国内有关遗传性痉挛性截瘫临床报告较少,现将一家系病例报告如下。 例1 女12岁,学生,湖北籍。5岁发现行走不稳,足趾拖地,6岁易跌倒,言语不清,常遗尿,症状进行性加重,于1978年3月18日入院。家族中姑母8岁始会行走,10岁死于麻疹,姨表兄一足趾呈连趾畸形,其父母系始表兄妹近亲结婚。检查:发育正常,营养中等,意识清楚,智能正常,一般检查无异常所见。言语欠清晰,双眼底视乳头色较淡,边缘清,双眼轻度水平性眼震,双上肢肌力及肌张力正 The domestic clinical reports of hereditary spastic paraplegia less, now a family of cases are reported as follows. Example 1 Female 12 years old, student, Hubei nationality. 5-year-old found walking unsteady, mop toe, 6-year-old fell easily, unclear speech, often enuresis, progressive symptoms, was admitted on March 18, 1978. The family aunt at the age of 8 will walk, 10-year-old died of measles, aunt cousin was toe deformity, his parents started cousins ​​cousins ​​married. Check: normal development, moderate nutrition, clear awareness, intelligence and normal, no abnormal findings in general inspection. Speech is not clear, the bottom of the eyes as the nipple color lighter, clear edge, mild horizontal eye nystagmus, upper extremity muscle strength and muscle tension positive