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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.
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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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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