Clinical study of paramyotonia congenita with and without myotonia in a warm environment

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Abstract
Fourteen patients with paramyotonia congenita were examined clinically. Patients of 3 farnilies had no myotonia in a warm environment while in a cold environment they developed paradoxical myotonia (myotonia aggravated by repeated muscle contraction). Patients of a 4th family had myotonia associated with after‐activity in a warm environment which was not paradoxical. This myotonia was aggravated by cooling. In a warm environment the resting muscles of all patients showed no spontaneous electromyographic activity except for occasional myotonic runs. On cooling, spontaneous fibrillations developed. This was most intense at 32°C–28°C (muscle temperature). On deeper cooling it ceased. In contrast, 5 patients with myotonia congenita did not show such activity during cooling. In all paramyotonic patients cooling (30°C–25°C) produced muscle paralysis, which outlasted rewarming by several hours. At 32°C–30°C muscle relaxation was slowed. Recording of electromyographic activity and isometric contractions of the long finger flexors during cooling revealed that the slowing of muscle relaxation in paramyotonia is not as closely linked to after–activity as is the slowing of muscle relaxation in myotonia congenita.

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