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Thymoma-Associated Myasthenia Gravis

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Book cover Myasthenia Gravis and Related Disorders

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

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.

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Authors and Affiliations

  1. Department of Pathology, University Medical Centre Mannheim, University of Heidelberg, Mannheim, Germany

    Alexander Marx MD & Cleo-Aron Weis MD, MSc

  2. Department of Pathology, University Medical Center Göttingen, University of Göttingen, Göttingen, Germany

    Philipp Ströbel MD

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  1. Alexander Marx MD
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  3. Cleo-Aron Weis MD, MSc
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Correspondence to Alexander Marx MD .

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  1. Department of Neurology, George Washington University, Washington, DC, USA

    Henry J. Kaminski

  2. Department of Pharmacology and Physiology, George Washington University, Washington, DC, USA

    Linda L. Kusner

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Marx, A., Ströbel, P., Weis, CA. (2018). Thymoma-Associated Myasthenia Gravis. In: Kaminski, H., Kusner, L. (eds) Myasthenia Gravis and Related Disorders. Current Clinical Neurology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-73585-6_8

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