Definition of Kearns-Sayre syndrome Kearns-Sayre syndrome: A
neuromuscular disorder characterized by three primary findings:
Kearns-Sayre syndrome is one of the mitochondrial
encephalomyopathies. These disorders are due to defects in the
DNA of the mitochondria, the cell structures that produce
energy. These defects cause the brain and muscles to function
abnormally (encephalomyopathy). In about 80% of cases of Kearns-
Sayre syndrome, tests reveal deletions in mitochondrial DNA
(mtDNA). There are many other names for the Kearns-Sayre
syndrome including: CPEO with myopathy; CPEO with ragged-red
fibers; KSS; Mitochondrial cytopathy, Kearns-Sayre type;
Oculocraniosomatic syndrome; Ophthalmoplegia-plus syndrome;
Ophthalmoplegia with myopathy; and Ophthalmoplegia with ragged-
red fibers.Source: MedTerms™ Medical Dictionary Last Editorial
Review: 6/14/2012 Medical Dictionary Definitions A - Z Search
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