Neues zur Myasthenia gravis

Zusammenfassung

Die Myasthenia gravis ist die klassische neurologische Autoimmunerkrankung. Bei dieser Erkrankung kommt es durch pathologische Autoantikörper zu einer Störung der neuromuskulären Übertragung. Bei der überwiegenden Zahl der Betroffenen lassen sich ursächlich Antikörper gegen den Acetylcholinrezeptor (AChR) nachweisen. Fortschritte wurden in den letzten Jahren insbesondere durch die Identifikation eines weiteren pathogenen Auto-Antikörpers bei der so genannten AChR-Antikörper-negativen Myasthenie gemacht. Außerdem liegen Untersuchungen vor, anhand derer der diagnostische Stellenwert der Antikörper gegen quergestreifte Muskulatur, insbesondere der Titin-Antikörper, besser beurteilt werden kann. In dieser Übersicht werden darüber hinaus die Datenlage zum Nutzen der Thymektomie und neue Entwicklungen in der Pharmakotherapie zusammengefasst.

Summary

Myasthenia gravis is an antibody-mediated autoimmune disease. Classically, antibodies against the acetylcholine receptor (AChR) cause inactivation and loss of acetylcholine receptors at the neuromuscular junction. Progress has been made not only in the identification of alternate autoantibodies in so called AChR-antibody negative patients but also concerning the diagnostic value of antibodies against striated muscle proteins. In this review, these advances in myasthenia gravis are summarized. Moreover, concerning therapy, the value of thymectomy is discussed and recent developments in immunotherapy are reported.

Schlüsselwörter

Antikörper-negative Myasthenie - MuSK-Antikörper - Titin-Antikörper - Thymektomie - Mycophenolat Mofetil

Keywords

Antibody-negative myasthenia - MuSK-antibody - Titin-antibody - thymectomy - mycophenolate mofetil

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