骨骼肌特异性酪氨酸受体激酶抗体阳性重症肌无力的低频重复电刺激特点  

Low frequency repetitive nerve stimulation in anti-muscle specific tyrosine kinase receptor antibody positive myasthenia gravis

作　　者：谭颖[1] 管宇宙[1] 朱立[2] 吴双[1] 丁青云[1] 胡又方 崔丽英[1,3] 

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]中国医学科学院北京协和医院核医学科,100730 [3]中国医学科学院神经科学中心

出　　处：《中华神经科杂志》2017年第10期725-729,共5页Chinese Journal of Neurology

摘　　要：目的 描述骨骼肌特异性酪氨酸受体激酶抗体阳性重症肌无力（MG）患者的重复电刺激特点及特殊电生理特点.方法 回顾性地分析和比较了2015年1月至2017年4月就诊于我院门诊、年龄和性别匹配的9例骨骼肌特异性酪氨酸受体激酶抗体阳性MG和19例AChR抗体阳性MG患者的临床资料,并分析比较两组患者经低频电流重复刺激尺神经、面神经和副神经的电生理结果.结果 骨骼肌特异性酪氨酸受体激酶抗体阳性组和AChR抗体阳性组经面神经重复电刺激异常比例分别为6/9和13/19,副神经异常比例为5/9和18/19,尺神经异常比例为0/9和7/19,总体阳性比例分别为8/9和19/19.经副神经刺激时,AChR抗体组异常比例明显较高.Logistic回归分析提示,新斯的明试验阴性是骨骼肌特异性酪氨酸受体激酶抗体阳性MG的独立影响因素（OR=4.25,95%CI 2.19-15.25,P=0.015）.结论 抗骨骼肌特异性酪氨酸受体激酶抗体MG的低频电刺激阳性率总体低于AChR抗体组,尤其经副神经刺激时差异明显.低频重复电刺激和血清抗体检测对诊断有较大帮助.Objective To describe the repetitive nerve stimulation （ RNS） in anti-muscle specific tyrosine kinase （anti-MuSK） receptor antibody positive myasthenia gravis （MG）, and compare with anti-acetylcholine receptor （ AChR ） positive myasthenia gravis , to figure out characteristics of anti-MuSK receptor MG.Methods We analyzed clinical and RNS data of nine anti-MuSK receptor MG and 19 age-and sex-matched anti-AChR MG.RNS was performed to the abductor digiti minimi , orbicularis oculi or musculus frontalis and trapezius .Results In anti-MuSK receptor MG , abnormal RNS in facial nerve was seen in 6/9 and in trapezius was 5/9, in limbs was 0.In anti-AChR MG, abnormal RNS in facial nerve was seen in 13/19, in trapezius was 18/19 and in limbs was 7/19.Abnormal in any of three parts was 8/9 and 19/19 in anti-MuSK receptor MG and anti-AChR MG, respectively.The RNS decrementing was more obvious in facial nerve in anti-Musk receptor MG than in anti-AChR MG.Negative prostigmin test was independently associated with anti-MuSK receptor MG （OR=4.25,95% CI 2.19 -15.25, P=0.015）. Conclusions Abnormal RNS in any of three parts is more pronounced in anti-AChR MG compared with anti-MuSK receptor MG.RNS decrementing in facial nerve is more obvious in anti-AChR MG.Negative prostigmin test can aid in early suspicion in anti-MuSK receptor MG.

关 键 词：重症肌无力 受体 胆碱能 新斯的明 骨骼肌特异性酪氨酸受体激酶 

分 类 号：R746.1[医药卫生—神经病学与精神病学]