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[卡恩斯综合征的心脏方面。关于3例报告,其中1例对传导组织进行了组织病理学研究]

[Cardiological aspect of Kearns' syndrome. Apropos of 3 cases, with histopathological study of the conduction tissue in one of them].

作者信息

Voisin M, Baissus C, Grolleau-Raoux R, Dumas R, Jean R

出版信息

Arch Mal Coeur Vaiss. 1979 May;72(5):521-8.

PMID:115405
Abstract

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.

摘要

报告了3例卡恩斯综合征病例。神经眼科体征与眼肌麻痹、色素性视网膜变性以及多形性神经肌肉和感觉缺陷相似。心电体征在病情发作后2至4年出现;每例的死亡原因均与完全性心脏传导阻滞有关。其中1例的尸检结果为中枢神经系统海绵状变性以及希氏束及其分支看似原始的变性。

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