摘要
目的探讨儿童抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点、治疗方法、预后及复发特点。方法回顾性分析长治医学院附属和济医院2016年6月至2019年6月诊治的抗NMDAR脑炎患儿20例的临床资料,分析其临床症状、辅助检查结果、合并肿瘤情况、治疗方案、复发情况及预后。结果女17例,男3例,发病年龄为(5.9±3.5)岁,呈急性或亚急性起病,首发症状抽搐12例。临床症状:精神行为异常18例,运动障碍18例,癫痫发作17例,语言障碍17例。均未发现肿瘤。脑电图均异常,4例可见δ刷,3例为复发病例。头颅核磁共振未见明显异常4例,异常16例。复发病例8例,单次复发7例,3次复发1例。17例采用利妥昔单抗治疗。20例均好转,2例遗留癫痫,1例左侧偏瘫。结论抗NMDAR脑炎在女性患儿中发病率较高,儿童各年龄段均可发病,首发症状以抽搐最常见。常见的临床症状依次为精神行为异常、运动障碍和癫痫发作、语言障碍,肿瘤合并率低,脑电图异常δ刷对疾病严重度有提示意义。抗NMDAR脑炎复发率较高,以未使用二线免疫治疗的较高。利妥昔单抗对一线免疫治疗效果欠佳病例及复发病例均具有较好疗效。
Objective To investigate the clinical characteristics,treatment methods,prognosis and relapse of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis in children.Methods The clinical data of 20 children with NMDAR encephalitis treated in Heji Hospital Affiliated to Changzhi Medical College of Shanxi Province from June 2016 to June 2019 were retrospectively analyzed.The clinical symptoms and auxiliary examination results,combination of tumor,treatment,relapse and prognosis were analyzed.Results There were 17 females,3 males,the age of onset was(5.9±3.5)years,which presented acute or subacute onset,the first symptom was convulsion in 12 cases.Clinical symptoms:18 cases of abnormal mental behavior,18 cases of dyskinesia,17 cases of epileptic seizure and 17 cases of language barrier.No tumor was found.The electroencephalogram was abnormal,the delta brush was visible in 4 cases,and 3 cases were recurrent cases,cranial MRI showed no significant abnormalities in 4 cases and abnormal in 16 cases.There were 8 cases of recurrence,7 cases of single recurrence and 1 case of 3 recurrence.These 17 cases were treated with Rituximab.All 20 cases were improved,2 cases with left epilepsy,1 case with left hemiplegia.Conclusion Anti-NMDAR encephalitis has a high incidence in female children and can occur in children of all ages.Convulsions are the most common first symptom.The common clinical symptoms are in order:mental behavior abnormalities,dyskinesia,seizures,and language barrier,the tumor merger rate is low,the abnormal delta brush is significant for disease severity.The recurrence rate of anti-NMDAR encephalitis is high,those who did not use second-line immunotherapy are higher.Rituximab is effective for patients with poor first-line immunotherapy and patients with recurrence.
作者
宋瑞娟
Song Ruijuan(Department of Pediatrics,Heji Hospital Affiliated to Changzhi Medical College,Changzhi,Shanxi 046000,China)
出处
《中国基层医药》
CAS
2020年第22期2713-2716,共4页
Chinese Journal of Primary Medicine and Pharmacy
基金
长治医学院科技启动基金项目(QDZ201618)。
关键词
脑炎
自身免疫
N-甲基-D-天冬氨酸受体
儿童
临床特点
免疫治疗
预后
复发
影响因素
Encephalitis
Autoimmunity
N-Methyl-D-Aspartate receptor
Child
Clinical characteristics
Immunotherapy
Prognosis
Relapse
Influence factor
