Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis Current Status
Journal of Clinical Neurology
;
: 53-64, 2009.
Article
Dans Anglais
| 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.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Échange plasmatique
/
Stéroïdes
/
Tyrosine
/
Protein-tyrosine kinases
/
Acétylcholine
/
Alabama
/
Anticholinestérasiques
/
Édrophonium
/
Muscles de la face
/
Immunomodulation
Limites du sujet:
Femelle
/
Humains
Pays comme sujet:
Amérique du Nord
langue:
Anglais
Texte intégral:
Journal of Clinical Neurology
Année:
2009
Type:
Article
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