【摘要】 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。更多还原

【Abstract】 Objective:To evaluate the features and clinical significance of repetitive nerve stimulation(RNS)in patients with amyotrophic 1ateral sclerosis(ALS)and myasthenia gravis(MG).Methods:The consecutive patients with definite or probable ALS and patients with MG,referral to our department during December 2013 and August 2015,were enrolled.RNS examination was performed for all patients and EMG examinmion only for ALS patients.Results:1Among the enrolled 68 ALS and 52 MG,there were 37 and 34cases showed positive RNS,in which the total positive rates of RNS were 54.4% and 65.4%,respectively,with no significant difference(χ2=0.993,P=0.319).2The positive rates of ulnar and accessory nerves were 4.4% and 51.5%(χ2= 6.972,P=0.000)in ALS patients;those of ulnar,median,facial and accessory nerves were 15.4%,38.7%,58.0% and 65.4%(χ2=30.152,P=0.000)in MG patients,respective-ly.3The decremental extent(%)for ulnar nerve was 15.5±7.5and 30.4±8.4(t=2.699,P=0.024),for accessory nerve 18.2±7.1and 27.3±10.7(t=17.442,P=0.000)in ALS and MG patients,respectively.4The rate of disease progression was 1.5±1.0and 1.0±0.9(t=2.11,P=0.039);CMAP amplitudes(mV)of ulnar nerve were 5.0±3.4and 7.0±3.0(t=-2.496,P=0.016),median nerve 2.8±2.4and 4.6±2.7(t=-2.993,P=0.004),in RNS positive and negative subgroup of ALS patients,respectively.Conclusion:Occurrence of decremental response to RNS in ALS patients is up to more than half of the cases,whereas the decremental extent is less than that in MG patients.The positive RNS and lower amplitude of CMAP in ALS patients suggest disease progression more rapidly,which would be helpful for follow-up assessment of the prognosis.更多还原