title: From Creutzfeldt-Jakob disease to the mad cow epidemic.
abstract: Hans-Gerhard Creutzfeldt and Alfons Jakob independently authored clinical and pathologic descriptions of a new syndrome in the 1920s. This syndrome, which subsequently came to be named after them, was characterized by dementia, motor and coordination abnormalities, a fatal course, and pathologic findings of diffuse spongiform neuronal degeneration. Although it appeared for many years to be little more than a medical curiosity, Creutzfeldt-Jakob disease attained widespread attention by its pathologic similarity to kuru and bovine spongiform encephalopathy, "mad cow disease." Because there are sporadic, familial, and iatrogenic forms of Creutzfeldt-Jakob disease, it is considered to have both genetic and infectious aspects. Although its causation has for some time been ascribed to "slow viruses," the etiology of Creutzfeldt-Jakob disease is currently thought to be due to prions, small proteinaceous infectious particles that have genetic encoding. The debate regarding whether the appearance of atypical Creutzfeldt-Jakob disease can be linked to the epidemic of "mad cow disease" is currently unresolved.
authors.affiliation: Emergency Medicine Service, Stanford University Medical Center, CA 94305, USA.
authors.forename: G
authors.initials: G
authors.lastname: Sternbach
authors.forename: C L
authors.initials: CL
authors.lastname: Dibble
authors.forename: J
authors.initials: J
authors.lastname: Varon
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000818:Animals; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000266:history* / Q000503:physiopathology; D004196:Disease Outbreaks; D016643:Encephalopathy, Bovine Spongiform / Q000453:epidemiology* / Q000503:physiopathology; D049673:History, 20th Century; D006801:Humans; D007729:Kuru / Q000503:physiopathology; D008875:Middle Aged; D011328:Prions; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D011328:Prions