Kang Qingyun, Liao Hongmei, Yang Liming, Fang Hongjun, Hu Wenjing, Wu Liwen
Department of Neurology, Hunan Children's Hospital, Changsha, China.
Front Pediatr. 2022 Jul 8;10:880693. doi: 10.3389/fped.2022.880693. eCollection 2022.
The incidence and prevalence of autoimmune encephalitis (AE) is gradually increasing in pediatric patients (between the ages of 3 months and 16 years). The aim of this retrospective observational study was to investigate the clinical characteristics and short-term prognosis of children with antibody-mediated AE at Hunan Children's Hospital.
Antibody analysis of blood and/or cerebrospinal fluid was performed in suspected AE patients admitted to the Department of Neurology, Hunan Children's Hospital from June 2014 to June 2021. Ultimately, 103 patients were diagnosed with antibody-mediated AE and were enrolled in this study. Clinical data and corresponding demographic, clinical characteristics, laboratory and imaging data, treatment, and prognosis data were collected and analyzed.
In our study, 103 AE patients with antibody-positive were identified. The main subtype of AE in our cohort was anti-NMDAR encephalitis. Few patients have anti-CASPR2 encephalitis, anti-GABABR encephalitis, or anti-LGI1 encephalitis. In our AE patients, the most common clinical manifestations were behavioral symptoms, seizures, and involuntary movements, with seizures being the most common initial symptom. All patients underwent brain magnetic resonance imaging (MRI) and electroencephalography (EEG). Forty-five (43.7%) patients had abnormal MRI findings. And 96 (93.2%) patients had abnormal EEG results. All 103 patients were given first-line immunotherapy, 21 of which were also treated with the combination of the second-line immunotherapy. All surviving patients were followed up for at least 6 months. Seventy-seven patients recovered completely, 23 had sequelae of different degrees, and 3 died. Eight patients had one or more relapses during the follow-up period.
AE is a treatable disease that can occur in children of all ages. The mortality rate is low, as most patients have a good response to immune therapy. Compared with the older children, infants and young children (≤ 3 years old) with anti-NMDAR encephalitis have a higher incidence of fever and status epilepticus, more severe condition, higher PICU admission rate and worse prognosis. AE patients with high maximum mRS scores and PICU admissions may require second-line immunotherapy.
自身免疫性脑炎（AE）在儿科患者（3个月至16岁）中的发病率和患病率正在逐渐上升。这项回顾性观察研究的目的是调查湖南省儿童医院抗体介导的AE患儿的临床特征和短期预后。
对2014年6月至2021年6月入住湖南省儿童医院神经内科的疑似AE患者进行血液和/或脑脊液抗体分析。最终，103例患者被诊断为抗体介导的AE并纳入本研究。收集并分析临床资料以及相应的人口统计学、临床特征、实验室和影像学资料、治疗及预后资料。
在我们的研究中，确定了103例抗体阳性的AE患者。我们队列中AE的主要亚型是抗NMDAR脑炎。很少有患者患有抗CASPR2脑炎、抗GABABR脑炎或抗LGI1脑炎。在我们的AE患者中，最常见的临床表现是行为症状、癫痫发作和不自主运动，癫痫发作是最常见的初始症状。所有患者均接受了脑磁共振成像（MRI）和脑电图（EEG）检查。45例（43.7%）患者MRI检查结果异常。96例（93.2%）患者EEG结果异常。所有103例患者均接受了一线免疫治疗，其中21例还接受了二线免疫治疗联合治疗。所有存活患者均进行了至少6个月的随访。77例患者完全康复，23例有不同程度的后遗症，3例死亡。8例患者在随访期间出现一次或多次复发。
AE是一种可治疗的疾病，可发生于各年龄段儿童。死亡率较低，因为大多数患者对免疫治疗反应良好。与年龄较大的儿童相比，抗NMDAR脑炎的婴幼儿（≤3岁）发热和癫痫持续状态的发生率更高，病情更严重，PICU入住率更高，预后更差。mRS最高评分和入住PICU的AE患者可能需要二线免疫治疗。
