Skip to main content

Myasthenia Gravis

  • Reference work entry
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 effected 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 immunosupression, 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.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 1,699.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

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

  • Several chapters on issues of myasthenia gravis, authored by specialists in the field. Autoimmunity (2010) 43(5–6):341–460

    Google Scholar 

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

    Google Scholar 

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

    Google Scholar 

  • 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

    Article  PubMed  CAS  Google Scholar 

  • Kaminski HJ, Barohn RJ (eds) (2008) Myasthenia gravis and related disorders, 11th international conference, vol 1132. New York Academy of Sciences, New York

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  • Lindstrom JM (2000) Acetylcholine receptors and myasthenia. Muscle Nerve 23:453–477

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

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

  • Willcox N (ed) (2008) Special issue in memory of John Newsom-Davis. J Neuroimmun 201-202:1–256

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Socrates Tzartos .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media, LLC

About this entry

Cite this entry

Lazaridis, K., Tzartos, S. (2013). Myasthenia Gravis. In: Pfaff, D.W. (eds) Neuroscience in the 21st Century. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-1997-6_104

Download citation

Publish with us

Policies and ethics