Efgartigimod improves muscle weakness in a mouse model for muscle-specific kinase myasthenia gravis.

In conclusion, our study shows that efgartigimod has clear therapeutic potential in MuSK myasthenia gravis and forms an exciting candidate drug for many autoantibody-mediated neurological and other disorders. PMID: 30851266 [PubMed - as supplied by publisher]
Source: Experimental Neurology - Category: Neurology Authors: Tags: Exp Neurol Source Type: research

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Non-dystrophic myotonias are a rare group of neuromuscular disorders caused by variants in skeletal muscle sodium (SCN4A) or chloride (CLCN1) channels genes. Variants in SCN4A are associated with different phenotypes such as paramyotonia congenita, hyper- or hypokalemic periodic paralysis, sodium channel myotonia but also with congenital myasthenic syndromes and congenital myopathies [1, 2]. Clinical symptoms are mainly characterized by episodes with muscle stiffness or paralysis triggered by exercise, cold, infections or fasting [2, 3].
Source: Neuromuscular Disorders - Category: Neurology Authors: Tags: Case report Source Type: research
Authors: McCarter SJ, Burkholder DB, Klaas JP, Martinez-Thompson JM, Boes CJ Abstract Mary Broadfoot Walker (1888-1974) was the first to demonstrate the 'Mary Walker effect' describing the weakness of other muscle groups following release of the arteriovenous occlusion of an unrelated exercising muscle group in patients with myasthenia gravis, which led to the search for a circulating causative agent for myasthenia gravis. She was the first to clearly demonstrate that strength temporarily improved in patients with myasthenia gravis with physostigmine or Prostigmin (neostigmine). This dramatic treatment response has...
Source: Journal of the Royal College of Physicians of Edinburgh - Category: General Medicine Tags: J R Coll Physicians Edinb Source Type: research
Myasthenia gravis (MG) is a rare and treatable antibody-mediated autoimmune disease. Pseudo internuclear ophthalmoplegia (-INO) or pyramidal tract damage is rarely observed in MG, and there were no known cases of MG with both pseudo-INO and pyramidal tract damage. Here, we report a case of a 61-year-old female suffering from MG accompanied by pseudo-INO and pyramidal tract damage with a rapid progressive course. Her blood and cerebrospinal fluid (CSF) tests were normal, except for the presence of the anti-acetylcholine receptor antibody. CT and contrast enhancement of the chest showed a thymic involution. MRI and contrast ...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Extended thymectomy is employed for patients with myasthenia gravis (MG) and/or thymoma with elevated serum antiacetylcholine receptor antibody (AchR) titers. However, MG symptoms occasionally worsen in postthymectomy patients. We explored the risk factors for exacerbation of MG symptoms after surgical therapy for patients with MG and/or thymoma with an elevated AchR titer. We retrospectively analyzed 90 patients suffering from MG and/or thymoma with an elevated AchR titer who underwent thymectomy in our institute.
Source: Seminars in Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: THORACIC – Original Submission Source Type: research
This study aimed to evaluate the impact of tumor location on the clinicopathological features and prognosis of thymomas.MethodsWe performed a retrospective review of patients at our institution who underwent an extended thymectomy for a thymoma from 1976 to 2015. The tumor location was classified as either the superior or inferior mediastinum based on the maximum tumor diameter. The clinicopathological characteristics of the thymoma were also evaluated. Kaplan ‐Meier estimates and Cox proportional hazards models were used to analyze the survival outcomes and risk factors for recurrence.ResultsA total of 194 patients with...
Source: Thoracic Cancer - Category: Cancer & Oncology Authors: Tags: ORIGINAL ARTICLE Source Type: research
New research finds biomarkers for myasthenia gravis, an autoimmune condition, that may help diagnose it and create individualized treatments in the future.
Source: Health News from Medical News Today - Category: Consumer Health News Tags: Medical Devices / Diagnostics Source Type: news
We report a case of our experience and review the current literature of MG exacerbated or occurring during immunotherapy to describe characteristics of this AE, warn the oncologist about this toxicity, and summarize the treatments conducted. Thirty-four cases of MG were reported, mostly with anti-programmed cell death protein 1 checkpoint inhibitor, and with melanoma. Onset was quick after the first or second infusion. Treatment comprised corticosteroids, prostigmine, and more or less plasmapheresis or immunoglobulins. Prognosis is poor, as 13 patients died after MG. MG is a rare immune-related AE that must be rapidly evok...
Source: Journal of Immunotherapy - Category: Allergy & Immunology Tags: Clincal Studies Source Type: research
Myasthenia gravis (MG) is an autoimmune disorder caused by antibodies targeting the neuromuscular junction, which in most cases are directed towards the skeletal muscle acetylcholine receptor (AChR) [1]. The pathophysiology of MG is accepted to be immune mediated [2]. Sporadic inclusion body myositis (IBM) is considered the most common inflammatory myopathy in patients over 50 years old, but its pathophysiology remains to this day an enigma: It is still unclear whether it is a primary degenerative disease with secondary dysimmune reaction or vice versa [3].
Source: Neuromuscular Disorders - Category: Neurology Authors: Tags: Case report Source Type: research
Conclusions: Rituximab is both safe and effective for the treatment of acquired angioedema with C1-inhibitor deficiency, ANCA-associated vasculitis, autoimmune hemolytic anemia, Behçet’s disease, bullous pemphigoid, Castleman’s disease, cryoglobulinemia, Goodpasture’s disease, IgG4-related disease, immune thrombocytopenia, juvenile idiopathic arthritis, membraneous nephropathy, relapsing-remitting multiple sclerosis, myasthenia gravis, nephrotic syndrome, neuromyelitis optica, pemphigus, rheumatoid arthritis, spondyloarthropathy, and systemic sclerosis. Conversely, rituximab failed to show an effec...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
The objective of this study is to determine the efficacy and safety of SCIg in the treatment of adult patients with myasthenia gravis. Relevant records were identified from August 2018 to January 2019 systematic search. Five relevant articles with a total of 34 patients with MG were included in this review. Data on functional disability score and adverse events were obtained. Based on the included uncontrolled studies, the functional disability scores of adult MG patients after SCIg administration showed consistent improvement. Headache and local site injection reactions were the most common adverse events reported. The ev...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
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