Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, 9# Jin Sui Road, 510623 Guangzhou, Guangdong Province, PR China.
Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, 9# Jin Sui Road, 510623 Guangzhou, Guangdong Province, PR China.
J Neuroimmunol. 2021 Mar 15;352:577479. doi: 10.1016/j.jneuroim.2021.577479. Epub 2021 Jan 15.
To study the clinical features of children diagnosed with anti-NMDAR encephalitis in southern China.
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.
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.
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.
研究中国南方地区抗 NMDAR 脑炎患儿的临床特征。
分析 2014 年 10 月至 2020 年 6 月期间，来自一家国家级区域医疗中心的抗 NMDAR 脑炎患儿的临床资料。采用改良 Rankin 量表（mRS）评估疾病全过程中的神经功能缺损。
共纳入 111 例抗 NMDAR 脑炎患儿（男/女=49/62；平均发病年龄 6.8 岁）。34.2%的患儿有前驱事件，以感染性事件最为常见。癫痫发作是最常见的首发症状，但运动障碍是最常见的疾病过程中的症状。9.9%的患儿与其他神经元自身抗体重叠。脑电图分别表现为慢波（100.0%）、癫痫放电（31.5%）和 δ 刷（8.1%）异常。41.4%的患儿脑 MRI 异常，以局灶性病变最为常见。无患儿存在肿瘤。80.9%的患儿对一线治疗（激素联合免疫球蛋白）反应良好，14 例患儿接受二线治疗（利妥昔单抗），均反应良好。男孩需要更多疗程激素的可能性显著更高。13.8%的患儿复发。2 例男性患儿死亡。治疗后 mRS 评分显著改善。51.4%的患儿完全康复，81.7%的患儿 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、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）。
中国南方地区的儿童抗 NMDAR 脑炎具有以下特点：发病年龄中位数约为 7 岁；女性多见；男孩预后可能较女孩差；癫痫发作或运动障碍分别为最常见的起病或病程症状；少数与其他神经元自身抗体重叠；罕见合并肿瘤；多数患儿对免疫治疗反应良好，预后良好；复发率相对较高；病死率相对较低；存在一些与不良预后相关的危险因素。
