29例遗传性小脑型共济失调的临床与诱发电位分析

Clinical and Evoked Potentials Analysis on 29 Cases of Hereditary Cerebeller Ataxias

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。

Objective: To evaluate the clinica characteristic of hereditary cerebellar ataxias and diagnostic and classificationvalues of MRI, CF and evoked potentials. Methods : A retrosective analysis of 29 cases of henditary ceretallar ataxias was pre-sental. Reslts: The results showed tha it is chronic mive fonns of muti - system degenerative cerebellar disease of CN S.MRI has higher sensitivity than CT in manifesting the degenerative Lesion . Evoked potentials had high positive rate (22% -86% ). Conclusion : Evoked potentials can convey the function condition of the neamn conduction pathways, especially asymp-tomatic Lesions . The Qassification of disoat be made only according to evoked potentials . The diagnosis and classification de- pends on the combination of clinical manifesfation , CT or MRI picture and evoked potentials.