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Open Access Highly Accessed Case report

Monozygotic twins concordant for Kleine-Levin syndrome

Taro Ueno12, Akira Fukuhara2, Azusa Ikegami2, Fumihiro Ohishi2 and Kazuhiko Kume12*

Author Affiliations

1 Institute of Molecular Embryology and Genetics, Kumamoto University, Honjo, Kumamoto, 860-0811, Japan

2 Sleep Center, Kuwamizu Hospital, Kuwamizu, Kumamoto, 862-0954, Japan

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BMC Neurology 2012, 12:31  doi:10.1186/1471-2377-12-31

Published: 30 May 2012

Abstract

Background

Kleine-Levin syndrome is a rare sleep disorder of unknown etiology. It is characterized by intermittent periods of excessive sleepiness, cognitive disturbances and behavioral abnormalities. Nine cases of familial Kleine-Levin syndrome have been identified, but there are no reported cases describing twins that are affected by the syndrome.

Case presentation

We report the cases of 16-year-old monozygotic twin boys who both suffered from Kleine-Levin syndrome. In both cases, the onset of the first episode was preceded by an influenza infection. During symptomatic periods they slept for the entire day except for meals and bathroom visits. Actimetry recordings revealed that during symptomatic periods, daily activity was lower than that of asymptomatic periods, on the other hand, activity during the night was significantly higher in symptomatic periods than asymptomatic periods. Polysomnography (PSG) data during symptomatic periods revealed a decrease in sleep efficiency. Human leukocyte antigen (HLA) typing revealed no DQB1*02 loci. They were administered lithium carbonate but the beneficial effect was limited.

Conclusions

Our observations suggest that Kleine-Levin syndrome may be due to genetic and autoimmune processes, although etiologic relationship to specific HLA type remains controversial.

Keywords:
Recurrent hypersomnia; Kleine-Levin syndrome; Monozygotic twins; HLA typing; Polysomnography; Actimetry