Dysphagia in Oculopharyngeal Muscular Dystrophy

Report of a case

Abstract

This paper describes a case of oculopharyngeal muscular dystrophy. Patho-physiological aspects of and surgical treatments for dysphagia in this clinical entity are discussed.
The patient was a fifty-nine year-old male who had developed dysphagia and hypernasality in speech at the age of forty-nine. Deglutition of the patient was investigated with a use of simultaneous recordings of X-ray video and EMG. The muscles which participated in deglutition presented normal temporary relationship from kinesiological aspects. However, passage of barium in the pharynx was markedly delayed because of weakened propeling force. This asynchronism between muscular activity and passage of barium made the problems more complicated.
Cricopharyngeal myotomy was fairly effective. Pharyngeal flap operation caused an aggravation of dysphagia.