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Kearns-Sayre syndrome (KSS) is a rare multisystem mitochondrial disorder associated with progressive external ophthalmoplegia, atypical pigmentary degeneration of the retina, and complete heart block. Kearns-Sayre Syndrome. The rare Kearns-Sayre syndrome (MIM 530000), also known as oculocraniosomatic disease or oculocraniosomatic neuromuscular disease with ragged red fibers, is characterized by myopathic abnormalities leading to ophthalmoplegia and progressive weakness in association with several endocrine abnormalities, including Cardiac Involvement in Kearns-Sayre Syndrome Article (PDF Available) in Revista Espa de Cardiologia 58(4):443-6 · May 2005 with 83 Reads How we measure 'reads' Le syndrome de Kearns et Sayre (KSS) fut decrit pour la premiere fois en 1958. Il s'agit d'une atteinte multisystemique definie par une triade caracteristique faite d'ophtalmoplegie externe progressive, de retinopathie pigmentaire et de bloc auriculoventriculaire. INSUFICIENCIA CARDIACA Vol. 6, N? 2, 2011 102 H de Castro Junior et al. Kearns-Sayre syndrome pacemaker implantation of a bicameral type. He moved to the floor on the third day of hospitalization and was Kearns-Sayre syndrome (KSS) is a rare mitochondrial cytopathy, first described at Mayo Clinic in 1958.1 KSS belongs to a group of mitochondrial DNA (mtDNA) deletion syndromes that also includes Pearson syndrome and progressive external ophthalmoplegia (PEO).2 Classically, KSS has a triad of features, including presence of PEO, pigmentary Kearns-sayre syndrome Kearns-sayre syndrome is when the mitochondria in DNA becomes abnormal, and does not do its proper fucntions. Slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Cochlear implant in Kearns-Sayre syndrome: case study of twin sisters Implante coclear na sindrome Kearns-Sayre: estudo de caso de irmas gemeas Leticia Sampaio de Oliveira1, Karina Costa Brosco2, Eduardo Boaventura Oliveira3, Katia de Freitas Alvarenga4 ABSTRACT Hearing impairment may be related to several factors, including hearing A 47-year-old woman with Kearns-Sayre syndrome (KSS) and an implanted pacemaker for complete heart block was admitted to the intensive care unit following a cardiac arrest due to ventricular tachycardia (torsade de pointes) in the setting of QT prolongation. Complete heart blocks and ventricular tachycardia are implicated as mechanisms of sudden deaths in KSS; such patients may require Kearns?Sayre syndrome (KSS) is a rare genetic abnormality. Classified as a mitochondrial cytopathy, the primary pathology of this syndrome is a disturbance of mitochondrial DNA, which codes for the proteins required for the respiratory chain reaction.