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Idiopathic hypersomnia

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https://www.readbyqxmd.com/read/29445282/orexin-hypocretin-levels-in-the-cerebrospinal-fluid-and-characteristics-of-patients-with-myotonic-dystrophy-type-1-with-excessive-daytime-sleepiness
#1
Yuki Omori, Takashi Kanbayashi, Aya Imanishi, Ko Tsutsui, Yohei Sagawa, Yuka S Kikuchi, Masahiro Takeshima, Kazuhisa Yoshizawa, Sachiko Uemura, Tetsuo Shimizu
Purpose: Myotonic dystrophy type 1 (DM1) is often characterized by excessive daytime sleepiness (EDS) and sleep-onset rapid eye movement periods caused by muscleblind-like protein 2. The EDS tends to persist even after treatment of sleep apnea. We measured the cerebrospinal fluid (CSF) orexin levels in DM1 patients with EDS and compared the clinical characteristics with narcolepsy type 1 and idiopathic hypersomnia (IHS) patients. Patients and methods: We measured the CSF orexin levels in 17 DM1 patients with EDS and evaluated subjective sleepiness using the Epworth Sleepiness Scale (ESS), objective sleepiness using mean sleep latency (MSL), and sleep apnea using apnea-hypopnea index (AHI)...
2018: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/29351827/nocturnal-rem-sleep-without-atonia-is-a-diagnostic-biomarker-of-pediatric-narcolepsy
#2
Saadoun Bin-Hasan, Aleksandar Videnovic, Kiran Maski
STUDY OBJECTIVES: Compare nocturnal REM sleep without atonia (nRWA) and REM sleep behavior disorder (RBD) between pediatric patients with and without narcolepsy and determine if the nRWA index is a valid diagnostic biomarker for narcolepsy. METHODS: Retrospective cohort study of children ages 6 to 18 years who completed a nocturnal polysomnogram (PSG) and Multiple Sleep Latency Test (MSLT). Our study sample included 11 patients with narcolepsy type 1 (NT1), 6 with narcolepsy type 2 (NT2), 12 with idiopathic hypersomnia (IH), and 11 with subjective hypersomnia (sHS)...
January 16, 2018: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/29340766/wanted-a%C3%A2-better-cut-off-value-for-the-epworth-sleepiness-scale
#3
Karin Trimmel, Magdalena Żebrowska, Marion Böck, Andrijana Stefanic, Daniel Mayer, Gerhard Klösch, Eduard Auff, Stefan Seidel
BACKGROUND: Excessive daytime sleepiness (EDS) is the main complaint in many neurological sleep disorders, such as idiopathic hypersomnia, narcolepsy, or obstructive sleep apnea/hypopnea syndrome (OSAS). The validity of the Epworth Sleepiness Scale (ESS) as a screening tool for EDS remains controversial. We therefore investigated (1) the interrelation of the ESS total score and the mean sleep latency (MSL) during the multiple sleep latency test (MSLT) and (2) the diagnostic accuracy of the ESS total score to detect EDS in patients with the chief complaint of subjective EDS...
January 16, 2018: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/29323727/alternative-diagnostic-criteria-for-idiopathic-hypersomnia-a-32-hour-protocol
#4
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
https://www.readbyqxmd.com/read/29276000/a-standardized-test-to-document-cataplexy
#5
Stefano Vandi, Fabio Pizza, Elena Antelmi, Giulia Neccia, Martina Iloti, Alice Mazzoni, Patrizia Avoni, Giuseppe Plazzi
OBJECTIVE/BACKGROUND: Cataplexy is the pathognomonic symptom of narcolepsy type 1 (NT1). Since it is considered difficult to be directly observed or documented by clinicians, its diagnosis relies mainly on history taking. Our study aimed at testing the feasibility of a standardized video recording procedure under emotional stimulation to document cataplexy in the diagnostic work-up of suspected hypersomnia of central origin. PATIENTS/METHODS: Two-hundred-eight consecutive patients underwent the diagnostic work-up and reached the final diagnosis of NT1 (n = 133), idiopathic hypersomnia or narcolepsy type 2 (IH/NT2 group, n = 33), or subjective excessive daytime sleepiness (sEDS group, n = 42)...
October 9, 2017: Sleep Medicine
https://www.readbyqxmd.com/read/29250981/update-on-treatment-for-idiopathic-hypersomnia
#6
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 Clinicaltrial.gov database from 1997 to 2017...
January 3, 2018: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/29198301/the-mslt-is-repeatable-in-narcolepsy-type-1-but-not-narcolepsy-type-2-a-retrospective-patient-study
#7
Chad Ruoff, Fabio Pizza, Lynn Marie Trotti, Karel Sonka, Stefano Vandi, Joseph Cheung, Swaroop Pinto, Mali Einen, Narong Simakajornboon, Fang Han, Paul Peppard, Sona Nevsimalova, Giuseppe Plazzi, David Rye, Emmanuel Mignot
STUDY OBJECTIVES: To examine repeatability of Multiple Sleep Latency Test (MSLT) results in narcolepsy type 1 (NT1) and narcolepsy type 2 (NT2) according to the criteria of the International Classification of Sleep Disorders, Third Edition (ICSD-3). METHODS: Repeatability of the MSLT was retrospectively evaluated in NT1 (n = 60) and NT2 (n = 54) cases, and controls (n = 15). All subjects had documented HLA-DQB1*06:02 status and/or hypocretin-1 levels from cerebrospinal fluid...
November 29, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/29099966/test-retest-reliability-of-the-multiple-sleep-latency-test-in-central-disorders-of-hypersomnolence
#8
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 non-cataplectic central disorders of hypersomnolence (NCHS): type 2 (NT2), idiopathic hypersomnia (IH) and unspecified hypersomnolence (UnsEDS). Methods: PSG-MSLT in drug-free conditions was administered twice (median interval of 1...
November 1, 2017: Sleep
https://www.readbyqxmd.com/read/29047171/effectiveness-and-side-effect-profile-of-stimulant-therapy-as-monotherapy-and-in-combination-in-the-central-hypersomnias-in-clinical-practice
#9
Chiraag Thakrar, Kishankumar Patel, Grainne D'ancona, Brian D Kent, Alexander Nesbitt, Hugh Selsick, Joerg Steier, Ivana Rosenzweig, Adrian J Williams, Guy D Leschziner, Panagis Drakatos
Effectiveness and side-effect profile data on pharmacotherapy for daytime sleepiness in central hypersomnias are based largely upon randomized controlled trials. Evidence regarding the use of combination therapy is scant. The aim of this study was to examine the effectiveness and occurrence of drug-related side effects of these drugs in routine clinical practice. Adult patients diagnosed with a central hypersomnia during a 54-month period at a tertiary sleep disorders centre were identified retrospectively...
October 19, 2017: Journal of Sleep Research
https://www.readbyqxmd.com/read/28958044/altered-regional-cerebral-blood-flow-in-idiopathic-hypersomnia
#10
Soufiane Boucetta, Jacques Montplaisir, Antonio Zadra, Francis Lachapelle, Jean-Paul Soucy, Paul Gravel, Thien Thanh Dang-Vu
Objectives: Idiopathic hypersomnia is characterized by excessive daytime sleepiness despite normal or long sleep time. Its pathophysiological mechanisms remain unclear. This pilot study aims at characterizing the neural correlates of idiopathic hypersomnia using single photon emission computed tomography. Methods: Thirteen participants with idiopathic hypersomnia and sixteen healthy controls were scanned during resting wakefulness using a high-resolution single photon emission computed tomography scanner with 99mTc-ethyl cysteinate dimer to assess cerebral blood flow...
August 23, 2017: Sleep
https://www.readbyqxmd.com/read/28853423/daytime-sleepiness-more-than-just-obstructive-sleep-apnea-osa
#11
Luigi Ferini-Strambi, Marco Sforza, Mattia Poletti, Federica Giarrusso, Andrea Galbiati
Excessive Daytime Sleepiness (EDS) is a common condition with a significant impact on quality of life and general health. A mild form of sleepiness can be associated with reduced reactivity and modest distractibility symptoms, but more severe symptomatic forms are characterized by an overwhelming and uncontrollable need to sleep, causing sudden sleep attacks, amnesia and automatic behaviors. The prevalence in the general population is between 10 and 25%. Furthermore, EDS has been considered a core symptom of obstructive sleep apnea (OSA), as well as being the main symptom of primary hypersomnias such as narcolepsy types 1 and 2, and idiopathic hypersomnia...
August 28, 2017: La Medicina del Lavoro
https://www.readbyqxmd.com/read/28822033/clinical-and-polysomnographic-characteristics-of-excessive-daytime-sleepiness-in-children
#12
Jiwon Lee, Geonyoub Na, Eun Yeon Joo, Munhyang Lee, Jeehun Lee
PURPOSE: This study aimed to delineate the clinical and polysomnography (PSG) characteristics of sleep disorders in children with excessive daytime sleepiness (EDS). METHODS: Between February 2002 and June 2015, 622 pediatric patients with EDS were evaluated with overnight PSG and the Multiple Sleep Latency Test at the Samsung Medical Center. The medical records; questionnaire responses about depression, sleepiness, sleep habits; and sleep study data of 133 patients without obstructive sleep apnea (OSA) were reviewed retrospectively...
August 18, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/28777172/narcolepsy-and-other-central-hypersomnias
#13
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
https://www.readbyqxmd.com/read/28771870/the-perception-and-attention-functions-test-battery-as-a-measure-of-neurocognitive-impairment-in-patients-with-suspected-central-disorders-of-hypersomnolence
#14
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
https://www.readbyqxmd.com/read/28735675/treatment-of-narcolepsy-and-other-organic-hypersomnias-in-children
#15
REVIEW
Suresh Kotagal
The comprehensive management of chronic disorders such as hypersomnias of childhood requires combining life-style changes with rational pharmacotherapy that is based on treating the symptoms that are most bothersome, the age, comorbidities, and metabolic and endocrine status of the patient. The excessive sleepiness of narcolepsy and idiopathic hypersomnia is best treated with dextroamphetamine or methylphenidate preparations or modafinil/armodafinil. Cataplexy treatment requires sodium oxybate, tricyclic agents, selective norepinephrine reuptake inhibitors or selective serotonin reuptake inhibitors...
June 20, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28656632/serum-brain-derived-neurotrophic-factor-bdnf-in-sleep-disordered-patients-relation-to-sleep-stage-n3-and-rapid-eye-movement-rem-sleep-across-diagnostic-entities
#16
Michael Deuschle, Michael Schredl, Christian Wisch, Claudia Schilling, Maria Gilles, Olga Geisel, Rainer Hellweg
Experimental and clinical evidence suggests an association between neuroplasticity, brain-derived neurotrophic factor and sleep. We aimed at testing the hypotheses that brain-derived neurotrophic factor is associated with specific aspects of sleep architecture or sleep stages in patients with sleep disorders. We included 35 patients with primary insomnia, 31 patients with restless legs syndrome, 17 patients with idiopathic hypersomnia, 10 patients with narcolepsy and 37 healthy controls. Morning serum brain-derived neurotrophic factor concentrations were measured in patients and controls...
June 28, 2017: Journal of Sleep Research
https://www.readbyqxmd.com/read/28376374/brain-mri-findings-in-patients-with-idiopathic-hypersomnia
#17
Lynn Marie Trotti, Donald L Bliwise
OBJECTIVE: Proper diagnosis of idiopathic hypersomnia necessitates the exclusion of neurologic or medical causes of sleepiness that better explain the clinical syndrome. However, there are no formal guidelines regarding the use of neuroimaging to identify such secondary causes of symptoms. We sought to characterize brain MRI findings in a series of patients with idiopathic hypersomnia. METHODS: We reviewed medical records on a consecutive series of 61 patients diagnosed with idiopathic hypersomnia to determine the frequency and results of brain magnetic resonance imaging (MRI)...
June 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28364448/cerebrospinal-fluid-biomarkers-of-neurodegeneration-are-decreased-or-normal-in-narcolepsy
#18
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 the 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)...
January 1, 2017: Sleep
https://www.readbyqxmd.com/read/28328583/case-report-of-a-patient-with-idiopathic-hypersomnia-and-a-family-history-of-malignant-hyperthermia-undergoing-general-anesthesia-an-overview-of-the-anesthetic-considerations
#19
Sena Aflaki, Sally Hu, Rami A Kamel, Frances Chung, Mandeep Singh
The pathophysiologic underpinnings of idiopathic hypersomnia and its interactions with anesthetic medications remain poorly understood. There is a scarcity of literature describing this patient population in the surgical setting. This case report outlines the anesthetic considerations and management plan for a 55-year-old female patient with a known history of idiopathic hypersomnia undergoing an elective shoulder arthroscopy in the ambulatory setting. In addition, this case offers a unique set of considerations and conflicts related to the patient having a family history of malignant hyperthermia...
March 17, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28243864/hypersomnolence-hypersomnia-and-mood-disorders
#20
REVIEW
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
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