case_images: PMC8384121_01
case_images: (A-F) Contrast-enhanced brain MRI taken on admission shows diffuse T2/fluid-attenuated inversion recovery (FLAIR) hyperintense lesions in bilateral cerebral white matter, left temporal pole, and right middle cerebellar peduncle without gadolinium enhancement. (G-I) On diffusion-weighted imaging (DWI), these lesions show faintly high intensities that can be explained by T2 shine-through.
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case_images: F1
case_images: Brain MRI on initial presentation revealed diffuse non-enhancing T2/fluid-attenuated inversion recovery hyperintense lesions in the bilateral cerebral white matter, left temporal pole, and right middle cerebellar peduncle (Figures 1A-F). On diffusion-weighted imaging (DWI), these lesions showed faintly high intensities; however, they could be explained by T2 shine-through (Figures 1G-I). We performed thyroid sonography, and it was characterized by isoechogenicity, very slightly internal heterogeneity, no diffuse goiter, and a few cysts (Supplementary Figure 1).
case_images: Electroencephalography (EEG) study 1.5 months after admission shows periodic synchronous discharge (PSD) suggesting Creutzfeldt-Jakob disease (CJD).
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case_images: F2
case_images: One and a half months after admission, her electroencephalogram (EEG) showed bilateral periodic synchronous discharge (PSD), typically suggestive of CJD (Figure 2). The clinical timeline is shown in Supplementary Figure 2.
case_images: Pathological findings. Brain biopsy from the left frontal lobe. (A) Hematoxylin and eosin staining. (B) CD20 immunohistochemical staining. (C) Ki-67 immunohistochemical staining. (D) CD3 immunohistochemical staining.
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case_images: F3
case_images: Pathological findings revealed infiltration of atypical lymphoid cells with large and irregularly shaped nuclei (Figure 3A). These atypical lymphoid cells were positive for cluster differentiation (CD) 20, with 80-90% of the Ki-67 proliferation index (Figures 3B,C). A few CD3-positive reactive T cells were also observed and did not show irregularities (Figure 3D).
case_images: (A,B) Contrast-enhanced brain MRI recorded 1 month after brain biopsy shows a spotty gadolinium enhancement in the left periventricular white matter (arrow) and post-biopsy scar (arrowhead) (A) with T2 hyperintensities (B). (C) This lesion does not show abnormal hyperintensities on diffusion-weighted imaging (DWI).
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case_images: F4
case_images: Contrast-enhanced MRI performed 1 month after brain biopsy revealed a spotty gadolinium enhancement in the left periventricular white matter without high intensity on DWI (Figures 4A-C).