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Combined Systemic Immunotherapy and Intrathecal Dexamethasone in Febrile Infection Related Epilepsy Syndrome
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Combined Systemic Immunotherapy and Intrathecal Dexamethasone in Febrile Infection Related Epilepsy Syndrome
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Journal Article
Combined Systemic Immunotherapy and Intrathecal Dexamethasone in Febrile Infection Related Epilepsy Syndrome
2025
Overview
Febrile infection related epilepsy syndrome (FIRES) is a rare presentation of refractory status epilepticus with immune dysregulation as a potential pathologic mechanism. Despite promising results from second‐line immunomodulators, approximately 30% remain refractory to treatment. We describe two children with FIRES who were unable to wean from anesthetic infusions with immunomodulatory treatment and subsequently received concurrent intrathecal dexamethasone and anakinra/tocilizumab as escalation of therapy. Following the initiation of this combined regimen, anesthetic infusions were decreased while maintaining seizure freedom. These cases demonstrate proof of principle that a multi‐modal approach may be beneficial and should be considered in the treatment of FIRES.
Publisher
John Wiley & Sons, Inc,John Wiley and Sons Inc,Wiley
Subject
/ Antibodies, Monoclonal, Humanized - administration & dosage
/ Child
/ Dexamethasone - administration & dosage
/ Dexamethasone - pharmacology
/ Drug Resistant Epilepsy - drug therapy
/ Epilepsy
/ febrile infection related epilepsy syndrome (FIRES)
/ Fever
/ Humans
/ Interleukin 1 Receptor Antagonist Protein - administration & dosage
/ Ketamine
/ Male
/ new onset refractory status epilepticus (NORSE)
/ Patients
/ Seizures, Febrile - drug therapy
/ Status Epilepticus - drug therapy
/ Status Epilepticus - etiology
/ Steroids
/ Weaning
