title: The nosology of Creutzfeldt-Jakob disease and conditions related to the accumulation of PrPCJD in the nervous system.
abstract: Although typical cases of Creutzfeldt-Jakob disease are readily recognized pathologically and clinically, variant forms often pose a diagnostic challenge. From the 1920's, when this disease was first characterized, until quite recently diagnosis relied heavily on morphologic changes. New advances in immunoassays and PrP gene analysis now provide important adjuncts in recognizing the spectrum of disorders of PrP metabolism associated with these transmissible encephalopathies.
authors.affiliation: Charles S Kubik Laboratory for Neuropathology, Massachusetts General Hospital, Boston 02114-2696, USA.
authors.forename: E P
authors.initials: EP
authors.lastname: Richardson
authors.forename: C L
authors.initials: CL
authors.lastname: Masters
mesh_terms: D001921:Brain / Q000378:metabolism / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification* / Q000235:genetics / Q000266:history / Q000473:pathology; D004252:DNA Mutational Analysis; D005260:Female; D016098:Gerstmann-Straussler-Scheinker Disease / Q000145:classification / Q000235:genetics / Q000473:pathology; D049673:History, 20th Century; D006801:Humans; D008297:Male; D018620:PrPSc Proteins / Q000235:genetics* / Q000378:metabolism; D017096:Prion Diseases / Q000145:classification* / Q000235:genetics / Q000266:history / Q000473:pathology
chemical_list: D018620:PrPSc Proteins