904 results match your criteria: "Paraneoplastic Cerebellar Degeneration"
Anti-Tr/DNER Antibody-associated Paraneoplastic Neurological Syndrome Presenting Limbic Encephalitis with Anaplastic Large Cell Lymphoma: A Case Report.
Intern Med
December 2024
Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.
An 82-year-old man presented with acute progressive disturbance of consciousness. We suspected autoimmune limbic encephalitis because of abnormal magnetic resonance imaging findings in the bilateral temporal lobes and cerebrospinal fluid pleocytosis. The patient tested positive for anti-Tr/Delta/Notch-like epidermal growth factor-related receptor (DNER) antibodies, and a tissue biopsy revealed complications of anaplastic large cell lymphoma.
View Article and Find Full Text PDFCase report: anti-IgLON5 disease combined with paraneoplastic cerebellar degeneration with the detection of anti-sulfatide IgG antibody, masquerading as meningoencephalitis.
BMC Neurol
December 2024
Department of Neurology, Guangdong Sanjiu Brain Hospital, Guangzhou, Guangdong, China.
Objective: Anti-IgLON5 disease is a rare autoimmune mediated disease. It is mainly featured by sleep-related disturbance, parkinsonism, chorea and limb ataxia. Previous studies had clarified its clinical manifestations and predisposing genes.
View Article and Find Full Text PDFParaneoplastic Syndromes of the Nervous System in Patients Suffering from SCLC. A Review of the Recent Literature.
Acta Med Acad
August 2024
Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Background: Paraneoplastic Neurological Syndromes (PNS) constitute a heterogeneous cluster of disease manifestations related to various cancers. Small Cell Lung Cancer (SCLC) is strongly related to PNS. This narrative review conducted a survey in the available PubMed literature to highlight the appearance of PNSs in SCLC cases and discuss published research highlights on the subject so that general practitioners can be acquainted with the medical phenomenon present in SCLC patients.
View Article and Find Full Text PDFFLOCCULAR SYNDROME- AN ATYPICAL PRESENTATION OF PARANEOPLASTIC CEREBELLAR DEGENERATION.
J Ayub Med Coll Abbottabad
December 2024
Liaquat National Hospital, Karachi-Pakistan.
Article Synopsis
CDR2 is a dynein adaptor recruited by kinectin to regulate ER sheet organization.
bioRxiv
November 2024
i3S - Instituto de Investigação e Inovação em Saúde, Universidade do Porto, 4200-135 Porto, Portugal.
The endoplasmic reticulum (ER) relies on the microtubule cytoskeleton for distribution and remodelling of its extended membrane network, but how microtubule-based motors contribute to ER organization remains unclear. Using biochemical and cell-based assays, we identify cerebellar degeneration-related protein 2 (CDR2) and its paralog CDR2-like (CDR2L), onconeural antigens with poorly understood functions, as ER adaptors for cytoplasmic dynein-1 (dynein). We demonstrate that CDR2 is recruited by the integral ER membrane protein kinectin (KTN1) and that double knockout of CDR2 and CDR2L enhances KTN1-dependent ER sheet stacking, reversal of which by exogenous CDR2 requires its dynein-binding CC1 box motif.
View Article and Find Full Text PDFAltered exosomal miRNA profiles in patients with paraneoplastic cerebellar degeneration.
Ann Clin Transl Neurol
December 2024
Department of Clinical Medicine, University of Bergen, Bergen, Norway.
Article Synopsis
- The study explores the relationship between ovarian cancer (OC) and anti-Yo-associated paraneoplastic cerebellar degeneration (PCD), focusing on changes in exosomal microRNA (miRNA) profiles in OC patients with PCD compared to healthy individuals and those without PCD.
- Serum exosomes from three groups (OC with PCD, OC without PCD, and healthy controls) were analyzed, revealing 103 differentially expressed miRNAs in PCD patients versus OC patients without PCD, and 139 versus healthy controls.
- Key miRNAs such as miR-486-5p and miR-21-5p showed potential as biomarkers for diagnosing PCD and indicated a link between these miRNAs and the
Blood-Brain Barrier Disruption in Neuroimmunological Disease.
Int J Mol Sci
October 2024
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube 755-8505, Japan.
Article Synopsis
- The blood-brain barrier (BBB) serves as a vital protective barrier for the brain, and its dysfunction is linked to various neuroimmunological diseases like multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD).
- BBB breakdown can allow harmful autoantibodies and lymphocytes to enter the central nervous system, leading to symptoms in diseases such as autoimmune encephalitis and paraneoplastic neurological syndrome.
- Recent findings indicate that therapies targeting BBB integrity could be promising in managing and preventing damage in these neuroimmunological conditions.
Article Synopsis
- The study evaluates the effectiveness of the new EUROLINE Neurologic Syndrome 15 Ag (IgG) test, which adds three new antigens to the existing EUROLINE Paraneoplastic Neurologic Syndrome 12 Ag test.
- The analysis involved testing 194 diagnosed samples and over 100 healthy controls, confirming high sensitivity (89-100%) and specificity (≥99%) for the autoantibodies associated with neurological disorders.
- The findings suggest that this new diagnostic tool can improve the detection of various neuronal autoantibodies, particularly by identifying double positivity for anti-CDR2 and anti-CDR2L, which is linked to paraneoplastic cerebellar degeneration.
What we've learnt about autoimmune neurological diseases from neuropathology.
Rev Neurol (Paris)
November 2024
Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria; Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria. Electronic address:
Article Synopsis
- * Diseases like paraneoplastic encephalitis feature T-cell-driven inflammation, causing neuronal damage and distinct changes in the brain's pathology at different disease stages.
- * In contrast, conditions driven by surface antibodies mainly disrupt neuron function without causing direct immune-related damage, with effects varying based on the specific antibodies involved.
Autoimmune Encephalitis: Insights Into Immune-Mediated Central Nervous System Injury.
Korean J Radiol
September 2024
Division of Neuroradiology, Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, Toronto, Canada.
Article Synopsis
- * It can be triggered by various factors, including infections or cancers, and shares imaging features with other types of encephalitis, particularly limbic encephalitis.
- * Increased awareness and understanding of AE have improved its detection, highlighting the importance of recognizing its signs in MRI reports for better treatment outcomes.
"One a penny, two a penny", I saw the hot cross bun sign".
Clin Imaging
October 2024
ChristianaCare Hospital, 4755 Ogletown-Stanton Road, Newark, DE 19718, United States.
Article Synopsis
- * The name comes from the visual resemblance of the brain's MRI images to the cross on a hot cross bun, first identified in a study by Schrag et al. in 1998.
- * Other conditions, such as spinocerebellar ataxia and progressive multifocal leukoencephalopathy, can also present similar imaging characteristics, broadening the differential diagnosis.
Immune Checkpoint Inhibitor-Related Cerebellar Toxicity: Clinical Features and Comparison with Paraneoplastic Cerebellar Ataxia.
Cerebellum
December 2024
Clinical Neurology, Department of Medicine (DMED), University of Udine, Udine, Italy.
Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy, and the association with immune-related adverse events (irAEs) is well-established. However, cerebellar irAEs are poorly defined and their relationship with paraneoplastic disorders remains unclear. Our aim was (i) to characterize cerebellar irAE; (ii) to compare it with paraneoplastic cerebellar ataxia (PCA).
View Article and Find Full Text PDFParaneoplastic neurological syndromes of small cell lung cancer.
Postep Psychiatr Neurol
June 2024
Department of Medical Virology, School of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
Purpose: This article reviews the relevant literature on paraneoplastic neurological syndromes of small cell lung cancer and discusses the clinical presentation, pathophysiology, and diagnosis of these syndromes. It also includes a summary of the current treatment options for the management of them.
Views: Paraneoplastic syndromes are a group of signs and symptoms that develop due to cancer in a remote site, mainly triggered by an autoantibody produced by the tissues involved or lymphocytes during anti-cancer defense.
Recent advances in diagnosis of immune-mediated cerebellar ataxias: novel concepts and fundamental questions on autoimmune mechanisms.
J Neurol
October 2024
Service de Neurologie, Médiathèque Jean Jacquy, CHU-Charleroi, 6000, Charleroi, Belgium.
Article Synopsis
- Immune-mediated cerebellar ataxias (IMCAs) are disorders where the immune system primarily attacks the cerebellum, with various types like gluten ataxia and Miller Fisher syndrome identified, leading to insights into diagnosis and treatment.
- Specific autoantibodies help pinpoint some IMCA causes, but others require careful interpretation, as the relationships between them and the conditions are not always clear.
- New classifications such as primary autoimmune cerebellar ataxia (PACA) and latent autoimmune cerebellar ataxia (LACA) have emerged to improve early treatment and management, while recent studies are exploring how autoimmunity affects synapses and nervous system function.
Paraneoplastic syndromes in patients with melanoma.
Postepy Dermatol Alergol
June 2024
Department of Dermatology and Vascular Anomalies, John Paul II Centre of Paediatrics, Sosnowiec, Poland.
Article Synopsis
- Paraneoplastic syndromes refer to rare conditions linked to malignant tumors, often caused by tumor-produced substances or autoimmune responses, with melanoma being a notable example associated with diverse systemic manifestations.
- Melanoma can lead to various paraneoplastic syndromes, including neurological and dermatological symptoms, with melanoma-associated retinopathy and neurological conditions like encephalitis being among the most common.
- Treatment mainly involves addressing the underlying melanoma, which may improve symptoms, along with possible immune-modulating therapies like corticosteroids and intravenous immunoglobulins for some cases; further research is needed to better understand these syndromes.
Significant improvement in paraneoplastic neurological syndromes without identifiable anti-neural antibodies in patients with breast cancer after breast surgery.
Int Cancer Conf J
July 2024
Department of Breast Surgery, Hokkaido University Hospital, Sapporo, Japan.
Article Synopsis
- Paraneoplastic neurological syndromes (PNS) are disorders linked to tumors, where anti-neural antibodies may be present, and cerebellar ataxia is a common example in breast cancer patients.
- Improvement in symptoms has typically been observed in patients with positive anti-neural antibodies, but this case highlights a 78-year-old woman whose neurological symptoms improved after surgery for breast cancer despite negative antibody tests.
- The patient’s ataxia was quantitatively assessed using the Scale for the Assessment and Rating of Ataxia (SARA), showing significant improvement post-surgery, suggesting that breast cancer treatment could benefit patients who are negative for anti-neural antibodies.
Paraneoplastic Cerebellar Degeneration Revealing Non-Small Cell Lung Cancer: A Case Report.
Cureus
May 2024
Department of Pulmonology, Research and Medical Sciences Laboratory, Faculty of Medicine and Pharmacy of Oujda, Mohammed VI University Hospital, Mohammed First University, Oujda, MAR.
Article Synopsis
- Paraneoplastic neurologic degeneration (PND) is a sudden or subacute neurological syndrome associated with underlying cancer, which can be either obvious or hidden.
- A specific type of PND, called subacute paraneoplastic cerebellar degeneration (PCD), is important but rare, and early diagnosis can improve patient outcomes for both neurological symptoms and cancer treatment.
- The text discusses a case study of a 61-year-old patient who was found to have subacute cerebellar degeneration, leading to the discovery of non-small cell lung carcinoma.
Multi-omics profiling reveals dysregulated ribosome biogenesis and impaired cell proliferation following knockout of CDR2L.
BMC Cancer
May 2024
Department of Clinical Medicine, University of Bergen, Bergen, Norway.
Article Synopsis
- - The study investigates cerebellar degeneration-related (CDR) proteins and their involvement in paraneoplastic cerebellar degeneration (PCD), a rare neurodegenerative disease caused by autoimmune responses to tumors, focusing specifically on the CDR2L protein in ovarian cancer.
- - Researchers employed RNA sequencing and mass spectrometry to analyze ovarian cancer cell lines lacking the CDR proteins (CDR1, CDR2, and CDR2L) and identified distinct changes in gene and protein expression linked to the absence of these proteins.
- - The findings revealed that knocking out CDR2L led to disrupted expression of genes related to ribosome function and cell cycle, resulting in decreased cell proliferation, thus offering new insights and
Immune Ataxias: The Continuum of Latent Ataxia, Primary Ataxia and Clinical Ataxia.
J Integr Neurosci
April 2024
Department of Medical Education, Tokyo Medical University, 160-0023 Tokyo, Japan.
Article Synopsis
- Immune-mediated cerebellar ataxias (IMCAs) have been recognized as a clinical category that arises from immune attacks on the cerebellum, which houses a significant number of brain neurons and various antigens.
- IMCAs manifest in acute or subacute phases and include conditions such as gluten ataxia, post-infectious cerebellitis, and anti-GAD ataxia, presenting symptoms like vestibulocerebellar and motor syndromes.
- Latent autoimmune cerebellar ataxia (LACA) serves as a precursor stage for patients who are asymptomatic or exhibit mild symptoms, potentially allowing for therapeutic intervention to maintain neuronal health before progressing to more severe autoimmune disorders.
18F-FDG PET/CT in assessment of paraneoplastic cerebellar degeneration as the first sign of occult fallopian tube serous cystadenocarcinoma: Case report.
Rofo
August 2024
Division of Molecular Imaging and Theranostics, Department of Nuclear Medicine, Paracelsus Medical University Salzburg, Salzburg, Austria.
Gynecologic clear cell carcinoma and paraneoplastic cerebellar Degeneration: A literature review and case study.
Gynecol Oncol Rep
April 2024
Division of Gynecologic Oncology, University of Kentucky Markey Comprehensive Cancer Center, 800 Rose Street, Lexington, KY 40536, United States.
• Paraneoplastic cerebellar degeneration (PCD) is rare condition associated with gynecologic malignancy. • PCD presents with progressive cerebellar dysfunction in the setting of malignancy and confers a poor neurologic prognosis. • PCD associated with ovarian clear cell cancer may have more favorable neurologic outcomes versus other histologies.
View Article and Find Full Text PDFOverview of treatment strategies in paraneoplastic neurological syndromes.
Handb Clin Neurol
March 2024
Department of Neurology, Erasmus Medical Center, Rotterdam, The Netherlands. Electronic address:
Treatment strategies in paraneoplastic neurological syndromes rely on the three pillars of tumor treatment, immunotherapy, and symptomatic treatment, the first one being by far the most important in the majority of patients and syndromes. Classically, antibodies against extracellular antigens are directly pathogenic, and patients with these syndromes are more responsive to immunomodulatory or immunosuppressive treatments than the ones with antibodies against intracellular targets. This chapter first discusses some general principles of tumor treatment and immunotherapy, followed by a closer look at specific treatment options for different clinical syndromes, focusing on symptomatic treatments.
View Article and Find Full Text PDFEpidemiology of paraneoplastic neurologic syndromes.
Handb Clin Neurol
March 2024
Department of Neurology, University of Utah, Salt Lake City, UT, United States; George E. Wahlen Department of Veterans Affairs Medical Center, Salt Lake City, UT, United States. Electronic address:
Article Synopsis
- Paraneoplastic neurologic syndromes (PNS) are rare, neurologic disorders linked to cancer that were first described in the early 20th century, with better understanding of their causes developed later on.
- Recent discoveries of new autoantigens have expanded the range of conditions associated with PNS, which may complicate the understanding of their incidence and underlying mechanisms.
- The increasing use of immunotherapy in cancer treatment is expected to raise the incidence of PNS, leading to a need for more detailed research on diverse patient populations to improve treatment and clinical trials.
Hematologic malignancies and hematopoietic stem cell transplantation.
Handb Clin Neurol
March 2024
Department of Medicine, Hematology Unit, University of Padova, Padova, Italy.
Article Synopsis
- Paraneoplastic neurologic syndromes are uncommon in blood cancers, but lymphomas frequently show these syndromes, often lacking high-risk antibodies except in specific cases like cerebellar degeneration and limbic encephalitis.* -
- Peripheral nervous system syndromes are rare but can occur in non-Hodgkin lymphoma, with POEMS being a noteworthy rare syndrome linked to plasma cell disorders; diagnosis relies on specific criteria and VEGF levels rather than antibodies.* -
- Treatment typically involves addressing the underlying cancer, and in cases like Ophelia syndrome/limbic encephalitis with anti-mGluR5 antibodies, patients may experience full recovery; hematopoietic stem-cell transplantation is preferred for POEMS syndrome.*
Paraneoplastic neurologic syndrome associated with gynecologic and breast malignancies.
Handb Clin Neurol
March 2024
French Reference Center for Paraneoplastic Neurological Syndromes, Hospices Civils de Lyon, Lyon, France; Synaptopathies and Autoantibodies (SynatAc) Team, Institut MeLis, Inserm U1314, UMR CNRS 5284, University Claude Bernard Lyon 1, Lyon, France.
Article Synopsis
- Gynecologic and breast cancers are commonly linked to paraneoplastic neurologic syndromes, particularly the Yo syndrome, which involves severe cerebellar ataxia in women, especially around their 60s.
- Diagnosis of Yo syndrome hinges on identifying Yo antibodies in the patient's serum or cerebrospinal fluid and always correlates with an underlying tumor, typically ovarian or breast cancer.
- While understanding the immune mechanisms behind these syndromes is improving, effective treatments remain limited, and other rarer syndromes related to these cancers exhibit unique immune and genetic characteristics.