Dou Qingyang, Li Renke, Shu Xiaomei
Department of Pediatrics, Affiliated Hospital of Zunyi Medical University, Zunyi, China.
Department of Pediatrics, Guizhou Provincial People's Hospital, Guiyang, China.
Front Pediatr. 2022 Oct 20;10:1004210. doi: 10.3389/fped.2022.1004210. eCollection 2022.
Anti-Contactin-associated protein-like 2 (CASPR2) antibody-associated encephalitis is a rare group of autoimmune diseases that causes extensive damage to the central and/or peripheral nervous system.
Here, we reported a case of anti-CASPR2 antibody-associated encephalitis in a 12-year-old male patient with symptoms of headache, consciousness disturbance, mental abnormalities, urinary incontinence, fasciculations in the extremity muscles, and involuntary movements. The testing for autoimmune encephalitis-associated antibodies showed that CASPR2-associated antibodies were positive, and electroencephalography showed diffuse slow waves. No tumor was found after screening for malignancies. The child's status significantly improved after receiving immunotherapy with intravenous methylprednisolone and immunoglobulin.
Anti-CASPR2 antibody-associated encephalitis has been rarely reported in children. It has a complex clinical presentation and a low incidence of tumor. Most pediatric patients have a favorable prognosis and relapse is uncommon.
抗接触蛋白相关蛋白样2(CASPR2)抗体相关脑炎是一组罕见的自身免疫性疾病,可对中枢和/或外周神经系统造成广泛损害。
在此,我们报告了一例12岁男性抗CASPR2抗体相关脑炎患者,其症状包括头痛、意识障碍、精神异常、尿失禁、肢体肌肉束颤和不自主运动。自身免疫性脑炎相关抗体检测显示CASPR2相关抗体呈阳性,脑电图显示弥漫性慢波。恶性肿瘤筛查后未发现肿瘤。该患儿接受静脉注射甲泼尼龙和免疫球蛋白免疫治疗后病情明显改善。
抗CASPR2抗体相关脑炎在儿童中鲜有报道。其临床表现复杂,肿瘤发生率低。大多数儿科患者预后良好,复发罕见。