MRI Evaluation of the Brain in Infantile Neuronal Ceroid-Lipofuscinosis. Part 1: Postmortem MRI With Histopathologic Correlation
MRI Evaluation of the Brain in Infantile Neuronal Ceroid-Lipofuscinosis. Part 1: Postmortem MRI With Histopathologic Correlation
复制标题
婴儿神经元蜡质脂褐质沉着症大脑的 MRI 评估。
作者：
S. Vanhanen;R. Raininko;P. Santavuori;T. Autti;M. Haltia
本研究的目的是关联死后的磁共振成像（MRI）与一系列尸检的婴儿神经元蜡样脂褐质沉积症，一个复发性遗传性进行性脑病患者的大脑组织病理学结果。八福尔马林固定的大脑（死亡年龄范围，7至13岁）进行了MRI检查。1例患者在死亡前2年还接受了脑部MRI。根据MRI扫描选择的标准区域进行组织学分析。尸检MRI结果与患者生前检查结果无显著差异。典型的表现是脑萎缩和T2加权像上相对于白色物质的灰质结构的低信号，这是一种与正常相反的模式。特征性组织学检查结果是皮质神经元几乎完全丧失，白色物质中的轴突和髓鞘继发性丧失。在MRI扫描上，灰质和白质结构的相对强度急剧变化，反映了神经元被充满储存物质的肥大星形胶质细胞和/或巨噬细胞所取代。（J Child Neurol 1995;10：438-443）。
The purpose of this study was to correlate postmortem magnetic resonance imaging (MRI) with histopathologic findings in brains of a series of autopsied patients with infantile neuronal ceroid-lipofuscinosis, a recessively inherited progressive encephalopathy. Eight formalin-fixed brains (age range at death, 7 to 13 years) were examined with MRI. One patient had also undergone brain MRI 2 years before death. Histopathologic analyses were made from standard areas selected on the basis of the MRI scans. Postmortem MRI findings did not differ significantly from the findings in the patient who was also examined during life. Typical findings were extreme cerebral atrophy and hypointensity of the gray-matter structures in relation to the white matter on T2-weighted images, a pattern the reverse of normal. Characteristic histologic findings were almost complete loss of cortical neurons and secondary loss of axons and myelin sheaths in the white matter. The drastically altered relative intensities of the gray- and white-matter structures on the MRI scans reflected replacement of the neurons with hypertrophic astrocytes and/or macrophages filled with storage material. (J Child Neurol 1995;10:438-443).
DOI：
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发表时间：
1986
期刊：
The Journal of biological chemistry
影响因子：
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作者：
Palmer,DN;Husbands,DR;Winter,PJ;Blunt,JW;Jolly,RD
通讯作者：
Jolly,RD
Rosenthal 纤维主要蛋白质成分的分离。
DOI：
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发表时间：
1988
期刊：
The American journal of pathology
影响因子：
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作者：
Goldman,JE;Corbin,E
通讯作者：
Corbin,E
艾滋病患者大脑异常：死后 MR 结果与神经病理学的相关性。
DOI：
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发表时间：
1990
期刊：
AJNR. American journal of neuroradiology
影响因子：
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作者：
Grafe,MR;Press,GA;Berthoty,DP;Hesselink,JR;Wiley,CA
通讯作者：
Wiley,CA