Serial diffusion-weighted, voxel-based morphometry MRI, and 123I-IMP SPECT in V180I genetic Creutzfeldt–Jakob disease before symptom onset

Medical Reports Pub Date : 2024-05-29 DOI:10.1016/j.hmedic.2024.100072

Masakazu Ozawa , Keisuke Shibata , Keiko Toyoda , Tomomichi Kitagawa , Masako Ikeda , Renpei Sengoku

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Abstract

An 84-year-old genetic Creutzfeldt–Jakob disease with V180I mutation in the prion protein gene (V180I gCJD) woman presented with rapid progressive dementia. Six months before symptom onset, diffusion-weighted imaging (DWI) and T2-weighted imaging (T2WI) were normal; however, voxel-based morphometry (VBM) evidenced volume loss of the right temporal pole and hippocampus. Two months after symptom onset, brain imaging revealed cortical DWI hyperintensities with swelling on T2WI, and hypoperfusion on ¹²³I-IMP single photon emission computed tomography in the right dominant bilateral temporal lobes, and right front-parietal lobes, striatum, and parahippocampus. The VBM loss had spread across the right temporal lobe and hippocampus. Considering spongiform changes and neuron loss, these imagings were useful for understanding the neuropathological mechanisms of V180I gCJD.

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V180I 遗传性克雅氏病患者症状出现前的连续弥散加权磁共振成像、基于体素的形态计量磁共振成像和 123I-IMP SPECT

一名 84 岁的遗传性克雅氏病患者因朊病毒蛋白基因 V180I 突变（V180I gCJD）而出现快速进展性痴呆。发病前六个月，弥散加权成像（DWI）和T2加权成像（T2WI）正常；然而，体素形态测量（VBM）显示右侧颞极和海马体积缩小。发病两个月后，脑成像显示皮质 DWI 高密度，T2WI 肿胀，123I-IMP 单光子发射计算机断层扫描显示右侧显性双侧颞叶、右侧前顶叶、纹状体和海马旁灌注不足。VBM损失遍及右侧颞叶和海马。考虑到海绵状改变和神经元缺失，这些图像有助于了解 V180I gCJD 的神经病理学机制。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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