title: Diagnosis of prion diseases.
abstract: Prion diseases are usually diagnosed clinically and confirmed by post-mortem histopathological examination of brain tissue. The only reliable molecular marker for prion diseases is PrP(Sc), the pathological conformer of the prion protein that accumulates in the central nervous system and, to a lesser extent, in lymphoreticular tissues. For BSE, several commercial diagnostic kits based on the post-mortem immunochemical detection of PrP(Sc) in brain tissue are now available. These rapid screening tests have been used in active surveillance of BSE and have greatly improved the detection of infected cattle before their entry into the human food chain. At present, no diagnostic test exists for the detection of prion diseases in live animals or humans. New diagnostic techniques aimed at increasing sensitivity and specificity of PrP(Sc) detection in body fluids and at identifying novel surrogate markers are under development. In this report, we review the classical diagnostic methods as well as present and future tools for the diagnosis of prion diseases.
authors.affiliation: Prionics AG, Schlieren, Switzerland.
authors.forename: Eric
authors.initials: E
authors.lastname: Kübler
authors.forename: Bruno
authors.initials: B
authors.lastname: Oesch
authors.forename: Alex J
authors.initials: AJ
authors.lastname: Raeber
mesh_terms: D000818:Animals; D001344:Autopsy; D015415:Biomarkers / Q000032:analysis; D015153:Blotting, Western; D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000175:diagnosis; D010450:Endopeptidases / Q000378:metabolism; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D006801:Humans; D007150:Immunohistochemistry; D008221:Lymphoid Tissue / Q000737:chemistry; D012157:Mononuclear Phagocyte System / Q000737:chemistry; D018622:PrP 27-30 Protein / Q000235:genetics; D018621:PrPC Proteins / Q000032:analysis / Q000378:metabolism; D018620:PrPSc Proteins / Q000032:analysis* / Q000235:genetics / Q000378:metabolism; D011237:Predictive Value of Tests; D017096:Prion Diseases / Q000175:diagnosis* / Q000473:pathology; D011933:Reagent Kits, Diagnostic; D012680:Sensitivity and Specificity; D015047:Zoonoses
chemical_list: D015415:Biomarkers; D018621:PrPC Proteins; D018620:PrPSc Proteins; D011933:Reagent Kits, Diagnostic; D018622:PrP 27-30 Protein; D010450:Endopeptidases