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.
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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
Several chapters on issues of myasthenia gravis, authored by specialists in the field. Autoimmunity (2010) 43(5–6):341–460
Christadoss P (ed) (2010) Myasthenia gravis: disease mechanisms and immune intervention, 2nd edn. Linus, New York
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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Kaminski HJ, Barohn RJ (eds) (2008) Myasthenia gravis and related disorders, 11th international conference, vol 1132. New York Academy of Sciences, New York
Lang B, Vincent A (2009) Autoimmune disorders of the neuromuscular junction. Curr Opin Pharmacol 9:336–340
Lindstrom JM (2000) Acetylcholine receptors and myasthenia. Muscle Nerve 23:453–477
Meriggioli MN, Sanders DB (2009) Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol 8:475–490
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
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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
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DOI: https://doi.org/10.1007/978-1-4614-1997-6_104
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4614-1996-9
Online ISBN: 978-1-4614-1997-6
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