眼肌型重症肌无力临床特征及转归分析——单中心研究

Clinical characteristics and outcome of patients with ocular myasthenia gravis

目的分析眼肌型重症肌无力患者的临床特征、治疗及其转归。方法单中心回顾性分析2022年5月—2023年5月期间就诊于复旦大学附属眼耳鼻喉科医院神经眼科门诊并诊断为眼肌型重症肌无力患者的临床资料,对其人口学特征、发病特征、临床表现、治疗及转归进行分析。结果共收集40例确诊为眼肌型重症肌无力患者,其中男性27例、女性13例。平均年龄为(50.45±18.03)岁(4~89岁),平均病程为(6.72±17.35)个月(0.25~72个月),平均随访时间为(14.00±2.63)个月(12~20个月)。单眼患者25例、双眼患者15例。单纯表现为眼睑下垂患者8例(20.00%)、单纯表现为眼肌麻痹患者5例(12.50%)、二者均有者27例(67.50%)。疲劳试验、冰试验、血清肌无力相关抗体阳性率分别为88.46%、82.35%及46.15%。甲状腺功能异常者为73.68%。34例患者进行了胸部CT检查,2例(5.88%)患者确诊胸腺瘤,并进行了手术切除,3例(8.82%)患者胸腺增生,未行手术。39例患者分别行如下4种治疗,单用溴吡斯的明、单用泼尼松、联合应用溴吡斯的明与泼尼松、联合应用泼尼松与非糖皮质激素类免疫抑制剂的患者各为1例、14例、12例及12例,平均用药时间为(13.00±7.63)个月(1~20个月),用药后达缓解或最轻临床表现状态的时间平均为(4.14±3.66)个月(0.25~12个月)。至随访结束,症状缓解或最轻临床表现状态者27例(67.50%),13例(32.50%)患者部分好转,症状轻微且维持稳定,未有患者转化为全身型重症肌无力。随访期间,29例(72.50%)患者仅发作1次,11例(27.50%)患者发生1次及以上复发。各治疗组间症状改善及复发状况差异无统计学意义。结论眼肌型重症肌无力为导致上睑下垂及复视的重要原因,疲劳试验、冰试验对于眼肌型重症肌无力的确诊具有重要意义,其阳性率明显高于血清肌无力抗体阳性率。经过免疫抑制治疗,患者总体复发率较低,并且未观察到向全身型重症肌无力的转化趋势。

Objective To evaluate the clinical characteristics,treatment and outcome of patients diagnosed with ocular myasthenia gravis.Methods Cases review study.Outpatients with ocular myasthenia gravis(OMG)presented to neuro-ophthalmology division between May 2022 and May 2023 were collected.The demographic data along with ophthalmologic examinations,were evaluated.Results A total of 40 patients(27 males and 13 females)with a mean age of(50.45±18.03)years(range:4-89)were included in the study.The mean course between presenting and final diagnosis was(6.72±17.35)months(range:0.25-72).The mean follow-up was(14.00±2.63)months(range:12-20).Monocular involvement occurred in 25 cases and binocular in 15 cases.Isolated ptosis was found in 8 cases(20.00%),isolated extraocular muscle paralysis in 5 cases(12.50%)and 27 cases(67.50%)had both manifestations.The positive rates for the fatigue test,ice test and AChR antibody testing were 88.46%,82.35%and 46.15%respectively.Thyroid dysfunction was found in 14 patients(73.68%,14/19).Chest CT scans revealed thymoma in 2 cases who subsequently underwent thymectomy and thymic hyperplasia in 3 cases.Thirty-nine patients accepted medical treatment,including 1 case with pyridostigmine bromide,14 cases with prednisone,12 cases with pyridostigmine bromide combined with prednisone and 12 cases with prednisone combined with non-steroidal immunosuppressants.The mean medication time was(13.00±7.63)months(range:1-20).After an average treatment period of(4.14±3.66)months(range:0.25-12),27 patients(67.50%)acquired remission or minimal manifestation status and 13 patients(32.50%)showed improvement.There was no case progressing to generalized MG.Twenty-nine cases(72.50%)had only one episode and 11 cases(27.50%)relapsed once or more during the period of follow-up.Conclusions OMG is a common cause leading to ptosis and diplopia.The fatigue test and ice test show higher positive rates compared to serum antibody tests and therefore have important diagnosis value for OMG.The immunosuppressive therapy,to a certain extent,reduces both recurrence risk and progression to generalized MG.