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Comparative analysis of patients with new onset refractory status epilepticus preceded by fever (febrile infection-related epilepsy syndrome) versus without prior fever: An interim analysis.

Anthony D JimenezMargaret GopaulHannah AsbellSeyhmus AydemirMaysaa Merhi BashaAyush BatraCharlotte DamienGregory S DayOnome EkaKrista EschbachSafoora FatimaMadeline C FieldsBrandon ForemanElizabeth E GerardTeneille E GoftonHiba A HaiderStephen T HantusSara HockerAmy JongelingMariel Kalkach AparicioPadmaja KandulaPeter KangKarnig KazazianMarissa A KelloggMinjee KimJong Woo LeeLara V MarcuseChristopher M McGrawWazim MohamedJanet OrozcoCederic PimentelVineet PuniaAlexandra M RamirezClaude SteriadeAaron F StruckOlga TaraschenkoAndrew K TreisterJi Yeoun YooSahar F ZafarDaniel J ZhouDeepti ZutshiNicolas GaspardLawrence J HirschAurélie Hanin
Published in: Epilepsia (2024)
Febrile infection-related epilepsy syndrome (FIRES) is a subset of new onset refractory status epilepticus (NORSE) that involves a febrile infection prior to the onset of the refractory status epilepticus. It is unclear whether FIRES and non-FIRES NORSE are distinct conditions. Here, we compare 34 patients with FIRES to 30 patients with non-FIRES NORSE for demographics, clinical features, neuroimaging, and outcomes. Because patients with FIRES were younger than patients with non-FIRES NORSE (median = 28 vs. 48 years old, p = .048) and more likely cryptogenic (odds ratio = 6.89), we next ran a regression analysis using age or etiology as a covariate. Respiratory and gastrointestinal prodromes occurred more frequently in FIRES patients, but no difference was found for non-infection-related prodromes. Status epilepticus subtype, cerebrospinal fluid (CSF) and magnetic resonance imaging findings, and outcomes were similar. However, FIRES cases were more frequently cryptogenic; had higher CSF interleukin 6, CSF macrophage inflammatory protein-1 alpha (MIP-1a), and serum chemokine ligand 2 (CCL2) levels; and received more antiseizure medications and immunotherapy. After controlling for age or etiology, no differences were observed in presenting symptoms and signs or inflammatory biomarkers, suggesting that FIRES and non-FIRES NORSE are very similar conditions.
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