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https://www.readbyqxmd.com/read/29885874/two-cases-of-childhood-narcolepsy-mimicking-epileptic-seizures-in-video-eeg-emg
#1
Tomoe Yanagishita, Susumu Ito, Yui Ohtani, Kaoru Eto, Takashi Kanbayashi, Hirokazu Oguni, Satoru Nagata
Narcolepsy is characterized by excessive sleepiness, hypnagogic hallucinations, and sleep paralysis, and can occur with or without cataplexy. Here, we report two children with narcolepsy presenting with cataplexy mimicking epileptic seizures as determined by long-term video-electroencephalography (EEG) and electromyography (EMG) monitoring. Case 1 was a 15-year-old girl presenting with recurrent episodes of "convulsions" and loss of consciousness, who was referred to our hospital with a diagnosis of epilepsy showing "convulsions" and "complex partial seizures"...
June 6, 2018: Brain & Development
https://www.readbyqxmd.com/read/29784823/continuous-intrathecal-orexin-delivery-inhibits-cataplexy-in-a-murine-model-of-narcolepsy
#2
Mahesh K Kaushik, Kosuke Aritake, Aya Imanishi, Takashi Kanbayashi, Tadashi Ichikawa, Tetsuo Shimizu, Yoshihiro Urade, Masashi Yanagisawa
Narcolepsy-cataplexy is a chronic neurological disorder caused by loss of orexin (hypocretin)-producing neurons, associated with excessive daytime sleepiness, sleep attacks, cataplexy, sleep paralysis, hypnagogic hallucinations, and fragmentation of nighttime sleep. Currently, human narcolepsy is treated by providing symptomatic therapies, which can be associated with an array of side effects. Although peripherally administered orexin does not efficiently penetrate the blood-brain barrier, centrally delivered orexin can effectively alleviate narcoleptic symptoms in animal models...
June 5, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29616151/narcolepsy-in-adolescence-a-missed-diagnosis-a-case-report
#3
Anoop K Gupta, Swapnajeet Sahoo, Sandeep Grover
<b/> Narcolepsy is an uncommon sleep cycle disorder with a usual onset in adolescence, but it is often misdiagnosed and underdiagnosed. Rarely is the tetrad of excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep paralysis seen in patients. The clinical characteristics of narcolepsy are often confused with many psychiatric and neurologic disorders. Lack of clinical awareness about narcolepsy leads to frequent prescriptions of antiepileptics and psychotropics, which can adversely affect the quality of life of children and adolescents...
July 2017: Innovations in Clinical Neuroscience
https://www.readbyqxmd.com/read/29522212/the-inappropriate-occurrence-of-rapid-eye-movement-sleep-in-narcolepsy-is-not-due-to-a-defect-in-homeostatic-regulation-of-rapid-eye-movement-sleep
#4
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 later three symptoms together with a short rapid eye movement (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...
June 1, 2018: Sleep
https://www.readbyqxmd.com/read/29512449/sodium-oxybate-treatment-in-pediatric-type-1-narcolepsy
#5
Monica Moresco, Fabio Pizza, Elena Antelmi, Giuseppe Plazzi
Narcolepsy type 1 (NT1) is a rare chronic neurologic disorder characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hallucinations and disrupted nocturnal sleep, usually with onset during childhood/adolescence. Pediatric NT1 is associated with limitations on children's activities and achievements, especially poor performance at school, difficulty with peers due to disease symptoms and comorbidities including depression, obesity, and precocious puberty. NT1 disease is caused by the selective loss of hypocretin-producing neurons in the lateral hypothalamus, most probably related to an autoimmune pathophysiology...
March 5, 2018: Current Drug Metabolism
https://www.readbyqxmd.com/read/29195824/pseudotumor-cerebri-syndrome-in-a-patient-with-narcolepsy-type-1
#6
Thomas Rossor, Ming Lim, Kirandeep VanDenEshof, Paul Gringras
Type 1 narcolepsy (NT1) is a chronic primary disorder of hypersomnolence characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations and disrupted nocturnal sleep. NT1 is linked to hypothalamic hypocretin deficiency, strongly associated with Human Leukocyte Antigen (HLA) marker DQB1*06:02 and of probable autoimmune origin. NT1 is usually associated with increased rates of overweight and obesity, and sometimes with increases in overnight blood pressure and increased rates of hypoventilation with raised CO2 levels overnight...
January 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29149785/association-study-of-hla-dqb1-0602-allele-in-iranian-patients-with-narcolepsy
#7
Demeke Geremew, Ania Rahimi-Golkhandan, Khosro Sadeghniiat-Haghighi, Yadollah Shakiba, Ahmad Khajeh-Mehrizi, Bita Ansaripour, Maryam Izad
Narcolepsy is a rare, disabling disorder characterized by excessive daytime sleepiness, cataplexy, hypnagogic hallucinations and sleep paralysis. Several studies demonstrated its association with HLA-DQB1*0602 in various ethnic groups. Our study aimed to determine the prevalence of HLA-DQB1*0602 allele in Iranian patients with narcolepsy and assess its predictive parameters for diagnosing narcolepsy. In addition, car accidents and job problems were assessed among narcoleptic patients. We studied 44 narcoleptic patients, 30 patients with other types of excessive daytime sleepiness (EDS)  and 50 healthy age and sex matched individuals in this case-control study...
October 2017: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/29145290/effective-treatment-of-narcolepsy-like-symptoms-with-high-frequency-repetitive-transcranial-magnetic-stimulation-a-case-report
#8
Jian-Bo Lai, Mao-Mao Han, Yi Xu, Shao-Hua Hu
RATIONALE: Narcolepsy is a rare sleep disorder with disrupted sleep-architecture. Clinical management of narcolepsy lies dominantly on symptom-driven pharmacotherapy. The treatment role of repetitive transcranial magnetic stimulation (rTMS) for narcolepsy remains unexplored. PATIENT CONCERNS: In this paper, we present a case of a 14-year-old young girl with excessive daytime sleepiness (EDS), cataplexy and hypnagogic hallucinations. DIAGNOSES: After excluding other possible medical conditions, this patient was primarily diagnosed with narcolepsy...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28956242/polysomnographic-findings-in-craniopharyngioma-patients
#9
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
https://www.readbyqxmd.com/read/28778234/neuroimaging-of-narcolepsy-and-kleine-levin-syndrome
#10
REVIEW
Seung Bong Hong
Narcolepsy is a chronic neurologic disorder with the abnormal regulation of the sleep-wake cycle, resulting in excessive daytime sleepiness, disturbed nocturnal sleep, and manifestations related to rapid eye movement sleep, such as cataplexy, sleep paralysis, and hypnagogic hallucination. Over the past decade, numerous neuroimaging studies have been performed to characterize the pathophysiology and various clinical features of narcolepsy. This article reviews structural and functional brain imaging findings in narcolepsy and Kleine-Levin syndrome...
September 2017: Sleep Medicine Clinics
https://www.readbyqxmd.com/read/28726523/investigational-therapies-for-the-treatment-of-narcolepsy
#11
REVIEW
Stefano de Biase, Annacarmen Nilo, Gian Luigi Gigli, Mariarosaria Valente
Narcolepsy is a chronic sleep disorder characterized by a pentad of excessive daytime sleepiness (EDS), cataplexy, sleep paralysis, hypnagogic/hypnopompic hallucinations, and disturbed nocturnal sleep. While non-pharmacological treatments are sometimes helpful, more than 90% of narcoleptic patients require a pharmacological treatment. Areas covered: The present review is based on an extensive Internet and PubMed search from 1994 to 2017. It is focused on drugs currently in development for the treatment of narcolepsy...
August 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28539902/arousal-in-nocturnal-consciousness-how-dream-and-sleep-experiences-may-inform-us-of-poor-sleep-quality-stress-and-psychopathology
#12
REVIEW
Nirit Soffer-Dudek
The term "sleep experiences," coined by Watson (2001), denotes an array of unusual nocturnal consciousness phenomena; for example, nightmares, vivid or recurrent dreams, hypnagogic hallucinations, dreams of falling or flying, confusional arousals, and lucid dreams. Excluding the latter, these experiences reflect a single factor of atypical oneiric cognitions ("general sleep experiences"). The current study is an opinionated mini-review on the associations of this factor-measured with the Iowa sleep experiences survey (ISES, Watson, 2001)-with psychopathological symptoms and stress...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28472332/the-spectrum-of-rem-sleep-related-episodes-in-children-with-type-1-narcolepsy
#13
Elena Antelmi, Fabio Pizza, Stefano Vandi, Giulia Neccia, Raffaele Ferri, Oliviero Bruni, Marco Filardi, Gaetano Cantalupo, Rocco Liguori, Giuseppe Plazzi
Type 1 narcolepsy is a central hypersomnia due to the loss of hypocretin-producing neurons and characterized by cataplexy, excessive daytime sleepiness, sleep paralysis, hypnagogic hallucinations and disturbed nocturnal sleep. In children, close to the disease onset, type 1 narcolepsy has peculiar clinical features with severe cataplexy and a complex admixture of movement disorders occurring while awake. Motor dyscontrol during sleep has never been systematically investigated. Suspecting that abnormal motor control might affect also sleep, we systematically analysed motor events recorded by means of video polysomnography in 40 children with type 1 narcolepsy (20 females; mean age 11...
June 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28424564/new-developments-in-the-management-of-narcolepsy
#14
REVIEW
Vivien C Abad, Christian Guilleminault
Narcolepsy is a life-long, underrecognized sleep disorder that affects 0.02%-0.18% of the US and Western European populations. Genetic predisposition is suspected because of narcolepsy's strong association with HLA DQB1*06-02, and genome-wide association studies have identified polymorphisms in T-cell receptor loci. Narcolepsy pathophysiology is linked to loss of signaling by hypocretin-producing neurons; an autoimmune etiology possibly triggered by some environmental agent may precipitate hypocretin neuronal loss...
2017: Nature and Science of Sleep
https://www.readbyqxmd.com/read/28258080/measurement-of-narcolepsy-symptoms-the-narcolepsy-severity-scale
#15
Yves Dauvilliers, Severine Beziat, Carole Pesenti, Regis Lopez, Lucie Barateau, Bertrand Carlander, Gianina Luca, Mehdi Tafti, Charles M Morin, Michel Billiard, Isabelle Jaussent
OBJECTIVE: To validate the Narcolepsy Severity Scale (NSS), a brief clinical instrument to evaluate the severity and consequences of symptoms in patients with narcolepsy type 1 (NT1). METHODS: A 15-item scale to assess the frequency and severity of excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, sleep paralysis, and disrupted nighttime sleep was developed and validated by sleep experts with patients' feedback. Seventy untreated and 146 treated adult patients with NT1 were evaluated and completed the NSS in a single reference sleep center...
April 4, 2017: Neurology
https://www.readbyqxmd.com/read/28162143/patient-reported-measures-of-narcolepsy-the-need-for-better-assessment
#16
REVIEW
Ulf Kallweit, Markus Schmidt, Claudio L Bassetti
STUDY OBJECTIVES: Narcolepsy, a chronic disorder of the central nervous system, is clinically characterized by a symptom pentad that includes excessive daytime sleepiness, cataplexy, sleep paralysis, hypnopompic/hypnagogic hallucinations, and disrupted nighttime sleep. Ideally, screening and diagnosis instruments that assist physicians in evaluating a patient for type 1 or type 2 narcolepsy would be brief, easy for patients to understand and physicians to score, and would identify or rule out the need for electrophysiological testing...
May 15, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/28123336/narcolepsy-and-cataplexy-a-pediatric-case-report
#17
Tülin Savaş, Ilknur Erol, Semra Saygı, Mehmet Ali Habeşoğlu
Narcolepsy is characterized by excessive sleepiness, cataplexy, hypnagogic hallucinations, and sleep paralysis during the rapid eye movement period of sleep. Herein, we present a boy aged eight years who was diagnosed as having narcolepsy and cataplexy about thirteen months after his first presentation. He was admitted with symptoms of daytime sleepiness. In the follow-up, cataplexy in the form of head dropping attacks developed seven months after the first admission. The patient was investigated for different prediagnoses and was eventually diagnosed as having narcolepsy and cataplexy through polysomnography and multiple sleep latency tests thirteen months after the first presentation...
December 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28118866/quantifying-leg-movement-activity-during-sleep
#18
REVIEW
Raffaele Ferri, Stephany Fulda
Currently, 2 sets of similar rules for recording and scoring leg movement (LM) exist, including periodic LM during sleep (PLMS) and periodic LM during wakefulness. The former were published in 2006 by a task force of the International Restless Legs Syndrome Study Group, and the second in 2007 by the American Academy of Sleep Medicine. This article reviews the basic recording methods, scoring rules, and computer-based programs for PLMS. Less frequent LM activities, such as alternating leg muscle activation, hypnagogic foot tremor, high-frequency LMs, and excessive fragmentary myoclonus are briefly described...
December 2016: Sleep Medicine Clinics
https://www.readbyqxmd.com/read/27865546/french-consensus-management-of-patients-with-hypersomnia-which-strategy
#19
REVIEW
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...
January 2017: Revue Neurologique
https://www.readbyqxmd.com/read/27838095/french-consensus-type-1%C3%A2-and-type-2%C3%A2-narcolepsy-investigations-and-follow-up
#20
REVIEW
C Monaca, P Franco, P Philip, Y Dauvilliers
In the new international classification of sleep disorders (ICSD-3), narcolepsy is differentiated into two distinct pathologies: type 1 narcolepsy (NT1) and type 2 narcolepsy (NT2). NT1 is characterised by periods of an irrepressible need to sleep, cataplexy (a sudden loss of muscle tone triggered by emotion) and in some cases the presence of symptoms such as hypnagogic hallucinations, sleep paralysis and disturbed night-time sleep. Its physiopathology is based on the loss of hypocretin neurons in the hypothalamus, seemingly connected to an auto-immune process...
January 2017: Revue Neurologique
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