Friedreich共济失调是遗传性共济失调的类型之一,是一种主要累及脊髓的进行性病变的疾病,多为常染色体隐性遗传,而显性遗传则不多见。本文将一家系4代9例患者报告如下: 病例介绍先证者,男,66岁,农民。自5岁起行走不稳易摔倒,病情缓慢进展并进行性加重。40岁以后逐渐出现双手动作苯拙及意向性震颤,言语含糊不清,现需双拐方 Friedreich’s ataxia is one of the types of ataxia, a disease mainly involved in progressive lesions of the spinal cord, mostly autosomal recessive, while dominant inheritance is rare. This article will be a family of 4 generations of 9 patients reported as follows: Case description proband, male, 66 years old, farmer. Since the age of 5, walking instability falls easily, the disease progresses slowly and aggravates. After 40 years of age, his hands slowly appear benzene humble and intention tremor, verbal vague, now need to double-shunted side