Síndrome paraneoplásica que envolve o sistema nervoso.
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Síndrome paraneoplásica que envolve o sistema nervoso.
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Sinais e sintomas
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Partes do corpo afetadas
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Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 142 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
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Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
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🇧🇷 Atendimento SUS — Síndrome neurológico paraneoplásico
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Publicações mais relevantes
Motor neuron disease can present as a paraneoplastic neurologic syndrome with various phenotypes.
Paraneoplastic motor neuron disease is an uncommon paraneoplastic neurologic syndrome whose existence has fallen into ambiguity. Epidemiologic studies that have addressed the association between cancer and motor neuron disease have provided conflicting results. Case studies that report motor neuron disease presentation at the time of active malignant disease, in the presence of another paraneoplastic neurologic syndrome or onconeural antibody or with neurologic response to antineoplastic treatment provide strong evidence for paraneoplastic motor neuron disease. However, conclusive evidence about the existence and the clinical and laboratory profiles of this neurologic syndrome is lacking. In this study, we report four new cases of paraneoplastic motor neuron disease, two of whom with expression of Sry-like high mobility group box 1 (SOX1) antibody. We also present a systematic review of all cases of paraneoplastic motor neuron disease reported to date that fulfill prespecified inclusion criteria with individual participant data meta-analysis of the demographic, clinical and laboratory features of the disease. Our data demonstrate that motor neuron disease can present as a paraneoplastic neurologic syndrome. Paraneoplastic motor neuron disease spans the whole motor neuron disease phenotypic spectrum, and it is associated with a wide variety of neoplastic diseases, onconeural antibodies and it may present concurrently with other well-recognized paraneoplastic neurologic syndromes. Paraneoplastic motor neuron disease may be clinically indistinguishable from idiopathic motor neuron disease. Its only distinctive clinical feature is the rapidly progressive course. A subset of cases display immune derangements in cerebrospinal fluid, including increased white cell count, elevated protein, albumin index, IgG index and/or oligoclonal band expression. Cancer-induced inflammatory pathways may trigger the disease in genetically predisposed individuals harboring amyotrophic lateral sclerosis-causing genetic deficits. A thorough evaluation for neoplastic diseases should be carried out upon strong suspicion of this rare paraneoplastic neurologic syndrome to increase the diagnostic yield for this entity. Paraneoplastic motor neuron disease apparently results from complex interactions between degenerative and immune pathways and its pathophysiology may elucidate previously unresolved aspects of idiopathic motor neuron disease pathogenesis.
The history of paraneoplastic neurologic disorders of the CNS, PNS, and autonomic nervous systems: Perspective on the past toward a brighter future.
Cases of paraneoplastic neurologic disease were described as early as the 1800s; despite two centuries of impressive progress in our understanding of immunity, autoimmunity, and paraneoplastic antibodies, we still have a lot to learn about these rare and devastating diseases. This chapter focuses on the history of paraneoplastic neurologic diseases of the central, peripheral, and autonomic nervous systems. Rather than attempting an exhaustive chronicle of every antibody discovered to date, we discuss key discoveries and concepts which laid the groundwork for our current understanding of paraneoplastic neurologic disease as a whole. The work of the pioneers described in this chapter, as well as many others, provides the foundation upon which future work in this field will be built. Reflecting on the incremental nature of scientific discovery, the critical observations which allowed for paradigm shifts, and the gaps in our current knowledge provides valuable lessons for future directions in this field.
Seronegative Autoimmune Encephalitis With Neuropsychiatric Presentation: A Case Report.
Seronegative autoimmune encephalitis (AE) is an immune-mediated inflammatory disorder of the central nervous system that presents with a broad spectrum of neuropsychiatric manifestations, including acute behavioral changes, cognitive dysfunction, catatonia, and altered levels of consciousness. The absence of identifiable neuronal autoantibodies in serum or cerebrospinal fluid contributes to diagnostic uncertainty, delayed treatment initiation, and increased morbidity. Given the potential reversibility of this condition, early recognition and prompt initiation of immunotherapy are critical to optimizing clinical outcomes. We report the case of a 68-year-old woman with probable seronegative AE who presented with subacute neuropsychiatric deterioration characterized by prominent psychiatric symptoms and catatonia. An extensive diagnostic evaluation, including cerebrospinal fluid analysis, comprehensive autoimmune antibody testing, and exclusion of infectious, metabolic, and structural etiologies, failed to identify an alternative diagnosis. Functional neuroimaging with 18F-fluorodeoxyglucose positron emission tomography revealed cerebral metabolic abnormalities supportive of an inflammatory encephalitic process. The patient demonstrated marked clinical improvement following early initiation of high-dose intravenous corticosteroid therapy. This case underscores the diagnostic complexity of seronegative AE when neuropsychiatric manifestations predominate and overlap with primary psychiatric disorders. It highlights the importance of maintaining a high index of clinical suspicion despite negative serological findings and supports the use of clinical diagnostic criteria and adjunctive functional neuroimaging to guide early immunotherapeutic intervention in suspected seronegative AE.
Clinical characteristics, treatment, and outcomes in anti-metabotropic glutamate receptor-1 encephalitis: A systematic review.
To characterize the clinical manifestations, paraclinical findings, treatment, and outcomes in 42 cases of anti-metabotropic glutamatergic receptor-1 (mGluR1) encephalitis. We conducted a systematic review of 42 reported cases of anti-mGluR1 encephalitis in accordance with the PRISMA statement. Clinical data were systematically extracted from published literature in PubMed, Web of Science, Embase, and Cochrane databases spanning January 2000 to August 2025. Statistical analysis was conducted on demographic characteristics, clinical presentations, paraclinical findings, treatment regimens, and clinical outcomes. Twenty-two articles with 42 cases were included. The median age at onset was 50.5 years (range: 3-81 years) with equal gender distribution. Prodromal symptoms were present in 26.2 % of cases. Cerebellar manifestations developed in nearly all cases (n = 41, 97.6 %) during the disease course. Lymphoma represented the most frequently associated malignancy. Magnetic resonance imaging (MRI) abnormalities were detected in 50.0 % of patients and inflammatory cerebrospinal fluid findings were frequently observed. Glucocorticoids constituted the most common immunotherapy (n = 29, 80.6 %). The majority of patients showed clinical improvement (n = 31), and a few of patients (n = 5) experienced disease relapses. Anti-mGluR1 encephalitis represents a rare yet treatable autoimmune disorder, characterized by predominant cerebellar involvement, typically of non-paraneoplastic origin. Initial comprehensive neoplastic screening appears sufficient for most cases. Immunotherapy yields favorable responses in most patients, with generally good long-term outcomes.
A case of MOGAD with rectal adenocarcinoma: Comorbidity or paraneoplastic neurological syndrome?
Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare autoimmune disease characterized primarily by central nervous system demyelination. We report a rare case of MOGAD coexisting with rectal adenocarcinoma. A 59-year-old female presented with fever and bilateral lower limb weakness. MRI of the brain revealed abnormal signals in multiple regions of the cerebrum, brainstem, and spinal cord. Both serum and cerebrospinal fluid tested positive for MOG antibodies. The symptoms improved after steroid therapy. During hospitalization, colonoscopy and pathological examination revealed rectal cancer, which was subsequently treated surgically. After six months of follow-up, neither the tumor nor MOGAD recurred. Paraneoplastic etiologies may also contribute to the development of MOGAD. To date, no cases of MOGAD associated with rectal cancer have been reported. It remains uncertain whether paraneoplastic neurologic syndrome (PNS) is involved in this patient.
Publicações recentes
Autoimmune encephalitis and paraneoplastic neurologic syndrome: clinical diagnosis.
Motor neuron disease can present as a paraneoplastic neurologic syndrome with various phenotypes.
Seronegative Autoimmune Encephalitis With Neuropsychiatric Presentation: A Case Report.
The history of paraneoplastic neurologic disorders of the CNS, PNS, and autonomic nervous systems: Perspective on the past toward a brighter future.
Cancer-related movement disorders: A scoping review and diagnostic approach.
📚 EuropePMC47 artigos no totalmostrando 83
Motor neuron disease can present as a paraneoplastic neurologic syndrome with various phenotypes.
Brain communicationsSeronegative Autoimmune Encephalitis With Neuropsychiatric Presentation: A Case Report.
CureusThe history of paraneoplastic neurologic disorders of the CNS, PNS, and autonomic nervous systems: Perspective on the past toward a brighter future.
Handbook of clinical neurologyCancer-related movement disorders: A scoping review and diagnostic approach.
Neuro-oncology practiceBaseline neuronal antibodies in patients with small cell lung cancer are not necessarily associated with post-immune checkpoint inhibitors neurotoxicities.
Frontiers in immunologyClinical characteristics, treatment, and outcomes in anti-metabotropic glutamate receptor-1 encephalitis: A systematic review.
Clinical neurology and neurosurgeryLeukemoid Reaction and Sudden Hearing Loss in Early Cervical Cancer: A Case Report and Review of the Literature.
International journal of women's healthAnti-Ri Paraneoplastic Neurologic Syndrome in a Patient With Untreated Breast Cancer.
CureusRapid Paralysis and Hidden Malignancy: Acute Motor Axonal Neuropathy Revealing Pleomorphic Liposarcoma.
European journal of case reports in internal medicineA case of MOGAD with rectal adenocarcinoma: Comorbidity or paraneoplastic neurological syndrome?
Brain, behavior, & immunity - healthAnti-RGS8 Paraneoplastic Neurologic Syndrome Presenting with Skew Deviation and Mild Cerebellar Dysfunction.
Cerebellum (London, England)Exacerbation of Paraneoplastic Neurologic Syndrome After Immune Checkpoint Inhibitor Treatment in Advanced Small Cell Lung Cancer: A Case Report.
CureusA Rare Cause of Rapid Progression to Intestinal Failure.
GastroenterologyParaneoplastic Leucine Zipper 4 IgG Associated Motor-Predominant Polyradiculoneuropathy.
Journal of the peripheral nervous system : JPNSParaneoplastic Neurologic Syndrome Associated with Fallopian Tube Cancer: A Case Report.
Case reports in oncologyClinical characteristics and immunotherapy response in paraneoplastic neurologic syndrome patients with increased number of high-risk antibodies.
Frontiers in immunologyThe limbic and extra-limbic encephalitis associated with glutamic acid decarboxylase (GAD)-65 antibodies: an observational study.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology[Anti-Hu syndrome - an unusual cause of vertigo].
PraxisAnti-Tr/DNER Antibody-associated Paraneoplastic Neurological Syndrome Presenting Limbic Encephalitis with Anaplastic Large Cell Lymphoma.
Internal medicine (Tokyo, Japan)Central sleep apnea as an initial presentation of small cell lung carcinoma with anti-Hu antibody-related paraneoplastic neurologic syndrome.
Respirology case reportsPerformance Assessment of the New EUROLINE Neurologic Syndrome 15 Ag (IgG) for the Determination of Autoantibodies Associated with Neurological Disorders.
NeurologyComprehensive Analysis of Paraneoplastic Neurologic Syndrome and PNS-CARE Diagnostic Criteria in Clinical Practice.
Neurology(R) neuroimmunology & neuroinflammationExtra-Limbic Seronegative Encephalitis Preceding Recurrent Hodgkin's Lymphoma and Gastric Diffuse Large B-Cell Lymphoma: A Case Report.
The NeurohospitalistComplexities in the Diagnosis and Management of Anti-Hu Antibody-Associated Paraneoplastic Syndrome.
CureusParaneoplastic Neurologic Disorders.
Continuum (Minneapolis, Minn.)Case report: PCA-2-associated encephalitis with different clinical phenotypes: a two-case series and literature review.
Frontiers in immunologyClinical and serological insights into paraneoplastic brachial amyotrophic diplegia.
Journal of neurologyClinical approach to diagnosis of paraneoplastic neurologic syndromes.
Handbook of clinical neurologyParaneoplastic neurologic syndrome and autoantibody accompaniments of germ cell tumors.
Handbook of clinical neurologyHematologic malignancies and hematopoietic stem cell transplantation.
Handbook of clinical neurologyParaneoplastic neurologic syndrome associated with gynecologic and breast malignancies.
Handbook of clinical neurologyClinical Impact of Pre-Existing Autoantibodies in Patients With SCLC Treated With Immune Checkpoint Inhibitor: A Multicenter Prospective Observational Study.
JTO clinical and research reportsParaneoplastic Anti-Neuronal Nuclear Antibody Type 3 Neurologic Autoimmunity.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesA Case of Primary Lung Adenocarcinoma With Two Uncommon Presentations: Neurological Paraneoplastic Syndrome and Pericardial Effusion.
CureusA not so incidental 'incidentaloma' - pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review.
Frontiers in neurologyIdentification of SKOR2 IgG as a novel biomarker of paraneoplastic neurologic syndrome.
Frontiers in immunologyDiagnosis and Treatment of Paraneoplastic Neurologic Syndromes.
Antibodies (Basel, Switzerland)Collapsin response mediator protein 5-associated optic neuropathy: clinical characteristics, radiologic clues, and outcomes.
Frontiers in neurologySubacute Horizontal Diplopia, Jaw Dystonia, and Laryngospasm.
Neurology(R) neuroimmunology & neuroinflammationComparison of mediastinal and non-mediastinal neuroblastoma and ganglioneuroblastoma associated with opsoclonus-myoclonus syndrome: a systematic review and meta-analysis.
Translational cancer researchMRI Features of Anti-Ma1/Ma2 Paraneoplastic Neurologic Syndrome.
NeurologyValidation of MATCH score: a predictive tool for identification of patients with kelch-like protein-11 autoantibodies.
Journal of neurology, neurosurgery, and psychiatrySuccessful Treatment of Paraneoplastic Neuropathy and Pruritis With Scrambler Therapy: A Case Report.
CureusCase Report: Presence of Anti-MAG in the CSF Can Be Associated With a Neurodegenerative Process With Frontal Involvement.
Frontiers in neurologyA Case of a Splitting Headache: Paraneoplastic Rhombencephalitis.
CureusPearls & Oy-sters: Gait Instability, Jaw Dystonia, and Horizontal Diplopia in a Woman With Anti-Ri Antibodies and Breast Cancer.
NeurologyEvaluation of the Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes in China.
Frontiers in immunologyAnti-Ri-Associated Paraneoplastic Neurological Syndrome Revealing Breast Cancer: A Case Report.
CureusMyasthenia Gravis and Myeloproliferative Neoplasms - Mere Association or Paraneoplastic Neurologic Syndrome: A Mini-Review.
Acta bio-medica : Atenei ParmensisAnti-Yo-Associated Paraneoplastic Cerebellar Degeneration: Case Series and Review of Literature.
CureusAnti-Ma2 Antibody-Associated Paraneoplastic Neurological Syndromes: A Pilot Study.
Brain sciencesLung cancer masquerading as a paraneoplastic neurologic syndrome without a primary lung mass: Case report and review of literature.
Lung India : official organ of Indian Chest SocietyCV2/CRMP5-antibody-related Paraneoplastic Neurologic Syndrome Associated with Gastrointestinal Stromal Tumor.
Internal medicine (Tokyo, Japan)Immunopathogenesis and proposed clinical score for identifying Kelch-like protein-11 encephalitis.
Brain communicationsGuillain-Barré Syndrome as a Presenting Symptom in Breast Cancer: The Importance of Considering Paraneoplastic Neurologic Syndrome.
CureusLow-dose interleukin-2 as a novel therapeutic option for refractory paraneoplastic neurologic syndrome: a case of chronic relapsing anti-Ma2/Ta paraneoplastic myeloradiculopathy.
Encephalitis (Seoul, Korea)Diffuse Large B-Cell Lymphoma Presenting as an Unusual Paraneoplastic Neurologic Syndrome Affecting the Central and Peripheral Nervous Systems.
CureusParaneoplastic Neurologic Syndrome in Ewing Sarcoma.
The American journal of medicineNeuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect.
International journal of molecular sciencesParaneoplastic Disorders of the Nervous System.
Continuum (Minneapolis, Minn.)Imaging Review of Paraneoplastic Neurologic Syndromes.
AJNR. American journal of neuroradiologyBrain paraneoplastic syndromes in a patient with mediastinal ganglioneuroma.
Radiology case reportsExpanded Clinical Phenotype, Oncological Associations, and Immunopathologic Insights of Paraneoplastic Kelch-like Protein-11 Encephalitis.
JAMA neurologyClinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.
Neurology(R) neuroimmunology & neuroinflammationWorsening of anti-Hu paraneoplastic neurological syndrome related to anti-PD-1 treatment: Case report and review of literature.
Journal of neuroimmunologyAn autoimmune-based, paraneoplastic neurologic syndrome following checkpoint inhibition and concurrent radiotherapy for merkel cell carcinoma: case report.
Strahlentherapie und Onkologie : Organ der Deutschen Rontgengesellschaft ... [et al]18 F-Fluorodeoxyglucose positron-emission tomography for the investigation of malignancy in patients with suspected paraneoplastic neurologic syndromes and negative or indeterminate conventional imaging: a retrospective analysis of the Ontario PET Access Program, with systematic review and meta-analysis.
Current oncology (Toronto, Ont.)Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors.
Neurology(R) neuroimmunology & neuroinflammationCase of anti-Hu antibody-mediated encephalopathy associated with ovarian cancer.
The journal of obstetrics and gynaecology researchParaneoplastic cerebellar ataxia in Merkel cell carcinoma of unknown primary.
JAAD case reportsParaneoplastic Lambert-Eaton syndrome in a patient with disseminated metastatic cancer.
Cirugia y cirujanosParaneoplastic neurologic syndrome as a presentation of underlying breast malignancy.
The breast journal[A case of hemichorea associated with lung adenocarcinoma].
Rinsho shinkeigaku = Clinical neurologyAnti-Ma2-antibody-associated encephalitis: An atypical paraneoplastic neurologic syndrome.
SA journal of radiology[Paraneoplastic Lambert-Eaton syndrome in a patient with disseminated metastatic cancer].
Cirugia y cirujanosThymoma-associated panencephalitis: a newly emerging paraneoplastic neurologic syndrome.
Neuroimmunology and neuroinflammationAcquired Hemophilia and Anti-Hu Paraneoplastic Neurologic Syndrome in Small Cell Lung Cancer.
The American journal of the medical sciencesImmune-mediated disorders.
Handbook of clinical neurologyAnti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report.
MedicineLimbic encephalitis - a report of four cases.
Central-European journal of immunologyFDG-Avid Keloid on PET/CT Imaging.
Clinical nuclear medicineCerebrospinal fluid markers of neuronal and glial cell damage in patients with autoimmune neurologic syndromes with and without underlying malignancies.
Journal of neuroimmunologyNeuronal Na+/K+ ATPase is an autoantibody target in paraneoplastic neurologic syndrome.
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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.
- Motor neuron disease can present as a paraneoplastic neurologic syndrome with various phenotypes.
- The history of paraneoplastic neurologic disorders of the CNS, PNS, and autonomic nervous systems: Perspective on the past toward a brighter future.
- Seronegative Autoimmune Encephalitis With Neuropsychiatric Presentation: A Case Report.
- Clinical characteristics, treatment, and outcomes in anti-metabotropic glutamate receptor-1 encephalitis: A systematic review.
- A case of MOGAD with rectal adenocarcinoma: Comorbidity or paraneoplastic neurological syndrome?
- Autoimmune encephalitis and paraneoplastic neurologic syndrome: clinical diagnosis.
- Cancer-related movement disorders: A scoping review and diagnostic approach.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:36388(Orphanet)
- MONDO:0018215(MONDO)
- GARD:7326(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q3508804(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
