Muscle-specific receptor tyrosine kinase antibody positive myasthenia gravis current status. Journal of clinical neurology (Seoul, Korea) [J Clin Neurol] Journal article | | Title | Muscle-specific receptor tyrosine kinase antibody positive myasthenia gravis current status. | | Author(s) | Oh SJ | | Institution | Distinguished Professor of Neurology, The University of Alabama at Birmingham, The Veterans Affairs Medical Center, Birmingham, Alabama, USA. | | Source | J Clin Neurol 2009 Jun; 5(2):53-64. | | Abstract | Muscle-specific tyrosine-kinase-antibody-positive myasthenia gravis (MuSK-MG) has emerged as a distinct entity since 2001. This disease has been reported worldwide, but with varying rates among patients with generalized acetylcholine-receptor-antibody-negative MG. MuSK-MG was detected in approximately 37% of generalized acetylcholine receptor antibody-negative MG. MuSK-MG patients were predominantly female with more prominent facial and bulbar involvement and more frequent crises. Disease onset tended to be earlier. Patients tended to have a relatively poor edrophonium response but showed prominent decrement in the repetitive nerve stimulation test in the facial muscles. Patients were more likely to display poor tolerance of, or a lack of improvement with, anticholinesterase agents. Somewhat better response was observed with steroids and plasma exchange. Most were managed successfully with aggressive immunomodulatory therapies, although a higher proportion of MuSK-MG patients had a refractory course when compared with other forms of generalized MG. I present here an up-to-date overview on MuSK-MG based on our experience at the University of Alabama at Birmingham and the existing literature. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 19587811 |
|
|
| |
Advertise on this site.
| | |
|
