Idiopathic Hypersomnia.
Idiopathic Hypersomnia.
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DOI：
10.1053/smrv.2001.0168
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发表时间：
2001-10-01
影响因子：
10.5
通讯作者：
Dauvilliers, Y
中科院分区：
文献类型：
--
作者：
Billiard, M;Dauvilliers, Y
与发作性睡病和克莱恩-莱文综合征不同，特发性嗜睡症是一种最近被描述的睡眠障碍。与前两种情况相比，缺乏相关的临床特征，如猝倒或巨咽症和特征性的多导睡眠图特征，使特发性嗜睡症的阳性诊断更加不确定。因此，有一种不幸的倾向，把所有难以分类的日间过度嗜睡的病例都贴上特发性嗜睡症的标签。目前，由于对上呼吸道抵抗综合征、无猝倒发作性睡症、睡眠阶段延迟综合征等新疾病的描述，以及对特发性嗜睡症“多症状”或“经典”形式的明确识别，疾病的界限变得更加精确。仍然有一些孤立的白天过度嗜睡，没有长时间的夜间睡眠，没有醒来困难的病例，这介于发作性睡病和真正的特发性嗜睡症之间。因此，明确需要进一步开展实验室调查，以帮助确定和分类这些病例。此外，病理生理学和发病机制仍处于起步阶段，必须在这一方向上努力。除了使用一种新的促进尾流的化合物莫达非尼（modafinil）外，治疗没有取得一致的进展，莫达非尼尚未在对照研究中进行评估。
In contrast to narcolepsy and the Kleine-Levin syndrome, idiopathic hypersomnia is a recently described sleep disorder. Absence of associated clinical features such as cataplexy or megaphagia and characteristic polysomnographic features such as sleep-onset REM episodes render positive diagnosis more uncertain in idiopathic hypersomnia than in the fwo former conditions. Consequently there has been an unfortunate tendency to label all difficult to classify cases of excessive daytime sleepiness as idiopathic hypersomnia. At present due to the description of new disorders such as upper airway resistance syndrome, narcolepsy without cataplexy, delayed sleep phase syndrome, all of which were formerly confused with idiopathic hypersomnia and the clear identification of a "polysymptomatic" or "classic" form of idiopathic hypersomnia, the limits of the disorder become more precise. Still there are a number of cases of isolated excessive daytime sleepiness with no prolonged night sleep, no difficulty waking up, which lay between narcolepsy and genuine idiopathic hypersomnia. Thus there is a definite need to further develop laboratory investigations to help identify and classify these cases. Moreover pathophysiology and pathogenesis are still in their infancy and efforts have to be pursued in this direction. Treatment has not made consistent progress except for the use of a new wake promoting compound, modafinil, which has not yet been evaluated in controlled studies.