眼肌型重症肌无力临床分析  

Clinical analysis of ocular myasthenia gravis

作　　者：马晓伟[1] 杨丽[1] 杨春生[1] 张大启[1] 翟翚[1] 程焱[1] 

机构地区：[1]天津医科大学总医院神经内科,天津市神经病学研究所神经生化室,300052

出　　处：《中国现代神经疾病杂志》2011年第1期88-92,共5页Chinese Journal of Contemporary Neurology and Neurosurgery

摘　　要：目的总结分析眼肌型重症肌无力患者的临床特征,以为诊断和治疗提供参考依据。方法回顾性分析113例眼肌型重症肌无力患者的临床资料。采用免疫荧光细胞染色方法检测血清乙酰胆碱受体(AChR)抗体和肌肉特异性受体酪氨酸激酶(MuSK)抗体表达水平,分析这两项免疫学指标对眼肌型重症肌无力向全身型转化的预测价值。结果成年发病的眼肌型重症肌无力好发于40岁以上男性,多以眼睑下垂(95例,84.07%)为首发症状,少数以复视(18例,1 5.93%)起病。疲劳试验和新斯的明试验阳性率分别为79.44%(85/107)和84.85%(84/99),低频重复神经电刺激和血清甲状腺抗体异常率分别为44.32%(39/88)和28%(14/50),胸腺增生和胸腺瘤阳性率分别为16.67%(17/102)和11.76%(12/102);血清AChR抗体阳性率为62.83%(71/113);但MuSK抗体均呈阴性。眼肌型重症肌无力向全身型转化率为12.39%(14/113),其中血清AChR抗体强阳性者(13例,28.26%)显著高于弱阳性者(1例,4%),二者差异有统计学意义(X^2=4.587,P=0.032)。结论成年发病的眼肌型重症肌无力好发于中年以上男性,主要表现为眼睑下垂和复视,大多数患者伴发胸腺和甲状腺异常。血清AChR抗体表达水平升高预示向全身型转化率升高,鲜有MuSK抗体阳性反应。Objective To study clinical characteristics of ocular myasthenia gravis （OMG） to provide evidence for diagnosis and treatment of this disease. Methods Clinical data of 113 OMG patients were retrospectively analysed. Immunofluoresence cell staining was applied to detect the acetylcholine receptor （AChR） antibody and muscle- specific receptor tyrosine kinase （MUSK） antibody, and their predictive value for developing to generalized myasthenia gravis （MG） was assessed. Results Adult-onset of OMG was often happened in males over age 40. The most common initial presentation was ptosis （n = 95, 84.07% ）, and a few started with diplopia （n = 18, 15.93% ）. The positive ratio of fatigue test and neostigmine test was 79,44% （85/107） and 84.85% （84/99） respectively, abnormal rate of repetitive nerve stimulation （RNS） and serum thyroid antibodies was 44.32% （39/88） and 28% （14/50） respectively. The incidence of thymic hyperplasia and thymoma was 16.67% （17/102） and 11.76% （12/102）, respectively. The positive rate of serum AChR antibody was 62.83% （71/113）, but the serum MuSK antibody was negative in all patients. There were 12.39% （14/113） OMG patients progressed to generalized MG. Patients with higher AChR antibody expression were with more risk to progress to generalized MG （X2= 4.587, P = 0.032）. Conclusion Adult- onset OMG often begins in middle-aged males. The main manifestation of OMG are ptosis and diplopia. Most patients are associated with abnormality of thymus and thyroid. Patients with higher AChR antibody expression are more at risk for progressing to generalized MG. MuSK antibody is uncommon in OMG.

关 键 词：重症肌无力 眼肌麻痹 受体 胆碱能 电刺激 荧光抗体技术 

分 类 号：R746.1[医药卫生—神经病学与精神病学]