Rasmussen syndrome in a pediatric patient: case report

Abstract

Introduction: Rasmussen’s Syndrome (RS) is a rare, chronic, and progressive encephalitis, presumably of autoimmune origin, that predominantly affects children. It is characterized by drug-resistant focal seizures, progressive neurological deterioration, and cerebral hemispheric atrophy. Objective: To describe the clinical course, therapeutic challenges, and outcome of a pediatric patient with Rasmussen’s syndrome. Case Report: This case describes an 8-year-old female patient with a history of delayed neuropsychomotor development who developed drugresistant focal epilepsy. The diagnosis of RS was confirmed following clinical and imaging investigations. Functional hemispherectomy was indicated due to refractoriness to medical treatment. The procedure was temporarily postponed due to SARS-CoV-2 infection and was later performed successfully. The patient developed complications postoperatively, including hemodynamic instability, electrolyte disturbances, diabetes insipidus, respiratory infections, and acute kidney injury. Complete seizure control was achieved after approximately 65 days of hospitalization. Results: This case highlights the complexity of managing RS in pediatric patients and emphasizes the importance of an individualized approach. Conclusion: Hemispherectomy proved effective in controlling seizures and stabilizing neurological progression.