Update on pediatric autoimmune encephalitis-associated epilepsy

Autoimmune encephalitis-associated epilepsy (AEAE) is initially caused by an aberrant innate and/or adaptive immune system, and then the seizure nidus persists from the ensuing structural damage resulting in chronic, often drug-resistant epilepsy. Early identification and targeted immunomodulation may help improve long-term outcomes in some cases. This review summarizes current advances in understanding the diagnosis, treatment, and long-term outcomes for pediatric patients with AEAE.

Enhanced recognition of the role of immune dysfunction in seizures has led to diagnostic and treatment advances. Predictive scores may help identify appropriate patients faster, allowing for earlier initiation of immunotherapy. Biomarkers are emerging to aide in diagnosis and prognostication, including EEG features and biochemical markers, such as neurofilament light chain and cytokines. Novel intrathecal and cytokine-targeted therapies may improve outcomes. Children are at a higher risk for developing AEAE after some types of autoimmune encephalitis compared to adults, but predicting those at risk for epilepsy remains a challenge. Long-term outcomes in children with AEAE are often poor, with refractory seizures and cognitive decline.

Pediatric AEAE is associated with significant morbidity, and research is underway to improve outcomes for these patients by determining risk factors, identifying biomarkers, and developing targeted therapeutic approaches.