Progressive Muscular Dystrophy With Ptosis and Dysphagia

In December, 1962, Victor, Hayes, and Adams9called attention to several patients with a unique combination of progressive dysphagia and ptosis which they appropriately named oculopharyngeal muscular dystrophy. As noted by these authors, Taylor,8in 1915, published a study of a French-Canadian family in which a number of members, all over 50 years of age, became affected with progressive ptosis and dysphagia, which he erroneously ascribed to "degeneration" of cranial nerve nuclei. In 1948 Amyot1described a similar condition in a number of patients, also of French-Canadian descent, in whom there was a familial incidence of ptosis and dysphagia of late onset and progressive severity. He suggested that the ptosis was due to myopathy of the levator muscles. Whereas a number of previous authors had considered progressive ptosis and extraocular muscle paresis to be due to degeneration of cranial nerve nuclei, Kiloh and Nevin,5in 1951