目的　评价遗传性小脑型共济失调的临床特征及CT ,MRI对该病的诊断价值。方法　回顾分析了2 5例遗传性小脑型共济失调者的临床及CT ,MRI表现。结果　CT ,MRI可发现不同程度的小脑或脑干萎缩 ,异常率为 61 1%～ 87 5 %。结论　MRI,CT为诊断该病极为重要的检查 ,其阳性率与病程长短有关 ,MRI优于CT。 Objective To evaluate the clinical features of hereditary cerebellar ataxia and the diagnostic value of CT and MRI in the diagnosis of the disease. Methods Retrospective analysis of 25 cases of hereditary cerebellar ataxia clinical and CT, MRI findings. Results CT and MRI showed different degree of cerebellar or brainstem atrophy, the abnormal rate was 61 1% ~ 87 5%. Conclusion MRI and CT are very important tests for the diagnosis of the disease. The positive rate is related to the duration of the disease, and MRI is superior to CT.