A 30-50 YEAR FOLLOW-UP STUDY OF NARCOLEPSY AND OTHER HYPERSOMNIAS

发作性睡病症状的长期结果尚未得到充分调查。一些文献认为预后不良是一种不治之症。但我们观察到部分发作性睡症患者随着病程的发展，发作性睡症症状逐渐消失。为了更好地估计发作性睡病的预后，我们对发作性睡病患者进行了长期（10-50年）随访研究，包括那些确诊但放弃药物治疗的患者。研究对象为1955 - 1985年在东京大学医院或1985 - 2000年在Seiwa医院睡眠门诊就诊并确诊的发作性睡病患者。共识别954例典型发作性睡病患者，收集问卷373份，有效问卷308份。应答率为32.3%。我们将他们分为两组，一组继续去门诊，另一组停止治疗，并检查两组之间的差异。白天过度嗜睡症状完全消失者，临床组为4.0%，滴药组为15.4%；白天很少入睡者，临床组为6.5%，滴药组为7.7%。猝倒变化方面，临床组症状完全消失者为10.7%，滴药组为13.2%，极少发生猝倒者为18.1%，滴药组为42.1%。这些差异具有统计学意义。另一方面，临床组（86.1%）比下降组（73.0%）更频繁地观察到日常生活困难的改善。上述结果提示发作性睡症状确实完全恢复，且滴药组预后较好。大多数发作性睡病患者（73-86%）的生活质量也有明显改善。

The long-term outcome of narcolepsy symptoms is not fully investigated. Some literature mentioned poor prognosis as an incurable disease. But we observed some narcolepsy patients showed disappearance of narcoleptic symptoms along with their clinical courses. In order to better estimate the prognosis of narcolepsy, we performed the long-term (10-50 year) follow-up study of narcolepsy patients including those who were diagnosed but dropped from medical treatment. Narcolepsy patients who visited and diagnosed in sleep clinics in Tokyo University Hospital from 1955 to 1985 or in Seiwa Hospital from 1985 to 2000, were included in this study.A total of 954 typical narcolepsy were identified and 373 answers were collected, among them 308 returned with valid answers. The response rate was 32.3%. We divided them into 2 groups, those who continuously visited outpatient clinic and those who dropped from the medical treatment, and checked the differences between groups.As for the excessive daytime sleepiness, those who showed the complete disappearance of symptoms were 4.0% in the clinic group and 15.4% in dropped group, and those who rarely fell asleep during daytime were 6.5% in clinic group and 7.7% in dropped group. As for the change in cataplexy, those who showed complete disappearance of symptoms were 10.7% in clinic group and 13.2% in dropped group, and those who rarely experienced cataplexy were 18.1% in clinic group and 42.1% in dropped group. These differences were statistically significant. On the other hand, the improvement of difficulty in daily life was more frequently observed in clinic group (86.1%) compared to dropped group (73.0%).These results indicated that complete recovery from narcolepsy symptoms did occur and the prognosis in the dropped group was better. Also the QOL were improved substantially in the majority (73-86%) of narcolepsy patients.