Ebook Sleepy or sleepless - Clinical approach to the sleep patient: Part 1

(BQ) Part 1 book Sleepy or sleepless - Clinical approach to the sleep patient presents the following contents: Key history and physical examination findings in the sleepy patient, diagnostic tools and testing in the sleepy patient, central nervous system hypersomnias, sleep deprivation, the sleepy child,...

Sleepy or Sleepless Clinical Approach to the Sleep Patient Raman K Malhotra Editor 123 Sleepy or Sleepless Raman K Malhotra Editor Sleepy or Sleepless Clinical Approach to the Sleep Patient Editor Raman K Malhotra, MD Director, Neurology Residency Program Co-Director, SLUCare Sleep Disorders Center Director, Sleep Medicine Fellowship Assistant Professor of Neurology Saint Louis University School of Medicine St Louis, MO, USA ISBN 978-3-319-18053-3 ISBN 978-3-319-18054-0 DOI 10.1007/978-3-319-18054-0 (eBook) Library of Congress Control Number: 2015942895 Springer Cham Heidelberg New York Dordrecht London © Springer International Publishing Switzerland 2015 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, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made Printed on acid-free paper Springer International Publishing AG Switzerland is part of Springer Science+Business Media (www.springer.com) Foreword Dr Raman K Malhotra, an authority in sleep medicine education, has assembled a group of exemplary authors who superbly summarize the major sleep disturbances in an effective, accurate, and efficient manner This book consists of practical and probabilistic approaches to any patient who may present with a sleep complaint It is with this foresight that Dr Malhotra, as book editor, embarks on his uniquely imaginative symptoms-based perspective of sleep medicine Given that the majority of patients who arrive in a sleep disorders clinic often complain of excessive sleepiness and insomnia, the title of the book is very appropriate indeed It is with this in mind that this book is strategically divided into two sections: Part I focuses on “The Sleepy Patient” and is subdivided into seven chapters addressing history and physical findings, diagnostic tools and tests, and then proceeds with a discussion of the two key clinical entities: sleep disordered breathing and central nervous system hypersomnias, which most commonly present with excessive sleepiness The chapter on sleepiness in industry is indispensable given the important contributions of shift work schedule requirements on human sleep Part I concludes with two chapters covering sleep deprivation, a pervasive problem in today’s society as frequently demonstrated by National Sleep Foundation polls, and the sleepy child, which is an often neglected problem but a recent epidemic for any number of reasons Part II “The Sleepless or Restless Patient” mirrors Part I in its organizational approach covering history, physical exam, and tests in addressing these patients This is followed by a discussion of key causes of sleeplessness including insomnia, circadian rhythm disorders, and movement disorders of sleep Separate discussion is later provided covering the sleepless child and problems with sleeplessness during and after pregnancy The authors have done a remarkable job in producing a text that is eloquent, practical, and concise All are authorities on the various topics assigned to them through their intimate knowledge of the subject area, which they have not only researched, but also have contributed to the evidence for diagnostic approaches and management strategies The readership should be aware that the authors’ credibility as writers is derived from their reputable clinical expertise, as well as serving as key opinion leaders and researchers in the field v vi Foreword Every clinician, whether a busy primary care physician, a subspecialist or a trainee needs to ask patients about their sleep quality Having a “road map” within reach to empower clinicians for making appropriate and well-reasoned decision is critical Given that sleep medicine is underrepresented in graduate medical education curriculum, resources such as Sleepy or Sleepless: Approach to the Sleep Patient are indispensible Dr Malhotra and his fellow authors should be congratulated on delivering this masterful textbook and for their commitment and determination in propelling sleep medicine education and clinical care Los Angeles, CA February 25, 2015 Alon Avidan, M.D., M.P.H Preface Sleep disorders are regularly encountered in clinical practice, and when left untreated can lead to significant consequences to patients, their families, and society as a whole Unfortunately, common sleep disorders, such as sleep apnea, insomnia, and insufficient sleep, remain undiagnosed and untreated by clinicians This is partially due to patients not bringing their sleep complaints to their health care providers’ attention; however, this also highlights inadequate exposure and education about sleep disorders during medical school, residency, and fellowship training Though sleep disorders can affect almost all functions and organ systems of the body, very little time is dedicated to sleep disorders in medical education Furthermore, though some medical schools and postgraduate training programs have increased formal teaching and rotations in sleep medicine, there remain limited resources, curricula, or references available for trainees that fit their needs Most rotators want the most up-to-date information on clinical sleep medicine and the ability to review relevant materials by the end of their short rotation This textbook seeks to fill this gap as a concise textbook that medical students, residents, fellows, or health care providers who are beginners to sleep medicine can review during a rotation This text is one of the first to incorporate new classification systems such as the International Classifications of Sleep Disorders—third Edition that was released in 2014 as well as the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) To further make this text more practical, the content is separated into two sections: (1) The Sleepy Patient and (2) The Sleepless/Restless Patient With a focus first on the patient’s chief complaint, this layout will provide the reader the proper framework to perform a complete clinical evaluation of their patient’s sleep complaint, make the proper diagnosis, and then choose the most effective management for their patient Each of the two sections provides a detailed chapter on proper history-taking and key physical examination findings that will be helpful in the clinical evaluation of sleep patients In addition, I have included a chapter in each section on common screening tools, tests, and “templates” that can be used in the clinical setting to assist in the office visit Much of these tools are validated and used frequently in clinical trials and research Other tools, on the other hand, were vii viii Preface included primarily due to the expert author’s own experience in using these tools successfully when seeing sleep patients in their own clinical practice In addition to chapters on common sleep disorders such as sleep-disordered breathing, insomnia, restless legs syndrome, hypersomnias, parasomnias, and circadian rhythm disorders, there are specialty chapters on sleep in special populations (pregnancy, children, high consequence industries) Sleep deprivation and its effects on the individual and society is highlighted in a separate chapter I hope this book serves useful for learners on the sleep medicine elective or learners on other rotations interested in how patients with sleep disorders present In addition, this book will provide a solid foundation to clinicians who want to better evaluate patients who present to their office with sleepiness or sleeplessness St Louis, MO, USA Raman K Malhotra, M.D Acknowledgments I would like to express my gratitude to all the accomplished authors who took time away from their families and friends to contribute to this book I would also like to thank my colleagues and staff at the SLUCare Sleep Disorders Center and in the Department of Neurology and Psychiatry at Saint Louis University for their support and guidance with this project I would like to give my sincere gratitude to my Chairman, Dr Henry Nasrallah, for his input and advice I would like to thank Dr Ronald Chervin who helped guide me early on in my career and drew me to the exciting field of sleep medicine while I was at the University of Michigan Sleep Disorders Center in Ann Arbor, Michigan I would like to thank Dr Alon Avidan who has provided me invaluable support and guidance throughout this project and throughout my career I would like to thank the publishing team from Springer who made this book possible and were wonderful to work with Lastly, I would like to thank my parents, my wife, Shalini, and my two sons, Yash and Sanjay, for their inspiration and understanding while I worked on this important endeavor St Louis, MO, USA Raman K Malhotra, M.D ix Sleep Deprivation 83 Commercial aviation is not free from the impact of sleep deprivation on pilots and engineers There were instances where pilots were found to be asleep in their cockpits [114] In one study, the maintenance engineers were found to have shifts longer than 12 h with an average of 50 h per week 10 % of the 1,209 engineers in that survey reported falling asleep while driving The National Transportation Safety Board examined accidents between 1978 and 1990 and found that half of the captains involved in accidents had been awake for more than 12 h and half of the first officers had been awake for more than 11 h Drowsiness has been identified as the reason behind many disastrous industrial accidents as well [115] The Exxon Valdez accident was found to be due to the fatigue caused by reduced sleep and extended working hours [116] Similar observations involving sleep deprivation were seen in the Chernobyl nuclear plant meltdown, Three Mile Island reactor accident in Pennsylvania [117], Davis–Besse reactor in Ohio, [118], Rancho Seco nuclear reactor in California [119], Bhopal disaster in India, space shuttle Challenger accident [115, 120], and a nearcatastrophic launch of the shuttle Columbia Many of them were associated with shift work-related sleep deprivation Sleep Deprivation in Medical Profession While residents in medical and surgical training are plagued by a variety of stressors, sleep deprivation has been consistently identified to be associated with a negative impact on their performance and an increased likelihood of committing a serious medical error Despite the recent efforts in cutting down the number of duty hours while on call and other measures to balance education with clinical duties, sleep deprivation continues to be rampant among physicians in training The formal study of the effect of residents’ long duty hours on performance began in 1971 when Friedman et al reported that interns made almost twice as many errors reading electrocardiograms (EKG) after an extended work shift than after a night of sleep [121] The topic on the resident duty hours gained more attention when a college freshman by the name Libby Zion died within h of her emergency admission in a New York teaching hospital [122] The cause of death is thought to be serotonin syndrome and a grand jury investigation found that the death was related to 36 h duty periods of the residents involved in the patient care along with inadequate supervision by the attending physicians [123] In another recent study, surgical residents made up to twice the number of technical errors in performing simulated laparoscopic surgical skills after an overnight work call than after a night of sleep [124] The “Intern Sleep and Patient Safety Study” which studied the performance of medical residents found that interns made more serious medical errors when they worked frequent shifts of 24 h or more than when they worked shorter shifts [125] Lockley et al found that eliminating the extended work shifts in the intensive care unit significantly increased sleep and reduced the number of attention failures during the night work hours [126] 84 P.C Bollu et al Management of Sleep Deprivation Non-pharmacological Treatments To put in simple terms, sleep is the treatment for sleepiness due to sleep deprivation People typically nap when they are sleepy secondary to a restricted sleep routine Naps tend to improve alertness and performance [127] In a study, the nap rate in adult population is as high as 74 % [128] If a nap happens after a period of prolonged wakefulness, it is called a “recuperative nap,” but if it happens prior to a prolonged wakeful period, it is a “prophylactic nap.” Longer naps, though always not feasible, are more efficient in improving alertness, reaction times, and shortterm memory [129–131] Naps shorter than 10 however are not very restorative and are associated with only limited improvement [132] Naps in the midafternoon (1–4 pm) were found to be more restorative than those in the early afternoon [133] Other than naps, some behavioral measures like walking while reading, reading out loudly, bright lighting, increasing the sound of music, etc are all adopted by people when they sleep However, their effect is limited and short lasting at best Pharmacological 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Physiological changes around adolescence make teenagers more prone to sleepiness These include a physiological delay in onset of the secretion of melatonin, which is the key sleep-inducing hormone Consequently, teens find themselves unable to fall asleep prior to 10:30 or 11:00 p.m Further, the use of electronic media such as cell phones, computers, and television in the 1–2 h prior to bedtime may also in interfere with timely sleep onset Their morning wake-up time on school mornings remains unaltered however at around 5:30 or 6:00 am Consequently, most teens receive only between and h of sleep on most school nights This is further substantiated by the longitudinal study of Iglowstein et al who found that S Kotagal, M.D (*) Division of Child Neurology and Center for Sleep Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA e-mail: Kotagal.Suresh@mayo.edu © Springer International Publishing Switzerland 2015 R.K Malhotra (ed.), Sleepy or Sleepless, DOI 10.1007/978-3-319-18054-0_7 91 S Kotagal 92 the mean sleep duration in 10-year-olds was 9.9 ± 0.6 h whereas the mean sleep duration in 16-year-olds had diminished to 8.1 ± 0.7 h [4] The need for optimum sleep however remains at 9–9.5 h Most teens are therefore chronically sleep deprived When specific sleep disorders are superimposed on the above increased propensity for sleepiness, clinically significant sleepiness is likely to emerge Sleep-wake function is driven centrally by dynamic interaction between two opposing processes—process C or the circadian drive for sleep and process S or the homeostatic drive for sleep The circadian drive for sleep is highest at the end of the night’s sleep, which is the time at which the homeostatic drive is at the lowest The homeostatic drive for sleep builds up gradually during wakefulness and dissipates progressively during sleep [5, 6] The timing of sleep and wakefulness is regulated by the circadian system, which is composed of the retinohypothalamic pathway, which transmits lightdependent impulses to the suprachiasmatic nucleus (SCN) of the hypothalamus The SCN serves as our biological clock At the cellular level, the activities of the biological clock are based upon a series of transcription–translation feedback loops that are regulated by a series of genes, including Clock, Bmal1, Period, and Cryptochrome [6] Some of the neurons located in the dorsolateral hypothalamus and the rostral midbrain are wake promoting Cells of the dorsolateral hypothalamus secrete hypocretin (orexin), which has widespread projections to the forebrain as well as the brain stem and plays a major role in enhancing alertness and motor activity [5] Narcolepsy is associated with loss of hypocretin-secreting neurons Histaminergic neurons located in the tuberomammillary nucleus and noradrenergic neurons of the locus ceruleus also play a role in enhancing alertness On the other hand, sleeppromoting structures include the anterior hypothalamus, which contains gamma amino butyric acid, a major inhibitory neurotransmitter What Are Some Common Disorders Leading to Hypersomnia in Childhood and Adolescence? Table 7.1 lists sleep disorders that are commonly associated with excessive daytime sleepiness The relative frequency of these disorders will likely vary from a community practice to a pediatric sleep clinic, with conditions like inadequate sleep Table 7.1 Disorders associated with excessive daytime sleepiness in childhood Health condition Inadequate sleep hygiene Sedation from drugs (prescription or over the counter) Depression Sleep apnea–hypopnea syndrome Narcolepsy Neoplastic, traumatic, and inflammatory brain lesions Kleine–Levin syndrome Idiopathic hypersomnia a Authors opinion based upon clinical practice Relative frequencya ++++ +++ ++ ++ ++ ++ + + The Sleepy Child 93 hygiene being more prevalent in the former setting and indolent problems like narcolepsy, idiopathic hypersomnia, and Kleine–Levin syndrome presenting more often in the sleep clinic environment What Are the Clinical Features of Childhood Excessive Daytime Sleepiness? Excessive sleepiness may be difficult to establish in preschool-age children as physiological daytime napping may still occur at this age Usually, however, most 3–4-year-olds take one nap per day that lasts about 1–2 h Sleepiness in excess of this is generally abnormal Sometimes daytime hyperactivity can be a manifestation of sleepiness at this age as sleepiness can lead to loss of affect control that is mediated by the ventrolateral prefrontal cortex In children of school-going age, mood swings, inattentiveness, and executive dysfunction may occur on a similar basis There may be a decline in school grades as a consequence of impaired attention span due to hypersomnolence In children of school-going age, there may be reappearance of the tendency to take daytime naps The child may be subjected to bullying by peers Patients with depression may have a flat affect or admit to feelings of sadness or anhedonia Obesity and precocious puberty may accompany daytime sleepiness of narcolepsy–cataplexy [7] Cataplexy, a condition characterized by abrupt muscle weakness in response to emotional stimuli like laughter or surprise, is seen in patients with narcolepsy–cataplexy (for details, please refer to the section dealing with narcolepsy) Sleepiness can be documented using questionnaires Some of the questionnaires that are available to the practitioner include the Pediatric Daytime Sleepiness Scale, the modified Epworth Sleepiness Scale, the Sleep Disturbance Scale for Children, the Cleveland Adolescent Sleepiness Scale, the Pediatric Sleep Questionnaire, and the Children’s Sleep Wake Scale [8] The clinician is advised to utilize the questionnaire that best meets the demands of the individual practice The author utilizes the Pediatric Daytime Sleepiness Scale because of its ease of use and numeric representation of the results The sleep history should also inquire into the bed-onset time and sleep-onset time, whether there is significant departure in the sleep time from school nights to non-school nights, feelings of restlessness in the legs prior to sleep onset, hypnagogic hallucinations and sleep paralysis, habitual snoring, periods of observed apnea, restless sleep, and the morning awakening time and whether the patient feels tired or refreshed upon awakening Patients with delayed sleep-phase syndrome generally indicate an inability to fall asleep prior to midnight or early morning and find it hard to awaken prior to the mid-morning hours Elements of the general medical history that are relevant include changes in weight and appetite over the preceding year, thyroid dysfunction, and use of medications that might impact weight such as corticosteroids The history should explore the possibility of alcohol or substance abuse If there have been previous sleep evaluations, their date and result should be documented Medications previously used for treating sleepiness and response to them should be recorded 94 S Kotagal Laboratory Investigations Wrist actigraphy and sleep diary maintained for 2–3 weeks are useful in the case of suspected circadian rhythm sleep disorders like delayed sleep-phase syndrome The wrist actigraph in delayed sleep-phase syndrome will demonstrate delayed sleep onset, relative absence of sleep fragmentation, but persistence of sleep into the midmorning hours A urine drug screen should be used in adolescents whenever there is a suspicion of drug-seeking behavior Nocturnal polysomnography is indicated in patients with hypersomnia suspected to be related to sleep disordered breathing, narcolepsy, or Idiopathic hypersomnia or sleepiness related to conditions like head injury or encephalitis Polysomnographic findings are discussed under specific sleep disorders The multiple sleep latency test (MSLT) is generally conducted on the morning after a nocturnal polysomnogram The test helps to quantify the degree of daytime sleepiness and the nature of the transitions from wakefulness to sleep, i.e., whether into REM or NREM sleep Normal values for the MSLT in children and adolescents vary with age and Tanner stage of sexual development (Table 7.2) Inadequate Sleep Hygiene This entity can occur along with other sleep disorders or may be the sole clinical disturbance It is usually encountered in adolescents There may be relatively late bed-onset time on school nights or non-school nights, with consequent late onset of sleep If the morning wake time, especially for school days remains unaltered around 6:00 a.m., there is a likelihood of daytime sleepiness simply due to insufficient sleep at night The history may reveal other factors that predispose to late sleep onset such as excessive consumption of caffeinated beverages; heavy use of electronic media such as cell phones, computers, and television in the 2–3 h prior to bed time; or exercising late in the evening A warm bath in the 2–3 h prior to bedtime might also postpone sleep by artificially raising body temperature The diagnosis can be facilitated by wrist actigraphy for 2–3 weeks and concurrent sleep logs Table 7.2 Medications commonly used for treating daytime sleepiness Medication Modafinil (Provigil) Armodafinil (Nuvigil) Methylphenidate hydrochloride (Ritalin, Concerta) Methylphenidate hydrochloride sustained release (SR) Methylphenidate hydrochloride (Concerta) Methylphenidate skin patch (Daytrana) Dextroamphetamine (Dexedrine, Dextrostat) Amphetamine/dextroamphetamine mixture (Adderall) Lisdexamfetamine (Vyvanse) Dose 50–200 mg/day 50–250 mg/day 5–60 mg/day in 2–3 divided doses 20–60 mg once a day 18–54 mg once a day 10–30 mg skin patch 5–40 mg once a day 10–40 mg/day 30–70 mg once a day The Sleepy Child 95 There may be a discrepancy between the patient’s record of bed-onset time and the actigraphically derived sleep-onset time The management consists of a frank discussion with the adolescent patient and his or her parents during which the importance of receiving adequate sleep and the adverse effects of sleep deprivation are discussed It is sometimes a good idea to build a buffer of 30–45 of “quiet time” for reflection prior to entering bed in which the use of electronic media is avoided The importance of avoiding nicotine, alcohol, and excessive caffeine should be also stressed Narcolepsy This is a chronic, lifelong disorder characterized by irresistible daytime sleepiness, hypnagogic hallucinations, cataplexy, and sleep paralysis Approximately % of narcolepsy has onset prior to age years Overall, about a third of all patients with narcolepsy have onset of their symptoms prior to the age of 15–16 years Two distinct forms can occur: narcolepsy (type 1) with cataplexy and narcolepsy (type 2) without cataplexy Some patients may initially present only with hypersomnia In these instances, cataplexy may appear 5–10 years after onset of the sleepiness The subtype of narcolepsy type is being increasingly recognized as the more common form in childhood In these cases, cataplexy is often present right at the very onset of symptoms Cataplexy is characterized by abrupt episodes of muscle weakness in response to emotional stimuli like laughter or surprise It is the most reliable manifestation of narcolepsy and is found in approximately 70 % of subjects Younger children with narcolepsy may sometimes manifest transient episodes of facial weakness which can almost appear choreiform There may not be a clear emotional trigger in some patients Precocious puberty and obesity may also accompany narcolepsy–cataplexy Mood swings and feelings of sadness may also accompany childhood narcolepsy The pathophysiology of narcolepsy–cataplexy is characterized by a genetic predisposition (HLA DQB1*0602 positivity), which is present in over 95 % of cases When the patient is subjected to an immunological challenge, such as influenza, influenza immunization, or Streptococcus pyogenes infection, there is an immunemediated degeneration of the hypocretin-secreting neurons of the dorsolateral hypothalamus [9] This region is the major site for the synthesis of hypocretin (orexin), a peptide that enhances alertness and motor activity The deficiency of hypocretin can be determined by assessing cerebrospinal fluid levels of hypocretin, which is below 110 pg/mL in narcolepsy–cataplexy The diagnosis of narcolepsy has traditionally been made on the basis of a combined battery of nocturnal polysomnogram and the MSLT The nocturnal polysomnogram may show a sleep-onset REM period (onset within 15 of sleep onset), a reduced latency to REM sleep of