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Progressive conduction defects and cardiac death in late infantile neuronal ceroid lipofuscinosis
Author(s) -
FUKUMURA SHINOBU,
SAITO YOSHIAKI,
SAITO TAKASHI,
KOMAKI HIROFUMI,
NAKAGAWA EIJI,
SUGAI KENJI,
SASAKI MASAYUKI,
OKA AKIRA,
TAKAMISAWA ITARU
Publication year - 2012
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/j.1469-8749.2011.04170.x
Subject(s) - medicine , cardiology , bradycardia , neuronal ceroid lipofuscinosis , electrical conduction system of the heart , electrocardiography , heart rate , disease , blood pressure
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.