Key result
Cold exposure during electromyography induced a significant fall in CMAP amplitude and decremental response to 2 Hz stimulation in paramyotonia congenita, distinguishing it from myotonia congenita.
Why the study?
Can electromyography distinguish between paramyotonia congenita and myotonia congenita?
Population
4 patients: 2 with paramyotonia congenita and 2 with dominantly inherited myotonia congenita
Comparison
Electromyography including 2 Hz nerve… vs Comparison between paramyotonia congenita and…
Design
Case_series
Authors
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May aid differentiation of paramyotonia congenita from myotonia congenita via cold-provoked EMG; hypothesis-generating and requires prospective validation.
Observational (n=4)
Can electromyography distinguish between paramyotonia congenita and myotonia congenita?
Electromyography with temperature variations and exercise can effectively distinguish paramyotonia congenita from myotonia congenita.
Subramony et al. (1983) conducted an observational in Paramyotonia congenita and myotonia congenita (n=4). Cold (20 degrees C) state during electromyography vs. Warm (34 degrees C) state and patients with myotonia congenita was evaluated on CMAP amplitudes, response to 2 Hz nerve stimulation, response to exercise and electromyographic needle electrode examination findings. Cold exposure during electromyography induced a significant fall in CMAP amplitude and decremental response to 2 Hz stimulation in paramyotonia congenita, distinguishing it from myotonia congenita.
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