Kearns-Sayre syndrome


Kearns-Sayre syn·drome

(kernz sār), [MIM*530000] a form of chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss; a sporadically occurring mitochondrial myopathy presenting in childhood. Synonym(s): ragged red fiber myopathy

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.

Kearns-Sayre syn·drome

(kĕrnz sār sindrōm) A form of chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss; a sporadically occurring mitochondrial myopathy presenting in childhood.

Kearns-Sayre syndrome (KSS)

A syndrome caused by major rearrangements of, and often large deletions from, the MITOCHONDRIAL DNA. The condition features paralysis of the eye-moving muscles with double vision; drooping eyelids; degeneration of the retinas; defects in the conducting muscle tissue of the heart; respiratory distress; and in some cases staggering walk, deafness and DIABETES.

Kearns,

Thomas P., U.S. ophthalmologist, 1922–. Kearns-Sayre syndrome - chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss.

Sayre,

George P., U.S. ophthalmologist, 1911–. Kearns-Sayre syndrome - see under Kearns

Kearns-Sayre syn·drome

(kĕrnz sār sindrōm) [MIM*530000] Chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss.