婴儿脊髓性进行性肌萎缩是一种少见的常染色体隐性遗传性疾病。本文收集11例,现分析报告如下。临床资料诊断依据:(1)1岁前发病,以进行性肌松弛为特征,伴有反复呼吸通感染,(2)肌萎缩、肌张力减弱、腱反射消失(或明显减弱)双侧对称,以双下肢为重。(3)血清肌酸磷酸激酶正常,新斯的明试验阴性。(4)肌电图提示神经源性肌损害.肌活检纤维结构模糊不 Infantile spinal muscular dystrophy is a rare autosomal recessive disease. This collection of 11 cases, the analysis is as follows. Clinical data on the basis of diagnosis: (1) onset before the age of 1, characterized by progressive muscle relaxation, with repeated respiratory infections, (2) muscle atrophy, decreased muscle tone, tendon reflex disappeared (or significantly weakened) bilateral symmetry, With both lower extremities as the most important. (3) normal serum creatine phosphokinase, neostigmine test negative. (4) EMG suggestive of neurogenic muscle damage, muscle biopsy fibrous structure is not fuzzy