猝睡症

Narcolepsy

猝睡症（narcolepsy）是大腦調控睡眠和清醒機制出了問題的一種腦部慢性疾病。1880 年首度由法國的醫師Jean-Baptiste Edouard Gelineau 定義猝睡症，他定義猝睡症為一種多重症狀的症候群，並提出猝睡症病人的猝倒發作（cataplexy）常伴隨著肌肉無力跌倒（astasia）發生。此種猝倒發作早在1916 年已被Henneberg 定義為猝倒症（cataplexy）。但因此疾病診斷不容易，且需與其他疾病做仔細的鑑別診斷，所以猝睡症常常被忽略或誤診。本文將針對猝睡症的診斷，治療與病理生理機轉做一大略探討，並介紹近年來各國的新研究與台灣相關的研究發現。

The word "narcolepsy" refers to a syndrome of unknown origin that is characterized by abnormal sleep tendencies, including excessive daytime sleepiness and frequent disturbed nocturnal sleep and pathological manifestations of rapid-eye-movement(REM) sleep. Excessive daytime sleepiness, cataplexy, and less often sleep paralysis and hypnagogic hallucinations are the major symptoms of the disease. To make the diagnosis patients have to receive a nocturnal polysomnography(PSG) and the following-day's multiple sleep latency test (MSLT). Although the etiology of human narcolepsy remains elusive, it has been suggested that the dysfunction of hypocretic-1 might play a role in the pathogenesis of this disorder. Moreover, Narcolepsy-cataplexy is associated with the presence of HLA DQB1 0602 independent of ethnicity in at least 92% of the patients. In recent years, the new finding of "T-cell receptive alpha-beta receptor subtype showed narcolepsy may be an autoimmune disease. Although, narcolepsy is a chronic sleep disorder and occurs during adolescence and young adulthood, and persists throughout the lifetime. There are some symptomatic medications were mentioned. Therefore, we will discuss the pathogenesis, clinical manifestation and treatment of narcolepsy, and up-to-date research directions will be introduced in this article.