Kobayashi Shunsuke, Saito Yuko, Maki Toshiyuki, Murayama Shigeo
Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Clin Neurol Neurosurg. 2010 Jul;112(6):520-3. doi: 10.1016/j.clineuro.2010.03.015. Epub 2010 Apr 20.
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.
一名患有朊蛋白（PrP）基因密码子180突变的克雅氏病（CJD 180）患者在死前1.5年期间出现认知功能衰退。系列磁共振成像（MRI）研究追踪了皮质异常肿胀和T2延长的逐步扩散情况。尸检时，受CJD影响相对较短时间的皮质区域伴有轻度海绵状改变，神经元数量基本保留。这些区域的残留神经元表现出细胞体的空泡样扩张。相比之下，受CJD影响较长时间的萎缩性皮质区域则以肥胖型星形细胞增生为主，伴有严重的神经元丢失。本报告描述了CJD 180独特的皮质扩散情况以及相应的放射学和病理学发现。通过皮质 - 皮质连接的轴突运输可能是该疾病扩散的基础。MRI似乎有助于区分不同的病理状态并追踪CJD 180的进展。
