Repetitive nerve stimulation of facial muscles in MuSK antibody-positive myasthenia gravis.
To better define electrophysiological abnormalities in myasthenia gravis (MG) patients with muscle-specific tyrosine kinase (MuSK) antibodies (Ab), we compared electrophysiological features of 14 MuSK Ab-positive, 73 acetylcholine receptor antibody (AChR Ab)-positive, and 22 MuSK and AChR Ab-negative (seronegative) patients with generalized disease. Repetitive nerve stimulation (RNS) abnormalities were observed in 86% of MuSK Ab-positive and 82% of AChR Ab-positive patients but in only 55% of seronegative patients. RNS decrements in the orbicularis oculi were more common and severe in the MuSK Ab-positive patients than the other two groups. Single-fiber electromyography (SFEMG) of the extensor digitorum communis was abnormal in 90% of MuSK Ab-positive patients. The high frequency of RNS abnormalities in facial muscles in the MuSK Ab-positive population reflects the propensity for facial muscle involvement in this form of MG and emphasizes the importance of including facial muscles in RNS protocols when evaluating these patients.
Oh
Shin J
SJ
Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama, USA. shinjoh@uab.edu
Hatanaka
Yuki
Y
Hemmi
Shoji
S
Young
Angela M
AM
Scheufele
Mia L
ML
Nations
Sharon P
SP
Lu
Liang
L
Claussen
Gwen C
GC
Wolfe
Gil I
GI
D000200:Action Potentials / Q000502:physiology; D000328:Adult; D000906:Antibodies / Q000032:analysis; D004558:Electric Stimulation; D004594:Electrophysiology; D005152:Facial Muscles / Q000294:innervation* / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D018482:Muscle, Skeletal / Q000294:innervation / Q000503:physiopathology; D009157:Myasthenia Gravis / Q000235:genetics* / Q000503:physiopathology; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics* / Q000276:immunology / Q000502:physiology; D011950:Receptors, Cholinergic / Q000235:genetics* / Q000276:immunology / Q000502:physiology
D000906:Antibodies; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases