Narcolepsy - clinical spectrum, aetiopathophysiology, diagnosis and treatment.

Details

Serval ID
serval:BIB_2FF2BBD31801
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Narcolepsy - clinical spectrum, aetiopathophysiology, diagnosis and treatment.
Journal
Nature reviews. Neurology
Author(s)
Bassetti CLA, Adamantidis A., Burdakov D., Han F., Gay S., Kallweit U., Khatami R., Koning F., Kornum B.R., Lammers G.J., Liblau R.S., Luppi P.H., Mayer G., Pollmächer T., Sakurai T., Sallusto F., Scammell T.E., Tafti M., Dauvilliers Y.
ISSN
1759-4766 (Electronic)
ISSN-L
1759-4758
Publication state
Published
Issued date
09/2019
Peer-reviewed
Oui
Volume
15
Number
9
Pages
519-539
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
Abstract
Narcolepsy is a rare brain disorder that reflects a selective loss or dysfunction of orexin (also known as hypocretin) neurons of the lateral hypothalamus. Narcolepsy type 1 (NT1) is characterized by excessive daytime sleepiness and cataplexy, accompanied by sleep-wake symptoms, such as hallucinations, sleep paralysis and disturbed sleep. Diagnosis is based on these clinical features and supported by biomarkers: evidence of rapid eye movement sleep periods soon after sleep onset; cerebrospinal fluid orexin deficiency; and positivity for HLA-DQB1*06:02. Symptomatic treatment with stimulant and anticataplectic drugs is usually efficacious. This Review focuses on our current understanding of how genetic, environmental and immune-related factors contribute to a prominent (but not isolated) orexin signalling deficiency in patients with NT1. Data supporting the view of NT1 as a hypothalamic disorder affecting not only sleep-wake but also motor, psychiatric, emotional, cognitive, metabolic and autonomic functions are presented, along with uncertainties concerning the 'narcoleptic borderland', including narcolepsy type 2 (NT2). The limitations of current diagnostic criteria for narcolepsy are discussed, and a possible new classification system incorporating the borderland conditions is presented. Finally, advances and obstacles in the symptomatic and causal treatment of narcolepsy are reviewed.
Pubmed
Web of science
Create date
04/08/2019 14:54
Last modification date
23/10/2019 5:13
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