84例重症肌无力患儿临床分析与随访

Clinical analysis and follow-up of 84children with myasthenia gravis

目的分析84例重症肌无力患儿的临床特点,并对其进行随访。方法收集2012年1月至2014年12月湖南省儿童医院神经二科收治重症肌无力患儿84例作为研究对象,对其临床资料进行详细分析。结果 84例患儿中眼肌型(Ⅰ型)66例(78.57%),全身型(Ⅱ型)10例(11.90%),重症激进型(Ⅲ型)5例(5.95%),迟发重症型(Ⅳ型)3例(3.57%);3岁之前发病40例(47.62%),3~7岁发病25例(29.76%),7岁以上发病19例(22.62%);发病前出现上呼吸道感染15例(17.86%)。随访期间有1例患儿因环磷酰胺疗效不好,转外院治疗,随访期间死亡,一共复发44例,复发1~2次36例(81.82%),复发3~5次6例(13.64%),复发>5次2例(4.55%)。采取激素治疗效果显著,随访中有1例患儿死亡。结论重症肌无力患儿中眼肌型较多,死亡率较低,预后较为良好。

Objective To analyze the clinical characteristics of 84 children with myasthenia gravis and to make follow-up.Methods From January 2012 to December 2014, 84 cases of myasthenia gravis in two Department of Neurology in Hunan Children＇s Hospital were collected as the research subjects, and the clinical data were analyzed in detail.Results There were 66 cases（78.57%） of musculus-ocularis type（type Ⅰ） in 84 cases, 10 cases（11.90%） of systematic type（type Ⅱ）, 5 cases（5.95%） of severe-radical type（type Ⅲ）, and 3 cases（3.57%） of late-severe type（type Ⅳ）; 40 cases（47.62%） had onset before 3 years of age,25 cases between 3 and 7（29.76%） and 19 cases（22.62%） over 7 years old. There were 15 cases（17.86%） of upper respiratory tract infection before onset. During the follow-up period, 1 case who responded poorly to cyclophosphamide was sent to another hospital for treatment and died during follow-up. There were 44 cases of recurrence, 1～2 times of recurrence in 36 cases（81.82%）, 3～5 times in 6 cases（13.64%）, and over 5 times in 2 cases（4.55%）. Hormone therapy was effective and 1 child died during the follow-up. Conclusion Myasthenia gravis children are more of musculus-ocularis type, with lower mortality and better prognosis