Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis Current Status
Journal of Clinical Neurology
;
: 53-64, 2009.
Artigo
em Inglês
| 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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Troca Plasmática
/
Esteroides
/
Tirosina
/
Proteínas Tirosina Quinases
/
Acetilcolina
/
Alabama
/
Inibidores da Colinesterase
/
Edrofônio
/
Músculos Faciais
/
Imunomodulação
Limite:
Feminino
/
Humanos
País/Região como assunto:
América do Norte
Idioma:
Inglês
Revista:
Journal of Clinical Neurology
Ano de publicação:
2009
Tipo de documento:
Artigo
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