Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Division of Epidemiology, Department of Health Sciences Research, Mayo Clinic, Rochester, Minnesota.
JAMA Neurol. 2018 Apr 1;75(4):503-509. doi: 10.1001/jamaneurol.2017.4243.
This article reviews the epidemiological evidence of features of α-synucleinopathies that precede clinical onset of disease, proposes a clinical timeline, and attempts to define the different premotor and clinical phenotypes associated with α-synucleinopathies.
The pathological hallmarks of the α-synucleinopathies (Parkinson disease, Parkinson disease dementia, dementia with Lewy bodies, and multisystem atrophy) begin years before a clinical diagnosis. Epidemiologic studies support the long gap between pathology and symptoms and suggest that certain nonmotor conditions (constipation, anxiety, and rapid eye movement sleep behavior disorder) precede the traditional motor Parkinson disease phenotype by long intervals.
Characterizing the temporal onset of these conditions will help to better recognize the premotor phase of the α-synucleinopathies and specific clinical phenotypes and will guide the search for predictive biomarkers and risk or protective factors for Parkinson disease and other synucleinopathies.
本文综述了 α-突触核蛋白病在临床发病前的特征性流行病学证据，提出了临床时间表，并尝试定义与 α-突触核蛋白病相关的不同前驱期和临床表型。
α-突触核蛋白病（帕金森病、帕金森病痴呆、路易体痴呆和多系统萎缩）的病理标志在临床诊断前多年就已经存在。流行病学研究支持病理学和症状之间的长时间差距，并表明某些非运动症状（便秘、焦虑和快速眼动睡眠行为障碍）在传统的运动型帕金森病表型之前就已经存在很长时间了。
描述这些情况的时间发生将有助于更好地识别 α-突触核蛋白病的前驱期和特定的临床表型，并将指导对帕金森病和其他突触核蛋白病的预测生物标志物以及风险或保护因素的研究。
