Department of Neurology, McGill University, Montreal General Hospital, Montreal, Canada.
Parkinsonism Relat Disord. 2014 Jan;20 Suppl 1:S1-4. doi: 10.1016/S1353-8020(13)00400-8.
REM sleep behavior disorder (RBD) is characterized by loss of REM atonia of sleep, such that patients act out the contents of their dreams. Perhaps the most important facet of idiopathic RBD is that it is a powerful prodromal marker of Parkinson's disease (PD) and other synucleinopathies. Several prospective studies have now established that patients with idiopathic RBD have up to an 80% risk of developing a defined neurodegenerative synucleinopathy. This has profound implications for understanding and treating early disease. First, the extremely high risk and long latency/time to intervene make RBD patients the ideal candidates for neuroprotective therapy against synucleinopathy. Second, RBD patients can be used as a 'test lab' to assess other potential prodromal predictors of PD, which could be applied to the general population in future large-scale screening programs. Third, assessing epidemiology of RBD allows us to study the epidemiology of PD and dementia with Lewy bodies 10-15 years earlier, reducing bias and opening new hypotheses as to the mechanism of action of selected risk factors. Finally, by prospectively observing RBD patients as they transition to full neurodegenerative synucleinopathy, one has an unprecedented window in which to directly observe evolution of PD from its prodromal stages. The evidence for RBD as a marker of prodromal PD and all these implications will be discussed.
快速眼动睡眠行为障碍（RBD）的特征是 REM 睡眠时的去弛缓丧失，以至于患者会将梦境内容付诸行动。或许特发性 RBD 最重要的一个方面是，它是帕金森病（PD）和其他突触核蛋白病的一个强有力的前驱标志物。目前已有几项前瞻性研究确立，特发性 RBD 患者有高达 80%的风险发展为明确的神经退行性突触核蛋白病。这对理解和治疗早期疾病具有深远的影响。首先，极高的风险和长潜伏期/干预时间使 RBD 患者成为针对突触核蛋白病的神经保护治疗的理想候选者。其次，RBD 患者可作为“测试实验室”，评估 PD 的其他潜在前驱预测因子，这些预测因子可在未来的大规模筛查计划中应用于一般人群。第三，评估 RBD 的流行病学使我们能够研究 PD 和路易体痴呆的流行病学早 10-15 年，减少偏倚并提出关于选定危险因素作用机制的新假说。最后，通过前瞻性观察 RBD 患者向完全神经退行性突触核蛋白病的转变，人们有了一个前所未有的窗口，可以直接观察 PD 从前驱阶段的演变。将讨论 RBD 作为 PD 前驱标志物的证据以及所有这些影响。
