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, Olga Taraschenko, Robertino Dilena, Eric T Payne, Nicola Specchio, Rima Nabbout, Sookyong Koh, Nicolas Gaspard, Lawrence J Hirsch, International NORSE Consensus Group
Author Information
Ronny Wickstrom: Neuropaediatric Unit, Department of Women´s and Children´s Health, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
Olga Taraschenko: University of Nebraska Medical Center, Department of Neurological Sciences, Omaha, NE, USA.
Robertino Dilena: Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Neuropathophysiology Unit, Milan, Italy.
Eric T Payne: Alberta Children's Hospital, Department of Pediatrics, Section of Neurology. Calgary, Canada.
Nicola Specchio: Rare and Complex Epilepsy Unit, Department of Neurosciences, Bambino Gesù Children's Hospital, IRCCS, Rome, Full Member of European Reference Network EpiCARE, Italy.
Rima Nabbout: Centre de reference epilepsies rares, department of pediatric Neurology, APHP, member of EPICARE ERN, Universite de Paris, Institut Imagine, INSERM 1163, Paris, France.
Sookyong Koh: Children's Hospital and Medical Center, Department of Pediatrics, University of Nebraska, Omaha, NE, USA.
Nicolas Gaspard: Université Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium.
Lawrence J Hirsch: Comprehensive Epilepsy Center, Department of Neurology, Yale University, New Haven, CT, USA.
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.
