Myasthenia gravis, MuSK antibody positive (Q88643): Difference between revisions
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Aproximadamente 50% dos pacientes sem autoanticorpos contra o receptor de acetilcolina possuem autoanticorpos contra a quinase muscular específica (MuSK). MG anti-MusK positivo é uma entidade de doença separada devido ao padrão característico de fraqueza, curso da doença e fisiopatologia. MG anti-MusK positivo é 20 vezes mais rara que MG anti-AChR positivo (prevalência de 1–2 por 10.000 pessoas). A maioria dos pacientes é do sexo feminino, tem início <40 anos, apresenta ptose e diplopia, pode desenvolver rapidamente fraqueza bulbar (apresentação oculobulbar) e apresenta maior risco de crise respiratória. Um terço a metade dos pacientes com MG anti-MuSK positivo podem apresentar crises miastênicas que requerem suporte ventilatório. A fraqueza dos músculos dos membros é menos grave. Pequenos timomas foram ocasionalmente relatados. | |||
| description / en | description / en | ||
Approximately 50% of the Acetylcholine receptor-negative patients have autoantibodies against muscle-specific kinase (MuSK). MuSK MG is a separate disease entity given the characteristic pattern of weakness, disease course and pathophysiology. MuSK MG is 20 x as rare as AChR –positive MG (prevalence 1–2 per 10,000 persons). Most patients are female, have an onset <40 years, present with ptosis and diplopia, may rapidly develop bulbar weakness (oculobulbar presentation) and are at higher risk of respiratory crisis. One-third to half of MuSK MG patients may experience myasthenic crises requiring ventilatory support. Limb muscle weakness is less severe. Small thymomas have occasionally been reported. | |||
Revision as of 14:57, 15 August 2026
Approximately 50% of the Acetylcholine receptor-negative patients have autoantibodies against muscle-specific kinase (MuSK). MuSK MG is a separate disease entity given the characteristic pattern of weakness, disease course and pathophysiology. MuSK MG is 20 x as rare as AChR –positive MG (prevalence 1–2 per 10,000 persons). Most patients are female, have an onset <40 years, present with ptosis and diplopia, may rapidly develop bulbar weakness (oculobulbar presentation) and are at higher risk of respiratory crisis. One-third to half of MuSK MG patients may experience myasthenic crises requiring ventilatory support. Limb muscle weakness is less severe. Small thymomas have occasionally been reported.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_422441512 |
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| English | Myasthenia gravis, MuSK antibody positive |
Approximately 50% of the Acetylcholine receptor-negative patients have autoantibodies against muscle-specific kinase (MuSK). MuSK MG is a separate disease entity given the characteristic pattern of weakness, disease course and pathophysiology. MuSK MG is 20 x as rare as AChR –positive MG (prevalence 1–2 per 10,000 persons). Most patients are female, have an onset <40 years, present with ptosis and diplopia, may rapidly develop bulbar weakness (oculobulbar presentation) and are at higher risk of respiratory crisis. One-third to half of MuSK MG patients may experience myasthenic crises requiring ventilatory support. Limb muscle weakness is less severe. Small thymomas have occasionally been reported. |
