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December 01, 1999; 53 (9) Brief Communications

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

G. Puoti, G. Giaccone, G. Rossi, B. Canciani, O. Bugiani, F. Tagliavini
First published December 1, 1999, DOI: https://doi.org/10.1212/WNL.53.9.2173
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Abstract

Article abstract Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been 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 sporadic CJD and found that both type 1 and type 2 PrPSc coexisted in 5 subjects. The distinct PrPSc isoforms were associated with different patterns of PrP deposition and severity of spongiform changes, suggesting that the PrPSc type plays a central role in determining the neuropathologic profile of CJD.

  • Accepted October 1, 1999.
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