Cholinergic neuromuscular hyperactivity in patients with myasthenia gravis seropositive for MuSK antibody

Muscle Nerve. 2006 Jul;34(1):111-5. doi: 10.1002/mus.20515.

Abstract

A 75-year-old man with severe oculobulbar myasthenia gravis (MG) treated with acetylcholine esterase inhibitors (AChEIs) was found to have muscle-specific tyrosine kinase (MuSK) antibodies. Neurophysiological examination displayed extra repetitive discharges after the compound motor action potential (CMAP) at low-frequency stimulation, possibly triggered by AChEI. This indicates an abnormal sensitivity to acetylcholine in patients with MuSK antibodies and may be a useful indicator of the adverse effect of AChEI treatment in these patients.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Acetylcholine*
  • Action Potentials / drug effects
  • Aged
  • Antibodies / blood*
  • Cholinesterase Inhibitors / therapeutic use
  • Electric Stimulation / methods
  • Electromyography / methods
  • Humans
  • Male
  • Muscle, Skeletal / innervation
  • Muscle, Skeletal / physiopathology
  • Myasthenia Gravis / drug therapy
  • Myasthenia Gravis / immunology*
  • Myasthenia Gravis / pathology
  • Myasthenia Gravis / physiopathology*
  • Neuromuscular Junction / drug effects
  • Neuromuscular Junction / physiopathology*
  • Receptor Protein-Tyrosine Kinases / immunology*
  • Receptors, Cholinergic / immunology*

Substances

  • Antibodies
  • Cholinesterase Inhibitors
  • Receptors, Cholinergic
  • MUSK protein, human
  • Receptor Protein-Tyrosine Kinases
  • Acetylcholine