Comparative analysis of new-onset refractory status epilepticus in adult and pediatric patients: immunotherapy timing and functional outcomes.
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OBJECTIVES: We aimed to compare the clinical course of adult and pediatric patients with new-onset refractory status epilepticus (NORSE), including study of the timing of initiation of immunotherapy timing, duration of intensive care unit (ICU) stay, and functional outcomes. METHODS: A retrospective review was conducted of patients with NORSE admitted to McGill University Health Centre (January 2013-July 2023). Data collected included demographics, diagnostics, treatments, ICU stay length, long-term anti-seizure medication use, and modified Rankin Scale (mRS) scores, with good outcomes defined as mRS 0-2 and poor outcomes as 3-6. Early initiation of immunotherapy was defined as administration of first-line agent within 7 days of admission and second line agent within 30 days. Comparisons were made between adult and pediatric patients. RESULTS: 15 patients were identified (10 adult, 5 pediatric) with median ages of 34 and 4 years, respectively. A causative etiology was identified in three adult patients (two anti-NMDA receptor encephalitis, one small cell lung cancer) and two pediatric patients (one anti-NMDA receptor encephalitis and one hemophagocytic lymphohistiocytosis); the remainder were considered to have cryptogenic NORSE. Good functional outcomes (mRS 0-2) were seen in 80% of the pediatric cohort but only 40% of adults. Children that had earlier escalation of chronic immunosuppression tended to have better functional outcomes, though the same trend was not seen in the adult cohort. DISCUSSION: There are likely differences in presentation and clinical course between adult and pediatric patients with NORSE, with children possibly having a better long-term prognosis; however, further study is needed. Investigation into age-specific factors may guide more targeted therapeutic approaches. Ideally, a prospective multicenter randomized controlled trial would be conducted in order to generate more robust data to help determine optimal treatment protocols for NORSE across all age groups.