眼肌型重症肌无力患者的临床特点及转归分析

Clinical feature and prognostic analysis on ocular myasthenia gravis patients

目的分析以单纯眼肌无力起病的重症肌无力(MG)患者的临床特点及其临床转归。方法回顾性分析2008年6月至2015年6月首都医科大学附属北京友谊医院收治的以单纯眼肌无力起病的96例MG患者的临床资料,包括性别、年龄、首发症状、其他自身免疫性疾病伴发情况、胸腺异常及疾病进展情况。结果 96例MG患者的男女比例为1. 18︰1,起病年龄男女无显著差异。最常见的首发症状为眼睑下垂,其次为复视。男女患者合并其他自身免疫病的比率分别为40. 4%和45. 5%,无显著差异(P> 0. 05)。30. 2%(29例)的患者合并胸腺异常。44例患者完成2年时间的随访,其中35例(79. 5%)患者进展为全身型,病情进展主要发生在发病前3个月内,9例(20. 5%)患者随访2年时仍为眼肌型。结论眼肌型MG首发症状以眼睑下垂最常见,三分之一患者合并胸腺异常,80%患者2年内进展为全身型MG,这些特征对于眼肌型MG的诊治和转归具有重要的临床指导意义。

Objective To analyze the clinical features and outcomes of myasthenia gravis patients with symptoms of ocular muscle weakness as the first manifestation.Methods A retrospective analysis of the clinical data of 96 ocular MG patients admitted to Beijing Friendship Hospital,Capital Medical University,during June 2008 to June 2015,including gender,age,first symptom,other autoimmune diseases,thymus abnormalities and disease progression.Results The ratio of male to female in 96 patients was 1.18:1.There was no significant difference in onset age between males and females.The most common first symptom was drooping eyelids,followed by diplopia.The proportion of male and female ocular MG patients with other autoimmune diseases was 40.4%and 45.5%,respectively,and there was no difference in males and females.30.2%of MG patients had thymic abnormalities.Forty-four patients were followed up for 2 years.Of them,35(79.5%)patients progressed to generalized MG.The disease progressed mainly within 3 months after onset,and 9 patients(20.5%)kept to be ocular type when they were followed for 2 years.Conclusion The most common first symptom of ocular MG was eyelid ptosis,one third of patients with thymus abnormalities,and 80%developed generalized MG within 2 years.These characteristics have important clinical guiding significance for the diagnosis and treatment of ocular MG.