Creutzfeldt–Jakob Disease and Related Transmissible Spongiform Encephalopathies

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Abstract
Until 30 years ago, Creutzfeldt–Jakob disease was an obscure form of dementia unknown to most physicians. The name is now familiar to the medical community as the major transmissible spongiform encephalopathy (or prion disease) in humans and to research scientists because of its strange causative agent with novel modes of replication and transmission. Furthermore, the term “Creutzfeldt–Jakob disease” has caused distress to patients who have been notified that they may have received tainted blood or blood products, and it has been popularized by extensive media coverage of bovine spongiform encephalopathy (“mad cow disease”), with its profound economic impact in Europe . . .