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Kleine-Levin syndrome (recurrent hypersomnia)

Authors
Adi Aran, MD
Ruth S Shalev, MD
Emmanuel Mignot, MD, PhD
Isabelle Arnulf, MD, PhD
Section Editors
Thomas E Scammell, MD
Ronald D Chervin, MD, MS
Deputy Editor
April F Eichler, MD, MPH

INTRODUCTION

Kleine-Levin syndrome (KLS), also called recurrent hypersomnia, is a rare sleep disorder characterized by recurrent episodes of severe hypersomnia associated with cognitive and behavioral disturbances such as confusion, derealization, apathy, compulsive eating, and hypersexuality. Episodes last a few days to several weeks and are separated by weeks or months of normal sleep and behavior [1]. KLS is categorized as a central disorder of hypersomnolence; other disorders in this category include narcolepsy type 1 and type 2 and idiopathic hypersomnia. (See "Classification of sleep disorders".)

The disease predominantly, but not exclusively, affects adolescent males, and most cases are sporadic. The cause of the disease is unknown, and the diagnosis is based on a unique constellation of clinical symptoms. In most cases, attacks cease spontaneously several years after onset. Treatment is primarily symptomatic, but stimulants and mood stabilizers (particularly lithium) can be considered in severe cases.

The epidemiology, pathogenesis, clinical features, diagnosis, and treatment of KLS will be reviewed here. A general approach to the patient with excessive daytime sleepiness and other central disorders of hypersomnolence are discussed elsewhere. (See "Approach to the patient with excessive daytime sleepiness" and "Clinical features and diagnosis of narcolepsy in adults" and "Treatment of narcolepsy in adults" and "Idiopathic hypersomnia".)

EPIDEMIOLOGY

Kleine-Levin syndrome (KLS) is a rare disorder with an estimated prevalence of one to five cases per million population [2-7]. Population-based studies have not been performed. Patients with primary KLS can be found worldwide, with a higher prevalence among Ashkenazi Jews. (See 'Genetic factors' below.)

KLS is more common in males. In several large observational series, the proportion of males ranges from 60 to 87 percent [4-7].

                                   

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Literature review current through: Nov 2016. | This topic last updated: Mon Jun 27 00:00:00 GMT 2016.
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