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多系统萎缩：临床特征与诊断

Authors
Stewart A Factor, DO
Christine Doss Esper, MD
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD

引言

多系统萎缩(multiple system atrophy, MSA)是将一组神经变性综合征集合在一起的一个统称术语，这些综合征包括：橄榄体脑桥小脑萎缩(olivopontocerebellar atrophy, OPCA)、纹状体黑质变性和Shy-Drager综合征，它们具有以不同程度的自主神经功能障碍、小脑功能异常、帕金森综合征和皮质脊髓变性为特征的相似的脑部病理改变。

本专题将总结MSA的流行病学、临床特征和诊断。MSA的预后和治疗以及其他类型的帕金森综合征将单独讨论。 (参见“多系统萎缩：预后与治疗”和“帕金森病的临床表现”和“帕金森病的诊断”)

历史背景

在1900年，Dejerine和Thomas写了第一份散发性OPCA的报告，该病后来成为MSA疾病谱的一部分[1]。1925年，直立性低血压作为自主神经功能障碍的一种表现得到研究描述[2]。1960年，Shy和Drager报道了伴直立性晕厥、阳痿和膀胱功能障碍的自主神经系统特征的患者接着发生了步态异常、震颤和肌束震颤等其他症状和体征[3]。该疾病逐渐被人们称为Shy-Drager综合征。

同样是在1960年，报道的首批病例以不对称性帕金森综合征为主，主要表现为运动不能和强直[4]。作者提示，纹状体黑质变性是与这些病例对应的的病理改变。

1969年，引入了将3种临床综合征(OPCA、Shy-Drager综合征以及纹状体黑质变性)都包含在内的术语“多系统萎缩”[5]。纹状体黑质变性后来被重新定义为以帕金森综合征为主的多系统萎缩(MSA with predominant parkinsonism, MSA-P)，而OPCA被重新定义为以小脑性共济失调为主的多系统萎缩(MSA with predominant cerebellar ataxia, MSA-C)[6]。当以自主神经功能障碍为主时，可使用术语Shy-Drager综合征。

                

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Literature review current through: 2017-07 . | This topic last updated: 2017-03-02.
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