Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts.

Journal Article (Journal Article;Multicenter Study)

INTRODUCTION: Myasthenia gravis (MG) patients with autoantibodies to muscle-specific tyrosine kinase (MuSK) represent a distinct subset of those with this disease. Treatment and outcomes data in these patients are limited and conflicting. METHODS: We reviewed 110 MuSK-MG patients from two large clinics in Italy and the USA. RESULTS: Thirty-nine to 49% of patients with generalized, acetylcholine receptor antibody (AChR-Ab)-negative MG had MuSK-MG. Eighty-five percent were female, with disease onset typically in the fourth decade. Ocular and/or bulbar symptoms were present at onset in 79% of those studied. Eighty-five percent were MGFA class III or greater, and crisis occurred in 28%. Plasma exchange (PLEX) produced improvement in 93%, whereas only 61% improved after intravenous immunoglobulin. Long-term outcomes were comparable to those of patients with AChR-Ab-positive MG. CONCLUSIONS: MuSK-MG has a marked female predominance with frequent oculobulbar weakness and crises. Many patients deteriorate rapidly early in the disease, and PLEX is usually the preferred treatment. Long-term outcomes are similar to those of patients with AChR-Ab(+) MG.

Full Text

Duke Authors

Cited Authors

  • Guptill, JT; Sanders, DB; Evoli, A

Published Date

  • July 2011

Published In

Volume / Issue

  • 44 / 1

Start / End Page

  • 36 - 40

PubMed ID

  • 21674519

Electronic International Standard Serial Number (EISSN)

  • 1097-4598

Digital Object Identifier (DOI)

  • 10.1002/mus.22006


  • eng

Conference Location

  • United States