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Rituximab in juvenile myasthenia gravis-an international cohort study and literature review.
Eur J Paediatr Neurol
September 2022
Department of Paediatric Neurology, Neuromuscular Service, Evelina's Children Hospital, Guy's and St Thomas' Hospital NHS Foundation Trust, London, UK; Randall Centre for Cell and Molecular Biophysics, Muscle Signalling Section, FoLSM, King's College, London, UK. Electronic address:
Article Synopsis
- - Juvenile myasthenia gravis (JMG) is a rare autoimmune disorder affecting the neuromuscular junction, and treatment is generally based on adult myasthenia gravis (MG) protocols.
- - A study from five centers found that 10 JMG patients treated with rituximab showed reduced hospital admissions, with 60% achieving remission after 24 months, and 7 patients reduced their immunomodulatory treatments.
- - The main side effect was infusion-related reactions in 30% of patients, but these were manageable; overall, rituximab appears to be a safe and effective treatment for moderate to severe JMG.
Myasthenia Gravis-An Analysis of Multimodal Evoked Potentials.
Brain Sci
August 2021
Department of Neurology, Wroclaw Medical University, 50-556 Wroclaw, Poland.
Introduction: The aim of this study is a comprehensive analysis of the parameters of exogenous evoked potentials (visual, brainstem auditory, and somatosensory) in patients with myasthenia gravis (MG), a prototype of both neuromuscular junction disease and autoimmune disease. The study also seeks to isolate electrophysiological changes that may indicate disorders within the central and/or peripheral nervous system.
Methods: A total of forty-two consecutive patients with myasthenia gravis (24 women, 18 men) were included in the study.
Myasthenia gravis--an unexpected cause of respiratory failure and reversible left ventricular dysfunction after cardiac surgery.
J Card Surg
November 2010
Department of Cardiothoracic Surgery, Naval Medical Center Portsmouth, Portsmouth, Virginia 23708, USA.
We report a case of postpericardiotomy myasthenia gravis. A 68-year-old male patient without prior history of neuromuscular or autoimmune disorders presented with respiratory failure and severe left ventricular dysfunction four weeks after mitral valve replacement. Markedly elevated acetylcholine receptor antibodies were noted, and the patient responded promptly to immunologic therapy.
View Article and Find Full Text PDFCCL2 recruitment of IL-6-producing CD11b+ monocytes to the draining lymph nodes during the initiation of Th17-dependent B cell-mediated autoimmunity.
Eur J Immunol
July 2008
Institute of Neuroinformatics and Laboratory for Brain and Mind, Dalian University of Technology, Dalian, China.
The development and function of Th17 cells are influenced in part by the cytokines TGF-beta, IL-23 and IL-6, but the mechanisms that govern recruitment and activity of Th17 cells during initiation of autoimmunity remain poorly defined. We show here that the development of autoreactive Th17 cells in secondary lymphoid organs in experimental autoimmune myasthenia gravis--an animal model of human myasthenia gravis--is modulated by IL-6-producing CD11b(+) cells via the CC chemokine ligand 2 (CCL2). Notably, acetylcholine receptor (AChR)-reactive Th17 cells provide help for the B cells to produce anti-AChR antibodies, which are responsible for the impairment of the neuromuscular transmission that contributes to the clinical manifestations of autoimmunity, as indicated by a lack of disease induction in IL-17-deficient mice.
View Article and Find Full Text PDF[Myasthenia gravis--an autoimmune neuromuscular disease].
Lakartidningen
October 2000
Myasthenia gravis Centrum, neurologiska kliniken, Karolinska sjukhuset, Stockholm.
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