PMC3934789_01
a [123I]-FP-CIT-SPECT. Transaxial view at striatal level with identical regions of interest covering the caudate nucleus and putamen (1 right, 2 left) and occipital reference region (3) showing decreased uptake bilaterally in the posterior putamen and in the left caudate nucleus compared to the right side. The striatal-to-occipital uptake ratio is: 2.24 on the right and 2.02 on the left side. b [18F]-FDG-PET. Summed image with transaxial and coronal views on the striatal level revealing widespread cortical hypometabolism in the frontal, temporal, parietal, and occipital lobes bilaterally as well as in striatum and thalami. A small cortical area of preserved uptake in the frontoparietal region can be seen in the coronal view. MR DWI with apparent diffusion coefficient (c) and TRACE maps (d). Restricted diffusion is noted in the cortical gray matter (especially temporally and occipitally) and in striatum. e Histologic sample with HE staining from the right thalamus, showing marked spongiform changes, gliosis, and neuronal loss.
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The scan showed clear reductions in tracer binding in the left caudate nucleus compared to the right side and bilaterally in the posterior putamen (striatal to occipital uptake ratio: 2.42 on the right and 2.02 on the left) (fig. 1a). FDG-PET showed widespread reductions in FDG binding bilaterally in cortical and deep grey matter, with a small area of preserved cortical binding in the frontoparietal region (fig. 1b). A brain MRI showed no structural abnormalities, whereas pathological changes in diffusion tensor imaging concurrent with CJD were seen (fig. 1c, d); however, other infectious or inflammatory etiology could not be excluded. The histological samples showed marked spongiform changes in the cerebral cortex (fig. 1d). Abundant spongiform changes and gliosis as well as nerve cell loss were found in the caudate nucleus, putamen, and thalamus (fig. 1e).