摘要
目的分析青少年起病的重症肌无力(MG)患者的临床特点。方法回顾性分析2006-2013年作者医院神经内科住院的351例起病年龄≤18岁MG患者的临床资料,包括人口统计学(性别、年龄),临床特点(临床分型、起病症状、疾病转归、胸腺情况)和合并疾病(自身免疫性疾病)。结果青少年起病的MG患者发病年龄小于5岁者占45.9%,女性多见占53.6%。根据美国重症肌无力协会(myasthenia gravis foundation of America,MGFA)分型,Ⅰ型占80.3%,Ⅱa型占8.5%,Ⅱb型占9.1%,Ⅲa型占0.3%,Ⅲb型占1.4%,Ⅳb型占0.3%。由眼肌型MG(OMG)转化为全身型MG(GMG)患者39例(13.8%),主要诱发转化的因素为不规律用药或停药。诱发病情复发或加重的因素中以受凉多见,占63.6%。合并胸腺异常患者有60例,其中28例行胸腺切除术,术后病理分型:胸腺增生20例,胸腺瘤4例。合并有其他自身免疫性疾病共20例,其中甲状腺功能亢进占4.6%,甲状腺功能减低占0.6%,甲状腺囊肿占0.6%。共有6例患者发生过危象,其中5例发生肌无力危象者为由OMG转化为GMG患者。结论青少年MG患者有自己独特的临床特点:5岁之前发病更多见;诱发病情复发或加重的因素中以受凉多见;合并胸腺增生者多于合并胸腺瘤者;最常见的甲状腺功能异常为甲状腺功能亢进。
Objective To analyze the clinical features of juvenile onset myasthenia gravis（MG）patients.Methods Retrospective analysis was adopted to investigate the clinical data of MG patients from inpatient department of neurology whose onset ages were not over 18 years old.The patients were admitted from2006 to 2013.Those data included demographics（gender and age）,clinical features（clinical classification,onset symptoms,the transformation of MG,thymus status）and accompanying diseases（autoimmune diseases）.Results The results showed that juvenile MG patients whose onset age was less than 5years old accounted for45.9%,and more common in women（accounted for 53.6%）.According to the Myasthenia Gravis Foundation of America（MGFA）classification,type Ⅰaccounted for 80.3%,typeⅡa accounted for 8.5%,typeⅡb accounted for 9.1%,typeⅢa accounted for 0.3%,type Ⅲb accounted for 1.4%,and type Ⅳb accounted for0.3%.Thirty-nine patients progressed from OMG to GMG which accounted for 13.8%,and the major inducing factor of progression was irregular medication or drug withdrawal.The most common predisposing factor was catching cold,accounted for 63.6%.Sixty patients combined with thymic abnormalities and 28 patients of them undergone thymectomy.As for the pathological types after thymectomy,there were 20 patients with thymic hyperplasia, 4 patients with thymoma. Twenty patients combined with other autoimmune disease,hyperthyroidism accounted for 4.6%,hypothyroidism accounted for 0.6%,thyroid cyst accounted for 0.6%,2patients combined with diabetes mellitus.Six patients experienced myasthenic crisis and 5of them were patients progressed from OMG to GMG.Conclusions The juvenile MG patients have their own unique clinical features as follows：onset before 5years old was more common,the most common predisposing factor was catching cold,it is more common of patients accompanying with thymic hyperplasia than accompanying with thymoma,the most common thyroid disorder was hyperthyroidism.
出处
《中国神经免疫学和神经病学杂志》
CAS
2016年第1期5-8,13,共5页
Chinese Journal of Neuroimmunology and Neurology
关键词
重症肌无力
青少年
临床特点
myasthenia gravis
adolescent
clinical manifestations
