DOCSLIB.ORG

Narcolepsy and Other Central Hypersomnias

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Narcolepsy and Other Central Hypersomnias Review Article Address correspondence to Dr Yves Dauvilliers, Centre Narcolepsy and Other National de Re´fe´rence Narcolepsie Hypersomnies, INSERM U1061, De´partement Central Hypersomnias de Neurologie, CHRU Gui de Chauliac, 80 avenue Augustin Fliche, 34295 Montpellier Yves Dauvilliers, MD, PhD; Lucie Barateau, MD Cedex 5, France, [email protected]. Relationship Disclosure: ABSTRACT Dr Dauvilliers has received personal compensation for Purpose of Review: This article focuses on the clinical presentation, pathophys- speaking engagements and iology, diagnosis, differential diagnosis, and management of narcolepsy type 1 and travel expenses, served as narcolepsy type 2, idiopathic hypersomnia, Kleine-Levin syndrome, and other consultant for, and received research/grant support central disorders of hypersomnolence, as defined in the International Classification from Bioprojet Pharma, of Sleep Disorders, Third Edition (ICSD-3). Flamel Technologies, Jazz Recent Findings: In ICSD-3, the names of some central disorders of hypersomnolence Pharmaceuticals, Theranexus, and UCB SA. Dr Dauvilliers have been changed: narcolepsy with cataplexy and narcolepsy without cataplexy have serves as specialty chief been renamed narcolepsy type 1 and narcolepsy type 2, respectively. A low level of editor of the Sleep and hypocretin-1/orexin-A in the CSF is now theoretically sufficient to diagnose narcolepsy Chronobiology section of Frontiers in Neurology. type 1, as it is a highly specific and sensitive biomarker. Conversely, other central Dr Barateau has received hypersomnias are less well-defined disorders with variability in the phenotype, and few reimbursement for travel reliable biomarkers have been discovered so far. The epidemiologic observation that expenses from Laidet Me´dical and has received personal influenza A (H1N1) infection and vaccination are potential triggering factors of compensation as a speaker narcolepsy type 1 (discovered during the 2009 H1N1 pandemic) has increased interest for UCB SA. in this rare disease, and progress is being made to better understand the process Unlabeled Use of (highly suspected to be autoimmune) responsible for the destruction of hypocretin Products/Investigational Use Disclosure: neurons. Treatment of narcolepsy remains largely symptomatic, usually initially with Drs Dauvilliers and Barateau modafinil or armodafinil or with higher-potency stimulants such as methylphenidate or report no disclosures. amphetamines. Several newer wake-promoting agents and psychostimulants have * 2017 American Academy also been developed, including sodium oxybate, which has a role in the treatment of of Neurology. cataplexy and as an adjunctive wake-promoting agent, and pitolisant, a selective histamine H3 receptor inverse agonist that is currently only available in Europe. Summary: Although far less common than many other sleep disorders, central hypersomnias are among the most severe and disabling diseases in the field of sleep medicine, and their early recognition is of major importance for patients, especially children, to maximize their quality of life and functioning in activities of daily living. Continuum (Minneap Minn) 2017;23(4):989–1004. INTRODUCTION hypersomnias has improved consider- Excessive daytime sleepiness is the ably over the past 2 decades because most common presenting symptom of of the integration of data from human rare sleep diseases, the hypersomnia and animal models. The diagnostic disorders of central origin. These in- workup includes medical history, sleep clude narcolepsy type 1 and narcolepsy logs, and polysomnography, followed type 2, idiopathic hypersomnia, Kleine- by the multiple sleep latency test in Levin syndrome, and other hyper- most patients and, in some, additional somnias (eg, due to a medical disorder evaluation with actigraphy, human leu- or substance or associated with psychi- kocyte antigen (HLA) genotyping, and atric disorders). Our understanding CSF examination for hypocretin. The of the pathophysiology of central current management of hypersomnias of Continuum (Minneap Minn) 2017;23(4):989–1004 ContinuumJournal.com 989 Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Narcolepsy and Hypersomnias KEY POINT h Narcolepsy type 1 is a central origin remains symptomatic, and conversation, writing something inap- well-defined entity good evidence for treatment (Level A) propriate or illegible, or doing an characterized by currently exists only for narcolepsy. This activity such as driving to an inappro- excessive daytime article reviews the clinical features, priate location with no memory of the sleepiness and pathophysiology, diagnostic criteria, event) can be seen. cataplexy, whereas and treatment options for narcolepsy Cataplexy is the pathognomonic narcolepsy type 2 is a and other primary hypersomnias. symptom of narcolepsy type 1, de- syndrome of sleepiness fined by a loss of muscle tone in full without cataplexy and NARCOLEPSY TYPE 1 AND consciousness triggered by emotions, is a considerably less TYPE 2 particularly positive ones such as specific and more Narcolepsy is classified into two dis- laughter or surprise (Case 3-1).5 Cat- heterogeneous syndrome. tinct disorders according to the In- aplexy can either be generalized and ternational Classification of Sleep lead to falls or partial, with lower limb Disorders, Third Edition (ICSD-3): collapse, head dropping, or dysarthria. narcolepsy type 1, formerly called Muscle stretch reflexes are abolished narcolepsy with cataplexy, and nar- during generalized cataplexy, since colepsy type 2.1 Narcolepsy type 1 the pathophysiology of cataplexy affects about 1 in 2000 people in the seems mediated by an intrusion of world, with a bimodal age at onset, physiologic rapid eye movement usually between 15 and 35 years of (REM) sleep atonia into wakefulness, age.2 The prevalence of narcolepsy thereby interrupting conscious volun- type 2 remains unclear, as this disorder tary motor activity and waking muscle is more heterogeneous, with an un- tone. Cataplectic attacks can be dif- known pathophysiologic mechanism.3,4 ferent in children. Sometimes they appear without a specific triggering Clinical Features factor; they can also arise in anticipa- Excessive daytime sleepiness is the tion of a strong emotion to come and major and most frequent initial symp- sometimes even during movement. Chil- tom of narcolepsy. Excessive daytime dren can also present with cataplectic sleepiness arises preferentially in mo- facial expressions, such as generalized notonous situations or during periods face hypotonia, abnormal movements, of relative inactivity. Typically, naps and tongue protrusion.6 Clinical signs are short and considered to be re- and biological markers that should freshing by patients, who may also make the physician reconsider the recall the experience of dream activity diagnosis of supposed cataplectic at- just after falling asleep. Similarly, noc- tacks are listed in Table 3-1. turnal sleep is usually considered Other symptoms are often asso- refreshing, and morning waking is ciated with narcolepsy, but they are usually not difficult. In children, the not specific to the condition. Sleep phenotype can be slightly different: paralysis is a transient paralysis lasting naps are inconsistently refreshing, pa- a few seconds or minutes while falling tients sometimes fight against sleepi- asleep or upon awakening. Hypna- ness and do not fall sleep, and they gogic (ie, while falling asleep) and may present with hyperactive behav- hypnopompic (ie, upon awakening) ioral symptoms that can mimic atten- hallucinations can occur at the same tion deficit hyperactivity disorder. time as the paralysis and can be very During episodes of sleepiness, auto- frightening. These symptoms are also matic activities (ie, saying something reported in the general population but inappropriate or out of context in a are more frequent and severe in 990 ContinuumJournal.com August 2017 Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Case 3-1 An 18-year-old man presented with a 2-year history of severe excessive daytime sleepiness that began a few months after a vaccination for influenza A (H1N1). He had to repeat a school year because of the excessive daytime sleepiness. He was overweight and had frequent sleep paralysis and nightmares. Six months after the onset of excessive daytime sleepiness, he developed episodes of weakness in his limbs and neck triggered by various emotional stimuli, especially when he laughed with his brother. The frequency was variable, from several episodes per day to several per week, depending on exposure to the stimuli. His brother had made a cell phone video that the neurologist was able to view, which led to the diagnosis of typical cataplectic attacks. After reviewing the patient’s sleep diary to ensure he was getting adequate nocturnal sleep, polysomnography was performed, followed by a multiple sleep latency test that showed fragmented sleep with no apnea, short sleep latency (2 minutes), and four sleep-onset rapid eye movement (REM) periods, confirming a clear-cut diagnosis of narcolepsy type 1. Human leukocyte antigen genotyping was positive for the DQB1*0602 allele, and CSF hypocretin-1 levels in the CSF were undetectable (less than 10 pg/mL). A modafinil treatment trial was started, with the dosage gradually increased to 200 mg in the morning and 200 mg at noon. Anticataplectic treatment (venlafaxine 37.5 mg/d) was introduced
Load more

Recommended publications
  • S46. Parasomnias.Pdf
    PARASOMNIAS S46 (1) Parasomnias Last updated: May 8, 2019 Clinical Features ............................................................................................................................... 2 SLEEP TERRORS (S. PAVOR NOCTURNUS) ............................................................................................... 2 SLEEPWALKING (S. SOMNAMBULISM) .................................................................................................... 3 CONFUSIONAL AROUSALS (S. SLEEP DRUNKENNESS, SEVERE SLEEP INERTIA) ........................................ 3 Diagnosis .......................................................................................................................................... 3 Management ..................................................................................................................................... 3 HYPNIC JERKS (S. SLEEP STARTS) .......................................................................................................... 4 RHYTHMIC MOVEMENT DISORDER ........................................................................................................ 4 SLEEP TALKING (S. SOMNILOQUY) ......................................................................................................... 4 NOCTURNAL LEG CRAMPS ..................................................................................................................... 4 REM SLEEP BEHAVIORAL DISORDER (RBD) ........................................................................................ 4
    [Show full text]
  • The Neurobiology of Narcolepsy-Cataplexy
    Progress in Neurobiology Vol. 41, pp. 533 to 541, 1993 0301-0082/93/$24.00 Printed in Great Britain. All rights reserved © 1993 Pergamon Press Ltd THE NEUROBIOLOGY OF NARCOLEPSY-CATAPLEXY MICHAEL S. ALDRICH Department of Neurology, Sleep Disorders Center, University of Michigan Medical Center, Ann Arbor, MI, U.S.A. (Received 17 July 1992) CONTENTS 1. Introduction 533 2. Clinical aspects 533 2.1. Sleepiness and sleep attacks 533 2.2. Cataplexy and related symptoms 534 2.3. Clinical variants 534 2.3.1. Narcolepsy without cataplexy 534 2.3.2. Idiopathic hypersomnia 534 2.3.3. Symptomatic narcolepsy 534 2.4. Treatment 534 3. Pathophysiology 535 4. Neurobiological studies 535 4.1. The canine model of narcolepsy 535 4.2. Pharmacology of human cataplexy 537 4.3. Postmortem studies 537 5. Genetic and family studies 537 6. Summary and conclusions 539 References 539 1. INTRODUCTION 2. CLINICAL ASPECTS Narcolepsy is a specific neurological disorder Narcolepsy has a prevalence that varies worldwide characterized by excessive sleepiness that cannot be from as little as 0.0002% in Israel to 0.16% in Japan; fully relieved with any amount of sleep and by in North America and Europe the prevalence is about abnormalities of rapid eye movement (REM) 0.03-0.06% (Dement et al., 1972; Honda, 1979; Lavie sleep. About two-thirds of patients also have brief and Peled, 1987). The onset of narcoleptic symptoms, episodes of muscle weakness usually brought on by usually in the second or third decade of life, may emotion, referred to as cataplexy. The disorder gener- occur over a few days or weeks or it may be so ally begins in adolescence and continues throughout gradual that the loss of full alertness is unrecognized life.
    [Show full text]
  • Post-Traumatic Stress Disorder (PTSD) and Sleep
    SHF-PTSD-0312 21/3/12 6:20 PM Page 1 Post-Traumatic Stress Disorder (PTSD) and Sleep Important Things to Know About PTSD and Sleep • PTSD can happen after a period of extreme trauma and stress. • One of the symptoms of PTSD may be problems with sleeping. • The treatment for this will depend on how the PTSD is affecting sleep. • There are many treatments available. How might PTSD affect sleep? • Insomnia. People with PTSD may have difficulty with getting to sleep or staying asleep. They may wake up There are may sleep problems that may be associated frequently during the night and be unable to get back with PTSD. For more information on the disorders to sleep. mentioned below see the relevant pages on this website. • Issues linked to the body clock, such as Delayed Sleep • The extreme anxiety of PTSD (caused by trauma or Phase Disorder may occur in a person with PTSD. If you catastrophe) can seriously disrupt sleep. In some cases can’t get to sleep until very late at night and then this starts a few months after the event. You might need to sleep in you may be experiencing this suffer from horror or strong fear and feel helpless. See problem. Anxiety and Sleep. • Obstructive Sleep Apnoea may be caused by weight • People with PTSD have higher rates of depression and gain due to the life style changes associated with the this is often associated with poor sleep. See PTSD. If the sleep apnoea is serious, medications such Depression and Sleep. as Seroquel can be an additional danger.
    [Show full text]
  • Sleep Disorders Preeti Devnani
    SPECIAL ISSUE 1: INVITED ARTICLE Sleep Disorders Preeti Devnani ABSTRACT Sleep disorders are an increasingly important and relevant burden faced by society, impacting at the individual, community and global level. Varied presentations and lack of awareness can make accurate and timely diagnosis a challenge. Early recognition and appropriate intervention are a priority. The key characteristics, clinical presentations and management strategies of common sleep disorders such as circadian rhythm disorders, restless legs syndrome, REM behavior disorder, hypersomnia and insomnia are outlined in this review. Keywords: Hypersomnia, Insomnia, REM behavior International Journal of Head and Neck Surgery (2019): 10.5005/jp-journals-10001-1362 INTRODUCTION Department of Neurology and Sleep Disorder, Cleveland Clinic, Abu Sleep disorders are becoming increasingly common in this modern Dhabi, United Arab Emirates era, resulting from several lifestyle changes. These complaints may Corresponding Author: Preeti Devnani, Department of Neurology present excessive daytime sleepiness, lack of sleep or impaired and Sleep Disorder, Cleveland Clinic, Abu Dhabi, United Arab Emirates, quality, sleep related breathing disorders, circadian rhythm disorder e-mail: [email protected] misalignment and abnormal sleep-related movement disorders.1 How to cite this article: Devnani P. Sleep Disorders. Int J Head Neck They are associated with impaired daytime functioning, Surg 2019;10(1):4–8. increased risk of cardiovascular and cerebrovascular disease, poor Source of support: Nil glycemic control, risk of cognitive decline and impaired immunity Conflict of interest: None impacting overall morbidity and mortality. Diagnosis of sleep disorders is clinical in many scenarios, The following circadian rhythm sleep–wake disorders adapted polysomnography is a gold standard for further evaluation of from the ICSD-3: intrinsic sleep disorder such as obstructive sleep apnea (OSA) • Delayed sleep–wake phase disorder and periodic limb movement disorder (PLMD).
    [Show full text]
  • Sleep Disturbances in Patients with Persistent Delusions: Prevalence, Clinical Associations, and Therapeutic Strategies
    Review Sleep Disturbances in Patients with Persistent Delusions: Prevalence, Clinical Associations, and Therapeutic Strategies Alexandre González-Rodríguez 1 , Javier Labad 2 and Mary V. Seeman 3,* 1 Department of Mental Health, Parc Tauli University Hospital, Autonomous University of Barcelona (UAB), I3PT, Sabadell, 08280 Barcelona, Spain; [email protected] 2 Department of Psychiatry, Hospital of Mataró, Consorci Sanitari del Maresme, Institut d’Investigació i Innovació Parc Tauli (I3PT), CIBERSAM, Mataró, 08304 Barcelona, Spain; [email protected] 3 Department of Psychiatry, University of Toronto, #605 260 Heath St. West, Toronto, ON M5T 1R8, Canada * Correspondence: [email protected] Received: 1 September 2020; Accepted: 12 October 2020; Published: 16 October 2020 Abstract: Sleep disturbances accompany almost all mental illnesses, either because sound sleep and mental well-being share similar requisites, or because mental problems lead to sleep problems, or vice versa. The aim of this narrative review was to examine sleep in patients with delusions, particularly in those diagnosed with delusional disorder. We did this in sequence, first for psychiatric illness in general, then for psychotic illnesses where delusions are prevalent symptoms, and then for delusional disorder. The review also looked at the effect on sleep parameters of individual symptoms commonly seen in delusional disorder (paranoia, cognitive distortions, suicidal thoughts) and searched the evidence base for indications of antipsychotic drug effects on sleep. It subsequently evaluated the influence of sleep therapies on psychotic symptoms, particularly delusions. The review’s findings are clinically important. Delusional symptoms and sleep quality influence one another reciprocally. Effective treatment of sleep problems is of potential benefit to patients with persistent delusions, but may be difficult to implement in the absence of an established therapeutic relationship and an appropriate pharmacologic regimen.
    [Show full text]
  • Original Articles
    ORIGINAL ARTICLES Improvement in Cataplexy and Daytime Somnolence in Narcoleptic Patients with Venlafaxine XR Administration Rafael J. Salin-Pascual, M.D., Ph.D. Narcoleptic patients have been treated with stimulants for sleep attacks as well as day- time somnolence, without effects on cataplexy, while this symptoms has been treated with antidepressants, that do not improve daytime somnolence or sleep attacks. Venlafaxine inhibits the reuptake of norepinephrine, serotonin, and to lesser extent dopamine, and also suppressed REM sleep. Because some of the symptoms of nar- colepsy may be related to REM sleep deregulation, venlafaxine was studied in this sleep disorder. Six narcoleptic patients were studied, they were drug-free and all of them had daytime somnolence and cataplexy attacks. They underwent the following sleep proce- dure: one acclimatization night, one baseline night, followed by multiple sleep latency test. After two days of the sleep protocol, patients received 150 mg of venlafaxine XR at 08:00 h. Two venlafaxine sleep nights recordings were performed. Patients were fol- lowed for two months with weekly visits for clinical evaluation. Sleep log and analog- visual scale for alertness and somnolence were performed on each visit. Venlafaxine XR was increase by the end of the first month to 300 mg/day. Sleep recordings showed that during venlafaxine XR two days acute administration the following findings: increase in wake time and sleep stage 1, while REM sleep time was reduced. No changes were observed in the rest of sleep architecture variables. Cataplexy attacks were reduced since the first week of venlafaxine administration. Daytime somnolence was reduced also, but until the 7th week and with 300 mg/day of venlafaxine XR administration.
    [Show full text]
  • Sleep Disorders As Outlined by Outlined As Disorders Sleep of Classification N the Ribe the Features and Symptoms of Each Disorder
    CHAPTER © Jones & Bartlett Learning, LLC © Jones & Bartlett Learning, LLC NOT FOR SALE OR DISTRIBUTION 2NOT FOR SALE OR DISTRIBUTION © Jones & Bartlett Learning, LLC © Jones & Bartlett Learning, LLC NOT FORSleep SALE OR DISTRIBUTION Disorders NOT FOR SALE OR DISTRIBUTION © Jones & Bartlett Learning, LLC © Jones & Bartlett Learning, LLC NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION © Agsandrew/Shutterstock © Jones & Bartlett Learning, LLC © Jones & Bartlett Learning, LLC NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION CHAPTER OUTLINE EEG arousal alveolar hypoventilation paradoxical breathing idiopathic central alveolar History of Sleep Disorders© Jones & Bartlett Learning, LLCmicrognathia © Joneshypoventilation & Bartlett Learning, LLC Classification of Sleep DisordersNOT FOR SALE OR DISTRIBUTIONretrognathia NOTsnoring FOR SALE OR DISTRIBUTION Insomnia apnea–hypopnea primary snoring Sleep-Related Breathing Disorders index (AHI) sleep-related groaning Central Disorders of Hypersomnolence respiratory disturbance catathrenia Circadian Rhythm Sleep–Wake Disorders index (RDI) CPAP therapy Parasomnias respiratory effort–related positional therapy Sleep-Related© Jones Movement & Bartlett Disorders Learning, LLC arousal (RERA)© Jones & Bartletttonsillectomy Learning, LLC OtherNOT Sleep FOR Disorders SALE OR DISTRIBUTION upper-airwayNOT resistance FOR SALEadenoidectomy OR DISTRIBUTION Chapter Summary syndrome bi-level therapy excessive daytime multiple sleep latency LEARNING OBJECTIVES sleepiness (EDS) test (MSLT) sudden infant
    [Show full text]
  • Narcolepsy Caused by Acute Disseminated Encephalomyelitis
    OBSERVATION Narcolepsy Caused by Acute Disseminated Encephalomyelitis Richard F. Gledhill, MD, MRCP; Peter R. Bartel, PhD; Yasushi Yoshida, MD, PhD; Seiji Nishino, MD, PhD; Thomas E. Scammell, MD Background: Narcolepsy with cataplexy is caused by ueduct, which are consistent with acute disseminated a selective loss of hypocretin-producing neurons, but nar- encephalomyelitis. colepsy can also result from hypothalamic and rostral brainstem lesions. Results: After treatment with steroids, this patient’s sub- jective sleepiness, hypersomnia, and hypocretin defi- Patient: We describe a 38-year-old woman with severe ciency partially improved. daytime sleepiness, internuclear ophthalmoplegia, and bilateral delayed visual evoked potentials. Her multiple Conclusions: Autoimmune diseases such as acute dis- sleep latency test results demonstrated short sleep laten- seminated encephalomyelitis can produce narcolepsy. cies and 4 sleep-onset rapid eye movement sleep peri- Most likely, this narcolepsy is a consequence of demy- ods, and her cerebrospinal fluid contained a low con- elination and dysfunction of hypocretin pathways, but centration of hypocretin. Magnetic resonance imaging direct injury to the hypocretin neurons may also occur. showed T2 and fluid-attenuated inversion recovery hy- perintensity along the walls of the third ventricle and aq- Arch Neurol. 2004;61:758-760 ARCOLEPSY IS CHARAC- REPORT OF A CASE terized by excessive daytime sleepiness and rapid eye movement A previously healthy, 38-year-old, black, (REM) sleep-related South African woman was admitted to Ga- Nsymptoms such as cataplexy. More than Rankuwa Hospital, Pretoria, Republic of 90% of people with narcolepsy with cata- South Africa, with 6 weeks of severe day- plexy have no detectable hypocretin/ time sleepiness and hypersomnia (total orexin in their cerebrospinal fluid sleep time about 16 of every 24 h).
    [Show full text]
  • The Co-Occurrence of Sexsomnia, Sleep Bruxism and Other Sleep Disorders
    Journal of Clinical Medicine Review The Co-Occurrence of Sexsomnia, Sleep Bruxism and Other Sleep Disorders Helena Martynowicz 1, Joanna Smardz 2, Tomasz Wieczorek 3, Grzegorz Mazur 1, Rafal Poreba 1, Robert Skomro 4, Marek Zietek 5, Anna Wojakowska 1, Monika Michalek 1 ID and Mieszko Wieckiewicz 2,* 1 Department of Internal Medicine, Occupational Diseases and Hypertension, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] (H.M.); [email protected] (G.M.); [email protected] (R.P.); [email protected] (A.W.); [email protected] (M.M.) 2 Department of Experimental Dentistry, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] 3 Department and Clinic of Psychiatry, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] 4 Division of Respiratory Critical Care and Sleep Medicine, Department of Medicine, University of Saskatchewan, Saskatoon, SK S7N 5A2, Canada; [email protected] 5 Department of Periodontology, Wroclaw Medical University, 50-367 Wroclaw, Poland; [email protected] * Correspondence: [email protected]; Tel.: +48-660-47-87-59 Received: 3 August 2018; Accepted: 19 August 2018; Published: 23 August 2018 Abstract: Background: Sleep sex also known as sexsomnia or somnambulistic sexual behavior is proposed to be classified as NREM (non-rapid eye movement) parasomnia (as a clinical subtype of disorders of arousal from NREM sleep—primarily confusional arousals or less commonly sleepwalking), but it has also been described in relation to REM (rapid eye movement) parasomnias. Methods: The authors searched the PubMed database to identify relevant publications and present the co-occurrence of sexsomnia and other sleep disorders as a non-systematic review with case series.
    [Show full text]
  • Circadian Rhythm Sleep Disorders and Narcolepsy
    TALK FOR NARCOLEPSY NETWORK CONFERENCE 2013: Circadian Rhythm Sleep Disorders and Narcolepsy Note: The slides for this talk may be viewed at http://www.circadiansleepdisorders.org/docs/talks/NNconf2013talkSlides.pdf . Slides with audio of the talk are at http://youtu.be/i70SqjCr-jY . I. Introduction [title slide] A. Hello Hi. I’m Peter Mansbach, and I’m president of Circadian Sleep Disorders Network. I’m really glad for this opportunity to talk about circadian sleep disorders, and also about possible connections with narcolepsy. B. Disclaimer Let me start by saying I am not a medical doctor. I don’t diagnose, and I don’t treat. C. Why should the narcolepsy community care? [Overview slide] The various sleep disorders overlap. I have DSPS, but I have some of the same symptoms as narcolepsy. And many of you have symptoms of DSPS. Diagnoses are fuzzy too, and in some cases another sleep disorder may be secondary or even dominant. I’ll talk more about this later. D. Intro How many of you have trouble waking up in the morning? How many of you like to stay up late? II. Circadian Rhythm Sleep Disorders A. What are circadian rhythms? [slide] 1. General Circadian means "approximately a day". Circadian rhythms are processes in living organisms which cycle daily. They are produced internally in all living things. They are also referred to as the body clock. 2. In Humans Humans have internal cycles lasting on average about 24 hours and 10 minutes, though the length varies from person to person. (Early experiments seemed to show a cycle of about 25 hours, and this still gets quoted, but it is now known to be incorrect.
    [Show full text]
  • A Comparison of Idiopathic Hypersomnia and Narcolepsy-Cataplexy Using Self Report Measures and Sleep Diary Data
    57676ournal ofNeurology, Neurosurgery, and Psychiatry 1996;60:576-578 SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.60.5.576 on 1 May 1996. Downloaded from A comparison of idiopathic hypersomnia and narcolepsy-cataplexy using self report measures and sleep diary data Dorothy Bruck, J D Parkes Abstract Published data comparing patients with IH Eighteen patients with idiopathic hyper- and patients with NLS outside the sleep labo- somnia (IH) were compared with 50 ratory are scarce. Using self report question- patients with the narcoleptic syndrome of naires and sleep diary data our aim was to cataplexy and daytime sleepiness (NLS) determine the extent of group differences and using self report questionnaires and a which variables best discriminated between IH diary of sleep/wake patterns. The IH and NLS. group reported more consolidated noc- turnal sleep, a lower propensity to nap, greater refreshment after naps, and a Patients and methods greater improvement in excessive day- DIAGNOSTIC CRITERIA time sleepiness since onset than the NLS Idiopathic hypersomnia group. In IH, the onset of excessive day- Patients were selected with a complaint of time sleepiness was predominantly asso- excessive daytime sleepiness without cataplexy ciated with familial inheritance or a viral and with no evidence of any medical, psycho- illness. Two variables-number of logical, drug related, or respiratory disorder. reported awakenings during nocturnal All patients met diagnostic criteria ascertained sleep and the reported change in sleepi- from questionnaire responses: Epworth sleepi- ness since onset-provided maximum ness scale score8 > 13; duration of excessive discrimination between the IH and NLS daytime sleepiness > five years, profile of groups.
    [Show full text]
  • Adult NREM Parasomnias: an Update
    Review Adult NREM Parasomnias: An Update Maria Hrozanova 1, Ian Morrison 2 and Renata L Riha 3,* 1 Department of Neuromedicine and Movement Science, Norwegian University of Science and Technology, N-7491 Trondheim, Norway; [email protected] 2 Department of Neurology, Ninewells Hospital and Medical School, DD1 9SY Dundee, UK; [email protected] 3 Department of Sleep Medicine, Royal Infirmary of Edinburgh, EH16 4SA Edinburgh, UK * Correspondence: [email protected] or [email protected]; Tel.: +44-013-242-3872 Received: 23 August 2018; Accepted: 15 November 2018; Published: 23 November 2018 Abstract: Our understanding of non-rapid eye movement (NREM) parasomnias has improved considerably over the last two decades, with research that characterises and explores the causes of these disorders. However, our understanding is far from complete. The aim of this paper is to provide an updated review focusing on adult NREM parasomnias and highlighting new areas in NREM parasomnia research from the recent literature. We outline the prevalence, clinical characteristics, role of onset, pathophysiology, role of predisposing, priming and precipitating factors, diagnostic criteria, treatment options and medico-legal implications of adult NREM parasomnias. Keywords: NREM parasomnias; slow-wave sleep disorders; parasomnias; adult; arousal disorders; review 1. Introduction Non-rapid eye movement (NREM) parasomnias constitute a category of sleep disorders characterised by abnormal behaviours and physiological events primarily arising from N3sleep [1–3] and occuring outside of conscious awareness. Due to their specific association with slow wave sleep (SWS), NREM parasomnias are also termed ‘SWS disorders’. Behaviours such as confusional arousals, sleepwalking, sleep eating (also called sleep-related eating disorder, or SRED), night terrors, sexualised behaviour in sleep (also called sexsomnia) and sleep-related violence are NREM parasomnias that arise from N3 sleep.
    [Show full text]