Bassetti, Claudio; Adamantidis, Antoine; Burdakov, Denis; Han, Fang; Gay, Steffen; Kallweit, Ulf; Khatami, Ramin; Koning, Frits; Kornum, Brigitte R; Lammers, Gert Jan; Liblau, Roland S; Luppi, Pierre H; Mayer, Geert; Pollmächer, Thomas; Sakurai, Takeshi; Sallusto, Federica; Scammell, Thomas E; Tafti, Mehdi; Dauvilliers, Yves (2019). Narcolepsy - clinical spectrum, aetiopathophysiology, diagnosis and treatment. Nature reviews. Neurology, 15(9), pp. 519-539. Springer Nature 10.1038/s41582-019-0226-9
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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.
Item Type: |
Journal Article (Review Article) |
|---|---|
Division/Institute: |
04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology |
UniBE Contributor: |
Bassetti, Claudio L.A., Adamantidis, Antoine Roger |
Subjects: |
600 Technology > 610 Medicine & health |
ISSN: |
1759-4766 |
Publisher: |
Springer Nature |
Language: |
English |
Submitter: |
Chantal Kottler |
Date Deposited: |
15 Nov 2019 15:21 |
Last Modified: |
02 Mar 2023 23:32 |
Publisher DOI: |
10.1038/s41582-019-0226-9 |
PubMed ID: |
31324898 |
BORIS DOI: |
10.7892/boris.134838 |
URI: |
https://boris.unibe.ch/id/eprint/134838 |
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