Sporadic ataxias in Japan - a population-based epidemiological study
Sporadic ataxias in Japan - a population-based epidemiological study
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DOI：
10.1007/s12311-008-0028-x
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发表时间：
2008-06-01
期刊：
影响因子：
3.5
通讯作者：
Nishizawa, Masatoyo
中科院分区：
文献类型：
--
作者：
Tsuji, Shoji;Onodera, Osamu;Nishizawa, Masatoyo
散发性脊髓小脑性共济失调 (SCA) 包括流行病学和病因学知之甚少的异质性疾病。描述了日本人群中散发性共济失调的基于人群的流行病学分析。日本人口中 SCA 的患病率估计为 18.5/100,000。散发性 SCA 占全部 SCA（包括遗传性 SCA）的 67.2%，其中橄榄桥小脑萎缩 (OPCA) 是最常见的散发性共济失调 (64.7%)。根据国际合作共济失调评定量表（ICARS）进行的自然史分析显示，只有33%的OPCA患者在发病后4-5年内能够至少用一根拐杖行走，这远低于皮质小脑萎缩（CCA）患者。同样，43% 的 OPCA 患者在发病后 4-5 年能够独立生活，而 76% 的 CCA 患者在相同病程内能够独立生活。基于人群的流行病学分析应提供有关 SCA 自然史的重要信息。
Sporadic spinocerebellar ataxias (SCAs) comprise heterogeneous diseases with poorly understood epidemiologies and etiologies. A population-based epidemiological analysis of sporadic ataxias in the Japanese population was described. The prevalence rate of SCAs in the Japanese population is estimated to be 18.5/100,000. Sporadic SCAs account for 67.2% of total SCAs including hereditary SCAs, with olivopontocerebellar atrophy (OPCA) being the most common form sporadic ataxia (64.7%). The natural history analysis conducted on the basis of International Cooperative Ataxia Rating Scale (ICARS) showed that only 33% of patients with OPCA were able to walk at least with one stick 4-5 years after the onset of OPCA, which is much less than that of patients with cortical cerebellar atrophy (CCA). Similarly, 43% of patients with OPCA were able to stand alone 4-5 years after the onset, while 76% of patients with CCA were able to stand alone at the same disease duration. A population-based epidemiological analysis should provide essential information on the natural history of SCAs.