Preliminary Findings of a Dedicated Ocular Myasthenia Gravis Rating Scale: The OMGRate

Source: Neuro-Ophthalmology - Category: Opthalmology Authors: Source Type: research

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Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction. Approximately 9% of MG patients have autoantibodies targeting the muscle specific kinase (MuSK), and are challenging therapeutically, since they often present with more severe symptoms. A useful therapy is plasmapheresis, but it is highly non-specific. Antigen-specific immunoadsorption would only remove the pathogenic autoantibodies, minimizing the possible side effects and maximizing the benefit. We used rats with human MuSK-induced experimental autoimmune MG to perform antigen-specific immunoadsorptions, and found it very effective, res...
Source: Journal of Neuroimmunology - Category: Allergy & Immunology Authors: Source Type: research
ConclusionA small subgroup of patients with generalized myasthenia gravis do not respond sufficiently to standard therapies. Refractory disease has considerable implications for both patients and health care providers and highlights an unmet need for new treatment options.
Source: Journal of Neurology - Category: Neurology Source Type: research
We describe 2 children from unrelated families who presented with hypotonia, ptosis and fatigability in early infancy with anti-AChR antibodies detected via ELISA on 2 separate occasions in the sera. Both were treated as refractory autoimmune MG due to poor clinical response to acetylcholinesterase inhibitor and immunotherapy. In view of the atypical clinical features, genetic studies of CMS were performed and both were confirmed to have novel pathogenic mutations in the COLQ gene. To the best of our knowledge, the presence of anti-AChR antibody in COLQ-related CMS has never been reported in the literature. The clinical pr...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
Immune checkpoint inhibitors (ICIs) can cause adverse reactions in the nervous system. The incidence rate is 0.1% –12% and 80% of nervous system adverse reactions occur within the first four months of application. ICIs can cause diseases of various parts of the nervous system including central nervous system diseases such as aseptic meningitis, meningeal encephalitis, necrotizing encephalitis, brainstem encep halitis, transverse myelitis, etc., and peripheral neuropathy such as cranial nerve peripheral neuropathy, multifocal nerve root neuropathy, Guillain‐Barré syndrome, spinal nerve root neuropathy, myasth...
Source: Thoracic Cancer - Category: Cancer & Oncology Authors: Tags: CLINICAL GUIDELINE Source Type: research
We thank Finsterer and associates for their interest in our study and for giving valuable comments.
Source: American Journal of Ophthalmology - Category: Opthalmology Authors: Tags: Correspondence Source Type: research
Juvenile myasthenia gravis (JMG) is an antibody mediated autoimmune disorder that manifests as progressive voluntary muscle weakness and fatigue. In medically refractory cases, thymectomy has been shown to abrogate symptoms and reduce glucocorticoid dependence. While transcervical or transsternal incisions have been the traditional approach, adult trends now favor thoracoscopic thymectomy. Little data exist to support this approach in children.
Source: Journal of Pediatric Surgery - Category: Surgery Authors: Source Type: research
Complement-dependent disruption of motor endplate is detected in anti-acetylcholine receptor (AChR) antibody-positive myasthenia gravis (MG). We measured serum AChR α1 subunit protein levels, which may be associated with neuromuscular damage, in 55 patients with MG (47 were seropositive and 8 were negative) and in 20 controls. Serum AChR α1 subunit protein concentrations were higher in patients with anti-AChR antibody-positive MG than those in controls (P =  .04), were negatively correlated with MG activities of daily living score (P = .01), and tended to be higher in ocular MG than in generalized MG.
Source: Journal of Neuroimmunology - Category: Allergy & Immunology Authors: Tags: Short Communication Source Type: research
Authors: Popa GA, Tomulescu V, Popescu I, Herlea V, Lupescu IG Abstract Thymic epithelial tumors have been traditionally classified into two main types: noninvasive and invasive thymoma. Several classifications have been proposed for thymic tumors, but according to these classifications, the prognosis of patients with thymomas varies considerably. Our purpose is to present different CT aspects according to various subtypes of thymic epithelial neoplasms based on the simplified World Health Organization classification. In this article, we will discuss and illustrate histologic and functional features of the thymus a...
Source: Journal of Medicine and Life - Category: General Medicine Tags: J Med Life Source Type: research
CONCLUSION: It was a 100% correlation between CT examinations and intra-operative findings regarding the presents of focal thymic masses in our study. In the diagnostic of focal thymic mass, the only inter-disciplinary correlation is between radiological and macroscopic aspects. CT examination has a limited value in differential diagnosis between thymic lymphoid follicular hyperplasia and thymoma. PMID: 31803292 [PubMed]
Source: Journal of Medicine and Life - Category: General Medicine Tags: J Med Life Source Type: research
Conclusions about the regulation of the MuSK kinase that were derived from molecular structures will be highlighted. In addition, the role of MuSK during development and disease will be discussed.
Source: Neuroscience Letters - Category: Neuroscience Source Type: research
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