[Cardiological aspect of Kearns' syndrome. Apropos of 3 cases, with histopathological study of the conduction tissue in one of them].
Three cases of Kearns syndrome are reported. The neuro-ophthalmological signs were comparable with ophthalmoplegia, pigmentary retinal degeneration and polymorphic neuro-muscular and sensory deficits. The electrocardiological signs were observed 2-4 years after the onset of the condition; the cause of death in each case was related to complete heart block. The post-mortem findings in one of the cases were spongial degeneration of the central nervous system and a seemingly primitive degeneration of the Bundle of His and its branches.
D000293:Adolescent; D000328:Adult; D002490:Central Nervous System / Q000473:pathology; D004562:Electrocardiography; D006327:Heart Block / Q000175:diagnosis / Q000473:pathology*; D006329:Heart Conduction System / Q000473:pathology*; D006801:Humans; D008297:Male; D009886:Ophthalmoplegia / Q000473:pathology*; D012174:Retinitis Pigmentosa / Q000473:pathology*; D013577:Syndrome
Aspect cardiologique du syndrome de Kearns. A propos de 3 cas dont 1 avec étude histo-pathologique du tissu de conduction.