本文报告家族性显性遗传之视动特征。32岁的母亲和9、8、4岁三个孩子中,母亲及其两个幼女早年发病,为非进行性向上跳眼震和轻度共济失调,长子为向上顿挫凝视眼震。核磁共振发现母亲及二女小脑蚓部萎缩,而子则正常。眼震电图检查:4人中3人有向上的自发性眼震。慢相速度为8°/sec,向上看速度加大,向下看降低;2例慢相呈线性,光线和头 This article reports the optokinetic characteristics of familial dominant inheritance. 32-year-old mother and 9,8,4-year-old three children, the mother and her two young girls early onset, progressive non-progressive choroid and mild ataxia, the eldest son upset and stared nystagmus. Nuclear magnetic resonance found that the mother and the second female cerebellar vermis atrophy, while the child is normal. 3. Eye electronystagmography: 3 of 4 people have spontaneous nystagmus. Slow phase velocity of 8 ° / sec, look faster and lower look down; 2 cases of slow phase was linear, light and head