Narcolepsy - clinical spectrum, aetiopathophysiology, diagnosis and treatment.

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

[img] Text
Bassetti, 2019, Narcolepsy.pdf - Published Version
Restricted to registered users only
Available under License Publisher holds Copyright.

Download (2MB) | Request a copy

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 and 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:

15 Nov 2019 15:21

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

Actions (login required)

Edit item Edit item
Provide Feedback