摘要： 目的 评价神经变性疾病相关睡眠障碍的治疗方法及其疗效，为神经变性疾病相关睡眠障碍提供具有循证证据的最佳治疗方案。方法 分别以睡眠障碍（sleep disorder or sleep disturbance）、神经变性疾病（neurodegenerative diseases）、帕金森病（Parkinson's disease or PD）、阿尔茨海默病（Alzheimer's disease or AD）、多系统萎缩（multiple system atrophy or MSA）等中英文词组作为检索词，检索PubMed、美国国立医学图书馆（MEDLINE）、英国Cochrane图书馆、万方数据库和中国知识基础设施工程（CNKI）等数据库获取关于神经变性疾病相关睡眠障碍的系统评价、随机对照临床试验、临床对照试验、病例观察、流行病学调查研究等文献，采用Jadad 量表对文献质量进行评价。结果 经筛选共纳入与神经变性疾病相关睡眠障碍治疗相关的英文文献35 篇，包括系统评价2 篇、随机对照临床试验5 篇、临床对照试验13 篇和病例观察13 篇，余2 篇为流行病学调查研究，与治疗无关。35 篇文献中13 篇被评为高质量文献，余22 篇被评为低质量文献。经对各项临床试验治疗原则和不同治疗方法的治疗效果及安全性评价显示：（1）帕金森病相关睡眠障碍的治疗建议为注意睡眠卫生、合理应用拟多巴胺类药物，可改善失眠；亮光疗法可改善昼夜节律失调；氯硝西泮可改善快速眼动睡眠期行为障碍等。但迄今为止尚缺乏标准治疗方案，仍待更多的临床研究。（2）阿尔茨海默病相关睡眠障碍的治疗包括药物治疗和非药物治疗，其中药物治疗以褪黑素、乙酰胆碱酯酶抑制药、抗精神病药物、抗抑郁药物等为主；非药物治疗包括亮光疗法及行为疗法，但仍待更多的临床研究证明其疗效。（3）多系统萎缩相关睡眠障碍主要有睡眠相关呼吸障碍和快速眼动睡眠期行为障碍，睡眠相关呼吸障碍以经鼻持续气道正压通气为一线治疗方案，快速眼动睡眠期行为障碍首选氯硝西泮。（4）与睡眠障碍相关的其他神经系统变性疾病还有肌萎缩侧索硬化症、路易体痴呆等，但鲜有临床治疗的研究报道。结论 借助循证医学评价方法可为神经变性疾病相关睡眠障碍的循证治疗提供临床证据。

关键词: 神经变性疾病, 睡眠障碍, 循证医学

Abstract: Objective To evaluate the effectiveness of the treatments for sleep disorders in neurodegenerative diseases so as to provide the best therapeutic regimens for the evidence-based treatment.  Methods Search PubMed, MEDLINE, Cochrane Library, Wanfang Data and China National Knowledge Infrastructure (CNKI) databases with "sleep disorder or sleep disturbance", "neurodegenerative diseases", "Parkinson's disease or PD", "Alzheimer's disease or AD", "multiple system atrophy or MSA" as retrieval words. The quality of the articles were evaluated with Jadad Scale.  Results A total of 35 articles, including 2 systematic reviews, 5 randomized controlled trials, 13 clinical controlled trials, 13 case series and 2 epidemiological investigation studies were included for evaluation, 13 of which were high grade and 22 were low grade articles. Clinical evidences showed that: 1) advice on sleep hygiene, careful use of dopaminergic drugs and hypnotic sedative agents should be considered for PD. Bright light therapy (BLT) may improve circadian rhythm sleep disorders and clonazepam may be effective for rapid eye movement sleep behavior disorder (RBD). However, to date, very few controlled studies are available to make a recommendation for the management of sleep disorders in PD; 2) treatments for sleep disorders in AD include drug therapy (e.g. melatonin, acetylcholinesterase inhibitors, antipsychotic drugs, antidepressants) and non-drug therapy (e.g. BLT, behavior therapy), but very limited evidence shows the effectiveness of these treatments; 3) the first line treatment for sleep-related breathing disorder in MSA is nasal continuous positive airway pressure (nCPAP), and clonazepam is effective for RBD in MSA; 4) there is rare evidence related to the treatment of sleep disorders in dementia with Lewy body (DLB) and amyotrophic lateral sclerosis (ALS).  Conclusion Evidence-based medicine can provide the best clinical evidence on sleep disorders' treatment in neurodegenerative diseases.

Key words: Neurodegenerative diseases, Sleep disorders, Evidence-based medicine