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发作性睡病

[Narcolepsy].

作者信息

Billiard M

机构信息

Service de neurologie B, Hôpital Gui-de-Chauliac, Montpellier.

出版信息

Rev Prat. 1996 Dec 15;46(20):2428-34.

PMID:9035528
Abstract

Narcolepsy is a rather unknown but not exceptional condition. Its prevalence, 2 to 6/10000, is ranging among this of multiple sclerosis. Narcolepsy is remarkable for clinical, polygraphic and immunogenetic features which make it a kind of model of disorders of alertness. It was first described in 1877. It has recently benefited from consistent pathophysiological progresses, which have been facilitated by the discovery of a natural canine model. The two main symptoms are irresistible and refreshing episodes of sleep and cataplexy a loss of muscle tone emotionally triggered. Polygraphically the sleep onset REM period is the major feature. Immunogenetically the condition is remarkable for an almost 100% association with HLA DR2-DQ1. Narcolepsy is a debilitating, chronic condition. Its treatment is threefold including stimulants against excessive daytime sleepiness and irresistible episodes of sleep, modafinil a new compound with awakening properties has just been introduced, antidepressants against cataplexy and associated symptoms and hypnotics against disrupted sleep.

摘要

发作性睡病是一种鲜为人知但并不罕见的病症。其患病率为万分之二至六,与多发性硬化症的患病率相当。发作性睡病在临床、多导睡眠图和免疫遗传学特征方面较为显著,使其成为警觉性障碍的一种模型。它于1877年首次被描述。最近,由于发现了天然犬类模型,其在病理生理学方面取得了持续进展。两个主要症状是不可抗拒且能提神的睡眠发作以及猝倒——一种由情绪触发的肌肉张力丧失。在多导睡眠图上,睡眠始发快速眼动期是主要特征。在免疫遗传学方面,该病症几乎与HLA DR2 - DQ1有100%的关联。发作性睡病是一种使人衰弱的慢性病症。其治疗包括三个方面,即使用兴奋剂对抗白天过度嗜睡和不可抗拒的睡眠发作,刚刚引入了具有觉醒特性的新化合物莫达非尼,使用抗抑郁药对抗猝倒及相关症状,以及使用催眠药对抗睡眠紊乱。

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