摘要
目的探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患儿的临床症状、诊断、治疗及预后,以期提高对该病的认识。方法选择2014年6月—2016年6月在湖南省儿童医院神经一科确诊的11例抗NMDAR脑炎患儿的临床症状、辅助检查、治疗方法及随访情况进行回顾性分析。结果 11例抗NMDAR脑炎患儿中7例以惊厥为首发症状,4例以精神症状为首发症状。11例均发生过不同程度的精神症状,10例发生过意识障碍,10例发生过不自主运动,9例发生过语言障碍,9例发生过睡眠障碍,8例发生过惊厥,6例发生过自主神经功能障碍。10例脑电图异常,以背景节律慢化为主要表现,可夹杂局限性痫性放电;10例颅脑MRI正常或脑沟裂增宽加深;10例脑脊液抗NMDAR抗体阳性,3例血清抗NMDAR抗体阳性。11例患儿均接受静脉用甲泼尼龙+丙种球蛋白(IVIG)冲击治疗,其中3例联合利妥昔单抗治疗。患儿随访2个月~1年,6例临床痊愈,5例存在不同程度的神经系统后遗症。结论惊厥或精神症状、意识障碍、锥体外系症状、语言障碍与睡眠障碍等症状是抗NMDAR脑炎患儿的常见临床症状,脑脊液及血清中抗NMDAR抗体检测有助于抗NMDAR脑炎患儿的早期诊断,抗NMDAR脑炎免疫治疗有效,患儿预后较好。
Objective To explore the clinical characteristics,diagnosis,treatment and outcomes of anti- N- methyl- D- aspartate receptor（ anti- NMDAR） encephalitis in children to improve the awareness of this disease in people. Methods We conducted a retrospective analysis of the clinical characteristics,auxiliary examination findings,treatment and follow- up outcomes of 11 children with anti- NMDAR encephalitis confirmed in First Department of Neurology of Hunan Children＇s Hospital from June 2014 to June 2016. Results Of the 11 patients,7 cases had convulsion at the onset of disease,the other 4 started with psychiatric symptoms. Psychiatric symptoms occurred in all the patients, specifically, 10 cases exhibited impaired consciousness,10 cases had dyskinesias,9 cases had speech disorders,9 cases had developed sleep disorders,8 cases had convulsion,and 6 cases had dysautonomia. EEG abnormalities occurred in 10 cases,with slowed background rhythm as the main performance,probably mixed with limited epileptic discharge. The results of MRI examination were normal or broadening and deepening in cerebral sulcus and fissure in 10 cases. Specific anti- NMDAR antibody was positive in cerebrospinal fluid in 10 cases,and it was positive in serum in 3 cases. All patients received pulse therapy with intravenous immunoglobulin（ IVIG） and methylprednisolone,of them,3 cases received combined therapy with rituximab additionally. Results of the follow- ups lasting for 2 to 12 months showed that,6 patients had clinical recovery,and 5 cases were found with varying degrees of neurological sequelae. Conclusion Convulsions or psychiatric symptoms, impaired consciousness, extrapyramidal symptoms, language disorders and sleep disorders are the common symptoms of anti- NMDAR encephalitis in children. Detecting the anti- NMDAR antibody in the serum and cerebrospinal fluid may help the diagnosis of anti- NMDAR encephalitis in children as early as possible. In treating this disease,immunotherapy proves effective,and the prognosis of anti- NMDAR
作者
康庆云
杨理明
陈波
张洁
宁泽淑
KANG Qing - yun YANG Li - ming CHEN Bo ZHANG Jie NING Ze-shu(First Department of Neurology, Hunan Children＇s Hospital, Changsha 410007, China)
出处
《中国全科医学》
CAS
北大核心
2017年第9期1117-1121,1126,共6页
Chinese General Practice
