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目的 研究运动神经元病 (MND)患者的单纤维肌电图 (SFEMG)改变、病理生理机制及临床意义。方法 对 6 8例 (男 4 3例 ,女 2 5例 )经病史、临床表现和神经电生理检查测定证实的MND患者进行了伸指总肌SFEMG测定 ,并与年龄匹配的正常人对照。结果 所有患者感觉神经传导速度测定均正常 ,肌电图为广泛神经源性损害。SFEMG检查发现 :颤抖值 (jitter)为 30～ 178μs,平均 (80 2± 32 6 ) μs ;jitter>5 5 μs占 5 0 %～ 10 0 0 % ,平均 6 0 6 %± 2 9 0 % ;阻滞 (block)所占百分比为 0 0 %～90 0 % ,平均 2 9 3%± 30 0 % ;纤维密度 (FD)为 1 4～ 4 0 ,平均 2 6± 0 6。其中 5 1例确诊和拟诊的肌萎缩侧索硬化患者jitter增宽、block和FD增高最明显。还发现伸指总肌肌无力的程度与jitter增宽和block百分比呈明显的负相关。结论 SFEMG的异常改变在确诊患者组最明显 ;jitter增宽、block百分比升高和FD增高与肌肉无力的严重程度呈明显的负相关 (P <0 0 0 1) ;jitter增宽、block百分比升高和FD增高反映进行性失神经、神经再生和神经肌肉接头处的传递情况
Objective To study the changes of single-fiber electromyography (EMG), pathophysiological mechanism and clinical significance in patients with motor neuron disease (MND). Methods SFEMG of 86 cases (43 males and 25 females) with MND confirmed by history, clinical manifestation and neuroelectrophysiological examination was compared with age-matched normal subjects. Results Sensory nerve conduction velocity was normal in all patients, and electromyography was extensive neurogenic damage. The results of SFEMG showed that jitter was 30 ~ 178μs with an average of (80 2 ± 32 6) μs; jitter> 55μs was 50% ~ 100% with an average of 6 0 6% ± 290%; The block percentage was 0 0% -90 0% with an average of 293% ± 30 0%. The fiber density (FD) was 14 ~ 40 with an average of 26 ± 0 6. Among them, 51 cases of amyotrophic lateral sclerosis diagnosed and diagnosed were jitter broadened, and the increase of block and FD was the most obvious. It was also found that extensor extensor total muscle weakness was significantly negatively correlated with jitter broadening and percentage of block. Conclusions The abnormal changes of SFEMG are the most obvious in the diagnosed patients. Jitter broadening, increasing of the percentage of block and increasing of FD are negatively correlated with the severity of muscle weakness (P <0.01); jitter broadening, percentage of block rising High and FD increases reflect the progression of progressive denervation, nerve regeneration and neuromuscular junction delivery