儿童抗NMDA受体脑炎患者的脑电图及临床分析

Pediatric anti-N-methyi-D-aspartate receptor encephalitis- EEG and clinical analysis in 8 patients

目的：探讨儿童抗N-甲基-D-天冬氨酸（NMDA）受体脑炎的临床及脑电图（EEG）特点，分析患者病情与EEG的关系。方法：①收集2015年7月-2018年1月临床诊断为抗NMDA受体脑炎患儿的临床资料，包括起病特点、发病年龄、治疗药物、住院时间及后遗症、病情转归及随访资料等方面；②收集患儿辅助检查资料：动态监测EEG变化及MRI等结果，自身抗体及肿瘤相关检测等。结果：①符合纳入标准者8例，其中男3例，女5例，起病年龄平均为8．75（±2．12）岁；均为急性或亚急性起病，其中4例患儿惊厥起病，7例患儿伴运动障碍，8例患儿均伴睡眠障碍及记忆缺陷；②8例患儿EEG显著异常，未发现8刷，主要表现为单侧或双侧（全面性）持续性8波，全面性8波者病程更长，预后更差。所有患儿血或脑脊液抗NMDA受体抗体阳性，均未发现肿瘤，仅1例患儿头颅MRI异常；③8例患儿入院后均予丙种球蛋白，甲基泼尼松龙免疫治疗，5例患儿因疗效差加用环磷酰胺治疗，缓解期长期口服强的松；④随访时间为15～30个月，住院期间死亡2例，余6例患儿中4例痊愈，1例明显缓解7个月后复发而再次予免疫治疗明显缓解，1例患儿（病例7）留轻度智力障碍及多动等后遗症。至最后1次随访，出院6例患儿均无癫痫发作。结论：本研究显示绝大多数抗NMDA受体脑炎患儿EEG异常，以持续性8波为特点，双侧持续性慢波者病情更重，病程更长。对于怀疑抗NMDA受体脑炎患儿，早期给予经验性免疫治疗，多数患儿预后良好。

Objective：To explore the electroencephalograph （EEG） and clinical features of children with anti-N-methyl-D-aspartate receptor （anti-NMDAR） encephalitis. Methods：Cinical and follow-up data of patients with anti-NMDAR encephalitis were collected, including symptoms and age of onset, therapy strategies, duration of hospitalization, outcome and follow-up data. Work-up data： monitor of EEG dynamically and data of tumor relative results and auto-antibody of encephalitis were analyzed. Results：8 patients with anti-NMDAR encephalitis were in conformity with the inclusion criteria, 5 female and 3 male. The average age of onset was 8.75（±2.12） years. All of the cases had acute or sub acute onset. The symptom in the initiate course of the disease was that 100% （8/8） suffered from sleep disturbance and memory deficit, and 50%（4/8） patients began seizures from convulsion. Dyskinesia was presented in 87% （7/8） patients. 100% （6/6） patients＇ EEG was abnormal, and δ-brush was not found in all patients. The mainly abnormal findings were persistent, bilateral or unilateral δ waves, patients with unilateral 3 waves have a better outcome than these with bilateral slow waves. Anti-NMDAR antibody were positive in blood or cerebrospinal fluid while all tumor related detection was negative, and only 1 pa tients＇ head MRI was abnormal. All patients were given first-line immunotherapy, and 62% （5/8） panents were given second-line immunotherapy. Duration of follow-up ranged from 15 months to 30 months, the prognosis was good in 50% （4/5） of the patientst 2 patients diedt one was relapse and one had a mild sequel with mental retardation （MR） and hyperactive disorder. All patients had not seizure attacks till the last follow-up. Conclusion, The EEG images of most children with Anti-NMDAR encephali- tis were abnormal, characterized by persistent δ waves, and children with bilateral δ waves have bad prognosis. Most patients suspected with anti-NMDAR encephalitis have a desirable outcome if early immunotherapy is given.