É uma doença rara e potencialmente fatal, uma condição cerebral grave causada por epilepsia. Ela se caracteriza pelo início súbito e muito intenso de crises convulsivas tônico-clônicas (que afetam o corpo todo) que se repetem e atingem várias áreas e os dois lados do cérebro. Tudo isso acontece depois de uma doença com febre sem uma causa específica. A síndrome se desenvolve sem uma causa aguda e óbvia (como um problema na estrutura do cérebro, uma toxina ou uma alteração no metabolismo do corpo), em um paciente que nunca teve epilepsia antes. A FIRES é um subtipo do que chamamos de "estado de mal epiléptico refratário de novo início" (NORSE). Isso significa crises epilépticas prolongadas e difíceis de controlar, que aparecem pela primeira vez. Para ser considerada FIRES, é obrigatório que o paciente tenha tido uma infecção com febre antes.
Introdução
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É uma doença rara e potencialmente fatal, uma condição cerebral grave causada por epilepsia. Ela se caracteriza pelo início súbito e muito intenso de crises convulsivas tônico-clônicas (que afetam o corpo todo) que se repetem e atingem várias áreas e os dois lados do cérebro. Tudo isso acontece depois de uma doença com febre sem uma causa específica. A síndrome se desenvolve sem uma causa aguda e óbvia (como um problema na estrutura do cérebro, uma toxina ou uma alteração no metabolismo do corpo), em um paciente que nunca teve epilepsia antes. A FIRES é um subtipo do que chamamos de "estado de mal epiléptico refratário de novo início" (NORSE). Isso significa crises epilépticas prolongadas e difíceis de controlar, que aparecem pela primeira vez. Para ser considerada FIRES, é obrigatório que o paciente tenha tido uma infecção com febre antes.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 6 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 12 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome epiléptico relacionado com infecção febril
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Publicações mais relevantes
From clinical practice to mechanistic insights in ketogenic diets for epilepsy.
Ketogenic diet therapies, including the classic ketogenic diet, modified Atkins diet, and low glycaemic index treatment, have shown effectiveness in controlling seizures, in part by shifting metabolism from glucose to ketone bodies. They improve mitochondrial function, reduce neuroinflammation, and modulate neurotransmitters. Ketogenic diet therapies also affect the gut microbiome, potentially impacting neurotransmitter balance in ways that contribute to seizure control. A classic ketogenic diet is effective yet restrictive, whereas the modified Atkins diet and low glycaemic index treatment offer greater flexibility, tolerability, and ease of implementation, particularly in resource-limited settings. Cochrane reviews and meta-analyses rank the certainty of randomised controlled trial evidence for ketogenic diet therapies as limited. Early initiation of ketogenic diet therapies, particularly in children or patients with metabolic epilepsies, improves seizure outcomes, potentially preventing further mitochondrial and neuronal damage and reducing the risk of developing resistance to antiseizure medications. Research using rigorous, large-scale comparative effectiveness study designs that accounts for differences in age, epilepsy type, dietary therapy modality, sociodemographic background, care delivery contexts, and that minimises performance and observation bias is needed to resolve remaining uncertainties regarding the efficacy and real-world challenges of ketogenic diet therapies in epilepsy.
[Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
A 55-year-old woman presented to our clinic with refractory status epilepticus, which developed after her symptoms of influenza had initially improved. Despite various tests, the definite cause could not be identified; however, her clinical presentation preceded by a febrile infection was consistent with that of febrile infection-related epilepsy syndrome (FIRES), a subtype of new-onset refractory status epilepticus (NORSE). Because the brain MRI on day seven revealed a claustrum sign, we administered aggressive immunotherapy. Thereafter, the patient recovered from status epilepticus, and the claustrum sign disappeared on brain MRI. Although the retrograde amnesia and chronic epilepsy persisted, the patient recovered and was able to live at home upon discharge. In patients with refractory status epilepticus that develops after a preceding infection, FIRES should be considered in the differential diagnosis regardless of age at onset, and early aggressive immunotherapy should be considered, especially in patients with a claustrum sign.
An increase in cerebrospinal fluid macrophage migration inhibitory factor levels in pediatric patients with acute neuroinflammatory diseases: A preliminary study.
Macrophage migration inhibitory factor (MIF) is a multifaceted cytokine involved in both the innate and adaptive immune systems. The roles of MIF in neuroinflammatory diseases have been described in adults, but remain unclear in the pediatric population. We herein retrospectively investigated the cerebrospinal fluid (CSF) levels of MIF and 19 types of related cytokines in pediatric patients with acute neuroinflammatory diseases, i.e., bacterial meningitis (n = 6), aseptic meningitis/encephalitis (n = 8), and febrile infection-related epilepsy syndrome (FIRES) (n = 5), and in patients with febrile seizure (n = 15). CSF MIF levels were significantly higher in the three neuroinflammatory disease groups, particularly in the bacterial meningitis group, than in the febrile seizure group. CSF MIF levels were significantly higher in the bacterial meningitis group than in the FIRES group. CSF MIF levels were higher in the FIRES group than in the febrile seizure group, and this result was independent of age. CSF MIF levels correlated more strongly with interleukin-10, an M2 promoter, than with interferon-γ, an M1 promoter. CSF MIF levels were elevated in pediatric patients with acute neuroinflammatory diseases. This is the first study to compare differences in CSF MIF levels among neuroinflammatory diseases and to show relationships between MIF and related cytokines.
[Expert consensus on the diagnosis and treatment of pediatric febrile infection-related epilepsy syndrome (2026)].
热性感染相关性癫痫综合征属于感染诱发性脑病综合征相关范畴,以儿童多见,急性期以发热性感染后迅速进展的超难治性癫痫持续状态为特征,慢性期常表现为药物难治性癫痫伴高级皮质功能障碍。在临床工作中,儿科医生对该疾病的认识不足,国内尚缺乏规范统一的诊疗方案。中华医学会儿科学分会神经学组、中华医学会儿科学分会急救学组和中华儿科杂志编辑委员会联合制订了“儿童热性感染相关性癫痫综合征诊疗专家共识(2026)”,提出17条推荐意见,旨在进一步规范该疾病的临床诊治。.
Status Epilepticus in Children With Hemophagocytic Lymphohistiocytosis: Literature Review.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder involving systemic inflammation and multi-organ damage, which often affects the central nervous system (CNS). Familial HLH, which is caused by mutations in genes such as PRF1 and UNC13D, impairs the function of cytotoxic cells, leading to uncontrolled immune activation. CNS involvement occurs in 30% to 73% of cases and manifests as seizures, encephalopathy or status epilepticus, which often mimics other neurologic conditions. This article presents the case of a 28-month-old boy with PRF1-related HLH who developed super-refractory status epilepticus during CNS relapse, despite having undergone a prior hematopoietic stem cell transplantation (HSCT). An initial MRI scan revealed diffuse cortical and cerebellar abnormalities, whereas systemic HLH findings emerged later. Treatment included antiseizure medications, immunotherapy and etoposide, but severe neurologic deficits persisted. A review of the literature on 20 pediatric HLH cases presenting with status epilepticus revealed a variety of presentations, including focal, generalized and febrile infection-related epilepsy syndrome (FIRES). Neuroimaging showed white matter lesions, atrophy or diffusion abnormalities, whereas CSF findings ranged from normal to elevated protein and neopterin levels. Mortality was high (45%), with survivors often experiencing cognitive or motor impairments. HLH relapse can initially present with isolated CNS involvement, emphasizing the need for early neuroimaging and cerebrospinal fluid (CSF) analysis in suspected cases. Despite aggressive treatment, outcomes remain poor, highlighting the need for further research into optimal management strategies.
Publicações recentes
[Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
Metabolism-corrected propofol exposure intensity and long-term intelligence quotient in pediatric febrile infection-related epilepsy syndrome: a retrospective cohort study.
An increase in cerebrospinal fluid macrophage migration inhibitory factor levels in pediatric patients with acute neuroinflammatory diseases: A preliminary study.
Extreme cryptogenic new onset refractory status epilepticus/febrile infection-related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury.
Pediatric new-onset super refractory status epilepticus: Potential of timely immunotherapy in a resource-limited PICU.
📚 EuropePMC148 artigos no totalmostrando 197
From clinical practice to mechanistic insights in ketogenic diets for epilepsy.
The Lancet. Neurology[Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
Rinsho shinkeigaku = Clinical neurologyMetabolism-corrected propofol exposure intensity and long-term intelligence quotient in pediatric febrile infection-related epilepsy syndrome: a retrospective cohort study.
Frontiers in medicineAn increase in cerebrospinal fluid macrophage migration inhibitory factor levels in pediatric patients with acute neuroinflammatory diseases: A preliminary study.
Brain & developmentExtreme cryptogenic new onset refractory status epilepticus/febrile infection-related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury.
EpilepsiaPediatric new-onset super refractory status epilepticus: Potential of timely immunotherapy in a resource-limited PICU.
Seizure[Expert consensus on the diagnosis and treatment of pediatric febrile infection-related epilepsy syndrome (2026)].
Zhonghua er ke za zhi = Chinese journal of pediatricsStatus Epilepticus in Children With Hemophagocytic Lymphohistiocytosis: Literature Review.
Journal of child neurologySecondary hemophagocytic lymphohistiocytosis concurrent with febrile infection-related epilepsy syndrome in a child.
SeizureEfficacy differences between tocilizumab and ketogenic diet during acute phase of febrile Infection-Related epilepsy syndrome in children: A retrospective cohort study.
Epilepsy & behavior : E&BFebrile infection-related epilepsy syndrome (FIRES) in adults: a case report and review of factors associated with survival.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyLidocaine as a potential anti-seizure therapy for pediatric FIRES: a promising therapeutic approach.
Frontiers in pediatricsKetogenic Diet Therapy for Adult Febrile Infection-Related Epilepsy Syndrome (FIRES): A Case Series of Five Patients and Clinical Efficacy Analysis.
Infection and drug resistanceStatus Epilepsy Syndromes Made Easy: Pediatric Perspectives.
Children (Basel, Switzerland)The symmetrical claustrum sign in pediatric febrile infection-related epilepsy syndrome: Diagnostic value and clinical implications.
EpilepsiaTocilizumab for super-refractory status epilepticus in children with FIRES: A case series.
SeizureCase Report: Successful multimodal management of FIRES in a pediatric patient using anakinra, ketogenic diet, and induced hypothermia.
Frontiers in pediatricsBeyond Anakinra and Tocilizumab: Additional Adjunctive Therapies in Pediatric New Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome - A Narrative Review.
Pediatric neurologyThe spectrum of pediatric acute leukoencephalopathy with restricted diffusion presenting as febrile infection-related refractory epilepsy syndrome.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyA Case Report of Bartonella henselae-Related New-Onset Refractory Status Epilepticus / Febrile Infection-Related Epilepsy Syndrome Complicated With Stimulus-Induced Rhythmic, Periodic, or Ictal Discharges.
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Frontiers in immunologyFebrile Infection-Related Epilepsy Syndrome (FIRES) in a Young Adult: A Case Report Highlighting Advanced Neuroimaging.
Clinical neuroradiologyVagus nerve stimulation combined with ketogenic diet versus ketogenic diet alone in the treatment of febrile infection-related epilepsy syndrome in children: efficacy and long-term prognosis study.
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EpilepsiaQuality of life over time after new onset refractory status epilepticus.
EpilepsiaHuman herpes virus-7-related severe encephalitis diagnosed using mNGS in immunocompetent pediatric patients.
Virology journalStandard complete blood count to predict long-term outcomes in febrile infection-related epilepsy syndrome (FIRES): A multicenter study.
EpilepsiaGastrointestinal symptoms are common in patients with new-onset refractory status epilepticus (NORSE).
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyUpdate in new-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.
Current opinion in pediatrics[Febrile infection-related epilepsy syndrome].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaCSF IL-6 in children with neuroinflammatory conditions.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyImmune dysregulation in new-onset refractory status epilepticus (NORSE): current insights and therapeutic perspectives.
Brain & development[Febrile infection-related epilepsy syndrome caused by hemophagocytic lymphohistiocytosis: a case report].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsClinical characteristics of 27 children with febrile infection-related epilepsy syndrome in a single center.
Pediatric discoveryRetrospective multicenter study on cryptogenic NORSE/FIRES patients treated with anakinra.
SeizureAdult-Onset Febrile Infection-Related Epilepsy Syndrome Treated with Short-Term Anakinra.
Journal of epilepsy researchObservational Study of Tocilizumab in Children With Febrile Infection-Related Epilepsy Syndrome.
Annals of clinical and translational neurologyClinical and neuroimaging features of patients with claustrum sign.
Frontiers in neurologyMinocycline in chronic management of febrile infection-related epilepsy syndrome (FIRES): a case series and literature review of treatment strategies.
Acta epileptologicaComparative clinical outcomes in children with Febrile Infection-Related Epilepsy Syndrome (FIRES).
Epilepsy & behavior : E&BSecondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series.
Frontiers in neurologyPediatric new-onset super-refractory status epilepticus (NOSRSE): a case-series.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyPerformance of cryptogenic new onset refractory status epilepticus score in a Brazilian cohort after testing for antineuronal antibodies with tissue-based and cell-based assays.
EpilepsiaA Case Series and Review of Febrile-Infection Related Epilepsy Syndrome (FIRES).
Children (Basel, Switzerland)Intrathecal Dexamethasone as a FIRES Extinguisher: A 12-Patient Clinical Experience with Usage of Intrathecal Dexamethasone for Febrile Infection-Related Epilepsy Syndrome.
Neurocritical careComparative analysis of new-onset refractory status epilepticus in adult and pediatric patients: immunotherapy timing and functional outcomes.
Journal of neurologyAdult-onset epilepsy with startle-induced seizure after febrile infection-related epilepsy syndrome: A case report.
Epileptic disorders : international epilepsy journal with videotapePotential inflammatory mechanisms of the ketogenic diet against febrile infection-related epilepsy syndrome.
Acta epileptologicaDiagnosing late-onset PKU in the shadow of refractory seizures.
Radiology case reportsClinical outcomes of cryptogenic new onset refractory status epilepticus (NORSE) in a tertiary hospital in Singapore: a case series.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyVagus nerve stimulation and fast parameter programming protocol in children with febrile infection-related epilepsy syndrome in ICU.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeuticsFebrile Infection-Related Epilepsy Syndrome (FIRES) in children: a retrospective cohort study.
European journal of pediatricsPotential Role of Perampanel in Reducing Barbiturate Dependency in Febrile Infection-Related Epilepsy Syndrome: A Case Report.
CureusCombined Systemic Immunotherapy and Intrathecal Dexamethasone in Febrile Infection Related Epilepsy Syndrome.
Annals of clinical and translational neurologyChallenges in Management of Febrile Infection-Related Epilepsy Syndrome: Real-World Experience From a Large Cohort of Pediatric Patients.
Pediatric neurologyEarly intrathecal dexamethasone (IT-Dexa) associated with faster recovery and good outcome in an adolescent with febrile infection-related epilepsy syndrome (FIRES).
BMJ case reportsNew-Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome Cases in Children: A Retrospective Cohort Study in South of Iran.
Iranian journal of child neurologyAge-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES Family Registry.
EpilepsiaNavigating life after New-onset refractory status epilepticus (NORSE) and Febrile infection-related epilepsy syndrome (FIRES): Insights from caregiver and patient interviews.
Epilepsy & behavior : E&BFavorable outcomes and FDG-PET changes following tocilizumab treatment for febrile infection-related epilepsy syndrome in a child.
International immunopharmacologyCorrigendum: A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.
Frontiers in neurologyComplex Febrile Seizures: Usual and the Unusual.
Indian journal of pediatricsMyelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis with super-refractory status epilepticus.
Brain & developmentCommunication trends over time in new-onset refractory status epilepticus (NORSE): Interim analysis from the NORSE/FIRES Family Registry.
Epilepsy & behavior : E&BSuccessful Management of Febrile Infection-Related Epilepsy Syndrome Using Cytokine-Directed Therapy.
Journal of child neurologyNORSE/FIRES: how can we advance our understanding of this devastating condition?
Frontiers in neurologyHemophagocytic lymphohistiocytosis in an adult patient with super-refractory status epilepticus.
Epilepsia openInternational consensus definitions for infection-triggered encephalopathy syndromes.
Developmental medicine and child neurologyThe Efficacy of a Ketogenic Diet in a Case With Febrile Infection-Related Epilepsy Syndrome in the Chronic Phase: A Case Report.
CureusUse of ketamine in Super Refractory Status Epilepticus: a systematic review.
Neurological research and practiceSerial MRI Changes in Febrile Infection-Related Epilepsy Syndrome (FIRES): A Clinical and Radiologic Exploration.
Annals of Indian Academy of NeurologyIdentification of etiologies according to baseline clinical features of pediatric new-onset refractory status epilepticus in single center retrospective study.
SeizureSteroid-Responsive Involuntary Movements as a Remote Symptom of Febrile Infection-Related Epilepsy Syndrome.
CureusAutoimmune-associated seizure disorders.
Epileptic disorders : international epilepsy journal with videotapeFADD gene pathogenic variants causing recurrent febrile infection-related epilepsy syndrome: Case report and literature review.
EpilepsiaNORSE seasonality may vary geographically in adults.
Epilepsia openComparative analysis of patients with new onset refractory status epilepticus preceded by fever (febrile infection-related epilepsy syndrome) versus without prior fever: An interim analysis.
EpilepsiaCase report: Febrile infection-related epilepsy syndrome in a 14-year-old girl with multiple organ failure and lethal outcome.
Frontiers in neuroscienceEfficacy and safety of ketamine for pediatric and adolescent super-refractory status epilepticus and the effect of cerebral inflammatory conditions.
Journal of the neurological sciencesStatus epilepticus: what's new for the intensivist.
Current opinion in critical careSecond-line immunotherapy in new onset refractory status epilepticus.
EpilepsiaFebrile infection-related epilepsy syndrome with claustrum lesion: an underdiagnosed inflammatory encephalopathy.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyThe Beneficial Outcome of Subsequent Treatment with Anakinra during the Chronic Phase of Febrile Infection-Related Epilepsy Syndrome (FIRES): A Case Report.
Neurology internationalNeuroinflammation and status epilepticus: a narrative review unraveling a complex interplay.
Frontiers in pediatricsRefractory Status Epilepticus Associated With a Pathogenic Variant in TNFRSF13B.
CureusSevere, Refractory Seizures: New-Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome.
The Medical clinics of North AmericaRasmussen's syndrome treated with anakinra.
Journal of neurologySpectrum, Evolution, and Clinical Relationship of Magnetic Resonance Imaging in 31 Children with Febrile Infection-Related Epilepsy Syndrome.
NeuropediatricsMultimodal Management of Febrile Infection-Related Epilepsy Syndrome in a 17-Year-Old Male.
Cureus[Electroencephalogram characteristics of 26 children with febrile infection-related epilepsy syndrome].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsFebrile infection-related epilepsy syndrome in childhood: A clinical review and practical approach.
SeizureTiming of therapeutic interventions against infection-triggered encephalopathy syndrome: a scoping review of the pediatric literature.
Frontiers in neuroscienceSevere and rare neurological manifestations following COVID-19 infection in children: A Malaysian tertiary centre experience.
Brain & developmentNo evidence of neuronal/glial autoantibodies in febrile infection-related epilepsy syndrome (FIRES): a prospective clinic-serologic analysis.
Frontiers in neuroscienceReal-world experience with cannabidiol as add-on treatment in drug-resistant epilepsy.
SeizureThe Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome (FIRES).
Journal of the Belgian Society of RadiologyNeuromodulation in new-onset refractory status epilepticus.
Frontiers in neurologyCoronavirus Disease 2019-Associated Neurological Manifestations in Children: A Large Single-Center Experience With Rare Cases.
Pediatric neurologyCSF neopterin and quinolinic acid are biomarkers of neuroinflammation and neurotoxicity in FIRES and other infection-triggered encephalopathy syndromes.
Annals of clinical and translational neurologyClaustrum sign in febrile infection-related epilepsy syndrome (FIRES).
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyA practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.
Frontiers in neurologyClaustrum sparing sign in seronegative limbic encephalitis.
eNeurologicalSciIntrathecal Dexamethasone in Febrile Infection-Related Epilepsy Syndrome: A Case Report.
Neurology. Clinical practicePerspective: Vagal nerve stimulation in the treatment of new-onset refractory status epilepticus.
Frontiers in neurologyKetogenic diet for super-refractory status epilepticus (SRSE) with NORSE and FIRES: Single tertiary center experience and literature data.
Frontiers in neurologyEarly identification of NORSE and transfer to care setting with appropriate supports: A proposed algorithm.
Frontiers in neurologyNeuropathology of New-Onset Refractory Status Epilepticus (NORSE).
Journal of neurologyInvestigating the genetic contribution in febrile infection-related epilepsy syndrome and refractory status epilepticus.
Frontiers in neurologyCannabidiol in the acute phase of febrile infection-related epilepsy syndrome (FIRES).
Epilepsia openReview and standard operating procedures for collection of biospecimens and analysis of biomarkers in new onset refractory status epilepticus.
EpilepsiaAn in vitro model of drug-resistant seizures for selecting clinically effective antiseizure medications in Febrile Infection-Related Epilepsy Syndrome.
Frontiers in neurologyThe seasonality of new-onset refractory status epilepticus (NORSE).
EpilepsiaLong-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra.
Frontiers in neurologyTreatment of new onset refractory status epilepticus/febrile infection-related epilepsy syndrome with tocilizumab in a child and a young adult.
EpilepsiaDrug-resistant seizures associated with hyperinflammatory monocytes in FIRES.
Annals of clinical and translational neurologyCytokines in New-Onset Refractory Status Epilepticus Predict Outcomes.
Annals of neurologyBridging the Gap: Tailoring an Approach to Treatment in Febrile Infection-Related Epilepsy Syndrome.
NeurologyNew-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.
Current opinion in neurologyMyoclonic super-refractory status epilepticus with favourable evolution in a teenager with FIRES: Is the association of vagus nerve stimulation and cannabidiol effective?
Brain & developmentInflammatory epilepsy (FIRES) and haemophagocytic lymphohistiocytosis (HLH): an adult case.
BMJ case reportsLong-term outcomes of adult cryptogenic febrile infection-related epilepsy syndrome (FIRES).
Frontiers in neurologyFebrile Seizures in Children: A Review.
CureusEarly clinical features of new-onset refractory status epilepticus (NORSE) in adults.
BMC neurologyFebrile infection-related Epilepsy Syndrome (FIRES): a severe encephalopathy with status epilepticus. Literature review and presentation of two new cases.
Italian journal of pediatricsSurviving status epilepticus: The patient and family perspective.
Epilepsy & behavior : E&BCannabidiol in refractory status epilepticus: A review of clinical experiences.
SeizureConnections Between Febrile Infection-Related Epilepsy Syndrome and Autoimmune Encephalitis. A Case Report of a Child With New Anti-neuronal Antibodies.
Frontiers in pediatricsInternational consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) incl. Febrile Infection-Related Epilepsy Syndrome (FIRES): Statements and Supporting Evidence.
EpilepsiaInternational consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) including Febrile Infection-Related Epilepsy Syndrome (FIRES): Summary and Clinical Tools.
EpilepsiaDNM1L mutation presenting as progressive myoclonic epilepsy associated with acute febrile infection-related epilepsy syndrome.
Epileptic disorders : international epilepsy journal with videotapeCombined immunoglobulin and plasmapheresis treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES).
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisEnteral lorazepam is a promising weaning strategy for midazolam-responsive febrile infection-related epilepsy syndrome (FIRES): a case series.
Epileptic disorders : international epilepsy journal with videotapeAnakinra and tocilizumab in the chronic phase of febrile infection-related epilepsy syndrome (FIRES): Effectiveness and safety from a case-series.
SeizureAnti-inflammatory and immune-mediated therapy for a case of febrile infection-related epilepsy syndrome with rapid recurrence.
Clinical case reportsFever-Associated Seizures or Epilepsy: An Overview of Old and Recent Literature Acquisitions.
Frontiers in pediatricsA case of febrile infection-related epilepsy syndrome (FIRES) in young adult: still a diagnostic and therapeutic challenge.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyInternational League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions.
EpilepsiaMultifocal multilobar focal cortical dysplasia type IIa in Febrile Infection Related Epilepsy Syndrome (FIRES).
Clinical neurology and neurosurgeryAn ultra-long new onset refractory status epilepticus: Winning the battle but losing the war?
Epilepsy & behavior reportsZebras Seize the Day: Rare Causes of Status Epilepticus Across the Continuum of Critical Care.
Critical care clinicsTargeting Inflammatory Mediators in Epilepsy: A Systematic Review of Its Molecular Basis and Clinical Applications.
Frontiers in neurologyFighting autoinflammation in FIRES: The role of interleukins and early immunomodulation.
Epilepsy & behavior reportsKetogenic Diet for a Young Adult Patient With Chronic-Phase Febrile Infection-Related Epilepsy Syndrome.
Cureus[A case of febrile infection-related epilepsy syndrome requiring prolonged intensive care management: a trial of intravenous ketamine and intrathecal dexamethasone therapy].
Rinsho shinkeigaku = Clinical neurologySpecific characteristics and current diagnostic and treatment modalities performance of super refractory status epilepticus in children: A comparative study.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyNew-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) of unknown aetiology: A comparison of the incomparable?
SeizureTeaching NeuroImage: Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome.
NeurologyDeep brain stimulation of the centromedian thalamic nucleus for the treatment of FIRES.
Epilepsia openUtility and Safety of Perampanel in Pediatric FIRES and Other Drug-Resistant Epilepsies.
Child neurology openA retrospective study of 92 children with new-onset refractory status epilepticus.
Epilepsy & behavior : E&BVagus nerve stimulation for super-refractory status epilepticus in febrile infection-related epilepsy syndrome: a pediatric case report and literature review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryKetogenic diet (KD) therapy in the acute phase of febrile infection-related epilepsy syndrome (FIRES): a case report.
Translational pediatricsUnraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies.
European journal of neurologySevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection-related epilepsy syndrome.
Epilepsia openVaccination in acute immune-mediated/inflammatory disorders of the central nervous system.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyDramatic Course of Paediatric Cryptogenic Febrile Infection-Related Epilepsy Syndrome with Unusual Chronic Phase Presentation-A Case Report with Literature Study.
Brain sciencesTherapeutic plasma exchange in clinical pediatric neurology practice: Experience from a tertiary referral hospital.
Clinical neurology and neurosurgeryReport of a Child With Febrile Status Epilepticus and Post-COVID Multi-System Inflammatory Syndrome.
Child neurology openConsidering safety and patient tolerance in the use of ketogenic diet in the management of refractory and super-refractory status epilepticus: a systematic review.
Expert review of neurotherapeuticsTowards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy.
International journal of molecular sciencesSeizure control after late introduction of anakinra in a patient with adult onset Rasmussen's encephalitis.
Epilepsy & behavior reportsHypothesis: Febrile infection-related epilepsy syndrome is a microglial NLRP3 inflammasome/IL-1 axis-driven autoinflammatory syndrome.
Clinical & translational immunologyParental perception of FIRES outcomes, emotional states, and social media usage.
Epilepsia openPermanent loss of independence in adult febrile-infection-related epilepsy syndrome survivors: an underestimated and unsolved challenge.
European journal of neurologyClinical presentation of new onset refractory status epilepticus in children (the pSERG cohort).
EpilepsiaSuper-refractory status epilepticus in autoimmune encephalitis treated with interleukin-1 receptor antagonist, anakinra.
Epileptic disorders : international epilepsy journal with videotapeDietary Management of Children With Super-Refractory Status Epilepticus: A Systematic Review and Experience in a Single UK Tertiary Centre.
Frontiers in neurologyHow 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 currentsProposal to optimize evaluation and treatment of Febrile infection-related epilepsy syndrome (FIRES): A Report from FIRES workshop.
Epilepsia openSuper refractory status in a case of Febrile Infection-Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis.
Epilepsia openNew-onset refractory status epilepticus (NORSE).
Practical neurologyDiagnosis and Treatment of Status Epilepticus.
Journal of epilepsy researchIntrathecal dexamethasone therapy for febrile infection-related epilepsy syndrome.
Annals of clinical and translational neurologyAnakinra usage in febrile infection related epilepsy syndrome: an international cohort.
Annals of clinical and translational neurologyClaustrum hyperintensity: a rare radiological correlate in Niemann-Pick disease.
BMJ case reportsInterleukin-6 Blockade With Tocilizumab in Anakinra-Refractory Febrile Infection-Related Epilepsy Syndrome (FIRES).
Child neurology openImproved seizure burden and cognitive performance in a child treated with responsive neurostimulation (RNS) following febrile infection related epilepsy syndrome (FIRES).
Epileptic disorders : international epilepsy journal with videotapeInflammation, ictogenesis, and epileptogenesis: An exploration through human disease.
EpilepsiaPossible Role of High-Dose Barbiturates and Early Administration of Parenteral Ketogenic Diet for Reducing Development of Chronic Epilepsy in Febrile Infection-Related Epilepsy Syndrome: A Case Report.
NeuropediatricsKetogenic diet and Neuroinflammation.
Epilepsy researchAn atypical case of febrile infection-related epilepsy syndrome following acute encephalitis: impact of physiotherapy in regaining locomotor abilities in a patient with neuroregression.
The Pan African medical journalWhole-exome and HLA sequencing in Febrile infection-related epilepsy syndrome.
Annals of clinical and translational neurologyDiminished toll-like receptor response in febrile infection-related epilepsy syndrome (FIRES).
Biomedical journalApplication of ketogenic diets for pediatric neurocritical care.
Biomedical journalProtecting the blossoming brain - Neurocritical care in children.
Biomedical journalEfficacy of different treatment modalities for acute and chronic phases of the febrile infection-related epilepsy syndrome: A systematic review.
SeizureNeurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome.
Biomedical journalTherapeutic hypothermia for pediatric refractory status epilepticus May Ameliorate post-status epilepticus epilepsy.
Biomedical journalExtinguishing Febrile Infection-Related Epilepsy Syndrome: Pipe Dream or Reality?
Seminars in neurologyGenetic diagnoses in epilepsy: The impact of dynamic exome analysis in a pediatric cohort.
EpilepsiaFebrile Infection-Related Epilepsy Syndrome Treated Successfully With Anakinra in a 21-Year-Old Woman.
WMJ : official publication of the State Medical Society of WisconsinCentromedian thalamic nuclei deep brain stimulation and Anakinra treatment for FIRES - Two different outcomes.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyFebrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies.
Neuropsychiatric disease and treatmentEtiology is the key determinant of neuroinflammation in epilepsy: Elevation of cerebrospinal fluid cytokines and chemokines in febrile infection-related epilepsy syndrome and febrile status epilepticus.
EpilepsiaInflammation and reactive oxygen species in status epilepticus: Biomarkers and implications for therapy.
Epilepsy & behavior : E&BTherapeutic effect of Anakinra in the relapsing chronic phase of febrile infection-related epilepsy syndrome.
Epilepsia openKetogenic Diet as a Treatment for Super-Refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome.
Frontiers in neurologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- From clinical practice to mechanistic insights in ketogenic diets for epilepsy.
- [Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
- An increase in cerebrospinal fluid macrophage migration inhibitory factor levels in pediatric patients with acute neuroinflammatory diseases: A preliminary study.
- [Expert consensus on the diagnosis and treatment of pediatric febrile infection-related epilepsy syndrome (2026)].
- Status Epilepticus in Children With Hemophagocytic Lymphohistiocytosis: Literature Review.
- Metabolism-corrected propofol exposure intensity and long-term intelligence quotient in pediatric febrile infection-related epilepsy syndrome: a retrospective cohort study.
- Extreme cryptogenic new onset refractory status epilepticus/febrile infection-related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury.
- Pediatric new-onset super refractory status epilepticus: Potential of timely immunotherapy in a resource-limited PICU.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:163703(Orphanet)
- MONDO:0015584(MONDO)
- Epilepsia(PCDT · Ministério da Saúde)
- GARD:11005(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q17143712(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
