PMC9020293_01
Diffusion weighted imaging B=1000 (A, C, E) and matching fluid-attenuated inversion recovery (B, D, F) images. Geographic regions of cerebral cortical abnormal diffusion restriction (A; arrowheads) with more subtle increase in T2 signal (B, arrowheads) most marked in the left hemisphere. Abnormal diffusion restriction (C) and increased T2 signal (D) involving the caudate nucleus heads (arrowheads), left posteromedial thalamus:pulvinar (arrows) and to a lesser degree bilateral lentiform nuclei (asterisks). The hippocampi demonstrate no convincing abnormal diffusion restriction (E; arrowheads) or T2 signal abnormality (F; arrowheads).
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F1
MRI demonstrated extensive geographic areas of abnormal diffusion restriction of the cerebral cortex, predominantly in the left cerebral hemisphere (figure 1).
PrPSc glycotyping of the patient's brain. Proteinase K digested patient (Pt) and glycotype control (T1, T2) brain homogenates were analysed. The patient's unglycosylated band (un-)resolves at approximately the same mobility as the T1 control, and the diglycosylated band (di-) is appreciably under-represented compared with the monoglycosylated and unglycosylated bands. Relative molecular weights are indicated.
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F2
Based on the electrophoretic mobility and relative abundance of the PrPSc bands, the patient's glycotype was determined to be type 1 (figure 2), with the final molecular subtype designated VV1.