Variant Creutzfeldt–Jakob disease

  title={Variant Creutzfeldt–Jakob disease},
  author={J. W. Ironside},
Summary.  Variant Creutzfeldt–Jakob disease (CJD) is an emerging form of human prion disease caused by oral exposure to the bovine spongiform encephalopathy agent. Most cases have occurred in the UK, but smaller numbers of cases have been identified in 10 other countries worldwide. All confirmed cases belong to a single genetic subgroup defined by methionine homozygosity at codon 129 in the prion protein gene. Variant CJD has a widespread distribution of infectivity in the body, involving… 

The importance of ongoing international surveillance for Creutzfeldt–Jakob disease

An up-to-date overview of the transmission of prion diseases in human populations is provided and it is argued 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.

[Creutzfeldt-Jakob disease in Peru: report of eleven cases].

Clinical features of six cases of the sporadic form of CJD with definitive diagnosis by histopathology, and five cases with probable diagnosis were reported in patients treated at the Peruvian National Institute of Neurological Sciences.

Variant CJD

It is ascertained that all blood components and leucoreduced blood in a sheep model of vCJD have the ability to transmit disease and the potential for peripheral infection in the absence of clinical disease.

Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review.

Keeping instruments moist was estimated to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease cases and associated costs, and the estimated costs per quality-adjusted life-year gained of these strategies in addition to keeping instruments moist were in excess of £1M.

Hemophilia A and Hemophilia B

Veterans health administration information systems as a resource for rare disorders research: Creutzfeldt-Jakob disease as a paradigm.

For certain rare diseases, VHA information systems can be used to assemble a substantive case series for clinical study, however, the VHA's distinctive demographic characteristics and population dynamics may limit the external validity of epidemiologic investigations.

Infections of the Nervous System

It is essential that the key features of these conditions are understood by critical care professionals and others to prevent a missed diagnosis and by those who may work in those parts of the world where these infections are more common.



Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia

Estimates of the relative risks of exposure through diet, surgery, endoscopy, blood transfusion and receipt of UK‐sourced plasma products suggest that by far the most likely route of infection in this patient was receipt ofUK plasma products.

Can Creutzfeldt-Jakob disease be transmitted by transfusion?

  • P. Brown
  • Medicine, Biology
    Current opinion in hematology
  • 1995
Epidemiologic studies do not support the contention that the administration of blood, blood components, or blood derivatives transmits the disease, and it will require an unremitting effort to screen from the blood donor population all individuals with a higher than average risk of harboring the infectious agent.

Pathological diagnosis of variant Creutzfeldt‐Jakob disease

The pathology of vCJD showed relatively uniform morphological and immunocytochemical characteristics, which were distinct from other forms of CJD, and there is a major need for widespread CJD surveillance.

Creutzfeldt‐Jakob disease: reflections on the risk from blood product therapy

  • P. Brown
  • Medicine, Biology
    Haemophilia : the official journal of the World Federation of Hemophilia
  • 2007
Strategies to prevent iatrogenic transmissions include low‐risk sourcing, leucodepletion, and a variety of infectivity‐reducing plasma processing steps; screening tests to detect infection in preclinical donors are under development.

Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent

It is shown that the strain of agent from cattle affected by bovine spongiform encephalopathy (BSE) produces a characteristic pattern of disease in mice that is retained after experimental passage through a variety of intermediate species, providing strong evidence that the same agent strain is involved in both BSE and vCJD.

Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns.

The epidemic of bovine spongiform encephalopathy in the United Kingdom is waning to a conclusion, but leaves in its wake an outbreak of human Creutzfeldt-Jakob disease, most probably resulting from the consumption of beef products contaminated by central nervous system tissue.