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      Clinical Presentation, Management, and Outcome in Patients With Myasthenia Gravis: A Retrospective Study From Two Tertiary Care Centers in Saudi Arabia

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          Abstract

          Introduction: A limited number of research studies were published to delineate the clinical pattern of myasthenia gravis in Saudi Arabia. This paper is an attempt to describe some of the clinical aspects related to this disease in two large centers from two main cities in Saudi Arabia.

          Methods: A retrospective multi-center observational study of patients diagnosed with myasthenia gravis was conducted. The study setting was King Abdulaziz Medical City in Jeddah and Riyadh, Saudi Arabia. The study period was 12 years, starting from January 2007 to May 2019.

          Results: A total of 144 patients were included in this study (60 males and 84 females). The most common symptoms at diagnosis were ocular symptoms in 118 patients (81.9%), diplopia in 84 patients (58.3%), and/or blurred vision in 30 patients (20.8%). The majority had positive anti-acetylcholine receptor antibodies (72.2%). Pyridostigmine was the most prescribed medication for 136 patients (94.4%). Immunosuppressive medications were prescribed for 114 patients (79.2%). Around 40% of patients had exacerbations, and approximately 20% were admitted to the ICU. Thymectomy was performed for 97 patients (67.4%).

          Conclusion: The present study indicates that the clinical presentation and management of myasthenia gravis remained the same in the last few years despite the introduction of new modalities of diagnosis such as the anti-muscle-specific kinase (anti-MuSK) and other autoantibodies tests. Furthermore, we observed that the number of exacerbations and ICU admission were high, which may indicate inadequate therapy. We are stressing the need for establishing specialized neuromuscular clinics with neurologists trained in neurophysiology to improve the diagnostic accuracy and outcomes for patients with myasthenia gravis. 

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          Myasthenia gravis

          Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thymectomy and immunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.
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            Myasthenia gravis: Association of British Neurologists' management guidelines.

            Myasthenia gravis is an autoimmune disease of the neuromuscular junction for which many therapies were developed before the era of evidence based medicine. The basic principles of treatment are well known, however, patients continue to receive suboptimal treatment as a result of which a myasthenia gravis guidelines group was established under the aegis of The Association of British Neurologists. These guidelines attempt to steer a path between evidence-based practice where available, and established best practice where evidence is unavailable. Where there is insufficient evidence or a choice of options, the guidelines invite the clinician to seek the opinion of a myasthenia expert. The guidelines support clinicians not just in using the right treatments in the right order, but in optimising the use of well-known therapeutic agents. Clinical practice can be audited against these guidelines.
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              Myasthenia gravis and the neuromuscular junction.

              Myasthenic disorders are a well characterized group of diseases of the neuromuscular junction. Their pathogenesis is diverse, including genetic and autoimmune mechanisms. We review recent findings on risk factors, pathogenesis and treatment of autoimmune myasthenia gravis. Better knowledge of congenital myasthenia has led to the development of efficient diagnostic algorithms that have therapeutic implications. New epidemiological and genetic risk factors have been identified and are considered to play a role in the development of myasthenia gravis. The study of the role of innate immunity in myasthenia gravis has identified relevant pathways to explain myasthenia gravis causes. The description of the pathogenic role of IgG4 anti-MuSK antibodies has revealed heterogeneous immune mechanisms that should lead to more specific therapies. Rituximab seems to be particularly effective in MuSK myasthenia gravis, and eculizumab arises as an option in refractory AChR myasthenia gravis. Therapeutic algorithms need to be tailored to each myasthenia subtype. Increasing knowledge about the environmental and genetic risk factors and basic immunopathogenesis of myasthenia gravis, including the role of innate immunity, regulatory T cell impairment and autoantibody heterogeneity, is providing a rationale for treatment with new biological agents. Current immunotherapies in myasthenia gravis undoubtedly provide benefits, but also cause side-effects. Controlled trials are, therefore, needed to confirm initial results from pilot studies.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                27 December 2021
                December 2021
                : 13
                : 12
                : e20765
                Affiliations
                [1 ] Department of Medicine, Neurology Section, King Abdulaziz Medical City, Jeddah, SAU
                [2 ] College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, SAU
                [3 ] Research Office, King Abdullah International Medical Research Center, Jeddah, SAU
                [4 ] Department of Neuroscience, King Faisal Specialist Hospital and Research Centre, Jeddah, SAU
                [5 ] Neuromuscular Integrated Practice Unit, Neuroscience Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, SAU
                Author notes
                Article
                10.7759/cureus.20765
                8794400
                35111450
                14621c28-15c9-4103-90ee-1f8d4a9a0b69
                Copyright © 2021, Algahtani et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 27 December 2021
                Categories
                Neurology

                thymectomy,clinical features,disease course,clinical pattern,myasthenia gravis

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