Prion diseases: an update

Prion diseases are fatal neurodegenerative disorders affecting a variety of vertebrates, including humans. Recently, a new variant of Creutzfeldt-Jakob disease (CJD) in unusually young people appeared in Great Britain. Considerable evidence argues that this novel disease phenotype is caused by dietary exposure to BSE-contaminated food products. In addition to this, the past year's literature holds tantalizing clues to the mechanisms of prion spread in the organism after peripheral infection. This should eventually lead to the development of diagnostic and therapeutic strategies for prion diseases.