Diagnostic Evaluation, Identification, and Treatment Recommendations for Seizure Control in NORSE & FIRES
The following are recommendations for the evaluation, identification, and treatment of New Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES). In varying degrees they all include diagnostic guidelines, with recommendations for blood/CSF/Genetic testing, EEGs, treatments for seizure suppression including ketogenic diet, immune and neuromodulation alternatives (anakinra, tocilizumab, IT-Dex and JAK inhibitors), initiating palliative care, and post-ICU or chronic phase evaluation and treatment recommendations.
International Consensus Recommendations
The first consensus guideline for NORSE and FIRES management based on a Delphi study of 48 experts in NORSE/FIRES. Includes 85 consensus statements on diagnosis, acute and post-acute treatment, and research priorities.
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, Epilepsia, 23 August 2022 https://doi.org/10.1111/epi.17397
Families can read a short version of the article here: https://onlinelibrary.wiley.com/doi/10.1111/epi.17391
NORSE Diagnostic Evaluation
A suggested guideline for the diagnostic investigation of NORSE and FIRES covering recommended laboratory, imaging, CSF, genetic, metabolic, and autoimmune testing.
The NORSE Institute gratefully acknowledges its Medical Advisory Board, and in particular Drs. Nicolas Gaspard, Claudine Sculier, Lawrence J Hirsch, Sara Hocker, Zubeda Sheikh, Shivani Ghoshal, and Yashwanth Pulluru for their contributions.
Disclaimer: This is not a complete list of tests, and should be adapted to the patient and institutional setting.
Additions to this checklist are welcome and can be sent via Contact page. (Updated: 2020)
In-Hospital Management of NORSE/FIRES
Provides an evaluation decision tree and broad guideline for multidisciplinary assessments and treatments in the ICU.
Sheikh Z, Hirsch LJ. A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome. Front Neurol. 2023 May 12;14:1150496. doi: 10.3389/fneur.2023.1150496. Erratum in: Front Neurol. 2024 Nov 11;15:1509148. doi: 10.3389/fneur.2024.1509148. PMID: 37251223; PMCID: PMC10213694.
Sample Treatment Algorithm
For treatment of adult patients in generalized convulsive status epilepticus.
Disclaimer: This treatment algorithm for adult patients in generalized convulsive status epilepticus is used at Yale New Haven Hospital, and generously shared for centers without an SE algorithm. Posted with the permission of Lawrence J. Hirsch, MD. The NORSE Institute is grateful to Dr. Hirsch, Abdalla Ammar, PharmD, Emily J. Gilmore, MD; the Yale New Haven Hospital Department of Pharmacy Services; and the Yale University Dept of Neurology.
Comments are welcome and can be sent to us via the Contact page. (Updated: 2023)