Oculopharyngeal Syndrome (progressive Muscular
Dystrophy with Ptosis and Dysphagia;
Oculopharyngeal Muscular Dystrophy) 959b18j 959b18j 959b18j 959b18j
General: Etiology unknown; autosomal dominant inheritance; no CNS pathology; muscles of pharynx, hypopharynx, and proximal third of esophagus involved with myopathy; onset late in life; progressive hereditary myopathy in which the levator palpebrae and pharyngeal muscles are selectively involved; progressive usually symmetrical blepharoptosis with or without dysphagia appears in the fifth decade.
Ocular: Ptosis.
Clinical: Dysphagia; occasionally, weakness of facial muscles.
Codere F. Oculopharyngeal muscular dystrophy. Can J Ophthalmol 1993; 28: 1-2.
Duranceau A, Forand MD, Fauteaux JP. Surgery in oculopharyngeal muscular dystrophy. Am J Surg 1980; 139:33-39.
Jordan DR, Addison DJ. Surgical results and pathological findings in the oculopharyngeal dystrophy syndrome. Can J Ophthalmol 1993; 28:15-l8.
Molgat YM, Rodrigue D. Correction of blepharoptosis in oculopharyngeal muscular dystrophy: review of 91 cases. Can J Ophthalmol 1993; 28:11-l4.
Murphy SF, Drachman DB. The oculopharyngeal syndrome. JAMA 1968; 203:1003.
Taylor EW. Progressive glossopharyngeal paralysis with ptosis: contribution to a group of family diseases. J Nerv Ment Dis 1915; 42:129.
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