Raras
Buscar doenças, sintomas, genes...
Encefalite límbica autoimune
ORPHA:623615CID-10 · G04.8CID-11 · 8E4A.0DOENÇA RARA

Uma inflamação rara no cérebro, causada pelo próprio sistema de defesa do corpo (autoimune), que afeta a parte interna dos lobos temporais. A condição se manifesta com um início subagudo (ou seja, os sintomas aparecem e pioram rapidamente em menos de três meses), incluindo problemas de memória recente, convulsões ou sintomas psiquiátricos, como mudanças no comportamento, ansiedade, depressão e psicose. Para o diagnóstico, também são considerados: alterações nos dois lados da parte interna dos lobos temporais, visíveis em exames de ressonância magnética do cérebro; aumento de células de defesa no líquor (o líquido que envolve o cérebro e a medula espinhal) e/ou padrões de atividade elétrica anormais nos lobos temporais, observados no eletroencefalograma (EEG); além da exclusão de outras possíveis causas para os sintomas. Também podem ser encontrados, no sangue ou no líquor, anticorpos (substâncias de defesa do corpo) que atacam as células nervosas, podendo ou não estar relacionados a algum tipo de câncer.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Uma inflamação rara no cérebro, causada pelo próprio sistema de defesa do corpo (autoimune), que afeta a parte interna dos lobos temporais. A condição se manifesta com um início subagudo (ou seja, os sintomas aparecem e pioram rapidamente em menos de três meses), incluindo problemas de memória recente, convulsões ou sintomas psiquiátricos, como mudanças no comportamento, ansiedade, depressão e psicose. Para o diagnóstico, também são considerados: alterações nos dois lados da parte interna dos lobos temporais, visíveis em exames de ressonância magnética do cérebro; aumento de células de defesa no líquor (o líquido que envolve o cérebro e a medula espinhal) e/ou padrões de atividade elétrica anormais nos lobos temporais, observados no eletroencefalograma (EEG); além da exclusão de outras possíveis causas para os sintomas. Também podem ser encontrados, no sangue ou no líquor, anticorpos (substâncias de defesa do corpo) que atacam as células nervosas, podendo ou não estar relacionados a algum tipo de câncer.

Publicações científicas
247 artigos
Último publicado: 2026 Mar

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
1.7
Europe
Início
All ages
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G04.8
🇧🇷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

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

🧠
Neurológico
7 sintomas
🛡️
Imunológico
2 sintomas

+ 15 sintomas em outras categorias

Características mais comuns

55%prev.
Deterioração mental
Frequente (79-30%)
55%prev.
EEG com atividade lenta focal
Frequente (79-30%)
55%prev.
Anticorpo anti-LGI1
Frequente (79-30%)
55%prev.
Irritabilidade
Frequente (79-30%)
55%prev.
Aumento da concentração de proteína no LCR
Frequente (79-30%)
55%prev.
Psicose
Frequente (79-30%)
24sintomas
Frequente (19)
Ocasional (3)
Muito raro (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.

Deterioração mentalMental deterioration
Frequente (79-30%)55%
EEG com atividade lenta focalEEG with focal slow activity
Frequente (79-30%)55%
Anticorpo anti-LGI1Anti-LGI1 antibody
Frequente (79-30%)55%
IrritabilidadeIrritability
Frequente (79-30%)55%
Aumento da concentração de proteína no LCRIncreased CSF protein concentration
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico247PubMed
Últimos 10 anos200publicações
Pico202229 papers
Linha do tempo
2026Hoje · 2026🧪 2011Primeiro ensaio clínico📈 2022Ano 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-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 3 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Encefalite límbica autoimune

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

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Ensaios clínicos abertos e novidades científicas recentes

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Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
200 papers (10 anos)
#1

Sjögren Syndrome Associated Limbic Encephalitis and Its Longitudinal Brain FDG PET Manifestations.

Clinical nuclear medicine2026 Apr 01

A 62-year-old woman with Sjögren syndrome, rheumatoid arthritis, and autoimmune hepatitis presented with subacute confusion and generalized seizures. MRI revealed bilateral mesial temporal T2/FLAIR hyperintensity. Serology revealed anti-SSA and anti-SSB antibodies elevated at 5 times above the upper limit of normal. FDG-PET demonstrated marked relative mesial temporal hypermetabolism with widespread hypometabolism in the rest of the brain; z-score mapping confirmed focal mesial temporal hyperactivity. They received immunotherapy and antiseizure therapy. After 1 year, cognition improved, though memory deficits persisted. Follow-up FDG-PET showed a transition from mesial temporal hypermetabolism to relative hypometabolism, with recovery of cortical metabolism, highlighting FDG-PET's role in diagnosing and monitoring autoimmune limbic encephalitis in Sjögren syndrome.

#2

Lentiform fork sign mimicking uremic encephalopathy in an atypical case of autoimmune limbic encephalitis.

Radiology case reports2026 Apr

The lentiform fork sign (LFS) on MRI is often associated with uremic encephalopathy but is not pathognomonic. We present a case of autoimmune limbic encephalitis initially suspected as uremic encephalopathy despite normal renal function. A 58-year-old woman presented with seizures and altered mental status. MRI showed bilateral basal ganglia hyperintensities with the classic LFS. Normal renal parameters prompted cerebrospinal fluid analysis, which revealed anti-NMDA receptor antibodies, confirming autoimmune encephalitis. The patient improved markedly after immunotherapy. This case emphasizes the need to consider autoimmune causes of LFS and the importance of early diagnosis and treatment in preventing adverse outcomes.

#3

T1w/T2w Ratio Identifies the Basolateral Amygdala as a Preferential Target in Autoimmune Limbic Encephalitis.

Journal of neuroimaging : official journal of the American Society of Neuroimaging2026

The amygdala plays a key role in the pathophysiology of autoimmune limbic encephalitis (ALE), contributing to epileptic seizures and neuropsychiatric symptoms. While no study has examined microstructural changes in individual amygdala nuclei in ALE, we used the T1-weighted/T2-weighted (T1w/T2w) ratio to explore amygdalar pathology and its associations with clinical manifestations, including epilepsy and neuropsychiatric symptoms. This single-center study examined 57 patients diagnosed with ALE and 16 healthy controls (HC). Patients underwent a comprehensive assessment that included clinical, electroencephalogram (EEG), magnetic resonance imaging (MRI), and neuropsychological assessments. Patients were stratified by epileptic focus based on long-term EEG. T1w/T2w ratio and volumetric measures of the amygdala and its nuclei were analyzed and correlated with epileptic focus and neuropsychiatric outcomes. EEG revealed 26 left temporal, 26 bitemporal, and five right temporal epileptic foci. The T1w/T2w ratio in the left amygdala was markedly reduced in patients with left temporal (p = 0.013) and bitemporal (p = 0.018) epileptic foci compared to HC. This reduction was most pronounced in the left basolateral complex (p = 0.011). Whereas amygdalar volumes were similar between patients and HC, exploratory analyses showed an increased volume of the left lateral nucleus in left temporal ALE (p = 0.036). Furthermore, we found no correlations between MRI measures and neuropsychiatric scores. Our findings indicate that the basolateral complex of the amygdala is preferentially affected in ALE, suggesting a region-specific vulnerability to autoimmune-mediated inflammation. T1w/T2w ratio alterations reflect the epileptogenic focus and may serve as a clinically accessible, noninvasive biomarker for early diagnosis and treatment monitoring in ALE.

#4

IDH-wildtype diffuse glioma initially presenting as limbic encephalitis: A case report.

Radiology case reports2026 Jan

Diffuse gliomas represent a heterogeneous group of infiltrative central nervous system tumors that may resemble inflammatory disorders in early stages. We report a rare case of an IDH-wildtype diffuse glioma initially mimicking autoimmune limbic encephalitis. A 49-year-old male presented with subacute neuropsychiatric symptoms, including confusion, hallucinations, and memory disturbances. MRI revealed widespread FLAIR hyperintensities involving the cingulate gyri, insula, basal ganglia, thalamus, corpus callosum, and frontal operculum, without contrast enhancement or perfusion abnormalities. MR spectroscopy showed increased Cho/Cr and Cho/NAA ratios, decreased NAA/Cr ratio, and elevated myo-inositol peak, suggesting a neoplastic process. The patient received corticosteroids, intravenous immunoglobulin, and plasmapheresis for presumed autoimmune encephalitis, but no clinical or radiological improvement was observed. Subsequent biopsy confirmed IDH-wildtype diffuse glioma. This case highlights the diagnostic overlap between encephalitis and gliomas, especially when radiological findings lack mass effect or enhancement. Extralimbic involvement, abnormal metabolic profiles on spectroscopy, and failure to respond to immunotherapy should raise suspicion for neoplasia. Awareness of these red flags is essential, as misclassification may delay biopsy and oncologic treatment. Early histopathological confirmation remains the only definitive step to secure accurate diagnosis and initiate timely therapy.

#5

Reprogramming Patient-Derived urine cells into iPSCs for Anti-GAD65 autoimmune encephalitis research.

Stem cell research2025 Sep

Urine cells from a patient with anti-GAD65autoantibody-associated autoimmune limbic encephalitis (ALE) were reprogrammed intoiPSCline IUFi020-A. Pluripotency was confirmed throughhiPSCore analysis while G-banding and CNVanalysis demonstrated that IUFi020-A exhibits characteristic features of a bona fide iPSC line with no genetic changes compared to the donor urine cells. STRanalysis further confirmed that IUFi020-A was derived from the parental urine cells. Additional characterization verified the absence of plasmidintegration and mycoplasma contamination.IUFi020-Aline provides a non-invasive alternative to fibroblast-based reprogramming and serves as a valuable model for investigating the pathogenic mechanisms of anti-GAD65-associated ALE.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC105 artigos no totalmostrando 198

2026

Sjögren Syndrome Associated Limbic Encephalitis and Its Longitudinal Brain FDG PET Manifestations.

Clinical nuclear medicine
2026

Lentiform fork sign mimicking uremic encephalopathy in an atypical case of autoimmune limbic encephalitis.

Radiology case reports
2026

T1w/T2w Ratio Identifies the Basolateral Amygdala as a Preferential Target in Autoimmune Limbic Encephalitis.

Journal of neuroimaging : official journal of the American Society of Neuroimaging
2025

Electroceuticals for Motherhood: Utility of responsive neurostimulation and Proactive antiseizure medication management in two pregnancies.

Epilepsy &amp; behavior reports
2026

IDH-wildtype diffuse glioma initially presenting as limbic encephalitis: A case report.

Radiology case reports
2025

Brain MRI Revealing the Probable Pathophysiology of Neuropsychiatric Lupus: A Case Report.

Cureus
2025

Ictal cold shiver caused by autoimmune limbic encephalitis: A case report and literature review.

Epilepsy &amp; behavior reports
2025

Reprogramming Patient-Derived urine cells into iPSCs for Anti-GAD65 autoimmune encephalitis research.

Stem cell research
2025

Where There's Smoke, There's Fire: Paraneoplastic Autoimmune Limbic Encephalitis.

Cureus
2025

Nine-Year Follow-Up of GAD65 Antibody Limbic Encephalitis With Clinical Remission Despite Persistent Imaging and Serological Abnormalities.

Cureus
2025

[Paraneoplastic limbic encephalitis - an important differential diagnosis in cases of unclear encephalopathy].

Praxis
2025

Limbic Encephalitis: An Unusual Presentation on 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (18F-FDG PET/CT).

Cureus
2025

Clinical Characteristics and Seizure Outcomes in Antibody-Positive Autoimmune Limbic Encephalitis.

Journal of inflammation research
2025

Lymphocyte signatures correspond to clinical phenotypes in autoimmune limbic encephalitis.

Brain communications
2025

Multimodal prognostication of autoimmune encephalitis: an Australian autoimmune encephalitis consortium study.

Journal of neurology
2025

Exploring autoantigens in autoimmune limbic encephalitis using phage immunoprecipitation sequencing.

Journal of neurology
2025

Retrograde Amnesia in LGI1 and CASPR2 Limbic Encephalitis: Two Case Reports and a Systematic Literature Review.

European journal of neurology
2025

Severe coxsackie virus B5 encephalitis mimics autoimmune limbic encephalitis in a young woman under long-term B-cell depletion with ocrelizumab: A case report.

Multiple sclerosis (Houndmills, Basingstoke, England)
2025

Clarithromycin-induced myoclonic status epilepticus.

Practical neurology
2025

Cognitive Deficits in Anti-LGI1 Encephalitis Are Linked to Immunotherapy-Resistant White Matter Network Changes.

Neurology(R) neuroimmunology &amp; neuroinflammation
2024

Anti-Gamma Aminobutyric Acid B (GABA B) Antibody Receptor-Associated Autoimmune Encephalitis in Pregnancy: A Rare Condition.

Cureus
2025

Anti-Tr/DNER Antibody-associated Paraneoplastic Neurological Syndrome Presenting Limbic Encephalitis with Anaplastic Large Cell Lymphoma.

Internal medicine (Tokyo, Japan)
2024

Anti-LGI1 Autoimmune Limbic Encephalitis: An Easy-To-Miss Diagnosis.

European journal of case reports in internal medicine
2025

Markedly Elevated IgG Index: A Key to Differentiating Neurosyphilis from Autoimmune Limbic Encephalitis.

Internal medicine (Tokyo, Japan)
2024

Immune checkpoint inhibitor-induced autoimmune limbic encephalitis with positivity for anti-Hu antibodies in a patient with small-cell lung cancer: A case report and literature review.

Respirology case reports
2024

Distinguishing seizures in autoimmune limbic encephalitis from mesial temporal lobe epilepsy with hippocampal sclerosis: Clues of a temporal plus network.

Journal of the neurological sciences
2024

Flow cytometry identifies changes in peripheral and intrathecal lymphocyte patterns in CNS autoimmune disorders and primary CNS malignancies.

Journal of neuroinflammation
2025

CSF and blood signatures support classification of limbic encephalitis subtypes.

Brain, behavior, and immunity
2025

Kikuchi-Fujimoto Disease Presenting as Autoimmune Limbic Encephalitis: A Case Report with Review of Literature.

Annals of Indian Academy of Neurology
2024

FDG-PET and ASL MRI identify largely overlapping hypermetabolic and hyperperfusion changes in limbic autoimmune encephalitis.

The quarterly journal of nuclear medicine and molecular imaging : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR), [and] Section of the Society of...
2024

Human stem cell-derived neurons and astrocytes to detect novel auto-reactive IgG response in immune-mediated neurological diseases.

Frontiers in immunology
2024

A Case of Anti-Leucine-Rich Glioma-Inactivated Protein 1 (Anti-LGI1) Limbic Encephalitis With Normal Imaging.

Cureus
2024

Anti-LGI1 Autoimmune Encephalitis in a Patient with Rheumatoid Arthritis and MGUS.

European journal of case reports in internal medicine
2024

[(Auto)immunity in focal epilepsy: mechanisms of (auto‑)immune-inflammatory epileptogenic neurodegeneration].

Der Nervenarzt
2024

Distinctive sleep complaints and polysomnographic findings in antibody subgroups of autoimmune limbic encephalitis.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2024

Two Cases of Pediatric Leucine-Rich Glioma-Inactivated Protein-1 Encephalitis: Clinical Course, Challenges, and Implications.

Pediatric neurology
2024

Differentiation between viral and autoimmune limbic encephalitis: a prospective cohort study with development and validation of a diagnostic model.

Journal of neurology
2025

Autoimmune-associated epilepsy - a challenging concept.

Seizure
2024

The role of the human hippocampus in decision-making under uncertainty.

Nature human behaviour
2024

Study on clinical features and factors related to long-term outcomes of antibody-negative autoimmune encephalitis.

Annals of clinical and translational neurology
2024

Remote digital cognitive assessment reveals cognitive deficits related to hippocampal atrophy in autoimmune limbic encephalitis: a cross-sectional validation study.

EClinicalMedicine
2024

Antibiomania: clarithromycin-induced neurotoxicity mimicking autoimmune limbic encephalitis.

Practical neurology
2023

Case Report: Limbic encephalitis following treatment with durvalumab for small-cell lung cancer.

Frontiers in immunology
2023

Paraneoplastic Autoimmune Limbic Encephalitis Associated with an Atypical Carcinoid Tumor of the Lung: A Case Report.

Case reports in neurology
2023

Antibody-Negative Autoimmune Encephalitis: A Single-Center Retrospective Analysis.

Neurology(R) neuroimmunology &amp; neuroinflammation
2023

Anti-Ma2 encephalitis in a phenotypic female with XY gonadal dysgenesis: A case report.

Epilepsy &amp; behavior reports
2023

Autoimmune Limbic Encephalitis in Patients with Hematologic Malignancies after Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplant Cyclophosphamide.

Cells
2023

Mimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria.

Neurology(R) neuroimmunology &amp; neuroinflammation
2024

Autoimmune encephalitis related to LGI1 antibodies with negative MRI study: Description of two cases.

Medicina clinica
2022

A case of leucine-rich glioma-inactivated 1 antibody encephalitis with schizophrenia-like symptoms as an initial clinical manifestation.

Encephalitis (Seoul, Korea)
2023

Autoimmune limbic encephalitis combined with leptomeningeal metastases of non-small cell lung cancer: treatment response to osimertinib, immunoglobulin, rituximab, and tocilizumab.

Encephalitis (Seoul, Korea)
2023

Limbic Encephalitis With Dual Positivity.

Cureus
2023

Rare Association of Autoimmune Limbic Encephalitis and Stiff Person Syndrome.

Rhode Island medical journal (2013)
2023

Translational imaging of TSPO reveals pronounced innate inflammation in human and murine CD8 T cell-mediated limbic encephalitis.

Science advances
2023

Double Positive Anti-CASPR2 and LGI1 Antibodies Associated with Two Episodes of Clinically Distinct Disorders.

The Israel Medical Association journal : IMAJ
2023

18 F-FDG PET and MRI Evaluation of Paraneoplastic Limbic Encephalitis.

Clinical nuclear medicine
2023

Autoimmune limbic encephalitis mimicking inferior myocardial infarction on ECG.

BMJ case reports
2023

Comparative Effects of Domain-Specific Human Monoclonal Antibodies Against LGI1 on Neuronal Excitability.

Neurology(R) neuroimmunology &amp; neuroinflammation
2023

A feline model of spontaneously occurring autoimmune limbic encephalitis.

Veterinary journal (London, England : 1997)
2022

Neurosyphilis: The Great Imitator.

Cureus
2023

Review and meta-analysis of neuropsychological findings in autoimmune limbic encephalitis with autoantibodies against LGI1, CASPR2, and GAD65 and their response to immunotherapy.

Clinical neurology and neurosurgery
2022

A Case of Anti-Leucine-Rich Glioma-Inactivated Protein 1 (Anti-LGI1) Encephalitis With an Unusual Frontomesial Motor Cortex T2 MRI Hyperintensity.

Cureus
2023

Early-Stage Contactin-Associated Protein-like 2 Limbic Encephalitis: Clues for Diagnosis.

Neurology(R) neuroimmunology &amp; neuroinflammation
2022

Cytokines/chemokines and soluble immune checkpoint molecules in anti-GABAB receptor encephalitis.

Multiple sclerosis and related disorders
2023

Autoimmune seronegative limbic encephalitis following ChAdOx1-S/nCoV-19 vaccination in a patient with ankylosing spondylitis.

Journal of medical virology
2022

Post-COVID-19 Vaccine Limbic Encephalitis: A Case Report.

Cureus
2022

Increased T- and B-cells associated with the phenotype of autoimmune limbic encephalitis with mainly memory dysfunction.

Journal of translational autoimmunity
2023

18F-FDG PET for the diagnosis and assessment of treatment response of autoimmune limbic encephalitis.

Revista espanola de medicina nuclear e imagen molecular
2022

Seronegative Autoimmune Limbic Encephalitis: A Case Report.

The Journal of the Association of Physicians of India
2022

Autoimmune neuromyotonia.

Current opinion in neurology
2022

Neurosyphilis presenting as autoimmune limbic encephalitis: A case report and literature review.

Medicine
2022

Mature Cystic Teratoma Combined With Autoimmune Limbic Encephalitis Mimicking Functional Ovarian Cyst.

Cureus
2022

A reverse genetics and genomics approach to gene paralog function and disease: Myokymia and the juxtaparanode.

American journal of human genetics
2022

A rare case of antileucine-rich glioma-inactivated 1 encephalitis in a 14-year-old girl.

The Turkish journal of pediatrics
2023

Distinct movement disorders in contactin-associated-protein-like-2 antibody-associated autoimmune encephalitis.

Brain : a journal of neurology
2022

Nonparaneoplastic Anti-GAD Limbic Encephalitis: Seizure Outcome and Long-term Neuropsychological Follow-up After Immunotherapy.

Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology
2022

Case report of anti-NMDA receptor encephalitis in a 24-year-old female: an uncommon presentation.

The Egyptian journal of neurology, psychiatry and neurosurgery
2022

Autoimmune Voltage-Gated Potassium Channel Limbic Encephalitis With Auditory and Visual Hallucinations.

Cureus
2022

Construction of an Assisted Model Based on Natural Language Processing for Automatic Early Diagnosis of Autoimmune Encephalitis.

Neurology and therapy
2022

Whole-brain metabolic pattern analysis in patients with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis.

European journal of neurology
2022

Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.

Brain : a journal of neurology
2022

HLA-DRB1*03:01 is associated with female sex and younger age of anti-LGI1 encephalitis.

European journal of neurology
2022

Pilomotor seizures in autoimmune limbic encephalitis: description of two GAD65 antibodies- related cases and literature review.

Seizure
2022

Adenylate kinase 5 (AK5) autoimmune encephalitis: Clinical presentations and outcomes in three new patients.

Journal of neuroimmunology
2022

Seronegative limbic encephalitis in association with Sjögren's syndrome: a rare case report.

The Pan African medical journal
2022

Bilaterality of temporal EEG findings in limbic encephalitis compared to other mesiotemporal epilepsies - A retrospective cohort study.

Seizure
2022

Neuropsychological implication in possible antibody-negative limbic encephalitis: a clinical case report.

The Journal of international medical research
2022

Neuropsychological Performance in Autoimmune Limbic Encephalitis: Evidence from an Immunotherapy-Naïve Cohort.

Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists
2022

Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study.

Annals of clinical and translational neurology
2021

Immunotherapy for Refractory Autoimmune Encephalitis.

Frontiers in immunology
2021

Recurrent Anti-AMPA Receptor Limbic Encephalitis: A Case Report and Literature Review.

Frontiers in neurology
2021

Impact of T cells on neurodegeneration in anti-GAD65 limbic encephalitis.

Annals of clinical and translational neurology
2021

Atypical Association of Autoimmune Limbic Encephalitis with Anti-NMDA Receptor Antibodies in a Young Male Patient: Clinical, Imaging, and Neuropsychological Characteristics.

Case reports in neurology
2021

Executive deficits in neuropsychological testing differentiate between autoimmune temporal lobe epilepsy caused by limbic encephalitis and temporal lobe epilepsies with non-autoimmune etiologies.

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

Role of LGI1 protein in synaptic transmission: From physiology to pathology.

Neurobiology of disease
2021

Comprehensive B-Cell Immune Repertoire Analysis of Anti-NMDAR Encephalitis and Anti-LGI1 Encephalitis.

Frontiers in immunology
2021

Autoimmune Limbic Encephalitis: A Review of Clinicoradiological Features and the Challenges of Diagnosis.

Cureus
2021

I can't count, but I can beat you playing cards: a case report on autoimmune encephalitis.

BMC neurology
2021

Anti-leucine-rich-glioma-inactivated 1 Limbic Encephalitis: An Underrecognised Disease.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
2021

Status epilepticus suspected autoimmune: Neuronal surface antibodies and main clinical features.

Epilepsia
2022

Feline temporal lobe epilepsy: seven cases of hippocampal and piriform lobe necrosis in England and literature review.

Journal of feline medicine and surgery
2021

Rare paraneoplastic syndrome of prostatic cancer: limbic encephalitis: a case report.

Journal of medical case reports
2022

Diagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis.

European journal of neurology
2021

Determinants of cognition in autoimmune limbic encephalitis-A retrospective cohort study.

Hippocampus
2021

Serum antiganglioside antibodies in patients with autoimmune limbic encephalitis.

Turkish journal of medical sciences
2021

Trans-synaptic LGI1-ADAM22-MAGUK in AMPA and NMDA receptor regulation.

Neuropharmacology
2021

Case Report: Autoimmune Encephalitis Associated With Anti-glutamic Acid Decarboxylase Antibodies: A Pediatric Case Series.

Frontiers in neurology
2021

ASL MRI and 18F-FDG-PET in autoimmune limbic encephalitis: clues from two paradigmatic cases.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2021

Takotsubo syndrome associated with autoimmune limbic encephalitis: a case report.

BMC cardiovascular disorders
2021

Orbitofrontal syndrome and "goosebumps"-A new manifestation of autoimmune epilepsy in anti-LGI1 encephalitis.

Seizure
2021

Autoimmune limbic encephalitis related to SARS-CoV-2 infection: Case-report and review of the literature.

Brain, behavior, &amp; immunity - health
2021

Initial cerebrospinal fluid-restricted oligoclonal bands associate with anti-N-methyl-D-aspartate receptor encephalitis severity: a pilot study.

Encephalitis (Seoul, Korea)
2020

Efficacy of rituximab on seizure control and cognitive symptoms in leucine-rich, glioma-inactivated 1 (LGI1) limbic encephalitis: a high-density electroencephalography case study.

Epileptic disorders : international epilepsy journal with videotape
2022

A scoping review on paraneoplastic autoimmune limbic encephalitis (PALE) psychiatric manifestations.

CNS spectrums
2020

Anti-N-methyl-d-aspartate Receptor Encephalitis Related Sinus Node Dysfunction and the Lock-Step Phenomenon.

American journal of medical case reports
2021

LGI1 encephalitis with squamous lung-cell carcinoma: Resolution after tumor resection.

Neurology(R) neuroimmunology &amp; neuroinflammation
2020

A Case of Wernicke-Korsakoff Syndrome Initially Diagnosed as Autoimmune Limbic Encephalitis: Differential Diagnosis of Delirium and Short-Term Memory Deficits.

The primary care companion for CNS disorders
2020

Psychiatric symptoms in anti glutamic acid decarboxylase associated limbic encephalitis in adults: a systematic review.

Neuroscience and biobehavioral reviews
2020

Double filtration plasmapheresis - 10-year pediatric experience as an alternative to plasma exchange.

Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
2021

Metabolic Imaging Patterns on 18F-FDG PET in Acute and Subacute LGI1 Autoimmune Limbic Encephalitis.

Clinical nuclear medicine
2021

Specific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis.

Journal of neurology
2020

Neurobehavioral Disorders: The Corticolimbic System in Health and Disease.

The Veterinary clinics of North America. Small animal practice
2020

Does hippocampal atrophy explain anterograde and retrograde amnesia following autoimmune limbic encephalitis?

Hippocampus
2020

Limbic encephalitis in a neuroscientist: CASPR 2 antibody-associated disease after antigen exposure.

Journal of neuroimmunology
2021

Clinical features of nine cases of leucine-rich glioma inactivated 1 protein antibody-associated encephalitis.

Acta neurologica Belgica
2020

Glioblastoma as an autoimmune limbic encephalitis mimic: A case and review of the literature.

Journal of neuroimmunology
2020

Efficacy of Tocilizumab in Limbic Encephalitis with Anti-CASPR2 Antibodies.

Case reports in neurological medicine
2020

Distinct Effects of Stereotactically Injected Human Cerebrospinal Fluid Containing Glutamic Acid Decarboxylase Antibodies into the Hippocampus of Rats on the Development of Spontaneous Epileptic Activity.

Brain sciences
2020

Very high-dose methylprednisolone for treatment of nivolumab-induced limbic encephalitis: A case report.

Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners
2019

Dopaminergic Hyperactivity in Neurological Patients with Delirium.

Archives of medical research
2020

Pathologic tearfulness after limbic encephalitis: A novel disorder and its neural basis.

Neurology
2020

Multimodality Imaging of Anti-Contactin-Associated Protein-Like 2 Antibody-Induced Nonparaneoplastic Limbic Encephalitis: Complementary Role of Fluorine-18 Fluorodeoxyglucose Positron Emission Tomography and Magnetic Resonance Imaging.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2019

LGI-1 antibody encephalitis in a seven-year-old girl.

Epileptic disorders : international epilepsy journal with videotape
2020

Low CSF CD4/CD8+ T-cell proportions are associated with blood-CSF barrier dysfunction in limbic encephalitis.

Epilepsy &amp; behavior : E&amp;B
2019

Autoimmune Limbic Encephalitis in a Patient with Acute Encephalopathy and Hyponatremia.

Case reports in medicine
2020

Diagnosing autoimmune encephalitis in a real-world single-centre setting.

Journal of neurology
2019

Predictive value of high titer of GAD65 antibodies in a case of limbic encephalitis.

Journal of neuroimmunology
2019

Characterizing memory loss in patients with autoimmune limbic encephalitis hippocampal lesions.

Hippocampus
2019

Syncope and Twitching at the Emergency Department.

The American journal of case reports
2019

Clinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series.

Internal medicine (Tokyo, Japan)
2019

Network-wide abnormalities explain memory variability in hippocampal amnesia.

eLife
2019

Impact of anti-CASPR2 autoantibodies from patients with autoimmune encephalitis on CASPR2/TAG-1 interaction and Kv1 expression.

Journal of autoimmunity
2019

Diagnosing autoimmune limbic encephalitis.

CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne
2019

Hippocampal network abnormalities explain amnesia after VGKCC-Ab related autoimmune limbic encephalitis.

Journal of neurology, neurosurgery, and psychiatry
2019

Conventional brain MRI features distinguishing limbic encephalitis from mesial temporal glioma.

Neuroradiology
2019

Hashimoto's Encephalopathy Presenting with Smoldering Limbic Encephalitis.

Internal medicine (Tokyo, Japan)
2019

Deleted in colorectal cancer (netrin-1 receptor) antibodies and limbic encephalitis in a cat with hippocampal necrosis.

Journal of veterinary internal medicine
2019

Antibody Prevalence in Epilepsy and Encephalopathy score: Increased specificity and applicability.

Epilepsia
2019

Development of the clinical assessment scale in autoimmune encephalitis.

Annals of neurology
2018

Gastaut-Geschwind Syndrome, Faciobrachial Dystonic Seizure, and Autoimmune Limbic Encephalitis.

Case reports in psychiatry
2018

Autoimmune Epilepsy and/or Limbic Encephalitis Can Lead to Changes in Sleep Spindles.

Noro psikiyatri arsivi
2019

Limbic encephalitis: Experience of a moroccan center.

Brain and behavior
2018

LGI1 antibodies alter Kv1.1 and AMPA receptors changing synaptic excitability, plasticity and memory.

Brain : a journal of neurology
2018

Use of intravenous immunoglobulin for the treatment of autoimmune encephalitis: audit of the NHS experience.

JRSM open
2018

Predictors of neural-specific autoantibodies and immunotherapy response in patients with cognitive dysfunction.

Journal of neuroimmunology
2018

Toxic Epidermal Necrolysis in a Patient with Autoimmune Limbic Encephalitis with Anti-Glutamate Receptor Antibodies.

Case reports in neurology
2018

Autoimmune limbic encephalitis in association with acute stroke.

Neurology. Clinical practice
2018

Distinction between anti-VGKC-complex seropositive patients with and without anti-LGI1/CASPR2 antibodies.

Journal of the neurological sciences
2018

Limbic encephalitis associated with systemic sclerosis.

Multiple sclerosis and related disorders
2018

Practical issues in measuring autoantibodies to neuronal cell-surface antigens in autoimmune neurological disorders: 190 cases.

Journal of the neurological sciences
2018

Striatal hypermetabolism in a case of IgG4-related disease.

Nuclear medicine review. Central &amp; Eastern Europe
2018

Simultaneous 18F- FDG PET/MRI in Autoimmune Limbic Encephalitis.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2017

Claustrum hyperintensities: A potential clue to autoimmune epilepsy.

Epilepsia open
2018

Possible anti-VGKC autoimmune limbic encephalitis associated with SIADH.

BMJ case reports
2017

Gamma-aminobutyric acid-B limbic encephalitis and asystolic cardiac arrest: a case report.

Journal of medical case reports
2017

Molecular cloning and characterization of the family of feline leucine-rich glioma-inactivated (LGI) genes, and mutational analysis in familial spontaneous epileptic cats.

BMC veterinary research
2018

Anti-Hu-Associated Autoimmune Limbic Encephalitis in a Patient with PD-1 Inhibitor-Responsive Myxoid Chondrosarcoma.

The oncologist
2017

Application of the 2016 diagnostic approach for autoimmune encephalitis from Lancet Neurology to Chinese patients.

BMC neurology
2017

Selective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies.

Frontiers in immunology
2017

A rare case of autoimmune limbic encephalitis: an uncharted territory!

Neurosciences (Riyadh, Saudi Arabia)
2017

[Autoimmune and Epilepsy].

Brain and nerve = Shinkei kenkyu no shinpo
2017

Dissociation findings between short-term and long-term memory in autoimmune limbic encephalitis.

Journal of the neurological sciences
2017

Comprehensive and Methodical: Diagnostic and Management Approaches to Rapidly Progressive Dementia.

Current treatment options in neurology
2017

Peripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review.

Brain and behavior
2017

Seronegative Paraneoplastic Limbic Encephalitis Associated with Thymoma.

Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology
2017

Autoimmune Limbic Encephalitis Associated with Type 1 Diabetes Mellitus.

Journal of clinical research in pediatric endocrinology
2017

AUTOIMMUNE LIMBIC ENCEPHALITIS (CASE REPORTS).

Georgian medical news
2017

GAD antibody-associated limbic encephalitis in a young woman with APECED.

Endocrinology, diabetes &amp; metabolism case reports
2017

Predictive models in the diagnosis and treatment of autoimmune epilepsy.

Epilepsia
2017

Confusion, Faciobrachial Dystonic Seizures, and Critical Hyponatremia in a Patient with Voltage-Gated Potassium Channel Encephalitis.

Korean journal of family medicine
2017

Autoimmune limbic encephalitis with anti-contactin-associated protein-like 2 antibody secondary to pembrolizumab therapy.

Journal of neuroimmunology
2017

Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies: A clinical subtype of Hashimoto encephalopathy.

Medicine
2016

[Review of the psychiatric aspects of anti-NMDA (N-methyl-D-aspartic acid) receptor encephalitis, case report, and our plans for a future study].

Neuropsychopharmacologia Hungarica : a Magyar Pszichofarmakologiai Egyesulet lapja = official journal of the Hungarian Association of Psychopharmacology
2017

N-Methyl-D-aspartate receptor antibody could be a cause of catatonic symptoms in psychiatric patients: case reports and methods for detection.

Neuropsychiatric disease and treatment
2017

[A case of neurosyphilis presenting with limbic encephalitis].

Rinsho shinkeigaku = Clinical neurology
2016

Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up.

Neurology
2016

Neuropsychological and FDG-PET profiles in VGKC autoimmune limbic encephalitis.

Brain and cognition
2016

Correlations of Clusters of Non-Convulsive Seizure and Magnetic Resonance Imaging in a Case With GAD65-Positive Autoimmune Limbic Encephalitis.

Journal of clinical medicine research
2016

Autoimmune Limbic Encephalitis and Syndrome of Inappropriate Antidiuretic Hormone Secretion Associated with Lamotrigine-induced Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome.

Internal medicine (Tokyo, Japan)
2016

[Autoimmune Associated Encephalitis and Dementia].

Brain and nerve = Shinkei kenkyu no shinpo
2016

Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort.

Neurology
2016

Limbic Encephalitis Associated With GAD65 Antibodies: Brief Review of the Relevant literature.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2016

A unique combination of autoimmune limbic encephalitis, type 1 diabetes, and Stiff person syndrome associated with GAD-65 antibody.

Annals of Indian Academy of Neurology
2017

Suspected antibody negative autoimmune limbic encephalitis: outcome of immunotherapy.

Acta neurologica Scandinavica
2016

Clinical characterization of autoimmune LGI1 antibody limbic encephalitis.

Epilepsy &amp; behavior : E&amp;B
2016

Cognitive Impairments Preceding and Outlasting Autoimmune Limbic Encephalitis.

Case reports in neurological medicine
2016

Autoimmune limbic encephalitis due to VGKC-Ab. Thanks for the memory. Leo Robin (Title of song, 1937).

Practical neurology
2015

[Faciobrachial dystonic seizures. Semiologic diagnosis in limbic encephalitis].

Medicina

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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. Sj&#xf6;gren Syndrome Associated Limbic Encephalitis and Its Longitudinal Brain FDG PET Manifestations.
    Clinical nuclear medicine· 2026· PMID 41771040mais citado
  2. Lentiform fork sign mimicking uremic encephalopathy in an atypical case of autoimmune limbic encephalitis.
    Radiology case reports· 2026· PMID 41694700mais citado
  3. T1w/T2w Ratio Identifies the Basolateral Amygdala as a Preferential Target in Autoimmune Limbic Encephalitis.
    Journal of neuroimaging : official journal of the American Society of Neuroimaging· 2026· PMID 41568938mais citado
  4. IDH-wildtype diffuse glioma initially presenting as limbic encephalitis: A case report.
    Radiology case reports· 2026· PMID 41142863mais citado
  5. Reprogramming Patient-Derived urine cells into iPSCs for Anti-GAD65 autoimmune encephalitis research.
    Stem cell research· 2025· PMID 40752256mais citado
  6. Anti-Glutamic Acid Decarboxylase 65 (Anti-GAD65) Autoimmune Encephalopathy: A Case of Diagnostic and Treatment Challenge.
    Cureus· 2026· PMID 41994747recente
  7. Autoimmune Limbic Encephalitis With Leucine-Rich Glioma-Inactivated 1 (LGI1) Antibodies Presenting as Rapidly Progressive Cognitive Decline and Psychiatric Symptoms: A Case Report.
    Cureus· 2026· PMID 41982561recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:623615(Orphanet)
  2. MONDO:0850097(MONDO)
  3. GARD:22492(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)

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 límbica autoimune
Compêndio · Raras BR

Encefalite límbica autoimune

ORPHA:623615 · MONDO:0850097
Prevalência
1-9 / 100 000
CID-10
G04.8 · Outras encefalites, mielites e encefalomielites
CID-11
Início
All ages
Prevalência
1.7 (Europe)
MedGen
UMLS
C2930824
EuropePMC
Wikipedia
Papers 10a
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