Variant CJD: Reflections a Quarter of a Century on

Abstract

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.

Diagnostic performance of CSF biomarkers in a well-characterized Australian cohort of sporadic Creutzfeldt-Jakob disease

“…. recognized to be transmissible through blood transfusion and likely work-place exposure (2), and iatrogenic CJD (iCJD), transmitted through medical interventions. The foundation of all prion diseases is the template directed conversion of normal cellular prion protein (PrP C ) into abnormally folded conformers (PrP Sc ), with the continued accumulation and deposition of PrP Sc in the brain (3,4).…”

“…Approximately 10–15% of human prion diseases are inherited, caused by mutations of the prion protein gene ( PRNP ). A minor percentage of human prion disease cases are considered acquired: these include variant CJD (vCJD), a zoonosis linked to bovine spongiform encephalopathy, and recognized to be transmissible through blood transfusion and likely work-place exposure ( 2 ), and iatrogenic CJD (iCJD), transmitted through medical interventions. The foundation of all prion diseases is the template directed conversion of normal cellular prion protein (PrP C ) into abnormally folded conformers (PrP Sc ), with the continued accumulation and deposition of PrP Sc in the brain ( 3 , 4 ).…”

Pellagra: Down Not Out If Down and Out (and South) – Part 2

“…Pellagra: Down Not out If Down and out (and South): Part 2 DOI: http://dx.doi.org/10.5772/intechopen.110061 CJD outbreak in the UK from unnatural farming and feeding practices associated with "industrial meat regimes" or in China "dragonhead enterprises"-COVID-19 highlights the need for better and safer meat supplies as did other earlier emergent zoonoses; pathogen spillover is more likely when there are rapid changes in bat ecology including climate change and deforestation and when they are hungry and nomadic themselves bringing them closer to intermediate viral hosts and the human food-chain [62, [278][279][280][281][282][283][284].…”

Recent Advances in Prion Inactivation by Plasma Sterilizer

“…Acquired prion diseases result from exposure to prion agents. For example, iatrogenic CJD (iCJD) has been confirmed following dural transplantation, pituitary growth hormone injection, and corneal transplantation [ 5 , 6 , 7 ], while variant CJD (vCJD) is derived from BSE-contaminated bovine materials [ 8 ]. Sporadic CJD (sCJD) accounts for approximately 85% of all human prion diseases [ 2 ].…”