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评价遗传性小脑型共济失调的临床特征及MRI、CT、诱发电位对该病的诊断及分类的价值。方法：回顾分析了29例遗传性小脑型共济失调患者的临床表现。结果：显示本病是以小脑症状体征为主要表现的系及中枢神经系统多部位的变性疾病，影像学检查MRI优于CT。诱发电位有较高的敏感性，异常率为22％－86％。结论府发电位可以了解传导路的功能状态，特别是亚临床损害，但不能单独作为分类依据。临床诊断及分类有赖于临床观察、影像学检查与诱发电位测定三结合。
To evaluate the clinical features of hereditary cerebellar ataxia and the value of MRI, CT and evoked potentials in the diagnosis and classification of the disease. Methods: A retrospective analysis of 29 patients with hereditary cerebellar ataxia in patients with clinical manifestations. Results: This disease showed that the disease is mainly manifested cerebellar symptoms and signs of the Department of central nervous system and multiple parts of the degenerative diseases, MRI imaging better than CT. Evoked potentials have a higher sensitivity, the abnormal rate was 22% -86%. Conclusions The power generation potential can understand the functional status of the conduction path, especially subclinical damage, but can not be used as a basis for classification alone. Clinical diagnosis and classification depends on clinical observation, imaging and evoked potential test of the three combined.