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Review
. 2018 Sep;22(3):613-623.
doi: 10.1007/s11325-017-1617-z. Epub 2018 Mar 12.

Kleine-Levin syndrome: clues to aetiology

Saad Mohammed AlShareef  1 , Richard Mark Smith  2 , Ahmed Salem BaHammam  3
Affiliations
Review

Kleine-Levin syndrome: clues to aetiology

Saad Mohammed AlShareef et al. Sleep Breath. 2018 Sep.

Abstract

Kleine-Levin syndrome (KLS) is the commonest recurrent sleep disorder, with a prevalence of 1-2 per million population. Clear diagnostic criteria are now defined, but effective treatment remains elusive. The significant body of published literature allows consideration of possible aetiological mechanisms, an understanding of which could guide the development of therapeutic strategies. Functional imaging studies have been inconclusive; although diencephalic abnormalities are a common finding, no consistent pattern has emerged, and these studies have not revealed the mechanism(s) underlying the development of the abnormalities detected. An autoimmune aetiology is consistent with the available data. In this review, we argue that, in order to further our understanding of KLS, there needs to be a co-ordinated international effort to standardise approaches to functional imaging studies, genetic analyses that specifically address the possibility of an autoimmune aetiology, and clinical trials of immunosuppressive therapies.

Keywords: Aetiology; Autoimmunity; Functional neuroimaging; Kleine-Levin syndrome; Recurrent hypersomnia.

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Conflict of interest statement

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animal performed by any of the authors.

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