PMC5075606_01
Antemortem and postmortem findings in an index case. (a) An EEG with pseudoperiodic generalized triphasic slow waves on the 23rd day of the disease. (b) Axial brain DWI shows hyperintense areas in bilateral caudate nuclei, bilateral frontal, and left insular cortex. (c) Macroscopic view of the left frontal lobe atrophy. (d) Immunohistochemical confirmation of prion protein with 6H4 monoclonal antibodies (granular layer of the cerebellar cortex, 400x zoom).
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fig1
The EEG taken on the 23rd day displayed marked diffuse slowing with pseudoperiodic generalized triphasic slow wave pattern (Figure 1(a)). Brain MRI performed on the 23rd day revealed slight symmetrical frontoparietal atrophy and high signal in both caudate nuclei and the bilateral frontal and left insular cortex on diffuse-weighted images (DWI) sequence, with no signs of CBS (Figure 1(b)). An autopsy revealed macroscopically a mild diffuse atrophy of the frontal, paracentral, and cerebellar cortex (Figure 1(c)). The typical histopathological triad of spongiosis, neuronal loss, and astrogliosis, as well as immunohistochemically detected pathological prion protein, confirmed the diagnosis of definite CJD (Figure 1(d)).