Atlas of Sleep Medicine Sudhansu Chokroverty, SECOND EDITION MD, FRCP, FACP Professor and Co-Chair of Neurology Program Director Clinical Neurophysiology and Sleep Medicine New Jersey Neuroscience Institute at JFK Medical Center and Seton Hall University Edison, New Jersey Clinical Professor of Neurology Robert Wood Johnson Medical School New Brunswick, New Jersey Robert J. Thomas, MD, MMSc Associate Professor of Medicine Harvard Medical School Department of Medicine Division of Pulmonary, Critical Care, and Sleep Beth Israel Deaconess Medical Center Boston, Massachusetts iii 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899 ATLAS OF SLEEP MEDICINE ISBN: 978-1-4557-1267-0 Copyright © 2014, 2005 by Saunders, an imprint of Elsevier Inc. Video Vignettes 1 to 8, “Narcolepsy,” “Obstructive Sleep Apnea Associated With Cerebral Hypoxemia,” “The Rapid Eye Movement Sleep Behavior Disorder Leading to a Subdural Hemorrhage,” “Isolated Sleep Paralysis,” “Confusional Arousals,” “Sleepwalking,” “Hypnagogic Hallucination,” and “Sleep Terrors” by Mark Eric Dyken. Mark Eric Dyken retains copyright to his original video vignettes. No part of this publication may be reproduced or transmitted in any form or by any means, elec- tronic 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 treat- ment may become necessary. Practitioners and researchers must always rely on their own experi- ence 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, instruc- tions, or ideas contained in the material herein. Library of Congress Cataloging-in-Publication Data Chokroverty, Sudhansu, author. Atlas of sleep medicine / Sudhansu Chokroverty, Robert J. Thomas. -- Second edition. p. ; cm. Includes bibliographical references and index. ISBN 978-1-4557-1267-0 (hardback : alk. paper) I. Thomas, Robert J. (Professor of medicine), author. II. Title. [DNLM: 1. Sleep Disorders--diagnosis--Atlases. 2. Polysomnography--Atlases. WL 17] RC547 616.8’498--dc23 2013032574 Content Strategist: Helene Caprari Content Development Specialist: Maria Holman Publishing Services Manager: Anne Altepeter Senior Project Manager: Doug Turner Design Direction: Louis Forgione Printed in Canada Last digit is the print number: 9 8 7 6 5 4 3 2 1 To my wife, Manisha Chokroverty, MD, my love and gratitude for your lifelong support in my academic endeavors despite being left alone for days and weeks during the production of this and other books SC To my wife, Shubha, who has developed the steel will to deal with my never-ending hyperdrive, and to my best friend, Sudha, who played a very important part in sculpting me into who I am today RJT Preface The field of sleep medicine continues to expand exponen- behavior disorder (RBD) has expanded in terms of clinical tially and is now reaching a wider audience in the world. presentation and relevance as a prodromal manifestation There is increasing awareness of the importance of sleep of future neurodegenerative disease, driving investigators and the impact of its deprivation (either from lifestyle or to develop quantifiable diagnostic criteria in the prodromal disorders of sleep). An early diagnosis of sleep disorder is a stage. Also, now there is PSG requirement for diagnosis of must to prevent consequences that include morbidity and RBD as mentioned in the International Classification of Sleep mortality. Laboratory techniques, particularly polysomno- Disorders (ICSD), Second Edition. Furthermore, evidence graphic (PSG) study and simultaneous audio-video record- in the literature points to the heterogeneity of electromyo- ings, remain the cornerstones for a definitive diagnosis of graphic (EMG) findings in RBD patients, showing dissocia- sleep disorders. It is, therefore, important to be conversant tion among cranial and upper- and lower-limb muscle tonic with laboratory techniques and familiar with illustrations and phasic activities, requiring multiple muscle recordings. illuminating the diagnostic features of sleep disorders. Chapters from the previous edition of this book have As noted in the first edition, the fundamental purpose of been revised with new text and illustrations. Some chapters an atlas is that the text can be augmented with illustrations, have been added that contain new materials (see Chapters proving the adage that a picture is worth a thousand words. 5, 7, 13, and 16) to address recent advances in our under- In addition, in the second edition we include online video standing of sleep disorders, and topics missing from the first segments of clinical events, permitting clinical-physiological edition have been included. The goal of this edition remains correlations of normal, abnormal, and unusual sleep-related the same as in the first edition: to produce a comprehensive events. Eight video vignettes are discussed in the appendix, and contemporary atlas that provides examples of PSG and with cross references to chapters that illustrate abnormal other tracings, while also adding short video segments with motor events including nocturnal seizures, motor para- clinical vignettes that correlate behavioral- physiological somnias, and sleep-related movement disorders, as well as features for correct diagnosis of normal, abnormal, and events that are apparently normal variants or of uncertain uncommon sleep-related events. This book is intended for significance. In some of these video vignettes, duplicate fig- a multidisciplinary field of clinicians taking care of patients ures have been intentionally kept for the convenience of with sleep disorders and, therefore, should be useful to the reader. neurologists, pulmonologists, and other internists including Since the publication of the first edition of the Atlas of cardiologists, psychiatrists, psychologists, dentists, otolar- Sleep Medicine in 2005, there has been an increasing inter- yngologists, and pediatricians. It is also intended for others est and demand for visual display of interesting and educa- who have an interest in advancing the practical knowledge tionally satisfying sleep-related events and recordings. Many in sleep medicine, such as electroencephalography and PSG topics are better served by pictorial or video display. A case technologists, respiratory therapists, fellows, residents, and in point is movement triggered by sleep or preferentially medical students. occurring during sleep (e.g., parasomnias, nocturnal frontal lobe epilepsy). In addition, new sleep disorders have been Sudhansu Chokroverty, MD, FRCP, FACP described (e.g., catathrenia, complex sleep apnea, alternat- Robert J. Thomas, MD, MMSc ing leg muscle activation) and the spectrum of REM sleep vii Acknowledgments We are most grateful to the contributing authors for their at Elsevier, for their time, patience, help, and toleration scholarly contributions. We would like to acknowledge Else- of the editors’ idiosyncrasies. We also wish to thank Jenny vier’s Adrianne Brigido, who initiated the project but left to Rodriguez, secretary at the NJ Neuroscience Institute, for accept a position in another department, and Helene Cap- help with typing. rari, this title’s current content strategist, for her dedication Finally, our patients and trainees deserve our thanks for and professionalism; two content development specialists continuously challenging our conventional wisdom, driving (Julia Bartz, Gabrielle Goodman White) who were initially us to deliver the highest standard of care and education, and assigned to this project to try to speed up the process with shaking our foundations when we thought we “got it right.” utmost dedication before they left for other assignments; Maria Holman, the current content development special- Sudhansu Chokroverty, MD, FRCP, FACP ist; Doug Turner, senior project manager; Anne Altepeter, Robert J. Thomas, MD, MMSc publishing services manager; and Louis Forgione, designer, ix Contributors FERNANDA R. ALMEIDA, DDS, MSc, PhD THIEN THANH DANG-VU, MD, PHD Department of Oral Health Sciences Neurologist and Assistant Professor Faculty of Dentistry Center for Studies in Behavioral Neurobiology University of British Columbia Department of Exercise Science Vancouver, British Columbia, Canada Concordia University Montreal, Quebec, Canada ALON Y. AVIDAN, MD, MPH Professor of Neurology FERNANDO DE PAOLIS, MD Director, UCLA Sleep Disorders Center Sleep Disorders Center Director, UCLA Neurology Clinic Department of Neuroscience David Geffen School of Medicine at UCLA University of Parma Los Angeles, California Parma, Italy SIDDHARTH BAJPAI, MB BS, MS MARTIN DESSEILLES, MD, PhD Chief Resident Psychiatry Head, Department of Psychology Department of Psychiatry University of Namur Medical School Roy J. and Lucille A. Carver College of Medicine Namur, Belgium University of Iowa Iowa City, Iowa MARK ERIC DYKEN, MD, FAHA, FAASM Professor of Neurology SUSHANTH BHAT, MD Director, Department of Neurology Sleep Disorders Center Assistant Attending Roy J. and Lucille A. Carver College of Medicine New Jersey Neuroscience Institute at JFK Medical Center University of Iowa and Seton Hall University Iowa City, Iowa Edison, New Jersey RAFFAELE FERRI, MD JEFFREY BOYLE, MD, PhD Sleep Research Centre Chief Resident Department of Neurology I.C. Department of Neurology Sleep Disorders Center Oasi Research Institute (IRCCS) Roy J. and Lucille A. Carver College of Medicine Troina, Italy University of Iowa Iowa City, Iowa JOHN A. FLEETHAM, MD, FRCP(C) Professor SUDHANSU CHOKROVERTY, MD, FRCP, FACP Department of Medicine Professor and Co-Chair of Neurology The University of British Columbia Program Director Vancouver, British Columbia, Canada Clinical Neurophysiology and Sleep Medicine New Jersey Neuroscience Institute at JFK Medical Center CHRISTINE L. GLENN, RPSGT, RST, REEG T and Seton Hall University Chief Clinical Laboratory Technologist Edison, New Jersey Department of Neurology Sleep Disorders Center Clinical Professor of Neurology Roy J. and Lucille A. Carver College of Medicine Robert Wood Johnson Medical School University of Iowa New Brunswick, New Jersey Iowa City, Iowa ELENA COLIZZI, MD TIMOTHY F. HOBAN Physician (Geriatrics) Professor of Pediatrics and Neurology Department of Clinical and Experimental Medicine The Michael S. Aldrich Sleep Disorders Laboratory Section of Geriatrics University of Michigan Endocrinology of Aging Unit Ann Arbor, Michigan University of Parma Parma, Italy xi xii CONTRIBUTORS BIRGIT HÖGL, MD ELI S. NEIMAN, DO Head of the Sleep Disorders Clinic Attending Neurologist and Epileptologist Department of Neurology Comprehensive Epilepsy Center Innsbruck Medical University New Jersey Neuroscience Institute Innsbruck, Austria JFK Medical Center Edison, New Jersey NIVEDITA JERATH, MD Assistant Professor of Neurology Fellow in Clinical Neurophysiology Seton Hall University School of Graduate Medical Department of Neurology Sleep Disorders Center Education Roy J. and Lucille A. Carver College of Medicine South Orange, New Jersey University of Iowa Iowa City, Iowa LIBORIO PARRINO, MD Professor of Neuroscience PATRICK LÉVY, MD, PhD Department of Neuroscience Director, HP2 Laboratory University of Parma President, Joseph Fourier University Parma, Italy Professor Locomotion Rehabilitation and Physiology Department JEAN-LOUIS PÉPIN, MD, PhD Grenoble University Hospital Head of Clinical Physiology, Sleep, and Exercise Clinic Grenoble, France HP2 Laboratory Head of the Clinical Research Team DEBORAH C. LIN-DYKEN, MD, FAAP Joseph Fourier University Clinical Associate Professor of Pediatrics Professor Division of Pediatric Neurology, Development, and Locomotion Rehabilitation and Physiology Department Behavior Grenoble University Hospital Roy J. and Lucille A. Carver College of Medicine Grenoble, France University of Iowa Iowa City, Iowa FEDERICA PROVINI, MD, PhD Assistant Professor of Neurology RAMAN K. MALHOTRA, MD IRCCS Istituto delle Scienze Neurologiche di Bologna Assistant Professor of Neurology Department of Biomedical and Neuromotor Sciences Co-Director, SLUCare Sleep Disorders Center University of Bologna Department of Neurology and Psychiatry Bellaria Hospital Saint Louis University School of Medicine Bologna, Italy St. Louis, Missouri GEORGE B. RICHERSON, MD, PhD RONEIL MALKANI, MD Professor and Head, Neurology Assistant Professor Professor, Molecular Physiology and Biophysics Department of Neurology The Roy J. Carver Chair in Neuroscience Northwestern University Feinburg School of Medicine Roy J. and Lucille A. Carver College of Medicine Chicago, Illinois University of Iowa Iowa City, Iowa MAURO MANCONI, MD, PhD Head of Service RENAUD TAMISIER, MD, PhD Sleep and Epilepsy Center Clinical Physiology, Sleep, and Exercise Clinic Neurocenter of the Southern Switzerland HP2 Laboratory Civic Hospital of Lugano Joseph Fourier University Lugano, Switzerland Associate Professor Locomotion Rehabilitation and Physiology Department PIERRE MAQUET, MD, PhD Grenoble University Hospital Professor and Director Grenoble, France Cyclotron Research Centre Department of Neurology MARIO GIOVANNI TERZANO, MD University of Liège Professor of Neurology Liège, Belgium Department of Neuroscience University of Parma JASON MAXFIELD, MD Parma, Italy Fellow in Sleep Medicine Department of Neurology Sleep Disorders Center Roy J. and Lucille A. Carver College of Medicine University of Iowa Iowa City, Iowa CONTRIBUTORS xiii ROBERT J. THOMAS, MD, MMSc MARCO ZUCCONI, MD Assistant Professor of Medicine Physician Harvard Medical School Sleep Disorders Center Instructor in Medicine, Pulmonary, Critical Care, and Department of Clinical Neurosciences Sleep Division San Raffaele Institute and Hospital Beth Israel Deaconess Medical Center Milan, Italy Boston, Massachusetts PHYLLIS C. ZEE, MD, PhD Benjamin and Virginia T. Boshes Professor in Neurology Professor of Neurobiology Director, Sleep Disorders Center Northwestern University Feinburg School of Medicine Chicago, Illinois C H A P T E R 1 Polysomnographic Recording Technique Sudhansu Chokroverty Sushanth Bhat The most important initial step in the evaluation of a Patient Preparation and Laboratory patient with sleep complaints is a detailed and focused in- Environment clinic interview and physical examination by an experienced provider, which helps generate a differential diagnosis. In The goal of a PSG study is to recreate a typical night’s sleep selected patients the diagnostic polysomnogram (PSG) for the patient so that recorded parameters are most clini- then plays a pivotal role in confirming the clinician’s suspi- cally relevant. Towards this end, most modern sleep labora- cions and helps guide further management. tories have bedrooms that are quiet, clean, comfortable, and A routine overnight PSG records multiple physiologi- tastefully decorated. Televisions, Internet and telephone cal characteristics simultaneously during sleep. In general, access, adjacent bathrooms, and individual temperature patients spend one entire night in the sleep laboratory control are standard in most laboratories. Many patients are with the goal of capturing a typical night’s sleep. The study encouraged to bring their own pillows, blankets, pajamas, assesses wakefulness and sleep stages, respiration, cardio- and reading material to improve the sense of familiarity, pulmonary function, and body movements. Electroenceph- which eases patient anxiety and makes sleep easier. Within alography (EEG), electro-oculography (EOG), and chin reason, lights-out and lights-on times (the beginning and the muscle electromyography (EMG) channels are used to stage ending of the recording) should match the patient’s regular sleep. Airflow and respiratory effort channels are used to bedtime and waking time, so as to prevent falsely shortened score sleep-disordered breathing. The finger pulse oximetry or prolonged sleep onset and rapid eye movement (REM) channel provides additional data in this regard, as well as sleep latencies. Children being studied in the sleep labora- being helpful in identifying sleep hypoxemia independent of tory may often present with special needs, and this is dis- apneic and hypopneic events. In patients undergoing contin- cussed in depth in Chapter 17. uous positive airway pressure (CPAP) titration for obstruc- It is equally important that the technologist performing the tive sleep apnea (OSA), the C-flow channel provides the study have a basic understanding of PSG equipment (includ- airflow signal, and CPAP pressure is continuously adjusted ing amplifiers, filters, sensitivities, and simple troubleshoot- during the night to eliminate respiratory events. Limb EMG ing), as well as knowledge of important sleep disorders and channels are typically placed on the legs (usually the tibialis the reason a particular patient is being studied in the sleep lab- anterior muscle) and aid in the scoring and evaluation of limb oratory. Well-designed questionnaires and a succinct clinical movements. Additional limb EMG channels may be used in summary from the referring physician help a great deal in this special montages (see Electromyography Recording During regard. With this information an experienced technician can Standard Polysomnography ). A single-channel electrocar- make the necessary protocol adjustments so that the desired diography (ECG) channel and a snore channel are part of clinical data are obtained through an efficient recording. Tech- the typical PSG setup. Video and audio recording are essen- nicians should also be ever vigilant for artifacts that may occur tial for recording position and evaluating abnormal move- during the recording and address them as soon as possible. ments and behavior in sleep (such as bruxism, catathrenia, As mentioned, simultaneous video and audio recording and various other parasomnias). Video-audio PSG (video- to monitor behavior during sleep is invaluable during the PSG) is now the recommended technique for in-labora- PSG. It is advantageous to use two cameras to view the tory evaluation of sleep disorders. Special techniques, not entire body. A low-light-level camera should be used to part of routine recording in most laboratories but used in obtain good quality video in the dark, and an infrared light selected patients, include measurements of intraesophageal source should be available after turning the laboratory lights pressure in patients with suspected upper airway resistance off. The monitoring station should have remote control that syndrome (UARS), which helps distinguish obstructive and can zoom, tilt, or pan the camera for adequate viewing, and central apneas; esophageal pH in patients with nocturnal technicians should be alert to any abnormal movements gastroesophageal reflux disease; and penile tumescence for and adjust the view accordingly. The camera should be assessment of patients with erectile dysfunction. These are mounted on the wall across from the head end of the bed. described in greater detail later. An intercom from a microphone near the patient should be 1 2 Atlas of Sleep Medicine available. Technicians should also document the nature of environmental noise, which is likely to be seen at the two any abnormal behavior and whether the patient was easily electrodes, is subtracted out and therefore cannot contami- awakened from it, and they should regularly inquire about nate the recording. The ability of the amplifier to suppress recollection of the episode, as well as dream recall (espe- an extraneous signal such as noise that is simultaneously cially in a patient with suspected REM behavior disorder). present in both electrodes is measured by the common mode rejection ratio. Ideally this ratio must exceed 1000 to 1, but most contemporary PSG amplifiers use a ratio in Technical Considerations and excess of 10,000 to 1. Polysomnography Equipment The amplifiers used consist of both alternating current Biological signals recorded during PSG are of very small (AC) and direct current (DC) amplifiers. The AC ampli- amplitude (EEG, EOG, and EMG activity is in the micro- fiers are used to record physiological characteristics showing volt range) and need to be amplified to be displayed and high frequencies, such as EEG, EOG, EMG, and ECG. The analyzed. These waveforms also need to be filtered to best AC amplifier contains both high- and low-frequency filters. visualize activity of interest and exclude artifact. PSG equip- DC amplifiers have no low-frequency filters and are typi- ment is thus basically a series of amplifiers that records and cally used to record potentials with slow frequency, such amplifies this activity and then passes it through adjustable as the output from the oximeter, the output from the pH filters for display at different sensitivity settings. meter, CPAP titration pressure changes, and intraesopha- High-frequency (or low-pass) filters attenuate all activity geal pressure readings. AC or DC amplifiers may be used to at frequencies higher than the value at which they are set, record respiratory flow and effort. while allowing lower frequency activity to pass. For exam- Sensitivity is expressed in microvolts per millimeter or ple, EEG channels are typically set with a high-frequency millivolts per centimeter. Sensitivity switches should be filter at 70 Hz. This setting would attenuate any activity adjusted to obtain sufficient amplitude for interpretation. above 70 Hz but allows lower-frequency activity to pass Sensitivity and filter settings vary according to the physi- through unchanged. Low-frequency (or high-pass) filters, ological characteristics recorded (Table 1.1). on the other hand, attenuate all activity lower than the value The standard speed for recording traditional PSG is 10 at which they are set, while allowing faster-frequency activ- mm/sec so that each monitor screen is a 30-second epoch, ity to pass. EEG channels, typically set at 0.3 Hz, would making sleep staging easiest. A 30-mm/sec speed is the tra- therefore attenuate out any activity slower than 0.3 Hz but ditional speed at which EEGs are analyzed, because it allows allow higher-frequency activity to pass through unchanged. easy identification of epileptiform activities. While review- These filters can be adjusted to eliminate known sources ing the PSG at the traditional 10-mm/sec speed, the poly- of artifact. For example, decreasing the high-frequency fil- somnographer may pick up EEG abnormalities that can be ter from 70 Hz to 35 Hz may eliminate faster-frequency better analyzed by slowing the recording down to 30 mm/ artifacts like muscle artifact, whereas increasing the low- sec. On the other hand, with experience, polysomnogra- frequency filter from 0.3 Hz to 1 Hz may attenuate slower- phers may choose a 5-mm/sec speed, rendering a 60-second frequency artifacts like sweat artifact. Most PSG amplifiers epoch, to better visualize respiratory events. also have a 60-Hz notch filter, which attenuates the main frequency while attenuating activity of surrounding fre- Electroencephalography quency less extensively. This is particularly useful in studies contaminated by 60-Hz artifact (the frequency of electrical The main purpose of EEG recording performed during PSGs activity in North America; the frequency in other countries is to distinguish between wakefulness and the various stages is usually 50 Hz), generally seen if the electrode application of sleep. This is further elaborated on in Chapter 3. Accord- and impedance are suboptimal. The use of the notch filter, ing to the 2007 AASM Manual for the Scoring of Sleep and however, is generally discouraged because some important Associated Events, a minimum of three channels (F4-M1, components in the recording, such as muscle activity and C4-M1, O2-M1) representing the right frontal, central, and epileptiform spikes, may be attenuated. Thus the technician occipital electrodes referenced to the contralateral mastoid should ideally attempt to eliminate this artifact at the time electrode is recommended, with corresponding backup of recording by reapplying electrodes and attempting to iso- electrodes over the left hemisphere (F3-M2, C3-M2, late and eliminate the source of the artifact. The differential O1-M2) referenced to the contralateral mastoid electrode, amplifier is generally sufficient to reject 60-Hz artifacts. in case of malfunction of the primary electrodes. Although PSG equipment uses differential amplifiers, which the montage just described would theoretically be suffi- amplify the potential difference between the two ampli- cient to detect a posterior dominant rhythm in wakefulness fier inputs. The result of this is that unwanted extraneous (best seen in occipital leads) and major sleep architecture Table 1.1 Filter and Sensitivity Settings for Polysomnographic Studies Characteristics High-Frequency Filter (Hz) Time Constant (sec) Low-Frequency Filter (Hz) Sensitivity Electroencephalogram 70 or 35 0.4 0.3 5-7 μV/mm Electro-oculogram 70 or 35 0.4 0.3 5-7 μV/mm Electromyogram 90 0.04 5.0 2-3 μV/mm Electrocardiogram 15 0.12 1.0 1 mV/cm to start; adjust Airflow and effort 15 1 0.1 5-7 μV/mm; adjust