Age-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES Family Registry

• Published in: Epilepsia (Copenhagen) 2025-01
• Main Authors: Kazazian, Karnig, Gaspard, Nicolas, Hirsch, Lawrence J, Kellogg, Marissa, Hocker, Sara E, Wong, Nora, Farias-Moeller, Raquel, Eschbach, Krista, Gofton, Teneille E
• Format: Article
• Language: English
• ISSN: 0013-9580; 1528-1167; 1528-1167
• DOI: 10.1111/epi.18260

Febrile infection-related epilepsy syndrome (FIRES) is a rare clinical presentation of refractory status epilepticus following a febrile infection. This study analyzes data from the NORSE/FIRES Family Registry, an international web-based registry available in six languages with data entered by patients, families, and clinicians to explore clinical presentations, survivorship, and long-term outcomes in adult and pediatric FIRES patients. We characterize and examine differences in demographics, prodromal symptoms, seizure frequency, anti-seizure medications (ASMs), quality of life, cognition, mood, and anxiety in adults vs pediatric populations with FIRES. Eighty-six participants were included in the study. Pediatric patients (n = 54) were predominantly male (77.8%) and experienced a significantly higher post-FIRES seizure burden than adult survivors (67.7% ≥12 seizures/month in pediatrics vs 11.8% in adults, p