Treatment? strategies for paediatric autoimmune encephalitis – current evidence, limitations and future directions

Abstract

Introduction and Objective. Autoimmune encephalitis (AE) is an inflammatory disorder in which the immune system attacks brain tissue, causing central nervous system (CNS) dysfunction. In children, it often manifests as seizures, behavioural changes, memory impairment and involuntary movements. The aim of the review is to summarise and discuss currently available treatment methods, their limitations, and the latest advances in the field. Review Methods. A narrative literature review was conducted using PubMed and Google Scholar databases. The search strategy included combinations of the following key words and their variations: ‘autoimmune encephalitis, ‘AE’ and ‘paediatric’ or ‘children’. Priority was given to recent peer-reviewed studies published from January 2020 onwards in order to capture the most up-to-date evidence. However, selected earlier landmark studies were also included when considered essential for contextual understanding. Brief description of the state of knowledge. Treatment of this condition primarily focuses on immunotherapy. First-line therapy includes high-dose corticosteroids, intravenous immunoglobulins and plasmapheresis. In cases refractory to first-line therapy, second-line treatments most commonly comprise rituximab and cyclophosphamide. Additional options include certain monoclonal antibodies, cytotoxic agents, as well as interleukin-2 (IL-2) . In addition to primary treatment, supportive therapy may be introduced. Summary. AE is a serious neurological disorder that can cause irreversible CNS damage without prompt treatment. Its growing recognition in children highlights the need for early diagnosis and timely therapy. While immunotherapy is often effective, some patients remain refractory, underscoring the need for further research, paediatric-specific guidelines and new treatments.