Esophageal manometry in oculopharyngeal dystrophy.

Four cases of oculopharyngeal dystrophy (OPD) seen within five years are presented. The cardinal features of OPD are dysphagia and ptosis. The manometric abnormalities in OPD are similar to those seen in other myopathies; low pharyngeal pressures, abnormalities in relaxation and coordination of the pharyngoesophageal sphincter and weak aperistaltic swallows in the proximal esophagus. In addition, weak aperistaltic swallows frequently occur in the distal esophagus and may exacerbate dysphagia. Although there is no specific therapy for ODP, one patient showed marked improvement following cricopharyngeal myotomy.