title: Sporadic and Infectious Human Prion Diseases.
abstract: 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.
authors: National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom.
authors: Robert G
authors: RG
authors: Will
authors: National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom.
authors: James W
authors: JW
authors: Ironside
mesh_terms: D000818:Animals; D001921:Brain / Q000000981:diagnostic imaging; D005060:Europe / Q000453:epidemiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D051379:Mice; D017096:Prion Diseases / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology*; D000072002:Prion Proteins / Q000235:genetics
chemical_list: D000072002:Prion Proteins