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

发作性睡病症状的长期结局尚未得到充分研究。一些文献提到预后不良是一种不治之症。但我们观察到部分发作性睡病患者发作性睡病症状随着临床病程的发展而沿着消失。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，包括那些被诊断但放弃药物治疗的患者。以1955 ~ 1985年和1985 ~ 2000年在东京大学医院睡眠门诊就诊并确诊的发作性睡病患者为研究对象，对954例典型发作性睡病患者进行问卷调查，共收集373份问卷，其中有效问卷308份。有效率为32.3%。将患者分为门诊连续就诊组和停药组，比较两组间的差异，白天嗜睡症状完全消失者门诊组为4.0%，停药组为15.4%。白天很少入睡者占门诊组的6.5%，下降组的7.7%。症状完全消失者占10.7%，很少出现症状者占18.1%，停药组占42.1%。这些差异具有统计学显著性。临床组日常生活困难改善率（86.1%）明显高于停药组（73.0%），提示临床组发作性睡病症状完全恢复，停药组预后较好。大多数（73-86%）发作性睡病患者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.