Marie 共济失调症是一种少见的家族遗传性疾病,它与 Friedreie 共济失调症一起构成遗传性小脑性共济失调症的两个主要类型.该型有时与 Friedreie型难以区别,但两者遗传特点,发病年龄和临床症状显著不同.本型特点是显性遗传,20～40岁发病,临床症状以缓慢进展小脑性共济失调为主.本文报告朝鲜族一家4代4例 Marie 共济失调,并附有系谱(附图). Marie ataxia is a rare familial hereditary disease that, together with Friedreie’s ataxia, constitutes the two main types of hereditary cerebellar ataxia. This type is sometimes indistinguishable from the Friedreie type, but both Genetic characteristics, age of onset and clinical symptoms were significantly different.This type of feature is dominant inheritance, 20 to 40-year-old onset, the clinical symptoms of slow progress in cerebellar ataxia.This article reports a Korean family 4 4 Marie Kyrgyzstan Disorders, with accompanying pedigree (with photos).