Raras
Buscar doenças, sintomas, genes...
Estado de mal epiléptico refratário de início precoce
ORPHA:363558CID-10 · G41.8DOENÇA RARA

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.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

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.

Pesquisas ativas
1 ensaio
1 total registrados no ClinicalTrials.gov
Publicações científicas
316 artigos
Último publicado: 2026 Apr 28

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G41.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
18 sintomas
🛡️
Imunológico
2 sintomas
😀
Face
1 sintomas

+ 16 sintomas em outras categorias

Características mais comuns

100%prev.
Estado de mal epiléptico
90%prev.
Anormalidade interictal no EEG
Muito frequente (99-80%)
90%prev.
Estado de mal epiléptico refratário
Muito frequente (99-80%)
90%prev.
Convulsão precipitada por infecção febril
Muito frequente (99-80%)
55%prev.
Crise motora focal consciente
Frequente (79-30%)
55%prev.
Febre
Frequente (79-30%)
37sintomas
Muito frequente (4)
Frequente (18)
Ocasional (13)
Muito raro (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 37 características clínicas mais associadas, ordenadas por frequência.

Estado de mal epilépticoStatus epilepticus
Muito frequente100%
Anormalidade interictal no EEGInterictal EEG abnormality
Muito frequente (99-80%)90%
Estado de mal epiléptico refratárioRefractory status epilepticus
Muito frequente (99-80%)90%
Convulsão precipitada por infecção febrilSeizure precipitated by febrile infection
Muito frequente (99-80%)90%
Crise motora focal conscienteFocal aware motor seizure
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico316PubMed
Últimos 10 anos200publicações
Pico202552 papers
Linha do tempo
2026Hoje · 2026📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Estado de mal epiléptico refratário de início precoce

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

1 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
300 papers (10 anos)
#1

[Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].

Rinsho shinkeigaku = Clinical neurology2026 Mar 11

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.

#2

New-onset refractory status epilepticus associated with Sjögren's disease.

BMJ neurology open2026

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.

#3

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 Neurophysiology2026 Jan 12

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.

#4

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 Neurophysiology2026 Jan 07

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.

#5

Refractory Status Epilepticus Treated With Bilateral Pulvinar Deep Brain Stimulation-A Case Study.

Annals of clinical and translational neurology2026 Mar

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

Ver todas no PubMed

📚 EuropePMC193 artigos no totalmostrando 195

2026

Vagus nerve stimulation as an adjunctive therapy for super-refractory status epilepticus including NORSE: a retrospective cohort study.

Epilepsy &amp; behavior : E&amp;B
2026

[Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].

Rinsho shinkeigaku = Clinical neurology
2026

A model of new-onset refractory status epilepticus (NORSE) using intrahippocampal kainic acid in adolescent mice.

Epilepsy research
2026

Safety and efficacy of intrathecal rituximab in refractory neuroimmune disorders- a case series.

Journal of neuroimmunology
2026

Exploring pathways leading to drug-resistant epilepsy for patients with cryptogenic new onset refractory status epilepticus.

Epilepsia
2026

Extreme cryptogenic new onset refractory status epilepticus/febrile infection-related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury.

Epilepsia
2026

Pediatric new-onset super refractory status epilepticus: Potential of timely immunotherapy in a resource-limited PICU.

Seizure
2026

Efficacy differences between tocilizumab and ketogenic diet during acute phase of febrile Infection-Related epilepsy syndrome in children: A retrospective cohort study.

Epilepsy &amp; behavior : E&amp;B
2026

New-onset refractory status epilepticus associated with Sjögren's disease.

BMJ neurology open
2026

The Use of Electroconvulsive Therapy (ECT) in the New Onset Refractory Status Epilepticus (NORSE)-Case Report and Systematic Review.

The journal of ECT
2026

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

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

Fatal retroperitoneal hemorrhage following enoxaparin administration in a patient with autoimmune encephalitis: a case report.

Encephalitis (Seoul, Korea)
2025

Predicting epilepsy after new onset refractory status epilepticus due to autoimmune encephalitis: The DAME score.

Epilepsia
2025

Ketogenic Diet Therapy for Adult Febrile Infection-Related Epilepsy Syndrome (FIRES): A Case Series of Five Patients and Clinical Efficacy Analysis.

Infection and drug resistance
2025

Status Epilepsy Syndromes Made Easy: Pediatric Perspectives.

Children (Basel, Switzerland)
2025

Claustrum Sign: An Increasingly Recognized Imaging Feature of New-Onset Refractory Status Epilepticus (NORSE).

The Neurohospitalist
2026

Refractory Status Epilepticus Treated With Bilateral Pulvinar Deep Brain Stimulation-A Case Study.

Annals of clinical and translational neurology
2025

Emergence of Atypical Lymphocytes Before Cryptogenic New Onset Refractory Status Epilepticus: A Case Report.

The Neurohospitalist
2025

Case Report: Successful multimodal management of FIRES in a pediatric patient using anakinra, ketogenic diet, and induced hypothermia.

Frontiers in pediatrics
2026

Factors associated with long-term outcomes in people with new onset refractory epilepticus: A single-center cohort study.

Epilepsia
2026

Beyond Anakinra and Tocilizumab: Additional Adjunctive Therapies in Pediatric New Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome - A Narrative Review.

Pediatric neurology
2025

Deep brain stimulation of the anterior nucleus of the thalamus for seizures after new-onset refractory status epilepticus: a case report.

Frontiers in human neuroscience
2026

Iatrogenic NORSE: Immune checkpoint inhibitor-related anti-GFAP autoimmune astrocytopathy.

Journal of neuroimmunology
2025

Successful Treatment of Cryptogenic NORSE Resistant to Immunosuppression With Intravenous Ganaxolone and Electroconvulsive Therapy.

The Neurohospitalist
2025

Intensive 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 journal
2025

The 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 Society
2025

Early Recovery from Cryptogenic NORSE Following Repeated C-NORSE Score Assessment and Cyclophosphamide Treatment: A Case Report.

Internal medicine (Tokyo, Japan)
2025

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 neurology
2025

Hemorrhagic cystitis induced by intravenous esketamine in the treatment of new-onset refractory status epilepticus in children: a case report.

Frontiers in pediatrics
2025

New onset refractory status epilepticus diagnosed in the second trimester: A case report.

Obstetric medicine
2025

The spectrum of cognitive outcomes following admission to hospital for refractory status epilepticus (RSE): A scoping review.

Seizure
2026

Quality of life over time after new onset refractory status epilepticus.

Epilepsia
2026

An extreme type of new onset refractory status epilepticus with stimulus-induced seizures in pharmacological isoelectric states.

Epilepsia
2025

Standard complete blood count to predict long-term outcomes in febrile infection-related epilepsy syndrome (FIRES): A multicenter study.

Epilepsia
2025

Gastrointestinal 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 Neurophysiology
2025

Update in new-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

Current opinion in pediatrics
2025

Rescue use of electroconvulsive therapy in new-onset refractory status epilepticus during pregnancy.

BMJ case reports
2025

Immune dysregulation in new-onset refractory status epilepticus (NORSE): current insights and therapeutic perspectives.

Brain &amp; development
2025

Hypocupraemia-related drug-refractory seizures in Wilson disease.

Practical neurology
2025

Retrospective multicenter study on cryptogenic NORSE/FIRES patients treated with anakinra.

Seizure
2025

Analysis of Early EEG Changes After Tocilizumab Treatment in New-Onset Refractory Status Epilepticus.

Brain sciences
2025

The Role of Metabolic Testing in the Diagnostic Evaluation of Adult NORSE: A Retrospective, Single-Centre Study.

European journal of neurology
2025

Patients with status epilepticus and new-onset refractory status epilepticus display drastically altered fecal microbiomes compared to chronic epilepsy patients.

Epilepsia
2025

Identification of Distinct Biological Groups of Patients With Cryptogenic NORSE via Inflammatory Profiling.

Neurology(R) neuroimmunology &amp; neuroinflammation
2025

Autoimmune encephalitis associated with anti-SOX1 autoantibodies in COVID-19: A case report.

IDCases
2025

Pediatric new-onset super-refractory status epilepticus (NOSRSE): a case-series.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2025

Performance of cryptogenic new onset refractory status epilepticus score in a Brazilian cohort after testing for antineuronal antibodies with tissue-based and cell-based assays.

Epilepsia
2025

A Case Series and Review of Febrile-Infection Related Epilepsy Syndrome (FIRES).

Children (Basel, Switzerland)
2025

Intrathecal Dexamethasone as a FIRES Extinguisher: A 12-Patient Clinical Experience with Usage of Intrathecal Dexamethasone for Febrile Infection-Related Epilepsy Syndrome.

Neurocritical care
2025

Comparative analysis of new-onset refractory status epilepticus in adult and pediatric patients: immunotherapy timing and functional outcomes.

Journal of neurology
2025

Adult-onset epilepsy with startle-induced seizure after febrile infection-related epilepsy syndrome: A case report.

Epileptic disorders : international epilepsy journal with videotape
2024

Cytokines in new-onset refractory status epilepticus: ready for clinical use?

Acta epileptologica
2025

Clinical analysis of 7 cases of cryptogenic new-onset refractory status epilepticus.

Medicine
2025

CSF Tau Is a Biomarker of Hippocampal Injury in Cryptogenic New-Onset Refractory Status Epilepticus.

Annals of clinical and translational neurology
2025

Dramatic 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 Neurophysiology
2025

Clinical 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 Neurophysiology
2025

Phenotype, outcome, interleukins, and miRNA levels assessment of new-onset refractory status epilepticus: A prospective cohort study.

Epileptic disorders : international epilepsy journal with videotape
2025

Potential Role of Perampanel in Reducing Barbiturate Dependency in Febrile Infection-Related Epilepsy Syndrome: A Case Report.

Cureus
2025

Combined Systemic Immunotherapy and Intrathecal Dexamethasone in Febrile Infection Related Epilepsy Syndrome.

Annals of clinical and translational neurology
2025

New-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 neurology
2024

Multidisciplinary Approaches and Molecular Diagnostics in New-Onset Refractory Status Epilepticus and Nontuberculous Mycobacterial CNS Infections in the ICU: A Case Report.

Cureus
2025

New onset refractory status epilepticus: Long-term outcomes beyond seizures.

Epilepsia
2024

New-onset refractory status epilepticus after SARS-CoV-2 infection: a review of literature.

Croatian medical journal
2025

Age-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES Family Registry.

Epilepsia
2025

NORSE secondary to anti-GAD65 antibody-positive encephalitis treated with novel adjunctive rapid titration VNS protocol.

Epilepsia open
2025

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) as a cause of new-onset refractory status epilepticus (NORSE): Case report and review of literature.

Journal of neuroimmunology
2025

Navigating life after New-onset refractory status epilepticus (NORSE) and Febrile infection-related epilepsy syndrome (FIRES): Insights from caregiver and patient interviews.

Epilepsy &amp; behavior : E&amp;B
2025

Early initiation of intravenous cyclophosphamide and one-year outcome in super-refractory cryptogenic-new onset refractory status epilepticus.

Epilepsia open
2024

Corrigendum: A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.

Frontiers in neurology
2025

New-Onset Refractory Status Epilepticus With Diffuse Cerebral Restricted Diffusion in Young Children: A Novel Clinical-Radiologic Presentation.

Pediatric neurology
2024

Polygenic Landscape of Cryptogenic New-Onset Refractory Status Epilepticus: A Comprehensive Whole-Genome Sequencing Study.

Annals of neurology
2025

Cryptogenic 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)
2024

Communication trends over time in new-onset refractory status epilepticus (NORSE): Interim analysis from the NORSE/FIRES Family Registry.

Epilepsy &amp; behavior : E&amp;B
2024

Prognosis prediction and immunotherapy optimisation for cryptogenic new-onset refractory status epilepticus.

Journal of neurology, neurosurgery, and psychiatry
2025

POLG epilepsy presenting as new-onset refractory status epilepticus (NORSE) in pregnancy.

Practical neurology
2024

New onset status epilepticus and its long-term outcome: A cohort study.

Epilepsy research
2024

NORSE/FIRES: how can we advance our understanding of this devastating condition?

Frontiers in neurology
2024

IT-DEX and B cell depletion in a child with anti-GAD 65 autoimmune encephalitis presenting as NORSE: A case report.

Journal of neuroimmunology
2024

Identification of etiologies according to baseline clinical features of pediatric new-onset refractory status epilepticus in single center retrospective study.

Seizure
2024

Trends in management of patients with new-onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis.

Epilepsia
2024

Autoimmune-associated seizure disorders.

Epileptic disorders : international epilepsy journal with videotape
2024

Single-Cell Transcriptomic Analyses of Brain Parenchyma in Patients With New-Onset Refractory Status Epilepticus (NORSE).

Neurology(R) neuroimmunology &amp; neuroinflammation
2024

Electroconvulsive therapy in new onset refractory status epilepticus (NORSE) in a pediatric patient.

Brain stimulation
2024

FADD gene pathogenic variants causing recurrent febrile infection-related epilepsy syndrome: Case report and literature review.

Epilepsia
2024

Treatment of Cryptogenic New-onset Refractory Status Epilepticus (C-NORSE) with Tocilizumab.

Internal medicine (Tokyo, Japan)
2024

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 Neurophysiology
2024

NORSE seasonality may vary geographically in adults.

Epilepsia open
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.

Epilepsia
2024

Clinical outcomes among initial survivors of cryptogenic new-onset refractory status epilepsy (NORSE).

Epilepsia
2024

Long-term outcome in new onset refractory status epilepticus: a retrospective study.

Critical care (London, England)
2024

Status epilepticus: what's new for the intensivist.

Current opinion in critical care
2024

Second-line immunotherapy in new onset refractory status epilepticus.

Epilepsia
2024

New onset refractory status epilepticus: Summary of the NORSE Institute roundtable during the 2023 American Epilepsy Society meeting, Orlando, Florida.

Epilepsia
2024

Successfully treated anti-GAD limbic encephalitis in a 15-year-old diabetic boy with intravenous immunoglobulin: case report.

Annals of medicine and surgery (2012)
2024

Time to FIRE NORSE: A single acronym for a heterogeneous presentation. Further information from a case series and discussion of the literature.

Journal of neuroimmunology
2023

Refractory Status Epilepticus Associated With a Pathogenic Variant in TNFRSF13B.

Cureus
2023

[Initial Management of Status Epilepticus].

No shinkei geka. Neurological surgery
2023

[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 neurology
2024

Severe, Refractory Seizures: New-Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome.

The Medical clinics of North America
2024

Rasmussen's syndrome treated with anakinra.

Journal of neurology
2024

Refractory status epilepticus arrested by vagus nerve stimulation.

Practical neurology
2023

Multimodal Management of Febrile Infection-Related Epilepsy Syndrome in a 17-Year-Old Male.

Cureus
2023

Lidocaine as a potential therapeutic option for super-refractory status epilepticus: A case report.

Journal of central nervous system disease
2023

A case of new onset refractory status epilepticus in a U.S. traveler with Japanese encephalitis.

Journal of neuroimmunology
2023

The role of neuronal antibodies in cryptogenic new onset refractory status epilepticus.

Epilepsia
2023

Anti-N-methyl D-aspartate receptor encephalitis presenting with the new-onset refractory status epilepticus.

Northern clinics of Istanbul
2023

The Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome (FIRES).

Journal of the Belgian Society of Radiology
2023

Neuromodulation in new-onset refractory status epilepticus.

Frontiers in neurology
2023

Safe and effective implantation and use of vagal nerve stimulation in new-onset refractory status epilepticus in early pregnancy: a case report.

Frontiers in neurology
2023

A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.

Frontiers in neurology
2023

Perspective: Vagal nerve stimulation in the treatment of new-onset refractory status epilepticus.

Frontiers in neurology
2023

Ketogenic diet for super-refractory status epilepticus (SRSE) with NORSE and FIRES: Single tertiary center experience and literature data.

Frontiers in neurology
2023

Early identification of NORSE and transfer to care setting with appropriate supports: A proposed algorithm.

Frontiers in neurology
2023

New-Onset Refractory Status Epilepticus Due to a Novel MT-TF Variant: Time for Acute Genetic Testing Before Treatment?

Neurology. Genetics
2023

New-onset refractory status epilepticus responsive to VNS: A case report.

Epileptic disorders : international epilepsy journal with videotape
2023

Neuropathology of New-Onset Refractory Status Epilepticus (NORSE).

Journal of neurology
2023

Investigating the genetic contribution in febrile infection-related epilepsy syndrome and refractory status epilepticus.

Frontiers in neurology
2023

Review and standard operating procedures for collection of biospecimens and analysis of biomarkers in new onset refractory status epilepticus.

Epilepsia
2023

The seasonality of new-onset refractory status epilepticus (NORSE).

Epilepsia
2023

Long-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra.

Frontiers in neurology
2023

Treatment of new onset refractory status epilepticus/febrile infection-related epilepsy syndrome with tocilizumab in a child and a young adult.

Epilepsia
2023

Blood-brain barrier damage and new onset refractory status epilepticus: An exploratory study using dynamic contrast-enhanced magnetic resonance imaging.

Epilepsia
2023

Cytokines in New-Onset Refractory Status Epilepticus Predict Outcomes.

Annals of neurology
2023

Specific profiles of new-onset vs. non-inaugural status epilepticus: From diagnosis to 1-year outcome.

Frontiers in neurology
2023

New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

Current opinion in neurology
2023

Seizure burden and neuropsychological outcomes of new-onset refractory status epilepticus: Systematic review.

Frontiers in neurology
2023

The etiology and mortality of new-onset refractory status epilepticus (NORSE) in adults: A systematic review and meta-analysis.

Epilepsia
2022

A case report: New-onset refractory status epilepticus in a patient with FASTKD2-related mitochondrial disease.

Frontiers in neurology
2022

Long-term outcomes of adult cryptogenic febrile infection-related epilepsy syndrome (FIRES).

Frontiers in neurology
2022

The Increased Interleukin-6 Levels Can Be an Early Diagnostic Marker for New-Onset Refractory Status Epilepticus.

Journal of epilepsy research
2023

Pearls & Oy-sters: Status Epilepticus and Cerebral Edema From Hyperammonemia Due to Disseminated Ureaplasma and Mycoplasma Species.

Neurology
2022

Early clinical features of new-onset refractory status epilepticus (NORSE) in adults.

BMC neurology
2022

New-onset refractory status epilepticus (NORSE) in paediatric patients: causes, characteristics and outcomes.

BMJ neurology open
2022

Febrile infection-related Epilepsy Syndrome (FIRES): a severe encephalopathy with status epilepticus. Literature review and presentation of two new cases.

Italian journal of pediatrics
2023

Surviving status epilepticus: The patient and family perspective.

Epilepsy &amp; behavior : E&amp;B
2022

Role of responsive neurostimulation and immunotherapy in refractory epilepsy due to autoimmune encephalitis: A case report.

Frontiers in neurology
2022

Cannabidiol in refractory status epilepticus: A review of clinical experiences.

Seizure
2023

Inflammation as Treatment Target for Status Epilepticus.

Current neuropharmacology
2023

Reversible Brain Atrophy in Cryptogenic New-onset Refractory Status Epilepticus.

Internal medicine (Tokyo, Japan)
2022

Intravenous ganaxolone in pediatric super-refractory status epilepticus: A single hospital experience.

Epilepsy &amp; behavior reports
2023

Multi-proteomic Analysis Revealed Distinct Protein Profiles in Cerebrospinal Fluid of Patients Between Anti-NMDAR Encephalitis NORSE and Cryptogenic NORSE.

Molecular neurobiology
2022

Parallel roles of neuroinflammation in feline and human epilepsies.

Veterinary journal (London, England : 1997)
2022

Long-term outcomes after NORSE: Treatment with vagus nerve stimulation.

Epilepsia open
2022

New-Onset Refractory Status Epilepticus Secondary to COVID-19 Infection in Adults: A Systematic Review.

Neuropsychiatric disease and treatment
2022

New-onset refractory status epilepticus due to autoimmune encephalitis after vaccination against SARS-CoV-2: First case report.

Frontiers in neurology
2022

International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) incl. Febrile Infection-Related Epilepsy Syndrome (FIRES): Statements and Supporting Evidence.

Epilepsia
2022

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

Child Neurology: Initial Presentation of PCDH19-Related Epilepsy With New-Onset Refractory Status Epilepticus and Treatment With Anakinra.

Neurology
2022

A retrospective cohort study of new-onset refractory status epilepticus (NORSE): clinical features, timing of immunotherapy and outcomes.

Epileptic disorders : international epilepsy journal with videotape
2023

Rapidly evolving Creutzfeldt-Jakob disease in COVID-19: from early status epilepticus to fatal outcome.

Acta neurologica Belgica
2022

Fever-Associated Seizures or Epilepsy: An Overview of Old and Recent Literature Acquisitions.

Frontiers in pediatrics
2022

A 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 Neurophysiology
2022

Neurosyphilis presenting as new onset refractory status epilepticus: one of the multifarious faces of a great imitator.

Sexual health
2022

Autoimmune encephalitis associated with COVID-19: A systematic review.

Multiple sclerosis and related disorders
2022

An ultra-long new onset refractory status epilepticus: Winning the battle but losing the war?

Epilepsy &amp; behavior reports
2022

Zebras Seize the Day: Rare Causes of Status Epilepticus Across the Continuum of Critical Care.

Critical care clinics
2022

New-onset refractory status epilepticus as an early manifestation of multisystem inflammatory syndrome in adults after COVID-19.

Epilepsia
2022

An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report.

Case reports in neurology
2022

New onset refractory status epilepticus and neuroendocrine tumour: a case report and review of the literature.

Acta neurologica Belgica
2022

Specific 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 Society
2022

New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) of unknown aetiology: A comparison of the incomparable?

Seizure
2022

New onset refractory status epilepticus: State of the art.

Revue neurologique
2021

New Onset Refractory Status Epilepticus as a Manifestation of Tuberculosis of the Central Nervous System.

Neurology India
2022

Cryptogenic new-onset refractory status epilepticus responded to anti-interleukin-6 treatment.

Journal of neuroimmunology
2022

Corrigendum to 'New onset refractory status epilepticus secondary to HIV CNS viral escape syndrome: Case report'.

Seizure
2022

High dose cannabidiol (CBD) in the treatment of new-onset refractory status epilepticus (NORSE).

Seizure
2022

New onset refractory status epilepticus secondary to HIV CNS viral escape syndrome: Case report.

Seizure
2021

A retrospective study of 92 children with new-onset refractory status epilepticus.

Epilepsy &amp; behavior : E&amp;B
2021

New-Onset Refractory Status Epilepticus (NORSE): The Queen Square Neuro-ICU experience.

Epilepsy &amp; behavior : E&amp;B
2021

A Role for Electroconvulsive Therapy in the Management of New Onset Refractory Status Epilepticus (NORSE) in a Young Child.

Neurology India
2021

Histopathology of new-onset refractory status epilepticus (NORSE) in adults.

Seizure
2021

Idiopathic Hypoparathyroidism Presenting As New Onset Refractory Status Epilepticus.

Journal of neurosciences in rural practice
2022

Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies.

European journal of neurology
2021

Anti-N-methyl-d-aspartate Receptor Encephalitis Presenting as New-onset Refractory Status Epilepticus Responding to Rituximab in an Adolescent Girl.

Journal of pediatric neurosciences
2021

Predictors and prognoses of epilepsy after anti-neuronal antibody-positive autoimmune encephalitis.

Seizure
2021

Infantile Hemiconvulsion-Hemiplegia and Epilepsy (IHHE) in a boy with tuberous sclerosis complex.

Epilepsy &amp; behavior reports
2021

Benzodiazepine administration patterns before escalation to second-line medications in pediatric refractory convulsive status epilepticus.

Epilepsia
2021

New-Onset Refractory Status Epilepticus (NORSE) as a Recurrence of Anti-Neuronal Nuclear Antibody 2 (ANNA-2) Encephalitis After Immune Checkpoint Inhibition Therapy.

Cureus
2021

Super-Refractory Status Epilepticus: Prognosis and Recent Advances in Management.

Aging and disease
2021

Understanding new-onset refractory status epilepticus from an immunological point of view.

Encephalitis (Seoul, Korea)
2021

New-onset refractory status epilepticus following the ChAdOx1 nCoV-19 vaccine.

Journal of neuroimmunology
2021

Parental perception of FIRES outcomes, emotional states, and social media usage.

Epilepsia open
2021

Permanent loss of independence in adult febrile-infection-related epilepsy syndrome survivors: an underestimated and unsolved challenge.

European journal of neurology
2021

Clinical presentation of new onset refractory status epilepticus in children (the pSERG cohort).

Epilepsia
2021

Super-refractory status epilepticus in autoimmune encephalitis treated with interleukin-1 receptor antagonist, anakinra.

Epileptic disorders : international epilepsy journal with videotape
2021

[Anti-NMDA Receptor Encephalitis].

Brain and nerve = Shinkei kenkyu no shinpo
2021

Cryptogenic new-onset refractory status epilepticus (NORSE) following blood transfusion in a patient with severe anemia.

Epileptic disorders : international epilepsy journal with videotape
2021

COVID-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 Neurophysiology
2021

Remote Teamwork Management of NORSE During the COVID-19 Lockdown.

Neurology. Clinical practice
2021

Predictors of outcome in super refractory status epilepticus.

Epilepsy &amp; behavior : E&amp;B
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.

Epilepsy currents
2021

Resolution of cryptogenic new onset refractory status epilepticus with tocilizumab.

Epilepsy &amp; behavior reports
2021

Intravenous immunoglobulin response in new-onset refractory status epilepticus (NORSE) COVID-19 adult patients.

Journal of neurology
2021

Proposal to optimize evaluation and treatment of Febrile infection-related epilepsy syndrome (FIRES): A Report from FIRES workshop.

Epilepsia open

Associações

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Doenças relacionadas

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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.

  1. [Febrile infection-related epilepsy syndrome (FIRES) with the claustrum sign in a 55-year-old woman: a case report].
    Rinsho shinkeigaku = Clinical neurology· 2026· PMID 41813187mais citado
  2. New-onset refractory status epilepticus associated with Sj&#xf6;gren's disease.
    BMJ neurology open· 2026· PMID 41561677mais citado
  3. 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
  4. 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
  5. Refractory Status Epilepticus Treated With Bilateral Pulvinar Deep Brain Stimulation-A Case Study.
    Annals of clinical and translational neurology· 2026· PMID 41345856mais citado
  6. Role of Tocilizumab in Severe CNS Inflammatory Presentations in Children.
    Neurology· 2026· PMID 41945878recente
  7. Vagus nerve stimulation as an adjunctive therapy for super-refractory status epilepticus including NORSE: a retrospective cohort study.
    Epilepsy Behav· 2026· PMID 41875755recente
  8. A model of new-onset refractory status epilepticus (NORSE) using intrahippocampal kainic acid in adolescent mice.
    Epilepsy Res· 2026· PMID 41762487recente
  9. Safety and efficacy of intrathecal rituximab in refractory neuroimmune disorders- a case series.
    J Neuroimmunol· 2026· PMID 41702141recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:363558(Orphanet)
  2. MONDO:0018199(MONDO)
  3. GARD:12244(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Estado de mal epiléptico refratário de início precoce
Compêndio · Raras BR

Estado de mal epiléptico refratário de início precoce

ORPHA:363558 · MONDO:0018199
Prevalência
Unknown
CID-10
G41.8 · Outros estados de mal epiléptico
Ensaios
1 ativos
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C4749462
EuropePMC
Wikidata
Papers 10a
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