PMC8384121_01
(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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F1
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).
Electroencephalography (EEG) study 1.5 months after admission shows periodic synchronous discharge (PSD) suggesting Creutzfeldt-Jakob disease (CJD).
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F2
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.
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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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).
(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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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).