Late-onset muscle dystrophy: oculopharyngoesophageal variety.

Oculopharyngeal muscular dystrophy is a localized or restricted variety of muscular dystrophy, characterized by bilateral ptosis, myopathic facies, external ophthalmoplegia and dysphagia. A patient with this unusual myopathy is described and detailed esophageal motility studies are presented that provide conclusive evidence of both striated and smooth muscle involvement.Because these unusual localized forms of muscular dystrophy exist and may be of late onset, previous rigid concepts concerning the dystrophies should be discarded.