Variant CJD: Reflections a Quarter of a Century on

Twenty-five years has now passed since variant Creutzfeldt-Jakob disease (vCJD) was first described in the United Kingdom (UK). Early epidemiological, neuropathological and biochemical investigations suggested that vCJD represented a new zoonotic form of human prion disease resulting from dietary ex...

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Autores principales:	Diane L. Ritchie, Alexander H. Peden, Marcelo A. Barria
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	variant Creutzfeldt-Jakob disease prion diseases BSE transmission blood transfusion prevalence Medicine R
Acceso en línea:	https://doaj.org/article/2fc169e9f4ed4a74b10d93de7ee127ec
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spelling	oai:doaj.org-article:2fc169e9f4ed4a74b10d93de7ee127ec2021-11-25T18:38:05ZVariant CJD: Reflections a Quarter of a Century on10.3390/pathogens101114132076-0817https://doaj.org/article/2fc169e9f4ed4a74b10d93de7ee127ec2021-10-01T00:00:00Zhttps://www.mdpi.com/2076-0817/10/11/1413https://doaj.org/toc/2076-0817Twenty-five years has now passed since variant Creutzfeldt-Jakob disease (vCJD) was first described in the United Kingdom (UK). Early epidemiological, neuropathological and biochemical investigations suggested that vCJD represented a new zoonotic form of human prion disease resulting from dietary exposure to the bovine spongiform encephalopathy (BSE) agent. This hypothesis has since been confirmed though a large body of experimental evidence, predominantly using animal models of the disease. Today, the clinical, pathological and biochemical phenotype of vCJD is well characterized and demonstrates a unique and remarkably consistent pattern between individual cases when compared to other human prion diseases. While the numbers of vCJD cases remain reassuringly low, with 178 primary vCJD cases reported in the UK and a further 54 reported worldwide, concerns remain over the possible appearance of new vCJD cases in other genetic cohorts and the numbers of asymptomatic individuals in the population harboring vCJD infectivity. This review will provide a historical perspective on vCJD, examining the origins of this acquired prion disease and its association with BSE. We will investigate the epidemiology of the disease along with the unique clinicopathological and biochemical phenotype associated with vCJD cases. Additionally, this review will examine the impact vCJD has had on public health in the UK and the ongoing concerns raised by this rare group of disorders.Diane L. RitchieAlexander H. PedenMarcelo A. BarriaMDPI AGarticlevariant Creutzfeldt-Jakob diseaseprion diseasesBSEtransmissionblood transfusionprevalenceMedicineRENPathogens, Vol 10, Iss 1413, p 1413 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	variant Creutzfeldt-Jakob disease prion diseases BSE transmission blood transfusion prevalence Medicine R
spellingShingle	variant Creutzfeldt-Jakob disease prion diseases BSE transmission blood transfusion prevalence Medicine R Diane L. Ritchie Alexander H. Peden Marcelo A. Barria Variant CJD: Reflections a Quarter of a Century on
description	Twenty-five years has now passed since variant Creutzfeldt-Jakob disease (vCJD) was first described in the United Kingdom (UK). Early epidemiological, neuropathological and biochemical investigations suggested that vCJD represented a new zoonotic form of human prion disease resulting from dietary exposure to the bovine spongiform encephalopathy (BSE) agent. This hypothesis has since been confirmed though a large body of experimental evidence, predominantly using animal models of the disease. Today, the clinical, pathological and biochemical phenotype of vCJD is well characterized and demonstrates a unique and remarkably consistent pattern between individual cases when compared to other human prion diseases. While the numbers of vCJD cases remain reassuringly low, with 178 primary vCJD cases reported in the UK and a further 54 reported worldwide, concerns remain over the possible appearance of new vCJD cases in other genetic cohorts and the numbers of asymptomatic individuals in the population harboring vCJD infectivity. This review will provide a historical perspective on vCJD, examining the origins of this acquired prion disease and its association with BSE. We will investigate the epidemiology of the disease along with the unique clinicopathological and biochemical phenotype associated with vCJD cases. Additionally, this review will examine the impact vCJD has had on public health in the UK and the ongoing concerns raised by this rare group of disorders.
format	article
author	Diane L. Ritchie Alexander H. Peden Marcelo A. Barria
author_facet	Diane L. Ritchie Alexander H. Peden Marcelo A. Barria
author_sort	Diane L. Ritchie
title	Variant CJD: Reflections a Quarter of a Century on
title_short	Variant CJD: Reflections a Quarter of a Century on
title_full	Variant CJD: Reflections a Quarter of a Century on
title_fullStr	Variant CJD: Reflections a Quarter of a Century on
title_full_unstemmed	Variant CJD: Reflections a Quarter of a Century on
title_sort	variant cjd: reflections a quarter of a century on
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/2fc169e9f4ed4a74b10d93de7ee127ec
work_keys_str_mv	AT dianelritchie variantcjdreflectionsaquarterofacenturyon AT alexanderhpeden variantcjdreflectionsaquarterofacenturyon AT marceloabarria variantcjdreflectionsaquarterofacenturyon
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Variant CJD: Reflections a Quarter of a Century on

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