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.
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.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
+ 15 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Encefalite límbica autoimune
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
Ensaios em destaque
🟢 Recrutando agora
1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
0 ensaios clínicos encontrados.
Publicações mais relevantes
Sjögren Syndrome Associated Limbic Encephalitis and Its Longitudinal Brain FDG PET Manifestations.
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.
Lentiform fork sign mimicking uremic encephalopathy in an atypical case of autoimmune limbic encephalitis.
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.
T1w/T2w Ratio Identifies the Basolateral Amygdala as a Preferential Target in Autoimmune Limbic Encephalitis.
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.
IDH-wildtype diffuse glioma initially presenting as limbic encephalitis: A case report.
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.
Reprogramming Patient-Derived urine cells into iPSCs for Anti-GAD65 autoimmune encephalitis research.
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
Anti-Glutamic Acid Decarboxylase 65 (Anti-GAD65) Autoimmune Encephalopathy: A Case of Diagnostic and Treatment Challenge.
Autoimmune Limbic Encephalitis With Leucine-Rich Glioma-Inactivated 1 (LGI1) Antibodies Presenting as Rapidly Progressive Cognitive Decline and Psychiatric Symptoms: A Case Report.
Sjögren Syndrome Associated Limbic Encephalitis and Its Longitudinal Brain FDG PET Manifestations.
Lentiform fork sign mimicking uremic encephalopathy in an atypical case of autoimmune limbic encephalitis.
T1w/T2w Ratio Identifies the Basolateral Amygdala as a Preferential Target in Autoimmune Limbic Encephalitis.
📚 EuropePMC105 artigos no totalmostrando 198
Sjögren Syndrome Associated Limbic Encephalitis and Its Longitudinal Brain FDG PET Manifestations.
Clinical nuclear medicineLentiform fork sign mimicking uremic encephalopathy in an atypical case of autoimmune limbic encephalitis.
Radiology case reportsT1w/T2w Ratio Identifies the Basolateral Amygdala as a Preferential Target in Autoimmune Limbic Encephalitis.
Journal of neuroimaging : official journal of the American Society of NeuroimagingElectroceuticals for Motherhood: Utility of responsive neurostimulation and Proactive antiseizure medication management in two pregnancies.
Epilepsy & behavior reportsIDH-wildtype diffuse glioma initially presenting as limbic encephalitis: A case report.
Radiology case reportsBrain MRI Revealing the Probable Pathophysiology of Neuropsychiatric Lupus: A Case Report.
CureusIctal cold shiver caused by autoimmune limbic encephalitis: A case report and literature review.
Epilepsy & behavior reportsReprogramming Patient-Derived urine cells into iPSCs for Anti-GAD65 autoimmune encephalitis research.
Stem cell researchWhere There's Smoke, There's Fire: Paraneoplastic Autoimmune Limbic Encephalitis.
CureusNine-Year Follow-Up of GAD65 Antibody Limbic Encephalitis With Clinical Remission Despite Persistent Imaging and Serological Abnormalities.
Cureus[Paraneoplastic limbic encephalitis - an important differential diagnosis in cases of unclear encephalopathy].
PraxisLimbic Encephalitis: An Unusual Presentation on 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (18F-FDG PET/CT).
CureusClinical Characteristics and Seizure Outcomes in Antibody-Positive Autoimmune Limbic Encephalitis.
Journal of inflammation researchLymphocyte signatures correspond to clinical phenotypes in autoimmune limbic encephalitis.
Brain communicationsMultimodal prognostication of autoimmune encephalitis: an Australian autoimmune encephalitis consortium study.
Journal of neurologyExploring autoantigens in autoimmune limbic encephalitis using phage immunoprecipitation sequencing.
Journal of neurologyRetrograde Amnesia in LGI1 and CASPR2 Limbic Encephalitis: Two Case Reports and a Systematic Literature Review.
European journal of neurologySevere 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)Clarithromycin-induced myoclonic status epilepticus.
Practical neurologyCognitive Deficits in Anti-LGI1 Encephalitis Are Linked to Immunotherapy-Resistant White Matter Network Changes.
Neurology(R) neuroimmunology & neuroinflammationAnti-Gamma Aminobutyric Acid B (GABA B) Antibody Receptor-Associated Autoimmune Encephalitis in Pregnancy: A Rare Condition.
CureusAnti-Tr/DNER Antibody-associated Paraneoplastic Neurological Syndrome Presenting Limbic Encephalitis with Anaplastic Large Cell Lymphoma.
Internal medicine (Tokyo, Japan)Anti-LGI1 Autoimmune Limbic Encephalitis: An Easy-To-Miss Diagnosis.
European journal of case reports in internal medicineMarkedly Elevated IgG Index: A Key to Differentiating Neurosyphilis from Autoimmune Limbic Encephalitis.
Internal medicine (Tokyo, Japan)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 reportsDistinguishing seizures in autoimmune limbic encephalitis from mesial temporal lobe epilepsy with hippocampal sclerosis: Clues of a temporal plus network.
Journal of the neurological sciencesFlow cytometry identifies changes in peripheral and intrathecal lymphocyte patterns in CNS autoimmune disorders and primary CNS malignancies.
Journal of neuroinflammationCSF and blood signatures support classification of limbic encephalitis subtypes.
Brain, behavior, and immunityKikuchi-Fujimoto Disease Presenting as Autoimmune Limbic Encephalitis: A Case Report with Review of Literature.
Annals of Indian Academy of NeurologyFDG-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...Human stem cell-derived neurons and astrocytes to detect novel auto-reactive IgG response in immune-mediated neurological diseases.
Frontiers in immunologyA Case of Anti-Leucine-Rich Glioma-Inactivated Protein 1 (Anti-LGI1) Limbic Encephalitis With Normal Imaging.
CureusAnti-LGI1 Autoimmune Encephalitis in a Patient with Rheumatoid Arthritis and MGUS.
European journal of case reports in internal medicine[(Auto)immunity in focal epilepsy: mechanisms of (auto‑)immune-inflammatory epileptogenic neurodegeneration].
Der NervenarztDistinctive 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 NeurophysiologyTwo Cases of Pediatric Leucine-Rich Glioma-Inactivated Protein-1 Encephalitis: Clinical Course, Challenges, and Implications.
Pediatric neurologyDifferentiation between viral and autoimmune limbic encephalitis: a prospective cohort study with development and validation of a diagnostic model.
Journal of neurologyAutoimmune-associated epilepsy - a challenging concept.
SeizureThe role of the human hippocampus in decision-making under uncertainty.
Nature human behaviourStudy on clinical features and factors related to long-term outcomes of antibody-negative autoimmune encephalitis.
Annals of clinical and translational neurologyRemote digital cognitive assessment reveals cognitive deficits related to hippocampal atrophy in autoimmune limbic encephalitis: a cross-sectional validation study.
EClinicalMedicineAntibiomania: clarithromycin-induced neurotoxicity mimicking autoimmune limbic encephalitis.
Practical neurologyCase Report: Limbic encephalitis following treatment with durvalumab for small-cell lung cancer.
Frontiers in immunologyParaneoplastic Autoimmune Limbic Encephalitis Associated with an Atypical Carcinoid Tumor of the Lung: A Case Report.
Case reports in neurologyAntibody-Negative Autoimmune Encephalitis: A Single-Center Retrospective Analysis.
Neurology(R) neuroimmunology & neuroinflammationAnti-Ma2 encephalitis in a phenotypic female with XY gonadal dysgenesis: A case report.
Epilepsy & behavior reportsAutoimmune Limbic Encephalitis in Patients with Hematologic Malignancies after Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplant Cyclophosphamide.
CellsMimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria.
Neurology(R) neuroimmunology & neuroinflammationAutoimmune encephalitis related to LGI1 antibodies with negative MRI study: Description of two cases.
Medicina clinicaA case of leucine-rich glioma-inactivated 1 antibody encephalitis with schizophrenia-like symptoms as an initial clinical manifestation.
Encephalitis (Seoul, Korea)Autoimmune limbic encephalitis combined with leptomeningeal metastases of non-small cell lung cancer: treatment response to osimertinib, immunoglobulin, rituximab, and tocilizumab.
Encephalitis (Seoul, Korea)Limbic Encephalitis With Dual Positivity.
CureusRare Association of Autoimmune Limbic Encephalitis and Stiff Person Syndrome.
Rhode Island medical journal (2013)Translational imaging of TSPO reveals pronounced innate inflammation in human and murine CD8 T cell-mediated limbic encephalitis.
Science advancesDouble Positive Anti-CASPR2 and LGI1 Antibodies Associated with Two Episodes of Clinically Distinct Disorders.
The Israel Medical Association journal : IMAJ18 F-FDG PET and MRI Evaluation of Paraneoplastic Limbic Encephalitis.
Clinical nuclear medicineAutoimmune limbic encephalitis mimicking inferior myocardial infarction on ECG.
BMJ case reportsComparative Effects of Domain-Specific Human Monoclonal Antibodies Against LGI1 on Neuronal Excitability.
Neurology(R) neuroimmunology & neuroinflammationA feline model of spontaneously occurring autoimmune limbic encephalitis.
Veterinary journal (London, England : 1997)Neurosyphilis: The Great Imitator.
CureusReview 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 neurosurgeryA Case of Anti-Leucine-Rich Glioma-Inactivated Protein 1 (Anti-LGI1) Encephalitis With an Unusual Frontomesial Motor Cortex T2 MRI Hyperintensity.
CureusEarly-Stage Contactin-Associated Protein-like 2 Limbic Encephalitis: Clues for Diagnosis.
Neurology(R) neuroimmunology & neuroinflammationCytokines/chemokines and soluble immune checkpoint molecules in anti-GABAB receptor encephalitis.
Multiple sclerosis and related disordersAutoimmune seronegative limbic encephalitis following ChAdOx1-S/nCoV-19 vaccination in a patient with ankylosing spondylitis.
Journal of medical virologyPost-COVID-19 Vaccine Limbic Encephalitis: A Case Report.
CureusIncreased T- and B-cells associated with the phenotype of autoimmune limbic encephalitis with mainly memory dysfunction.
Journal of translational autoimmunity18F-FDG PET for the diagnosis and assessment of treatment response of autoimmune limbic encephalitis.
Revista espanola de medicina nuclear e imagen molecularSeronegative Autoimmune Limbic Encephalitis: A Case Report.
The Journal of the Association of Physicians of IndiaAutoimmune neuromyotonia.
Current opinion in neurologyNeurosyphilis presenting as autoimmune limbic encephalitis: A case report and literature review.
MedicineMature Cystic Teratoma Combined With Autoimmune Limbic Encephalitis Mimicking Functional Ovarian Cyst.
CureusA reverse genetics and genomics approach to gene paralog function and disease: Myokymia and the juxtaparanode.
American journal of human geneticsA rare case of antileucine-rich glioma-inactivated 1 encephalitis in a 14-year-old girl.
The Turkish journal of pediatricsDistinct movement disorders in contactin-associated-protein-like-2 antibody-associated autoimmune encephalitis.
Brain : a journal of neurologyNonparaneoplastic 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 NeurologyCase report of anti-NMDA receptor encephalitis in a 24-year-old female: an uncommon presentation.
The Egyptian journal of neurology, psychiatry and neurosurgeryAutoimmune Voltage-Gated Potassium Channel Limbic Encephalitis With Auditory and Visual Hallucinations.
CureusConstruction of an Assisted Model Based on Natural Language Processing for Automatic Early Diagnosis of Autoimmune Encephalitis.
Neurology and therapyWhole-brain metabolic pattern analysis in patients with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis.
European journal of neurologySeronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.
Brain : a journal of neurologyHLA-DRB1*03:01 is associated with female sex and younger age of anti-LGI1 encephalitis.
European journal of neurologyPilomotor seizures in autoimmune limbic encephalitis: description of two GAD65 antibodies- related cases and literature review.
SeizureAdenylate kinase 5 (AK5) autoimmune encephalitis: Clinical presentations and outcomes in three new patients.
Journal of neuroimmunologySeronegative limbic encephalitis in association with Sjögren's syndrome: a rare case report.
The Pan African medical journalBilaterality of temporal EEG findings in limbic encephalitis compared to other mesiotemporal epilepsies - A retrospective cohort study.
SeizureNeuropsychological implication in possible antibody-negative limbic encephalitis: a clinical case report.
The Journal of international medical researchNeuropsychological Performance in Autoimmune Limbic Encephalitis: Evidence from an Immunotherapy-Naïve Cohort.
Archives of clinical neuropsychology : the official journal of the National Academy of NeuropsychologistsClinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study.
Annals of clinical and translational neurologyImmunotherapy for Refractory Autoimmune Encephalitis.
Frontiers in immunologyRecurrent Anti-AMPA Receptor Limbic Encephalitis: A Case Report and Literature Review.
Frontiers in neurologyImpact of T cells on neurodegeneration in anti-GAD65 limbic encephalitis.
Annals of clinical and translational neurologyAtypical Association of Autoimmune Limbic Encephalitis with Anti-NMDA Receptor Antibodies in a Young Male Patient: Clinical, Imaging, and Neuropsychological Characteristics.
Case reports in neurologyExecutive deficits in neuropsychological testing differentiate between autoimmune temporal lobe epilepsy caused by limbic encephalitis and temporal lobe epilepsies with non-autoimmune etiologies.
Epilepsy & behavior : E&BRole of LGI1 protein in synaptic transmission: From physiology to pathology.
Neurobiology of diseaseComprehensive B-Cell Immune Repertoire Analysis of Anti-NMDAR Encephalitis and Anti-LGI1 Encephalitis.
Frontiers in immunologyAutoimmune Limbic Encephalitis: A Review of Clinicoradiological Features and the Challenges of Diagnosis.
CureusI can't count, but I can beat you playing cards: a case report on autoimmune encephalitis.
BMC neurologyAnti-leucine-rich-glioma-inactivated 1 Limbic Encephalitis: An Underrecognised Disease.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPStatus epilepticus suspected autoimmune: Neuronal surface antibodies and main clinical features.
EpilepsiaFeline temporal lobe epilepsy: seven cases of hippocampal and piriform lobe necrosis in England and literature review.
Journal of feline medicine and surgeryRare paraneoplastic syndrome of prostatic cancer: limbic encephalitis: a case report.
Journal of medical case reportsDiagnostic challenges in patients with temporal lobe seizures and features of autoimmune limbic encephalitis.
European journal of neurologyDeterminants of cognition in autoimmune limbic encephalitis-A retrospective cohort study.
HippocampusSerum antiganglioside antibodies in patients with autoimmune limbic encephalitis.
Turkish journal of medical sciencesTrans-synaptic LGI1-ADAM22-MAGUK in AMPA and NMDA receptor regulation.
NeuropharmacologyCase Report: Autoimmune Encephalitis Associated With Anti-glutamic Acid Decarboxylase Antibodies: A Pediatric Case Series.
Frontiers in neurologyASL 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 NeurophysiologyTakotsubo syndrome associated with autoimmune limbic encephalitis: a case report.
BMC cardiovascular disordersOrbitofrontal syndrome and "goosebumps"-A new manifestation of autoimmune epilepsy in anti-LGI1 encephalitis.
SeizureAutoimmune limbic encephalitis related to SARS-CoV-2 infection: Case-report and review of the literature.
Brain, behavior, & immunity - healthInitial cerebrospinal fluid-restricted oligoclonal bands associate with anti-N-methyl-D-aspartate receptor encephalitis severity: a pilot study.
Encephalitis (Seoul, Korea)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 videotapeA scoping review on paraneoplastic autoimmune limbic encephalitis (PALE) psychiatric manifestations.
CNS spectrumsAnti-N-methyl-d-aspartate Receptor Encephalitis Related Sinus Node Dysfunction and the Lock-Step Phenomenon.
American journal of medical case reportsLGI1 encephalitis with squamous lung-cell carcinoma: Resolution after tumor resection.
Neurology(R) neuroimmunology & neuroinflammationA 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 disordersPsychiatric symptoms in anti glutamic acid decarboxylase associated limbic encephalitis in adults: a systematic review.
Neuroscience and biobehavioral reviewsDouble 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 HaemapheresisMetabolic Imaging Patterns on 18F-FDG PET in Acute and Subacute LGI1 Autoimmune Limbic Encephalitis.
Clinical nuclear medicineSpecific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis.
Journal of neurologyNeurobehavioral Disorders: The Corticolimbic System in Health and Disease.
The Veterinary clinics of North America. Small animal practiceDoes hippocampal atrophy explain anterograde and retrograde amnesia following autoimmune limbic encephalitis?
HippocampusLimbic encephalitis in a neuroscientist: CASPR 2 antibody-associated disease after antigen exposure.
Journal of neuroimmunologyClinical features of nine cases of leucine-rich glioma inactivated 1 protein antibody-associated encephalitis.
Acta neurologica BelgicaGlioblastoma as an autoimmune limbic encephalitis mimic: A case and review of the literature.
Journal of neuroimmunologyEfficacy of Tocilizumab in Limbic Encephalitis with Anti-CASPR2 Antibodies.
Case reports in neurological medicineDistinct 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 sciencesVery 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 PractitionersDopaminergic Hyperactivity in Neurological Patients with Delirium.
Archives of medical researchPathologic tearfulness after limbic encephalitis: A novel disorder and its neural basis.
NeurologyMultimodality 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, IndiaLGI-1 antibody encephalitis in a seven-year-old girl.
Epileptic disorders : international epilepsy journal with videotapeLow CSF CD4/CD8+ T-cell proportions are associated with blood-CSF barrier dysfunction in limbic encephalitis.
Epilepsy & behavior : E&BAutoimmune Limbic Encephalitis in a Patient with Acute Encephalopathy and Hyponatremia.
Case reports in medicineDiagnosing autoimmune encephalitis in a real-world single-centre setting.
Journal of neurologyPredictive value of high titer of GAD65 antibodies in a case of limbic encephalitis.
Journal of neuroimmunologyCharacterizing memory loss in patients with autoimmune limbic encephalitis hippocampal lesions.
HippocampusSyncope and Twitching at the Emergency Department.
The American journal of case reportsClinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series.
Internal medicine (Tokyo, Japan)Network-wide abnormalities explain memory variability in hippocampal amnesia.
eLifeImpact of anti-CASPR2 autoantibodies from patients with autoimmune encephalitis on CASPR2/TAG-1 interaction and Kv1 expression.
Journal of autoimmunityDiagnosing autoimmune limbic encephalitis.
CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienneHippocampal network abnormalities explain amnesia after VGKCC-Ab related autoimmune limbic encephalitis.
Journal of neurology, neurosurgery, and psychiatryConventional brain MRI features distinguishing limbic encephalitis from mesial temporal glioma.
NeuroradiologyHashimoto's Encephalopathy Presenting with Smoldering Limbic Encephalitis.
Internal medicine (Tokyo, Japan)Deleted in colorectal cancer (netrin-1 receptor) antibodies and limbic encephalitis in a cat with hippocampal necrosis.
Journal of veterinary internal medicineAntibody Prevalence in Epilepsy and Encephalopathy score: Increased specificity and applicability.
EpilepsiaDevelopment of the clinical assessment scale in autoimmune encephalitis.
Annals of neurologyGastaut-Geschwind Syndrome, Faciobrachial Dystonic Seizure, and Autoimmune Limbic Encephalitis.
Case reports in psychiatryAutoimmune Epilepsy and/or Limbic Encephalitis Can Lead to Changes in Sleep Spindles.
Noro psikiyatri arsiviLimbic encephalitis: Experience of a moroccan center.
Brain and behaviorLGI1 antibodies alter Kv1.1 and AMPA receptors changing synaptic excitability, plasticity and memory.
Brain : a journal of neurologyUse of intravenous immunoglobulin for the treatment of autoimmune encephalitis: audit of the NHS experience.
JRSM openPredictors of neural-specific autoantibodies and immunotherapy response in patients with cognitive dysfunction.
Journal of neuroimmunologyToxic Epidermal Necrolysis in a Patient with Autoimmune Limbic Encephalitis with Anti-Glutamate Receptor Antibodies.
Case reports in neurologyAutoimmune limbic encephalitis in association with acute stroke.
Neurology. Clinical practiceDistinction between anti-VGKC-complex seropositive patients with and without anti-LGI1/CASPR2 antibodies.
Journal of the neurological sciencesLimbic encephalitis associated with systemic sclerosis.
Multiple sclerosis and related disordersPractical issues in measuring autoantibodies to neuronal cell-surface antigens in autoimmune neurological disorders: 190 cases.
Journal of the neurological sciencesStriatal hypermetabolism in a case of IgG4-related disease.
Nuclear medicine review. Central & Eastern EuropeSimultaneous 18F- FDG PET/MRI in Autoimmune Limbic Encephalitis.
Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, IndiaClaustrum hyperintensities: A potential clue to autoimmune epilepsy.
Epilepsia openPossible anti-VGKC autoimmune limbic encephalitis associated with SIADH.
BMJ case reportsGamma-aminobutyric acid-B limbic encephalitis and asystolic cardiac arrest: a case report.
Journal of medical case reportsMolecular cloning and characterization of the family of feline leucine-rich glioma-inactivated (LGI) genes, and mutational analysis in familial spontaneous epileptic cats.
BMC veterinary researchAnti-Hu-Associated Autoimmune Limbic Encephalitis in a Patient with PD-1 Inhibitor-Responsive Myxoid Chondrosarcoma.
The oncologistApplication of the 2016 diagnostic approach for autoimmune encephalitis from Lancet Neurology to Chinese patients.
BMC neurologySelective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies.
Frontiers in immunologyA rare case of autoimmune limbic encephalitis: an uncharted territory!
Neurosciences (Riyadh, Saudi Arabia)[Autoimmune and Epilepsy].
Brain and nerve = Shinkei kenkyu no shinpoDissociation findings between short-term and long-term memory in autoimmune limbic encephalitis.
Journal of the neurological sciencesComprehensive and Methodical: Diagnostic and Management Approaches to Rapidly Progressive Dementia.
Current treatment options in neurologyPeripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review.
Brain and behaviorSeronegative Paraneoplastic Limbic Encephalitis Associated with Thymoma.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive NeurologyAutoimmune Limbic Encephalitis Associated with Type 1 Diabetes Mellitus.
Journal of clinical research in pediatric endocrinologyAUTOIMMUNE LIMBIC ENCEPHALITIS (CASE REPORTS).
Georgian medical newsGAD antibody-associated limbic encephalitis in a young woman with APECED.
Endocrinology, diabetes & metabolism case reportsPredictive models in the diagnosis and treatment of autoimmune epilepsy.
EpilepsiaConfusion, Faciobrachial Dystonic Seizures, and Critical Hyponatremia in a Patient with Voltage-Gated Potassium Channel Encephalitis.
Korean journal of family medicineAutoimmune limbic encephalitis with anti-contactin-associated protein-like 2 antibody secondary to pembrolizumab therapy.
Journal of neuroimmunologyLimbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies: A clinical subtype of Hashimoto encephalopathy.
Medicine[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 PsychopharmacologyN-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[A case of neurosyphilis presenting with limbic encephalitis].
Rinsho shinkeigaku = Clinical neurologyAnti-LGI1 encephalitis: Clinical syndrome and long-term follow-up.
NeurologyNeuropsychological and FDG-PET profiles in VGKC autoimmune limbic encephalitis.
Brain and cognitionCorrelations of Clusters of Non-Convulsive Seizure and Magnetic Resonance Imaging in a Case With GAD65-Positive Autoimmune Limbic Encephalitis.
Journal of clinical medicine researchAutoimmune 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)[Autoimmune Associated Encephalitis and Dementia].
Brain and nerve = Shinkei kenkyu no shinpoRituximab treatment for autoimmune limbic encephalitis in an institutional cohort.
NeurologyLimbic Encephalitis Associated With GAD65 Antibodies: Brief Review of the Relevant literature.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesA unique combination of autoimmune limbic encephalitis, type 1 diabetes, and Stiff person syndrome associated with GAD-65 antibody.
Annals of Indian Academy of NeurologySuspected antibody negative autoimmune limbic encephalitis: outcome of immunotherapy.
Acta neurologica ScandinavicaClinical characterization of autoimmune LGI1 antibody limbic encephalitis.
Epilepsy & behavior : E&BCognitive Impairments Preceding and Outlasting Autoimmune Limbic Encephalitis.
Case reports in neurological medicineAutoimmune limbic encephalitis due to VGKC-Ab. Thanks for the memory. Leo Robin (Title of song, 1937).
Practical neurology[Faciobrachial dystonic seizures. Semiologic diagnosis in limbic encephalitis].
MedicinaAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Encefalite límbica autoimune.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Encefalite límbica autoimune
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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.
- Sjögren Syndrome Associated Limbic Encephalitis and Its Longitudinal Brain FDG PET Manifestations.
- Lentiform fork sign mimicking uremic encephalopathy in an atypical case of autoimmune limbic encephalitis.
- 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
- IDH-wildtype diffuse glioma initially presenting as limbic encephalitis: A case report.
- Reprogramming Patient-Derived urine cells into iPSCs for Anti-GAD65 autoimmune encephalitis research.
- Anti-Glutamic Acid Decarboxylase 65 (Anti-GAD65) Autoimmune Encephalopathy: A Case of Diagnostic and Treatment Challenge.
- Autoimmune Limbic Encephalitis With Leucine-Rich Glioma-Inactivated 1 (LGI1) Antibodies Presenting as Rapidly Progressive Cognitive Decline and Psychiatric Symptoms: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:623615(Orphanet)
- MONDO:0850097(MONDO)
- GARD:22492(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
