Variant Creutzfeldt-Jakob disease

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Variant Creutzfeldt-Jakob disease (vCJD) or new variant Creutzfeldt-Jakob disease (nvCJD) is a transmissible spongiform encephalopathy which was identified in 1996 by the National CJD Surveillance Unit in Edinburgh. Over 170 cases of vCJD have been recorded in the United Kingdom, and around 30 cases in the rest of the world. The fact that the epidemiology of the disease coincided with an epidemic of Bovine spongiform encephalopathy led to the hypothesis that consumption of BSE-infected beef caused the disease.[1][2]

Despite the consumption of contaminated beef in the UK being reckoned to be quite high, vCJD has infected a comparatively small cohort of people. One explanation for this can be found in the genetics of patients with the disease. All tested vCJD patients have been shown to have two copies of an altered version of the prion protein gene.[3][4]

References[edit]

  1. ^ Ironside, JW (Jul 2010). "Variant Creutzfeldt-Jakob disease.". Haemophilia : the official journal of the World Federation of Hemophilia. 16 Suppl 5: 175–80. doi:10.1111/j.1365-2516.2010.02317.x. PMID 20590878. 
  2. ^ Ironside, JW (2012). "Variant Creutzfeldt-Jakob disease: an update.". Folia neuropathologica / Association of Polish Neuropathologists and Medical Research Centre, Polish Academy of Sciences 50 (1): 50–6. PMID 22505363. 
  3. ^ Saba, R; Booth, SA (2013). "The genetics of susceptibility to variant Creutzfeldt-Jakob disease.". Public health genomics 16 (1-2): 17–24. doi:10.1159/000345203. PMID 23548713. 
  4. ^ Sikorska, B; Liberski, PP (2012). "Human prion diseases: from Kuru to variant Creutzfeldt-Jakob disease.". Sub-cellular biochemistry 65: 457–96. doi:10.1007/978-94-007-5416-4_17. PMID 23225013.