In 1920 and 1921, Creutzfeldt and Jakob described a rapidly progressive form of dementia that was apparently sporadic; the description of the familial form appeared shortly thereafter.1 Between 1928 and 1936, Gerstmann, Sträussler, and Scheinker described a slowly progressive familial disease characterized by cerebellar signs. The discovery in 1957 of a third such disease, kuru, a subacute condition affecting a New Guinean tribe that practiced cannibalism, suggested that Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker disease, and kuru were related. Since all three diseases were shown to be transmissible to animals, they were grouped under the name “transmissible spongiform encephalopathies” and later came to . . .
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Gambetti et al. (1999) studied this question.
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