MRI of neuronal ceroid lipofuscinosis. II. Postmortem MRI and histopathological study of the brain in 16 cases of neuronal ceroid lipofuscinosis of juvenile or late infantile type
MRI of neuronal ceroid lipofuscinosis. II. Postmortem MRI and histopathological study of the brain in 16 cases of neuronal ceroid lipofuscinosis of juvenile or late infantile type
复制标题
神经元蜡质脂褐质沉积症的 MRI。
作者：
T. Autti;R. Raininko;P. Santavuori;S. Vanhanen;V. Poutanen;M. Haltia
本文对14例幼年型和2例晚期婴儿型神经元蜡样质脂褐质沉积症（一种变异型，另一种经典型）进行了死后MRI检查。2例JNCL患者在生前也接受过MRI检查。MRI检查后，从大脑皮质、深核和白色物质的标准区域采集标本进行组织病理学分析。脑室周围白色物质的信号强度通常高于周围白色物质，这一发现与组织学观察到的严重脑室周围髓鞘丢失和胶质增生相关。丘脑的信号强度通常低于壳核;在某些患者中，丘脑的信号强度等于或甚至低于白色物质的信号强度。然而，髓鞘丢失，神经胶质增生，存储过程或丘脑中的神经元丢失与MRI结果无关。由于在1例JNCL患者中，生前和死后MRI基本上没有差异，因此在MRI上体内检测到的脑室周围变化可能是由于本研究中观察到的髓鞘严重缺失和神经胶质增生所致。然而，当尸检和体内MRI相关时，必须考虑固定过程引起的变化。
Abstract Postmortem MRI was carried out on the formalin-fixed brains of 14 patients with juvenile (JNCL) and two with late infantile neuronal ceroid lipofuscinosis, one of variant and the other of classical type. Two patients with JNCL had also undergone MRI during life. After MRI, specimens for histopathological analysis were taken from standard areas of the cerebral cortex, deep nuclei and white matter. The signal intensity of the periventricular white matter was usually higher than that of the peripheral white matter, a finding which correlated with the severe periventricular loss of myelin and gliosis observed histologically. The signal intensity was usually lower in the thalamus than in the putamen; in some patients the signal intensity of the thalamus was equal to or even lower than that of the white matter. However, myelin loss, gliosis, the storage process or neuronal loss in the thalamus did not correlate with the MRI findings. Since in one patient with JNCL the ante- and postmortem MRI did not differ basically, it appears probable that the periventricular changes detected in vivo on MRI are due to the severe loss of myelin and gliosis observed in this study. However, changes resulting from the fixation process must be considered, when postmortem and in vivo MRI are correlated.
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
神经元蜡样质脂褐质沉积症亚型的临床分类。
DOI：
10.1002/ajmg.1320310608
发表时间：
1988
期刊：
American journal of medical genetics. Supplement
影响因子：
--
作者：
Boustany,RM;Alroy,J;Kolodny,EH
通讯作者：
Kolodny,EH
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：
--
发表时间：
1990
期刊：
AJNR. American journal of neuroradiology
影响因子：
--
作者：
Grafe,MR;Press,GA;Berthoty,DP;Hesselink,JR;Wiley,CA
通讯作者：
Wiley,CA