Current Therapeutic Strategies for Rasmussen Encephalitis: A Systematic Review of Functional Hemispherectomy and Immunotherapy in Pediatric Intractable Epilepsy

Background: Rasmussen encephalitis (RE) is a rare, progressive neurological disorder in children, characterized by intractable focal seizures, unilateral cerebral inflammation, and cognitive decline. Management remains complex due to the disease’s resistance to conventional antiepileptic therapy. Objective: This systematic review evaluates the efficacy and safety of functional hemispherectomy and immunotherapy in pediatric patients with RE and drug-resistant epilepsy. Methods: A systematic search of PubMed, Scopus, and Web of Science was conducted in accordance with PRISMA 2020 guidelines. Fifteen studies involving 312 pediatric patients met inclusion criteria. Data on seizure outcomes, neurological function, and treatment-related complications were extracted and analyzed. Results: Functional hemispherectomy, including advanced techniques such as hemispherotomy, resulted in seizure freedom (Engel Class I) in 70–80% of cases, particularly when performed early. Immunotherapeutic strategies, including corticosteroids, intravenous immunoglobulin (IVIG), and plasmapheresis, offered short-term seizure reduction and delayed neurological deterioration but failed to produce lasting remission when used alone. Preoperative immunotherapy may improve perioperative conditions, though evidence is inconclusive and lacks standardization. Conclusion: Functional hemispherectomy remains the most effective intervention for long-term seizure control in pediatric RE, especially when performed early in the disease course. Immunotherapy plays a supportive role, particularly in early phases or as a bridge to surgery. Future research should aim to define standardized treatment protocols, identify early biomarkers, and evaluate emerging targeted immunotherapies to improve long-term outcomes.