儿童抗N-甲基-D-天冬氨酸受体脑炎90例临床特点分析

Clinical characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children: an analysis of 90 cases

摘要:

目的：研究儿童抗N-甲基-D-天门冬氨酸受体（N-methyl-D-aspartate receptor encephalitis，NMDAR）脑炎的临床特点、疗效及预后，提高对该病的认识。方法：回顾性分析重庆医科大学附属儿童医院2012年8月至2018年1月确诊的90例抗NMDAR脑炎患者临床资料。结果：90例中男性38例（42.2%），女性52例（57.8%），年龄8个月至13岁。首发症状以惊厥发作（47.8%）、精神行为异常（33.3%）最常见。常见临床症状依次为运动障碍81例（90%），精神行为异常70例（77.8%），惊厥发作58例（64.4%），睡眠障碍51例（56.7%），认知功能障碍35例（38.9%）。所有患者脑脊液抗NMDAR抗体均阳性，90%以上出现脑电图异常，约50%头颅磁共振（magnetic resonance imaging，MRI）肿瘤筛查阴性。74例（82.2%）给予丙种球蛋白（intravenous im－munoglobulin，IVIG）及甲基泼尼松龙治疗，8例（8.9%）仅予IVIG治疗，2例（2.2%）仅予甲基泼尼松龙治疗，2例（2.2%）给予血浆置换治疗，无缓解及复发的3例患者中分别给予利妥昔单抗、吗替麦考酚酯、硫唑嘌呤治疗。随访2~52个月，94.3%预后良好，3.2%死亡，23.4%复发。结论：抗NMDAR脑炎临床症状多样，部分临床症状可能与疾病严重程度有关，脑脊液抗NMDAR抗体阳性可确诊该病，儿童抗NMDAR脑炎肿瘤发生率低，早期诊断和及时免疫治疗可改善预后。

Abstract:

Objective：To investigate the clinical characteristics，treatment outcome，and prognosis of anti-N-methyl-D-aspartate re－ceptor（NMDAR） encephalitis in children，and to improve the understanding of the disease. Methods：The clinical data of 90 children with anti-NMDAR encephalitis diagnosed in Children’s Hospital of Chongqing Medical University from August 2012 to January 2018 were retrospectively analyzed. Results：Of the 90 children aged from 8 months to 13 years，38（42.2%） were males，and 52（57.8%） were females. The first symptoms of the disease were seizure（47.8%） and psychiatric disorders（33.3%），and the most common clinical symptoms were dyskinesia in 81 cases（90%），psychiatric disorders in 70 cases（77.8%），seizure in 58 cases（64.4%），sleep disorders in 51 cases（56.7%），and cognitive dysfunction in 35 cases（38.9%）. Anti-NMDAR antibodies in cerebrospinal fluid were positive in all patients，more than 90% of the patients had abnormal electroencephalogram，about 50% had abnormal brain magnetic resonance imaging，and tumor screening was negative in all patients. Seventy-four patients（82.2%） were treated with intravenous immunoglobulin（IVIG） and methylprednisolone，8 patients（8.9%） were treated with IVIG only，2 patients（2.2%） were treated with methylpred－nisolone only，2 patients（2.2%） received plasma exchange treatment，and 3 patients with recurrence and no remission received ritux－imab，mycophenolate mofetil，and azathioprine. After 2 to 52 months of follow-up，94.3% of the patients had a good prognosis，3.2% died，and 23.4% relapsed. Conclusion：Anti-NMDAR encephalitis has various clinical symptoms，and some clinical symptoms may be associated with the severity of the disease. The positive finding of anti-NMDAR antibody in cerebrospinal fluid can confirm the diag－nosis of the disease. The incidence of tumor in children with anti-NMDAR encephalitis is low. Early diagnosis and timely immunotherapy can improve prognosis.