晚发型重症肌无力临床特征研究  

Study on the clinical characteristics of late-onset myasthenia gravis

作　　者：李平[1] 钟士江[1] 张巧莲[1] 李双英[1] 

机构地区：[1]武警后勤学院附属医院神经内科,天津300162

出　　处：《武警后勤学院学报（医学版）》2017年第2期141-143,147,共4页Journal of Logistics University of PAP（Medical Sciences）

摘　　要：【目的】探讨晚发型重症肌无力(late-onset myasthenia gravis,LOMG)的临床特征。【方法】回顾性分析LOMG患者的临床资料,包括性别、年龄分布、临床分型、转归、血浆抗体的表达、有无自身免疫性疾病和其他共病的发生。记录所有LOMG患者复发加重住院的次数、使用免疫球蛋白的次数、肌无力危象发生的例数、死亡例数、基线水平和近期QMG和MMT评分。评估症状缓解后长期口服糖皮质激素和硫唑嘌呤的疗效及不良反应。【结果】LOMG患者男性18例(56.3%),高于女性;眼肌型7例(21.9%)、全身型25例(78.1%);咽喉部肌肉受累者17例(53.1%);ACh R抗体阳性率46.9%;发病最初2年复发率高,后期病情趋于稳定,危象发生率和死亡率不高;自身免疫性疾病发病率低,53.1%患者有共病存在。硫唑嘌呤与糖皮质激素比较疗效无明显差异,但硫唑嘌呤不良反应出现少。【结论】LOMG的临床表现、病情转归和预后有自身的特点,在治疗过程中应考虑到其临床特征和共病的存在而采取更合理的措施,硫唑嘌呤口服依从性更高,远期疗效可能更佳。[Objective] To investigate the clinical characteristics of late-onset myasthenia gravis （LOMG）. [ Methods ] The clinical data of LOMG cases were retrospectively analyzed, including gender, age, clinical types, prognosis, the expression of plasma antibodies and the occurrence of autoimmune diseases and other morbidities. The following was recorded, including the number of recurrent hospitalization, the times of using immunoglobulin, the cases of myasthenia crisis, dead cases, as well as baseline and recent scores of QMG and MMT. The efficacy and side effects of long-term oral glucocorticoid and azathioprine were evaluated. [ Results] There were 18 male cases（56.3%）, more than female patients, There were 7 ocular-type cases （21.9%） and 25 systemic-type cases （78.1%）. A total of 17 cases （53.1%） were involved in pharyngeal muscles. The positive rate of AChR antibody was 46.9%. The recurrence rate was high in the first 2 years, while the rates of crisis morbidity and mortality were not high in the late period when the state of this disease was stable. The incidence of autoimmune diseases was low, and 53.1% patients suffered co-morbidities. There was no obvious difference in the efficacy between azathioprine and glucocorticoid. However, the side effects of azathioprine appeared less. [ Conclusion ] LOMG is of its own characteristics in clinical manifestation and prognosis. The clinical features and co-morbidities should be considered in the LOMG treatment for more reasonable plans. Oral azathioprine is of higher compliance, and its long-term efficacy may be better.

关 键 词：重症肌无力 晚发型 硫唑嘌呤 糖皮质激素 临床特征 

分 类 号：R746.1[医药卫生—神经病学与精神病学]