calcimycin and Myotonia

Platelet function tests were carried out on 21 patients with myotonic dystrophy (MyD) and 7 patients with myotonia congenita (MC) together with 22 healthy subjects. Compared to the controls, the MyD and MC patients showed significantly greater sensitivity to the calcium ionophore A23187 but not to adenosine diphosphate, collagen, ristocetin or adrenaline. In addition, with the MyD patients, a significantly higher concentration of chlorpromazine and lignocaine was required to inhibit aggregation induced by a standard dose of adrenaline. A similar trend was noted with the MC patients but because of the small numbers tested, statistical analysis was not possible. Plasma levels of betathromboglobulin were also significantly higher in MyD patients than in controls. Platelet adenine nucleotide levels were within the normal range in both groups of patients and were not significantly different from those in controls. These preliminary results support the view that there is a defect in calcium metabolism in myotonic dystrophy and suggest that it may be possible to use the blood platelet as a model with which to carry out further studies in this disorder.

Topics: Adenosine Diphosphate; Adenosine Triphosphate; Adult; beta-Thromboglobulin; Blood Platelets; Calcimycin; Chlorpromazine; Epinephrine; Female; Humans; Lidocaine; Male; Middle Aged; Myotonia; Platelet Aggregation; Platelet Factor 4