Raras
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Encefalite receptor NMDA
ORPHA:217253CID-10 · G13.1DOENÇA RARA

É uma inflamação grave e repentina do cérebro, conhecida como encefalite aguda, causada por uma reação autoimune. Isso acontece quando os anticorpos do próprio corpo atacam uma parte específica do cérebro chamada receptor NMDA de glutamato. Geralmente afeta mulheres e, na maioria dos casos, está associada à presença de um tumor, sendo o mais comum um teratoma no ovário. Quando há um tumor nesses pacientes, a encefalite é considerada uma "síndrome paraneoplásica". Isso significa que o problema no cérebro é uma reação do sistema imunológico ao tumor que está em outra parte do corpo. Os sintomas incluem alterações psiquiátricas (como confusão, agitação, delírios) e crises epilépticas (convulsões). É uma condição que pode ser fatal. No entanto, geralmente é reversível se o tumor for removido rapidamente.

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Introdução

O que você precisa saber de cara

📋

É uma inflamação grave e repentina do cérebro, conhecida como encefalite aguda, causada por uma reação autoimune. Isso acontece quando os anticorpos do próprio corpo atacam uma parte específica do cérebro chamada receptor NMDA de glutamato. Geralmente afeta mulheres e, na maioria dos casos, está associada à presença de um tumor, sendo o mais comum um teratoma no ovário. Quando há um tumor nesses pacientes, a encefalite é considerada uma "síndrome paraneoplásica". Isso significa que o problema no cérebro é uma reação do sistema imunológico ao tumor que está em outra parte do corpo. Os sintomas incluem alterações psiquiátricas (como confusão, agitação, delírios) e crises epilépticas (convulsões). É uma condição que pode ser fatal. No entanto, geralmente é reversível se o tumor for removido rapidamente.

Pesquisas ativas
3 ensaios
9 total registrados no ClinicalTrials.gov
Publicações científicas
984 artigos
Último publicado: 2026 Apr 16

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
All ages
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G13.1
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Sinais e sintomas

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

Partes do corpo afetadas

🧠
Neurológico
10 sintomas
💪
Músculos
2 sintomas
🫃
Digestivo
2 sintomas
🧬
Pele e cabelo
2 sintomas
🩸
Sangue
1 sintomas
❤️
Coração
1 sintomas

+ 34 sintomas em outras categorias

Características mais comuns

90%prev.
Déficit de atenção
Muito frequente (99-80%)
90%prev.
Positividade para anticorpo anti-receptor NMDA
Muito frequente (99-80%)
90%prev.
Pleocitose do LCR
Muito frequente (99-80%)
90%prev.
Comprometimento da memória
Muito frequente (99-80%)
90%prev.
Confusão
Muito frequente (99-80%)
90%prev.
Comportamento atípico
Muito frequente (99-80%)
53sintomas
Muito frequente (8)
Frequente (14)
Ocasional (25)
Muito raro (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 53 características clínicas mais associadas, ordenadas por frequência.

Déficit de atençãoShort attention span
Muito frequente (99-80%)90%
Positividade para anticorpo anti-receptor NMDAAnti-NMDA receptor antibody positivity
Muito frequente (99-80%)90%
Pleocitose do LCRCSF pleocytosis
Muito frequente (99-80%)90%
Comprometimento da memóriaMemory impairment
Muito frequente (99-80%)90%
ConfusãoConfusion
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico984PubMed
Últimos 10 anos200publicações
Pico202580 papers
Linha do tempo
2026Hoje · 2026🧪 2012Primeiro ensaio clínico📈 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.
2Fase 21
·Pré-clínico7
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 8 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Encefalite receptor NMDA

🗺️

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

🟢 Recrutando agora

3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

9 ensaios clínicos encontrados, 3 ativos.

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

Timeline de publicações
782 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 782

#1

Complications, outcomes, and implications of a prolonged vegetative state in anti-NMDA receptor encephalitis: a retrospective international cohort study.

The Lancet. Neurology2026 Apr

In anti-NMDA receptor (NMDAR) encephalitis, delayed recovery and slow improvement makes it difficult to assess treatment refractoriness, leaving a knowledge gap about prolonged impaired consciousness. We aimed to assess treatment response, long-term outcomes, and their predictors in patients with NMDAR encephalitis and a prolonged vegetative state. In this international retrospective cohort study, we included patients with NMDAR encephalitis from Jan 1, 2007 to Sept 30, 2024 who remained in a vegetative state (unresponsive wakefulness) for 9 months or longer in 21 hospitals across Austria, Brazil, Chile, China, Germany, Hong Kong, Japan, the Netherlands, Spain, Switzerland, and the USA. Data on disease presentation and long-term outcomes were collected via medical record review and a structured questionnaire sent to referring physicians. Outcomes were death, ability to follow commands, reaching a modified Rankin Scale (mRS) score of 2, or complete recovery (mRS 0 with return to premorbid activities). Competing risks analysis was used to assess survival and predictors of death, mRS score 2, and recovery. 45 patients were identified (38 female and seven male; median age 22 [IQR 19-31] years). The patients had impaired consciousness a median of 9 days after symptom onset (IQR 5-16). All 45 patients received first-line immunotherapy; 41 (91%) received second-line, and 18 (40%) received third-line immunotherapies. 21 (47%) of 45 patients had ovarian teratomas, which were removed in 20 patients. Median durations were: vegetative state 399 days (IQR 307-698); intensive care unit stay 275 days (189-354); mechanical ventilation 270 days (195-394); and hospitalisation 474 days (349-715). 13 (28%) patients were resuscitated from dysautonomic cardiac arrest. After a median of 5 years (IQR 3-7), 15 (33%) of 45 patients completely recovered (mRS score ≤1 and returned to all previous activities), 13 (29%) substantially improved (mRS score 2), 11 (24%) had mRS score 3-5, and six (13%) died. Five patients began command-following a median of 11 months (IQR 2-21) after last immunotherapy. Estimated cumulative incidence for reaching an mRS score of 2 was 66% at 5 years and 76% at 10 years, and for recovery was 32% at 5 years and 54% at 10 years. Teratomas were associated with a lower probability of reaching mRS score 2 (sub-distribution hazard ratio 0·39 [95% CI 0·18-0·84], p=0·0160), whereas older age (1·10 per year [1·04-1·23], p=0·0052) and higher NMDAR encephalitis 1-Year Functional Status (NEOS)2 score (1·51 [1·12-2·04], p=0·0072) were associated with increased mortality. In people with NMDAR encephalitis and prolonged impairment of consciousness, full or substantial recovery was reached in approximately two-thirds of cases. Consequently, early assessment of treatment response or refractoriness might underestimate delayed improvement, prolonged therapy needs, or spontaneous recovery. Futility decisions should therefore be individualised with multidisciplinary input and based on extended follow-up. Instituto de Salud Carlos III and Fundació La Caixa.

#2

Determinants of disease severity and 12-month functional outcome in pediatric ovarian Teratoma-Associated Anti-NMDA receptor encephalitis.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society2026 Feb 06

Pediatric ovarian teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) represents a clinically important but relatively uncommon subgroup, and evidence regarding factors associated with acute severity and long-term functional outcome in this population remains limited. We conducted a single-center retrospective cohort study of pediatric patients with surgically resected, histopathologically confirmed ovarian teratoma-associated NMDARE. Baseline and acute-phase characteristics were summarized in the full cohort (N = 97). Acute severity was defined by ICU admission with organ support and/or mechanical ventilation. Twelve-month functional outcome was assessed using the modified Rankin Scale (mRS) and dichotomized as good (mRS≤2) versus poor (mRS>2); outcome analyses were performed in the complete-case cohort (N = 71). Univariable and multivariable logistic regression identified factors independently associated with poor outcome. Discriminative performance was evaluated using ROC curve analysis. Among 97 patients, median age at onset was 14.2 years (IQR 12.3-16.5) and 95.9% were female. Mature teratoma accounted for 80.4%. The median onset-to-resection interval was 18 days (IQR 11-29), and 34.0% underwent resection >21 days after onset. Severe disease occurred in 29 patients (29.9%) and was associated with higher symptom burden (median symptom categories 5.0 vs 3.0, P < 0.001), more frequent MRI abnormalities (62.1% vs 33.8%, P = 0.009) and multifocal MRI lesions (27.6% vs 7.4%, P = 0.013), and higher rates of EEG background slowing (89.7% vs 61.8%, P = 0.014) and extreme delta-brush (44.8% vs 13.2%, P = 0.001). In the 12-month outcome cohort (N = 71), 76.1% achieved mRS≤2 and 23.9% had mRS>2; relapse occurred in 8.5% and death in 2.8%. Multivariable analysis identified central hypoventilation (aOR 4.18, 95%CI 1.62-10.78), symptom categories ≥4 (aOR 2.94, 1.14-7.61), multifocal MRI lesions (aOR 3.12, 1.01-9.65), extreme delta-brush (aOR 3.37, 1.18-9.63), and onset-to-resection >21 days (aOR 2.81, 1.05-7.49) as independent correlates of poor functional outcome. The combined clinical plus imaging-EEG model showed the highest discrimination (AUC 0.86, 95%CI 0.76-0.95), with sensitivity 88.2% and specificity 74.1% at the optimal cutoff. In pediatric ovarian teratoma-associated NMDARE, higher acute-phase clinical burden, multifocal MRI abnormalities, extreme delta-brush, central hypoventilation, and delayed tumor resection were independently associated with poor 12-month functional outcome.

#3

Transition Readiness and Care Needs Among Youth With Neuroimmune Disorders: A Descriptive Survey Study.

Journal of child neurology2026 Jan 06

Transition of care (TOC) poses challenges across medical subspecialties, each with distinct patient needs. Understanding patient and caregiver awareness and readiness within specific patient populations is essential for developing effective population-specific TOC programs. Limited data exist describing TOC readiness in patients with neuroimmune disorders. We conducted a cross-sectional survey on youth with neuroimmune disorders and their caregivers assessing TOC readiness, challenges, and support needs. Thirty-one participants completed the survey (39% Hispanic, 29% Caucasian, 19% African American). Respondents with MOG antibody-associated disease (29%), multiple sclerosis (26%), and anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis (10%) were represented in this study. Eighty-three percent of caregivers reported their child was not ready for transition, whereas 32% of patients described feeling curious about TOC. The primary concern for both patients (53%) and caregivers (65%) was that adult providers would be unfamiliar with the patient's history. The most common patient-perceived readiness gaps included lack of knowledge of clinic information (81%), not having an emergency plan (77%), and poor understanding of insurance changes (81%). The most frequent patient-reported facilitators were access to an educational curriculum (35%) or a dedicated transition appointment (35%). More than half of patients (52%) and caregivers (61%) preferred a continuity-provider TOC model, and most supported participation in TOC support groups (55% of patients, 87% of caregivers). To conclude, significant gaps exist in TOC readiness among the neuroimmune disorder population. Key facilitators include structured education, provider continuity, and support programming-elements that could strengthen preparedness, reduce care disruption, and increase confidence in navigating adult health care systems.

#4

Therapeutic perspectives in refractory anti-NMDA receptor encephalitis.

The Lancet. Neurology2026 Apr
#5

Epilepsia partialis continua as an isolated manifestation of anti-NMDA receptor encephalitis: a case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026 Feb 02

Publicações recentes

Ver todas no PubMed

📚 EuropePMC702 artigos no totalmostrando 198

2026

Complications, outcomes, and implications of a prolonged vegetative state in anti-NMDA receptor encephalitis: a retrospective international cohort study.

The Lancet. Neurology
2026

Therapeutic perspectives in refractory anti-NMDA receptor encephalitis.

The Lancet. Neurology
2026

Anti-NMDA receptor encephalitis in the setting of inherited chromosomally integrated human herpesvirus 6B (iciHHV-6B): A case report.

IDCases
2026

Refractory anti-NMDA receptor encephalitis driven by a contralateral ovarian teratoma following unilateral oophorectomy: a case report.

International journal of surgery case reports
2026

CAR T cell therapy in autoantibody-mediated neurological disorders: a promising strategy.

Journal of neuroinflammation
2026

Neuropsychiatric Systemic Lupus Erythematosus Diagnosed Following Treatment Initiated for Acute Encephalitis.

Cureus
2026

Determinants of disease severity and 12-month functional outcome in pediatric ovarian Teratoma-Associated Anti-NMDA receptor encephalitis.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2026

Extreme delta brush EEG pattern in anti-NMDA receptor encephalitis.

QJM : monthly journal of the Association of Physicians
2026

Abnormal Magnetic Resonance Imaging Patterns in Patients with Neuropsychiatric Disorders Due to Anti-NMDA Receptor Encephalitis: A Comparative Study.

Diagnostics (Basel, Switzerland)
2026

Anti-NMDA Receptor Encephalitis Presenting as Acute Psychosis.

Clinical case reports
2026

Two Cases of vNOTES Assisted Vaginal Adnexectomy in Management of Paraneoplastic Anti-N-Methyl-D-Aspartate Receptor Encephalitis Secondary to Ovarian Teratoma.

Asian journal of endoscopic surgery
2026

Epilepsia partialis continua as an isolated manifestation of anti-NMDA receptor encephalitis: a case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2025

Anti-NMDA receptor encephalitis unmasking Sjögren's disease: a case report and literature review.

Frontiers in immunology
2026

Transition Readiness and Care Needs Among Youth With Neuroimmune Disorders: A Descriptive Survey Study.

Journal of child neurology
2025

Trapped in Silence: Severe Catatonia From Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis in a Neurofibromatosis Type 1 Patient With Craniopharyngioma.

Cureus
2026

Immature ovarian teratomas in anti-NMDA receptor encephalitis.

Journal of neuroimmunology
2025

Immune involvement in neuropsychiatric disorders: Insights from single-cell transcriptomic studies.

Psychiatry and clinical neurosciences
2026

Electroencephalography, microRNA, and anti-NMDA receptor encephalitis.

Clinica chimica acta; international journal of clinical chemistry
2025

Anti-NMDA receptor encephalitis with coexisting autoimmune GFAP astrocytopathy presenting with psychiatric symptoms leading to a suicide attempt: a case report.

International journal of emergency medicine
2025

Life-Threatening Bradycardia in Anti-NMDA-Receptor Encephalitis and a Novel Use for Permanent Pacing.

Annals of clinical and translational neurology
2025

Paediatric myelin oligodendrocyte glycoprotein antibody-associated disease with NMDA receptor encephalitis: overlap syndrome and challenges in the antibody testing.

BMJ case reports
2026

[Clinical challenge in anti-NMDA receptor encephalitis-A psychiatric case report].

Der Nervenarzt
2025

Comparison of the Efficacy of Polyvalent Intravenous Immunoglobulins and Therapeutic Plasma Exchange in Anti-NMDAR Encephalitis in the ICU.

Neurocritical care
2025

Severe anti-NMDA receptor encephalitis in a pediatric patient presenting with status dystonicus as the primary clinical manifestation: A case report (CARE).

Medicine
2025

Characterizing CSF inflammatory proteomics in pediatric post-hemorrhagic hydrocephalus and Anti-NMDAR encephalitis.

Journal of neuroinflammation
2025

Deciphering the molecular landscape of NMDAR-E associated ovarian teratomas: a Multi-Omics approach.

Journal of ovarian research
2025

Anti-NMDA receptor encephalitis following Murray Valley encephalitis: a case report.

ASM case reports
2025

Association between visual hallucinations and α-synuclein oligomers in patients with dementia with Lewy bodies.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2025

Combination therapy of ofatumumab and daratumumab in patients with severe anti-NMDA receptor encephalitis.

Frontiers in immunology
2025

Diagnostic Challenges in a Patient With Systemic Lupus Erythematosus (SLE) and Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis: A Case Report.

Cureus
2025

N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis Without Electroencephalogram Abnormalities or Cerebrospinal Fluid Pleocytosis Associated With a Tiny Ovarian Teratoma: A Case Report.

Cureus
2025

Paraneoplastic Anti-NMDA Receptor Encephalitis in a Nigerian Female Teenager: A Case Report.

Nigerian medical journal : journal of the Nigeria Medical Association
2025

Autoimmune neuropsychiatric disorders manifesting with psychosis.

The Journal of clinical investigation
2025

Systemic Complications of Status Epilepticus.

Current neurology and neuroscience reports
2025

Seronegative but Cerebrospinal Fluid-positive NMDA Receptor Encephalitis in an Alzheimer's Dementia Patient with Good Treatment Outcome: A Case Report.

Acta neurologica Taiwanica
2025

Child Neurology: Parainfectious Anti-NMDAR Encephalitis With Anti-MOG and Anti-AQP4 Seropositivity in an Infant.

Neurology
2025

Electroconvulsive Therapy for Catatonia in NMDA Receptor Encephalitis Unresponsive to Immunotherapy in a Five Year Old.

Pediatrics
2025

Atypical Anti-NMDAR encephalitis presenting with pharyngeal-cervical-bibrachial weakness: A rare case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2025

Acquired epileptic aphasia in NMDA encephalitis: a unique electroclinical presentation with pathophysiological considerations.

BMJ case reports
2025

A case of anti-N-methyl-D-aspartate receptor encephalitis with associated ovarian teratoma in a 23-year-old female.

Radiology case reports
2025

Neurochemical and molecular characteristics of altered brain functional activity in the anti-NMDAR encephalitis.

Neurobiology of disease
2025

Posterior Reversible Encephalopathy Syndrome: A Case Report With Contemporary Literature Review and Neuropathological Evidence From Autopsy.

Cureus
2025

Anti-NMDA receptor encephalitis following SARS-CoV-2 infection in children.

Diagnostic microbiology and infectious disease
2025

Anti-NMDA receptor encephalitis in a patient with 18p deletion syndrome.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2025

Brain Metabolite Levels in the Post-Acute Stage of Anti-NMDA Receptor Encephalitis and Schizophrenia: A Longitudinal Case-Control Study.

Biological psychiatry
2025

Anti-N-methyl-ᴅ-aspartate receptor encephalitis preceding stroke: report of an unusual case with literature review.

Encephalitis (Seoul, Korea)
2025

Mind in Crisis, Ovary in Question: A Case of Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis Associated With Ovarian Teratoma.

Cureus
2025

NMDAR autoimmune encephalitis associated with Sjögren's syndrome.

BMJ case reports
2025

Monoclonal humanized monovalent antibody blocking therapy for anti-NMDA receptor encephalitis.

Nature communications
2025

Anti-NMDA Receptor Encephalitis in the Setting of an Immature Ovarian Teratoma: A Case Report and Literature Review.

Case reports in obstetrics and gynecology
2025

Anti-NMDA receptor encephalitis in a child with H3K27M mutant pontine diffuse midline glioma.

BMJ case reports
2025

Striking pattern on [18F]FDG PET/CT in anti-NMDA receptor encephalitis.

Revista espanola de medicina nuclear e imagen molecular
2025

An updated, comprehensive meta-analysis of the treatment of anti-NMDAR encephalitis: Analysis, equipoise, and the urgent need for evidence over anecdote.

Journal of neuroimmunology
2025

Reversal of profound cortical atrophy with immunotherapy in pediatric anti-NMDA receptor encephalitis.

Journal of neuroimmunology
2025

Anti-NMDA Receptor Encephalitis: A Narrative Review.

Brain sciences
2025

Atypical Autoimmune Encephalitis: Diagnostic Challenges and Therapeutic Insights From a Case Series.

Cureus
2025

Risk factors for poor response to initial first-line immunotherapy and subsequent immunotherapy on prognosis in pediatric anti-NMDA receptor encephalitis.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2025

The Clinical Significance of Abnormal Electroencephalography (EEG) Patterns in Patients with Neuropsychiatric Disorders Due to Anti-NMDA Receptor Encephalitis: A Comparative Study.

Diagnostics (Basel, Switzerland)
2025

Development of anti-NMDA receptor encephalitis in a patient with multiple sclerosis.

BMJ case reports
2025

Spectrum of Neurological Manifestations of HHV-6 Encephalitis in Immunocompetent Children - A Retrospective Study at a Tertiary Care Center in South India.

Annals of Indian Academy of Neurology
2025

Autoimmune psychosis: Psychopathological patterns and outcome after immunotherapy.

Schizophrenia research
2025

A case report of NMDAR encephalopathy - A neurologic condition that a gynecologist can cure.

International journal of surgery case reports
2025

Red flag: A notable proportion of anti-NMDAR encephalitis cases may be admitted to psychiatric units in general hospitals.

General hospital psychiatry
2025

GTE score on evaluating the degree of neurological impairment in anti-NMDA receptor encephalitis.

Scientific reports
2025

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

Journal of neurology
2025

Longitudinal Evolution of Posterior Cortical Atrophy: Diagnostic Delays, Overlapping Phenotypes, and Clinical Outcomes.

Neurology
2025

Double Trouble: Herpes Simplex Encephalitis Triggering Autoimmune Encephalitis.

Cureus
2025

Recurrent anti-NMDA receptor encephalitis in first-trimester pregnancy with initially antibody-negative CSF.

Journal of neuroimmunology
2025

Diagnosis and Management of Children With Atypical Neuroinflammation.

Neurology
2025

Successful Treatment of Anti-N-Methyl-D-Aspartate Receptor Encephalitis With Bilateral Ovarian Teratomas Through Three Surgeries Without Loss of Fertility.

Cureus
2025

A Case of Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis With Video-Documented Psychogenic Nonepileptic Seizures (PNES)-Mimicking Episodes Initially Considered as Somatic Symptom Disorder.

Cureus
2025

Current and emerging therapies for autoimmune encephalitis.

Expert review of neurotherapeutics
2025

Safety and efficacy of long-term gantenerumab treatment in dominantly inherited Alzheimer's disease: an open-label extension of the phase 2/3 multicentre, randomised, double-blind, placebo-controlled platform DIAN-TU trial.

The Lancet. Neurology
2025

Immunotherapy-responsive parkinsonism and anosmia in anti-NMDA receptor encephalitis with seropositive NMOSD: A case report.

Journal of the neurological sciences
2025

A Complex Case of Triumphantly Treated ​​​​​​Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis.

Cureus
2025

Long-term maintenance of mycophenolate mofetil in anti-NMDA receptor encephalitis (LEARN): a multicentre, open-label, blinded-endpoint, randomised controlled trial.

Journal of neurology, neurosurgery, and psychiatry
2025

Abnormal Brain MRI in Anti-NMDA Receptor Encephalitis: Clinical and Prognostic Implications.

Neurology(R) neuroimmunology &amp; neuroinflammation
2025

Time moving 100-fold slower: time distortion as a diagnostic clue in anti-NMDA receptor encephalitis.

BMC neurology
2025

Successful sequential therapy with rituximab and telitacicept in refractory Anti-NMDA receptor encephalitis and MOG-associated demyelination: a case report and literature review.

Frontiers in immunology
2025

Safety and efficacy of long-term gantenerumab treatment in dominantly inherited Alzheimer's disease: an open label extension of the phase 2/3 multicentre, randomised, double-blind, placebo-controlled platform DIAN-TU Trial.

medRxiv : the preprint server for health sciences
2025

Successful treatment with efgartigimod as an add-on therapy for acute attack of anti-NMDA receptor encephalitis: a case report.

BMC neurology
2025

Clinical Analysis of MOG Antibody-Associated Disease Overlapped With Anti-NMDA Receptor Encephalitis: A Long-Term Retrospective Study.

The European journal of neuroscience
2025

Seizures and status epilepticus in anti-NMDA receptor encephalitis.

Journal of neurology
2025

Molecular scalpels dissect new GluN1 hot spots in anti-NMDA receptor encephalitis.

Science immunology
2025

Precision Assessment of Anti-NMDA Receptor Encephalitis: A Case Report on Integrating Clinical Course, Immunophenotyping, and Comprehensive Symptomatology in a Pediatric Patient With Adjunctive Hydrogen Therapy.

In vivo (Athens, Greece)
2025

Neuro-immunobiology and treatment assessment in a mouse model of anti-NMDAR encephalitis.

Brain : a journal of neurology
2024

Asymmetric Bilateral Medial Temporal Lobe Encephalitis Associated With Anti-N-Methyl-D-Aspartate (NMDA) Receptor Antibodies: A Case Report.

Cureus
2025

Long-Term Clinical and Biological Prognostic Factors of Anti-NMDA Receptor Encephalitis in Children.

Neurology(R) neuroimmunology &amp; neuroinflammation
2024

Catatonia As Initial Presentation in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis: A Case Report of a 19-Year-Old Patient.

Cureus
2025

Evidence-based diagnostic prediction score for pediatric NMDA receptor encephalitis.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2024

To analyze the efficacy and safety of plasma exchange in the treatment of anti-NMDA receptor encephalitis.

Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy
2025

Patient-Reported Outcome Measures in NMDA Receptor Encephalitis.

Neurology(R) neuroimmunology &amp; neuroinflammation
2025

Anti-N-methyl-D-aspartate receptor encephalitis following Human norovirus infection: A case report.

Diagnostic microbiology and infectious disease
2024

Anti-NMDA Receptor Encephalitis Presenting with Prosopagnosia.

Annals of Indian Academy of Neurology
2024

Alternative treatment in adolescent with anti-NMDA receptor encephalitis catatonia.

Neurocase
2024

ANTI -NMDA RECEPTOR ENCEPHALITIS: A POSSIBLE APPROACH TO DEVELOP A COST-EFFECTIVE TEST FOR ANTI NMDA RECEPTOR ANTIBODY DETECTION.

Journal of Ayub Medical College, Abbottabad : JAMC
2024

When the Mind Fails: A Mysterious Case of Concurrent Neurosyphilis, Herpes Simplex Virus Encephalitis, and Suspected Autoimmune Encephalitis.

Cureus
2025

Anti-NMDA receptor autoimmune encephalitis associated with ovarian teratoma: A case series and literature review.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics
2024

Oligoclonal IgG bands revealed to be constituted with NMDAR autoantibodies in a patient with anti-NMDA receptor encephalitis.

Journal of the neurological sciences
2024

Catatonia in anti-NMDA receptor encephalitis: a case series and approach to improve outcomes with electroconvulsive therapy.

BMJ neurology open
2025

Forme-Fruste of Anti-NMDA Receptor Encephalitis: Sydenham's Chorea Mimic.

Indian journal of pediatrics
2024

Encephalitis lethargica: clinical features and aetiology.

Brain communications
2024

Editors' Note: Teaching NeuroImage: Anti-NMDA Receptor Encephalitis Presenting With Cerebellitis in a Pediatric Patient.

Neurology
2024

Reader Response: Teaching NeuroImage: Anti-NMDA Receptor Encephalitis Presenting With Cerebellitis in a Pediatric Patient.

Neurology
2024

MRI findings in autoimmune encephalitis.

Revue neurologique
2023

Consensus recommendations for clinical assessment tools for the diagnosis of posterior cortical atrophy syndrome from the Atypical AD PIA of ISTAART.

Alzheimer's &amp; dementia (Amsterdam, Netherlands)
2024

From Psychosis to Recovery: A Case Report of N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis.

Cureus
2024

Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis Secondary to an Ovarian Dermoid Cyst.

Cureus
2024

Assessment of long-term psychosocial outcomes in N-methyl-D-aspartate receptor encephalitis - the SAPIENCE study protocol.

BMC neurology
2024

Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Life-Threatening Sinus Node Dysfunction: A Case Report, Literature Review, and Analysis of 23 Cases.

Cureus
2024

A short-term functional recovery comparison of therapeutic plasma exchange and immunoadsorption in severe acute neuroimmune diseases.

International immunopharmacology
2024

Isolated Psychiatric Symptoms in Children With Anti-N-Methyl-d Aspartate Receptor Encephalitis.

Pediatric neurology
2024

Eigenvector Centrality Mapping Reveals Volatility of Functional Brain Dynamics in Anti-NMDA Receptor Encephalitis.

Biological psychiatry. Cognitive neuroscience and neuroimaging
2024

Autoimmune 'secondary synaptopathies': do NMDAR antibodies cause a primary extra-synaptopathy?

Brain : a journal of neurology
2025

Correspondence: Electroconvulsive therapy in N-methyl-D-aspartate receptor encephalitis.

Journal of neuroimmunology
2024

An elderly case of paraneoplastic anti-NMDA receptor encephalitis associated with large cell neuroendocrine carcinoma of the lung.

BMC pulmonary medicine
2024

Hippocampal hub failure is linked to long-term memory impairment in anti-NMDA-receptor encephalitis: insights from structural connectome graph theoretical network analysis.

Journal of neurology
2024

Examining Associations Between Smartphone Use and Clinical Severity in Frontotemporal Dementia: Proof-of-Concept Study.

JMIR aging
2024

REM and NREM Sleep Parasomnia in Anti-NMDA Receptor Encephalitis.

Neurology(R) neuroimmunology &amp; neuroinflammation
2024

[A case report of anti-NMDA receptor encephalitis with mental disturbances manifestation].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2024

Autoimmune-associated seizure disorders.

Epileptic disorders : international epilepsy journal with videotape
2024

Aberrant Brain Networks and Relative Band Power in Patients with Acute Anti-NMDA Receptor Encephalitis: A Study of Resting-State EEG.

Journal of integrative neuroscience
2024

Memory loss and aberrant neurogenesis in mice exposed to patient anti-N-methyl-d-aspartate receptor antibodies.

Experimental neurology
2024

Case report: Isolated brainstem-cerebellar symptoms in a patient with anti-NMDA receptor encephalitis.

Frontiers in immunology
2024

Molecular neuroimaging in dominantly inherited versus sporadic early-onset Alzheimer's disease.

Brain communications
2024

H-intensity scale score to estimate CSF GluN1 antibody titers with one-time immunostaining using a commercial assay.

Frontiers in immunology
2024

Anti-NMDA encephalitis secondary to an ovarian teratoma presenting as altered mental status in a 32-year-old woman: A case report.

Case reports in women's health
2024

Racial and Ethnic Disparities in the Incidence of Anti-NMDA Receptor Encephalitis.

Neurology(R) neuroimmunology &amp; neuroinflammation
2024

A case report of anti-NMDA receptor encephalitis in a 23-year-old female with acute psychiatric symptoms.

Clinical case reports
2023

Regional cerebral blood flow in a patient with asystole episodes associated with anti-NMDA receptor encephalitis.

Neurocase
2024

Anti-NMDA Receptor Encephalitis Related to Renal Cell Carcinoma: A Case Report.

The Neurohospitalist
2024

[Late recognition of anti-NMDA receptor encephalitis: the effect of a one-track mind in multiple specialties].

Tijdschrift voor psychiatrie
2024

Very Long-Term Functional Outcomes and Dependency in Children With Anti-NMDA Receptor Encephalitis.

Neurology(R) neuroimmunology &amp; neuroinflammation
2025

Anti-NMDA Receptor Encephalitis, Human Papillomavirus, and microRNA.

Current medicinal chemistry
2024

Unveiled: A Case of N-Methyl-D-Aspartate Receptor Antibody Encephalitis With Delayed Diagnosis of Ovarian Teratoma.

Cureus
2024

Clinicoradiological and neuropathological evaluation of primary progressive aphasia.

Journal of neurology, neurosurgery, and psychiatry
2024

Teaching NeuroImage: Anti-NMDA Receptor Encephalitis Presenting With Cerebellitis in a Pediatric Patient.

Neurology
2024

Case report: Rapid recovery after intrathecal rituximab administration in refractory anti-NMDA receptor encephalitis: report of two cases.

Frontiers in immunology
2024

Paraneoplastic encephalitis.

Handbook of clinical neurology
2024

MyD88-mediated signaling is critical for the generation of seizure responses and cognitive impairment in a model of anti-N-methyl-D-aspartate receptor encephalitis.

Epilepsia
2024

[Paraneoplastic Anti-N-methyl-D-aspartate Receptor Encephalitis Associated with Anterior Mediastinal Mature Teratoma].

Kyobu geka. The Japanese journal of thoracic surgery
2024

Possible temporal relationship between SARS-CoV-2 infection and anti-NMDA receptor encephalitis: a meta-analysis.

Translational psychiatry
2024

Anti-NMDA receptor encephalitis presenting as aseptic meningitis.

Irish medical journal
2024

Mapping the global research landscape and trends of autoimmune encephalitis: A bibliometric analysis.

Heliyon
2024

Blood and CSF findings of cellular immunity in anti-NMDAR encephalitis.

International immunopharmacology
2024

Anti-NMDA-receptor encephalitis and concurrent neuroborreliosis misdiagnosed for post-COVID-19-syndrome: a case report.

Therapeutic advances in neurological disorders
2024

Examining amyloid reduction as a surrogate endpoint through latent class analysis using clinical trial data for dominantly inherited Alzheimer's disease.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2024

Ovarian teratoma-associated anti-NMDA receptor encephalitis with severe features.

BMJ case reports
2024

Significant efficacy of electroconvulsive therapy on the behavioural symptoms of anti-N-methyl-d-aspartate receptor encephalitis.

BMJ case reports
2024

Longitudinal Disability, Cognitive Impairment, and Mood Symptoms in Patients With Anti-NMDA Receptor Encephalitis.

Neurology
2024

Anti-NMDA receptor encephalitis presenting as a progression of disease in a patient with underlying congenital leukodystrophy: Case report.

The Neurohospitalist
2025

Ocular Electrophysiologic Studies in a Patient With Anti-NMDA Receptor Encephalitis and Visual Dysfunction: A Case Report.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2024

Cortical atrophy in early-stage patients with anti-NMDA receptor encephalitis: a machine-learning MRI study with various feature extraction.

Cerebral cortex (New York, N.Y. : 1991)
2024

Electroconvulsive therapy for catatonia in anti-NMDA receptor encephalitis: A case series.

Journal of neuroimmunology
2024

Examining the Features of Neuroleptic Malignant Syndrome in Anti-NMDA Receptor Encephalitis: A Case-Control Study.

Journal of the Academy of Consultation-Liaison Psychiatry
2024

The Patient Perspective in Encephalitis Research.

Neurology(R) neuroimmunology &amp; neuroinflammation
2023

Paraneoplastic Neurologic Syndromes.

Continuum (Minneapolis, Minn.)
2024

Single-cell RNA sequencing reveals diverse B cell phenotypes in patients with anti-NMDAR encephalitis.

Psychiatry and clinical neurosciences
2023

Recurrent Anti-NMDA Receptor Encephalitis After Mycoplasma Pneumonia Infection.

The American journal of psychiatry
2024

Intestinal dysbiosis exacerbates susceptibility to the anti-NMDA receptor encephalitis-like phenotype by changing blood brain barrier permeability and immune homeostasis.

Brain, behavior, and immunity
2023

Ofatumumab, a Fully Human Anti-CD20 Monoclonal Antibody, in the Treatment of Severe Refractory Anti-N-methyl-D-Aspartate Receptor Encephalitis: Two Case Reports.

Annals of Indian Academy of Neurology
2024

Teratoma pathology and genomics in anti-NMDA receptor encephalitis.

Annals of clinical and translational neurology
2023

Brazilian autoimmune encephalitis network (BrAIN): antibody profile and clinical characteristics from a multicenter study.

Frontiers in immunology
2023

Elevated phospholipids and acylcarnitines C4 and C5 in cerebrospinal fluid distinguish viral CNS infections from autoimmune neuroinflammation.

Journal of translational medicine
2023

Chimeric autoantibody receptor T cells deplete NMDA receptor-specific B cells.

Cell
2023

T1 and FLAIR signal intensities are related to tau pathology in dominantly inherited Alzheimer disease.

Human brain mapping
2023

Hero or Antagonist: A Case Series on the Tolerability of Memantine as Salvage Therapy for Catatonia from Anti-NMDA Receptor Encephalitis.

Harvard review of psychiatry
2023

Pathogenic variants in the Longitudinal Early-onset Alzheimer's Disease Study cohort.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2024

The T1-weighted/T2-weighted ratio as a biomarker of anti-NMDA receptor encephalitis.

Journal of neurology, neurosurgery, and psychiatry
2023

A Multimedia Description of Catatonia Secondary to Anti-NMDA-Receptor Encephalitis.

The primary care companion for CNS disorders
2023

Human NMDAR autoantibodies disrupt excitatory-inhibitory balance, leading to hippocampal network hypersynchrony.

Cell reports
2024

Investigation of sex differences in mutation carriers of the Dominantly Inherited Alzheimer Network.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2024

Glucocorticoid-dependent multiple sclerosis overlapping anti-NMDA receptor encephalitis: a case report and literature review update.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

VH2+ Antigen-Experienced B Cells in the Cerebrospinal Fluid Are Expanded and Enriched in Pediatric Anti-NMDA Receptor Encephalitis.

Journal of immunology (Baltimore, Md. : 1950)
2023

Temporal transformer-spatial graph convolutional network: an intelligent classification model for anti N-methyl-D-aspartate receptor encephalitis based on electroencephalogram signal.

Frontiers in neuroscience
2023

Amyloid and tau-PET in early-onset AD: Baseline data from the Longitudinal Early-onset Alzheimer's Disease Study (LEADS).

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2023

A small mature cystic ovarian teratoma associated with anti-NMDA receptor encephalitis and acute respiratory failure: A case report.

Taiwanese journal of obstetrics &amp; gynecology
2023

Anti-NMDA receptor encephalitis in a 73-year-old female with secondary progressive multiple sclerosis: A case report.

Epilepsy &amp; behavior reports
2023

Controversies in immunotherapy for anti-NMDA receptor encephalitis: a scoping review with a proposal of operational definitions.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

Association of Plasma Biomarkers of Alzheimer Disease With Cognition and Medical Comorbidities in a Biracial Cohort.

Neurology
2023

Anti-N-Methyl-D-Aspartate Receptor Encephalitis With Diffuse Demyelinating Plaques: A Case Report of an Atypical Presentation.

Cureus
2024

Age-Specific Barriers and Facilitators to Research Participation Amongst African Americans in Observational Studies of Memory and Aging.

Journal of racial and ethnic health disparities
2023

Brain rhythmic abnormalities in convalescent patients with anti-NMDA receptor encephalitis: a resting-state EEG study.

Frontiers in neurology
2023

The relevance of NMDA receptor antibody-specific index for diagnosis and prognosis in patients with anti-NMDA receptor encephalitis.

Scientific reports
2023

Clinico-Etiological Spectrum and Functional Outcomes of Children with Pre-Status Dystonicus and Status Dystonicus (SD): A Descriptive Study.

Annals of Indian Academy of Neurology
2023

Case report: Coexistence of Labbe vein thrombosis and autoimmune encephalitis with two different antibodies.

Frontiers in neurology
2023

Optimizing animal models of autoimmune encephalitis using active immunization.

Frontiers in immunology
2023

Sensitivity and specificity of alternative screening methods for systematic reviews using text mining tools.

Journal of clinical epidemiology
2023

Cerebrospinal fluid biomarkers in the Longitudinal Early-onset Alzheimer's Disease Study.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2023

Elevated blood and cerebrospinal fluid biomarkers of microglial activation and blood‒brain barrier disruption in anti-NMDA receptor encephalitis.

Journal of neuroinflammation
2023

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

Northern clinics of Istanbul
2023

Positron emission tomography and magnetic resonance imaging methods and datasets within the Dominantly Inherited Alzheimer Network (DIAN).

Nature neuroscience
2023

Effectiveness of intra-thecal methotrexate in refractory Anti-N-methyl-d-aspartate receptor encephalitis.

BMC neurology
2023

Abundant infiltration of B cells and plasma cells in brain biopsy of a male patient with severe anti-NMDA receptor encephalitis: A case report.

Medicine
2024

Adjunctive Memantine for Catatonia Due to Anti-NMDA Receptor Encephalitis.

The Journal of neuropsychiatry and clinical neurosciences
2023

Acute pediatric encephalitis: etiology, course, and outcome of a 12-year single-center immunocompetent cohort.

Journal of neurology
2023

Anti-NMDAR encephalitis in Southeast Asia - A single-centre, longitudinal study.

Clinical neurology and neurosurgery
2023

Anti-amyloid therapies for Alzheimer disease: finally, good news for patients.

Molecular neurodegeneration
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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. Complications, outcomes, and implications of a prolonged vegetative state in anti-NMDA receptor encephalitis: a retrospective international cohort study.
    The Lancet. Neurology· 2026· PMID 41864234mais citado
  2. Determinants of disease severity and 12-month functional outcome in pediatric ovarian Teratoma-Associated Anti-NMDA receptor encephalitis.
    European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society· 2026· PMID 41740198mais citado
  3. Transition Readiness and Care Needs Among Youth With Neuroimmune Disorders: A Descriptive Survey Study.
    Journal of child neurology· 2026· PMID 41493952mais citado
  4. Therapeutic perspectives in refractory anti-NMDA receptor encephalitis.
    The Lancet. Neurology· 2026· PMID 41864220mais citado
  5. Epilepsia partialis continua as an isolated manifestation of anti-NMDA receptor encephalitis: a case report.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2026· PMID 41622303mais citado
  6. When imaging fails: surgical discovery of an occult ovarian teratoma in severe refractory anti-NMDA receptor encephalitis.
    BMJ Case Rep· 2026· PMID 41991229recente
  7. Reversible Splenial Lesion Syndrome Due to Anti-NMDA Receptor Encephalitis Manifesting as Delusional Disorder: A Case Report and Literature Review.
    Brain Neurorehabil· 2026· PMID 41953803recente
  8. The role of brain MRI in autoimmune encephalitis.
    Curr Opin Neurol· 2026· PMID 41947644recente
  9. Glioblastoma mimicking anti-NMDA receptor encephalitis: a case series.
    Intern Med J· 2026· PMID 41919365recente
  10. Anti-NMDAR encephalitis impairs intrinsic hippocampal dynamics through neuronal hypercoupling, hub dominance, and aberrant ensembles.
    Mol Psychiatry· 2026· PMID 41917496recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:217253(Orphanet)
  2. MONDO:0021081(MONDO)
  3. GARD:20513(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55786136(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

Encefalite receptor NMDA
Compêndio · Raras BR

Encefalite receptor NMDA

ORPHA:217253 · MONDO:0021081
Prevalência
Unknown
Herança
Not applicable
CID-10
G13.1 · Outra atrofia sistêmica que afeta primariamente o sistema nervoso central em doenças neoplásicas
Ensaios
3 ativos
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C5700343
EuropePMC
Wikidata
Papers 10a
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