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经过连续4年观察,发现和证实一个家系两代5例Gerstmann—strāussler综合征。发病年龄17～26岁。首发症状为小脑性共济失调,伴下肢肌萎缩。CT示小脑萎缩。4例发病后4～17年死亡。经小脑活体组织检查、免疫组织化学染色及实验室内动物传递等研究,证明国内确有此病,应予注意。
After 4 consecutive years of observation, found and confirmed a family of two generations of 5 cases of Gerstmann-strāussler syndrome. Age of onset 17 to 26 years old. The first symptom is cerebellar ataxia, with lower limb muscle atrophy. CT showed cerebellar atrophy. 4 cases died 4 to 17 years after onset. After the cerebellum biopsy, immunohistochemical staining and laboratory animal transmission studies have proved that the domestic disease indeed, should be noted.