Bassetti C, Aldrich M S
Department of Neurology, University of Michigan Medical Center, Ann Arbor, USA.
Brain. 1997 Aug;120 ( Pt 8):1423-35. doi: 10.1093/brain/120.8.1423.
The features of idiopathic hypersomnia are not well defined. We reviewed clinical and laboratory information on 42 subjects with idiopathic hypersomnia and obtained detailed follow-up evaluations on 28 of them. Only 29% of subjects had 'classic' idiopathic hypersomnia with non-imperative sleepiness, long unrefreshing naps, prolonged night-time sleep, difficult awakening with sleep drunkenness and prominent mood disturbances. Thirty-two percent had clinical features similar to narcolepsy, i.e. irresistible sleepiness, short and refreshing naps, few problems with awakening and good response to stimulants, without cataplexy or any indication of abnormal REM (rapid eye movement) sleep. The other 39% had intermediate clinical characteristics. We found no increase in the frequency of the human leucocyte antigens associated with narcolepsy. Overall, response to stimulants was good in three-quarters of the patients and spontaneous improvement of sleepiness occurred in one-quarter. Possible aetiologies identified in 10 patients included viral illness, head trauma and primary mood disorder. Idiopathic hypersomnia is a rare syndrome in which clinical heterogeneity suggests a variable or multifactoral pathogenesis. Only a minority of cases correspond to classical descriptions. Stimulants are often beneficial and spontaneous improvement appears to be more common than in narcolepsy.
特发性嗜睡症的特征尚未明确界定。我们回顾了42例特发性嗜睡症患者的临床和实验室资料，并对其中28例进行了详细的随访评估。只有29%的患者患有“典型”特发性嗜睡症，表现为非强制性嗜睡、长时间睡眠后仍感不清醒、夜间睡眠时间延长、睡眠醉酒状态下难以唤醒以及明显的情绪障碍。32%的患者具有与发作性睡病相似的临床特征，即不可抗拒的嗜睡、短暂且能提神的午睡、唤醒困难较少以及对兴奋剂反应良好，但无猝倒现象或任何异常快速眼动（REM）睡眠的迹象。另外39%的患者具有中间型临床特征。我们发现与发作性睡病相关的人类白细胞抗原频率没有增加。总体而言，四分之三的患者对兴奋剂反应良好，四分之一的患者嗜睡症状出现自发改善。在10例患者中确定的可能病因包括病毒性疾病、头部外伤和原发性情绪障碍。特发性嗜睡症是一种罕见的综合征，其临床异质性提示发病机制可变或为多因素。只有少数病例符合经典描述。兴奋剂通常有益，且自发改善似乎比发作性睡病更为常见。
