Quantitative EMG of facial muscles in myasthenia patients with MuSK antibodies.
OBJECTIVE
Our aim was to study the pathophysiological process leading to facial muscle atrophy in 13 patients with MuSK antibody positive myasthenia gravis (MuSK-MG), and to compare with findings from 12 acetylcholine receptor antibody positive myasthenia patients (AChR-MG), selected because they suffered from the same degree of disease severity and required similar treatment.


METHODS
Motor unit action potential (MUAP) and interference pattern analysis from orbicularis oculi (O oculi) and orbicularis oris (O oris) muscles were studied using a concentric needle electrode, and compared with findings in 20 normal subjects, 6 patients receiving botulinum toxin injections (representing a neurogenic model) and 6 patients with a muscle dystrophy (representing a myopathic model). The techniques and control data have been reported previously.


RESULTS
The mean MUAP durations for O oculi and O oris were significantly reduced (p<0.001) in both MG cohorts when compared with healthy subjects, and were similar to those in the myopathic control group. They were significantly different from those obtained from the neurogenic control group (p<0.001 for both O oculi and O oris). The MUAP findings in O oculi occurred independently from neuromuscular blocking on single fibre EMG (SFEMG) in the same muscle. On turns amplitude analysis (TAA), 50% of MuSK-MG patients and 42% of AChR-MG patients had a pattern in O oculi which was similar to that in the myopathic control group, and 62% of MuSK-MG patients and 50% of AChR-MG patients had a pattern in O oris that was also similar to that in the myopathic control group. The TAA findings for O oculi and O oris in both MG cohorts were different from those obtained from the neurogenic control group.


CONCLUSIONS
Facial muscle atrophy in MuSK-MG patients is not neurogenic and the pathophysiological changes are akin to a myopathic process. The selected AChR-MG patients also show evidence of a similar pathophysiological process in the facial muscles albeit to a lesser degree.


CONCLUSIONS
We propose that muscle atrophy in MuSK-MG is a myopathic process consisting of either muscle fibre shrinkage or loss of muscle fibres from motor units. The duration of disease and long-term steroid treatment may be further contributory factors.
Farrugia
Maria E
ME
Department of Clinical Neurophysiology, The Radcliffe Infirmary, Oxford OX2 6HE, UK. m.e.farrugia@doctors.org.uk
Kennett
Robin P
RP
Hilton-Jones
David
D
Newsom-Davis
John
J
Vincent
Angela
A
D000200:Action Potentials; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D001905:Botulinum Toxins / Q000494:pharmacology; D004576:Electromyography / Q000379:methods*; D005152:Facial Muscles / Q000294:innervation / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons; D018485:Muscle Fibers, Skeletal / Q000276:immunology / Q000473:pathology; D009133:Muscular Atrophy / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology*; D009136:Muscular Dystrophies / Q000175:diagnosis / Q000503:physiopathology; D009157:Myasthenia Gravis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology*; D009469:Neuromuscular Junction / Q000503:physiopathology; D011237:Predictive Value of Tests; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology*; D011950:Receptors, Cholinergic / Q000276:immunology*; D011978:Receptors, Nicotinic / Q000276:immunology
D001323:Autoantibodies; D011950:Receptors, Cholinergic; D011978:Receptors, Nicotinic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases; D001905:Botulinum Toxins