Friedreich氏共济失调症,是一种神经系统的遗传性疾病。患者有脊髓及小脑受损的体征,病程进行缓慢,多在起病后10年内严重残废而失去生活能力,常因继发感染或心脏病而死亡(1,2,3)。我们对一个近亲结婚所生子中4人均罹患本症的家系进行了调查,并对其遗传规律进行了初步探讨,现报告如下。 Friedreich’s ataxia is a hereditary disease of the nervous system. The patient has signs of spinal cord and cerebellar damage. The course of the disease is slow, with severe disability and loss of life within 10 years after onset, often resulting from secondary infection or heart disease (1, 2, 3). We investigated a pedigree of 4 people who suffered from this disease in a child born of a close relative and conducted a preliminary study on their genetic laws. The report is as follows.