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Cerebroventricular infusion of pentosan polysulphate in human variant Creutzfeldt-Jakob disease

Lookup NU author(s): Dr Duddy Duddy


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Variant Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy believed to be caused by the bovine spongiform encephatopathy agent, an abnormal isoform of the prion protein (PrPSC). At present there is no specific or effective treatment available for any form of CJD. Pentosan polysulphate (PPS), a large polyglycoside molecule with weak heparin-like activity, has been shown to prolong the incubation period of the intracerebral. infection when administered to the cerebral ventricles in a rodent scrapie model. PIPS also prevents the production of further PrPSC in cell culture models. These properties of PPS prompted its cerebroventricular administration in a young man with vCJD. Long-term continuous infusion of PPS at a dose of 11 mu g/kg/day for 18 months did not cause drug-related side effects. Follow-up CT scans demonstrated progressive brain atrophy during PIPS administration. Further basic and clinical research is needed in order to address the issue of efficacy of PIPS in vCJD and in other prion diseases. (c) 2004 The British Infection Society. Published by Elsevier Ltd. All rights reserved.

Publication metadata

Author(s): Todd NV, Morrow J, Doh-ura K, Dealler S, O'Hare S, Farling P, Duddy M, Rainov NG

Publication type: Article

Publication status: Published

Journal: Journal of Infection

Year: 2005

Volume: 50

Issue: 5

Pages: 394-396

ISSN (print): 0163-4453

ISSN (electronic): 1532-2742

Publisher: WB Saunders Co. Ltd.


DOI: 10.1016/j.jinf.2004.07.015


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