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克莱恩-莱文综合征;最新进展与简要综述。

Kleine-Levin syndrome; An update and mini-review.

作者信息

Gadoth Natan, Oksenberg Arie

机构信息

Department of Neurology, Maynei Hayeshua Medical Center, Bnei Brak, Israel; The Sackler Faculty of Medicine, Tel-Aviv University, Israel; The Sleep Disorders Unit, Loewenstein Hospital - Rehabilitation Center, Raanana, Israel.

The Sleep Disorders Unit, Loewenstein Hospital - Rehabilitation Center, Raanana, Israel.

出版信息

Brain Dev. 2017 Sep;39(8):665-671. doi: 10.1016/j.braindev.2017.04.003. Epub 2017 Apr 20.

Abstract

Since 1962, when Critchley and Hoffman coined the term Kleine-Levin Syndrome (KLS) for the triad of hypersomnia, excessive eating and "often abnormal behavior" which they have observed in 11 adolescent boys, the number of patients recognized with this rare syndrome expanded, the spectrum of the clinical presentation, disease course, prognosis, gender specificity and the presence of familial cases were established. However, in spite of the progress made in neuroscience, the search for the cause, neuroanatomy, pathophysiology and drug treatment of KLS is still ongoing. In this mini-review we will describe in some detail the scientific efforts made to understand in depth the complex symptomatology of KLS and refer also to updated findings reached up till now.

摘要

自1962年克里奇利和霍夫曼为他们在11名青春期男孩身上观察到的嗜睡、暴饮暴食和“常伴有异常行为”三联征创造了克莱恩-莱文综合征(KLS)一词以来,被确诊患有这种罕见综合征的患者数量有所增加,其临床表现范围、病程、预后、性别特异性以及家族性病例的情况也得以明确。然而,尽管神经科学取得了进展,但对KLS病因、神经解剖学、病理生理学和药物治疗的探索仍在继续。在这篇小型综述中,我们将详细描述为深入理解KLS复杂症状所做的科学努力,并提及截至目前所取得的最新研究成果。

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