Comparative analysis of new-onset refractory status epilepticus in adult and pediatric patients: immunotherapy timing and functional outcomes.

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.