18 July 2000
Experts hope to learn from CJD outbreak

By FWi staff

BSE experts are hopeful that the cluster of cases of the human form of the disease in a Leicestershire village will shed some light on its transmission.

Government BSE advisors Spongiform Encephalopathy Advisory Committee (SEAC) gathered on Monday (17 July) to consider the outbreak in Queniborough.

Three victims of new variant of Creutzfeldt-Jakob Disease (nvCJD) who died in 1998 either lived there or visited there regularly, it emerged this month.

A fourth person from the county died from CJD this May, while a fifth has been described as a “highly probable” case.

Acting SEAC chairman Peter Smith told the BBC Radio 4 Farming Today programme this cluster could help establish if contaminated beef was the source of the disease.

“Its possible that by investigating this cluster we will get a better insight into possible ways in which the human population acquired this infection.”

However, Prof Smith warned that his previous clusters of other diseases often yielded disappointing results.

He said it was too early to say whether a programme of studying human tonsils for signs of the disease would be extended to Leicestershire.

But if the cluster gave real clues, then that would be considered.

Recent figures show for the first time an upward trend in nvCJD cases of about 20-30% per year, but Prof Smith is unsure of the long-term significance of this.

“We would expect all epidemics to show an increase and one of the surprising things is that it hasnt shown an increase before now.”

The number of definite and probable cases of nvCJD now stands at 76.

Prof Smith was sceptical about American reports that sheep in the USA imported from Europe had a BSE-type disease.

It was claimed that the animals, which came from Holland and Belgium, suffered from a transmissible spongiform encephalopathy (TSE) which was not scrapie.

Prof Smith said: “Id be interested to know how they knew it was not scrapie. At the moment we dont have any reliable tests for distinguishing scrapie and BSE in sheep.

“It seems a little bit unlikely if there is BSE in sheep that it should be identified in the United States.”

Prof Smith said as sheep had been fed contaminated feed there was a possibility of BSE transmission, but no evidence had yet been found.