论文部分内容阅读
目的分析幼儿脊髓性肌萎缩症的临床及电生理特点,探讨电生理检查对本病的诊断意义。方法回顾性分析11例幼儿脊髓性肌萎缩症患儿的临床资料,患儿血清肌酸磷酸激酶水平,正中神经、尺神经、胫神经和腓神经运动传导检查,包括远端潜伏期、运动传导速度和复合肌肉动作电位波幅。结果幼儿脊髓性肌萎缩症的主要临床特征为出生后不久出现进行性加重的四肢弛缓性瘫痪。所有患儿的复合肌肉动作电位波幅显著下降,伴有远端潜伏期延长和运动传导速度减缓。肌电图显示神经源性损伤。结论婴儿型脊髓性肌萎缩症有较典型的临床及电理特征,电生理检查为本病的诊断提供重要依据。
Objective To analyze the clinical and electrophysiological characteristics of children with spinal muscular atrophy and to explore the significance of electrophysiological examination in the diagnosis of this disease. Methods The clinical data of 11 children with spinal muscular atrophy were analyzed retrospectively. Serum creatine phosphokinase level, median nerve, ulnar nerve, tibial nerve and peroneal nerve were measured in children with preschool children with spinal muscular atrophy, including distal latency, motor conduction velocity And compound muscle action potential amplitude. Results The main clinical features of preschool spinal muscular atrophy were flaccid paralysis of the extremities that progressed exponentially soon after birth. The amplitude of the compound muscle action potential was significantly decreased in all children with prolonged distal latency and slowed motor conduction. Electromyography shows neurogenic injury. Conclusion Infantile spinal muscular atrophy has typical clinical and electrophysiological characteristics. Electrophysiological examination provides an important basis for the diagnosis of this disease.