Enhanced Creutzfeldt‐Jakob disease surveillance in the older population: Assessment of a protocol for screening brain tissue donations for prion disease

• DOI: 10.1111/bpa.13214
• Publication Date: 2023-09-29T05:02:49Z
• Authors: Alexander H. Peden; Adriana Libori; Diane L. Ritchie; Helen Yull; Colin Smith; Lovney Kanguru; Anna Molesworth; Richard Knight; Marcelo A. Barria
• Keywords: Bovine spongiform encephalopathy; Brain tissue; Creutzfeldt-Jakob Syndrome; Transmissible spongiform encephalopathy

Abstract Human prion diseases, including Creutzfeldt‐Jakob disease (CJD), occur in sporadic, genetic, and acquired forms. Variant (vCJD) first reported 1996 the United Kingdom (UK), resulted from contamination of food with bovine spongiform encephalopathy. There is a concern that UK national surveillance mechanisms might miss some CJD cases (including vCJD), particularly older population where other neurodegenerative disorders are more prevalent. We developed highly sensitive protocol for analysing autopsy brain tissue misfolded protein (PrP Sc ) associated disease, which could be used to screen elderly. Brain samples 331 donors Edinburgh Tissue Bank (EBTB), 2005 2022, were analysed, using immunohistochemical analysis on fixed tissue, five biochemical tests frozen specimens six regions, based different principles detecting PrP . An algorithm was established classifying results. To test effectiveness protocol, several neuropathologically confirmed controls, vCJD, included blinded study cohort. On unblinding, all positive control had been correctly identified. No tested positive; our uncovered no overlooked cases. Our effective handling anomalous overall suggested reduced employing only three two regions gave sufficient sensitivity specificity. conclude this may useful as UK‐wide screening programme human selected brains autopsies Further improvements by enhancements vitro conversion assays made during course study.