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Washington University Experience | PRION DISEASES | Prion Diseases | 19C9 CJD (Case 19) PRP 8.jpg
The dentate nucleus is stained in nerve terminals and Purkinje cells (3F4 IHC). ---- As part of this evaluation, brain tissues were sent to the NPDPSC for analysis. The Western immunoblot reveals the presence of abnormal protease resistant prion protein (PrPSc) commonly identified as PrP 27-30 confirming the diagnosis of prion disease based on the current criteria (but is not supportive of variant Creutzfeldt-Jakob disease (vCJD). The sequencing of the prion protein (PrP) gene confirm the diagnosis of prion disease with the characteristics of sporadic Creutzfeldt-Jakob disease (sCJD) MV1-2. The PrP gene sequencing rules out the presence of a pathogenic mutation in the coding region of the PrP gene, i.e., the prion disease in the case submitted is not familial according to the current criteria for familial prion disease (Kong et al. Prion Biology and Diseases 2 ed. Prusiner, S. Editor., 2004. 673-775).
