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Idiopathic hypersomnia

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https://www.readbyqxmd.com/read/27865546/french-consensus-management-of-patients-with-hypersomnia-which-strategy
#1
R Lopez, I Arnulf, X Drouot, M Lecendreux, Y Dauvilliers
Central hypersomnias principally involves type 1 narcolepsy (NT1), type 2 narcolepsy (NT2) and idiopathic hypersomnia (IH). Despite great progress made in understanding the physiopathology of NT1 with low cerebrospinal fluid hypocretin-1 levels, current treatment remains symptomatic. The same applies to NT2 and IH, for which the physiopathology is still largely unknown. Controlling excessive daytime sleepiness (EDS), cataplexy, hypnagogic hallucinations, sleep paralysis and disturbed night-time sleep are key therapeutic targets in NT1...
November 16, 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27864522/comorbidity-between-central-disorders-of-hypersomnolence-and-immune-based-disorders
#2
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...
November 18, 2016: Neurology
https://www.readbyqxmd.com/read/27810179/diurnal-and-nocturnal-cardiovascular-variability-and-heart-rate-arousal-response-in-idiopathic-hypersomnia
#3
Emilia Sforza, Frédéric Roche, Jean Claude Barthélémy, Vincent Pichot
OBJECTIVES: Autonomic nervous system dysfunction has been described in narcolepsy with cataplexy affecting sympathetic functions. In this study we analyzed whether altered diurnal and nocturnal cardiovascular control is present in idiopathic hypersomnia (IH). METHODS: Fourteen drug-free patients aged 26.2 ± 7 years and 14 age-matched controls were examined. Clinical data, 24-h polysomnography, heart rate (HR) variability, and the HR response to spontaneous arousal were available...
August 2016: Sleep Medicine
https://www.readbyqxmd.com/read/27692973/waking-up-is-the-hardest-thing-i-do-all-day-sleep-inertia-and-sleep-drunkenness
#4
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
https://www.readbyqxmd.com/read/27634789/cerebrospinal-fluid-biomarkers-of-neurodegeneration-are-decreased-or-normal-in-narcolepsy
#5
Poul Jørgen Jennum, Lars Østergaard Pedersen, Justyna Maria Czarna Bahl, Signe Modvig, Karina Fog, Anja Holm, Birgitte Rahbek Kornum, Steen Gammeltoft
OBJECTIVES: To investigate whether cerebrospinal fluid (CSF) biomarkers of neurodegeneration are altered in narcolepsy in order to evaluate whether the hypocretin deficiency and abnormal sleep-wake pattern in narcolepsy leads to neurodegeneration. METHODS: Twenty-one patients with central hypersomnia (10 type 1 narcolepsy, 5 type 2 narcolepsy, and 6 idiopathic hypersomnia cases) aged 33 years on average, and with a disease duration of 2-29 years, and 12 healthy controls underwent CSF analyses of levels of β-amyloid, total tau protein (T-tau), phosphorylated tau protein (P-tau181), α-synuclein, neurofilament light chain (NF-L), and chitinase 3-like protein-1 (CHI3L1)...
September 9, 2016: Sleep
https://www.readbyqxmd.com/read/27568897/urine-toxicology-in-adults-evaluated-for-a-central-hypersomnia-and-how-the-results-modify-the-physician-s-diagnosis
#6
Christopher A Kosky, Anastasios Bonakis, Arthee Yogendran, Gihan Hettiarachchi, Paul I Dargan, Adrian J Williams
STUDY OBJECTIVES: Drugs and psychoactive substances can cause sleepiness and when undetected, may lead to over diagnosis of central hypersomnias. We performed urine drug testing using gas chromatography-mass spectrometry in adults undergoing multiple sleep latency testing (MSLT) for a suspected central hypersomnia. We examined how the drug test results modified the treating physician's diagnosis. METHODS: One hundred eighty-six consecutive patients with a suspected central hypersomnia who underwent clinical assessment, MSLT and urine drug testing by gas chromatography-mass spectrometry were retrospectively studied...
November 15, 2016: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/27568806/aberrant-food-choices-after-satiation-in-human-orexin-deficient-narcolepsy-type-1
#7
Ruth Janke van Holst, Lisa van der Cruijsen, Petra van Mierlo, Gert Jan Lammers, Roshan Cools, Sebastiaan Overeem, Esther Aarts
STUDY OBJECTIVES: Besides influencing vigilance, orexin neurotransmission serves a variety of functions, including reward, motivation, and appetite regulation. As obesity is an important symptom in orexin-deficient narcolepsy, we explored the effects of satiety on food-related choices and spontaneous snack intake in patients with narcolepsy type 1 (n = 24) compared with healthy matched controls (n = 19). In additional analyses, we also included patients with idiopathic hypersomnia (n = 14) to assess sleepiness-related influences...
November 1, 2016: Sleep
https://www.readbyqxmd.com/read/27548094/the-effects-of-transcranial-direct-current-stimulation-tdcs-on-idiopathic-hypersomnia-a-pilot-study
#8
Andrea Galbiati, Jubin Abutalebi, Sandro Iannaccone, Virginia Maria Borsa, Stela Musteata, Marco Zucconi, Enrico Giora, Luigi Ferini-Strambi
Idiopathic Hypersomnia (IH) is a rare sleep disorder characterised by excessive daytime sleepiness (EDS) that leads to invalidating daytime consequences. Till now the treatment of IH has mirrored that of sleepiness in narcolepsy, and it is mainly focused on symptoms' management. We employed an anodal transcranic Direct Current Stimulation (tDCS) treatment in order to induce a shift toward arousal in IH patients' cortex during the day. Every patients underwent a 4 weeks treatment (3 stimulations per week, for a total of 12 stimulations over a period of 28 days) with an assessment at the baseline and after treatment aimed to the evaluation of subjective daytime sleepiness, neurocognitive functions, and attentional domain tested by means of the Attentional Network Task (ANT)...
April 1, 2016: Archives Italiennes de Biologie
https://www.readbyqxmd.com/read/27542882/disorders-of-excessive-daytime-sleepiness-including-narcolepsy-and-idiopathic-hypersomnia
#9
REVIEW
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
https://www.readbyqxmd.com/read/27538329/-management-of-central-hypersomnias
#10
Yves Dauvilliers, Régis Lopez
Central hypersomnias include narcolepsy type 1, type 2 and idiopathic hypersomnia with daytime sleepiness excessive in the foreground of the clinical symptoms. Despite major advances in our understanding of the mechanisms of the narcolepsy type 1 with a low level of hypocretin-1 in cerebrospinal fluid, its current management is only symptomatic. The current management is also only symptomatic for type 2 narcolepsy and idiopathic hypersomnia with an unknown pathophysiology. Treatment options may vary from a single drug targeting several symptoms or several drugs treating a specific symptom...
June 2016: La Revue du Praticien
https://www.readbyqxmd.com/read/27538325/-clinical-investigation-of-an-excessive-sleepiness-complaint
#11
Elisa Evangelista, Lucie Barateau, Yves Dauvilliers
Excessive sleepiness is a common problem, defined by a complaint of excessive daytime sleepiness almost daily with an inability to stay awake and alert dosing periods at sleep, with episodes of irresistible sleep need or drowsiness or non-intentional sleep, or by a night's sleep time overly extended often associated with sleep inertia. This sleepiness is variable in terms of phenotype and severity to be specified by the out-patient clinic. It is considered to be chronic beyond three months and often responsible for significant functional impairment of school and professional performance, of the accidents and cardiovascular risk...
June 2016: La Revue du Praticien
https://www.readbyqxmd.com/read/27417057/decreased-sleep-stage-transition-pattern-complexity-in-narcolepsy-type-1
#12
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
https://www.readbyqxmd.com/read/27315195/absence-of-%C3%AE-aminobutyric-acid-a-receptor-potentiation-in-central-hypersomnolence-disorders
#13
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
https://www.readbyqxmd.com/read/27166243/psychosocial-profile-and-quality-of-life-in-children-with-type-1-narcolepsy-a-case-control-study
#14
Francesca Letizia Rocca, Elena Finotti, Fabio Pizza, Francesca Ingravallo, Michela Gatta, Oliviero Bruni, Giuseppe Plazzi
STUDY OBJECTIVES: To investigate behavioral aspects and quality of life in children and adolescents with type 1 narcolepsy (NT1). METHODS: We performed a case-control study comparing 29 patients with NT1 versus sex- and age-matched patients with idiopathic epilepsy (n = 39) and healthy controls (n = 39). Behavior and quality of life were evaluated by self-administered questionnaires (Child Behavior Checklist, Pediatric Quality of Life Inventory). Patient groups were contrasted and scale results were correlated with clinical and polysomnographic parameters, and cerebrospinal fluid hypocretin-1 levels...
2016: Sleep
https://www.readbyqxmd.com/read/26943713/examining-the-frequency-of-stimulant-misuse-among-patients-with-primary-disorders-of-hypersomnolence-a-retrospective-cohort-study
#15
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
https://www.readbyqxmd.com/read/26935190/kleine-levin-syndrome-an-overview-and-relevance-to-nursing-practice
#16
Jacqueline M Arnone, Richard P Conti
Kleine-Levin syndrome (KLS) is a neuropsychiatric sleep disorder primarily affecting adolescent males. Onset is insidious, idiopathic, and hastened by neurological incident or infection. Typically, the initial onset occurs during the teen years or after the second decade, although cases have been documented in early childhood, adulthood, and senescence. KLS is marked by unexpected debilitating, yet reversible, episodic hypersomnia, with varying recurrence rates; cognitive and behavioral impairment; compulsive eating; and feelings of derealization, hypersexuality, apathy, and depressed mood...
March 2016: Journal of Psychosocial Nursing and Mental Health Services
https://www.readbyqxmd.com/read/26864219/which-diagnostic-findings-in-disorders-with-excessive-daytime-sleepiness-are-really-helpful-a-retrospective-study
#17
Ute Kretzschmar, Esther Werth, Christian Sturzenegger, Ramin Khatami, Claudio L Bassetti, Christian R Baumann
Due to extensive clinical and electrophysiological overlaps, the correct diagnosis of disorders with excessive daytime sleepiness is often challenging. The aim of this study was to provide diagnostic measures that help discriminating such disorders, and to identify parameters, which don't. In this single-center study, we retrospectively identified consecutive treatment-naïve patients who suffered from excessive daytime sleepiness, and analyzed clinical and electrophysiological measures in those patients in whom a doubtless final diagnosis could be made...
June 2016: Journal of Sleep Research
https://www.readbyqxmd.com/read/26847972/benefits-and-risk-of-sodium-oxybate-in-idiopathic-hypersomnia-versus-narcolepsy-type-1-a-chart-review
#18
Smaranda Leu-Semenescu, Pauline Louis, Isabelle Arnulf
BACKGROUND: Few stimulants have been evaluated for the treatment of idiopathic hypersomnia (IH). Sodium oxybate (indicated in narcolepsy type 1, NT1) has not been tested in IH patients. OBJECTIVE: The aim of this study is to retrospectively evaluate the benefit/risk ratio of sodium oxybate in IH versus NT1 using a chart review. METHODS: We reviewed the files of 46 patients with IH (35.7 ± 12.6 years old, 78% women) and 47 patients with NT1 (44...
January 2016: Sleep Medicine
https://www.readbyqxmd.com/read/26788889/behavioral-sleep-medicine-services-for-hypersomnia-disorders-a-survey-study
#19
Ariel B Neikrug, Megan R Crawford, Jason C Ong
Patients with hypersomnia disorders (HD) suffer from debilitating symptoms that result in reduced functioning, depression, anxiety, and overall worse quality of life. Little is known about the need and desire of this population to utilize behavioral sleep medicine (BSM) interventions that focus on psychosocial functioning and quality of life, and there have been limited attempts to develop such interventions. The purpose of this survey study was to gather patient-centered data on engagement in pharmacological and nonpharmacological interventions, the psychosocial impact of HD symptoms on quality of life and mental health, and potential interest in BSM services, such as cognitive behavioral therapy, mindfulness or yoga, and support groups...
January 20, 2016: Behavioral Sleep Medicine
https://www.readbyqxmd.com/read/26738920/decline-of-csf-orexin-hypocretin-levels-in-prader-willi-syndrome
#20
Mayu Omokawa, Tadayuki Ayabe, Toshiro Nagai, Aya Imanishi, Ayumi Omokawa, Seiji Nishino, Yohei Sagawa, Tetsuo Shimizu, Takashi Kanbayashi
Prader-Willi syndrome is a congenital neurodevelopmental disorder resulting from deletion of the paternal copies of genes within the chromosome region 15q11-q13. Patients with Prader-Willi syndrome often exhibit excessive daytime sleepiness, excessive appetite, and obesity. As is the case in narcolepsy, orexin (hypocretin) may be responsible for these symptoms. However, reports showing cerebrospinal fluid orexin levels in Prader-Willi syndrome patients have been limited. The aim of this study was to examine the relationship between the characteristic symptoms of Prader-Willi syndrome and cerebrospinal fluid orexin levels...
May 2016: American Journal of Medical Genetics. Part A
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