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      International consensus guidance for management of myasthenia gravis : Executive summary

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          Abstract

          Objective:

          To develop formal consensus-based guidance for the management of myasthenia gravis (MG).

          Methods:

          In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness methodology was used to develop consensus guidance statements. Definitions were developed for goals of treatment, minimal manifestations, remission, ocular MG, impending crisis, crisis, and refractory MG. An in-person panel meeting then determined 7 treatment topics to be addressed. Initial guidance statements were developed from literature summaries. Three rounds of anonymous e-mail votes were used to attain consensus on guidance statements modified on the basis of panel input.

          Results:

          Guidance statements were developed for symptomatic and immunosuppressive treatments, IV immunoglobulin and plasma exchange, management of impending and manifest myasthenic crisis, thymectomy, juvenile MG, MG associated with antibodies to muscle-specific tyrosine kinase, and MG in pregnancy.

          Conclusion:

          This is an international formal consensus of MG experts intended to be a guide for clinicians caring for patients with MG worldwide.

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          Most cited references 3

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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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            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 in pregnancy: best practice guidelines from a U.K. multispecialty working group.

              A national U.K. workshop to discuss practical clinical management issues related to pregnancy in women with myasthenia gravis was held in May 2011. The purpose was to develop recommendations to guide general neurologists and obstetricians and facilitate best practice before, during and after pregnancy. The main conclusions were (1) planning should be instituted well in advance of any potential pregnancy to allow time for myasthenic status and drug optimisation; (2) multidisciplinary liaison through the involvement of relevant specialists should occur throughout pregnancy, during delivery and in the neonatal period; (3) provided that their myasthenia is under good control before pregnancy, the majority of women can be reassured that it will remain stable throughout pregnancy and the postpartum months; (4) spontaneous vaginal delivery should be the aim and actively encouraged; (5) those with severe myasthenic weakness need careful, multidisciplinary management with prompt access to specialist advice and facilities; (6) newborn babies born to myasthenic mothers are at risk of transient myasthenic weakness, even if the mother's myasthenia is well-controlled, and should have rapid access to neonatal high-dependency support.
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                Author and article information

                Contributors
                Journal
                Neurology
                Neurology
                neurology
                neur
                neurology
                NEUROLOGY
                Neurology
                Lippincott Williams & Wilkins (Hagerstown, MD )
                0028-3878
                1526-632X
                26 July 2016
                26 July 2016
                : 87
                : 4
                : 419-425
                Affiliations
                From the Department of Neurology (D.B.S., J.M.M.), Duke University Medical Center, Durham, NC; Department of Neurology (G.I.W.), University at Buffalo School of Medicine and Biomedical Sciences, State University of New York; Department of Neurology (M.B.), University of Miami, Miller School of Medicine, FL; Department of Neurology (A.E.), Catholic University, Rome, Italy; Department of Clinical Medicine (N.E.G.), University of Bergen, Norway; Department of Neurology (I.I.), Hospital Santa Creu i Sant Pau, Universitat Autònoma de Barcelona; CIBERER U762 (I.I.), Barcelona, Spain; Departments of Pediatrics and Neurology (N.K.), Northwestern Feinberg School of Medicine, Chicago, IL; Neurologische Klinik (A.M.), Universitätsklinikum Erlangen; Hertie Institute for Clinical Research (A.M.), University of Tübingen, Germany; Department of Neurological Therapeutics (H.M.), Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan; Department of Clinical Neurological Sciences (M.N.), Western University, London, Canada; Department of Clinical Neurology (J.P.), John Radcliffe Hospital, Oxford University Hospitals Trust, Oxford, UK; Department of Neurology (D.P.R.), University of California, Davis, CA; Department of Neurology (J.V.), Leiden University Medical Centre, Leiden, the Netherlands; and Department of Neurology, Beth Israel Deaconess Medical Center/Harvard Medical School (P.N.), Boston, MA.
                Author notes
                Correspondence to Dr. Sanders: donald.sanders@ 123456duke.edu

                Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. The Article Processing Charge was paid by the Myasthenia Gravis Foundation of America.

                [*]

                These authors contributed equally to this work.

                Article
                NEUROLOGY2015689232
                10.1212/WNL.0000000000002790
                4977114
                27358333
                © 2016 American Academy of Neurology

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.

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