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rem narcolepsy

Alexander Neergaard Olesen, Matteo Cesari, Julie Anja Engelhard Christensen, Helge Bjarup Dissing Sorensen, Emmanuel Mignot, Poul Jennum
OBJECTIVE: To evaluate rapid eye movement (REM) muscular activity in narcolepsy by applying five algorithms to electromyogram (EMG) recordings, and to investigate its value for narcolepsy diagnosis. PATIENTS/METHODS: A modified version of phasic EMG metric (mPEM), muscle activity index (MAI), REM atonia index (RAI), supra-threshold REM EMG activity metric (STREAM), and Frandsen method (FR) were calculated from polysomnography recordings of 20 healthy controls, 18 clinic controls (subjects suspected with narcolepsy but finally diagnosed without any sleep abnormality), 16 narcolepsy type one without REM sleep behavior disorder (RBD), nine narcolepsy type one with RBD, and 18 narcolepsy type two...
April 2018: Sleep Medicine
Alexis Roman, Soraya Meftah, Sébastien Arthaud, Pierre-Hervé Luppi, Christelle Peyron
Narcolepsy type 1 is a disabling disorder with four primary symptoms: excessive-daytime-sleepiness, cataplexy, hypnagogic hallucinations and sleep paralysis. The three latter symptoms together with a short REM sleep latency have suggested impairment in REM sleep homeostatic regulation with an enhanced propensity for (i.e. tendency to enter) REM sleep. To test this hypothesis, we challenged REM sleep homeostatic regulation in a recognized model of narcolepsy, the orexin knock-out (Orex-KO) mice and their wild-type (WT) littermates...
March 7, 2018: Sleep
A V Strobel, H Tankisi, N B Finnerup, A Fuglsang-Frederiksen, P Jennum, K B Svendsen, F I Kirov, M Otto
BACKGROUND: Idiopathic REM sleep behaviour disorder (iRBD) has been recognised as a significant biomarker for developing a neurodegenerative alpha-synucleinopathy, which is why iRBD is considered to be a prodromal state for alpha-synucleinopathies including Parkinson's disease (PD). Many patients with PD suffer from complaints of pain and present impaired somatosensory function. We hypothesized that pain perception and somatosensory function could be altered already in a preclinical stage of PD including iRBD...
February 2018: Sleep Medicine
Helle Sadam, Arno Pihlak, Anri Kivil, Susan Pihelgas, Mariliis Jaago, Priit Adler, Jaak Vilo, Olli Vapalahti, Toomas Neuman, Dan Lindholm, Markku Partinen, Antti Vaheri, Kaia Palm
BACKGROUND: Neuropathological findings support an autoimmune etiology as an underlying factor for loss of orexin-producing neurons in spontaneous narcolepsy type 1 (narcolepsy with cataplexy; sNT1) as well as in Pandemrix influenza vaccine-induced narcolepsy type 1 (Pdmx-NT1). The precise molecular target or antigens for the immune response have, however, remained elusive. METHODS: Here we have performed a comprehensive antigenic repertoire analysis of sera using the next-generation phage display method - mimotope variation analysis (MVA)...
February 2, 2018: EBioMedicine
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
Delphine Oudiette, Pauline Dodet, Nahema Ledard, Emilie Artru, Inès Rachidi, Thomas Similowski, Isabelle Arnulf
Breathing is irregular during rapid eye-movement (REM) sleep, whereas it is stable during non-REM sleep. Why this is so remains a mystery. We propose that irregular breathing has a cortical origin and reflects the mental content of dreams, which often accompany REM sleep. We tested 21 patients with narcolepsy who had the exceptional ability to lucid dream in REM sleep, a condition in which one is conscious of dreaming during the dream and can signal lucidity with an ocular code. Sleep and respiration were monitored during multiple naps...
February 8, 2018: Scientific Reports
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)...
February 15, 2018: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
Adriana Bosco, Régis Lopez, Lucie Barateau, Sofiene Chenini, Carole Pesenti, Jean-Louis Pépin, Isabelle Jaussent, Yves Dauvilliers
OBJECTIVE: To assess the effect of psychostimulant treatments on the 24-hour blood pressure (BP) profile of patients with narcolepsy type 1 (NT1). METHODS: Heart rate (HR) and BP were monitored for 24 hours and morning endothelial function was evaluated in 160 consecutive patients with NT1: 68 untreated (41 male, median age 34.9 years), 54 treated (32 male, median age 40.9 years), and 38 evaluated twice (21 male, median age 32 years), before and during treatment...
January 10, 2018: Neurology
Yves Dauvilliers, Thomas Roth, Diane Guinta, Sarah Alvarez-Horine, Efim Dynin, Jed Black
OBJECTIVE: To assess the effects of three narcolepsy treatment modalities on sleep stage shifts associated with disrupted nighttime sleep (DNS) using data from a clinical trial. METHODS: Polysomnograms were reviewed from 155 patients (who had these data available at baseline and 8 weeks) of the 278 patients who were randomized to placebo, 9-g sodium oxybate (SXB)/nightly, 200-600 mg/d modafinil, or SXB + modafinil. Major outcomes of these post hoc analyses, analyzed using analysis of covariance, were change from baseline in number of shifts from Stages N2/3/rapid eye movement (REM) to Stage N1/Wake, and from Stage N1/Wake to REM...
December 2017: Sleep Medicine
Hiromi Toyoda, Yoshiko Honda, Susumu Tanaka, Taku Miyagawa, Makoto Honda, Kazuki Honda, Katsushi Tokunaga, Tohru Kodama
Narcolepsy is caused by the loss of hypocretin (Hcrt) neurons and is associated with multiple genetic and environmental factors. Although abnormalities in immunity are suggested to be involved in the etiology of narcolepsy, no decisive mechanism has been established. We previously reported chemokine (C-C motif) receptor 3 (CCR3) as a novel susceptibility gene for narcolepsy. To understand the role of CCR3 in the development of narcolepsy, we investigated sleep-wake patterns of Ccr3 knockout (KO) mice. Ccr3 KO mice exhibited fragmented sleep patterns in the light phase, whereas the overall sleep structure in the dark phase did not differ between Ccr3 KO mice and wild-type (WT) littermates...
2017: PloS One
John Peever, Patrick M Fuller
Considerable advances in our understanding of the mechanisms and functions of rapid-eye-movement (REM) sleep have occurred over the past decade. Much of this progress can be attributed to the development of new neuroscience tools that have enabled high-precision interrogation of brain circuitry linked with REM sleep control, in turn revealing how REM sleep mechanisms themselves impact processes such as sensorimotor function. This review is intended to update the general scientific community about the recent mechanistic, functional and conceptual developments in our current understanding of REM sleep biology and pathobiology...
November 20, 2017: Current Biology: CB
Claudio L Bassetti, Panagiotis Bargiotas
Rapid eye movement sleep behavior disorder (RBD) is a brain disorder, characterized by the dream enactment during rapid eye movement (REM) sleep due to a lack of physiologic muscle atonia and increased muscle twitching. Schenk was the first to describe this disorder in 1986; however, few authors reported in the 1970-1980s loss of physiological muscle atonia combined with dream enactment in the course of brainstem disorders and as a consequence of alcoholism and antidepressant treatment. RBD affects less than 1% of the adult population, but can be found in up to 25-50% of neurodegenerative disorders including Parkinson's disease, multisystem atrophy, and dementia with Lewy body...
2018: Frontiers of Neurology and Neuroscience
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
T Murer, L L Imbach, M Hackius, R N Taddei, E Werth, R Poryazova, Y V Gavrilov, S Winkler, D Waldvogel, C R Baumann, P O Valko
Objective: Multiple sleep onset rapid eye movement (R) periods (SOREMPs) and a mean sleep latency ≤8min on the multiple sleep latency test (MSLT) are diagnostic criteria of narcolepsy (NC), but occur also in other conditions with increased sleep pressure, including insufficient sleep syndrome (ISS), sleep disordered breathing (SDB) or Parkinson's disease (PD). These false positives are common, may create diagnostic uncertainty, and highlight the need for complementary MSLT measures with high specificity for narcolepsy...
October 23, 2017: Sleep
Sarah F Schoch, Esther Werth, Rositsa Poryazova, Thomas E Scammell, Christian R Baumann, Lukas L Imbach
Study Objectives: Patients with narcolepsy suffer from poor maintenance of wakefulness and fragmented night sleep, but the underlying mechanism of sleep boundary dysregulation remains little understood. The goal of this study was to quantify abnormal sleep-wake regulation in narcolepsy patients. Methods: Using a model-based approach (state space analysis), we analyzed overnight EEG recordings in ten patients with narcolepsy type 1 and age- and gender-matched healthy control subjects...
October 5, 2017: Sleep
Chiara Berteotti, Alessandro Silvani
Narcolepsy is a rare disease that entails excessive daytime sleepiness, often associated with sudden episodes of muscle weakness known as cataplexy. Narcolepsy with cataplexy (NC) is due to the loss of hypothalamic neurons that release the neuropeptides orexin A and B. Orexin neuron projections prominently target brain structures involved in wake-sleep state switching and the central autonomic network. This review provides an updated summary of the links between NC and autonomic cardiovascular dysfunction from a translational perspective...
October 10, 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
Line Pickering, Marianne Klose, Ulla Feldt-Rasmussen, Poul Jennum
PURPOSE: The purpose of this study is to evaluate whether damage to the hypothalamus due to craniopharyngioma or consequent surgery may involve the sleep-wake regulatory system, resulting in sleep disturbances and sleepiness. METHODS: Seven craniopharyngioma patients and 10 healthy controls were evaluated with sleep questionnaires including the Epworth Sleepiness Scale, polysomnography, and a multiple sleep latency test (MSLT). Five patients and eight controls had lumbar puncture performed to determine hypocretin-1 levels...
December 2017: Sleep & Breathing, Schlaf & Atmung
Elie Matar, Simon Jg Lewis
Rapid eye movement (REM) sleep behaviour disorder (RBD) is a parasomnia characterised by the loss of the normal atonia during the REM stage of sleep, resulting in overt motor behaviours that usually represent the enactment of dreams. Patients will seek medical attention due to sleep-related injuries or unpleasant dream content. Idiopathic RBD which occurs independently of any other disease occurs in up to 2% of the older population. Meanwhile, secondary RBD is very common in association with certain neurodegenerative conditions...
September 18, 2017: Medical Journal of Australia
Suresh Kotagal
PURPOSE OF REVIEW: Sleep-wake disorders occur in 10% to 28% of children and differ somewhat in pathophysiology and management from sleep-wake disorders in adults. This article discusses the diagnosis and management of key childhood sleep disorders. RECENT FINDINGS: The role of sleep in memory consolidation and in the facilitation of learning has been increasingly recognized, even at the toddler stage. Cataplexy, a key feature of narcolepsy type 1, may be subtle in childhood and characterized by transient muscle weakness isolated to the face...
August 2017: Continuum: Lifelong Learning in Neurology
Michael H Silber
PURPOSE OF REVIEW: This article provides a clinical approach to the appropriate investigation and diagnosis of sleep disorders commonly seen by neurologists. RECENT FINDINGS: Home sleep apnea testing in appropriate situations can replace laboratory polysomnography in many cases of uncomplicated sleep apnea. Multiple sleep latency tests must be performed meticulously and interpreted in the clinical setting to avoid overdiagnoses of narcolepsy. Human leukocyte antigen testing has limited utility in establishing a diagnosis of narcolepsy because a positive test has low specificity...
August 2017: Continuum: Lifelong Learning in Neurology
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