A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset

Figure 1

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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