婴儿期起病的自身免疫性重症肌无力临床研究  

Clinical research of infantile-onset autoimmune myasthenia gravis

作　　者：张炜华[1] 方方[1] 吴沪生[1] 吕俊兰[1] 丁昌红[1] 肖静[1] 陈春红[1] 王旭[1] 金洪[1] 韩彤立[1] 王晓慧[1] 伍云[1] 李久伟[1] 王红梅[1] 杨欣英[1] 刘丽英[1] 

机构地区：[1]首都医科大学附属北京儿童医院神经科,北京100056

出　　处：《中国实用儿科杂志》2014年第10期776-780,共5页Chinese Journal of Practical Pediatrics

摘　　要：目的总结分析婴儿期起病的自身免疫性重症肌无力临床特点。方法首都医科大学附属北京儿童医院神经内科2006年6月至2012年2月收治的婴儿期起病的自身免疫性重症肌无力患儿16例,对其临床表现、实验室检查及预后进行回顾性总结。并将其临床分型和发生肌无力危象情况与同期住院的1岁以上重症肌无力患儿338例进行比较。结果发病年龄平均(7.6±2.8)个月;首诊时病程4d至6个月。主诉症状主要包括眼睑下垂、精神差以及进食困难等,其中首诊误诊为中枢神经系统感染4例,消化系统疾病1例,误诊率31.25%。眼肌型4例(25.0%),全身型12例(75.0%),全身型所占比例较1岁以上组(17.5%)高(χ2=23.83,P<0.01)。发生肌无力危象4例(25.0%),与1岁以上组(2.1%)比较,差异有统计学意义(χ2=12.04,P<0.01)。新斯的明实验阳性率93.7%,乙酰胆碱受体抗体阳性率为57.0%,神经低频重复电刺激阳性率50.0%,眼肌型与全身型比较,各指标阳性率差异均无统计学意义(P>0.05)。16例接受溴吡斯的明治疗,15例正规应用肾上腺糖皮质激素治疗,1例应用6 d后出现肌无力危象。糖皮质激素应用后平均(6.8±5.6)d好转。对其中10例随访,随访时间为1年1个月至5年3个月,其中完全缓解3例、药物缓解1例、轻度异常4例,改善和无效各1例。结论婴儿期起病的自身免疫性重症肌无力以全身型为主,起病症状不典型,易误诊,易发肌无力危象。Abstracts： Objective To summarize the clinical characteristics of infantile-onset autoimmune myasthenia gravis and improve diagnostic skills. Methods The investigation summarized retrospectively the features including clinical manifestation, laboratory examination and the prognosis of infantile-onset autoimmune myasthenia gravis from June 2006 to February 2012 in 16 patients in Neurology Department of Beijing Children＇ s Hospital affiliated to Capital University of Medicine, and analysed the difference of the clinical classification and myasthenia crisis incidence between infatile group and the more than l year group during the corresponding period. Results Sixteen cases were revolved, including 9 boys and 7 gifts. The youngest was 4 months old, the average age was 7.6 months. The average course of disease was 1.8 months at first visiting. Main symptoms included ptosis, poor spirit and dysphagia.Among them 4 cases were misdiagn-osed as central nervous system infection and 1 as digestive system disease. Ocular and generalized type was respectively 4（25%） and 12（75%） cases; 4 （25%） cases underwent myasthenia crisis. Generalized myasthenia gravis component ratio and myasthenia crisis incidence rate of infantile-onset autoimmune myasthenia gravis was increasing significantly. Positive rate of Neostigmine test, acetylcholine receptor antibody and repetitive nerve stimulation was respectively 93.7%, 57% and 50%, and no significance was found between ocular and generalized type. All 16 cases received pyridostigmine bromide therapy; 15 cases received glucocorticoids therapy. Mean improvement time was （6.8±5.6） days after glucocorticoids therapy, but 1 of them deteriorate 6 days later; 10 chlidren was follwed-up for 1 year and 1 month to 5 years and 3 months, 3 cases were with completely stable remission （CSR）, 1 case pharmacologic remission （PR）, 4 cases were minimal manifestation （MM）, 1 case was improved （I） and 1 case was unchanged （U）. Conclusion Infantile-onset autoimmune mya

关 键 词：自身免疫 重症肌无力 婴儿 肌无力危象 

分 类 号：R72[医药卫生—儿科]