中国实用儿科杂志

中国实用儿科杂志

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婴儿期起病的自身免疫性重症肌无力临床研究

张炜华，方方，吴沪生，吕俊兰，丁昌红，肖静，陈春红，王旭，金洪，韩彤立，王晓慧，伍云，李久伟，王红梅，杨欣英，刘丽英   

  1. 作者单位： 首都医科大学附属北京儿童医院神经科， 北京 100056
  • 出版日期:2014-10-06 发布日期:2014-10-08
  • 通讯作者: 方方 

Clinical research of infantile-onset autoimmune myasthenia gravis.

ZHANG Wei-hua，FANG Fang，WU Hu-sheng，LÜ Jun-lan，DING Chang-hong，XIAO Jing，CHEN Chun-hong，WANG Xu，JIN Hong，HAN Tong-li，WANG Xiao-hui，WU Yun，LI Jiu-wei，WANG Hong-mei，YANG Xin-ying，LIU Li-ying.   

  1. Department of Neurology，Beijing Children’s Hospital ，Capital University of Medicine，Beijing 100056，China
  • Online:2014-10-06 Published:2014-10-08

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4 d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。

关键词: 自身免疫, 重症肌无力, 婴儿, 肌无力危象

Abstract:

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 1 year group during the corresponding period. Results Sixteen cases were revolved， including 9 boys and 7 girls. 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 misdiagnosed 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 myasthenia gravis ，mainly manifested as generalized type，cam be easily misdiagnosed because of atypical onset-symptoms， and incidence rate of myasthenia crisis is high.

Key words: autoimmune, myasthenia gravis, infantile, myasthenia crisis

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