Sporadic and Infectious Human Prion Diseases

Research output: Contribution to journalLiterature review


Human prion diseases are rare neurodegenerative diseases that have become the subject of public and scientific interest because of concerns about interspecies transmission and the unusual biological properties of the causal agents: prions. These diseases are unique in that they occur in sporadic, hereditary, and infectious forms that are characterized by an extended incubation period between exposure to infection and the development of clinical illness. Silent infection can be present in peripheral tissues during the incubation period, which poses a challenge to public health, especially because prions are relatively resistant to standard decontamination procedures. Despite intense research efforts, no effective treatment has been developed for human prion diseases, which remain uniformly fatal.

Original languageEnglish
JournalCold spring harbor perspectives in medicine
Early online date28 Oct 2016
Publication statusE-pub ahead of print - 28 Oct 2016


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