Cardiovascular observations in dystrophia myotonica.

Abstract

Dystrophia myotonica (or myotonia atrophica) is a progressive, generalized disease characterized chiefly by atrophy of skeletal muscles in a characteristic distribution, with associated myotonia. Other features include cataract, frontal baldness, gonadal atrophy and depression of the basal metabolic rate. The disease has a definite hereditary tendency. Excellent discussions of its symptomatology, pathology and clinical features are given by Waring, Ravin and Walker, 1 and by Maas and Paterson. 2 Among the findings in this disorder, abnormalities in the cardiovascular system have received scant attention in the American literature. On the other hand, the European literature contains a number of reports emphasizing cardiac abnormalities. Because of the rarity of this disorder it is not possible for one observer to study enough cases to discover their true incidence. It is our purpose to summarize the cardiac observations found in the literature, with particular emphasis on electrocardiographic observations, and to present the cardiovascular