PMC9124891_01
Case report timeline.
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F1
The MRI showed asymmetric (right>left) diffusion-weighted imaging (DWI) signal hyperintensities in the cortical ribbon of the parietal, frontal, and temporal lobes, hippocampi, caudate, and putamen (Figures 1A,B).
Diffusion-weighted imaging (DWI) axial view, apparent diffusion coefficient (ADC) view, and regional volumetric studies. Asymmetric DWI increased signal intensity in the cortical ribbon of the parietal, frontal, and temporal lobes, hippocampi, caudate, and putamen. The signal hyperintensities are greater on the right. (A) Image shows increased signal intensity in the cingulate cortex (yellow arrow). (B) More superior image shows diffuse asymmetric involvement of parietal, frontal, and temporal lobes. There is notable involvement of the caudate head that is greater on the right (red arrow). (C) ADC sequence illustrating caudate changes (red arrow) consistent with the DWI hyperintensities. (D) Brain atrophy pattern (highlighted in red), mainly on the right frontal, temporal, and parietal lobes, consistent with areas of abnormal DWI in MRI.
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F2
The illness duration from symptom onset (neuropsychiatric symptoms) to death was 32 months (Figure 2).
Histological and immunohistochemical features. Spongiform degeneration with intermediate size vacuoles affecting cortical (A,B) and subcortical regions (C); inset in (A) a ballooned neuron (arrowhead) and a reactive astrocyte (arrow). (D) Immunohistochemistry showing PrP granules (arrow) and dispersed deposits of larger PrP aggregates (inset). (A) Parietal cortex; (B) hippocampus; (C,D) putamen. Antibody: 3F4.
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F3
Western blot findings demonstrated PK-resistant PrPD type 1, with the unglycosylated PrPD isoform migrating to ~ 20 kDa (data not shown), and histopathological features of the VV1 subtype (Figure 3).