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

Publication ,  Journal Article
Guptill, JT; Sanders, DB; Evoli, A
Published in: Muscle Nerve
July 2011

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.

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Published In

Muscle Nerve

DOI

EISSN

1097-4598

Publication Date

July 2011

Volume

44

Issue

1

Start / End Page

36 / 40

Location

United States

Related Subject Headings

  • Young Adult
  • Treatment Outcome
  • Receptors, Cholinergic
  • Receptor Protein-Tyrosine Kinases
  • Plasma Exchange
  • Neurology & Neurosurgery
  • Myasthenia Gravis
  • Middle Aged
  • Male
  • Humans
 

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Guptill, J. T., Sanders, D. B., & Evoli, A. (2011). Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve, 44(1), 36–40. https://doi.org/10.1002/mus.22006
Guptill, Jeffrey T., Donald B. Sanders, and Amelia Evoli. “Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts.Muscle Nerve 44, no. 1 (July 2011): 36–40. https://doi.org/10.1002/mus.22006.
Guptill JT, Sanders DB, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve. 2011 Jul;44(1):36–40.
Guptill, Jeffrey T., et al. “Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts.Muscle Nerve, vol. 44, no. 1, July 2011, pp. 36–40. Pubmed, doi:10.1002/mus.22006.
Guptill JT, Sanders DB, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve. 2011 Jul;44(1):36–40.
Journal cover image

Published In

Muscle Nerve

DOI

EISSN

1097-4598

Publication Date

July 2011

Volume

44

Issue

1

Start / End Page

36 / 40

Location

United States

Related Subject Headings

  • Young Adult
  • Treatment Outcome
  • Receptors, Cholinergic
  • Receptor Protein-Tyrosine Kinases
  • Plasma Exchange
  • Neurology & Neurosurgery
  • Myasthenia Gravis
  • Middle Aged
  • Male
  • Humans