儿童重症抗N-甲基-D-天冬氨酸受体脑炎4例临床分析

发布时间：2018-03-08 15:51

本文选题：N-甲基-D-天冬氨酸受体　切入点：脑炎　出处：《临床儿科杂志》2017年06期 　论文类型：期刊论文

【摘要】：目的探讨重症抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患儿的临床特点及治疗方案。方法回顾性分析4例重症抗NMDAR脑炎患儿的临床资料以及随访情况。结果 4例患儿,男1例,女3例,年龄10~13岁,其中1例合并畸胎瘤。患儿均以精神症状和不自主运动为主要首发症状,1个月内进展为癫痫发作和意识障碍及中枢低通气性呼吸衰竭。脑脊液抗NMDAR抗体均为阳性,脑电图出现慢波,诊断时颅脑磁共振无改变。应用大剂量丙种球蛋白(IVIG)和甲基泼尼松龙冲击治疗,呼吸机治疗5~95天,其中气管切开2例,1例因感染死亡。随访21~27个月,1例临床痊愈;2例持续应用免疫抑制剂和抗癫痫药物至今,临床症状均明显改善,合并肿瘤患儿脑电图和抗NMDAR抗体持续异常,另1例复发。结论重症抗NMDAR脑炎多见于较大年龄女童,病程早期即发生中枢性低通气性呼吸衰竭,合并畸胎瘤是高危因素,常频呼吸机和反复应用激素联合IVIG治疗有效,病程长,恢复缓慢,1年后仍可出现复发。
[Abstract]:Objective to investigate the clinical characteristics and treatment of severe anti-N- methyl-Daspartic acid receptor (NMDAR) encephalitis in children. Methods the clinical data and follow-up data of 4 children with severe anti NMDAR encephalitis were retrospectively analyzed. Three women, aged 10 to 13, Among them, one case was complicated with teratoma. The main symptoms were mental symptoms and involuntary movement. The children developed epilepsy, disturbance of consciousness and central hypopnea respiratory failure within one month. Cerebrospinal fluid anti NMDAR antibody was positive. There were slow waves in EEG and no change in MRI at the time of diagnosis. The patients were treated with high dose gamma globulin (IVIGG) and methylprednisolone for 5 to 95 days. One case died of infection after tracheotomy, and 1 case was cured from 21 to 27 months, 2 cases were treated with immunosuppressant and antiepileptic drug, the clinical symptoms were improved obviously, EEG and anti NMDAR antibody were abnormal in children with tumor. Conclusion severe anti NMDAR encephalitis is more common in older girls. Central hypopnea respiratory failure occurs in the early stage of the disease. Teratoma is a high risk factor. Frequent ventilator and repeated use of hormone combined with IVIG are effective. The course of disease is long, the recovery is slow, after 1 year still can appear relapse.
【作者单位】：青岛大学附属医院儿童医学中心;首都医科大学附属北京儿童医院;
【分类号】：R742.9

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