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1、重症肌无力胸腺切除术及围术期处理上海长海医院胸心外科杨立信历史ThomasWillis(1621-1675)1672Firstwrittendescription重症肌无力MyastheniaGravis(MG)AChR-Ab介导的、细胞免疫及补体参与的一种NMJ处传递障碍的自身免疫性疾病。病变主要累及NM突触后膜上的acetylcholinereceptor(AChR)。机制胸腺病毒或其他非特异性因子感染胸腺肌样细胞表面的AchR构型发生变化启动免疫系统AchR―Ab产生与AchR发生应答使AchR大量破坏?突触后膜传递障碍肌肉无力免疫反应引起MG的机制1980年Drachman
2、提出抗体介导的免疫反应可能通过三个方面的机制影响正常神经肌肉传递。自体免疫反应的证据1983年Seybold等在80%的MG患者检测到了AchR1985年Destron等证实了AchR抗体存在于MG患者的NMJ处。临床诊断流行病学临床表现诊断鉴别诊断严重程度分型病因分型定量评分(QMGscore)改良Osserman分型MGFA临床分型Osserman分型gradeIocularinvolvement;gradeIIAmildgeneralizedocularMG;gradeIIBmoderategeneralizedMGinvolvingbulbarmusculature;grad
3、eIIIacutefulminantform;gradeIVseverelatemyasthenia.MGFA分型ClassⅠAnyocularmuscleweaknessMayhaveweaknessofeyeclosureAllothermusclestrengthisnormalClassⅡMildweaknessaffectingotherthanocularmusclesMayalsohaveocularmuscleweaknessofanyseverityⅡaPredominantlyaffectinglimb,axialmuscles,orbothMayalsohave
4、lesserinvolvementoforopharyngealmusclesⅡbPredominantlyaffectingoropharyngeal,respiratorymuscles,orbothMayalsohavelesserorequalinvolvementoflimb,axialmuscles,orbothClassⅢModerateweaknessaffectingotherthanocularmusclesMayalsohaveocularmuscleweaknessofanyseverityⅢaPredominantlyaffectinglimb,axialm
5、uscles,orbothMayalsohavelesserinvolvementoforopharyngealmusclesⅢbPredominantlyaffectingoropharyngeal,respiratorymuscles,orbothMayalsohavelesserorequalinvolvementoflimb,axialmuscles,orbothClassⅣSevereweaknessaffectingotherthanocularmusclesMayalsohaveocularmuscleweaknessofanyseverityⅣaPredominant
6、lyaffectinglimband/oraxialmusclesMayalsohavelesserinvolvementoforopharyngealmusclesⅣbPredominantlyaffectingoropharyngeal,respiratorymuscles,orbothMayalsohavelesserorequalinvolvementoflimb,axialmuscles,orbothClassVDefinedbyintubation,withorwithoutmechanicalventilation,exceptwhenemployedduringrou
7、tinepostoperativemanagement.TheuseofafeedingtubewithoutintubationplacesthepatientinclassIVb治疗对症治疗ChEI免疫针对性治疗长效免疫治疗激素其他免疫抑制剂胸腺切除术短效免疫治疗免疫球蛋白血浆置换对症治疗ChEI治疗1934年MaryWalker首先报道了针对MG的特效药物Prostigmin(Neostigmine)。现广泛应用溴吡斯的明。根据MG治疗指南,任何有症