Table of Contents
Washington University Experience | PRION DISEASES | Prion Diseases | 18B3 CJD (Case 18) H&E 5.jpg
Higher magnification of image #18B2. ---- Comment: The National Prion Disease Surveillance Center reported the presence of abnormal protease resistant prion protein identified as PrP 27-30 (not variant CJD). Immunostaining with 3F4, the monoclonal antibody to the prion protein, reveals granular deposits as seen in prion disease. Also reported was ELISA Immunoassay for the tau protein amount of 5373 pg/ml in CSF (decision point: 1150 pg/ml). The 14-3-3 test also is positive. Thus, in the context of dementia, the finding is consistent with prion disease, probably CJD. The sequencing of the prion protein (PrP) gene confirm the diagnosis of prion disease with the characteristics of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 according to the classification of sporadic prion disease proposed by Parchi et al. (PMID: 22744790).
