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Muskelerkrankungen

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Neurologische Pathophysiologie

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Zusammenfassung

Die Myasthenia gravis ist eine Erkrankung der neuromuskulären Endplatte mit Bildung von Autoantikörpern gegen nikotinerge ACh-Rezeptoren sowie unterschiedliche andere Antigene der postsynaptischen Membran (MuSK, LRP4, Agrin etc.). In einigen Fällen, wie einer paraneoplastisch bedingten Myasthenie, lassen sich Antikörper gegen Proteine der Muskulatur (z. B. Titin) oder, bei einem Lambert-Eaton-Myasthenie-Syndrom (LEMS), gegen präsynaptische spannungsgesteuerte Kalziumkanäle (VGCC) nachweisen. Klinisch imponiert eine abnorme belastungsabhängige Ermüdbarkeit der Muskulatur, die entweder okulär oder generalisiert (mit teils okulärer oder bulbärer Betonung) auftreten kann. Akute myasthene Krisen können lebensbedrohlich sein und müssen intensivmedizinisch behandelt werden. Im vorliegenden Beitrag werden die Grundlagen der neuromuskulären Transmission wiederholt. Anschließend wird auf die Ursachen und die Immunpathogenese sowie auf häufig beteiligte Antikörper eingegangen.

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Authors and Affiliations

  1. St.-Josef-Hospital, Klinik für Neurologie, Ruhr-Universität Bochum, Bochum, Deutschland

    Dr. med. A.-S. Biesalski

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  1. Dr. med. A.-S. Biesalski
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Correspondence to A.-S. Biesalski .

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Editors and Affiliations

  1. Agaplesion Bethesda KH Wuppertal, Wuppertal, Deutschland

    Dietrich Sturm

  2. Ruhr-Universität Bochum, Bochum, Deutschland

    Anne-Sophie Biesalski  & Oliver Höffken  & 

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© 2019 Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature

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Biesalski, AS. (2019). Muskelerkrankungen. In: Sturm, D., Biesalski, AS., Höffken, O. (eds) Neurologische Pathophysiologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-56784-5_7

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  • DOI: https://doi.org/10.1007/978-3-662-56784-5_7

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-56783-8

  • Online ISBN: 978-3-662-56784-5

  • eBook Packages: Medicine (German Language)

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