@article{986d38f8f2b14acfa11844c1b4b8746e,
title = "International consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile infection-related epilepsy syndrome (FIRES): Summary and clinical tools",
abstract = "Objective: To develop consensus-based recommendations for the management of adult and pediatric patients with new-onset refractory status epilepticus (NORSE)/febrile infection-related epilepsy syndrome (FIRES) based on best available evidence and expert opinion. Methods: The Delphi methodology was followed. A facilitator group of nine 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 that were then voted on using 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 than or equal to 7, and inappropriate if the median score was less than or equal to 3. 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. These are summarized in this article along with two practical clinical flowsheets: one for diagnosis and evaluation and one for acute treatment. A corresponding evidence-based analysis of all 85 recommendations alongside responses by the Delphi panel is presented in a companion article. Significance: 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 = "Delphi, adult, anti-seizure medication, epilepsy, immunotherapy, ketogenic diet, pediatric, refractory, status epilepticus",
author = "{the International NORSE Consensus Group} and Ronny Wickstr{\"o}m and Olga Taraschenko and Robertino Dilena and Payne, {Eric T.} and Nicola Specchio and Rima Nabbout and Sookyong Koh and Nicolas Gaspard and Hirsch, {Lawrence J.} and Stephane Auvin and {van Baalen}, Andreas and Ettore Beghi and Benseler, {Susanne M.} and Peter Bergin and Tom Bleck and Andreas Brunklaus and Caraballo, {Roberto H.} and Mackenzie Cervenka and Daniel Costello and Frank Drislane and Raquel Farias-Moeller and William Gallantine and Gilmore, {Emily J.} and Teneille Gofton and Jimenez, {Maria Angeles Perez} and Sara Hocker and Marios Kaliakatsos and Marissa Kellogg and Lee, {Jong Woo} and Tobias Loddenkemper and Stefano Meletti and Masashi Mizugushi and Eyal Muscal and Riviello, {James J.} and Rosenthal, {Eric S.} and Rossetti, {Andrea O.} and Stefan Ruegg and Rana Said and Claudine Sculier and Sarah Schmitt and Stephan Schuele and Coral Stredny and Eugen Trinka and Mark Wainwright and Stephen VanHaerents and Elisabeth Wells and Elaine Wirrell and Zuberi, {Sameer M.}",
note = "Funding Information: RW was supported by Region Stockholm (clinical research appointment) and by research grants from StratNeuro, Karolinska Institutet. OT was supported by research grants from the National Institutes of Health (NIH) (P20GM130447) and the American Epilepsy Society‐NORSE Institute Seed grant. SK was supported by American Epilepsy Society‐NORSE Institute Seed grant. RD was supported by Foundation IRCCS Ca′ Granda Ospedale Maggiore Policlinico Milan (Epiexome grant). ETP was supported by American Epilepsy Society‐NORSE Institute grant. Funding Information: RW has served on scientific advisory boards for GW Pharma and Octapharma and has received speaker honoraria from Eisai and Sanofi. OT was supported by research grant from the National Institutes of Health (NIH) (P20GM130447). RD has received speaker honorarium fee from Sobi. ETP has received a speaker honorarium from Eisai. RN has no disclosures for this study. SK has served on scientific advisory boards for Zogenix and Neurelis. NG has no disclosures for this study. LJH 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 co‐authoring 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. RW, OT, RN, SK, NG, and LJH are members of the Scientific Board of the NORSE Institute. 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. Publisher Copyright: {\textcopyright} 2022 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.",
year = "2022",
month = nov,
doi = "10.1111/epi.17391",
language = "English (US)",
volume = "63",
pages = "2827--2839",
journal = "Epilepsia",
issn = "0013-9580",
publisher = "Wiley-Blackwell",
number = "11",
}