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Myasthenia gravis

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Klinische Neurologie

Part of the book series: Springer Reference Medizin ((SRM))

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Zusammenfassung

Die Myasthenia gravis ist eine erworbene Autoimmunerkrankung, die durch belastungsinduzierte vorzeitige Ermüdbarkeit der Muskulatur gekennzeichnet ist. Bei 85 % der Patienten sind Antikörper gegen den Acetylcholinrezeptor der neuromuskulären Synapse nachweisbar. Daneben finden sich Antikörper gegen den muskelspezifischen Tyrosinkinaserezeptor und das Low-density lipoprotein receptor-related protein 4 (LRP4). Bei etwa 20 % der Erkrankten wird ein Thymom nachgewiesen (häufig mit Titin-Antikörpern). Die Therapie umfasst eine Immunsuppression ergänzt durch Acetylcholinesterasehemmer. Die Thymektomie wird bei der generalisierten Myasthenie mit AChR-Antikörpern im Frühstadium der Erkrankung empfohlen und ist bei Thymomverdacht immer indiziert.

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Literatur

  • Berrih-Aknin S, Le Panse R (2014) Myasthenia gravis: a comprehensive review of immune dysregulation and etiological mechanisms. J Autoimmun 52:90–100

    Article  CAS  Google Scholar 

  • Besinger UA, Toyka KV, Homberg M, Heininger K, Hohlfeld R, Fateh-Moghadam A (1983) Myasthenia gravis: long-term correlation of binding and bungarotoxin blocking antibodies against acetylcholine receptors with changes in disease severity. Neurology 33(10):1316–1321

    Article  CAS  Google Scholar 

  • Binks S, Vincent A, Palace J (2016) Myasthenia gravis: a clinical-immunological update. J Neurol 263(4):826–834

    Article  CAS  Google Scholar 

  • Dalakas MC (2015) Future perspectives in target-specific immunotherapies of myasthenia gravis. Ther Adv Neurol Disord 8(6):316–327

    Article  CAS  Google Scholar 

  • Della Marina A, Trippe H, Lutz S, Schara U (2014) Juvenile myasthenia gravis: recommendations for diagnostic approaches and treatment. Neuropediatrics 45(2):75–83

    Article  Google Scholar 

  • Detterbeck FC, Stratton K, Giroux D, Asamura H, Crowley J, Falkson C, Filosso PL, Frazier AA, Giaccone G, Huang J, Kim J, Kondo K, Lucchi M, Marino M, Marom EM, Nicholson AG, Okumura M, Ruffini E, Van Schil P, Staging and Prognostic Factors Committee, Members of the Advisory Boards, Participating Institutions of the Thymic Domain (2014) The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: proposal for an evidence-based stage classification system for the forthcoming (8th) edition of the TNM classification of malignant tumors. J Thorac Oncol 9(Suppl 2):S65–S72

    Google Scholar 

  • Evoli A (2010) Acquired myasthenia gravis in childhood. Curr Opin Neurol 23(5):536–540

    Article  Google Scholar 

  • Evoli A, Iorio R, Bartoccioni E (2016) Overcoming challenges in the diagnosis and treatment of myasthenia gravis. Expert Rev Clin Immunol 12(2):157–168

    Article  CAS  Google Scholar 

  • Fang F, Sveinsson O, Thormar G, Granqvist M, Askling J, Lundberg IE, Ye W, Hammarstrom L, Pirskanen R, Piehl F (2015) The autoimmune spectrum of myasthenia gravis: a Swedish population-based study. J Intern Med 277(5):594–604

    Article  CAS  Google Scholar 

  • Gilhus NE, Verschuuren JJ (2015) Myasthenia gravis: subgroup classification and therapeutic strategies. Lancet Neurol 14(10):1023–1036

    Article  CAS  Google Scholar 

  • Grob D, Brunner N, Namba T, Pagala M (2008) Lifetime course of myasthenia gravis. Muscle Nerve 37(2):141–149

    Article  Google Scholar 

  • Hohlfeld R, Toyka KV, Besinger UA, Gerhold B, Heininger K (1985) Myasthenia gravis: reactivation of clinical disease and of autoimmune factors after discontinuation of long-term azathioprine. Ann Neurol 17(3):238–242

    Article  CAS  Google Scholar 

  • Jaretzki A 3rd, Barohn RJ, Ernstoff RM, Kaminski HJ, Keesey JC, Penn AS, Sanders DB (2000) Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology 55(1):16–23

    Article  Google Scholar 

  • Jordan B, Zierz S (2016) Myasthenie und Thymus. Nervenheilkunde 10:665–672

    Google Scholar 

  • Jordan B, Eger K, Zierz S (2009) Polymyositis associated with thymoma. Nervenarzt 80(6):708–711

    Article  CAS  Google Scholar 

  • Jordan B, Kellner J, Jordan K, Bahre M, Behrmann C, Zierz S (2016) Thymic pathologies in myasthenia gravis: a preoperative assessment of CAT scan and nuclear based imaging. J Neurol 263(4):641–648

    Article  CAS  Google Scholar 

  • Leite MI, Jacob S, Viegas S, Cossins J, Clover L, Morgan BP, Beeson D, Willcox N, Vincent A (2008) IgG1 antibodies to acetylcholine receptors in ‚seronegative‘ myasthenia gravis. Brain 131(Pt 7):1940–1952

    Article  Google Scholar 

  • Marx A, Pfister F, Schalke B, Saruhan-Direskeneli G, Melms A, Strobel P (2013) The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes. Autoimmun Rev 12(9):875–884

    Article  CAS  Google Scholar 

  • Nacu A, Andersen JB, Lisnic V, Owe JF, Gilhus NE (2015) Complicating autoimmune diseases in myasthenia gravis: a review. Autoimmunity 48(6):362–368

    Article  Google Scholar 

  • Nicolaou S, Muller NL, Li DK, Oger JJ (1996) Thymus in myasthenia gravis: comparison of CT and pathologic findings and clinical outcome after thymectomy. Radiology 201(2):471–474

    Article  CAS  Google Scholar 

  • Norwood F, Dhanjal M, Hill M, James N, Jungbluth H, Kyle P, O’Sullivan G, Palace J, Robb S, Williamson C, Hilton-Jones D, Nelson-Piercy C (2014) Myasthenia in pregnancy: best practice guidelines from a U.K. multispecialty working group. J Neurol Neurosurg Psychiatry 85(5):538–543

    Article  Google Scholar 

  • O’Donovan P, Perrett CM, Zhang X, Montaner B, Xu YZ, Harwood CA, McGregor JM, Walker SL, Hanaoka F, Karran P (2005) Azathioprine and UVA light generate mutagenic oxidative DNA damage. Science 309(5742):1871–1874

    Article  Google Scholar 

  • Oosterhuis HJ (1989) The natural course of myasthenia gravis: a long term follow up study. J Neurol Neurosurg Psychiatry 52(10):1121–1127

    Article  CAS  Google Scholar 

  • Palace J, Newsom-Davis J, Lecky B (1998) A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis. Myasthenia Gravis Study Group. Neurology 50(6):1778–1783

    Article  CAS  Google Scholar 

  • Priola AM, Priola SM (2014) Imaging of thymus in myasthenia gravis: from thymic hyperplasia to thymic tumor. Clin Radiol 69(5):e230–e245

    Article  CAS  Google Scholar 

  • Rodriguez Cruz PM, Huda S, Lopez-Ruiz P, Vincent A (2015) Use of cell-based assays in myasthenia gravis and other antibody-mediated diseases. Exp Neurol 270:66–71

    Article  CAS  Google Scholar 

  • Sanders DB, Wolfe GI, Benatar M, Evoli A, Gilhus NE, Illa I, Kuntz N, Massey JM, Melms A, Murai H, Nicolle M, Palace J, Richman DP, Verschuuren J, Narayanaswami P (2016) International consensus guidance for management of myasthenia gravis: executive summary. Neurology 87(4):419–425

    Article  Google Scholar 

  • Shin HY, Park HJ, Lee HE, Choi YC, Kim SM (2014) Clinical and electrophysiologic responses to acetylcholinesterase inhibitors in MuSK-Antibody-positive Myasthenia Gravis: evidence for Cholinergic neuromuscular hyperactivity. J Clin Neurol 10(2):119–124

    Article  Google Scholar 

  • Skeie GO, Romi F (2008) Paraneoplastic myasthenia gravis: immunological and clinical aspects. Eur J Neurol 15(10):1029–1033

    Article  CAS  Google Scholar 

  • Weksler B, Lu B (2014) Alterations of the immune system in thymic malignancies. J Thorac Oncol 9(Suppl 2):S137–S142

    Article  Google Scholar 

  • Wiendl H, Hohlfeld R, Melms A, Melzer N, Tackenberg B, Schalke B, Schneider-Gold C, Zimprich F (2015) Diagnostik und Therapie der Myasthenia gravis und des Lambert Eaton Syndroms. In: Diener H, Gerloff C (Hrsg) Leitlinien für Diagnostik und Therapie in der Neurologie, 5. Aufl. (2012). Thieme Verlag, Berlin

    Google Scholar 

  • Wolfe GI, Herbelin L, Nations SP, Foster B, Bryan WW, Barohn RJ (1999) Myasthenia gravis activities of daily living profile. Neurology 52(7):1487–1489

    Article  CAS  Google Scholar 

  • Wolfe GI, Kaminski HJ, Aban IB, Minisman G, Kuo HC, Marx A, Strobel P, and M. S. Group et al (2016) Randomized Trial of Thymectomy in Myasthenia Gravis. N Engl J Med 375(6):511–522

    Article  Google Scholar 

  • Wong SH, Huda S, Vincent A, Plant GT (2014) Ocular myasthenia gravis: controversies and updates. Curr Neurol Neurosci Rep 14(1):421

    Article  Google Scholar 

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Authors and Affiliations

  1. Klinik und Poliklinik für Neurologie, Martin-Luther-Universität Halle-Wittenberg, Halle, Deutschland

    S. Zierz

  2. Neurologische Klinik, Universitätsklinikum Heidelberg, Heidelberg, Deutschland

    B. Jordan

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  1. B. Jordan
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  2. S. Zierz
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Correspondence to B. Jordan .

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  1. Essen, Germany

    Peter Berlit

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Jordan, B., Zierz, S. (2020). Myasthenia gravis. In: Berlit, P. (eds) Klinische Neurologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-44768-0_41-2

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  • DOI: https://doi.org/10.1007/978-3-662-44768-0_41-2

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  • Print ISBN: 978-3-662-44768-0

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Chapter history

  1. Latest

    Myasthenia gravis
    Published:
    09 February 2020

    DOI: https://doi.org/10.1007/978-3-662-44768-0_41-2

  2. Original

    Myasthenia gravis
    Published:
    23 November 2017

    DOI: https://doi.org/10.1007/978-3-662-44768-0_41-1

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