Thymoma-Associated Myasthenia Gravis

  • Alexander Marx
  • Philipp Ströbel
  • Cleo-Aron Weis
Part of the Current Clinical Neurology book series (CCNEU)


Thymoma-associated MG (TAMG) is almost always due to autoantibodies that recognize soluble nicotinic acetylcholine receptors (AChR). Alongside early-onset MG (EOMG) and late-onset MG (LOMG), TAMG represents the smallest subset (10–15%) among the anti-AChR MG subtypes. TAMG-associated thymomas are unique among cancers because almost all maintain thymus-like features, including intratumorous thymopoiesis. Therefore, it is thought that key immune tolerance-inducing mechanisms of the normal thymus go awry in thymomas. We describe the widely accepted three-step pathogenetic model of TAMG that comprises (1) intratumorous biased positive selection of T cells that preferentially recognizes skeletal muscle autoantigens, (2) intratumorous defective negative selection of potentially autoreactive effector T cells and deficient generation of regulatory T cells, and (3) the extra-tumorous, self-perpetuating activation of T and B cells and autoantibody-producing cells following enigmatic triggers. Molecular features underlying the three-step autoimmunization are also addressed. Current pathogenetic models of EOMG and LOMG are described for comparison.


Myasthenia gravis Thymoma Thymus Thymic follicular hyperplasia Autoimmune regulator AIRE CTLA4 Myoid cell 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Alexander Marx
    • 1
  • Philipp Ströbel
    • 2
  • Cleo-Aron Weis
    • 1
  1. 1.Department of PathologyUniversity Medical Centre Mannheim, University of HeidelbergMannheimGermany
  2. 2.Department of PathologyUniversity Medical Center Göttingen, University of GöttingenGöttingenGermany

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