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 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

Comparative Study

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 et al. Epilepsia. 2024 Jun.

. 2024 Jun;65(6):e87-e96.

doi: 10.1111/epi.17988. Epub 2024 Apr 16.

Authors

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.

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References

REFERENCES

1. Hirsch LJ, Gaspard N, van Baalen A, Nabbout R, Demeret S, Loddenkemper T, et al. Proposed consensus definitions for new‐onset refractory status epilepticus (NORSE), febrile infection‐related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018;59(4):739–744.
1. Ismail FY, Kossoff EH. AERRPS, DESC, NORSE, FIRES: multi‐labeling or distinct epileptic entities? Epilepsia. 2011;52(11):e185–e189.
1. van Baalen A, Häusler M, Boor R, Rohr A, Sperner J, Kurlemann G, et al. Febrile infection‐related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia. 2010;51(7):1323–1328.
1. Kramer U, Chi C‐S, Lin K‐L, Specchio N, Sahin M, Olson H, et al. Febrile infection‐related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia. 2011;52(11):1956–1965.
1. Körtvelyessy P, Lerche H, Weber Y. FIRES and NORSE are distinct entities. Epilepsia. 2012;53(7):1276.

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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

Affiliations

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

Authors

Affiliations

Abstract

References

REFERENCES

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Medical

Research Materials

Miscellaneous