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A clinical perspective on muscle specific kinase antibody positive myasthenia gravis.
Front Immunol
December 2024
Department of Neurology, Medical University of Vienna, Vienna, Austria.
The discovery of autoantibodies directed against muscle-specific kinase (MuSK) in "seronegative" myasthenia gravis (MG) patients marked a milestone in MG research. In healthy muscle, MuSK regulates a phosphorylation pathway, which is essential for the development and maintenance of acetylcholine receptor (AChR) clusters at the neuromuscular junction. Autoantibodies directed against MuSK are predominantly of the IgG4 subclass, but there is increasing evidence that IgG1-3 could also contribute to the pathology underlying MuSK-MG.
View Article and Find Full Text PDFSurgical treatment of thymic epithelial tumor and myasthenia gravis.
Front Surg
November 2024
Department of Thoracic Surgery, Istanbul University-Cerrahpaşa, Cerrahpaşa Medical Faculty, Istanbul, Türkiye.
Article Synopsis
- * Thymomas grow slowly and are localized, while thymic carcinomas grow quickly and can spread, with respective 5-year survival rates of 78% and 30%.
- * Treatment depends on tumor resectability, starting with surgery for early-stage cases, followed by chemotherapy for more advanced stages, while conditions like myasthenia gravis are associated with these tumors and can be treated through various methods, including extended thymectomy.
Heavy duty.
Surv Ophthalmol
November 2024
Ratner Children's Eye Center Within the Viterbi Family Department of Ophthalmology, University of California San Diego, La Jolla, CA, USA.
A 54-year-old woman with myopia, hypothyroidism, and asthma presented with intermittent double vision. Serum was positive for anti-low density lipoprotein receptor-related protein 4 (LRP4) antibodies suggesting the diagnosis of myasthenia gravis. There was no improvement with pyridostigmine, prednisone, or azathioprine.
View Article and Find Full Text PDFLipoprotein Receptor-Related Protein 4 Antibody Positivity in the Youngest Patient in the Caucasus Region: A Case Report.
Cureus
September 2024
Pediatric Neurology, Güven Hospital, Ankara, TUR.
Juvenile myasthenia gravis is a rare disorder where antibodies targeting the acetylcholine receptor or, less frequently, muscle-specific kinase can be detected in the serum while about half of the patients can be seronegative. A pediatric patient with ocular myasthenia is presented whose serum was negative for acetylcholine receptor and muscle-specific kinase antibodies but tested positive for low-density lipoprotein receptor-related protein 4 antibodies. A favourable clinical response was observed to medical treatment with pyridostigmine and prednisolone, as expected in isolated ocular juvenile myasthenia gravis.
View Article and Find Full Text PDFPrevalence, Incidence, and Mortality of Myasthenia Gravis and Myasthenic Syndromes: A Systematic Review.
Neuroepidemiology
October 2024
Department of Epidemiology, Lazio Regional Health Service, Rome, Italy.
Article Synopsis
- A systematic review was conducted to estimate the global prevalence, incidence, and mortality rates of myasthenia gravis (MG) and related syndromes, due to a gap in recent investigations.
- The review analyzed 94 studies published from 1952 to 2022, finding a mean prevalence of 173.3 cases per million, a mean incidence of 15.7 cases per million person-years, and a mean mortality rate of 1.4 cases per million person-years, with acetylcholine receptor (AChR)-MG being the most common subtype.
- The review highlighted that the significant increase in reported MG cases may stem from enhanced research methods and diagnostic tools, though variations in MG frequencies exist due to
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