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

Author:

Jimenez Anthony D.1ORCID,Gopaul Margaret1,Asbell Hannah2,Aydemir Seyhmus3,Basha Maysaa M.4ORCID,Batra Ayush5ORCID,Damien Charlotte6,Day Gregory S.7,Eka Onome8,Eschbach Krista2ORCID,Fatima Safoora9,Fields Madeline C.8,Foreman Brandon10,Gerard Elizabeth E.5,Gofton Teneille E.11ORCID,Haider Hiba A.1213ORCID,Hantus Stephen T.14,Hocker Sara15,Jongeling Amy16,Kalkach Aparicio Mariel9ORCID,Kandula Padmaja3ORCID,Kang Peter7,Kazazian Karnig11,Kellogg Marissa A.17,Kim Minjee5,Lee Jong Woo18ORCID,Marcuse Lara V.8,McGraw Christopher M.19,Mohamed Wazim4ORCID,Orozco Janet18,Pimentel Cederic20,Punia Vineet14ORCID,Ramirez Alexandra M.10,Steriade Claude16ORCID,Struck Aaron F.9,Taraschenko Olga21ORCID,Treister Andrew K.17,Yoo Ji Yeoun8ORCID,Zafar Sahar19ORCID,Zhou Daniel J.21,Zutshi Deepti4ORCID,Gaspard Nicolas16,Hirsch Lawrence J.1ORCID,Hanin Aurelie12223ORCID

Affiliation:

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

Abstract

AbstractFebrile 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.

Funder

Philippe Foundation

Swebilius Foundation

Institut Servier

Publisher

Wiley

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