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The importance of ongoing international surveillance for Creutzfeldt–Jakob disease
Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD),...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Nature Publishing Group UK
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8109225/ https://www.ncbi.nlm.nih.gov/pubmed/33972773 http://dx.doi.org/10.1038/s41582-021-00488-7 |
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author | Watson, Neil Brandel, Jean-Philippe Green, Alison Hermann, Peter Ladogana, Anna Lindsay, Terri Mackenzie, Janet Pocchiari, Maurizio Smith, Colin Zerr, Inga Pal, Suvankar |
author_facet | Watson, Neil Brandel, Jean-Philippe Green, Alison Hermann, Peter Ladogana, Anna Lindsay, Terri Mackenzie, Janet Pocchiari, Maurizio Smith, Colin Zerr, Inga Pal, Suvankar |
author_sort | Watson, Neil |
collection | PubMed |
description | Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. Emerging evidence from peripheral tissue distribution studies employing high-sensitivity assays suggests that all forms of human prion disease carry a theoretical risk of iatrogenic transmission. Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments. |
format | Online Article Text |
id | pubmed-8109225 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Nature Publishing Group UK |
record_format | MEDLINE/PubMed |
spelling | pubmed-81092252021-05-11 The importance of ongoing international surveillance for Creutzfeldt–Jakob disease Watson, Neil Brandel, Jean-Philippe Green, Alison Hermann, Peter Ladogana, Anna Lindsay, Terri Mackenzie, Janet Pocchiari, Maurizio Smith, Colin Zerr, Inga Pal, Suvankar Nat Rev Neurol Review Article Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. Emerging evidence from peripheral tissue distribution studies employing high-sensitivity assays suggests that all forms of human prion disease carry a theoretical risk of iatrogenic transmission. Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments. Nature Publishing Group UK 2021-05-10 2021 /pmc/articles/PMC8109225/ /pubmed/33972773 http://dx.doi.org/10.1038/s41582-021-00488-7 Text en © Springer Nature Limited 2021 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic. |
spellingShingle | Review Article Watson, Neil Brandel, Jean-Philippe Green, Alison Hermann, Peter Ladogana, Anna Lindsay, Terri Mackenzie, Janet Pocchiari, Maurizio Smith, Colin Zerr, Inga Pal, Suvankar The importance of ongoing international surveillance for Creutzfeldt–Jakob disease |
title | The importance of ongoing international surveillance for Creutzfeldt–Jakob disease |
title_full | The importance of ongoing international surveillance for Creutzfeldt–Jakob disease |
title_fullStr | The importance of ongoing international surveillance for Creutzfeldt–Jakob disease |
title_full_unstemmed | The importance of ongoing international surveillance for Creutzfeldt–Jakob disease |
title_short | The importance of ongoing international surveillance for Creutzfeldt–Jakob disease |
title_sort | importance of ongoing international surveillance for creutzfeldt–jakob disease |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8109225/ https://www.ncbi.nlm.nih.gov/pubmed/33972773 http://dx.doi.org/10.1038/s41582-021-00488-7 |
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