Department of Pediatric Intensive Care Unit, The First Hospital of Jilin University, No. 1 Xinmin Street, Changchun, 130021, Jilin, China.
Acta Neurol Belg. 2023 Oct;123(5):1663-1678. doi: 10.1007/s13760-023-02174-5. Epub 2023 Jan 20.
To ascertain the clinical characteristics of pediatric patients with contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis (AEs).
Two cases of CASPR2 antibody-associated AEs have been reported. In addition, a systematic search of literature published between January 2010 and March 2022 through six online databases was conducted to identify the pediatric patients with CASPR2 antibody-associated AEs. Data on demographics, clinical symptoms, laboratory examinations, imaging, treatment, and outcome were collected.
Our updated literature search yielded 1,837 publications, of which 21 were selected, and 40 patients in this study met the diagnostic criteria for AE. There were 25 males and 15 females with a mean age of 9.2 years. The most common presenting symptoms are psychiatric symptoms (72.5%), sleep changes (62.5%), and movement disorders (60%). The psychiatric symptoms included mood changes (39.1%), behavior changes (25%), and hallucination (7.5%). In total, 23 cases (57.5%) combined with autonomic dysfunction, such as gastrointestinal dysmotility, cardiovascular-related symptoms, and sweating. No tumors were observed in children. Thirty-eight patients received first-line immunotherapy, and eight received first-line and second-line immunotherapy. All patients had a good clinical response to immune therapy. Mean mRS at onset was 3.4; It was 0.88 at the last follow-up. There was no recurrence during follow-up.
Psychiatric symptoms, sleep disorders, movement disorders, and cardiovascular-related symptoms are the most common presentation in pediatric patients with CASPR2 antibody-associated AEs. Tumor, particularly with thymoma, is uncommon in children diagnosed with CASPR2 antibody-associated AEs. In addition, prompt diagnosis and immunotherapy can relieve symptoms and improve the prognosis.
确定接触蛋白相关蛋白样 2 (CASPR2) 抗体相关自身免疫性脑炎 (AE) 患儿的临床特征。
报告了 2 例 CASPR2 抗体相关 AE 病例。此外，通过 6 个在线数据库对 2010 年 1 月至 2022 年 3 月期间发表的文献进行了系统检索，以确定 CASPR2 抗体相关 AE 的儿科患者。收集了人口统计学、临床症状、实验室检查、影像学、治疗和预后的数据。
我们更新的文献检索产生了 1837 篇文献，其中 21 篇被选中，本研究共有 40 例患者符合 AE 的诊断标准。男性 25 例，女性 15 例，平均年龄为 9.2 岁。最常见的首发症状是精神症状 (72.5%)、睡眠改变 (62.5%)和运动障碍 (60%)。精神症状包括情绪改变 (39.1%)、行为改变 (25%)和幻觉 (7.5%)。共有 23 例 (57.5%)合并自主神经功能障碍，如胃肠动力障碍、心血管相关症状和出汗。儿童中未发现肿瘤。38 例患者接受一线免疫治疗，8 例患者接受一线和二线免疫治疗。所有患者对免疫治疗均有良好的临床反应。发病时平均 mRS 为 3.4；最后一次随访时为 0.88。随访期间无复发。
精神症状、睡眠障碍、运动障碍和心血管相关症状是儿童 CASPR2 抗体相关 AE 最常见的表现。肿瘤，特别是胸腺瘤，在诊断为 CASPR2 抗体相关 AE 的儿童中并不常见。此外，及时诊断和免疫治疗可以缓解症状，改善预后。
