OBJECTIVE To study the clinical features of children diagnosed with anti-NMDAR encephalitis in southern China. METHODS Clinical data of children diagnosed with anti-NMDAR encephalitis from October 2014 to June 2020 from one national regional medical center were analyzed. Neurological disability was assessed by modified Rankin Scale (mRS) throughout the course of disease. RESULTS 111 children (M/F = 49/62; mean onset age = 6.8 y) with anti-NMDAR encephalitis were involved. Prodromal events occurred in 34.2% of patients with infectious events being the most common. Seizure was the most common initial symptom, though movement disorder served as the most common event throughout the course of disease. 9.9% of patients had overlapped with other neuronal autoantibodies. Electroencephalogram showed abnormalities with slow wave (100.0%), epileptic discharge (31.5%) and delta brush (8.1%) respectively. 41.4% of patients had abnormal brain MRI, with focal lesions being the most common. None patients had tumor. 80.9% of patients had good response to first line therapy (steroid plus immunoglobulin), while 14 patients accepted second-line therapy (Rituximab) and all had a good response. Boys were significantly more likely to need more course of steroid. 13.8% of patients relapsed. 2 male patients died. mRS score was significantly improved after treatment. 51.4% of patients had a full recovery and 81.7% had mRS score ≤ 2. The median mRS score of boys after treatment was higher than that of girls. Non-infectious prodromal event, past medical history, perivascular lesions in brain MRI, hospital stay, initial mRS score higher than 3, and RTX treatment were independent risk factors associated with poor prognosis, defined as mRS score > 2. CONCLUSION Of pediatric anti-NMDAR encephalitis in southern China: median onset age around 7 years; girls more common; boys might have poor outcome than girls; seizure or movement disorder respectively being most common onset or course symptom; a few overlapped with other neuronal autoantibodies; rare combined with tumor; most had a good response to immunotherapy and a good prognosis; relapse rate relatively high; fatality rate relatively low; some risk factors associated with poor prognosis.

中文翻译：

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华南地区小儿抗-N-甲基-d-天冬氨酸受体脑炎111例分析

目的探讨华南地区抗NMDAR脑炎患儿的临床特征。方法 对2014年10月至2020年6月某国家区域医疗中心确诊为抗NMDAR脑炎患儿的临床资料进行分析。在整个疾病过程中，通过改良的兰金量表 (mRS) 评估神经功能障碍。结果 111 名儿童（男/女 = 49/62；平均发病年龄 = 6.8 岁）患有抗 NMDAR 脑炎。前驱事件发生在 34.2% 的患者中，感染事件最常见。癫痫发作是最常见的初始症状，尽管运动障碍是整个疾病过程中最常见的事件。9.9% 的患者与其他神经元自身抗体重叠。脑电图显示异常慢波（100.0%），分别为癫痫放电 (31.5%) 和 delta 刷 (8.1%)。41.4% 的患者有脑部 MRI 异常，局灶性病变最为常见。没有患者有肿瘤。80.9%的患者对一线治疗（类固醇加免疫球蛋白）反应良好，14例患者接受二线治疗（利妥昔单抗），反应良好。男孩更可能需要更多的类固醇疗程。13.8%的患者复发。2名男性患者死亡。治疗后mRS评分明显改善。51.4%的患者完全康复，81.7%的患者mRS评分≤2。治疗后男孩的mRS评分中位数高于女孩。非感染性前驱事件、既往病史、脑 MRI 血管周围病变、住院时间、初始 mRS 评分高于 3、和 RTX 治疗是与预后不良相关的独立危险因素，定义为 mRS 评分 > 2。结论 华南地区儿童抗 NMDAR 脑炎：中位发病年龄约 7 岁；女孩更常见；男孩的结果可能比女孩差；癫痫发作或运动障碍分别是最常见的发作或病程症状；少数与其他神经元自身抗体重叠；罕见合并肿瘤；大多数对免疫疗法反应良好，预后良好；复发率比较高；死亡率相对较低；一些与预后不良相关的危险因素。癫痫发作或运动障碍分别是最常见的发作或病程症状；少数与其他神经元自身抗体重叠；罕见合并肿瘤；大多数对免疫疗法反应良好，预后良好；复发率比较高；死亡率相对较低；一些与预后不良相关的危险因素。癫痫发作或运动障碍分别是最常见的发作或病程症状；少数与其他神经元自身抗体重叠；罕见合并肿瘤；大多数对免疫疗法反应良好，预后良好；复发率比较高；死亡率相对较低；一些与预后不良相关的危险因素。