Department of Neurology, McGill University, Montreal General Hospital, Montreal, Quebec, Canada.
Parkinsonism Relat Disord. 2009 Dec;15 Suppl 3:S105-9. doi: 10.1016/S1353-8020(09)70793-X.
Parkinson's disease (PD) is a progressive disorder with a presymptomatic interval; that is, there is a period during which the pathologic process has begun, but motor signs required for the clinical diagnosis are absent. There is considerable interest in discovering markers to diagnose this preclinical stage. Current predictive marker development stems mainly from two principles; first, that pathologic processes occur in lower brainstem regions before substantia nigra involvement and second, that redundancy and compensatory responses cause symptoms to emerge only after advanced degeneration. Decreased olfaction has recently been demonstrated to predict PD in prospective pathologic studies, although the lead time may be relatively short and the positive predictive value and specificity are low. Screening patients for depression and personality changes, autonomic symptoms, subtle motor dysfunction on quantitative testing, sleepiness and insomnia are other potential simple markers. More invasive measures such as detailed autonomic testing, cardiac MIBG-scintigraphy, transcranial ultrasound, and dopaminergic functional imaging may be especially useful in those at high risk or for further defining risk in those identified through primary screening. Despite intriguing leads, direct testing of preclinical markers has been limited, mainly because there is no reliable way to identify preclinical disease. Idiopathic RBD is characterized by loss of normal atonia with REM sleep. Approximately 50% of affected individuals will develop PD or dementia within 10 years. This provides an unprecedented opportunity to test potential predictive markers before clinical disease onset. The results of marker testing in idiopathic RBD with its implications for disease prediction will be detailed.
帕金森病(PD)是一种进行性疾病,有一个前驱期;也就是说,在病理过程已经开始,但还没有出现临床诊断所需的运动迹象的时期。人们对发现能够诊断这个临床前阶段的标志物非常感兴趣。目前预测标志物的开发主要基于两个原则;首先,病理过程发生在黑质受累之前的中脑下部区域,其次,冗余和代偿反应导致症状只有在高级别退化后才会出现。嗅觉减退最近已被证明可以在前瞻性病理研究中预测 PD,尽管潜伏期可能相对较短,阳性预测值和特异性较低。对抑郁和人格改变、自主症状、定量测试中的细微运动功能障碍、嗜睡和失眠等进行筛查,也是其他一些潜在的简单标志物。更具侵入性的措施,如详细的自主神经测试、心脏 MIBG 闪烁扫描、经颅超声和多巴胺能功能成像,在高风险人群中或在通过初步筛查确定的风险人群中进一步确定风险时可能特别有用。尽管有诱人的线索,但对临床前标志物的直接测试受到限制,主要是因为目前还没有可靠的方法来识别临床前疾病。特发性 REM 睡眠行为障碍(RBD)的特征是 REM 睡眠期正常弛缓消失。大约 50%的受影响者会在 10 年内患上 PD 或痴呆症。这为在临床疾病发作前测试潜在的预测标志物提供了前所未有的机会。本文将详细介绍特发性 RBD 中标志物检测的结果及其对疾病预测的影响。