摘要
目的探讨婴儿型脊髓性肌萎缩症(SMA)的临床和电生理特点。方法回顾性分析20例婴儿型SMA患儿的临床资料。结果 20例SMA患儿临床表现为出生后进行性加重的四肢弛缓性瘫痪,肌张力低下,腱反射消失。肌电图表现为神经源性损害,所检测的50条运动神经均示神经肌肉复合动作电位波幅衰减,其中10条合并末端潜伏期延长及传导速度轻度减慢;所检测的25条感觉神经传导速度在正常范围。肌肉活检为典型的神经源性肌萎缩。结论婴儿型SMA的临床特点为出生后进行性加重的四肢弛缓性瘫痪,肌电图检查显示为神经源性损害。
Objective To explore the clinical and electrophysiological features of infantile spinal muscular atrophy（SMA）. Methods The clinical data of 20 SMA children were analyzed retrospectively. Results The clinical manifestations of the 20 SMA children were postnatal progressive flaccid paralysis of four extremities, hypomyotonia, and deep tendon reflexes were disappeared. The examination of electromyography （EMG） showed neurogenous damage. The wave amplitude of nerve-muscle compound action potential was decreased in all of the 50 determined motor nerves, and 10 of them showed distal latent period prolonged and motor conduction velocity reduced slightly. The conduction velocity in 25 determined sensory nerves was in normal renge. The result of muscle biopsy was typical nervine myatrophy. Conclusions The chief clinical features of infantile SMA are postnatal progressive symmetrical flaccid paralysis in four extremities. EMG demonstration is motoneuron degeneration.
出处
《临床神经病学杂志》
CAS
北大核心
2011年第3期209-210,共2页
Journal of Clinical Neurology
关键词
脊髓性肌萎缩
婴儿型
临床特点
spinalmuscular atrophy, infantile
clinical feature
