Electromyographic evidence of subclinical myopathy in hypertrophic cardiomyopathy.
Hypertrophic cardiomyopathy (HCM) is due to a number of mutations of contractile protein genes such as beta-cardiac myosin, myosin binding protein-C, and troponin-T. Unlike troponin-T, beta-myosin is a constituent of slow skeletal muscle and its mutations generally have a better prognosis. In order to investigate the usefulness of electromyography in detecting skeletal muscle involvement in HCM, 46 patients were examined using both conventional electromyography (EMG) and quantitative electromyography (QEMG) methods. The QEMG involved motor unit potential (MUP) analysis, turns/amplitude (TAA) analysis, and power spectrum analysis of the interference pattern. Using conventional EMG, myopathic findings were demonstrated in 13 patients (28%). Receiver operating characteristic (ROC) analysis of the results of a discriminant function extracted using QEMG values, identified correctly 10 out of 11 normal controls and all 9 myopathic control patients, and displayed a 15% presence of myopathy (7 patients) among the cardiomyopathy group. The duration of MUPs was the most sensitive among the quantitative parameters in differentiating normal from myopathic subjects. Since skeletal muscle involvement may be due to distinct gene mutations, normal and myopathic EMG findings may reflect HCM subpopulations with a different genetic substrate.
Karandreas
N
N
EMG Laboratory, Department of Neurology, Aeginition Hospital, University of Athens, Vas. Sofias 72-74, 11528 Athens, Greece. nekaran@hotmail.com
Stathis
P
P
Anastasakis
A
A
Rigopoulos
A
A
Piperos
P
P
Theopistou
A
A
Stefanadis
C
C
Toutouzas
P
P
D000293:Adolescent; D000328:Adult; D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000503:physiopathology*; D004576:Electromyography / Q000379:methods*; D019054:Evoked Potentials, Motor; D005189:False Positive Reactions; D006801:Humans; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology*; D009135:Muscular Diseases / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D015203:Reproducibility of Results