Progressive conduction defects and cardiac death in late infantile neuronal ceroid lipofuscinosis
Progressive conduction defects and cardiac death in late infantile neuronal ceroid lipofuscinosis
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DOI：
10.1111/j.1469-8749.2011.04170.x
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发表时间：
2012-07-01
影响因子：
3.8
通讯作者：
Takamisawa, Itaru
中科院分区：
文献类型：
--
作者：
Fukumura, Shinobu;Saito, Yoshiaki;Takamisawa, Itaru
本文报告一位患有晚期婴儿神经元蜡样质脂褐质沉积症的女性，在23岁时出现左右前束分支传导阻滞和阵发性心动过缓。在23岁和27岁时出现几次室上性心动过速发作。此外，在27岁时也出现了短暂的二度房室传导阻滞。房颤和房室传导阻滞加重导致28岁时进行性心动过缓和心源性死亡。在青少年神经元蜡样质脂褐质沉积症中，心肌和心脏传导系统中的脂肪色素的心脏受累和积累已被确认，但这是第一份报告描述晚期婴儿神经元蜡样质脂褐质沉积症病例中的进行性传导缺陷。
This article reports the case of a female with late infantile neuronal ceroid lipofuscinosis who developed right and left anterior bundle branch blocks and episodic bradycardia at 23 years of age. Several episodes of supraventricular tachycardia manifested at 23 and 27 years of age. In addition, a transient second-degree atrioventricular conduction block also emerged at 27 years of age. Atrial fibrillation and aggravation of the atrioventricular conduction block resulted in progressive bradycardia and cardiac death at the age of 28 years. Cardiac involvement and accumulation of lipopigments in the myocardium and cardiac conduction system have been recognized in juvenile neuronal ceroid lipofuscinosis, but this is the first report to describe progressive conduction defects in a case of late infantile neuronal ceroid lipofuscinosis.