NR ANVX
AU McConnell,J.
TI Still no trend discernible in new-variant CJD incidence, say experts
QU Lancet 1997 Jul 12; 350(9071): 120
PT Article
VT
The latest information on nvCJD (new-variant Creutzfeldt-Jakob disease) - the fatal condition tentatively linked to eating beef infected with bovine spongiform encephalopathy (BSE) - was revealed at the 20th International Congress of Chemotherapy in Sydney, Australia (June 29-July 3). Robert Will (National CJD Surveillance Unit, Edinburgh, UK) said there are 18 confirmed cases and one probable case of nvCJD in the UK plus one confirmed case in France. There is no discernable trend in the rate of occurrence of nvCJD cases and "it is impossible to say whether there will be a handful more cases or thousands". If cases continue to occur at the current rate, it may be 4 years before the likely total number of cases can be estimated.
John Collinge (St Mary's Hospital Medical School, London, UK) described how abnormal prion protein PrPres from patients with nvCJD has a distinct glycoform pattern very similar to PrPres from BSE-infected animals. But, for BSE PrPres to infect human beings it must cross a species barrier and Collinge reported that studies in a humanised mouse model show that there is an effective species barrier between the BSE agent and human beings. This finding may mean the nvCJD epidemic will be small.
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Paul Brown (National Institutes of Health, Bethesda, MD, USA) said that many drugs, from antineoplastics to vitamin C, have been unsuccessfully tried as therapies for CJD. Since CJD is an infectious amyloidosis, attempts are being made to direct therapy against amyloid accumulation. Sulphonated glycosaminoglycans have such an action in vitro but only work in a mouse scrapie model if given close to the time of infection. Doxorubicin, another amyloid-binding drug, also delays experimental disease onset. Brown pointed out that for any therapy to be useful, a test for early infection with the CJD agent is needed - there is no point in stabilising patients with advanced disease.
John McConnell
SP englisch
PO England