Department of Neurology, Dysautonomia Center, New York University School of Medicine, 530 First Avenue, Suite 9Q, New York, NY, 10016, USA.
J Neural Transm (Vienna). 2014 May;121(5):507-12. doi: 10.1007/s00702-013-1133-7. Epub 2013 Dec 15.
Multiple system atrophy (MSA) is a neurodegenerative disease with two motor phenotypes: parkinsonian (MSA-P) and cerebellar (MSA-C). To elucidate whether in addition to the motor abnormalities there are other significant differences between these phenotypes, we performed a retrospective review of 100 patients (61 males, 39 females) with a diagnosis of possible (12 %), or probable (88 %) MSA. Four patients eventually had post-mortem confirmation (i.e., definite MSA). Sixty percent were classified as having MSA-P and 40 % as MSA-C. MSA-C and MSA-P patients had similar male prevalence (60 %), age of onset (56 ± 9 years), and frequency of OH (69 %). Brain MRI abnormalities were more frequent in MSA-C patients (p < 0.001). Mean survival was 8 ± 3 years for MSA-C and 9 ± 4 years for MSA-P patients (p = 0.22). Disease onset before 55 years predicted longer survival in both phenotypes. Initial autonomic involvement did not influence survival. We conclude that patients with both motor phenotypes have mostly similar survivals and demographic distributions. The differences here identified could help counseling of patients with MSA.
多系统萎缩（MSA）是一种神经退行性疾病，有两种运动表型：帕金森型（MSA-P）和小脑型（MSA-C）。为了阐明除了运动异常之外，这些表型之间是否还有其他显著差异，我们对 100 名可能（12%）或可能（88%）MSA 的患者进行了回顾性审查。4 名患者最终进行了尸检确认（即明确的 MSA）。60%的患者被归类为 MSA-P，40%的患者为 MSA-C。MSA-C 和 MSA-P 患者的男性患病率（60%）、发病年龄（56±9 岁）和 OH 频率（69%）相似。MSA-C 患者的脑 MRI 异常更为常见（p<0.001）。MSA-C 和 MSA-P 患者的平均存活时间分别为 8±3 年和 9±4 年（p=0.22）。两种表型中，发病年龄<55 岁预测存活时间更长。初始自主神经受累并不影响生存。我们的结论是，两种运动表型的患者的存活率和人口统计学分布大多相似。这里确定的差异可能有助于对 MSA 患者进行咨询。
