Pharmacological treatment for Kleine-Levin syndrome

Background

Kleine-Levin syndrome (KLS) is a rare disorder that mainly affects adolescent men. It is characterised by recurrent episodes of hypersomnia (excessive sleepiness), hyperphagia (overeating), and abnormal behaviour. The frequency and nature of the attacks can disrupt the individual's social, professional, and family life. The cause of KLS is not known. Several treatments have been used, including stimulant, anti-epileptic, anti-depressant, and anti-psychotic drugs, with some benefit reported, but because of the rarity of the condition, long-term follow-up of participants is difficult.

Objectives

The authors of this review aimed to identify and evaluate randomised controlled trials (RCTs) studying the effectiveness of pharmacological treatment for Kleine-Levin syndrome.

Results

We were not able to find any RCTs. Good-quality evidence is therefore lacking, and therapeutic trials with a double-blind, placebo-controlled design are needed.

The evidence was current to 7 April 2016.

Authors' conclusions: 

Therapeutic trials of pharmacological treatment for Kleine-Levin syndrome with a double-blind, placebo-controlled design are needed.

Read the full abstract...
Background: 

This is an updated version of the original Cochrane review, published in 2009, Issue 2.

Kleine-Levin syndrome (KLS) is a rare disorder that mainly affects adolescent men. It is characterised by recurrent episodes of hypersomnia, usually accompanied by hyperphagia, cognitive and mood disturbances, abnormal behaviour, such as hypersexuality, and signs of dysautonomia.

In 1990, the diagnostic criteria for Kleine-Levin syndrome were modified in the International Classification of Sleep Disorders, where KLS was defined as a syndrome comprised of recurring episodes of undue sleepiness lasting some days, which may or may not be associated with hyperphagia and abnormal behaviour. According to the International Classification of Sleepiness Disorders, 3rd version (ICSD-3), revised in 2014, the Kleine-Levin syndrome is a disorder characterized by recurrent episodes of hypersomnia that last from two days to four weeks, with at least annual recurrence, and hyperphagia (rapid consumption of a large amount of food), usually with onset in early adolescence in males but occasionally in later life and in women. A monosymptomatic form of the disorder with hypersomnia only can occur without binge eating or hypersexuality.

The cause of Kleine-Levin syndrome remains unknown, and several treatment strategies have been used. Some medications have been reported to provide benefit in the treatment of patients with KLS, but because of the rarity of the condition, no long-term follow-up therapies have yet been described.

Objectives: 

This review aimed to evaluate:

1. whether pharmacological treatment for Kleine Levin syndrome was effective and safe.
2. which drug or category of drugs was effective and safe.

Search strategy: 

For the latest update, we searched the following sources: the Cochrane Epilepsy Group Specialized Register (7 April 2016); the Cochrane Central Register of Controlled Trials (CENTRAL) via the Cochrane Register of Studies Online CRSO (7 April 2016); MEDLINE (1946 to April 2016); LILACS (7 April 2016); ClinicalTrials.gov (7 April 2016); WHO International Clinical Trials Registry Platform ICTRP (7 April 2016); reference lists of sleep medicine textbooks; review articles and reference lists of articles identified by the search strategies.

Selection criteria: 

All randomised controlled trials (RCTs) and quasi-randomised controlled trials looking at pharmacological interventions for Kleine-Levin syndrome were eligible. We had planned to include both parallel-group and cross-over studies.

Data collection and analysis: 

Two review authors (MMO and CC) had planned to extract the data reported in the original articles.

Main results: 

No studies met the inclusion criteria for this systematic review.

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