我国西北地区眼肌型重症肌无力的临床特点  

Clinical features of ocular myasthenia gravis in northwest China

作　　者：党丹[1] 吴芳[1] 郭俊[1] 李宏增[1] 张慜[1] 李川[1] 汪杰[1] 李柱一[1] 

机构地区：[1]陕西省西安市第四军医大学唐都医院神经内科,710038

出　　处：《中国神经免疫学和神经病学杂志》2014年第5期344-347,351,共5页Chinese Journal of Neuroimmunology and Neurology

基　　金：国家自然科学基金项目(31200665;31270952)

摘　　要：目的 探讨我国西北地区眼肌型重症肌无力（ocular myasthenia gravis,OMG）的临床特点.方法 回顾性分析2008-06-2012-06作者医院就诊的以眼外肌受累起病的392例OMG患者的临床资料.结果 该组OMG患者男女比例为1.2∶1.OMG第一个发病高峰在0～15岁,发病人数占47.4％,男女发病均等;第二个发病高峰在50～60岁,男性占优势.首发症状以单纯上睑下垂多见（67.6％）.最常见的眼球活动受限类型是眼球固定,最常受累的眼外肌是外直肌.新斯的明试验阳性率为95.8％.低频重复频率电刺激波幅递减的总体发生率为34.3％,其中面神经的阳性率最高.OMG合并甲状腺功能异常的发生率为18.3％,以合并甲状腺功能亢进者最多.OMG合并胸腺影像学异常的发生率为20.7％,以合并胸腺瘤者最多.结论 本文总结了我国西北地区OMG的临床表型特点,充分认识其临床特点有利于更好地了解疾病的演变.Objective To investigate the clinical features of ocular myasthenia gravis （OMG） in northwest China.Methods Retrospective analysis was held on 392 patients with an initial diagnosis of OMG who were admitted to Tangdu Hospital of Fourth Military Medical University from June 2008 to June 2012.Results Among 392 OMG patients,there were 210 men and 182 women,and male-to-female ratio was 1.2 ∶ 1.Both men and women had the first peak incidence before the age of 15 （47.4％）,and a second peak incidence of the ages between 50 and 60 years.Pure ptosis was the most frequent initial symptom （67.6％）; total ophthalmoplegia was the most common type of limitation of ocular movement; lateral rectus was most frequently involved.Neostigmine test was positive in 95.8％ of the patients.Totally,repetitive nerve stimulation （RNS） decrement of amplitude was observed in 34.3％ patients,with the highest positive rate on stimulating facial nerves.There were 31 of the 169 tested OMG patients （18.3 ％） combined with abnormal thyroid function,and hyperthyroidism was the most frequently seen.There were 58 of the 280 examined OMG patients （20.7 ％） combined with thymus pathology,and thymoma was most frequently seen.Conclusions The clinical phenotype of OMG in southwest of China is reviewed.An extensive recognition of the clinical features may facilitate a better understanding of the evolution of MG.

关 键 词：重症肌无力 临床特点 眼肌型重症肌无力 

分 类 号：R746.1[医药卫生—神经病学与精神病学]