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Myasthenia Gravis

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Neuroscience in the 21st Century

Abstract

Autoimmune diseases are becoming increasingly important with respect to the quality of life and health in modern societies. Myasthenia gravis (MG) is a prototype autoimmune disease since it has well-studied antigenic targets, pathogenic mechanisms, and experimental animal models of the disease. It is, therefore, well characterized and often serves as a model for other similar disorders. The hallmark of MG is muscle weakness and fatigability, caused by disruption of the neuromuscular junction function. This is mediated by autoantibodies, which in the majority of patients are directed against the acetylcholine receptor in the muscle membrane. Accurate diagnosis is crucial in administering treatment, relying on both clinical examination and a combination of electrophysiological, serological, and pharmacological tests. The most common therapy is immunosuppression, which can have a number of side effects, due to the long-term treatment regimes required. Thymectomy is recommended in many patients since the thymus seems to be central in the pathogenesis of MG. Other approaches, such as acetylcholinesterase inhibitors, intravenous immunoglobulin, and plasmapheresis, have also proven useful in the management of MG. Nevertheless, there is a pressing need for more sensitive diagnostic tests and more specific therapies with fewer side effects. To this end, efforts are currently being made for an even better understanding of the pathophysiology of MG. Importantly, this will not only aid in the management of MG but will lead to advances in other autoimmune disorders as well.

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Abbreviations

ACh:

Acetylcholine

AChE:

Acetylcholinesterase

ACHE-I:

Acetylcholinesterase inhibitor

AChR:

Nicotinic acetylcholine receptor

AIRE:

Autoimmune regulator gene

EAMG:

Experimental autoimmune myasthenia gravis

ECD:

Extracellular domain

EPP:

End-plate potential

HLA:

Human leukocyte antigen

IFN:

Interferon

IL:

Interleukin

IVIg:

Intravenous immunoglobulin

LNC:

Lymph node cell

MAC:

Membrane attack complex

MG:

Myasthenia gravis

MHC:

Major histocompatibility complex

MIR:

Main immunogenic region

MuSK:

Muscle-specific kinase

NMJ:

Neuromuscular junction

PDE:

Phosphodiesterase

RIPA:

Radioimmunoprecipitation assays

SF-EMG:

Single-fiber electromyography

TEC:

Thymic epithelial cell

VGSC:

Voltage-gated sodium channels

Further Reading

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  • (2018) Myasthenia gravis and related disorders Vol I & II, 13th international conference, vols 1412 & 1413. New York Academy of Sciences, New York

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  • Christadoss P (ed) (2010) Myasthenia gravis: disease mechanisms and immune intervention, 2nd edn. Linus, New York

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  • Drachman DB (2008) Therapy of myasthenia gravis. In: Engel AG (ed) Handbook of clinical neurology, vol 91. Neuromuscular junction disorders. Elsevier B.V, Edinburgh

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  • Kalamida D, Poulas K, Avramopoulou V, Fostieri E, Lagoumintzis G, Lazaridis K, Sideri A, Zouridakis M, Tzartos SJ (2007) Muscle and neuronal nicotinic acetylcholine receptors: structure, function and pathogenicity. FEBS J 274:3799–3845

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  • Lang B, Vincent A (2009) Autoimmune disorders of the neuromuscular junction. Curr Opin Pharmacol 9:336–340

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  • Lazaridis K, Tzartos SJ (2020) Autoantibody specificities in myasthenia gravis; implications for improved diagnostics and therapeutics. Front Immunol 11:212

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  • Meriggioli MN, Sanders DB (2009) Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol 8:475–490

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  • Myasthenia Gravis Foundation of America. http://www.myasthenia.org

  • The Myasthenia Gravis Association. http://www.mgauk.org

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Correspondence to Konstantinos Lazaridis .

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Lazaridis, K., Tzartos, S. (2021). Myasthenia Gravis. In: Pfaff, D.W., Volkow, N.D., Rubenstein, J. (eds) Neuroscience in the 21st Century. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6434-1_104-3

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  • DOI: https://doi.org/10.1007/978-1-4614-6434-1_104-3

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6434-1

  • Online ISBN: 978-1-4614-6434-1

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