The nosology of Creutzfeldt-Jakob disease and conditions related to the accumulation of PrPCJD in the nervous system.

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.