Cortical propagation of Creutzfeldt-Jakob disease with codon 180 mutation
Cortical propagation of Creutzfeldt-Jakob disease with codon 180 mutation
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DOI：
10.1016/j.clineuro.2010.03.015
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发表时间：
2010-07-01
影响因子：
1.9
通讯作者：
Murayama, Shigeo
中科院分区：
文献类型：
--
作者：
Kobayashi, Shunsuke;Saito, Yuko;Murayama, Shigeo
一名患有克雅氏病（CJD）的朊蛋白（PrP）基因密码子180突变（CJD 180）患者在去世前的1.5年内出现认知功能下降。系列磁共振成像（MRI）研究跟踪逐步传播的皮质异常肿胀和T2 elongations.On尸检检查，皮质区受CJD影响的相对较短的时间与轻度海绵状变化与神经元的数量被大量保存。残留神经元胞体呈空泡样扩张。与此相反，萎缩的皮质区受CJD的长期表现出主要的双生细胞星形胶质细胞增生与严重的神经元损失。本报告描述了独特的皮质传播的CJD 180与相应的放射学和病理结果。通过皮质连接的轴突运输可能是疾病传播的基础。磁共振成像似乎是有用的不同病理状态之间的区别和跟踪CJD 180的进展。(C)2010 Elsevier B.V.保留所有权利。
A patient with Creutzfeldt-Jakob disease (CJD) with prion protein (PrP) gene codon 180 mutation (CJD 180) experienced cognitive decline over the 1.5-year period before her death. Serial magnetic resonance imaging (MRI) studies tracked stepwise propagation of cortical abnormal swelling and T2 elongations.On postmortem examination, the cortical areas affected by CJD for relatively short periods were associated with mild spongiform changes with the number of neurons being largely preserved. The residual neurons in these areas exhibited vacuole-like dilatation of their cell body. In contrast, the atrophic cortical areas affected by CJD for long periods exhibited predominant gemistocytic astrocytosis with severe neuronal loss. The present report depicts the unique cortical propagation of CJD 180 with corresponding radiological and pathological findings. Axonal transport through corticocortical connections might underlie the disease's propagation. MRI appeared to be useful for discriminating between different pathological states and tracking the progression of CJD 180. (C) 2010 Elsevier B.V. All rights reserved.