我科收治六例脊髓性进行性肌萎缩均为男孩。一例七天发病,其余五例婴儿期发病,其中两例为兄弟。六例均有四肢肌肉松驰、萎缩、肌张力减低,以双上肢为显著,腱反射消失,不能站立。四例生后均不会坐,不会翻身,六例智力均正常。三例血钾降低。两例X光照片显示骨质疏松,骨皮质变薄。本病尚无特殊疗法。死亡原因多为呼吸麻痹和继发呼吸道感染。 My department admitted six cases of progressive spinal muscular atrophy were boys. A case of seven days of onset, the other five cases of infancy, of which two were brothers. Six cases had limb muscle relaxation, atrophy, reduced muscle tone, with both upper extremities as significant, tendon reflex disappeared, can not stand. Four will not sit after birth, will not stand up, six cases of intelligence are normal. Three cases of potassium decreased. Two cases of X-ray showed osteoporosis, cortical bone thinning. The disease is no special treatment. Most of the causes of death are respiratory paralysis and secondary respiratory tract infections.