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Myasthenia gravis with antibodies to MuSK: an update.
- Source :
- Annals of the New York Academy of Sciences; Jan2018, Vol. 1412 Issue 1, p82-89, 8p, 2 Graphs
- Publication Year :
- 2018
-
Abstract
- Abstract: Myasthenia gravis with antibodies to the muscle‐specific tyrosine kinase (MuSK<superscript>+</superscript> MG) is a rare disease with distinctive pathogenic mechanisms and clinical features. An acute onset and predominant bulbar muscle weakness are very common and highly suggestive of the disease. On the other hand, a more indolent course, atypical ocular presentation, and signs of cholinergic hyperactivity may complicate the diagnosis. Though MuSK<superscript>+</superscript> MG is still a severe disease, over the years we have observed a steady reduction in the rate of respiratory crisis and a significant improvement in the clinical outcome, both likely related to earlier diagnosis and timely treatment. Despite the improved management, MuSK<superscript>+</superscript> MG patients tend to remain dependent on long‐term immunosuppressive treatment and may develop permanent disabling weakness. In uncontrolled studies, B cell depletion with rituximab proved effective in most patients with refractory disease, inducing prolonged clinical responses associated with a sustained reduction of serum antibody levels. Promising results from experimental studies and case reports suggest that both 3,4‐diaminopyridine and albuterol may be effective as symptomatic agents. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 00778923
- Volume :
- 1412
- Issue :
- 1
- Database :
- Complementary Index
- Journal :
- Annals of the New York Academy of Sciences
- Publication Type :
- Academic Journal
- Accession number :
- 127679413
- Full Text :
- https://doi.org/10.1111/nyas.13518