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http://dx.doi.org/10.1136/jnnp-2020-325569 | DOI Listing |
Cureus
December 2024
Department of Medicine, Division of Endocrinology and Metabolism, King Abdulaziz Medical City, Riyadh, SAU.
Epithelioid hemangioendothelioma (EHE) is a rare form of vascular neoplasm that can manifest with various symptoms or be discovered incidentally in asymptomatic patients. In this report, we describe a case of a 56-year-old male who presented with progressive lower limb weakness over four years. The evaluation revealed severe hypophosphatemia, an inappropriately normal fibroblast growth factor 23 C-terminal (cFGF-23) level, and a 30 x 20 mm hypermetabolic right pleural mass, which was subsequently proven to be EHE.
View Article and Find Full Text PDFJ Peripher Nerv Syst
March 2025
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Background And Aims: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is difficult to distinguish from mimicking disorders, with misdiagnosis resulting in IVIG overutilization. We evaluate a clinical-electrophysiological model to facilitate CIDP versus mimic neuropathy prediction.
Methods: Using the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 CIDP guidelines we derived 26 clinical and 144 nerve conduction variables.
Semin Arthritis Rheum
December 2024
Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Rheumatology and Clinical Immunology, Istituti di Ricovero e Cura a Carattere Scientifico (IRCCS) Humanitas Research Hospital, Rozzano, Italy.
Objective: Older age, dermatomyositis, and specific serum autoantibodies such as anti-TIF1-γ are associated with higher cancer risk in patients with myositis. We evaluated a vast cohort of patients with myositis for the prevalence of cancer, the association to disease features, and the performance of the recent IMACS guidelines.
Methods: A retrospective cohort analysis was performed and in all cases serum autoantibodies were tested using HEp-2, immunoassays, RNA- and protein-immunoprecipitation.
Int J Mol Sci
December 2024
Cerebro, Emoción y Conducta, School of Medicine, Universidad de las Américas (UDLA), Quito 170124, Ecuador.
Paraneoplastic cerebellar degeneration (PCD) is a rapidly progressive, immune-mediated syndrome characterized by the degeneration of Purkinje cells, often associated with the presence of antibodies targeting intracellular antigens within these cells. These autoantibodies are implicated in the induction of cytotoxicity, leading to Purkinje cell death, as demonstrated in in vitro models. However, the precise roles of antibodies and T lymphocytes in mediating neuronal injury remain a subject of ongoing research, with T cells appearing to be the main effectors of cerebellar injury.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
December 2024
Division of Cardiothoracic Surgery, University of California, San Diego, California.
Thymomas have been associated with the generation of paraneoplastic autoantibodies to neurogenic epitopes, collapsin-response-mediator protein-5 receptor (CRMP-5) and alpha-amino-3-hydroxyl-5methyl-4isoxazolepropionic acid receptor (AMPAR), in patients with acute viral infection. We report a patient with thymoma and myasthenia gravis, with SARS-CoV-2 infection, who became comatose secondary to autoimmune encephalitis. Plasmapheresis, high-dose steroids, pyridostigmine, eculizumab, and rituximab did not restore neurologic function.
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