Kearns-Sayre syndrome (kûrnz sâr)n. A syndrome usually occurring before the age of 20 and characterized by pigmentary retinal dystrophy, cardiomyopathy, and varied cranial nerve impairment. |
Kearns-Sayre syndrome
[kernz sār]
Etymology: Thomas P. Kearns, American ophthalmologist, b. 1922; George P. Sayre, American pathologist, b. 1911
progressive ophthalmoplegia, pigmentary degeneration of the retina, myopathy, ataxia, and cardiac conduction defect, with onset before the age of 20 years. Almost all patients have large mitochondrial DNA deletions, and ragged red fibers are seen on muscle biopsy. Also called ophthalmoplegia plus.
Kearns-Sayre syndrome
A mitochondrial disease characterized by chronic progressive external ophthalmoplegia–paralysis of ocular muscles and mitochondrial myopathy combined with retinal deterioration, heart disease, hearing loss, DM,
renal disease See Mitochondrial disease.
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