Systemic inflammatory markers and EEG features of children with FIRES receiving anakinra
Yi-Chen Lai1, Gabriella Abou-El-Kheir1, Thao Nguyen1
1Division of Pediatric Critical Care Medicine, Baylor College of Medicine, Houston, Texas, USA.
Insights
Anakinra treatment helped most children with cryptogenic Febrile Infection-Related Epilepsy Syndrome (FIRES) stop anesthesia within weeks. Changes in EEG, CRP, and cytokines may signal treatment progress.
Area of Science:
- Pediatric Neurology
- Neuroinflammation
- Epileptology
Background:
- Febrile Infection-Related Epilepsy Syndrome (FIRES) is a rare, severe epilepsy syndrome.
- Anakinra, an IL-1 receptor antagonist, is being explored for FIRES treatment.
- Understanding early clinical course and biomarkers is crucial for managing FIRES.
Purpose of the Study:
- To describe the early clinical course of children with cryptogenic FIRES treated with anakinra.
- To identify potential biomarkers correlating with clinical progression during anakinra therapy.
Main Methods:
- Retrospective case series of 10 children with cryptogenic FIRES.
- Analysis of clinical data, including anesthetic withdrawal timelines.
- Evaluation of electroencephalography (cEEG), C-reactive protein (CRP), and serum cytokine levels (IL-6, IL-10).
Main Results:
- Six out of 10 children achieved anesthetic withdrawal within 3 weeks; one more by week 4.
- Temporal changes observed in epileptiform bursts on cEEG, CRP, IL-6, and IL-10 levels.
- These changes potentially correlate with clinical improvement.
Conclusions:
- Anakinra shows promise in managing cryptogenic FIRES, facilitating faster anesthetic withdrawal.
- CRP, IL-6, IL-10, and cEEG findings may serve as candidate biomarkers for monitoring treatment response.
- Further investigation is warranted to validate these biomarkers in larger studies.
Abstract:
In a retrospective case series of 10 children with cryptogenic FIRES, we sought to describe the early clinical course and potential biomarkers following anakinra initiation. Six children achieved anesthetic withdrawal within 3 weeks of therapy and one in week four. Of the available cEEG (six children), CRP (10 children), and serum cytokine (six children) studies, there were temporal changes in highly epileptiform bursts (observed in three children), CRP, IL-6, and IL-10 levels that might parallel clinical progression. These observations may represent candidate biomarkers for monitoring clinical progression and therapeutic interventions including anakinra, which merits further investigation in future studies.
