The importance of ongoing international surveillance for Creutzfeldt-Jakob disease.


Journal

Nature reviews. Neurology
ISSN: 1759-4766
Titre abrégé: Nat Rev Neurol
Pays: England
ID NLM: 101500072

Informations de publication

Date de publication:
06 2021
Historique:
accepted: 18 03 2021
pubmed: 12 5 2021
medline: 18 1 2022
entrez: 11 5 2021
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 33972773
doi: 10.1038/s41582-021-00488-7
pii: 10.1038/s41582-021-00488-7
pmc: PMC8109225
doi:

Substances chimiques

Prions 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

362-379

Subventions

Organisme : Medical Research Council
ID : MR/J016071/1
Pays : United Kingdom

Commentaires et corrections

Type : CommentIn

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Auteurs

Neil Watson (N)

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.

Jean-Philippe Brandel (JP)

Cellule Nationale de référence des MCJ, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.

Alison Green (A)

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.

Peter Hermann (P)

National Reference Centre for TSE, Department of Neurology, University Medical Centre Göttingen, Göttingen, Germany.

Anna Ladogana (A)

Registry of Creutzfeldt-Jakob Disease, Department of Neuroscience, Istituto Superiore di Sanità, Rome, Italy.

Terri Lindsay (T)

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.

Janet Mackenzie (J)

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.

Maurizio Pocchiari (M)

Registry of Creutzfeldt-Jakob Disease, Department of Neuroscience, Istituto Superiore di Sanità, Rome, Italy.

Colin Smith (C)

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.

Inga Zerr (I)

National Reference Centre for TSE, Department of Neurology, University Medical Centre Göttingen, Göttingen, Germany.

Suvankar Pal (S)

National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK. suvankar.pal@ed.ac.uk.

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