Skip to main content

Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis.

Publication ,  Journal Article
Guptill, JT; Sanders, DB
Published in: Curr Opin Neurol
October 2010

PURPOSE OF REVIEW: Important concepts regarding the pathogenesis, clinical features, diagnosis and treatment of muscle-specific tyrosine kinase (MuSK) antibody positive myasthenia gravis will be reviewed. Special attention will be paid to clinical phenotypes and treatment, particularly encouraging responses that have been reported to rituximab. RECENT FINDINGS: Worldwide studies confirm three major phenotypes in MuSK antibody positive myasthenia gravis (MMG) patients: indistinguishable from acetylcholine receptor antibody positive patients, prominent faciopharyngeal weakness, usually with marked muscle atrophy, and relatively isolated neck extensor and respiratory weakness. MMG predominates in women and weakness is typically more severe, with more frequent respiratory crises than non-MuSK myasthenia gravis. Patients with sub-acute bulbar, shoulder, and neck weakness pose unique challenges in terms of differential diagnosis and electrodiagnosis. Electrodiagnostic studies evaluating for disorders of neuromuscular transmission should focus on proximal limb and facial muscles, as well as clinically weak muscles. The response to acetylcholinesterase inhibitors is often disappointing. Long-term outcomes appear favorable though patients typically require more aggressive immunosuppression. Uncontrolled observations report encouraging results with rituximab in the treatment of refractory MMG. The role of thymectomy in the management of these patients remains uncertain. SUMMARY: MuSK antibody positive patients represent a unique subset of myasthenia gravis. Identification of these patients has important diagnostic and disease management implications.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Curr Opin Neurol

DOI

EISSN

1473-6551

Publication Date

October 2010

Volume

23

Issue

5

Start / End Page

530 / 535

Location

England

Related Subject Headings

  • Receptors, Cholinergic
  • Receptor Protein-Tyrosine Kinases
  • Neurology & Neurosurgery
  • Myasthenia Gravis
  • Humans
  • Autoantibodies
  • 3209 Neurosciences
  • 3202 Clinical sciences
  • 1702 Cognitive Sciences
  • 1109 Neurosciences
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Guptill, J. T., & Sanders, D. B. (2010). Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis. Curr Opin Neurol, 23(5), 530–535. https://doi.org/10.1097/WCO.0b013e32833c0982
Guptill, Jeffrey T., and Donald B. Sanders. “Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis.Curr Opin Neurol 23, no. 5 (October 2010): 530–35. https://doi.org/10.1097/WCO.0b013e32833c0982.
Guptill JT, Sanders DB. Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis. Curr Opin Neurol. 2010 Oct;23(5):530–5.
Guptill, Jeffrey T., and Donald B. Sanders. “Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis.Curr Opin Neurol, vol. 23, no. 5, Oct. 2010, pp. 530–35. Pubmed, doi:10.1097/WCO.0b013e32833c0982.
Guptill JT, Sanders DB. Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis. Curr Opin Neurol. 2010 Oct;23(5):530–535.

Published In

Curr Opin Neurol

DOI

EISSN

1473-6551

Publication Date

October 2010

Volume

23

Issue

5

Start / End Page

530 / 535

Location

England

Related Subject Headings

  • Receptors, Cholinergic
  • Receptor Protein-Tyrosine Kinases
  • Neurology & Neurosurgery
  • Myasthenia Gravis
  • Humans
  • Autoantibodies
  • 3209 Neurosciences
  • 3202 Clinical sciences
  • 1702 Cognitive Sciences
  • 1109 Neurosciences