Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis Current Status.
- Author:
Shin Joong OH
1
Author Information
- Publication Type:Review
- Keywords: myasthenia gravis; muscle-specific tyrosine-kinase-antibody; seronegative myasthenia gravis
- MeSH: Acetylcholine; Alabama; Cholinesterase Inhibitors; Edrophonium; Facial Muscles; Female; Humans; Immunomodulation; Myasthenia Gravis; Plasma Exchange; Protein-Tyrosine Kinases; Steroids; Tyrosine
- From:Journal of Clinical Neurology 2009;5(2):53-64
- CountryRepublic of Korea
- Language:English
- 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.
