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本病为一种较少见的家族遗传疾病。现将我院半年内所见二例报告如下: 病例报告张××:女、41岁、锦县人。因渐进性走路不稳,说话不清二十年,于1979年3月9日就诊。患者二十岁前一切正常。二十岁时发现走路不稳,两下肢活动时发笨,尚能坚持体力劳动,后逐渐加重。至30岁时走路不稳更为严重,不能参加田间劳动,可坐在床上做针线活。35岁不能承担家务劳动,走路蹒跚,需他人扶行,并出现吐词不清,手足麻木,38岁时出现尿急,视物不清,面部肌肉颤抖,喝水呛。既往否认其它病史。家族病史:祖母20岁左右走路不稳,逐年加重,至37岁时似不倒翁。父亦患同样病,30岁左右发病。母健在。本人19岁结婚,爱人健在,生一子一女:长子,男、17岁,十四岁时发现走路笨。女,14岁,现正常。
The disease is a rare family genetic disease. Now I see two cases within six months of hospital reports are as follows: Case Report Zhang × ×: Female, 41 years old, Jinxian people. Due to progressive walking instability, talk for two decades, on March 9, 1979 treatment. The patient was normal before the age of twenty. Twenty years of age found walking unsteadily, two stupid activities when stupid, still able to adhere to manual labor, and then gradually increased. To 30 years of age walking more serious instability, can not participate in field work, can sit in bed doing needlework. 35-year-old can not afford to housework, walking hobbled, need help others, and there is unclear articulation, numbness, 38-year-old urinary emergencies, blurred vision, facial muscle trembling, choking drink. Previously deny other medical history. Family history: Grandmothers walk around 20 years of instability, increased year by year, to 37 years old like tumbler. Father also suffer from the same disease, 30-year-old onset. Mother healthy. I am 19 years old married, lover alive, giving birth to a son and a daughter: the eldest son, male, 17 years old, fourteen years old found walking stupid. Female, 14 years old, is normal.