Overview
Febrile infection-related epilepsy syndrome (FIRES) is a rare neurological disorder characterized by refractory epilepsy developing in children following a febrile infection, often without evidence of direct brain infection 1. It typically presents with sudden onset of seizures and progressive neurological decline despite initial benign febrile illness 1.Diagnosis
Key Criteria: Sudden onset of refractory seizures following a febrile illness without identifiable central nervous system infection 1.
Recommended Tests: Comprehensive blood tests including complete blood count (CBC), C-reactive protein (CRP), and lumbar puncture to rule out other infectious causes 1.
Risk Factors: Elevated CRP levels and prolonged fever may indicate higher risk for serious bacterial infections (SBI) in infants <3 months 1.Management
First-Line Treatment: Anticonvulsants such as valproate or levetiracetam for seizure control 1.
Adjunctive Treatments: Immunomodulatory therapies like intravenous immunoglobulin (IVIG) or corticosteroids may be considered in refractory cases, though evidence is limited 1.
Supportive Care: Intensive care monitoring, management of complications, and multidisciplinary approach including neurology, infectious disease, and critical care 1.Special Populations
Pediatrics: Infants <3 months are at higher risk for serious bacterial infections (SBI) and require thorough evaluation and empirical antibiotic therapy if clinical suspicion remains high 1.
Comorbidities: No specific guidance provided in the abstracts regarding comorbidities; management should focus on addressing both febrile illness and neurological deterioration 1.Key Recommendations
Thorough Evaluation for SBI in Febrile Infants <3 Months: Perform comprehensive clinical and laboratory assessments including CRP and blood cultures to identify risk of serious bacterial infections 1 (Evidence: Moderate).
Empirical Antibiotic Therapy in Suspected SBI: Initiate empirical antibiotic therapy in infants <3 months with prolonged fever and elevated inflammatory markers until infectious causes are ruled out 1 (Evidence: Moderate).
Early Anticonvulsant Therapy for Seizures: Initiate appropriate anticonvulsant therapy promptly for management of refractory seizures in FIRES 1 (Evidence: Expert opinion).References
1 Moya I M, Couble P B, Piñera M C, Suau C T, Fritis L A, Roa R C et al.. Utility of basic clinical and laboratory parameters to predict Serious Bacterial Infection in children younger than 3 months old hospitalized for Febrile Syndrome without Source. Revista chilena de pediatria 2020. link