A comparison of tau and 14-3-3 protein in the diagnosis of Creutzfeldt-Jakob disease.

Objective: To compare the respective efficiency of CSF tau (quantitative) and CSF 14-3-3 protein (qualitative) in the diagnosis of prion disease.

Methods: We made measurements on 420 live subjects, who subsequently underwent a postmortem neuropathology examination, including protein chemistry, immunohistochemistry, and histology. We performed tau by ELISA.

A novel human disease with abnormal prion protein sensitive to protease.

Objective: To report a novel prion disease characterized by distinct histopathological and immunostaining features, and associated with an abnormal isoform of the prion protein (PrP) that, contrary to the common prion diseases, is predominantly sensitive to protease digestion.

Methods: Eleven subjects were investigated at the National Prion Disease Pathology Surveillance Center for clinical, histopathological, immunohistochemical, genotypical, and PrP characteristics.

Results: Patients presented with behavioral and psychiatric manifestations on average at 62 years, whereas mean disease duration was 20 months.

Washington statewide pathology surveillance for prion disease.

In February 2004, we initiated an epidemiological investigation within a US state to enhance autopsy surveillance for clinically suspected prion disease. During the first 30 months, 30 cases of suspected prion disease were referred from throughout Washington. Of these, 18 cases had prion disease, and all of these were classified as either familial or sporadic Creutzfeldt-Jakob disease (CJD); there was no case of variant CJD.