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多系统萎缩的病程演变及其预后影响因素：基于前驱期至晚期的全程解读
Evolution of disease course and prognostic determinants in multiple system atrophy: A comprehensive interpretation from prodromal to advanced stages
多系统萎缩（multiple system atrophy，MSA）是一种罕见的神经退行性疾病，全球流行病学资料显示其发病率和患病率极低，临床表现为自主神经功能障碍、帕金森综合征和小脑性共济失调的异质性组合。本文系统综述了MSA的流行病学特征、亚型分布、自然病程及预后影响因素，重点阐述了其前驱期表现（如快速眼动期睡眠行为障碍和纯自主神经衰竭）、确诊后的典型临床“里程碑”事件（如反复跌倒、轮椅依赖、吞咽困难和喘鸣）以及影响疾病进展的关键因素（包括发病年龄、自主神经受累程度、亚型和左旋多巴反应性等）。本文还特别探讨了早发型与晚发型MSA的临床特点与预后差异，并指出未来的研究方向应聚焦于生物标志物开发、早期诊断与前驱期识别以及多学科综合管理策略的优化，旨在为临床实践与干预性研究提供参考。
Multiple system atrophy (MSA) is a rare neurodegenerative disorder with very low incidence and prevalence worldwide. Clinically, it presents as a heterogeneous combination of autonomic failure, Parkinsonism and cerebellar ataxia. This review synthesizes current evidence on the epidemiology, subtype distribution, natural history and prognostic modifiers of MSA, with particular emphasis on the prodromal phase (e.g., rapid eye movement sleep behavior disorder and pure autonomic failure), hallmark clinical milestones after diagnosis (e.g., recurrent falls, wheelchair dependence, dysphagia, and inspiratory stridor), and key determinants of disease progression (including age at onset, severity of autonomic involvement, motor subtype and levodopa responsiveness). The clinical features and prognostic contrasts between young-onset and late-onset MSA are examined, and the future priorities focusing on biomarker development, early diagnosis and prodromal detection, and optimization of multidisciplinary care strategies are highlighted, aiming to inform clinical practice and interventional research.
多系统萎缩 / 自然病程 / 预后因素 / 临床里程碑 / 生物标志物
Multiple system atrophy / Natural history / Prognostic factors / Clinical milestones / Biomarkers
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