Department of Neuropsychiatry, University of Birmingham, Edgbaston, Birmingham, UK.
Neuropsychiatr Dis Treat. 2011;7:507-18. doi: 10.2147/NDT.S23624. Epub 2011 Sep 8.
Narcolepsy is a lifelong sleep disorder characterized by a classic tetrad of excessive daytime sleepiness with irresistible sleep attacks, cataplexy (sudden bilateral loss of muscle tone), hypnagogic hallucination, and sleep paralysis. There are two distinct groups of patients, ie, those having narcolepsy with cataplexy and those having narcolepsy without cataplexy. Narcolepsy affects 0.05% of the population. It has a negative effect on the quality of life of its sufferers and can restrict them from certain careers and activities. There have been advances in the understanding of the pathogenesis of narcolepsy. It is thought that narcolepsy with cataplexy is secondary to loss of hypothalamic hypocretin neurons in those genetically predisposed to the disorder by possession of human leukocyte antigen DQB1*0602. The diagnostic criteria for narcolepsy are based on symptoms, laboratory sleep tests, and serum levels of hypocretin. There is no cure for narcolepsy, and the present mainstay of treatment is pharmacological treatment along with lifestyle changes. Some novel treatments are also being developed and tried. This article critically appraises the evidence for diagnosis and treatment of narcolepsy.
发作性睡病是一种终身性睡眠障碍,其特征为日间过度嗜睡伴有不可抗拒的睡眠发作、猝倒(突发双侧肌肉无力)、催眠幻觉和睡眠瘫痪,可分为伴有猝倒的发作性睡病和不伴有猝倒的发作性睡病两组患者。发作性睡病的发病率为 0.05%,会对患者的生活质量造成负面影响,并限制他们从事某些职业和活动。人们对发作性睡病的发病机制有了进一步的了解,认为伴有猝倒的发作性睡病继发于下丘脑食欲素神经元缺失,而这种缺失是由 HLA-DQB1*0602 等遗传易感性因素导致的。发作性睡病的诊断标准基于症状、实验室睡眠测试和下丘脑分泌素的血清水平。目前尚无治愈发作性睡病的方法,主要的治疗方法是药物治疗和生活方式的改变,一些新的治疗方法也在开发和尝试中。本文对发作性睡病的诊断和治疗证据进行了批判性评估。