Department of Neurology, McGill University, Montreal General Hospital, Montreal, Quebec, Canada.
Neurobiol Dis. 2012 Jun;46(3):553-8. doi: 10.1016/j.nbd.2011.10.003. Epub 2011 Oct 14.
REM sleep behavior disorder is a unique parasomnia characterized by dream enactment behavior during REM sleep. Unless triggered by pharmacologic agents such as antidepressants, it is generally related to damage of pontomedullary brainstem structures. Idiopathic REM sleep behavior disorder (RBD) is a well-established risk factor for neurodegenerative disease. Prospective studies have estimated that at least 40-65% of patients with idiopathic RBD will eventually develop a defined neurodegenerative phenotype, almost always a 'synucleinopathy' (Parkinson's disease, Lewy Body dementia or multiple system atrophy). In most cases, patients appear to develop a syndrome with overlapping features of both Parkinson's disease and Lewy body dementia. The interval between RBD onset and disease onset averages 10-15 years, suggesting a promisingly large window for intervention into preclinical disease stages. The ability of RBD to predict disease has major implications for design and development of neuroprotective therapy, and testing of other predictive markers of synuclein-mediated neurodegeneration. Recent studies in idiopathic RBD patients have demonstrated that olfaction, color vision, severity of REM atonia loss, transcranial ultrasound of the substantia nigra, and dopaminergic neuroimaging can predict development of neurodegenerative disease.
快速眼动睡眠行为障碍是一种独特的睡眠障碍，其特征是在快速眼动睡眠期间出现梦境行为。除非是由抗抑郁药等药物触发，否则它通常与桥脑延髓脑干部位结构损伤有关。特发性快速眼动睡眠行为障碍（RBD）是神经退行性疾病的一个明确危险因素。前瞻性研究估计，至少有 40-65%的特发性 RBD 患者最终会发展出明确的神经退行性表型，几乎总是一种“突触核蛋白病”（帕金森病、路易体痴呆或多系统萎缩）。在大多数情况下，患者似乎会出现一种具有帕金森病和路易体痴呆重叠特征的综合征。RBD 发病与疾病发病之间的间隔平均为 10-15 年，这表明在临床前疾病阶段进行干预有很大的希望。RBD 能够预测疾病，这对神经保护治疗的设计和开发以及其他突触核蛋白介导的神经退行性变的预测标志物的检测具有重要意义。最近在特发性 RBD 患者中的研究表明，嗅觉、色觉、REM 失弛缓症的严重程度、黑质经颅超声和多巴胺能神经影像学可以预测神经退行性疾病的发展。
