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Oculopharyngeal syndrome (progressive muscular dystrophy with ptosis and dysphagia; oculopharyngeal muscular dystrophy)


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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