Clinical Manifestations of 35 Cases of Narcolepsy

三十五例猝睡症之臨床表現

陳信如(Shihn-Rur Chen) ； 蕭光明(Guang-Ming Shiao) ； 劉勝義(Shen-Yi Liu) ； 彭瑞鵬(Reury-Perng Perng)

《胸腔醫學》 17卷3期 (2002/09) Pp. 198-209

摘要

猝睡症（narcolepsy）為一種慢性睡眠疾病，其特性為白日過度嗜睡、猝倒（cataplexy）、睡眠痲痺（sleep paralysis）及臨睡幻覺（hypnagogic hallucinations）。我們回溯性地蒐集民國81年至民國90年間台北榮民總醫院猝睡症病患之病例紀錄及睡眠檢查資料，並分析病患之特性、症狀及治療情形。我們並以電話問卷方式，調查猝睡症對學業、工作之影響以及因嗜睡引發相關車禍之機率，另外利用Epworth Sleepiness Scale （ESS）調查病患的嗜睡程度。並以統計之方法分析症狀嚴重度不同的個組之間，其睡眠檢查結果及ESS scores是否有差異。
總計35位病患包含19位男性、16位女性。症狀發作之平均年齡為21歲，但發病到確定診斷的時間則平均達12年。病患皆有白日過度嗜睡之症狀，63%之病人有猝倒，54%有睡眠痲痺、46%有臨睡幻覺。符合全部四項症狀者僅有31%。猝睡症對生活品質有重大影響。20歲前發病者，64%有明顯學業成績退步、45%成績很差。已經從事工作的患者，有38%工作表現不佳。有開車習慣之患者，有78%曾在駕駛時睡著、52%曾因嗜睡而發生過車禍。我們發現對於猝睡症之病患，多重睡眠潛伏期試驗（Multiple Sleep Latency Test）及ESS不僅為主要診斷工具，亦對病患之預後提供了重要的資訊。目前對於猝睡症之治療多為症狀控制的療法，效果不盡理想。亟待更多的研究報告，以增進對猝睡症其致病機轉及症狀之了解，以期早期診斷並避免嚴重之後果，例如車禍。

關鍵字

猝睡症 ； 猝倒 ； 多重睡眠潛伏期試驗

並列摘要

Background: Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness (EDS), cataplexy, sleep paralysis, and hypnagogic hallucinations. This study was designed to evaluate the clinical presentation of narcolepsy patients during the past 10 years at Taipei Veterans General Hospital (Taipei VGH).
Methods: We retrospectively reviewed the medical charts and polysomnographic results of 35 narcoleptics seen between 1992 and 2001 at Taipei VGH. Patient characteristics, clinical symptoms, response to treatment, and a telephone questionnaire regarding quality of life, academic achievement, job performance, and history of automobile accidents, were analyzed.
Results: There were 19 male and 16 female patients in total. The mean age at onset of symptoms was 21 years. The average delay between symptom onset and the diagnosis of narcolepsy was 12 years. While EDS (100%) was the principal symptom, 22 (63%) had cataplexy, 19 (54%) had sleep paralysis, and 16 (46%) had hypnagogic hallucinations. Only 11 (31%) experienced the full tetrad. Among the 22 patients whose symptoms developed before 20 years of age, 14 (64%) experienced a marked deterioration in academic performance and 10 (45%) had bad grades. For patients older than 20 years of age (n = 26), 10 (38%) reported a poor job performance. Among the 23 who drove a vehicle, 18 (78%) reported falling asleep during driving, and 12 (52%) had had sleep-related driving accidents. Patients with bad grades and bad job performance had significantly shorter mean sleep latency and more sleep onset rapid eye movement periods (SOREMP) on the Multiple Sleep Latency Test (MSLT) than patients with good grades and good job performance. Patients with sleep-related driving accidents had significantly higher Epworth Sleepiness Scale (ESS) scores than patients without driving accidents. All patients were treated with methylphenidate and/or antidepressants. However, 24 patients did not receive regular treatment and follow-up. Of these, 13 reported ineffective treatment and 10 disliked lifelong therapy with medication.
Conclusions: Narcolepsy has a great impact on quality of life. Its diagnosis is often not made until a decade after symptoms develop. During the initial investigation, the MSLT and ESS provide important information about the prognosis. Current drug therapies are symptomatic and only modestly effective. A greater awareness of the pathophysiology and symptoms of narcolepsy may lead to an early diagnosis and an avoidance of serious consequences, such as traffic accidents.

並列關鍵字

narcolepsy ； cataplexy ； multiple sleep latency test