Sporadic Creutzfeldt-Jakob disease: Co-occurrence of different types of PrPSc in the same brain

Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has be en linked to biochemically distinct types of the protease-resistant form of the prion protein (type 1 and type 2 PrPSc). We investigated 14 cases of s poradic CJD and found that both type I and type 2 PrPSc coexisted in 5 subj ects. The distinct PrPSc isoforms were associated with different patterns o f PrP deposition and severity of spongiform changes, suggesting that the Pr PSc type plays a central role in determining the neuropathologic profile of CJD.