2024
Comparative analysis of patients with new onset refractory status epilepticus preceded by fever (febrile infection‐related epilepsy syndrome) versus without prior fever: An interim analysis
Jimenez A, Gopaul M, Asbell H, Aydemir S, Basha M, Batra A, Damien C, Day G, Eka O, Eschbach K, Fatima S, Fields M, Foreman B, Gerard E, Gofton T, Haider H, Hantus S, Hocker S, Jongeling A, Aparicio M, Kandula P, Kang P, Kazazian K, Kellogg M, Kim M, Lee J, Marcuse L, McGraw C, Mohamed W, Orozco J, Pimentel C, Punia V, Ramirez A, Steriade C, Struck A, Taraschenko O, Treister A, Yoo J, Zafar S, Zhou D, Zutshi D, Gaspard N, Hirsch L, Hanin A. Comparative analysis of patients with new onset refractory status epilepticus preceded by fever (febrile infection‐related epilepsy syndrome) versus without prior fever: An interim analysis. Epilepsia 2024, 65: e87-e96. PMID: 38625055, DOI: 10.1111/epi.17988.Peer-Reviewed Original ResearchFebrile infection-related epilepsy syndromeRefractory status epilepticusMacrophage inflammatory protein-1 alphaOnset refractory status epilepticusStatus epilepticusCerebrospinal fluidFIRES patientsCerebrospinal fluid interleukin-6Magnetic resonance imaging findingsComparative analysis of patientsAnalysis of patientsChemokine ligand 2Presenting symptomsFebrile infectionsClinical featuresGastrointestinal prodromeImaging findingsInflammatory biomarkersAntiseizure medicationsInterleukin-6Epilepsy syndromesInterim analysisPatientsEpilepticusFeverSecond‐line immunotherapy in new onset refractory status epilepticus
Hanin A, Muscal E, Hirsch L. Second‐line immunotherapy in new onset refractory status epilepticus. Epilepsia 2024, 65: 1203-1223. PMID: 38430119, DOI: 10.1111/epi.17933.Peer-Reviewed Original ResearchFebrile infection-related epilepsy syndromeSecond-line immunotherapyOnset refractory status epilepticusRefractory status epilepticusIntrathecal dexamethasoneStatus epilepticusCytokine levelsFunctional outcomesCerebrospinal fluid cytokine levelsInitiation of anakinraReduction of seizure frequencyLong-term functional outcomeResponse to treatmentSE onsetElevated serumFebrile infectionsImmune dysregulationLong-term disabilityTreatment initiationCase reportCytokine measurementsProspective studyAnakinraImmunotherapyTocilizumab
2022
International consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile infection‐related epilepsy syndrome (FIRES): Summary and clinical tools
Wickström R, Taraschenko O, Dilena R, Payne ET, Specchio N, Nabbout R, Koh S, Gaspard N, Hirsch LJ, Group T, Auvin S, van Baalen A, Beghi E, Benseler S, Bergin P, Bleck T, Brunklaus A, Caraballo R, Cervenka M, Costello D, Drislane F, Farias‐Moeller R, Gallantine W, Gilmore E, Gofton T, Jimenez M, Hocker S, Kaliakatsos M, Kellogg M, Lee J, Loddenkemper T, Meletti S, Mizugushi M, Muscal E, Riviello J, Rosenthal E, Rossetti A, Ruegg S, Said R, Sculier C, Schmitt S, Schuele S, Stredny C, Trinka E, Wainwright M, VanHaerents S, Wells E, Wirrell E, Zuberi S. International consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile infection‐related epilepsy syndrome (FIRES): Summary and clinical tools. Epilepsia 2022, 63: 2827-2839. PMID: 35951466, PMCID: PMC9826478, DOI: 10.1111/epi.17391.Peer-Reviewed Original ResearchFebrile infection-related epilepsy syndromeNew-onset refractory status epilepticusAcute treatmentRecommendation statementMedian scoreRefractory status epilepticusManagement of adultsManagement of patientsPost-acute phaseConsensus-based recommendationsInternational consensus recommendationsBest available evidenceEvidence-based analysisPediatric patientsStatus epilepticusEpilepsy syndromesDisease characteristicsConsensus recommendationsDiagnostic testingClinical toolAvailable evidenceDiagnosisPatientsTreatmentDelphi methodology
2021
How to Help Your Patients Enroll in the New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES) Family Registry, and Other Rare Epilepsy Registries.
Kazazian K, Kellogg M, Wong N, Eschbach K, Moeller RF, Gaspard N, Hirsch LJ, Hocker S, Gofton T. How to Help Your Patients Enroll in the New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES) Family Registry, and Other Rare Epilepsy Registries. Epilepsy Currents 2021, 1535759721998329. PMID: 33663265, DOI: 10.1177/1535759721998329.Peer-Reviewed Original ResearchFebrile infection-related epilepsy syndromeNew-onset refractory status epilepticusRefractory status epilepticusStatus epilepticusFamily RegistryRare clinical presentationPast medical historyQuality of lifeEpilepsy registryFebrile infectionsActive epilepsyDisease courseClinical outcomesClinical presentationEpilepsy syndromesMedical historyNeurologic disordersRigorous research studiesEpilepticusRegistryPatient enrollsFamily membersOutcomesMorbidityEpilepsy
2019
New onset refractory status epilepticus research: What is on the horizon?
Gofton TE, Gaspard N, Hocker SE, Loddenkemper T, Hirsch LJ. New onset refractory status epilepticus research: What is on the horizon? Neurology 2019, 92: 802-810. PMID: 30894443, DOI: 10.1212/wnl.0000000000007322.Peer-Reviewed Original ResearchConceptsNew-onset refractory status epilepticusRefractory status epilepticusStatus epilepticusFebrile infection-related epilepsy syndromeMore rescue medicationsRescue medicationNew onsetActive epilepsyClinical featuresClinical presentationEpilepsy syndromesMetabolic causesNeurologic disordersPediatric EvaluationSpecific diagnosisAge groupsEpilepticusFamily supportFuture collaborative researchMedicationsPatientsFeverEpilepsyPathophysiologySyndrome
2018
New‐onset refractory status epilepticus (NORSE) and febrile infection–related epilepsy syndrome (FIRES): State of the art and perspectives
Gaspard N, Hirsch LJ, Sculier C, Loddenkemper T, van Baalen A, Lancrenon J, Emmery M, Specchio N, Farias‐Moeller R, Wong N, Nabbout R. New‐onset refractory status epilepticus (NORSE) and febrile infection–related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia 2018, 59: 745-752. PMID: 29476535, DOI: 10.1111/epi.14022.Peer-Reviewed Original ResearchConceptsNew-onset refractory status epilepticusFebrile infection-related epilepsy syndromeRefractory status epilepticusStatus epilepticusCytokine-mediated mechanismOptimal treatment optionsAdult-onset formStrong scientific evidenceStandardized consensus definitionsMulticenter registryPrompt treatmentKetogenic dietUncontrolled trialsEpilepsy syndromesImmune therapyTreatment optionsEarly recognitionClinical careConsensus definitionClinical situationsPatient careEpilepticusStandardized definitionsElectroencephalography featuresScientific evidenceProposed consensus definitions for new‐onset refractory status epilepticus (NORSE), febrile infection‐related epilepsy syndrome (FIRES), and related conditions
Hirsch LJ, Gaspard N, van Baalen A, Nabbout R, Demeret S, Loddenkemper T, Navarro V, Specchio N, Lagae L, Rossetti AO, Hocker S, Gofton TE, Abend NS, Gilmore EJ, Hahn C, Khosravani H, Rosenow F, Trinka E. Proposed consensus definitions for new‐onset refractory status epilepticus (NORSE), febrile infection‐related epilepsy syndrome (FIRES), and related conditions. Epilepsia 2018, 59: 739-744. PMID: 29399791, DOI: 10.1111/epi.14016.Peer-Reviewed Original ResearchConceptsNew-onset refractory status epilepticusFebrile infection-related epilepsy syndromeRefractory status epilepticusStatus epilepticusConsensus definitionEpilepsy syndromesFebrile infectionsCritical Care EEG Monitoring Research ConsortiumSuper-refractory status epilepticusRelated conditionsHemiconvulsion-HemiplegiaNew onsetActive epilepsyImproved communicationClinical presentationNeurocritical careMetabolic causesPediatric expertsEpilepticusRelevant neurological disordersConsensus conferenceSpecific diagnosisNeurological disordersMulticenter researchSyndrome