Comparative clinical outcomes in children with Febrile Infection-Related Epilepsy Syndrome (FIRES)

Area of Science:

• Pediatric Neurology
• Neuroinflammation
• Epilepsy Syndromes

Background:

• Febrile Infection-Related Epilepsy Syndrome (FIRES) is a severe, rare encephalopathy in children.
• Understanding the long-term functional, emotional, and behavioral outcomes is critical for comprehensive patient care.
• Treatment advancements include biologics and ketogenic diets (KD), necessitating outcome evaluation.

Purpose of the Study:

• To compare functional, emotional, and behavioral outcomes in children with FIRES.
• To assess outcomes in patients receiving conventional treatments versus those also treated with biologics and/or KD.
• To analyze outcomes over a 24-month period post-diagnosis.

Main Methods:

• Retrospective chart review of pediatric patients (<18 years) diagnosed with FIRES.
• Analysis of two cohorts based on biologic availability (pre-2018 vs. 2018 onwards).
• Functional assessment using modified Rankin Scale (mRS) and WeeFIM; behavioral screening via MOAS, ADHD, anxiety, and depression diagnoses.

Main Results:

• Children requiring biologics and/or KD showed lower functional scores (WeeFIM) at discharge and 24 months.
• Aggression was more prevalent (53.5%) and severe (higher MOAS scores) in the later period (2018-2023).
• Inattention and mood disorders occurred later than aggression, with similar proportions across treatment groups.

Conclusions:

• Behavioral and emotional sequelae are common in FIRES, irrespective of advanced treatments like biologics or KD.
• Aggression typically manifests earlier, while inattention and mood disorders appear later in the disease course.
• Routine screening for long-term emotional and behavioral issues is essential for all children recovering from FIRES.