婴儿型进行性脊髓性肌萎缩是小儿神经系统的少见病,文献报导国内仅见1例尸检报告。我院对2例新生儿起病,婴儿期死亡的典型病例进行了尸检,现报告如下。病例报告例1,男,47天,住院号68559。患儿系第1胎,足月剖腹产。生后12小时即发现四肢活动少,吸吮无力,哭声低微。父母非近亲婚配。母孕期健康,胎动正常。无家族史。入院检查:体重4kg, Infantile progressive spinal muscular atrophy is a rare disease of the nervous system in children, reported in the literature only one case of autopsy reported. Our hospital on 2 cases of neonatal onset, the typical case of infant death autopsy, are as follows. Case report 1, male, 47 days, hospital number 68559. Children in the first child, full-term caesarean section. 12 hours after birth that limb activity is less, sucking weakness, low cry. Parents non-relatives marriage. Mother during pregnancy, normal fetal movement. No family history. Admission examination: weight 4kg,