PMC6134320_01
Brain MRI showing faint hyperintensity on Fluid Attenuated Inversion Recovery (FLAIR) images and positive diffusion-weighted (DWI) signal in the right lenticular and caudate nuclei, posterior insular and fronto-parietal cortex, without cortical atrophy or gadolinum enhancement.
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F1
Brain computed tomography (CT) scan was normal, while brain magnetic resonance imaging (MRI) showed faint hyperintensity on Fluid Attenuated Inversion Recovery (FLAIR) images and positive diffusion-weighted (DWI) signal in the right lenticular and caudate nuclei, posterior insular and fronto-parietal cortex, without cortical atrophy or gadolinum enhancement (Figure 1).
EEG showing nonperiodic and right-prevailing abnormal slow waves in fronto-temporal-parietal regions.
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F2
Notably, repeated electroencephalography (EEG) showed nonperiodic right-prevailing abnormal slow waves in fronto-temporal-parietal regions (Figure 2) without typical periodic triphasic sharp waves.
Western Blot showing type 1 abnormal isoform of the prion protein (PrPSc) in both cingulate gyrus and cerebellum brain samples.
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F3
Post-mortem immunoblotting for PrP protein on nervous tissue sampled from right brain emisphere confirmed the diagnosis of sCJD, by the detection of type 1 abnormal isoform of the prion protein (Figure 3).