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克莱恩-莱文综合征——锂盐治疗的效果——

The Kleine-Levin syndrome - effects of treatment with lithium -.

作者信息

Poppe M, Friebel D, Reuner U, Todt H, Koch R, Heubner G

机构信息

Department of Neuropediatrics, Technical University Dresden, Dresden, Germany.

出版信息

Neuropediatrics. 2003 Jun;34(3):113-9. doi: 10.1055/s-2003-41273.

Abstract

Kleine-Levin syndrome (KLS) is a rare disorder which affects mainly adolescents. Periods of extreme somnolence alternate with megaphagia, psychomental changes and behavioural symptoms. The cause and pathogenesis of KLS remains unknown. Several treatments have been tried and recently lithium has been proposed for a prophylactic use in single cases. In view of the rarity of KLS, long-term results of lithium therapy have not been described yet. We report the clinical course of five adolescents with KLS who were treated with lithium. All patients showed significant EEG and polysomnographic changes during the episodes and had normal results in the interval. All patients had relapses while being treated with lithium. But episodes of hypersomnia under lithium therapy were shorter and monosymptomatic with lack of behavioural symptoms. Statistical modelling showed that the risk for a relapsing episode under maintenance of lithium drops per months of therapy from 100 % to 93 %, and furthermore that the maintenance of lithium shortens the mean duration of episodes to 19 %. No severe side effects were observed. In conclusion, in KLS with a high frequency of episodes and severe behavioural changes lithium may become a treatment option.

摘要

克莱恩-莱文综合征(KLS)是一种主要影响青少年的罕见疾病。极度嗜睡期与贪食、精神心理变化及行为症状交替出现。KLS的病因及发病机制尚不清楚。已经尝试了多种治疗方法,最近有人提出锂盐可用于个别病例的预防性治疗。鉴于KLS的罕见性,锂盐治疗的长期结果尚未见报道。我们报告了5例接受锂盐治疗的KLS青少年的临床过程。所有患者在发作期间脑电图和多导睡眠图均有显著变化,间歇期结果正常。所有患者在接受锂盐治疗期间均有复发。但锂盐治疗期间的嗜睡发作时间较短,且为单一症状,无行为症状。统计模型显示,在锂盐维持治疗期间,复发发作的风险每月从100%降至93%,此外,锂盐维持治疗可将发作的平均持续时间缩短至19%。未观察到严重副作用。总之,对于发作频繁且有严重行为改变的KLS患者,锂盐可能成为一种治疗选择。

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