Fears grow over 'mad el...( THE death of three hunters from Creutzf...)

THE death of three hunters from Creutzfeldt-Jakob disease is likely to heighten fears that people in North America are contracting a new form of the fatal brain disorder from deer. But the surveillance system in the US is so woefully inadequate that even if these fears are unfounded, it is impossible for researchers to rule out the possibility.

Chronic wasting disease (CWD) is a prion disease spreading among elk and mule deer in North America. It is similar to mad cow disease, raising fears people eating contaminated venison could develop a form of Creutzfeldt-Jakob disease (CJD) like that blamed on contaminated beef, which has killed about 200 people in Britain.

This is why the latest cases of CJD in hunters, to be reported at the American Academy of Neurology's meeting in Honolulu this week, are causing concern. Two of the men, one aged 64 from Washington state and the other aged 54 from Alaska, were treated by neurologists Natalia Murinova and Ali Samii at the Seattle VA hospital. "These two cases may well have no relationship to CWD in elk and deer," says Samii. "But the fact that it happened in these two patients brings up that question."

The third man died in a different hospital but was from the same town as the hunter from Washington. The two were friends and hunted together.

However, the Centers for Disease Control and Prevention (CDC) will not be investigating the cases because there is no evidence that the men ate CWD-infected meat, says spokesman Ermias Belay. So far the CDC has only investigated CJD cases from states where deer are known to have the disease. But Washington and Alaska are not necessarily disease-free: states do not have to test for CWD until the disease is known to be present.

The CDC has investigated a few suspect CJD cases in the past, and found no compelling evidence of CWD transmission to humans. However, "the data seeking such evidence are very limited", Belay admits.

And that's because not many doctor
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Contact: Claire Bowles
claire.bowles@rbi.co.uk
207-331-2751
New Scientist
2-Apr-2003

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