Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions

Lawrence J Hirsch; Nicolas Gaspard; Andreas van Baalen; Rima Nabbout; Sophie Demeret; Tobias Loddenkemper; Vincent Navarro; Nicola Specchio; Lieven Lagae; Andrea O Rossetti; Sara Hocker; Teneille E Gofton; Nicholas S Abend; Emily J Gilmore; Cecil Hahn; Houman Khosravani; Felix Rosenow; Eugen Trinka

doi:10.1111/epi.14016

Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions

Epilepsia. 2018 Apr;59(4):739-744. doi: 10.1111/epi.14016. Epub 2018 Feb 5.

Authors

Lawrence J Hirsch¹, Nicolas Gaspard², Andreas van Baalen³, Rima Nabbout⁴, Sophie Demeret⁵, Tobias Loddenkemper⁶, Vincent Navarro⁷, Nicola Specchio⁸, Lieven Lagae⁹, Andrea O Rossetti¹⁰, Sara Hocker¹¹, Teneille E Gofton¹², Nicholas S Abend¹³, Emily J Gilmore¹, Cecil Hahn¹⁴, Houman Khosravani^{15

16}, Felix Rosenow¹⁷, Eugen Trinka^{18

19}

Affiliations

¹ Comprehensive Epilepsy Center, Department of Neurology, Yale University, New Haven, CT, USA.
² Department of Neurology, Université Libre de Bruxelles - Hôpital Erasme, Bruxelles, Belgium.
³ Department of Neuropediatrics, Christian-Albrechts University, University Medical Center Schleswig-Holstein, Kiel, Germany.
⁴ Reference Center for Rare Epilepsies, Department of Child Neurology, Necker Enfants Malades Hospital, AP-HP, Paris Descartes University, Paris, France.
⁵ AP-HP, Neurological Intensive Care Unit, Pitié-Salpêtrière Hospital, Paris, France.
⁶ Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital & Harvard Medical School, Boston, MA, USA.
⁷ AP-HP, GH Pitie-Salpêtrière-Charles Foix, Epilepsy Unit, Brain and Spine Institute, Sorbonne University, UPMC University, Paris, France.
⁸ Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁹ Department of Development and Regeneration, Section of Pediatric Neurology, University Hospitals Leuven, Leuven, Belgium.
¹⁰ Department of Neurology, CHUV and University of Lausanne, Lausanne, Switzerland.
¹¹ Division of Critical Care Neurology, Department of Neurology, Mayo Clinic, Rochester, MN, USA.
¹² Department of Clinical Neurological Sciences, Western University, Schulich School of Medicine and Dentistry, London, ON, Canada.
¹³ Departments of Neurology and Pediatrics, Children's Hospital of Philadelphia and University of Pennsylvania, Philadelphia, PA, USA.
¹⁴ Division of Neurology, The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Toronto, ON, Canada.
¹⁵ Division of Neurology and Interdepartmental Division of Critical Care, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada.
¹⁶ Interdepartmental Division of Critical Care, Department of Medicine, University of Toronto, Toronto, ON, Canada.
¹⁷ Epilepsy Center Frankfurt Rhine-Main, Center of Neurology and Neurosurgery, Goethe-University Frankfurt, Frankfurt/Main, Germany.
¹⁸ Department of Neurology, Christian Doppler Medical Center, Paracelsus Medical University, Center for Cognitive Neuroscience, Salzburg, Austria.
¹⁹ Institute of Public Health, Department of Public Health, Health Services and HTA, UMIT, Hall in Tirol, Austria.

PMID: 29399791
DOI: 10.1111/epi.14016

Abstract

We convened an international group of experts to standardize definitions of New-Onset Refractory Status Epilepticus (NORSE), Febrile Infection-Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in-person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care. The proposed consensus definitions are as follows: NORSE is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause. FIRES is a subcategory of NORSE, applicable for all ages, that requires a prior febrile infection starting between 2 weeks and 24 hours prior to onset of refractory status epilepticus, with or without fever at onset of status epilepticus. Proposed consensus definitions are also provided for Infantile Hemiconvulsion-Hemiplegia and Epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus. This document has been endorsed by the Critical Care EEG Monitoring Research Consortium. We hope these consensus definitions will promote improved communication, permit multicenter research, and ultimately improve understanding and treatment of these conditions.

Keywords: autoimmune; cryptogenic; encephalitis; febrile infection-related epilepsy syndrome; febrile status epilepticus; infantile hemiconvulsion-hemiplegia and epilepsy syndrome; new-onset refractory status epilepticus; prolonged febrile convulsions; refractory status epilepticus; super-refractory status epilepticus.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Consensus*
Drug Resistant Epilepsy / classification*
Drug Resistant Epilepsy / diagnosis
Epileptic Syndromes / classification*
Epileptic Syndromes / diagnosis
Humans
Status Epilepticus / classification*
Status Epilepticus / diagnosis