O estado epiléptico refratário de início recente é uma encefalopatia aguda com estado epiléptico mediado por inflamação, caracterizado por um estado epiléptico refratário agudo, tipicamente do tipo tônico-clônico, após sintomas prodrômicos de confusão, febre, fadiga, dor de cabeça, sintomas de infecção gastrointestinal ou do trato respiratório superior, alterações comportamentais ou alucinações. Anormalidades na ressonância magnética cerebral e achados anormais no LCR, incluindo pleocitose e/ou níveis elevados de proteínas, são frequentemente encontrados durante episódios agudos. A epilepsia resistente ao tratamento e as deficiências cognitivas e psiquiátricas são consequências habituais.
Introdução
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O estado epiléptico refratário de início recente é uma encefalopatia aguda com estado epiléptico mediado por inflamação, caracterizado por um estado epiléptico refratário agudo, tipicamente do tipo tônico-clônico, após sintomas prodrômicos de confusão, febre, fadiga, dor de cabeça, sintomas de infecção gastrointestinal ou do trato respiratório superior, alterações comportamentais ou alucinações. Anormalidades na ressonância magnética cerebral e achados anormais no LCR, incluindo pleocitose e/ou níveis elevados de proteínas, são frequentemente encontrados durante episódios agudos. A epilepsia resistente ao tratamento e as deficiências cognitivas e psiquiátricas são consequências habituais.
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Publicações mais relevantes
[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.
New-onset refractory status epilepticus associated with Sjögren's disease.
Sjögren's disease (SjD) is a chronic autoimmune connective tissue disorder primarily affecting the salivary and lacrimal glands. Extra-glandular involvement may occur, sometimes preceding the diagnosis of SjD or in the absence of sicca symptoms, contributing to diagnostic delay. Central nervous system (CNS) manifestations have been reported in SjD, though establishing a causal relationship is challenging given possible overlapping neuroinflammatory and autoimmune aetiologies. We report two patients who presented with acute encephalitis and new-onset refractory status epilepticus (NORSE), in whom features supportive of primary SjD were subsequently identified. Both patients underwent extensive evaluation that excluded infectious causes, other systemic autoimmune connective tissue diseases and known autoantibody-mediated forms of autoimmune encephalitis. These cases highlight the importance of considering systemic autoimmune diseases in the evaluation of unexplained encephalitis and NORSE, and support the early use of immunotherapy when an immune-mediated pathology is suspected.
Febrile infection-related epilepsy syndrome (FIRES) in adults: a case report and review of factors associated with survival.
Febrile infection-related epilepsy syndrome (FIRES) is a subtype of new onset refractory status epilepticus (NORSE) that has, in recent years, been recognized to not only affect pediatric but adult patients too. While inpatient mortality in adult-onset FIRES has been suggested to be higher than in pediatric patients, little is known about the factors associated with survival, hampering research into developing prognostic and stratification models and also posing a challenge for clinical communications and decision making. We report a fatal case of FIRES in a 21-year-old lady and performed a review of literature involving adult-onset FIRES from 1 Aug 2022 to 17 Aug 2024, analyzing the data to identify factors associated with survival of the initial hospital stay. Including our case, 49 patients of adult-onset FIRES were identified. Of the 47 patients who had MRI brain scan findings described at any time during the illness, 35 patients (74.5%) had abnormal findings in at least one scan, and the most common site of abnormality was the temporal lobe (59.6%). A total of 41 patients survived the initial hospital stay (83.7%). The presence of MRI temporal lobe abnormalities was associated with survival at discharge (p = 0.013), and the association remained significant on multivariate analysis with corticosteroid usage. In adult-onset FIRES, MRI temporal lobe abnormalities may be associated with survival beyond the acute phase. Our study findings suggest that the site of MRI abnormalities in FIRES may have value in the clinical stratification and prognostication of adult-onset FIRES.
Clinical characteristics, etiology and outcome of new onset refractory status epilepticus in adults.
We analyzed clinical features and predictors of outcome in adult patients with new-onset refractory status epilepticus(NORSE). In this retrospective cohort, we analyzed the data of 72 patients with NORSE followed-up for two or more years. Outcome was assessed by Glasgow Outcome Scale(GOS) at discharge and latest follow-up. Mean age of the population was 35.13 ± 16.42 years and average follow-up was 60.34 ± 18.12 months. Average length of stay in hospital was 19.62 ± 14.68 days and mean ICU stay was14.43 ± 13.89 days. Of 72 patients with NORSE, 37(51.3%) patients progressed to super-refractory status epilepticus(SRSE). The most common etiology was cryptogenic(54%) followed by viral encephalitis(25%). Overall mortality was 27.8%. At discharge, 37 of 72 cases(51.4%) achieved a poor outcome (GOS 1-3). Among the survivors(n = 52), functional outcome improved over time and 84.6%(n = 44) achieved good outcome(GOS 4-5); however significant cognitive impairment was reported in 29(56%) and drug-resistant epilepsy in 31(60%). Although none of the factors predicted poor GOS outcome at discharge; etiology of viral encephalitis was associated with poor outcome at latest follow-up. Viral encephalitis, not able to achieve burst suppression on cEEG, medical complications and progression to SRSE predicted mortality during hospital stay. Despite a prolonged clinical course and high mortality rate, more than 80% of adult survivors with NORSE attained a favourable functional outcome at long-term follow-up. None of the factors predicted the functional outcome at discharge; viral encephalitis was associated with poor outcome at latest follow-up. Significant cognitive impairment and drug-resistant epilepsy were reported in more than 50% of the survivors.
Refractory Status Epilepticus Treated With Bilateral Pulvinar Deep Brain Stimulation-A Case Study.
New-onset refractory status epilepticus (NORSE) arises without an identifiable cause or prior epilepsy history, with a 16%-27% mortality rate and significant long-term neurological sequelae. Neuromodulation such as deep brain stimulation (DBS) targeting the anterior and centromedian thalamic nuclei has shown promise when the traditional approach of anti-seizure medications (ASMs), anesthetics, and immunomodulation fails. We present a case of cryptogenic NORSE in a 30-year-old male with autism and developmental delay, with refractory seizures localized to bilateral posterior quadrants. Sensing-enabled DBS targeting the pulvinar thalami led to decreased seizure burden and clinical improvement, highlighting the importance of tailoring neuromodulatory targets to seizure localization.
Publicações recentes
Role of Tocilizumab in Severe CNS Inflammatory Presentations in Children.
Vagus nerve stimulation as an adjunctive therapy for super-refractory status epilepticus including NORSE: a retrospective cohort study.
[Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
A model of new-onset refractory status epilepticus (NORSE) using intrahippocampal kainic acid in adolescent mice.
Safety and efficacy of intrathecal rituximab in refractory neuroimmune disorders- a case series.
📚 EuropePMC193 artigos no totalmostrando 195
Vagus nerve stimulation as an adjunctive therapy for super-refractory status epilepticus including NORSE: a retrospective cohort study.
Epilepsy & behavior : E&B[Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
Rinsho shinkeigaku = Clinical neurologyA model of new-onset refractory status epilepticus (NORSE) using intrahippocampal kainic acid in adolescent mice.
Epilepsy researchSafety and efficacy of intrathecal rituximab in refractory neuroimmune disorders- a case series.
Journal of neuroimmunologyExploring pathways leading to drug-resistant epilepsy for patients with cryptogenic new onset refractory status epilepticus.
EpilepsiaExtreme 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.
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&BNew-onset refractory status epilepticus associated with Sjögren's disease.
BMJ neurology openThe Use of Electroconvulsive Therapy (ECT) in the New Onset Refractory Status Epilepticus (NORSE)-Case Report and Systematic Review.
The journal of ECTFebrile 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 NeurophysiologyClinical characteristics, etiology and outcome of new onset refractory status epilepticus in adults.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyFatal retroperitoneal hemorrhage following enoxaparin administration in a patient with autoimmune encephalitis: a case report.
Encephalitis (Seoul, Korea)Predicting epilepsy after new onset refractory status epilepticus due to autoimmune encephalitis: The DAME score.
EpilepsiaKetogenic 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)Claustrum Sign: An Increasingly Recognized Imaging Feature of New-Onset Refractory Status Epilepticus (NORSE).
The NeurohospitalistRefractory Status Epilepticus Treated With Bilateral Pulvinar Deep Brain Stimulation-A Case Study.
Annals of clinical and translational neurologyEmergence of Atypical Lymphocytes Before Cryptogenic New Onset Refractory Status Epilepticus: A Case Report.
The NeurohospitalistCase Report: Successful multimodal management of FIRES in a pediatric patient using anakinra, ketogenic diet, and induced hypothermia.
Frontiers in pediatricsFactors associated with long-term outcomes in people with new onset refractory epilepticus: A single-center cohort study.
EpilepsiaBeyond Anakinra and Tocilizumab: Additional Adjunctive Therapies in Pediatric New Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome - A Narrative Review.
Pediatric neurologyDeep brain stimulation of the anterior nucleus of the thalamus for seizures after new-onset refractory status epilepticus: a case report.
Frontiers in human neuroscienceIatrogenic NORSE: Immune checkpoint inhibitor-related anti-GFAP autoimmune astrocytopathy.
Journal of neuroimmunologySuccessful Treatment of Cryptogenic NORSE Resistant to Immunosuppression With Intravenous Ganaxolone and Electroconvulsive Therapy.
The NeurohospitalistIntensive Intracranial Pressure and Temperature Monitoring May Improve the Outcome of a Young Patient with Severe Cryptogenic New-onset Refractory Status Epilepticus: A Case Report.
NMC case report journalThe 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 SocietyEarly Recovery from Cryptogenic NORSE Following Repeated C-NORSE Score Assessment and Cyclophosphamide Treatment: A Case Report.
Internal medicine (Tokyo, Japan)A 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.
Journal of child neurologyHemorrhagic cystitis induced by intravenous esketamine in the treatment of new-onset refractory status epilepticus in children: a case report.
Frontiers in pediatricsNew onset refractory status epilepticus diagnosed in the second trimester: A case report.
Obstetric medicineThe spectrum of cognitive outcomes following admission to hospital for refractory status epilepticus (RSE): A scoping review.
SeizureQuality of life over time after new onset refractory status epilepticus.
EpilepsiaAn extreme type of new onset refractory status epilepticus with stimulus-induced seizures in pharmacological isoelectric states.
EpilepsiaStandard 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 pediatricsRescue use of electroconvulsive therapy in new-onset refractory status epilepticus during pregnancy.
BMJ case reportsImmune dysregulation in new-onset refractory status epilepticus (NORSE): current insights and therapeutic perspectives.
Brain & developmentHypocupraemia-related drug-refractory seizures in Wilson disease.
Practical neurologyRetrospective multicenter study on cryptogenic NORSE/FIRES patients treated with anakinra.
SeizureAnalysis of Early EEG Changes After Tocilizumab Treatment in New-Onset Refractory Status Epilepticus.
Brain sciencesThe Role of Metabolic Testing in the Diagnostic Evaluation of Adult NORSE: A Retrospective, Single-Centre Study.
European journal of neurologyPatients with status epilepticus and new-onset refractory status epilepticus display drastically altered fecal microbiomes compared to chronic epilepsy patients.
EpilepsiaIdentification of Distinct Biological Groups of Patients With Cryptogenic NORSE via Inflammatory Profiling.
Neurology(R) neuroimmunology & neuroinflammationAutoimmune encephalitis associated with anti-SOX1 autoantibodies in COVID-19: A case report.
IDCasesPediatric 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 videotapeCytokines in new-onset refractory status epilepticus: ready for clinical use?
Acta epileptologicaClinical analysis of 7 cases of cryptogenic new-onset refractory status epilepticus.
MedicineCSF Tau Is a Biomarker of Hippocampal Injury in Cryptogenic New-Onset Refractory Status Epilepticus.
Annals of clinical and translational neurologyDramatic response to delayed treatment with tocilizumab in new-onset refractory status epilepticus.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyClinical 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 NeurophysiologyPhenotype, outcome, interleukins, and miRNA levels assessment of new-onset refractory status epilepticus: A prospective cohort study.
Epileptic disorders : international epilepsy journal with videotapePotential 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 neurologyNew-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 neurologyMultidisciplinary Approaches and Molecular Diagnostics in New-Onset Refractory Status Epilepticus and Nontuberculous Mycobacterial CNS Infections in the ICU: A Case Report.
CureusNew onset refractory status epilepticus: Long-term outcomes beyond seizures.
EpilepsiaNew-onset refractory status epilepticus after SARS-CoV-2 infection: a review of literature.
Croatian medical journalAge-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES Family Registry.
EpilepsiaNORSE secondary to anti-GAD65 antibody-positive encephalitis treated with novel adjunctive rapid titration VNS protocol.
Epilepsia openMyelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) as a cause of new-onset refractory status epilepticus (NORSE): Case report and review of literature.
Journal of neuroimmunologyNavigating life after New-onset refractory status epilepticus (NORSE) and Febrile infection-related epilepsy syndrome (FIRES): Insights from caregiver and patient interviews.
Epilepsy & behavior : E&BEarly initiation of intravenous cyclophosphamide and one-year outcome in super-refractory cryptogenic-new onset refractory status epilepticus.
Epilepsia openCorrigendum: A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.
Frontiers in neurologyNew-Onset Refractory Status Epilepticus With Diffuse Cerebral Restricted Diffusion in Young Children: A Novel Clinical-Radiologic Presentation.
Pediatric neurologyPolygenic Landscape of Cryptogenic New-Onset Refractory Status Epilepticus: A Comprehensive Whole-Genome Sequencing Study.
Annals of neurologyCryptogenic New-onset Refractory Status Epilepticus with Hyperintensity of T1-weighted Magnetic Resonance Imaging in the Bilateral Basal Ganglia: An Autopsy Report.
Internal medicine (Tokyo, Japan)Communication trends over time in new-onset refractory status epilepticus (NORSE): Interim analysis from the NORSE/FIRES Family Registry.
Epilepsy & behavior : E&BPrognosis prediction and immunotherapy optimisation for cryptogenic new-onset refractory status epilepticus.
Journal of neurology, neurosurgery, and psychiatryPOLG epilepsy presenting as new-onset refractory status epilepticus (NORSE) in pregnancy.
Practical neurologyNew onset status epilepticus and its long-term outcome: A cohort study.
Epilepsy researchNORSE/FIRES: how can we advance our understanding of this devastating condition?
Frontiers in neurologyIT-DEX and B cell depletion in a child with anti-GAD 65 autoimmune encephalitis presenting as NORSE: A case report.
Journal of neuroimmunologyIdentification of etiologies according to baseline clinical features of pediatric new-onset refractory status epilepticus in single center retrospective study.
SeizureTrends in management of patients with new-onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis.
EpilepsiaAutoimmune-associated seizure disorders.
Epileptic disorders : international epilepsy journal with videotapeSingle-Cell Transcriptomic Analyses of Brain Parenchyma in Patients With New-Onset Refractory Status Epilepticus (NORSE).
Neurology(R) neuroimmunology & neuroinflammationElectroconvulsive therapy in new onset refractory status epilepticus (NORSE) in a pediatric patient.
Brain stimulationFADD gene pathogenic variants causing recurrent febrile infection-related epilepsy syndrome: Case report and literature review.
EpilepsiaTreatment of Cryptogenic New-onset Refractory Status Epilepticus (C-NORSE) with Tocilizumab.
Internal medicine (Tokyo, Japan)Responsive neurostimulation as a therapy for epilepsy following new-onset refractory status epilepticus: Case series and review of the literature.
Clinical neurophysiology : official journal of the International Federation of Clinical NeurophysiologyNORSE 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.
EpilepsiaClinical outcomes among initial survivors of cryptogenic new-onset refractory status epilepsy (NORSE).
EpilepsiaLong-term outcome in new onset refractory status epilepticus: a retrospective study.
Critical care (London, England)Status epilepticus: what's new for the intensivist.
Current opinion in critical careSecond-line immunotherapy in new onset refractory status epilepticus.
EpilepsiaNew onset refractory status epilepticus: Summary of the NORSE Institute roundtable during the 2023 American Epilepsy Society meeting, Orlando, Florida.
EpilepsiaSuccessfully treated anti-GAD limbic encephalitis in a 15-year-old diabetic boy with intravenous immunoglobulin: case report.
Annals of medicine and surgery (2012)Time to FIRE NORSE: A single acronym for a heterogeneous presentation. Further information from a case series and discussion of the literature.
Journal of neuroimmunologyRefractory Status Epilepticus Associated With a Pathogenic Variant in TNFRSF13B.
Cureus[Initial Management of Status Epilepticus].
No shinkei geka. Neurological surgery[A case of cryptogenic new-onset refractory status epilepticus (NORSE) in which cerebrospinal fluid IL-6 was elevated with increased seizure frequency early in the disease: a case report].
Rinsho shinkeigaku = Clinical neurologySevere, 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 neurologyRefractory status epilepticus arrested by vagus nerve stimulation.
Practical neurologyMultimodal Management of Febrile Infection-Related Epilepsy Syndrome in a 17-Year-Old Male.
CureusLidocaine as a potential therapeutic option for super-refractory status epilepticus: A case report.
Journal of central nervous system diseaseA case of new onset refractory status epilepticus in a U.S. traveler with Japanese encephalitis.
Journal of neuroimmunologyThe role of neuronal antibodies in cryptogenic new onset refractory status epilepticus.
EpilepsiaAnti-N-methyl D-aspartate receptor encephalitis presenting with the new-onset refractory status epilepticus.
Northern clinics of IstanbulThe Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome (FIRES).
Journal of the Belgian Society of RadiologyNeuromodulation in new-onset refractory status epilepticus.
Frontiers in neurologySafe and effective implantation and use of vagal nerve stimulation in new-onset refractory status epilepticus in early pregnancy: a case report.
Frontiers in neurologyA practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.
Frontiers in neurologyPerspective: 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 neurologyNew-Onset Refractory Status Epilepticus Due to a Novel MT-TF Variant: Time for Acute Genetic Testing Before Treatment?
Neurology. GeneticsNew-onset refractory status epilepticus responsive to VNS: A case report.
Epileptic disorders : international epilepsy journal with videotapeNeuropathology 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 neurologyReview and standard operating procedures for collection of biospecimens and analysis of biomarkers in new onset refractory status epilepticus.
EpilepsiaThe 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.
EpilepsiaBlood-brain barrier damage and new onset refractory status epilepticus: An exploratory study using dynamic contrast-enhanced magnetic resonance imaging.
EpilepsiaCytokines in New-Onset Refractory Status Epilepticus Predict Outcomes.
Annals of neurologySpecific profiles of new-onset vs. non-inaugural status epilepticus: From diagnosis to 1-year outcome.
Frontiers in neurologyNew-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.
Current opinion in neurologySeizure burden and neuropsychological outcomes of new-onset refractory status epilepticus: Systematic review.
Frontiers in neurologyThe etiology and mortality of new-onset refractory status epilepticus (NORSE) in adults: A systematic review and meta-analysis.
EpilepsiaA case report: New-onset refractory status epilepticus in a patient with FASTKD2-related mitochondrial disease.
Frontiers in neurologyLong-term outcomes of adult cryptogenic febrile infection-related epilepsy syndrome (FIRES).
Frontiers in neurologyThe Increased Interleukin-6 Levels Can Be an Early Diagnostic Marker for New-Onset Refractory Status Epilepticus.
Journal of epilepsy researchPearls & Oy-sters: Status Epilepticus and Cerebral Edema From Hyperammonemia Due to Disseminated Ureaplasma and Mycoplasma Species.
NeurologyEarly clinical features of new-onset refractory status epilepticus (NORSE) in adults.
BMC neurologyNew-onset refractory status epilepticus (NORSE) in paediatric patients: causes, characteristics and outcomes.
BMJ neurology openFebrile 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&BRole of responsive neurostimulation and immunotherapy in refractory epilepsy due to autoimmune encephalitis: A case report.
Frontiers in neurologyCannabidiol in refractory status epilepticus: A review of clinical experiences.
SeizureInflammation as Treatment Target for Status Epilepticus.
Current neuropharmacologyReversible Brain Atrophy in Cryptogenic New-onset Refractory Status Epilepticus.
Internal medicine (Tokyo, Japan)Intravenous ganaxolone in pediatric super-refractory status epilepticus: A single hospital experience.
Epilepsy & behavior reportsMulti-proteomic Analysis Revealed Distinct Protein Profiles in Cerebrospinal Fluid of Patients Between Anti-NMDAR Encephalitis NORSE and Cryptogenic NORSE.
Molecular neurobiologyParallel roles of neuroinflammation in feline and human epilepsies.
Veterinary journal (London, England : 1997)Long-term outcomes after NORSE: Treatment with vagus nerve stimulation.
Epilepsia openNew-Onset Refractory Status Epilepticus Secondary to COVID-19 Infection in Adults: A Systematic Review.
Neuropsychiatric disease and treatmentNew-onset refractory status epilepticus due to autoimmune encephalitis after vaccination against SARS-CoV-2: First case report.
Frontiers in neurologyInternational 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.
EpilepsiaChild Neurology: Initial Presentation of PCDH19-Related Epilepsy With New-Onset Refractory Status Epilepticus and Treatment With Anakinra.
NeurologyA retrospective cohort study of new-onset refractory status epilepticus (NORSE): clinical features, timing of immunotherapy and outcomes.
Epileptic disorders : international epilepsy journal with videotapeRapidly evolving Creutzfeldt-Jakob disease in COVID-19: from early status epilepticus to fatal outcome.
Acta neurologica BelgicaFever-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 NeurophysiologyNeurosyphilis presenting as new onset refractory status epilepticus: one of the multifarious faces of a great imitator.
Sexual healthAutoimmune encephalitis associated with COVID-19: A systematic review.
Multiple sclerosis and related disordersAn 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 clinicsNew-onset refractory status epilepticus as an early manifestation of multisystem inflammatory syndrome in adults after COVID-19.
EpilepsiaAn Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report.
Case reports in neurologyNew onset refractory status epilepticus and neuroendocrine tumour: a case report and review of the literature.
Acta neurologica BelgicaSpecific 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?
SeizureNew onset refractory status epilepticus: State of the art.
Revue neurologiqueNew Onset Refractory Status Epilepticus as a Manifestation of Tuberculosis of the Central Nervous System.
Neurology IndiaCryptogenic new-onset refractory status epilepticus responded to anti-interleukin-6 treatment.
Journal of neuroimmunologyCorrigendum to 'New onset refractory status epilepticus secondary to HIV CNS viral escape syndrome: Case report'.
SeizureHigh dose cannabidiol (CBD) in the treatment of new-onset refractory status epilepticus (NORSE).
SeizureNew onset refractory status epilepticus secondary to HIV CNS viral escape syndrome: Case report.
SeizureA retrospective study of 92 children with new-onset refractory status epilepticus.
Epilepsy & behavior : E&BNew-Onset Refractory Status Epilepticus (NORSE): The Queen Square Neuro-ICU experience.
Epilepsy & behavior : E&BA Role for Electroconvulsive Therapy in the Management of New Onset Refractory Status Epilepticus (NORSE) in a Young Child.
Neurology IndiaHistopathology of new-onset refractory status epilepticus (NORSE) in adults.
SeizureIdiopathic Hypoparathyroidism Presenting As New Onset Refractory Status Epilepticus.
Journal of neurosciences in rural practiceUnraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies.
European journal of neurologyAnti-N-methyl-d-aspartate Receptor Encephalitis Presenting as New-onset Refractory Status Epilepticus Responding to Rituximab in an Adolescent Girl.
Journal of pediatric neurosciencesPredictors and prognoses of epilepsy after anti-neuronal antibody-positive autoimmune encephalitis.
SeizureInfantile Hemiconvulsion-Hemiplegia and Epilepsy (IHHE) in a boy with tuberous sclerosis complex.
Epilepsy & behavior reportsBenzodiazepine administration patterns before escalation to second-line medications in pediatric refractory convulsive status epilepticus.
EpilepsiaNew-Onset Refractory Status Epilepticus (NORSE) as a Recurrence of Anti-Neuronal Nuclear Antibody 2 (ANNA-2) Encephalitis After Immune Checkpoint Inhibition Therapy.
CureusSuper-Refractory Status Epilepticus: Prognosis and Recent Advances in Management.
Aging and diseaseUnderstanding new-onset refractory status epilepticus from an immunological point of view.
Encephalitis (Seoul, Korea)New-onset refractory status epilepticus following the ChAdOx1 nCoV-19 vaccine.
Journal of neuroimmunologyParental 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 videotape[Anti-NMDA Receptor Encephalitis].
Brain and nerve = Shinkei kenkyu no shinpoCryptogenic new-onset refractory status epilepticus (NORSE) following blood transfusion in a patient with severe anemia.
Epileptic disorders : international epilepsy journal with videotapeCOVID-19-related refractory status epilepticus with the presence of SARS-CoV-2 (RNA) in the CSF: a case report.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyRemote Teamwork Management of NORSE During the COVID-19 Lockdown.
Neurology. Clinical practicePredictors of outcome in super refractory status epilepticus.
Epilepsy & behavior : E&BHow 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 currentsResolution of cryptogenic new onset refractory status epilepticus with tocilizumab.
Epilepsy & behavior reportsIntravenous immunoglobulin response in new-onset refractory status epilepticus (NORSE) COVID-19 adult patients.
Journal of neurologyProposal to optimize evaluation and treatment of Febrile infection-related epilepsy syndrome (FIRES): A Report from FIRES workshop.
Epilepsia openAssociações
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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.
- [Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
- New-onset refractory status epilepticus associated with Sjögren's disease.
- Febrile 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 Neurophysiology· 2026· PMID 41524901mais citado
- Clinical characteristics, etiology and outcome of new onset refractory status epilepticus in adults.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2026· PMID 41498863mais citado
- Refractory Status Epilepticus Treated With Bilateral Pulvinar Deep Brain Stimulation-A Case Study.
- Role of Tocilizumab in Severe CNS Inflammatory Presentations in Children.
- Vagus nerve stimulation as an adjunctive therapy for super-refractory status epilepticus including NORSE: a retrospective cohort study.
- A model of new-onset refractory status epilepticus (NORSE) using intrahippocampal kainic acid in adolescent mice.
- Safety and efficacy of intrathecal rituximab in refractory neuroimmune disorders- a case series.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:363558(Orphanet)
- MONDO:0018199(MONDO)
- GARD:12244(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55787789(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.
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