MRI Evaluation of the Brain in Infantile Neuronal Ceroid-Lipofuscinosis
MRI Evaluation of the Brain in Infantile Neuronal Ceroid-Lipofuscinosis
复制标题
婴儿神经元蜡质脂褐质沉着症脑部 MRI 评估
DOI：
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发表时间：
1995
影响因子：
1.9
通讯作者：
P. Santavuori
中科院分区：
文献类型：
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作者：
S. Vanhanen;R. Raininko;T. Autti;P. Santavuori
本研究的目的是用脑磁共振成像(MRI)显示3个月至11岁儿童神经元性蜡样脂褐素沉着症的病程。对21例患者和46例同龄神经科正常对照进行了检查。对图像进行视觉评估；然后测量信号强度并与参考文献的信号强度相关联。MRI异常在临床症状出现前即可被发现。脑部的放射影像随病程的不同而不同。本病早期MRI表现为广泛性脑萎缩，13个月以后T2加权像上白质和基底节丘脑呈强低信号，脑室周围高信号缘变薄。在4岁以上的患者中，大脑萎缩极度严重，整个白质的信号强度高于灰质，这与正常情况相反。这项研究表明，在生命的头4年，MRI上所见的异常迅速发展，然后稳定下来，这与婴儿神经性蜡样-脂褐素沉积症的临床和组织病理学特征相一致。(《儿童神经科杂志》1995；10：444-450)。
The purpose of this study was to demonstrate the course of infantile neuronal ceroid-lipofuscinosis with brain magnetic resonance imaging (MRI) in children aged 3 months to 11 years. Twenty-one patients and 46 neurologically normal controls of the same age were examined. The images were evaluated visually; then signal intensities were measured and related to those of references. MRI abnormalities were detectable before clinical symptoms. The radiologic picture of the brain varied with the duration of the disease. Pathognomonic MRI findings in the early stage of the disease were generalized cerebral atrophy, strong thalamic hypointensity to the white matter and to the basal ganglia, and thin periventricular high-signal rims from 13 months onward on T2-weighted images. In patients over 4 years old, cerebral atrophy was extreme, and the signal intensity of the entire white matter was higher than that of the gray matter, which is the reverse of normal. This study showed that the abnormalities seen on MRI progress rapidly during the first 4 years of life, then stabilize, in conformity with the clinical and histopathologic pictures of infantile neuronal ceroid-lipofuscinosis. (J Child Neurol 1995; 10:444-450).
神经元蜡质脂褐质沉积症婴儿晚期形式的新亚型。
影响因子：
1.4
作者：
Wisniewski,KE;Kida,E;Connell,F;Elleder,M;Eviatar,L;Konkol,RJ
通讯作者：
Konkol,RJ