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Roussy-Levy综合征,又名遗传性无反射性共济失调,是一少见的遗传性家族性疾病。1926年首次由Roussy及Levy二氏报告7例。国内尚未见报道。现将我科观察的一家4例报告如下。例1,女19岁,例2,女17岁,例3,男15岁,例4,男10岁。来诊时间:1981年3月。个人史:均在3岁左右才会走路,且双腿走路无力。4、5岁后出现四肢肌肉消瘦,脚心凹陷,手动作不灵活。用眼睛看着才能扣衣扣。晚上没灯穿不上鞋。现均在中、小学学习,学习成绩中等。患儿父母有较近血缘关系(其父的祖母与其母的祖父为同胞兄妹)。检查发现其父双手小鱼际肌瘦小,小指细且向尺侧弯曲,双手轮替运动较笨
Roussy-Levy syndrome, also known as hereditary aphakic ataxia, is a rare hereditary familial disease. In 1926 for the first time reported by Roussy and Levy two cases of 7 cases. Not yet reported in China. Now we observe a family of 4 reports are as follows. Example 1, female 19 years old, case 2, female 17 years old, case 3, male 15 years old, case 4, male 10 years old. Visiting time: March 1981. Personal history: all walk around 3 years old, and legs weakness. 4,5 years after the emergence of limb muscle wasting, soles of feet depression, manual action is not flexible. Watched with my eyes to button buckle. No light at night wear no shoes. Are in primary and secondary schools are learning, academic performance is medium. Parents of children with a closer relationship (his father’s grandmother and his grandfather’s siblings). Examination revealed that his father’s hands and small intertidal muscle is small, the little finger fine and curved to the ulnar side, his hands stupid exercise stupid