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

N. V. Todd, J. Morrow, K. Doh-ura, S. Dealler, S. O'Hare, P. Farling, M. Duddy, N. G. Rainov

研究成果: Article査読

68 被引用数 (Scopus)


Variant Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy believed to be caused by the bovine spongiform encephalopathy 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. PPS 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 μg/kg/day for 18 months did not cause drug-related side effects. Follow-up CT scans demonstrated progressive brain atrophy during PPS administration. Further basic and clinical research is needed in order to address the issue of efficacy of PPS in vCJD and in other prion diseases.

ジャーナルJournal of Infection
出版ステータスPublished - 2005 6

ASJC Scopus subject areas

  • Microbiology (medical)
  • Infectious Diseases

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