眼肌型重症肌无力临床分析

A clinical assessment of ocular myasthenia gravis

目的分析眼肌型重症肌无力(OMG)的临床特点及眼部征象。方法采用回顾性研究的方法,对92例OMG、40例全身型重症肌无力(GMG)的临床资料进行统计分析。结果①OMG好发于1￣24岁,17.4%有复发史,91.9%儿童重症肌无力MG表现为OMG。②OMG有95.6%表现为上睑下垂,70.5%表现为眼球活动障碍(斜视),多表现为单侧上睑下垂(57.6%),双侧上睑下垂率(38.0%)显著低于GMG(70.0%),OMG眼球垂直与水平运动障碍率分别是48.9%、43.2%,垂直与水平方向同时发生障碍者占21.6%。③OMG新斯的明试验和疲劳试验阳性率(88.0%、86.4%)与GMG相似,胸腺瘤阳性率(5.2%)、神经低频重复电刺激阳性率(23.2%)显著低于GMG。④28.6%OMG胸腺CT正常,经病理诊断为胸腺增生。结论OMG好发于青春期,病程长,易复发;表现为不对称的上睑下垂、斜视;即使胸腺CT结果正常也存在胸腺异常的可能性。

Objective To investigate clinical manifestations and ocular findings in patients with ocular myasthenia gravis (OMG). Methods Medical records of 92 patients with OMG and 40 patients with general myasthenia gravis (GMG) were reviewed retrospectively. Results ① OMG was liable to be onset at age from 1 to 24. 17.4% patients had relapsed. 91.9% childhood MG manifested as OMG. ② Ptosis was 95.6%, and strabismus was 70.5% in patients with OMG respectively. Single side ptosis was more prevalent in OMG (57.6%). In OMG, double ptosis was lower (38.0%) than in GMG (70.0%). Vertical heterotropia was in 48.9%, horizontal strabismus was in 43.2%, and the combined of both was in 21.6% of patients with OMG. ③ Sensitivity of neostigmine test (88.0%), fatigue test(86.4%) in OMG were no different from that in GMG, but thymus tumour (5.2%) and repetitive nerve stimulation (23.2%) in OMG were markedly lower than in GMG. ④ 28.6% OMG patients with a normal thymus CT were found to have a thymic hyperplasia on histology. Conclusions Onset of OMG is often at preadolescence or adolescence. Patients with OMG present as asymmetrical and multiform weakness of extraocular muscles, comparatively have a long course, and some times have one or more relapse once more. It is possible for patient with OMG presented with a normal thymus CT, but with an abnormal thymus on histology. [