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Sudhansu Chokroverty Pietro Cortelli Editors Autonomic Nervous System and Sleep Order and Disorder 123 Autonomic Nervous System and Sleep Sudhansu Chokroverty Pietro Cortelli Editors Autonomic Nervous System and Sleep Order and Disorder Editors Sudhansu Chokroverty Pietro Cortelli School of Graduate Medical Education IRCCS-ISNB, PADG1, Osp Bellaria - Pad G1 Seton Hall University DIBINEM University of Bologna South Orange, NJ Bologna USA Italy ISBN 978-3-030-62262-6 ISBN 978-3-030-62263-3 (eBook) https://doi.org/10.1007/978-3-030-62263-3 © Springer Nature Switzerland AG 2021 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland Foreword In the past several decades, a great deal has been learned about sleep, the state in which human beings spend about one third of their lives. It has become evident that sleep is crucial to the normal consolidation of memories, a function so vital that perhaps it is the long-sought reason that the phenomenon of sleep has weathered the challenges of evolution. Disordered breathing during sleep is now one of the most common medical problems diagnosed in people of all ages. Abnormal sleep is a major symptom of psychiatric disorders and may contribute to the cause of exacerbations of depression and anxiety. It is also related to weight control and vari- ous endocrine functions. Unproductive sleep is a risk factor for an array of cardiac disorders, and it is a widely held hope that one might die peacefully in one’s sleep. If so, we would have the connection between sleep and the heart to thank. It is becoming clear that there is virtually no system that is not impacted by sleep. What is the interface between the protean functions of the nervous system and sleep? Clearly, this is mainly the autonomic nervous system. The autonomic nervous system has also undergone intense study since its discovery just over 100 years ago. Yet, there has never been a single volume dedicated to the relationship of the autonomic nervous system and sleep, until now. In Autonomic Nervous System and Sleep: Order and Disorder, two distinguished experts, one in sleep and the other in the autonomic nervous system, collaborated to produce and edit an authoritative tome addressing virtually every important facet of the interface between these two interlocking, largely unconscious aspects of the function of the human ner- vous system. For the benefit of the newcomer, the book begins with the basics of the anatomy and physi- ology of the autonomic nervous system, the sleep-wakefulness network, and the control over the cardiovascular system. Part II is a review of the most important laboratory testing proce- dures used in the evaluation of patients suspected of harboring a disorder of sleep and/or the autonomic nervous system. The bulk of the book is in Part III, in which virtually every impor- tant clinical syndrome is reviewed in an authoritative manner. The volume editors have chosen a cast of superstars, each with deep expertise in the various disorders of the sleep–autonomic interface. The chapters are both scientifically rigorous and clinically useful, such that the book can be used either as a reference volume or a handbook, rigorously edited by Professors Chokroverty and Cortelli, such that it will be a classic that will be procured and valued by students, residents, faculty, and experts. Founding Chair Emeritus, Department of Neurology, Martin A. Samuels, MD Brigham and Women’s Hospital, Miriam Sydney Joseph Distinguished Professor of Neurology, Harvard Medical School, Boston, MA, USA v Preface Since its early discovery in the late nineteenth century, the autonomic nervous system (ANS), subserving a very important function of the human body along with its significance, remained largely neglected or poorly recognized by the clinicians for the next 75 years or so. It is now recognized that there is a dynamic coupling among the ANS, particularly the central auto- nomic network (CAN), somatic central (CNS), peripheral nervous system, and sleep-wake neurons in the CNS. Traditional thinking has been centered on the role of ANS in wakefulness, but there are profound changes in the ANS during sleep (normal sleep is now considered a vital function of human health). There is not a single textbook available describing this significant interaction between the ANS and sleep, although there are journal articles and a few scattered chapters in textbooks. It is therefore now an opportune moment to bring out this dynamic bidi- rectional relationship (anatomically and functionally), discussing its significance and clinical implications in a comprehensive (yet not encyclopedic) textbook. This close coupling between the ANS, sleep, and the CNS reflects a mind–body interaction during human sleep-wake states. We, the editors, have succeeded in attracting some of the world’s leading experts in the fields of autonomic and sleep medicine to contribute to this volume, which bodes well and will enhance the stature of this textbook. The volume is divided into three broad parts: I. Basics, II. Laboratory Evaluation, and III. Clinical Aspects, addressing the order and disorder of the interaction between the ANS and sleep and capturing many of the recent advances in 30 chapters. It should be useful not only to sleep specialists and senior trainees but also to general internists, pulmonary physicians, pedia- tricians, family physicians, otolaryngologists, dentists , neurosurgeons, and neuroscientists interested in patients suffering from sleep and autonomic dysfunction as well as those para- medical and ancillary staff with an interest in sleep and autonomic medicine ( e.g., nurses, technologists, and other healthcare workers). Finally, this book should be useful to both the beginners and those advanced in the field. Edison, NJ, USA Sudhansu Chokroverty, MD Bologna, Italy Pietro Cortelli, MD, PhD vii Acknowledgments First, we like to thank all the contributors for their scholarly and insightful writings and their patience and dedication to see the volume in production. Additionally, we wish to thank all those (authors, editors, and publishers) responsible for granting permission to reproduce illus- trations, tables, and boxes that have been published previously in other books and journals. We owe a great deal to Gregory Sutorius, senior editor of clinical medicine at Springer Nature, New York, NY, for his professionalism, dedication, and commitment to see the book in print as quickly as possible. We must thank Katherine Kreilkamp at Springer whose functions included formatting, preparation of proofs, and pushing through production. We also like to thank the other dedicated staff at Springer, New York, who played a significant role in produc- ing the book in its final format. The senior editor (SC) wishes to thank Chelsea Reimer, editorial assistant to the Sleep Medicine journal, for her valiant support and dedication for organizing the table of contents, keeping an eye on the contributors for prompt revision, making corrections, editing, and typ- ing, in addition to her major function as editorial assistant to the journal. Last but not least, the senior editor must acknowledge his wife, Manisha Chokroverty, MD, for her usual and unfail- ing and continued support, love, patience, and tolerance throughout the long period of writing, editing, and proof reading during the production and publication of the book. We would like to acknowledge with gratitude Professor Martin A. Samuels, Professor of Neurology at Harvard Medical School and Emeritus Chairman of Neurology at Brigham and Women’s Hospital, Boston, MA, for his thoughtful “FOREWORD” written for this book. ix Contents 1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Sudhansu Chokroverty and Pietro Cortelli Part I Basics 2 Central Autonomic Network . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 Kamal Shouman and Eduardo E. Benarroch 3 Functional Neuroanatomy of the Peripheral Autonomic Nervous System . . . . . . 19 Sudhansu Chokroverty and Sushanth Bhat 4 Functional Neuroanatomy and Mechanism of Sleep . . . . . . . . . . . . . . . . . . . . . . . . 29 Sudhansu Chokroverty 5 Physiological Changes in the Autonomic Nervous System During Sleep . . . . . . . . 43 Roberto Amici and Giovanna Zoccoli 6 Brain–Heart Interaction: Cardiovascular Reflexes . . . . . . . . . . . . . . . . . . . . . . . . . 51 Alessandro Silvani 7 Sleep and Circadian Regulation of the Autonomic Nervous System . . . . . . . . . . . 63 Massimiliano de Zambotti and Fiona C. Baker 8 Nocturnal Arousal Phenomenon and the Autonomic Nervous System . . . . . . . . . 71 Liborio Parrino Part II L aboratory Evaluation 9 Methods of Laboratory Evaluation of the Autonomic Nervous System in Wakefulness and Sleep . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79 Jacquie Baker and Kurt Kimpinski 10 Interpreting Heart Rate Variability in Sleep: Why, When, and How? . . . . . . . . . 99 Massimo Pagani, Pietro Guaraldi, Francesca Baschieri, Daniela Lucini, and Pietro Cortelli 11 Laboratory Evaluation of Sleep Disorders: PSG, MSLT, MWT, and Actigraphy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .117 Sushanth Bhat and Sudhansu Chokroverty Part III Clinical Aspects 12 An Approach to a Patient with Suspected Autonomic and Sleep Dysfunction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .131 Sudhansu Chokroverty xi xii Contents 13 Obstructive Sleep Apnea in Adults and the Autonomic Nervous System . . . . . . .159 Jose-Alberto Palma and Horacio Kaufmann 14 Primary Insomnia and Dysautonomia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .165 Luigi Ferini-Strambi, Sara Marelli, and Maria Salsone 15 Restless Legs Syndrome, Periodic Limb Movements, and Dysautonomia . . . . . .173 Marie-Hélène Pennestri, Rebecca Burdayron, and Alex Desautels 16 NREM-Related Parasomnias and Dysautonomia . . . . . . . . . . . . . . . . . . . . . . . . . .181 Federica Provini and Giuseppe Plazzi 17 Narcolepsy, Idiopathic Hypersomnia, and Dysautonomia . . . . . . . . . . . . . . . . . . .187 Lucie Barateau and Yves Dauvilliers 18 Fatal Familial Insomnia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .199 Luca Baldelli, Giovanna Calandra-Buonaura, Federica Provini, and Pietro Cortelli 19 Sleep-Related Epilepsy, Dysautonomia, and Sudden Nocturnal Death . . . . . . . . .213 Paola Proserpio, Thea Giacomini, Elio Clemente Agostoni, and Lino Nobili 20 The Postural Tachycardia Syndrome (PoTS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .229 Christopher J. Mathias, Andrew P. Owens, and Valeria Iodice 21 Congenital Central Hypoventilation Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . .239 Kevin S. Gipson and Christian Guilleminault 22 Autonomic Dysfunction in Parasomnias of REM Sleep . . . . . . . . . . . . . . . . . . . . .249 Jennifer Zitser and Mitchell G. Miglis 23 Multiple System Atrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .261 Roberto Vetrugno 24 Pure Autonomic Failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .273 Ramesh K. Khurana 25 Acute Autonomic Neuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .289 Walter Struhal 26 Familial Dysautonomia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .301 Jose-Alberto Palma and Horacio Kaufmann 27 Diabetic Autonomic Neuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .307 Anastasios Tentolouris and Nikolaos Tentolouris 28 Spinal Cord Lesion and Dysautonomia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .317 Valeria Iodice and Christopher J. Mathias 29 Dysautonomia and Sleep Dysfunction in Pediatric Practice . . . . . . . . . . . . . . . . . .327 Suresh Kotagal 30 Autonomic Regulation of Sleep-Related Gastrointestinal Function . . . . . . . . . . .333 William C. Orr and Samih Raad Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .341 Contributors Editors Sudhansu Chokroverty, MD, FRCP Hackensack Meridian Health, Neuroscience Institute at JFK, JFK University Medical Center, Edison, NJ, USA Seton Hall University & Hackensack Meridian School of Medicine at Seton Hall, South Orange, NJ, USA Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA Pietro Cortelli, MD, PhD IRCCS Istituto di Scienze Neurologiche, Bologna, Italy Department of Biomedical and NeuroMotor Sciences (DIBINEM), University of Bologna, Bologna, Italy Contributors Elio Clemente Agostoni, MD Sleep Medicine Center, Department of Neuroscience, Grande Ospedale Metropolitano Niguarda, Milan, Italy Roberto Amici, MD Department of Biomedical and NeuroMotor Sciences (DIBINEM), University of Bologna, Bologna, Italy Fiona C. Baker, PhD SRI International, Menlo Park, CA, USA Jacquie Baker, PhD University Hospital, London Health Sciences Centre, London, ON, Canada Luca Baldelli, MBBS Department of Biomedical and NeuroMotor Sciences (DIBINEM), University of Bologna, Bologna, Italy Lucie Barateau, MD Sleep-Wake Disorders Center, Department of Neurology, National Reference Network for Narcolepsy, Gui-de-Chauliac Hospital, CHU Montpellier, Montpellier, France PSNREC, University Montpellier, INSERM 1061, Montpellier, France Francesca Baschieri, MD Department of Biomedical and NeuroMotor Sciences (DIBINEM), University of Bologna, Bologna, Italy Eduardo E. Benarroch, MD Division of Autonomic Disorders, Department of Neurology, Mayo Clinic, Rochester, MN, USA Sushanth Bhat, MD Neuroscience Institute, Hackensack Meridian Health, JFK Medical Center, Seton Hall University, Edison, NJ, USA Rebecca Burdayron, BSc Department of Educational and Counselling Psychology, McGill University, Montréal, QC, Canada xiii

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