The case has renewed concern about the potential for transmission of the disease bovine spongiform encephalopathy (BSE) to man through contact with infected animals, eating meat, or drinking milk. No link has been proven but a leaked government memo says that it is "difficult to explain this [case] as a chance phenomenon".
A third case in an unnamed cattle farmer was detailed in the Lancet last month, with doctors concluding that it was "a matter for concern". Dr Rob Will, a leading expert on Creutzfeldt-Jakob disease, conceded that the chances of a third case were "statistically low". This contrasted sharply with the scientific community's response to two previous reports of CJD in cattle farmers in 1993 which were regarded as blips.
Earlier this month a report from the CJD Surveillance Unit in Edinburgh, set up to monitor the disease since the emergence of BSE in the 1980s, said the number of cases of CJD doubled between 1985 and 1994 to 55, the highest ever figure.
The rise may be due to increased surveillance, according to Dr Will. However, three confirmed cases among cattle farmers known to have had cows infected with BSE in their herds have failed to reassure the public and some scientists.
The fourth man, who is believed to be from North Wales, is dying of a degenerative brain disorder but only a post-mortem examination will confirm if he has CJD. His case came to light after a memo from the government's Spongiform Encephalopathy Advisory Committee was reportedly faxed to a wrong number.
The memo said: "The Committee concluded that it was difficult to explain this simply as chance phenomenon. There is a statistical excess of cases in cattle farmers compared with the general population."
The memo says there are no reported cases among vets or abattoir workers who might be expected to have an exposure to the infectious BSE agent, a prion. It also says that CJD has shown a similar incidence in farmers from countries with no cases, or very few, of BSE.Reuse content