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Autoantibody Testing in the Diagnosis and Management of Autoimmune Disorders of Neuromuscular Transmission and Related Diseases

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Myasthenia Gravis and Related Disorders

Abstract

The neuromuscular junction is the site of at least three distinct antibody-mediated diseases, myasthenia gravis (MG), the Lambert-Eaton myasthenic syndrome (LEMS), and acquired neuromyotonia. Antibodies to AChR and MuSK can be helpful in the diagnosis of myasthenia gravis, and their detection in patients with neuromuscular transmission disorders has helped define new subtypes of the disease. With increasing reluctance to use the classical radioimmunoprecipitation assays, other techniques such as ELISA and cell-based assays (CBAs) have been established; CBAs have also been used to detect antibodies to LRP4, agrin and other antigens, in some otherwise negative MG patients, but the roles of these antibodies are not clear yet. Antibodies to VGCC in the Lambert-Eaton myasthenic syndrome and to the VGKC-complex proteins, CASPR2 and LGI1, in acquired neuromyotonia are becoming more widely available. Although often helpful, these methods still need to be validated and standardized. Moreover, although each of these antibodies has the potential to be pathogenic, since they bind to extracellular aspects of membrane proteins, the pathogenic mechanisms need further studies.

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Correspondence to Angela Vincent MBBS, MSc, FRCPath, FRS .

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Cao, M., Vincent, A. (2018). Autoantibody Testing in the Diagnosis and Management of Autoimmune Disorders of Neuromuscular Transmission and Related Diseases. 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_10

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  • DOI: https://doi.org/10.1007/978-3-319-73585-6_10

  • Published:

  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-73584-9

  • Online ISBN: 978-3-319-73585-6

  • eBook Packages: MedicineMedicine (R0)

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