AUTHOR=Dou Qingyang , Li Renke , Shu Xiaomei TITLE=Anti-contactin-associated protein-like 2 antibody-associated encephalitis in children: A case report and literature review JOURNAL=Frontiers in Pediatrics VOLUME=Volume 10 - 2022 YEAR=2022 URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2022.1004210 DOI=10.3389/fped.2022.1004210 ISSN=2296-2360 ABSTRACT=Background: 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. Case presentation: 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, twitching in the extremity muscles, and involuntary movements. The autoimmune encephalitis-associated antibodies testing showed that CASPR2-associated antibodies were positive, and electroencephalography showed diffuse slow waves. The child's status significantly improved after receiving immunotherapy with intravenous methylprednisolone and immunoglobulin. Conclusions: Anti-CASPR2 antibody-associated encephalitis has been rarely reported in children, with a complex clinical presentation and a low incidence of combined tumors. Most pediatric patients have a favorable prognosis, and relapse is uncommon.