Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis Current Status
Journal of Clinical Neurology
;
: 53-64, 2009.
Article
in English
| WPRIM
| ID: wpr-72318
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Plasma Exchange
/
Steroids
/
Tyrosine
/
Protein-Tyrosine Kinases
/
Acetylcholine
/
Alabama
/
Cholinesterase Inhibitors
/
Edrophonium
/
Facial Muscles
/
Immunomodulation
Limits:
Female
/
Humans
Country/Region as subject:
North America
Language:
English
Journal:
Journal of Clinical Neurology
Year:
2009
Type:
Article
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