进行性肌营养不良的神经电生理检测

Analysis of Electroneurophysiological Detection on Children with Progressive Muscular Dystrophy

目的探讨神经电生理检测对小儿进行性肌营养不良(PMD)的诊断价值。方法对32例PMD患儿的临床特征及实验室资料进行分析,并作针极肌电图(EMG)及神经传导(NCV)检测。观察静息状态时自发电位,测定轻收缩时运动单位电位(MUP)的时限、波幅,大力收缩时的幕集相。测定运动、感觉神经的传导速度(MCV、SCV)及动作电位的末端潜伏期(ML)、波幅(AMP)。结果PMD患儿32例自发电位的异常率为49.2%,胫前肌达80.9%,MUP时限缩短了29.7%～62.4%,大力收缩时波幅降低,与正常值比较有显著差异(P<0.01)。动作电位的ML轻度延长3.70%,AMP降低5.56%。结论神经电生理检测在PMD患儿的诊断、鉴别诊断及早期诊断中有重要意义。

Objective To explore the value of electroneurophysiological detection in diagnosis of children progressive muscular dystrophy （PMD）.Methods The clinical features and laboratory data were analyzed in 32 children with PMD ,and electromyography （EMG） and nerve conduction velocity（NCV） were performed. Parameters studied included spontaneous activity , duration and amplitude of motor unit potential（MUP）, pattern of recruitment as strong contracting, sensory conduction velocity（SCV）, motor conduction velocity（MCV）, distal latency and distal amplitude. Results The abnormality rates of spontaneous potentials was 49.2% and 80.9% in tibialis anterior. The decrease of duration of MUP was 29.7% - 62.4%. Amplitude of strong contracting was significantly decreased.There were different from those in normal children（P〈0.01 ）. Slightly prolongation of the distal latency was found 3.70%, and decrease of distal amplitude wag found 5.56 %. Conclusion Electroneurophysiological detection plays an important role in diagnosis, identily diagnosis and early diagnosis of PMD.