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. 2022 Aug 23;63(11):2840-2864.
doi: 10.1111/epi.17397. Online ahead of print.

International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) incl. Febrile Infection-Related Epilepsy Syndrome (FIRES): Statements and Supporting Evidence

Ronny Wickstrom  1 , Olga Taraschenko  2 , Robertino Dilena  3 , Eric T Payne  4 , Nicola Specchio  5 , Rima Nabbout  6 , Sookyong Koh  7 , Nicolas Gaspard  8 , Lawrence J Hirsch  9 ; International NORSE Consensus Group
Collaborators, Affiliations

International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) incl. Febrile Infection-Related Epilepsy Syndrome (FIRES): Statements and Supporting Evidence

Ronny Wickstrom et al. Epilepsia. .

Abstract

Objective: To develop consensus-based recommendations for the management of adult and paediatric patients with NORSE/FIRES based on best evidence and experience.

Methods: The Delphi methodology was followed. A facilitator group of 9 experts was established, who defined the scope, users and suggestions for recommendations. Following a review of the current literature, recommendation statements concerning diagnosis, treatment and research directions were generated which were then voted on a scale of 1 (strongly disagree) to 9 (strongly agree) by a panel of 48 experts in the field. Consensus that a statement was appropriate was reached if the median score was greater or equal to 7, and inappropriate if the median score was less than or equal to 3. The analysis of evidence was mapped to the results of each statement included in the Delphi survey.

Results: Overall, 85 recommendation statements achieved consensus. The recommendations are divided into five sections: 1) disease characteristics, 2) diagnostic testing and sampling, 3) acute treatment, 4) treatment in the post-acute phase, and 5) research, registries and future directions in NORSE/FIRES. The detailed results and discussion of all 85 statements are outlined herein. A corresponding summary of findings and practical flowsheets are presented in a companion article.

Significance: This detailed analysis offers insight into the supporting evidence and the current gaps in the literature that are associated with expert consensus statements related to NORSE/FIRES. The recommendations generated by this consensus can be used as a guide for the diagnosis, evaluation, and management of patients with NORSE/FIRES, and for planning of future research.

Keywords: Adult; Anti-seizure medication; Delphi; Epilepsy; Immunotherapy; Ketogenic diet; Pediatric; Refractory status epilepticus; Status epilepticus.

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Conflict of interest statement

R.W. has served on scientific advisory boards for GW Pharma and Octapharma; and has received speaker honoraria from Eisai and Sanofi. O.T. was supported by a research grant from the National Institutes of Health (P20GM130447). R.D. has received a speaker honorarium fee from Sobi. E.T.P. has received a speaker honorarium from Eisai. S.K. has served on scientific advisory boards for Zogenix and Neurelis. L.J.H. has received consultation fees for advising from Accure, Aquestive, Ceribell, Eisai, Marinus, Medtronic, Neurelis, Neuropace, and UCB; royalties from Wolters‐Kluwer for authoring chapters for UpToDate–Neurology and from Wiley for coauthoring the book Atlas of EEG in Critical Care by Hirsch and Brenner; and honoraria for speaking from Neuropace, Natus, and UCB. NS has served on scientific advisory boards for GW Pharma, BioMarin, Arvelle, Marinus, and Takeda; has received speaker honoraria from Eisai, BioMarin, LivaNova, and Sanofi; and has served as an investigator for Zogenix, Marinus, BioMarin, UCB, and Roche. R.N. and N.G. have no disclosures for this study. The present study is not industry‐sponsored. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.

References

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