Experts study possible CJD breakthrough

Published on Monday 14 July 2008 10:20

EXPERTS at Edinburgh University's National CJD Surveillance Unit are reviewing all of their past research following the discovery of a new CJD-like disease that may have claimed the lives of ten people in America.

Dr Mark Head fears some patients previously identified as suffering from Creutzfeldt-Jakob Disease – the human form of BSE or "mad cow disease" – may have been suffering from a fast-advancing form of fatal dementia called PSPr.

There are between 50 and 100 new cases of so-called sporadic CJD diagnosed in the UK every year. This differs from "variant CJD" which is linked to the ingestion of BSE-infected beef.

Post-mortems on those who died in the States revealed familiar "spongy" brain tissue, covered with tiny holes, that is seen in CJD patients but with significant differences in symptoms.

Dr Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center, in Ohio, said that he believed the newly discovered type had probably "been around for years, unnoticed".

His study has prompted a review by the Edinburgh scientists. Dr Head said: "What is interesting about this is thatIt may mean there are other genes out there waiting to be found which are associated with prion disease, and looking at these patients in the US could help find them."