TY - JOUR
T1 - Kearns-Sayre syndrome
T2 - electro-vectorcardiographic evolution for left septal fascicular block of the his bundle
AU - Pérez Riera, Andrés Ricardo
AU - Kaiser, Elisabeth
AU - Levine, Paul
AU - Schapachnik, Edgardo
AU - Dubner, Sergio
AU - Ferreira, Celso
AU - Filho, Celso Ferreira
AU - Bayés de Luna, Antoni
AU - Zhang, Li
N1 - 1 Department Electro-Vectorcardiographic Section, Cardiology Division, ABC's, Medical School, ABC Foundation, Santo André, São Paulo, Brazil. [email protected] 1 Department Electro-Vectorcardiographic Section, Cardiology Division, ABC's, Medical School, ABC Foundation, Santo André, São Paulo, Brazil. [email protected] The Kearns-Sayre syndrome is a neuromyopathic disorder associated with mitochondrial abnormalities and characterized by the triad of chronic external ophthalmoplegia, atypical pigmentary retinopathy, and progressive conduction system disorders.
PY - 2008/11
Y1 - 2008/11
N2 - The Kearns-Sayre syndrome is a neuromyopathic disorder associated with mitochondrial abnormalities and characterized by the triad of chronic external ophthalmoplegia, atypical pigmentary retinopathy, and progressive conduction system disorders. Ragged red muscle fibers that seem to contain an excess of altered mitochondria are observed. The disease affects both sexes alike, during the first or the second decade of life. The following manifestations are observed: central bilateral sensorineural deafness, pyramidal signs, ataxia, asymmetrical ptosis, external ophthalmoplegia, and progressive muscular weakness secondary to myopathy associated with a significant increase of proteins of cephalorachidian liquid. A variety of endocrinopathies may occur.
AB - The Kearns-Sayre syndrome is a neuromyopathic disorder associated with mitochondrial abnormalities and characterized by the triad of chronic external ophthalmoplegia, atypical pigmentary retinopathy, and progressive conduction system disorders. Ragged red muscle fibers that seem to contain an excess of altered mitochondria are observed. The disease affects both sexes alike, during the first or the second decade of life. The following manifestations are observed: central bilateral sensorineural deafness, pyramidal signs, ataxia, asymmetrical ptosis, external ophthalmoplegia, and progressive muscular weakness secondary to myopathy associated with a significant increase of proteins of cephalorachidian liquid. A variety of endocrinopathies may occur.
KW - Fascicular blocks
KW - Kearns-Sayre syndrome
KW - Left septal fascicular block
KW - Prominent anterior forces
KW - Trifascicular block
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U2 - 10.1016/j.jelectrocard.2008.04.001
DO - 10.1016/j.jelectrocard.2008.04.001
M3 - Article
C2 - 18490026
SN - 0022-0736
VL - 41
SP - 675
EP - 678
JO - Journal of Electrocardiology
JF - Journal of Electrocardiology
IS - 6
ER -