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      Clinical Profile and Outcome of Postthymectomy versus Non-Thymectomy Myasthenia Gravis Patients in the Philippine General Hospital: A 6-Year Retrospective Study

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          Abstract

          Background

          Myasthenia gravis is an autoimmune neuromuscular disorder characterized by the production of abnormal autoantibodies directed against the receptors present in the neuromuscular junction. It has been the standard practice to offer thymectomy in all generalized myasthenia gravis patients despite the lack of robust evidence.

          Objectives

          The objectives of this study are to describe the clinical profile and differentiate the clinical outcomes of thymectomy versus non-thymectomy and thymomatous versus non-thymomatous myasthenia gravis patients in the Philippine General Hospital.

          Methodology

          Between 2009 and 2014, a total of 69 postthymectomy and 16 non-thymectomy patient records were successfully retrieved. The demographic characteristics, surgical approach, and histopathologic results were obtained. The clinical outcome after 6 months or 1 year-follow-up was also determined and grouped according to the following: (1) complete remission, (2) pharmacological remission, (3) no clinical change, (4) worsening symptoms, and (5) mortality.

          Results

          Majority of the patients were females (68.0%) with a mean age of 39.8 years and a mean duration of myasthenic symptoms of 21 months. Using the Myasthenia Gravis Foundation of America classification, 54.1% of patients fell under Class II and 48.2% of them presented with generalized weakness. In this study, 60.8% of postthymectomy myasthenia gravis patients had either complete remission or pharmacologic remission compared with 12.5% among non-thymectomy patients ( p-value <0.001). No significant difference in the clinical outcome was found between thymomatous and non-thymomatous myasthenia gravis after thymectomy ( p-value = 0.29).

          Conclusion

          This study showed that both thymomatous and non-thymomatous myasthenia gravis patients who underwent thymectomy had a higher incidence of complete stable remission and pharmacologic remission as compared with myasthenia gravis patients who did not undergo thymectomy.

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          Most cited references 29

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          Myasthenia gravis.

           D Drachman (1994)
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            Lifetime course of myasthenia gravis.

             D Grob,  N Brünner,  T Namba (2008)
            Between 1940 and 2000 a total of 1976 patients with myasthenia gravis (MG) were studied. Diagnosis was made by improvement in weakness after anticholinesterase medication. The historical developments in diagnosis and treatment of MG are reviewed. We analyzed the clinical course of MG as influenced by age, gender, thymectomy, thymomectomy, and the presence of antibodies to acetylcholine receptors (AChR). The clinical course of MG was significantly influenced by age and gender, and these need special attention in managing patients. The most severe level of weakness and high mortality occurred during the first 1 to 2 years of the disease, after which many patients experienced improvement. For treating MG patients the usefulness of thymectomy remains to be proven, and novel drugs need to be developed to increase the number as well as normal functioning of the AChRs and other components of the neuromuscular system.
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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

                Contributors
                URI : http://frontiersin.org/people/u/336532
                Journal
                Front Neurol
                Front Neurol
                Front. Neurol.
                Frontiers in Neurology
                Frontiers Media S.A.
                1664-2295
                21 June 2016
                2016
                : 7
                Affiliations
                1Department of Neurosciences, Philippine General Hospital , Manila, Philippines
                2Department of Surgery, Philippine General Hospital , Manila, Philippines
                3Department of Pathology, Philippine General Hospital , Manila, Philippines
                Author notes

                Edited by: Ghazala Hayat, St. Louis University, USA

                Reviewed by: Holli A. Horak, University of Arizona, USA; Stanley Iyadurai, Ohio State University, USA

                *Correspondence: Ranhel C. De Roxas, rhainderoxas@ 123456yahoo.com

                Specialty section: This article was submitted to Neuromuscular Diseases, a section of the journal Frontiers in Neurology

                Article
                10.3389/fneur.2016.00096
                4914503
                27445963
                Copyright © 2016 De Roxas, Bagnas, Baldonado, Rivera and Roxas.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                Page count
                Figures: 0, Tables: 5, Equations: 0, References: 37, Pages: 7, Words: 4807
                Categories
                Neuroscience
                Original Research

                Neurology

                thymectomy, myasthenia gravis, thymoma, non-thymomatous

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