The pharynx and esophagus in ocular muscular dystrophy

THE TOPOGRAPHIC PATTERN of clinically demonstrable muscle involvement remains an essential feature in classifying the muscular dystrophies.l-7 Occasionally, it is questionable whether variation in the distribution of muscle involvement in the phenotype reflects genetic heterogeneity or provides a useful basis for further subdivision within the classical syndromes. The significance of dysphagia in ocular myopathy has recently been debated and these questions raised.% This unusual association of muscle weakness in a rare muscular dystrophy seemed to merit further study. The following investigation was designed to determine the cause of the dysphagia and establish the incidence and discriminatory value in classification of involvement of the muscles of swallowing in ocular muscular dystrophy.