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November 1940


Author Affiliations

From the Department of Ophthalmology and the Department of Neurology, State University of Iowa, College of Medicine.

Arch Ophthalmol. 1940;24(5):867-884. doi:10.1001/archopht.1940.00870050017001

After Erb's classic description of Thomsen's disease in 1886, several so-called atypical cases (Thomsen's disease with muscular atrophy) were described sporadically. Deleage reported the first of these in 1890. But it remained for Batten and Gibb 1 and Steinert2 in 1909 to correlate these reports with cases of their own and to describe myotonia atrophica, a disease entity distinct from other neuromuscular maladies. The chief interest in the syndrome centered about the myotonia and atrophy, with the extramuscular symptoms being considered incidental. However, after Greenfield,3 Kennedy and Oberndorf 4 and Ormond 5 had described cataracts in some of their cases, Curschmann 6 recognized the significance of the extramuscular manifestations and proposed the name dystrophia myotonica for the disease. Subsequent reports, made principally in the European literature,7 have brought the total number of cases to approximately 300.