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 Jimenez; Margaret Gopaul; Hannah Asbell; Seyhmus Aydemir; Maysaa M Basha; Ayush Batra; Charlotte Damien; Gregory S Day; Onome Eka; Krista Eschbach; Safoora Fatima; Madeline C Fields; Brandon Foreman; Elizabeth E Gerard; Teneille E Gofton; Hiba A Haider; Stephen T Hantus; Sara Hocker; Amy Jongeling; Mariel Kalkach Aparicio; Padmaja Kandula; Peter Kang; Karnig Kazazian; Marissa A Kellogg; Minjee Kim; Jong Woo Lee; Lara V Marcuse; Christopher M McGraw; Wazim Mohamed; Janet Orozco; Cederic Pimentel; Vineet Punia; Alexandra M Ramirez; Claude Steriade; Aaron F Struck; Olga Taraschenko; Andrew K Treister; Ji Yeoun Yoo; Sahar Zafar; Daniel J Zhou; Deepti Zutshi; Nicolas Gaspard; Lawrence J Hirsch; Aurelie Hanin

doi:10.1111/epi.17988

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

Epilepsia. 2024 Apr 16. doi: 10.1111/epi.17988. Online ahead of print.

Authors

Anthony D Jimenez¹, Margaret Gopaul¹, Hannah Asbell², Seyhmus Aydemir³, Maysaa M Basha⁴, Ayush Batra⁵, Charlotte Damien⁶, Gregory S Day⁷, Onome Eka⁸, Krista Eschbach², Safoora Fatima⁹, Madeline C Fields⁸, Brandon Foreman¹⁰, Elizabeth E Gerard⁵, Teneille E Gofton¹¹, Hiba A Haider^{12

13}, Stephen T Hantus¹⁴, Sara Hocker¹⁵, Amy Jongeling¹⁶, Mariel Kalkach Aparicio⁹, Padmaja Kandula³, Peter Kang⁷, Karnig Kazazian¹¹, Marissa A Kellogg¹⁷, Minjee Kim⁵, Jong Woo Lee¹⁸, Lara V Marcuse⁸, Christopher M McGraw¹⁹, Wazim Mohamed⁴, Janet Orozco¹⁸, Cederic Pimentel²⁰, Vineet Punia¹⁴, Alexandra M Ramirez¹⁰, Claude Steriade¹⁶, Aaron F Struck⁹, Olga Taraschenko²¹, Andrew K Treister¹⁷, Ji Yeoun Yoo⁸, Sahar Zafar¹⁹, Daniel J Zhou²¹, Deepti Zutshi⁴, Nicolas Gaspard^{1

6}, Lawrence J Hirsch¹, Aurelie Hanin^{1

22

23}

Affiliations

¹ Department of Neurology, Comprehensive Epilepsy Center, Yale University School of Medicine, New Haven, Connecticut, USA.
² Section of Neurology, Department of Pediatrics, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, USA.
³ Department of Neurology, Weill Cornell Medicine, New York, New York, USA.
⁴ Wayne State University School of Medicine, Detroit, Michigan, USA.
⁵ Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA.
⁶ Department of Neurology, Hôpital Universitaire de Bruxelles-Hôpital Erasme, Brussels, Belgium.
⁷ Washington University School of Medicine, Saint Louis, Missouri, USA.
⁸ Icahn School of Medicine at Mount Sinai, New York, New York, USA.
⁹ Department of Neurology, University of Wisconsin, Madison, Wisconsin, USA.
¹⁰ Division of Neurocritical Care, Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, Ohio, USA.
¹¹ University Hospital London Health Sciences Center, London, Ontario, Canada.
¹² Epilepsy Center, Emory University School of Medicine, Atlanta, Georgia, USA.
¹³ Department of Neurology, University of Chicago, Chicago, Illinois, USA.
¹⁴ Epilepsy Center, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, USA.
¹⁵ Mayo Clinic, Minnesota, Rochester, Minnesota, USA.
¹⁶ NYU Comprehensive Epilepsy Center, NYU Langone Medical Center, New York, New York, USA.
¹⁷ Oregon Health & Science University, Portland, Oregon, USA.
¹⁸ Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
¹⁹ Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.
²⁰ Neurocritical Care, Emory University School of Medicine, Atlanta, Georgia, USA.
²¹ Department of Neurological Sciences, University of Nebraska Medical Center, Omaha, Nebraska, USA.
²² Sorbonne Université, Institut du Cerveau, Paris Brain Institute, ICM, Inserm, CNRS, Assistance Publique- Hôpitaux de Paris, Hôpital de la Pitié-Salpêtrière, Paris, France.
²³ Assistance Publique - Hôpitaux de Paris, Hôpital de la Pitié-Salpêtrière, DMU Neurosciences, Epilepsy Unit and Department of Clinical Neurophysiology, Paris, France.

PMID: 38625055
DOI: 10.1111/epi.17988

Abstract

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.

Keywords: age; febrile infection‐related epilepsy syndrome; new onset refractory status epilepticus; outcome.