New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives

Nicolas Gaspard, Lawrence J Hirsch, Claudine Sculier, Tobias Loddenkemper, Andreas van Baalen, Judette Lancrenon, Michel Emmery, Nicola Specchio, Raquel Farias-Moeller, Nora Wong, Rima Nabbout

Research output: Contribution to journalReview article

Abstract

We report the proceedings of the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. To promote awareness of this condition and foster research efforts, we conveyed the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. The conference was supported by The NORSE Institute (http://www.norseinstitute.org). This article summarizes the discussions that were held during the Symposium and presents our strategy to unravel the cause of these disorders and to improve patient care. The standardized definitions for these disorders that have been developed, are required to improve communication and facilitate the development of multicenter registries and biobanks. A distinction between childhood- and adult-onset forms of the syndrome is not supported by strong scientific evidence and it is argued that both should be studied together. Although the pathophysiology remains elusive, nascent evidence suggests a role for a postinfectious cytokine-mediated mechanism, which should be further investigated. It also appears important to develop tools for their early recognition and prompt treatment. Recent evidence suggests that specific electroencephalography (EEG) features might be helpful. The optimal treatment options remain to be determined; immune therapies are usually disappointing, but the ketogenic diet has proved effective in uncontrolled trials. NORSE and FIRES represent a very delicate clinical situation with specific communication issues between physicians and with patients and families. Standardized consensus definitions and a multidisciplinary multicenter strategy will help research efforts and improve clinical care for patients with NORSE and FIRES.

Original languageEnglish
Pages (from-to)745-752
Number of pages8
JournalEpilepsia
Volume59
Issue number4
DOIs
Publication statusPublished - Apr 2018

Fingerprint

Status Epilepticus
Epilepsy
Fever
Infection
Patient Care
Communication
Ketogenic Diet
Research
Registries
Electroencephalography
Therapeutics
Cytokines
Physicians

Keywords

  • Age Factors
  • Consensus
  • Drug Resistant Epilepsy/classification
  • Electroencephalography/methods
  • Epileptic Syndromes/classification
  • Humans
  • Status Epilepticus/classification

Cite this

Gaspard, N., Hirsch, L. J., Sculier, C., Loddenkemper, T., van Baalen, A., Lancrenon, J., ... Nabbout, R. (2018). New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia, 59(4), 745-752. https://doi.org/10.1111/epi.14022

New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) : State of the art and perspectives. / Gaspard, Nicolas; Hirsch, Lawrence J; Sculier, Claudine; Loddenkemper, Tobias; van Baalen, Andreas; Lancrenon, Judette; Emmery, Michel; Specchio, Nicola; Farias-Moeller, Raquel; Wong, Nora; Nabbout, Rima.

In: Epilepsia, Vol. 59, No. 4, 04.2018, p. 745-752.

Research output: Contribution to journalReview article

Gaspard, N, Hirsch, LJ, Sculier, C, Loddenkemper, T, van Baalen, A, Lancrenon, J, Emmery, M, Specchio, N, Farias-Moeller, R, Wong, N & Nabbout, R 2018, 'New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives', Epilepsia, vol. 59, no. 4, pp. 745-752. https://doi.org/10.1111/epi.14022
Gaspard, Nicolas ; Hirsch, Lawrence J ; Sculier, Claudine ; Loddenkemper, Tobias ; van Baalen, Andreas ; Lancrenon, Judette ; Emmery, Michel ; Specchio, Nicola ; Farias-Moeller, Raquel ; Wong, Nora ; Nabbout, Rima. / New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) : State of the art and perspectives. In: Epilepsia. 2018 ; Vol. 59, No. 4. pp. 745-752.
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abstract = "We report the proceedings of the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. To promote awareness of this condition and foster research efforts, we conveyed the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. The conference was supported by The NORSE Institute (http://www.norseinstitute.org). This article summarizes the discussions that were held during the Symposium and presents our strategy to unravel the cause of these disorders and to improve patient care. The standardized definitions for these disorders that have been developed, are required to improve communication and facilitate the development of multicenter registries and biobanks. A distinction between childhood- and adult-onset forms of the syndrome is not supported by strong scientific evidence and it is argued that both should be studied together. Although the pathophysiology remains elusive, nascent evidence suggests a role for a postinfectious cytokine-mediated mechanism, which should be further investigated. It also appears important to develop tools for their early recognition and prompt treatment. Recent evidence suggests that specific electroencephalography (EEG) features might be helpful. The optimal treatment options remain to be determined; immune therapies are usually disappointing, but the ketogenic diet has proved effective in uncontrolled trials. NORSE and FIRES represent a very delicate clinical situation with specific communication issues between physicians and with patients and families. Standardized consensus definitions and a multidisciplinary multicenter strategy will help research efforts and improve clinical care for patients with NORSE and FIRES.",
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