Variant CJD
نویسندگان
چکیده
Abbreviations: BSE, bovine spongiform encephalopathy; CWD, chronic wasting disease; GSS, Gerstman–Str€aussler–Scheinker disease; M, methionine; PPS, pentosan polysulphate; PrP, protease-resistant prion protein; PrP, abnormal prion protein; QuIC, quaking-induced conversion; TSE, transmissible spongiform encephalopathy; V, valine; vCJD, variant Creutzfeldt–Jakob disease; VPSPr, variably protease-sensitive prionopathy
منابع مشابه
Description and analysis of 12 years of surveillance for Creutzfeldt-Jakob disease in Denmark, 1997 to 2008.
Prospective surveillance of Creutzfeldt–Jakob disease (CJD) was initiated in Denmark in 1997, following the observation of variant CJD in the United Kingdom. Demographic, clinical and diagnostic information was collected for each patient with clinical suspicion of CJD. Here we describe the methods for surveillance and the observed outcomes between 1 January 1997 and 31 December 2008. A total of...
متن کاملDefinition and Evaluation of MRI-Based Measures for the Neuroradiological Investigation of Creutzfeld-Jakob Diseases
Of great concern has been the occurrence in the United Kingdom in the 1990s of variant CJD (vCJD), a form of human environmentally acquired CJD, also known as 'mad cow disease'. Although the number of vCJD cases has decreased since 2001, a new risk exists of transmission by blood transfusion. The disease raises a number of challenges for neuroradiological centres, since there exists only limite...
متن کاملThe nigrostriatal pathway in Creutzfeldt-Jakob disease.
Parkinsonism, chorea, and dystonia are well-known clinical manifestations of Creutzfeldt-Jakob disease (CJD), but lesions of the nigrostriatal pathway have never been thoroughly studied. We performed a detailed neuropathologic study of the nigrostriatal pathway in 15 sporadic CJD and 2 variant CJD cases that included clinical correlations and assessment of neuron subtype loss, distribution of p...
متن کاملUse of 14-3-3 in the diagnosis of Creutzfeldt-Jakob disease.
The transmissible spongiform encephalopathies include human diseases such as Creutzfeldt-Jakob disease (CJD) and kuru as well as animal diseases such as scrapie and bovine spongiform encephalopathy (BSE). The emergence of variant CJD, which is causally related to BSE, has generated much interest in the development of rapid and sensitive diagnostic tests for the pre-mortem diagnosis of CJD. In 1...
متن کاملIatrogenic Creutzfeldt-Jakob disease: the waning of an era.
The outbreaks of iatrogenic Creutzfeldt-Jakob disease (CJD) from cadaveric human growth hormone and dura mater are winding down and, like the only other environmentally acquired form of CJD (variant CJD due to infection with the agent of bovine spongiform encephalopathy), iatrogenic disease seems to have reached its high water mark during the 1990s. The total number of cases has reached 405, an...
متن کاملCreutzfeldt-Jakob disease: recent developments
Creutzfeldt-Jakob disease (CJD) is a rare prion disorder that has been the subject of both professional and public interest following the identification of variant CJD as a zoonotic disorder. There have been recent advances in diagnostic techniques, including real-time quaking-induced conversion and magnetic resonance imaging brain scan, that have allowed more accurate case recognition in all f...
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