PRNP and Creutzfeldt Jacob disease: The white matter of brains with inherited CJD prion disease show in the majority of cases filamentous PrP deposition in the frontal, temporal parietal and occipital regions of cerebral, subcortical white matter and to a much lesser extent in the cerebellar white matter, and the brain stem (Table 2, Figures 1, 2, 3, 4, 5, 6).