Figure 2

Serial brain MRIs, classic for CJD. Brain MRIs at 17 (a-c), 25 (d-f), and 28 (g-i) months. Solid arrows indicate cortical ribboning. Dotted arrows indicate deep nuclei hyperintensity. (a) Initial brain MRI was read by us as “ambiguous” for CJD, but was felt to show mild frontal-parietal atrophy and punctate subcortical white matter hyperintensities on FLAIR (b) DWI shows subtle cortical ribboning in the anterior and median cingulate and right posterior cingulate and subtle striatal hyperintensity. (c) ADC map shows hypointensities in the same regions as on DWI. (d) FLAIR (axial) brain MRI at 25 months reveals moderate diffuse cortical atrophy. (e) DWI now shows cortical ribboning in the right frontal cortex, and anterior and median cingulate, and hyperintensities in bilateral caudate and putamen with anterior to posterior gradient. (f) ADC map shows hypointensities in the areas of DWI hyperintensity. (g) FLAIR MRI at 28 months shows more prominent cingulate cortical ribboning hyperintensity in the striatum. (h) Cortical ribboning is more prominent than three months prior. (i) ADC map shows hypointensities consistent with restricted diffusion.