contemporary Neurology Series) (4th ed.). ClinicalTrials.gov New York: Oxford University Press. Ropper, A. H., & Samuels, M. A. (Eds.), (2009). Adams and Victor’s Principles of Neurology (9th ed.). New York: McGraw Hill, Waltham Mass., Academic Press, 2014. ix P -R , P N HYSICIAN EADERS LEASE OTE Kaufman’s Clinical Neurology for Psychiatrists discusses including doing nothing. Some aspects of medical care medications, testing, procedures, and other aspects of that this book discusses are widely and successfully used medical care. Despite their purported effectiveness, many for particular purposes not approved by the Food and are fraught with side effects and other adverse outcomes. Drug Administration (FDA). Regarding these “off-label” Discussions in this book neither recommend nor offer treatments, as well as conventional ones, this book is medical advice, and they do not apply to individual reporting – not endorsing – their use by neurologists or patients. The physician, who should consult the package other physicians. Finally, because medical practices insert and the medical literature, remains responsible for rapidly evolve, readers should expect that sooner or later medications’ indications, dosage, contraindications, pre- new diagnostic criteria and treatments will replace those cautions, side effects, adverse reactions, and alternatives, discussed in this edition. x P REFACE PURPOSE Many chapters contain outlines for a bedside examina- tion; reproductions of standard bedside tests, such as the We have written Kaufman’s Clinical Neurology for Psychia- Montreal Cognitive Assessment (MoCA) and Abnormal trists – a collegial straightforward guide – from our per- Involuntary Movement Scale (AIMS), references to spective as neurologists at a major, urban academic recent medical literature, and pertinent web sites. One medical center. In a format combining traditional neuro- chapter provides a compilation of computed tomography anatomic correlations with symptom-oriented discus- (CT), magnetic resonance imaging (MRI), and positron sions, the book will assist psychiatrists in learning modern emission tomography (PET) images that other chapters neurology. It emphasizes neurologic conditions that reference. Appendices contain information pertaining are frequently occurring, common to psychiatry and to most chapters: Patient and Family Support Groups neurology, illustrative of a scientific principle, or have (Appendix 1); Costs of Various Tests and Treatments prominent psychiatric manifestations. It also includes (Appendix 2); Diseases Transmitted by Chromosome or descriptions of numerous neurologic conditions that may Mitochondria Abnormalities (Appendix 3); and Chemical underlie aberrant behavior, disturbances in mood, or cog- and Biological Neurotoxins (Appendix 4). nitive impairment – symptoms that prompt patients or In addition, the book reviews neurologic conditions medical colleagues to solicit psychiatry consultations. that have entered the public arena because, willingly or Kaufman’s Clinical Neurology for Psychiatrists does not unwillingly, psychiatrists are liable to be drawn into intend to replace comprehensive neurology textbooks or debates involving their own patients or the medical convert psychiatrists into semiprofessional neurologists; community. Psychiatrists should be well versed in the however, this book contains essential information intricacies of the following conditions that this book required of psychiatrists. describes: • Amyotrophic lateral sclerosis and multiple sclerosis as battlegrounds of assisted suicide ORGANIZATION AND CONTENT • Meningomyelocele with Arnold–Chiari malforma- tion as an indication for abortion and the value of The organization and content of Kaufman’s Clinical Neu- spending limited resources on this fatal or severely rology for Psychiatrists arose from our experience as faculty debilitating condition at the Albert Einstein College of Medicine, attending • Chronic pain as the fulcrum for legalizing marijuana physicians at Montefiore Medical Center, and supervisors and heroin of numerous neurology and psychiatry residents; consul- • Parkinson disease, spinal cord injury, and other dis- tation with our colleagues, many of whom are world- orders amenable to research and treatment with renowned physicians; and feedback from many of the stem cells 20,000 psychiatrists who have attended the course, “Clin- • Persistent vegetative state and continuing life-sup- ical Neurology for Psychiatrists,” and the more than port technology 50,000 individuals who have purchased previous editions • Cost of medical testing and treatment. of this book. Learning the material in this book should help readers prepare for examinations, perform effective ADDITIONS AND OTHER CHANGES consultations, and improve their practice and teaching. Section 1 reviews classic anatomic neurology and FOR THE EIGHTH EDITION describes how to approach patients with a suspected neu- rologic disorder, identify central or peripheral nervous The first seven editions of Kaufman’s Clinical Neurology for system disease, and correlate physical signs. Section 2 Psychiatrists have enjoyed considerable success in the discusses common and otherwise important clinical areas, United States, Canada, and abroad. The book has been emphasizing aspects a psychiatrist may encounter. Topics translated into Japanese, Italian, Korean, and Spanish. In include neurologic illnesses, such as multiple sclerosis, the eighth edition, written 3 years after the seventh, we brain tumors, strokes, and traumatic brain injury; and have clarified the presentations, discussed recent develop- common symptoms, such as headaches, chronic pain, epi- ments in many areas, and added many clinical, anatomic, lepsy, and involuntary movement disorders. For each and radiologic illustrations. To give the question-and- topic, chapters describe the relevant symptoms including answer sections greater power, we have increased the psychiatric comorbidity, easily performed office and number of questions, refined them, expanded the discus- bedside examinations, appropriate laboratory tests, dif- sions, and provided more illustrations. We have increased ferential diagnoses, and some management options. the usage of questions based on clinical vignettes because xi xii PREFACE they mimic the clinical experience and the trend of national In addition, the book reproduces neurologic test results, specialty examinations. which are also visual records, such as CT, MRI, and In a major new feature of this edition, Kaufman’s Clini- electroencephalography (EEG). cal Neurology for Psychiatrists refers to the diagnostic cri- Kaufman’s Clinical Neurology for Psychiatrists comple- teria for various neurologic disorders in the Diagnostic and ments the text with question-and-answer sections at Statistical Manual of Mental Disorder, 5th Edition (DSM-5). the end of most chapters and at the conclusion of the It compares and contrasts DSM-5 diagnostic criteria to book. Sections at the end of chapters generally refer neurologists’ diagnostic criteria, which admittedly remain to material discussed within that chapter, whereas for the most part uncodified and variable. With a few those questions at the book’s conclusion tend to require exceptions, DSM-5 criteria rely entirely on the nature comparison of neurologic disorders that have appeared and duration of symptoms, but neurologists, depending under different headings. In Chapter 4, before the on the illness, rely on genetic testing, biopsy results, question-and-answer review of the preceding chapters’ blood tests, various laboratory testing, or physical find- material, the book offers a guide to preparing for stan- ings, but only sometimes exclusively on the patient’s dardized tests. symptoms, to make a diagnosis in their field. The Albert Einstein College of Medicine and many This edition updates and expands most topics and adds other medical schools rely on similar “problem-based new ones: interactive studying” – case-based question-and-answer • New nomenclature for seizures and epilepsy problems – as the optimum meaningful and efficient • Revised diagnostic criteria for multiple sclerosis learning strategy. Not merely quizzing the reader, the • New treatments for epilepsy, Alzheimer disease, Par- book’s questions-and-answers form an integral part of the kinson disease, multiple sclerosis, and headaches learning experience. In fact, many readers find that these • New diagnostic modalities and treatments for sections are the single most informative portion of the several movement disorders book and term them “high yield.” In keeping with the • New imaging techniques visual emphasis of the book, many of the questions are • New organization of sleep–wake disorders based on visual material, including sketches of patients • Current guidelines for the diagnosis of concussions and reproductions of MRIs, CTs, and EEGs. and their management • Psychiatric comorbidity of neurologic illnesses • New paraneoplastic disorders. ONE CAVEAT Kaufman’s Clinical Neurology For Psychiatrists expects well- DIDACTIC DEVICES: THE VISUAL APPROACH educated and thoughtful readers. It demands attention AND QUESTION-AND-ANSWER SECTIONS and work, and asks them to follow a rigorous course. Readers should find the book, like the practice of medi- Kaufman’s Clinical Neurology for Psychiatrists – like much cine, complex and challenging, but at the same time rich of the practice of neurology – relies on a visual approach. and fulfilling. It provides abundant illustrations, including numerous Even with the additions of text, illustrations, and ques- sketches of “patients” that personify or reinforce clinical tions, the eighth edition of Kaufman’s Clinical Neurology descriptions, correlate the basic science with clinical find- for Psychiatrists remains manageable in size, depth, and ings, and serve as the basis for question-and-answer scope, but still succinct enough for psychiatrists to read learning. The visual approach conforms to neurologists’ and enjoy from cover to cover. predilection to “diagnose by inspection.” For example, David Myland Kaufman, MD they rely on their observations for the diagnoses of gait Howard L. Geyer, MD, PhD abnormalities, psychogenic neurologic deficits, neurocu- Mark J. Milstein, MD taneous disorders, strokes, and involuntary movements. C H A P T E R 1 F E W IRST NCOUNTER ITH A P : E ATIENT XAMINATION AND F ORMULATION Despite the ready availability of sophisticated tests, the somewhat of an art and inapplicable in several important “hands on” examination remains the fundamental aspect neurologic illnesses, such as Alzheimer disease. of neurology. Beloved by neurologists, the neurologic The examination is not only of historical interest, but examination provides a vivid portrayal of both function also remains irreplaceable in diagnosis. It consists of a and illness. When neurologists say they have seen a case functional neuroanatomy demonstration: mental status, of a particular illness, they mean that they have really seen cranial nerves, motor system, reflexes, sensation, cerebel- a patient with it. lar system, and gait (Box 1.1). This format should be When a patient’s history suggests a neurologic illness, followed during most examinations. Trainees still master- the neurologic examination may unequivocally demon- ing this structure may bring a printed copy to the patient’s strate it. Even if psychiatrists themselves do not perform bedside to serve both as a reminder and as a place to the examination, they should be able to appreciate neu- record neurologic findings. rologic signs and assess a neurologist’s conclusion. The examination usually starts with an assessment Neurologists systematically examine the nervous sys- of the mental status, because cognition is the most tem’s major components, paying particular attention to fundamental neurologic function and cognitive impair- areas of interest in an individual patient in light of his or ments may preclude an accurate assessment of other neu- her symptoms. Neurologists try to adhere to the routine rologic functions. The examiner should consider specific while avoiding omissions and duplications. Despite intellectual deficits, such as language impairment (see obvious dysfunction of one part of the nervous system, Aphasia, Chapter 8), as well as general cognitive impair- they evaluate all major areas. A neurologist can usually ment (see Dementia, Chapter 7). Tests of cranial nerves complete an initial or screening examination in 20 may reveal malfunction of nerves either individually or minutes or less and return to perform detailed or other- in groups, such as the ocular motility nerves (III, IV, and wise special testing of particular areas, such as the mental VI) or the cerebellopontine angle nerves (V, VII, and VIII) status. (see Chapter 4). The examination of the motor system is usually per- formed more to detect the pattern than the severity of EXAMINATION weakness. Whether weakness is mild to moderate (paresis) or complete (plegia), the pattern rather than severity Neurologists usually begin by noting a patient’s age, sex, offers more clues to localization. On a practical level, of and handedness, and then review the primary symptom, course, the severity of the paresis determines the patient’s present illness, medical history, family history, and social functional capacity, e.g., whether a patient walks, requires history. They explore the primary symptom, associated a wheelchair, or stays bedridden. symptoms, and possible etiologic factors. If a patient cannot When neurologists detect paresis they attempt to clas- relate the history, the neurologist might interrupt the sify its pattern. They frequently speak of three patterns. process to look for language, memory, or other cognitive If the lower face, arm, and leg on one side of the body deficits. Many chapters in Section 2 contain outlines of the are paretic, they call the pattern hemiparesis. They usually standard questions that relate to common symptoms. attribute hemiparesis to damage in the contralateral cere- After obtaining the history, the neurologist should be bral hemisphere or brainstem. They call weakness of able to anticipate the patient’s deficits and prepare to both legs paraparesis and usually attribute it to spinal cord look for disease primarily of the central nervous system damage. If the paresis predominantly involves the distal (CNS) or the peripheral nervous system (PNS). At this portion of all four limbs, distal quadriparesis, they usually point, without yielding to rigid preconceptions, the phy- ascribe it to PNS rather than CNS damage. sician should have developed some sense of the problem Eliciting two categories of reflexes assists in determin- at hand. ing whether paresis – or other neurologic abnormality Then neurologists should look for the site of involve- – originates in CNS or PNS injury. Deep tendon reflexes ment (i.e., “localize the lesion”). “Localization,” one of (DTRs) are normally present with uniform reactivity the initial goals of most neurologic examinations, is valu- (speed and forcefulness) in all limbs, but neurologic able in the majority of cases. However, it is often injury often alters their activity or symmetry. In general, 3

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