Distinguishing paramyotonia congenita and myotonia congenita by electromyography

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Abstract
Compound muscle action potential (CMAP) amplitudes, response to 2 Hz nerve stimulation, response to exercise and electromyographic needle electrode examination findings from the thenar muscles of two patients with paramyotonia congenita were compared with those from two patients with dominantly inherited myotonia congenita in warm (34°C) and cold (20°C) states. Cold induced a significant fall in CMAP amplitude, induced/worsened a significant decremental response to 2 Hz stimulation, and virtually abolished myotonia and voluntary recruitment of motor unit potentials in patients with paramyotonia congenita; none of these occurred in myotonia congenita. Though exercise induced a mild fall in CMAP amplitude in both groups, postexercise fibrillations occurred only in patients with paramyotonia congenita. These findings serve to distinguish these two entities in the clinical electromyography laboratory.