Age-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based...

Area of Science:

• Neurology
• Pediatric Neurology
• Epileptology

Background:

• Febrile infection-related epilepsy syndrome (FIRES) is a rare, severe neurological condition characterized by refractory status epilepticus after infection.
• Understanding the long-term outcomes and differences between pediatric and adult FIRES survivors is crucial for targeted management.

Purpose of the Study:

• To analyze and compare the clinical presentations, seizure burden, treatment, and long-term outcomes (quality of life, cognition, mood, anxiety) in adult and pediatric FIRES patients.
• To identify age-specific challenges in managing FIRES survivors.

Main Methods:

• Data from 86 participants (54 pediatric, 32 adult) were analyzed from the international NORSE/FIRES Family Registry.
• Demographic data, prodromal symptoms, seizure frequency, anti-seizure medication (ASM) use, quality of life, cognition, mood, and anxiety were collected and compared between age groups.

Main Results:

• Pediatric patients (predominantly male) had a significantly higher seizure burden (≥12 seizures/month) compared to adults (p < .001).
• Adults were more likely to be female and report flu-like prodromal symptoms (90.6%).
• Both groups required high ASM use (≥3 medications); pediatric patients reported worse mood, anxiety, and cognitive outcomes.

Conclusions:

• FIRES presents distinct challenges in pediatric and adult populations, with children experiencing greater seizure burden and poorer long-term neurological and emotional outcomes.
• Age-tailored management approaches are essential to address the specific needs and improve outcomes for FIRES survivors, especially in pediatric cases.