title: Rapid screening and confirmatory methods for biochemical diagnosis of human prion disease.
abstract: Transmissible spongiform encephalopathies (TSEs) are characterised by accumulation of an abnormal isoform of prion protein (PrP(sc)), mainly in the brain but also in various peripheral tissues. Home-made assays consisting of non-standardised protocols are used currently for laboratory diagnosis of human TSE. The purpose of the present study was to test the ability of two commercial assays, TeSeE™ CJD ELISA and TeSeE™ Western blot, to detect PrPsc in cerebral and lymphoid tissues of TSE patients. Both tests detected a PrPsc-significant signal in the brains of 54 affected patients and not in 51 controls, yielding 100% specificity and 100% sensitivity. Furthermore, three post-mortem spleens and two pre-mortem tonsils from three patients with variant Creutzfeldt-Jakob disease (vCJD) were detected correctly. The expected PrPsc molecular patterns were found in TSE patient brain tissue and in the tonsils and spleens of the three vCJD patients. In conclusion, these rapid and robust in vitro tools were suitable for routine human TSE diagnosis and characterisation. CJD could also be diagnosed during the patient's lifetime by detection of PrPsc in the tonsil. A diagnostic strategy associating TeSeE™ CJD ELISA screening to biochemical confirmation by TeSeE™ Western blot is proposed.
authors.affiliation: Prion Diseases Diagnosis Laboratory, Centre de Biologie et Pathologie Est, Hospices Civils de Lyon, 59 Boulevard Pinel, 69677 Bron Cedex, France.
authors.forename: S
authors.initials: S
authors.lastname: Ugnon-Café
authors.forename: A
authors.initials: A
authors.lastname: Dorey
authors.forename: J M
authors.initials: JM
authors.lastname: Bilheude
authors.forename: N
authors.initials: N
authors.lastname: Streichenberger
authors.forename: G
authors.initials: G
authors.lastname: Viennet
authors.forename: D
authors.initials: D
authors.lastname: Meyronet
authors.forename: A
authors.initials: A
authors.lastname: Maues de Paula
authors.forename: A
authors.initials: A
authors.lastname: Perret-Liaudet
authors.forename: I
authors.initials: I
authors.lastname: Quadrio
mesh_terms: D015153:Blotting, Western / Q000379:methods; D001921:Brain / Q000473:pathology; D019411:Clinical Laboratory Techniques / Q000379:methods*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D006801:Humans; D008403:Mass Screening / Q000379:methods*; D014066:Palatine Tonsil / Q000473:pathology; D017096:Prion Diseases / Q000175:diagnosis*; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013154:Spleen / Q000473:pathology