The nosologic distinction between paramyotonia congenita and hyperkalemic periodic paralysis is somewhat blurred. Muscle membrane inexcitability induced by cooling seems to be characteristic of paramyotonia congenita. The effect of cooling on the maximal compound muscle action potential (CMAP) in patients with paramyotonia congenita was compared to that in patients with hyperkalemic periodic paralysis. Diminution in CMAP amplitude and area, which was observed in paramyotonia congenita, did not occur in hyperkalemic periodic paralysis. We suggest that this effect of cooling on the CMAP can be utilized in the differentiation of these two syndromes.
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