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February 1967

The Heart in Myotonia Atrophica

Author Affiliations


From the Division of Cardiology, University of Washington School of Medicine, Seattle. Dr. Church was formerly associated with the Department of Medicine, University Hospital, Columbus, Ohio.

Arch Intern Med. 1967;119(2):176-181. doi:10.1001/archinte.1967.00290200100008

D ÉLÈAGE,1 in 1890, was the first to describe a case of myotonia atrophica as a distinct clinical entity; but it was Rossolimo,2 in 1902, who first associated myotonia with muscular atrophy, giving the disease its present name. Batten and Gibb3 simultaneously with Steinert,4 in 1909, clarified the disease complex and separated it from other forms of myotonia. Myotonia atrophica is a hereditary disease which characteristically occurs in the third or fourth decade and progresses slowly thereafter until death in the sixth or seventh decade. The most constant course is myotonia, a tonic spasm of muscle, followed by atrophy. The myotonia is usually demonstrable by voluntary, mechanical, or electrical means in the muscles of the forearms, hands, tongue, and jaw. Myotonia occurs when the muscle is strongly stimulated as in a tight grip; it disappears with repetition of the stimulus; and it is intensified by cold. When the patient is

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