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泛美多系统萎缩协作组(PANMSA)。一项多系统萎缩的泛美多中心队列研究。

Pan-American Consortium of Multiple System Atrophy (PANMSA). A Pan-American multicentre cohort study of multiple system atrophy.

机构信息

Department of Movement Disorders, Instituto Neurociencias de Buenos Aires, INEBA, Buenos Aires, Argentina Department of Neurology, Sanatorio de la Trinidad Mitre, Buenos Aires, Argentina.

Instituto Nacional de Neurología y Neurocirugía, México City, Mexico.

出版信息

J Parkinsons Dis. 2014;4(4):693-8. doi: 10.3233/JPD-140434.

DOI:10.3233/JPD-140434
PMID:25213997
Abstract

BACKGROUND

Multiple system atrophy (MSA) is an adult-onset and rapidly progressive, neurodegenerative condition that presents with autonomic dysfunction, parkinsonism, cerebellar ataxia and corticospinal deficits. Clinical, demographic and epidemiological data from different regions have provided valuable information concerning the natural history of MSA. There are no published data of Multiple System Atrophy (MSA) in Latin American countries.

OBJECTIVE

To describe clinical and epidemiological data of patients with "possible" MSA from seven referral movement disorders centers from Argentina, Chile, Mexico, Peru and United States.

METHODS

We conducted a retrospective, observational, cross-sectional Pan-American multicentre cohort study of MSA.

RESULTS

The sample was composed of 82 females and 77 men with the diagnosis of "possible" MSA with a mean age at onset of 65 ± 10 years. 67.29% of the individuals had a MSA-P variant with a mean age at onset of 61.47 ± 10.28 years, whereas the mean age at onset in the MSA-C patients was 57.44 ± 10.58 years. Interestingly, MSA-C-was more prevalent in Non-Caucasian (50-Mestizo and 2 Asian patients) than Caucasians (51.92% vs. 20.79%, p = 0.0001). Dysautonomic symptoms were present in 95.6% of the patients, parkinsonism in 85.5%, pyramidal signs in 25.8% and depression in 48.4% of the patients.

CONCLUSIONS

Our epidemiological and clinical data appears to be similar to other Western international series, however, of note, the MSA-C phenotype was predominant in Non-Caucasians, more specifically the Mestizo population. This observation opens a new path to explore. Larger prospective epidemiologic studies in Latin America may provide valuable information concerning MSA in the region.

摘要

背景

多系统萎缩(MSA)是一种成人发病且进展迅速的神经退行性疾病,其特征为自主神经功能障碍、帕金森病、小脑共济失调和皮质脊髓束缺陷。来自不同地区的临床、人口统计学和流行病学数据为 MSA 的自然史提供了有价值的信息。目前尚无关于拉丁美洲国家 MSA 的发表数据。

目的

描述来自阿根廷、智利、墨西哥、秘鲁和美国的七个转诊运动障碍中心的“可能”MSA 患者的临床和流行病学数据。

方法

我们进行了一项回顾性、观察性、跨地区的泛美多中心队列研究。

结果

该样本由 82 名女性和 77 名男性组成,他们被诊断为“可能”MSA,平均发病年龄为 65 ± 10 岁。67.29%的个体为 MSA-P 变异型,平均发病年龄为 61.47 ± 10.28 岁,而 MSA-C 患者的平均发病年龄为 57.44 ± 10.58 岁。有趣的是,MSA-C 在非白种人(5 名梅斯蒂索人和 2 名亚洲人)中比白种人更为普遍(50% vs. 20.79%,p = 0.0001)。95.6%的患者存在自主神经症状,85.5%存在帕金森病,25.8%存在锥体束征,48.4%存在抑郁。

结论

我们的流行病学和临床数据似乎与其他西方国际系列相似,然而,值得注意的是,MSA-C 表型在非白种人中更为常见,特别是在梅斯蒂索人群中。这一观察结果开辟了新的探索途径。在拉丁美洲进行更大规模的前瞻性流行病学研究可能会为该地区的 MSA 提供有价值的信息。

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