Trends in management of patients with new-onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis

Aurelie Hanin^{1

2

3}, Anthony D Jimenez¹, Margaret Gopaul¹, Hannah Asbell⁴, Seyhmus Aydemir⁵, Maysaa Merhi Basha⁶, Ayush Batra⁷, Charlotte Damien⁸, Gregory S Day⁹, Onome Eka¹⁰, Krista Eschbach⁴, Safoora Fatima¹¹, Madeline C Fields¹⁰, Brandon Foreman¹², Elizabeth E Gerard⁷, Teneille E Gofton¹³, Hiba A Haider¹⁴, Stephen T Hantus¹⁵, Sara Hocker¹⁶, Amy Jongeling¹⁷, Mariel Kalkach Aparicio¹¹, Padmaja Kandula⁵, Peter Kang⁹, Karnig Kazazian¹³, Marissa A Kellogg¹⁸, Minjee Kim⁷, Jong Woo Lee¹⁹, Lara V Marcuse¹⁰, Christopher M McGraw²⁰, Wazim Mohamed⁶, Janet Orozco¹⁹, Cederic M Pimentel²¹, Vineet Punia¹⁵, Alexandra M Ramirez¹², Claude Steriade¹⁷, Aaron F Struck¹¹, Olga Taraschenko²², Andrew K Treister¹⁸, Mark S Wainwright²³, Ji Yeoun Yoo¹⁰, Sahar Zafar²⁰, Daniel J Zhou²², Deepti Zutshi⁶, Nicolas Gaspard^{1

8}, Lawrence J Hirsch¹

Affiliations

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

PMID: 38837761
DOI: 10.1111/epi.18014

Multicenter Study

Trends in management of patients with new-onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis

Aurelie Hanin et al. Epilepsia. 2024 Aug.

. 2024 Aug;65(8):e148-e155.

doi: 10.1111/epi.18014. Epub 2024 Jun 5.

Authors

Affiliations

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

PMID: 38837761
DOI: 10.1111/epi.18014

Abstract

In response to the evolving treatment landscape for new-onset refractory status epilepticus (NORSE) and the publication of consensus recommendations in 2022, we conducted a comparative analysis of NORSE management over time. Seventy-seven patients were enrolled by 32 centers, from July 2016 to August 2023, in the NORSE/FIRES biorepository at Yale. Immunotherapy was administered to 88% of patients after a median of 3 days, with 52% receiving second-line immunotherapy after a median of 12 days (anakinra 29%, rituximab 25%, and tocilizumab 19%). There was an increase in the use of second-line immunotherapies (odds ratio [OR] = 1.4, 95% CI = 1.1-1.8) and ketogenic diet (OR = 1.8, 95% CI = 1.3-2.6) over time. Specifically, patients from 2022 to 2023 more frequently received second-line immunotherapy (69% vs 40%; OR = 3.3; 95% CI = 1.3-8.9)-particularly anakinra (50% vs 13%; OR = 6.5; 95% CI = 2.3-21.0), and the ketogenic diet (OR = 6.8; 95% CI = 2.5-20.1)-than those before 2022. Among the 27 patients who received anakinra and/or tocilizumab, earlier administration after status epilepticus onset correlated with a shorter duration of status epilepticus (ρ = .519, p = .005). Our findings indicate an evolution in NORSE management, emphasizing the increasing use of second-line immunotherapies and the ketogenic diet. Future research will clarify the impact of these treatments and their timing on patient outcomes.

Keywords: age; etiology; immunotherapy; new‐onset refractory status epilepticus; outcome.

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References

REFERENCES

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Trends in management of patients with new-onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis

Affiliations

Trends in management of patients with new-onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis

Authors

Affiliations

Abstract

References

REFERENCES

Publication types

MeSH terms

Substances

Grants and funding

LinkOut - more resources

Full Text Sources