NR AOUJ
AU Ironside,J.W.; Head,M.W.; McCardle,L.; Ritchie,D.; Will,R.G.; Ward,H.J.T.; Knight,R.S.G.
TI Pathological approaches to study the epidemiology of variant CJD
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Oral sessions OS-02
PT Konferenz-Vortrag
AB Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disease in which the transmissible agent exhibits identical strain properties to the bovine spongiform encephalopathy (BSE) agent. Surveillance of CJD in the UK since has so far identified 136 patients with definite and probable vCJD. However, there are concerns that the surveillance programme might not detect all cases of vCJD, particularly in the elderly, where the incidence of other neurodegenerative disorders which might be confused with vCJD is higher than in younger age groups. To address this question, two tissue-based projects have been established; first, a retrospective review of CJD and atypical dementias in most of the neuropathology departments across the UK. Most of the cases entered into this study are in the elderly population. Second, a retrospective study to detect abnormal PrP in paraffin sections of appendix and tonsil removed from younger patients with no apparent neurological abnormalities at the time of operation. Both studies are still incomplete, but so far the national retrospective review has not identified any "missed " cases of vCJD. The appendix and tonsil study has so far identified one case with detectable PrP in germinal centre tissues within the wall of 1 appendix specimen out of 8,318 cases studied. The results from this study will help inform ongoing epidemiological predications of likely future numbers of vCJD cases in the UK. At present, there is evidence of a decline in the rates for both onsets of and deaths from vCJD. However, given the large uncertainties that still surround variables such as genetic susceptibility to vCJD, the infectious dose of BSE required to cause disease and the possibility of other disease phenotypes due to BSE infection, continuing surveillance of all types of prion disease in the UK is required for the foreseeable future.
AD J.W. Ironside, M.W. Head, L. McCardle, D. Ritchie, R.G. Will, H. Ward, R. Knight, National CJD Surveillance Unit, University of Edinburgh, UK
SP englisch
PO Deutschland