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目的:比较重复神经刺激(RNS)应用于肌萎缩侧索硬化(ALS)和重症肌无力(MG)患者的特点及临床意义。方法:收集2013年12月至2015年8月就诊于我院,临床确诊或拟诊型ALS患者和临床诊断的MG患者,比较远、近端神经低频RNS时,复合肌肉动作电位(CMAP)波幅及其异常递减,对两组患者诊断性评估的价值。结果:1 ALS患者68例、MG 52例,RNS阳性者分别为37例(54.4%)、34例(65.4%),两组总阳性率无差异(χ2=0.993,P=0.319)。2RNS阳性率在ALS组的尺、副神经分别为4.4%、51.5%(χ2=6.972,P=0.000),MG组尺、正中、面、副神经15.4%、38.7%、58.0%、65.4%(χ2=30.152,P=0.000)。3RNS递减幅度(%)在ALS和MG组的尺神经分别为15.5±7.5、30.4±8.4(t=2.699,P=0.024),副神经18.2±7.1、27.3±10.7(t=17.442,P=0.000)。4ALS组中RNS阳性和阴性亚组,病情进展速度1.5±1.0、1.0±0.9(t=2.11,P=0.039);尺神经运动波幅(mV)为5.0±3.4、7.0±3.0(t=-2.496,P=0.016),正中神经2.8±2.4、4.6±2.7(t=-2.993,P=0.004)。结论:ALS患者中RNS递减反应者高达50%以上,但递减幅度不如MG;RNS阳性及运动反应波幅低提示ALS病情进展速度快,有助于预后的随访评估。
Objective: To compare the characteristics and clinical significance of repeated nerve stimulation (RNS) in patients with amyotrophic lateral sclerosis (ALS) and myasthenia gravis (MG). Methods: From September 2013 to August 2015, patients with MG clinically diagnosed or diagnosed as ALS and clinical diagnosis of MG were collected. The amplitude of compound muscle action potential (CMAP) And its abnormal decline, the diagnostic value of the two groups of patients. Results: 1 ALS patients 68 cases, MG 52 cases, RNS positive were 37 cases (54.4%), 34 cases (65.4%), there was no difference between the two groups (χ2 = 0.993, P = 0.319). The positive rates of 2RNS in ALS group were respectively 4.4%, 51.5% (χ2 = 6.972, P = 0.000). In MG group, the rates of ulnar, median, facial and accessory nerve were 15.4%, 38.7%, 58.0% and 65.4% χ2 = 30.152, P = 0.000). The decrement (%) of 3RNS in the ALS and MG groups was 15.5 ± 7.5 and 30.4 ± 8.4 (t = 2.699, P = 0.024), and the accessory nerves were 18.2 ± 7.1 and 27.3 ± 10.7 (t = 17.442, P = 0.000 ). In the 4ALS group, the RNS positive and negative subgroups showed a progression of 1.5 ± 1.0,1.0 ± 0.9 (t = 2.11, P = 0.039). The mV of ulnar nerve was 5.0 ± 3.4,7.0 ± 3.0 (t = -2.496 , P = 0.016), median nerve 2.8 ± 2.4, 4.6 ± 2.7 (t = -2.993, P = 0.004). CONCLUSIONS: The patients with ALS have more than 50% reduction in RNS, but the decreasing rate is not as good as MG. The positive RNS and the low amplitude of motor response suggest that the progression of ALS is fast and contributes to the follow-up evaluation of prognosis.