Challenges and Advances in Antemortem Diagnosis of Human Transmissible Spongiform Encephalopathies.

ABSTRACT: Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, arise from the structural conversion of the monomeric, cellular prion protein (PrP^C) into its multimeric scrapie form (PrP^Sc). These pathologies comprise a group of intractable, rapidly evolving neurodegenerative diseases. Currently, a definitive diagnosis of TSE relies on the detection of PrP^Sc and/or the identification of pathognomonic histological features in brain tissue samples, which are usually obtained postmortem or, in rare cases, by brain biopsy (antemortem). Over the past two decades, several paraclinical tests for antemortem diagnosis have been developed to preclude the need for brain samples. Some of these alternative methods have been validated and can provide a pr

SUBMITTER: Ascari LM 

PROVIDER: S-EPMC7606880 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature