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This case study describes the successful use of ravulizumab in treating a 71-year-old woman with myasthenia gravis experiencing a myasthenic crisis. The patient initially presented with hypernasality and dysphagia; her medical history included untreated, complicated type 1 diabetes. The patient received several treatments approved in Japan for general myasthenia gravis, including immunoadsorption plasmapheresis, tacrolimus, intravenous immunoglobulin, and intravenous methylprednisolone.
View Article and Find Full Text PDFSemin Respir Crit Care Med
December 2024
Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, Ohio.
Neuromuscular disorders can cause respiratory impairment by affecting the muscle fibers, neuromuscular junction, or innervation of respiratory muscles, leading to significant morbidity and mortality. Over the past few years, new disease-modifying therapies have been developed and made available for treating different neuromuscular disorders. Some of these therapies have remarkable effectiveness, resulting in the prevention and reduction of respiratory complications.
View Article and Find Full Text PDFClin Neurophysiol
December 2024
Suna and Inan Kıraç Foundation, Neurodegeneration Research Laboratory, NDAL-KUTTAM, School of Medicine, Koc University, Istanbul, Turkey. Electronic address:
Orphanet J Rare Dis
December 2024
Department of Neurology, ERN EURO-NMD Center, University Hospital Brno, Brno, Czech Republic.
Background: Myasthenia gravis (MG) is a rare autoimmune disorder with significant clinical implications, including life-threatening myasthenic crises and exacerbations. Understanding real-world treatment patterns, especially associated direct medical costs, is essential for the effective management of healthcare delivery.
Methods: We conducted a descriptive cohort study using health administrative claims data from the Czech Republic covering more than 1,500 prevalent MG patients.
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.
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