Dysphagia in oculopharyngeal muscular dystrophy: a series of 22 French cases.

Twenty-two patients (mean age = 67.9 years) with oculopharyngeal muscular dystrophy (OPMD) were referred for dysphagia from 1987 to January 1995. Six patients had suffered aspiration pneumonia, and three had significantly lost weight, while 19 complained of discomfort during swallowing but without weight loss. Swallowing was assessed by fiberscopy during swallowing (last eight patients), videofluoroscopy (12 cases) and manometry (19 cases). Twelve patients underwent a cricopharyngeal (CP) myotomy: 10 showed improvement, one had a partial improvement, and the procedure failed in one (mean follow-up = 29.6 months). In the other cases, CP myotomy was postponed, refused or contraindicated. Of the 22 patients, three died from OPMD consequences. Factors associated with favorable outcome were adequate residual pharyngeal propulsion and no weight loss. In a majority of cases, CP myotomy constitutes an effective treatment of dysphagia with adequate residual propulsion but does not modify the final prognosis and is contraindicated in cases with pharyngeal aperistalsis.