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Idiopathic hypersomnolence

Yoko Fujii, Mutsumi Okura, Hidekazu Uemori, Mitsutaka Taniguchi, Motoharu Ohi
The rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia characterized by dream-enacting behaviors related to the loss of the normal generalized skeletal muscle atonia during REM sleep, and shows REM sleep without atonia (RWA) during polysomnography (PSG). Patients with idiopathic RBD have been known to have a siginificantly increased risk of developing one of the α-synucleiopathies later in life, therefore the diagnosis of RBD is very important and must be dealt with carefully. A 51-year-old man was identified presenting dream-enacting behaviors and unpleasant dreams suggesting the diagnosis of RBD, in addition to snoring and excessive daytime sleepiness...
February 28, 2018: Rinshō Shinkeigaku, Clinical Neurology
Elisa Evangelista, Régis Lopez, Lucie Barateau, Sofiene Chenini, Adriana Bosco, Isabelle Jaussent, Yves Dauvilliers
OBJECTIVE: To assess the diagnostic value of extended sleep duration on a controlled 32-hour bed-rest protocol in idiopathic hypersomnia (IH). METHODS: 116 patients with high suspicion of IH (37 clear-cut-IH according to multiple sleep latency test criteria and 79 probable-IH), 32 with hypersomnolence associated with a comorbid disorder (non-IH) and 21 controls underwent a polysomnography, modified-sleep latency tests and a 32-h bed-rest protocol. Receiver operating characteristic curves were used to find optimal total sleep time (TST) cut-off values on various periods that discriminate patients to controls...
January 11, 2018: Annals of Neurology
Elisa Evangelista, Régis Lopez, Yves Dauvilliers
Idiopathic hypersomnia (IH) is a poorly characterized orphan central disorder of hypersomnolence responsible for excessive daytime sleepiness (EDS), prolonged nighttime sleep and sleep inertia that often require long-term symptomatic stimulant medication. To date, no drug has currently the authorization for the treatment of IH patients worldwide. Areas covered: The authors reviewed data on pharmacological treatment of IH obtained from published literature (Medline/PubMed/Web of Science) and database from 1997 to 2017...
January 3, 2018: Expert Opinion on Investigational Drugs
Régis Lopez, Anis Doukkali, Lucie Barateau, Elisa Evangelista, Sofiene Chenini, Isabelle Jaussent, Yves Dauvilliers
Study Objectives: To assess the test-retest reliability of the polysomnography-multiple sleep latency test (PSG-MSLT) diagnostic classification and measures and to study the determinants of its variability in patients with narcolepsy type 1 (NT1) or with noncataplectic central disorders of hypersomnolence (NCHS): type 2 (NT2), idiopathic hypersomnia (IH), and unspecified hypersomnolence (unspecified excessive daytime sleepiness [UnsEDS]). Methods: PSG-MSLT in drug-free conditions was administered twice (median interval of 1...
December 1, 2017: Sleep
Yves Dauvilliers, Lucie Barateau
PURPOSE OF REVIEW: This article focuses on the clinical presentation, pathophysiology, diagnosis, differential diagnosis, and management of narcolepsy type 1 and narcolepsy type 2, idiopathic hypersomnia, Kleine-Levin syndrome, and other central disorders of hypersomnolence, as defined in the International Classification of Sleep Disorders, Third Edition (ICSD-3). RECENT FINDINGS: In ICSD-3, the names of some central disorders of hypersomnolence have been changed: narcolepsy with cataplexy and narcolepsy without cataplexy have been renamed narcolepsy type 1 and narcolepsy type 2, respectively...
August 2017: Continuum: Lifelong Learning in Neurology
Markus Ramm, Arsalan Jafarpour, Matthias Boentert, Nelly Lojewsky, Peter Young, Anna Heidbreder
The aim of the current study was to evaluate an attention test as a discriminative tool to measure neurocognitive impairment in patients with disorders of hypersomnolence. Chronic excessive daytime sleepiness is the main symptom in central disorders of hypersomnolence. For diagnostic purposes and treatment evaluation, reliable assessment of excessive daytime sleepiness is required. Thirty-six patients with central disorders of hypersomnolence were compared with 20 healthy controls. All participants performed the 'Perception and Attention Functions' (WAF) of the Vienna Test System...
August 2, 2017: Journal of Sleep Research
Lucie Barateau, Régis Lopez, Jean Arthur Micoulaud Franchi, Yves Dauvilliers
Relationships between symptoms of hypersomnolence, psychiatric disorders, and hypersomnia disorders (i.e., narcolepsy and idiopathic hypersomnia) are complex and multidirectional. Hypersomnolence is a common complaint across mood disorders; however, patients suffering from mood disorders and hypersomnolence rarely have objective daytime sleepiness, as assessed by the current gold standard test, the Multiple Sleep Latency Test. An iatrogenic origin of symptoms of hypersomnolence, and sleep apnea syndrome must be considered in a population of psychiatric patients, often overweight and treated with sedative drugs...
February 2017: Current Psychiatry Reports
Prabhjyot Saini, David B Rye
Most central disorders of hypersomnolence are conditions with poorly understood pathophysiologies, making their identification, treatment, and management challenging for sleep clinicians. The most challenging to diagnose and treat is idiopathic hypersomnia. There are no FDA-approved treatments, and off-label usage of narcolepsy treatments seldom provide benefit. Patients are largely left on their own to alleviate the compound effects of this disorder on their quality of life. This review covers the major points regarding clinical features and diagnosis of idiopathic hypersomnia, reviews current evidence supporting the available treatment options, and discusses the psychosocial impact and effects of idiopathic hypersomnia...
March 2017: Sleep Medicine Clinics
Lucie Barateau, Régis Lopez, Isabelle Arnulf, Michel Lecendreux, Patricia Franco, Xavier Drouot, Smaranda Leu-Semenescu, Isabelle Jaussent, Yves Dauvilliers
OBJECTIVE: To assess and compare the frequencies of personal and family history of autoimmune diseases (AID), autoinflammatory disorders (ID), and allergies in a population of patients, adults and children, with narcolepsy type 1 (NT1), narcolepsy type 2 (NT2), and idiopathic hypersomnia (IH), 3 central hypersomnia disorders, and healthy controls. METHODS: Personal and family history of AID, ID, and allergies were assessed by questionnaire and medical interview in a large cohort of 450 consecutive adult patients (206 NT1, 106 NT2, 138 IH) and 95 pediatric patients (80 NT1) diagnosed according to the third International Classification of Sleep Disorders criteria in national reference centers for narcolepsy in France and 751 controls (700 adults, 51 children) from the general population...
January 3, 2017: Neurology
Lynn M Trotti
The transition from sleep to wake is marked by sleep inertia, a distinct state that is measurably different from wakefulness and manifests as performance impairments and sleepiness. Although the precise substrate of sleep inertia is unknown, electroencephalographic, evoked potential, and neuroimaging studies suggest the persistence of some features of sleep beyond the point of awakening. Forced desynchrony studies have demonstrated that sleep inertia impacts cognition differently than do homeostatic and circadian drives and that sleep inertia is most intense during awakenings from the biological night...
October 2017: Sleep Medicine Reviews
Joseph Andrew Berkowski, Anita Valanju Shelgikar
Central disorders of hypersomnolence are rare conditions with a poorly understood pathophysiology, making the identification and management challenging for sleep clinicians. Clinical history is essential for ruling out secondary causes of hypersomnolence and distinguishing among diagnoses. Current diagnostic criteria rely heavily on the polysomnogram and multiple sleep latency test. The current focus of treatment of hypersomnolence is on drugs that promote alertness. Additionally, in the case of narcolepsy type 1, medication management addresses control of cataplexy, the hallmark symptom of this disorder...
September 2016: Sleep Medicine Clinics
Raffaele Ferri, Fabio Pizza, Stefano Vandi, Martina Iloti, Giuseppe Plazzi
OBJECTIVE: To analyze the complexity of the nocturnal sleep stage sequence in central disorders of hypersomnolence (CDH), with the hypothesis that narcolepsy type 1 (NT1) might exhibit distinctive sleep stage sequence organization and complexity. METHODS: Seventy-nine NT1 patients, 22 narcolepsy type 2 (NT2), 22 idiopathic hypersomnia (IH), and 52 patients with subjective hypersomnolence (sHS) were recruited and their nocturnal sleep was polysomnographically recorded and scored...
August 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Yves Dauvilliers, Elisa Evangelista, Regis Lopez, Lucie Barateau, Isabelle Jaussent, Thierry Cens, Matthieu Rousset, Pierre Charnet
OBJECTIVE: The pathophysiology of idiopathic hypersomnia (IH) remains unclear. Recently, cerebrospinal fluid (CSF)-induced enhancement of γ-aminobutyric acid (GABA)-A receptor activity was found in patients with IH compared to controls. METHODS: Fifteen unrelated patients (2 males and 13 females) affected with typical IH, 12 patients (9 males and 3 females) with narcolepsy type 1, and 15 controls (9 males and 6 females) with unspecified hypersomnolence (n = 7) and miscellaneous neurological conditions (n = 8) were included...
August 2016: Annals of Neurology
William G Mantyh, R Robert Auger, Timothy I Morgenthaler, Michael H Silber, Wendy R Moore
STUDY OBJECTIVES: Narcolepsy and idiopathic hypersomnia are commonly treated by sleep specialists and encountered by other medical providers. Although pharmacotherapy with modafinil and traditional stimulants is considered the mainstay of treatment, physicians are often uncomfortable with their prescription because of concerns regarding misuse. The goal of this study was to assess the frequency of stimulant misuse in this population. METHODS: A retrospective cohort study was performed evaluating patients 18 years and older diagnosed with narcolepsy with and without cataplexy and idiopathic hypersomnia with and without long sleep between 2003-2008...
May 15, 2016: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
Michel Billiard, Karel Sonka
Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood...
October 2016: Sleep Medicine Reviews
T Kim, J H Lee, C S Lee, I Y Yoon
BACKGROUND: Excessive daytime sleepiness (EDS) is a symptom frequently presented in sleep clinics. Only a paucity of data has addressed clinical courses of sleep disorders with EDS. Therefore, we sought to compare clinical outcomes of patients presenting EDS. METHODS: A retrospective observational study was performed in the setting of sleep laboratory and outpatient department in a university hospital. One hundred and eight patients who presented EDS underwent polysomnography and multiple sleep latency test...
July 2016: Acta Neurologica Scandinavica
Kristin T Avis, Jiabin Shen, Patrick Weaver, David C Schwebel
BACKGROUND: Hypersomnia of central origin from narcolepsy or idiopathic hypersomnia (IHS) is characterized by pathological levels of excessive daytime sleepiness (EDS). Central hypersomnia has historically been underdiagnosed and poorly understood, especially with respect to its impact on daytime functioning and quality of life in children. OBJECTIVE: Describe the psychosocial adjustment of children treated for narcolepsy or IHS on school performance, quality of life, and physical/extracurricular activities...
November 2015: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
Zeeshan Khan, Lynn Marie Trotti
The central disorders of hypersomnolence are characterized by severe daytime sleepiness, which is present despite normal quality and timing of nocturnal sleep. Recent reclassification distinguishes three main subtypes: narcolepsy type 1, narcolepsy type 2, and idiopathic hypersomnia (IH), which are the focus of this review. Narcolepsy type 1 results from loss of hypothalamic hypocretin neurons, while the pathophysiology underlying narcolepsy type 2 and IH remains to be fully elucidated. Treatment of all three disorders focuses on the management of sleepiness, with additional treatment of cataplexy in those patients with narcolepsy type 1...
July 2015: Chest
Fabio Pizza, Isabelle Jaussent, Regis Lopez, Carole Pesenti, Giuseppe Plazzi, Xavier Drouot, Smaranda Leu-Semenescu, Severine Beziat, Isabelle Arnulf, Yves Dauvilliers
BACKGROUND: Drowsiness compromises driving ability by reducing alertness and attentiveness, and delayed reaction times. Sleep-related car crashes account for a considerable proportion of accident at the wheel. Narcolepsy type 1 (NT1), narcolepsy type 2 (NT2) and idiopathic hypersomnia (IH) are rare central disorders of hypersomnolence, the most severe causes of sleepiness thus being potential dangerous conditions for both personal and public safety with increasing scientific, social, and political attention...
2015: PloS One
Fabio Pizza, Stefano Vandi, Martina Iloti, Christian Franceschini, Rocco Liguori, Emmanuel Mignot, Giuseppe Plazzi
STUDY OBJECTIVES: To evaluate the reliability of nocturnal sleep dynamics in the differential diagnosis of central disorders of hypersomnolence. DESIGN: Cross-sectional. SETTING: Sleep laboratory. PATIENTS: One hundred seventy-five patients with hypocretin-deficient narcolepsy type 1 (NT1, n = 79), narcolepsy type 2 (NT2, n = 22), idiopathic hypersomnia (IH, n = 22), and "subjective" hypersomnolence (sHS, n = 52). INTERVENTIONS: None...
August 2015: Sleep
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