International consensus recommendations for management of new onset refractory status epilepticus including febrile infection‐related epilepsy syndrome: Statements and supporting evidence

Abstract

Objective This study was undertaken 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 evidence and experience. 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, which were then rated 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 ≥7 and inappropriate if the median score was ≤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 postacute 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.

International consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile infection‐related epilepsy syndrome (FIRES): Summary and clinical tools

“…Experience was >13 years for 78% of respondents, 10–12 years for 8%, 7–9 years for 6%, and 4–6 years for 8%. Participants completed two rounds to develop a set of consensus statements following an initial pre‐questionnaire (details concerning how consensus statements were reached can be found in the longer and complete document) 9 . NORSE and FIRES were considered jointly for all ages.…”

“…Participants completed two rounds to develop a set of consensus statements following an initial pre‐questionnaire (details concerning how consensus statements were reached can be found in the longer and complete document). 9 NORSE and FIRES were considered jointly for all ages. The statements were divided into five sections.…”

Does the ketogenic ratio matter when using ketogenic diet therapy in pediatric epilepsy?

“…For some medical conditions, achieving a high level of ketosis as quickly as possible may be of paramount importance, for example, in super‐refractory status epilepticus or new‐onset infantile spasms 43 . The risk of gastroparesis, aspiration pneumonia, and other systemic side effects in the intensive‐care setting may limit the use of high ratios of the KD in the setting of super‐refractory status epilepticus.…”

The nuances of immunotherapy for NORSE/FIRES

“…The nuances of immunotherapy for NORSE/FIRES Dear Editors, I read with interest the consensus-based recommendations for management of new-onset refractory status epilepticus (NORSE) including febrile infection-related epilepsy syndrome (FIRES) authored by Wickstrom and colleagues. 1,2 The consensus recommendations not only crystallized the experiences/opinions from an international panel of experts but also identified knowledge gaps in this field. Among other issues, the authors draw attention to the putative neuroinflammatory mechanisms underlying NORSE/FIRES, as reflected by the recommended investigations and timeline of immunotherapies.…”