Abstract
Prions are proteinaceous particles that due to an alteration of its secondary structure on a post-translational process, they can become infectious pathogens responsible for Transmissible Spongiform Encephalopaties (TSEs). The most common TSE in humans is the Creutzfeldt-Jakob Disease (CJD), which can be divided into 4 forms according to its cause and its clinico-pathological presentation: Sporadic CJD, Genetic CJD, Iatrogenic CJD and Variant CJD. This disease has an annual incidence of 1 case per million and is manifested as a rapidly progressive dementia in middle-aged and elderly persons. This article presents a systematic review on CJD based on articles published in the past ten years from MedLine and Embase databases
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