Oxford Parkinson's Disease Centre, University of Oxford, UK; Nuffield Department of Clinical Neurosciences, University of Oxford, UK.
Neurobiol Dis. 2020 Sep;143:104996. doi: 10.1016/j.nbd.2020.104996. Epub 2020 Jun 26.
Since its first description in 1986 by Dr. Carlos Schenck, and his group's subsequent report of the delayed emergence of a Parkinsonian disorder in idiopathic RBD patients one decade later, RBD has emerged in recent years as one of the most promising markers of prodromal Parkinson's (References 2, 3). RBD is present in 25-58% of patients with Parkinson's disease and up to 90% of those with Dementia with Lewy Bodies (DLB) or Multiple System Atrophy (MSA). In a substantial proportion of these patients RBD onset occurs before motor symptoms. Critically, when seen in isolation, RBD is a highly specific marker of future synucleinopathy: long-term cohort studies indicate that more than 80% of people who develop isolated RBD will go on to develop an alpha-synuclein related neurodegenerative disorder. Recently, the largest ever study of 1280 polysomnographically-diagnosed RBD subjects from 24 International RBD Study Group sleep centres by a single author group, found an overall conversion rate from iRBD to an overt neurodegenerative syndrome of 6.3% per year. RBD is therefore common, representative of a large proportion of sporadic disease, and provides a unique window for the study of prodromal neurodegeneration, whether it be Parkinson's or Dementia.
自 1986 年 Carlos Schenck 博士首次描述该病以来，他的团队在 10 年后报告了特发性 RBD 患者出现迟发性帕金森病。近年来，RBD 已成为前驱期帕金森病最有希望的标志物之一（参考文献 2、3）。帕金森病患者中有 25-58%存在 RBD，而在路易体痴呆（DLB）或多系统萎缩（MSA）患者中高达 90%。在这些患者中，相当一部分患者的 RBD 发病发生在运动症状之前。至关重要的是，当孤立存在时，RBD 是未来突触核蛋白病的高度特异性标志物：长期队列研究表明，超过 80%出现孤立 RBD 的人将发展为与α-突触核蛋白相关的神经退行性疾病。最近，一个由单个作者团队对来自 24 个国际 RBD 研究组睡眠中心的 1280 名经多导睡眠图诊断的 RBD 受试者进行了迄今为止最大规模的研究，发现 iRBD 向显性神经退行性综合征的总体转化率为每年 6.3%。因此，RBD 很常见，代表了很大一部分散发性疾病，为前驱性神经退行性变的研究提供了独特的窗口，无论是帕金森病还是痴呆症。
