The high CSF p-tau levels (140 pg/ml) and the low Aβ42 levels (402 pg/ml) in the single case of VPSPr examined in this study correlated with a significant brain Aβ load (Thal phase IV, Aβ score 37) and a mixed brain tauopathy comprising features of PART/AD (Braak stage III, AD-tau score 15), prion-related tauopathy (PrP-tau score 3.5) and ARTAG. Here, PRNP is linked to Alzheimer disease.