Paramyotonia congenita: successful treatment with tocainide. Clinical and electrophysiologic findings in seven patients

Abstract

Seven patients with paramyotonia congenita (PC) from two families were studied. Voluntary exercise of the hand muscles was performed at different hand temperatures, both before and after treatment with tocainide. All patients developed stiffness, prolonged weakness, and small compound muscle action potentials (CMAPs) following exercise; the temperature at which this occurred was individually different. Two patients with PC and associated episodes of generalized weakness underwent potassium loading. A prolonged exercise test was performed both immediately before and 90 minutes after K-loading. Exercise-induced weakness and CMAP-decline occurred only with high serum K levels. Thiazide treatment in these two patients was ineffective. All seven patients responded well to tocainide. Treatment response and side effects were dose-dependent. Good clinical improvement has been maintained in all patients for more than 6 months, with relatively small doses of tocainide (400-1200 mg/day).