Comparable progression of spinocerebellar ataxias between Caucasians and Chinese
Comparable progression of spinocerebellar ataxias between Caucasians and Chinese
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DOI：
10.1016/j.parkreldis.2018.12.023
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发表时间：
2019-05-01
影响因子：
4.1
通讯作者：
Soong, Bing-Wen
中科院分区：
文献类型：
--
作者：
Lin, Yi-Cheng;Lee, Yi-Chung;Soong, Bing-Wen
简介：本研究的目的是重新评估中国人群中五种最常见的脊髓小脑共济失调 (SCA) 的进展，并与其他种族群体进行急需的批判性比较。关于亚洲人群 SCA 的纵向队列研究很少。早期研究中的一项有趣发现表明，中国人的 SCA 进展速度比白人更快。 方法：使用共济失调评估和评级量表 (SARA) 连续评估 SCA1、SCA2、SCA3、SCA6 或 SCA17 患者五年。使用线性混合模型来比较不同 SCA 亚型患者中使用 SARA 测量的年进展率。分析了进展率的预测因子。 结果：总共纳入 199 例 SCA 患者（10 例 SCA1、37 例 SCA2、118 例 SCA3、25 例 SCA6 和 9 例 SCA17）。 SARA分数的年平均增幅为：SCA1为1.23分，SCA2为1.52分，SCA3为1.60分，SCA6为0.99分，SCA17为3.26分。较大的 CAG 重复长度 (>= 74) 与 SCA3 的进展较快相关，而首次就诊时的 SARA 总分较低（
Introduction: The aim of this study is to reappraise the progression of the five most common spinocerebellar ataxias (SCAs) in the Chinese population and to establish a much-needed critical comparison with that in other ethnic groups. There are very few longitudinal cohort studies of SCAs in Asian populations. An intriguing finding in an earlier study demonstrated a faster progression of SCA among Chinese than that among Caucasians.Methods: Patients with SCA1, SCA2, SCA3, SCA6 or SCA17 were consecutively assessed using the scale for the assessment and rating of ataxia (SARA) for five years. A linear mixed model was used to compare the annual progression rates measured using the SARA among patients with different SCA subtypes. Predictors of the progression rates were analyzed.Results: A total of 199 patients with SCA (10 with SCA1, 37 with SCA2, 118 with SCA3, 25 with SCA6 and 9 with SCA17) were enrolled. The mean annual increase in SARA scores was 1.23 points for SCA1, 1.52 points for SCA2, 1.60 points for SCA3, 0.99 points for SCA6 and 3.26 points for SCA17. A larger CAG repeat length (>= 74) was associated with faster progression in SCA3, whereas a lower total SARA score at the first visit (