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Idiopathic hypersomnolence

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...
September 4, 2016: 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...
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
Karel Šonka, Marek Šusta, Michel Billiard
BACKGROUND: The successive editions of the International Classification of Sleep Disorders (ICSD) reflect the evolution of the concepts of various sleep disorders. This is particularly the case for central disorders of hypersomnolence, with continuous changes in terminology and divisions of narcolepsy, idiopathic hypersomnia, and recurrent hypersomnia. According to the ICSD 2nd Edition (ICSD-2), narcolepsy with cataplexy (NwithC), narcolepsy without cataplexy (Nw/oC), idiopathic hypersomnia with long sleep time (IHwithLST), and idiopathic hypersomnia without long sleep time (IHw/oLST) are four, well-defined hypersomnias of central origin...
February 2015: Sleep Medicine
Marco Filardi, Fabio Pizza, Monica Martoni, Stefano Vandi, Giuseppe Plazzi, Vincenzo Natale
OBJECTIVE: To evaluate the reliability of actigraphy to distinguish the features of estimated daytime and nighttime sleep between patients with central disorders of hypersomnolence and healthy controls. METHODS: Thirty-nine drug-naïve patients with Narcolepsy Type 1, twenty-four drug-naïve patients with Idiopathic Hypersomnia, and thirty age- and sex- matched healthy controls underwent seven days of actigraphic and self-report monitoring of sleep/wake behavior...
January 2015: Sleep Medicine
Anja Holm, Claus Heiner Bang-Berthelsen, Stine Knudsen, Signe Modvig, Birgitte Rahbek Kornum, Steen Gammeltoft, Poul J Jennum
MicroRNAs (miRNAs) are involved in the pathogenesis of many human diseases, including some neurological disorders. Recently, we have reported dysregulated miRNAs in plasma from patients with central hypersomnias including type 1 and type 2 narcolepsy, and idiopathic hypersomnia. This study addressed whether miRNA levels are altered in the cerebrospinal fluid (CSF) of patients with central hypersomnias. We conducted high-throughput analyses of miRNAs in CSF from patients using quantitative real-time polymerase chain reaction panels...
December 15, 2014: Journal of the Neurological Sciences
Soumiya Mudgal, R C Jiloha, Manish Kandpal, Aparna Das
Kleine-Levin syndrome (KLS) alias sleeping beauty syndrome, is a rare sleep disorder. Clinically presenting as episodes of hypersomnolence, behavioral and cognitive disturbances, hyperphagia and hypersexuality. KLS may have an idiopathic onset or may be precipitated by neurological event or infection. Until date, no definite underlying cause is established and neither there are any definitive management guidelines. It remains a diagnosis of exclusion after other psychiatric and neurological causes have been ruled out...
July 2014: Indian Journal of Psychiatry
Geert Mayer, Heike Benes, Peter Young, Marion Bitterlich, Andrea Rodenbeck
In 2010 the European Medicines Agency withdrew the indication of modafinil for the treatment of obstructive sleep apnea, shift work sleep disorder and for idiopathic hypersomnia (IH). In uncontrolled studies, modafinil has been reported to be efficacious in the treatment of sleep disorders. We therefore performed a randomized, placebo-controlled study with the aim of proving the efficacy of modafinil treatment in these patients. Drug-free IH patients without long sleep according to ICSD2 criteria, age >18 years and disease duration >2 years were included...
February 2015: Journal of Sleep Research
Anja Holm, Claus Heiner Bang-Berthelsen, Stine Knudsen, Birgitte R Kornum, Signe Modvig, Poul Jennum, Steen Gammeltoft
STUDY OBJECTIVES: MicroRNAs (miRNAs) have been implicated in the pathogenesis of human diseases including neurological disorders. The aim is to address the involvement of miRNAs in the pathophysiology of central hypersomnias including autoimmune narcolepsy with cataplexy and hypocretin deficiency (type 1 narcolepsy), narcolepsy without cataplexy (type 2 narcolepsy), and idiopathic hypersomnia. DESIGN: We conducted high-throughput analysis of miRNA in plasma from three groups of patients-with type 1 narcolepsy, type 2 narcolepsy, and idiopathic hypersomnia, respectively-in comparison with healthy controls using quantitative real-time polymerase chain reaction (qPCR) panels...
September 2014: Sleep
Olufemi Adenuga, Hrayr Attarian
In the absence of sleep deprivation (either because of behavioral or medical causes) or pharmacologically induced sleepiness, hypersomnia is a manifestation of one of the central disorders of hypersomnolence, such as narcolepsy types 1 and 2, idiopathic hypersomnia, and recurrent hypersomnias such as Kleine-Levin syndrome. Narcolepsy and most primary hypersomnias are chronic conditions, thus, before committing an individual to chronic, possibly, life-long treatments, an accurate diagnosis is important. The key to effective management of hypersomnia, thus, lies in a thorough history, detailed physical examination, and appropriate diagnostic tests...
September 2014: Current Treatment Options in Neurology
Smaranda Leu-Semenescu, Nandy Nittur, Jean-Louis Golmard, Isabelle Arnulf
OBJECTIVE: To evaluate the benefits and risks of pitolisant (a wake-enhancing drug that increases the histamine release in the brain by blocking presynaptic H3 histamine reuptake) in patients with idiopathic (IH) and symptomatic (SH) hypersomnia plus sleepiness refractory to available stimulants (modafinil, methylphenidate, mazindol, sodium oxybate, and d-amphetamine). METHODS: Through retrospective analyses of patient files, the benefit (the score from the Epworth Sleepiness Scale [ESS], authorization renewal) and tolerance (side-effects) of pitolisant were assessed...
June 2014: Sleep Medicine
Pierre Philip, Cyril Chaufton, Jacques Taillard, Aurore Capelli, Olivier Coste, Damien Léger, Nicholas Moore, Patricia Sagaspe
STUDY OBJECTIVE: Patients with excessive daytime sleepiness (EDS) are at high risk for driving accidents, and physicians are concerned by the effect of alerting drugs on driving skills of sleepy patients. No study has up to now investigated the effect of modafinil (a reference drug to treat EDS in patients with hypersomnia) on on-road driving performance of patients suffering from central hypersomnia. The objective is to evaluate in patients with central hypersomnia the effect of a wake-promoting drug on real driving performance and to assess the relationship between objective sleepiness and driving performance...
March 1, 2014: Sleep
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