In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing field. First, we highlight the non-motor features at disease onset when MSA can be indistinguishable from pure autonomic failure or other chronic neurodegenerative conditions. We describe the progression of clinical features to aid the diagnosis of MSA early in the disease course. We go on to describe the levels of diagnostic certainty and we discuss MSA subtypes that do not fit into the current diagnostic criteria, highlighting the complexity of the disease as well as the need for revised diagnostic tools. Second, we describe the pathology, clinical description, and investigations of cardiovascular autonomic failure, urogenital and sexual dysfunction, orthostatic hypotension, and respiratory and REM-sleep behavior disorders, which may precede the motor presentation by months or years. Their presence at presentation, even in the absence of ataxia and parkinsonism, should be regarded as highly suggestive of the premotor phase of MSA. Finally, we discuss how the recognition of the broader spectrum of clinical features of MSA and especially the non-motor features at disease onset represent a window of opportunity for disease-modifying interventions.

中文翻译：

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MSA 的更新：运动前和非运动特征为早期诊断和干预打开了机会之窗。


在这篇综述中，我们描述了多系统萎缩 (MSA) 中常见的广泛临床特征，重点关注运动前阶段和非运动症状，提供了对该领域当前认识的最新概述。快速增长的领域。首先，我们强调疾病发作时的非运动特征，此时 MSA 与纯粹的自主神经衰竭或其他慢性神经退行性疾病无法区分。我们描述临床特征的进展，以帮助在病程早期诊断 MSA。我们继续描述诊断确定性水平，并讨论不符合当前诊断标准的 MSA 亚型，强调疾病的复杂性以及修订诊断工具的需要。其次，我们描述了心血管自主神经衰竭、泌尿生殖和性功能障碍、直立性低血压以及呼吸和快速眼动睡眠行为障碍的病理学、临​​床描述和研究，这些障碍可能先于运动表现数月或数年。即使没有共济失调和帕金森病，它们在就诊时的出现也应被视为高度提示 MSA 的运动前阶段。最后，我们讨论了对 MSA 更广泛临床特征的认识，尤其是疾病发作时的非运动特征，如何代表疾病缓解干预措施的机会之窗。