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Myasthenia gravis: State of the art and new therapeutic strategies.

Published on Oct 15, 2019in Journal of Neuroimmunology2.832
· DOI :10.1016/j.jneuroim.2019.577080
Eliana B. Souto46
Estimated H-index: 46
(UC: University of Coimbra),
Bernardo Lima1
Estimated H-index: 1
(UC: University of Coimbra)
+ 3 AuthorsAmélia M. Silva23
Estimated H-index: 23
(UTAD: University of Trás-os-Montes and Alto Douro)
Abstract
Abstract Myasthenia Gravis (MG) – an autoimmune neuromuscular disease – is known by the production of autoantibodies against components of the neuromuscular junction mainly to the acetylcholine receptor, which cause the destruction and compromises the synaptic transmission. This disease is characterized by fluctuating and fatigable muscle weakness, becoming more intensive with activity, but with an improvement under resting. There are many therapeutic strategies used to alleviate MG symptoms, either by improving the transmission of the nerve impulse or by ameliorating autoimmune reactions with e.g. steroids, immunosuppressant drugs, or monoclonal antibodies (rituximab and eculizumab). Many breakthroughs in the discovery of new therapeutic targets have been reported, but MG remains to be a chronic disease where the symptoms are kept in the majority of patients. In this review, we discuss the different therapeutic strategies that have been used over the years to alleviate MG symptoms, as well as innovative therapeutic approaches currently under study.
  • References (136)
  • Citations (1)
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References136
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Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ). Autoantibodies target key molecules at the NMJ, such as the nicotinic acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4), that lead by a range of different pathogenic mechanisms to altered tissue architecture and reduced densities or functionality of AChRs, reduced neuromuscular transmission, and therefore a severe fatigable skeletal muscle...
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Abstract This document is the consensus of international experts on the current status of Single Fiber EMG (SFEMG) and the measurement of neuromuscular jitter with concentric needle electrodes (CNE – CN-jitter). The panel of authors was chosen based on their particular interests and previous publications within a specific area of SFEMG or CN-jitter. Each member of the panel was asked to submit a section on their particular area of interest and these submissions were circulated among the panel me...
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Abstract The accuracy of the ice-pack test for diagnosing myasthenia gravis (MG) has been reported to be excellent, however, the sensitivity is low in patients with mild ptosis which is common in Asians who have absent or low double eyelid folds. Herein, we performed the ice-pack test after sustained upgaze and tested its validity for diagnosing MG. The study included 30 newly diagnosed MG patients with ptosis (median age 47 years, range 2–87 years) and 30 controls with ptosis other than MG (med...
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Myasthenia gravis is a rare, heterogeneous, classical autoimmune disease characterized by fatigable skeletal muscle weakness, which is directly mediated by autoantibodies targeting various components of the neuromuscular junction, including the acetylcholine receptor, muscle specific tyrosine kinase, and lipoprotein-related protein 4. Subgrouping of myasthenia gravis is dependent on the age of onset, pattern of clinical weakness, autoantibody detected, type of thymic pathology, and response to i...
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The humanized monoclonal antibody eculizumab (Soliris®) is a complement inhibitor indicated for use in anti-acetylcholine receptor (AChR) antibody-positive adults with generalized myasthenia gravis (gMG) in the USA, refractory gMG in the EU, or gMG with symptoms that are difficult to control with high-dose IVIg therapy or PLEX in Japan. It is the first complement inhibitor to be approved for use in these patients. In the well-designed, 26-week REGAIN study in patients with anti-AChR-positive ref...
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#1Lin Wang (PRC: China Medical University (PRC))H-Index: 1
#2Lijuan Zhang (PRC: China Medical University (PRC))H-Index: 1
Myasthenia gravis (MG) is a rare acquired autoimmune neuromuscular disease. Autoantibodies, cellular immunity, complement, and cytokines are involved in the pathogenesis of MG. It is characterized by the dysfunction of neuromuscular junction transmission and skeletal muscle weakness. MicroRNAs (miRNAs) are non-coding small molecule ribonucleic acids that regulate various biological processes (e.g., development, differentiation, and immunity) at the transcriptional and post-transcriptional levels...
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Resume La myasthenie auto-immune (MAI) est la plus frequente des pathologies de la jonction neuromusculaire. L’antigene cible le plus frequent est le recepteur a l’acetylcholine (RAch) avec la presence d’anticorps anti-RAch chez 70 a 80 % des patients avec myasthenie generalisee. L’identification d’autres auto-anticorps tels que les anti-MuSK (muscle specific tyrosine kinase) et les anti-LRP4 (low density lipoporotein receptor-related protein 4) a defini de nouvelles formes de MAI. D’autres auto...
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