本文分析了55例小脑型遗传性共济失调的临床资料，男女之比为4：1，2/3患者年龄在37～50之间，并发症状多为行走不稳。主要临床表现为兼有躯干和肢体共济失调（78.2％），眼震（56.4），构音障碍（69.l％），约1/4病人有病理征及家族史。讨论了本病与其他疾病所致共济失调的鉴别诊断，以及本病的病理学基础。 This article analyzes 55 cases of cerebellar ataxia clinical data, male to female ratio of 4: 1, 2/3 patients aged between 37 to 50, mostly complicated by walking instability. The main clinical manifestations of both trunk and limb ataxia (78.2%), nystagmus (56.4), dysarthria (69.1%), about 1/4 of patients with pathological signs and family history. Discusses the differential diagnosis of ataxia caused by this disease and other diseases, and the pathological basis of the disease.