Publication Date
4-1-2025
Journal
Annals of Clinical and Translational Neurology
DOI
10.1002/acn3.70011
PMID
39932904
PMCID
PMC12040501
PubMedCentral® Posted Date
2-11-2025
PubMedCentral® Full Text Version
Post-print
Abstract
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.
Keywords
Child, Preschool, Humans, Male, Antibodies, Monoclonal, Humanized, Dexamethasone, Drug Resistant Epilepsy, Drug Therapy, Combination, Immunotherapy, Injections, Spinal, Interleukin 1 Receptor Antagonist Protein, Seizures, Febrile, Status Epilepticus, Child, anakinra, CNS immune modulation, febrile infection related epilepsy syndrome (FIRES), intrathecal dexamethasone, new onset refractory status epilepticus (NORSE), systemic immune modulation, tocilizumab
Published Open-Access
yes
Recommended Citation
Fisher, Kristen S; Ankar, Alexander; Cokley, Jon; et al., "Combined Systemic Immunotherapy and Intrathecal Dexamethasone in Febrile Infection Related Epilepsy Syndrome" (2025). Faculty and Staff Publications. 5752.
https://digitalcommons.library.tmc.edu/baylor_docs/5752