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Concise Neurology A Focused Review SECOND EDITION Alberto J. Espay, MD, MSc, FAAN, FANA Professor and Endowed Chair James J. and Joan A. Gardner Center for Parkinson’s disease and Movement Disorders Department of Neurology University of Cincinnati Cincinnati, Ohio José Biller, MD, FACP, FAAN, FAHA, FANA Professor and Chairman Department of Neurology Loyola University Chicago Stritch School of Medicine Maywood, Illinois Acquisitions Editor: Chris Teja Development Editor: Ariel Winter Editorial Coordinator: Cody Adams Production Project Manager: Bridgett Dougherty Design Coordinator: Steve Druding Manufacturing Coordinator: Beth Welsh Prepress Vendor: TNQ Technologies Copyright © 2021 Wolters Kluwer. All rights reserved. This book is protected by copyright. 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To the maximum extent permitted under applicable law, no responsibility is assumed by the publisher for any injury and/or damage to persons or property, as a matter of products liability, negligence law or otherwise, or from any reference to or use by any person of this work. shop.lww.com Dedication To my children, Landen, Caid, Isabelle, Samuel, and Elena. AJE To my grandchildren, Selim, Ira, and Oz. JB Preface to the Second Edition The birth of Concise Neurology in 2011 still counts as one of the greatest labors of love we have undertaken. If we count from the first few drafts, it took us approximately 7 years to complete. Two major objectives made our work deliberately harder. First, we decided that each page, within the realm of possibilities, was to contain a complete theme, concisely. Second, we were to avoid “grocery lists” as much as possible. This was not to be another review book but a reference that could serve both as a very short textbook as well as a thoughtful and efficient review reference. The extra work required to fulfill these objectives paid off. Concise Neurology was readily adopted by residents and general practitioners as a trusted reference. Seeing a neurology resident hand carry a worn copy very recently was a motivating stimulus to complete this second edition, in which most pages have been completely rewritten. Anticipating a second edition early on, we begun the work of updating the original book in a manner that seemed as if we may have never gone into book-- revising hibernation. From the outset, we noted the need for changes both in style and in evidence-based practice recommendations (Screenshot 1). Some tables became obsolete; some needed to be replaced by a more complicated story —or perhaps an extra page. Screenshot 1. Edits on the “Thrombolysis” section from Concise Neurology 1st ed., p. 52. Advances in neurogenetics may have alone driven at least 35% of changes enacted throughout the book, in many cases leading to a reconfiguration of the established nosology (Screenshot 2). While diseases have largely retained a phenotype-based classification, there is an emerging trend to move into a genotype-based classification, particularly for disorders in which a given phenotype can emerge in the context of many genetic etiologies (such as in the DYT classification for isolated dystonias). Screenshot 2. Edits on the “Dystonia-Plus” section from Concise Neurology 1st ed., p. 173. Perhaps this second edition can be seen as reflecting our tentative march toward the end of the clinico-pathologic axis of nosology, on which we have spent more than a century. Many autoimmune disorders now populating this edition were unknown or insufficiently characterized in the 2011 version, including the “wakeful unresponsive” encephalopathy of NMDA receptor autoantibodies or the faciobrachial dystonic seizures of anti-LGI1 antibodies. These disorders were hidden in the waste basket of limbic encephalopathies of unknown origin and were often presumed to be due to unrecognized viral agents. Nowhere is the challenge to the primacy of clinico-pathology nosology clearer than in the field of neurodegenerative diseases (Screenshot 3). Alzheimer’s, Parkinson’s, and the many related progressive dementias and parkinsonisms have remained stuck to their clinico-pathologic convergent roots despite the embracement of systems biology divergence by the wider world of medicine. We have loyally insisted on recognizing disorders by clinical criteria validated by correlations with a given set of microscopic findings on biopsy or autopsy. But we have begun to walk the talk that a clinical disorder represents many biological disruptions. Two decades after the onset of the “biomarker discovery” era for neurodegenerative diseases, we are no close to unleashing the promise of precision medicine at the bedside. We have no effective disease-modifying strategy for the most common neurodegenerative disorders. As of this writing, there have been 33 published negative trials in Alzheimer’s and nearing 20 in Parkinson’s. While many of these failures in disease modification have been followed by incremental improvements in clinical trial design, it is likely that they will require scrutinizing the underlying hypothesis and disease nosology supporting them. Materializing our first success, however small it may be, will require replacing the model of clinico-pathologic diseases with their many genetico-- biological subtypes. We are confident that a future third edition of Concise Neurology will document this leap before the 2020s come to a close. Screenshot 3. Editing work on Focal Atrophy Syndromes in Dementias from Concise Neurology 1st ed., p. 229. The second edition of Concise Neurology stays true to its roots by summarizing the ever-increasing survey of our field in the shortest, most efficient document available. Each page remains structured with the same self-contained identity as was the case with the first edition. Even if there is only time for one page of reading, we hope this will be the book to bring rewards to such a concise endeavor. As we promised the first time around, it is excellent for “a quick yet thoughtful summary of key points… particularly useful for novice or experienced neurologists held to time pressures.” We trust that every page of Concise Neurology will again reflect our dedication to both message and conduit, and serve as a practical reference to the experienced and as trustworthy springboard for further reading to the younger readers. Please take a brief time to let us know what you think may be important to consider as we start the work on what is to become the third edition in the next decade. Cheers! Alberto J. Espay ([email protected]) José Biller ([email protected]) August 18, 2019 Preface to the First Edition Throughout our training and subsequent practice in Neurology, everyone stumbles upon an enormous amount of information using many different outfits: review articles, landmark papers, course handouts, conference notes, online cases, and crowning it all, textbooks of every imaginable kind. In a few years, these sources morph into an impressive but hopelessly scattered collection of highlighted sentences, asterisked paragraphs, and handwritten footnotes. It would seem a Herculean task to revisit these sources if only to retrieve what was felt to be the four-line jest of any 20-page manuscript prior to a test, preparing for a conference, or just for the sheer pleasure of remembering the data, whenever one can bask under such joy. What follows is the account of our trip to the fountainhead. Every curricular subject matter in neurology has been identified, insightfully stripped to the core, and collected into what might be thought of as the essential body of neurological knowledge. Here they are, just about in their original spirit: lean facts devoid of rhetorical discussions but also unshackled by the oppression of bullet-fed handouts. The scope of this vade mecum of sorts is deliberately designed to lie halfway between the encyclopedic coverage of a textbook and the shallow doctrinal tidbits found in all-you-need-to-know-to-pass review books, which rely excessively on “grocery lists” for comprehensive topical coverage. Traces of biochemical reactions or glances at ultrastructural cellular organelles are shown in a fragmentary yet systematic fashion only to help engrave in memory a critical piece of data that draws on the scholarship of neurology. Those with specialized interests within our field will exercise their frustration muscles, as the outline of the text is not bound to satisfy the need for an expanded overview of specific areas of basic science progress, however exciting they have become. Risking a pedantic style, it will soon become evident a round-oriented factual collection of the basic but comprehensive neurologic knowledge we have come to learn as essential for the day-to-day practice survival and for the