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      Outcomes and characteristics of myasthenia gravis: A 10-year retrospective cross-sectional study at King Fahad Medical City

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      , MD, , MD , , MD, , MD
      Neurosciences
      Riyadh : Armed Forces Hospital

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          Abstract

          Objectives:

          To investigate the distribution of muscle weakness in Myasthenia gravis (MG) and the therapeutic response in each category.

          Methods:

          This is a retrospective cross-sectional study included all MG patients presented to our clinic between 2010 and 2020. The demographic, clinical, serological, electrophysiological, radiological, and histopathological data of the patients were recorded. The details of the treatment administered were also documented. Muscle weakness was divided into: ocular, bulbar, and generalized.

          Results:

          The mean age of the 147 patients included in this study was 34.2±16.6 years. The most common presentation was ocular MG (57.1%). There was no significant association between the gender of the patients and the MG subgroups. Antibodies against AChR were reported in 95.2%, 75%, and 87% of the patients with ocular, bulbar, and generalized myasthenia, respectively. Anti-MuSK antibodies were detected in 20% of the patients with bulbar weakness. Most of the patients with ocular (91.7%) and bulbar (90%) presentation developed generalized weakness. At the end of the follow-up, 82.6%, 70.2%, and 57.5% of the patients with generalized, ocular, and bulbar presentations, respectively demonstrated well-controlled weakness.

          Conclusion:

          The most common initial presentation was ocular weakness. Most patients with ocular and bulbar presentation developed generalized weakness during the follow up period. The most frequently reported autoantibody was against AChR. Most patients with generalized, ocular, and bulbar presentation demonstrated well-controlled weakness at the end of the follow up period.

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          Most cited references42

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          Mammalian nicotinic acetylcholine receptors: from structure to function.

          The classical studies of nicotine by Langley at the turn of the 20th century introduced the concept of a "receptive substance," from which the idea of a "receptor" came to light. Subsequent studies aided by the Torpedo electric organ, a rich source of muscle-type nicotinic receptors (nAChRs), and the discovery of alpha-bungarotoxin, a snake toxin that binds pseudo-irreversibly to the muscle nAChR, resulted in the muscle nAChR being the best characterized ligand-gated ion channel hitherto. With the advancement of functional and genetic studies in the late 1980s, the existence of nAChRs in the mammalian brain was confirmed and the realization that the numerous nAChR subtypes contribute to the psychoactive properties of nicotine and other drugs of abuse and to the neuropathology of various diseases, including Alzheimer's, Parkinson's, and schizophrenia, has since emerged. This review provides a comprehensive overview of these findings and the more recent revelations of the impact that the rich diversity in function and expression of this receptor family has on neuronal and nonneuronal cells throughout the body. Despite these numerous developments, our understanding of the contributions of specific neuronal nAChR subtypes to the many facets of physiology throughout the body remains in its infancy.
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            Myasthenia gravis: subgroup classification and therapeutic strategies.

            Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated myasthenia gravis might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. Pyridostigmine is the preferred symptomatic treatment, and for patients who do not adequately respond to symptomatic therapy, corticosteroids, azathioprine, and thymectomy are first-line immunosuppressive treatments. Additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies. Long-term drug treatment is essential for most patients and must be tailored to the particular form of myasthenia gravis.
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              A systematic review of population based epidemiological studies in Myasthenia Gravis

              Background The aim was to collate all myasthenia gravis (MG) epidemiological studies including AChR MG and MuSK MG specific studies. To synthesize data on incidence rate (IR), prevalence rate (PR) and mortality rate (MR) of the condition and investigate the influence of environmental and technical factors on any trends or variation observed. Methods Studies were identified using multiple sources and meta-analysis performed to calculate pooled estimates for IR, PR and MR. Results 55 studies performed between 1950 and 2007 were included, representing 1.7 billion population-years. For All MG estimated pooled IR (eIR): 5.3 per million person-years (C.I.:4.4, 6.1), range: 1.7 to 21.3; estimated pooled PR: 77.7 per million persons (C.I.:64.0, 94.3), range 15 to 179; MR range 0.1 to 0.9 per millions person-years. AChR MG eIR: 7.3 (C.I.:5.5, 7.8), range: 4.3 to 18.0; MuSK MG IR range: 0.1 to 0.32. However marked variation persisted between populations studied with similar methodology and in similar areas. Conclusions We report marked variation in observed frequencies of MG. We show evidence of increasing frequency of MG with year of study and improved study quality. This probably reflects improved case ascertainment. But other factors must also influence disease onset resulting in the observed variation in IR across geographically and genetically similar populations.
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                Author and article information

                Journal
                Neurosciences (Riyadh)
                nsj
                Neurosciences
                Neurosciences
                Riyadh : Armed Forces Hospital
                1319-6138
                1319-6138
                October 2022
                : 27
                : 4
                : 237-243
                Affiliations
                From the Department of Neurology (Ali, Alenazi), King Fahad Medical City, Riyadh, from the Department of Medicine (Almalki, Kotb), College of Medicine, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia, and from the Department of Neurology (Kotb), Faculty of Medicine, Minia University, Minia, Egypt
                Author notes
                Address correspondence and reprint request to: Dr. Daifallah M. Almalki, Department of Medicine, College of Medicine, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia. E-mail: dr_daifullah@ 123456hotmail.com ORCID ID: https://orcid.org/0000-0002-9498-6303
                Article
                Neurosciences-27-4-237
                10.17712/nsj.2022.4.20220038
                9749576
                36252965
                6e036841-0916-40e2-bc5f-2a9217b8d378
                Copyright: © Neurosciences

                Neurosciences is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work.

                History
                : 7 April 2022
                : 6 July 2022
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