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October 1931


Author Affiliations

With the Assistance of J. K. Miller and E. Janecek OMAHA

From the Department of Biochemistry and the University Hospital, University of Nebraska College of Medicine.

Arch Intern Med (Chic). 1931;48(4):569-575. doi:10.1001/archinte.1931.00150040035002

Myotonia atrophica or dystrophica is one of the rarer forms of myopathy, which, in addition to the typical myotonia, is characterized by the following conditions: (1) onset usually in the third decade; (2) limitation of the myotonia to special groups of muscles, namely, those of the hands, the tongue and the legs; (3) typical distribution of the atrophy to the face, the sternocleidomastoid muscles, the hand and the forearm and the muscles of the leg; (4) general emaciation; (5) baldness; (6) testicular atrophy; (7) cataract; (8) defect in speech; (9) combination with ataxia and Westphal's sign.1

The etiology of this disease is obscure. Naegeli2 regarded the pathogenesis of the disease as originating in a polyglandular disturbance, presumably with a marked hereditary predisposition. This relation to endocrine involvement was also assumed by Barkman3 and others, though the evidence for this is more circumstantial than direct. The occurrence of