AChR及MuSK双抗体阳性重症肌无力三例并文献复习  

Three AChR-Ab and MuSK-Ab double-seropositive myasthenia gravis patients and review of literature

作　　者：母艳蕾[1] 张华[1] 国红[1] 侯世芳[1] 殷剑[1] 蒋云[1] 赵鸿[2] MU Yanlei;ZHANG Hua;GUO Hong;HOU Shifang;YIN Jian;JIANG Yun;ZHAO Hong(Department of Neurology of Beijing Hospital,National Center for Gerontology,Beijing 100073)

机构地区：[1]北京医院神经内科国家老年医学中心,100073 [2]北京大学第三医院急诊科,100191

出　　处：《中国神经免疫学和神经病学杂志》2018年第2期80-86,共7页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的报道3例乙酰胆碱受体(AChR)抗体和肌肉特异性酪氨酸激酶(MuSK)抗体双抗体阳性重症肌无力(myasthenia gravis,MG)患者的临床特点、诊疗和预后,并复习既往相关文献,以提高对这一特殊MG类型的认识。方法对作者医院3例AChR及MuSK双抗体阳性的确诊MG病例进行报道,并检索近20年Medline和万方中文数据库,选择国内外报道的上述双抗体阳性的确诊MG病例中包含临床特点、实验室检查及诊疗结果的文章,结合作者医院病例分析和总结AChR及MuSK双抗体阳性MG的临床特点及诊治情况。结果8篇文献中的7例MG,包括作者医院的3例,共10例AChR及MuSK双抗体阳性MG纳入分析,男4例、女6例,平均起病年龄(45.5±24.1)岁,起病症状包括眼睑下垂到呼吸困难轻重不等,受累肌群可包括眼外肌(9/10)、球部肌肉(9/10)、颈肌(7/10)、四肢肌(8/10)和呼吸肌(6/10),肌无力危象发生率高(5/10)。9例行肌电图检查,8例重复电刺激(RNS)阳性,4例行单纤维肌电图(SFEMG)检测者均为阳性。双抗体阳性可在发病之初即为双阳性,也可在AChR抗体阳性基础上随着病情进展而发展形成,多在病情加重时出现,病情好转后亦可出现抗体转阴。溴吡斯的明+免疫抑制剂治疗对多数患者有效(8例中4例有效、4例部分有效);4例患者肌无力危象时使用血浆置换(PE)、2例重症患者使用利妥昔单抗治疗均有效,2例患者使用环孢素治疗,其中1例有效,2例患者硫唑嘌呤治疗均无改善。结论双抗体阳性MG患者任何年龄均可发病,女性居多,总体受累范围广,病情重,免疫抑制剂治疗个体差异大,PE对于危象效果好,双抗体表达情况随病情动态波动。Objective To analyze the clinical features,diagnosis and management of double seropositive myasthenia gravis(MG),which means both antibodies to acetylcholine receptors(AChR-Ab)and muscle specific tyrosine kinase(MuSK-Ab)are positive.The data of our three patients and cases which have been reported were analyzed together for making a full understanding of this rare condition.Methods A search was conducted for papers published on Medline and Wanfang database in the recent 20 years to study cases on double seropositive MG in terms of clinical features,electrophysiology and laboratory tests,management and prognosis.Data of our three patients and previous reported cases were analyzed together.Results Eight papers reporting seven patients met the inclusion criteria.In total ten patients were analyzed,including four males and six females,mean age(45.5土24.1)yeavs.The onset symptoms varied form,ptosis,diplopia to dyspnea.The involved muscles included extra ocular muscles(9/10),bulbar muscles(9/10),proximal limbs(8/10),neck extensor(7/10)and respiratory muscles(6/10).The incidence of myasthenic crisis was as high as 50%.9 patients underwent electromyography(EMG),the positive rate of repetitive nerve stimulation(RNS)of was 88.9%,the positive rate of single fiber EMG(SFEMG)was 100%.The double positive antibodies could not only be identified in the deterioration phase of anti-AChR positive MG,but also identified the first examination.The patients were often responsive to the treatments of pyridostigmine and immunosupressive drugs,and patientswith crisis(4/4)can be markedly improved by plasma exchange(PE),Rituximab was beneficial for severe symptoms such as dyspnea(2/2),cyclosporine was partial beneficial in 1 of 2 and not responsive in the rest,azathioprine was not responsive to all.The double positive antibodies might change during remission.Conclusions The double seropositive MG can be involved at any age,with a female dominance,and generalized muscle involvement,and are more likely to be in severe conditions.The benefit

关 键 词：重症肌无力 乙酰胆碱受体抗体 肌肉特异性酪氨酸激酶抗体 血清双抗体阳性 

分 类 号：R746.1[医药卫生—神经病学与精神病学]