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Review:
Michael B. Coulthart and Neil R. Cashman
Variant Creutzfeldt–Jakob disease: a summary of current scientific knowledge in relation to public health
CMAJ 2001; 165: 51-58 [Abstract] [Full text] [PDF]
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[Read eLetter] Bioterrorism - another explanation for the BSE case in Canada?
John H. Lange, E.O. Talbott, G. Mastrangelo, U. Fedeli   (3 June 2003)

Bioterrorism - another explanation for the BSE case in Canada? 3 June 2003
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John H. Lange,
Consultant
Envirosafe Training and Consultants,
E.O. Talbott, G. Mastrangelo, U. Fedeli

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Re: Bioterrorism - another explanation for the BSE case in Canada?

john.pam.lange{at}worldnet.att.net John H. Lange, et al.

The recent occurrence of Bovine Spongiform Encephalopathy (BSE) in Canada raises many concerns and issues(1). Four theories have emerged in explaining this single case(2). First, is the event arose spontaneously (a “’De Novo’ event”); second, the cow was born in a county where BSE exit or be the offspring of an infected cow (“vertical transmission”); third, the animal in this case consumed contaminated feed; and forth, there has been an interspecies transfer.

There have been numerous studies showing that prions can jump species, including BSE. Some have suggested that Chronic Wasting Disease (CWD), BSE and variant Bovine Spongiform Encephalopathy (vBSE) arose from scrapie(3). If prions can jump species(4), then transfer to man, as has been previously reported in a few cases (table), is certainly possible, although not widely accepted. Such a suggestion makes the case in Canada of great importance and provides credence to the later two theories. Certainly, if cattle consume feed and become infected, this may be a mechanism for interspecies transfer, including to man.

We are proposing another possibility for the occurrence of BSE in Canada, and that is a bioterrorist event. There is little stopping a terrorist group from obtaining animal prions and injecting this material into cattle or other organisms, including wildlife, and/or placing it into feed stocks for the purpose of crippling a segment of the economy. If interspecies transfer is possible, animal prions would easily be obtained from wildlife. Such an act would be difficult to detect and discover. Both livestock and wildlife are important to the economy of many areas of Canada and other places in the world. Even the threat of such actions could be devastating on these industries.

This is not to indicate that a bioterrorist event occurred, but in our unsettled world this is in the realm of possibilities. Thus, additional safeguards are need for two of the most important industries in Canada and many other world locations, livestock and wildlife.

Table: Reports on prions jumping the species barrier to man*

Agent Species Involved Reference
vCJD Cattle - Man (5)
CJD Squirrel - Man (6)
CJD Wild goats - Man (6)
CJD Wild pigs/Squirrel - Man (7)
vCJD/CWD Deer/Elk - Man (8)
CWD Rabbit/Squirrel/Bear - Man (8)

* believed that the species barrier is related to the amino acid sequence heterology between PrPsc of donor and PrPc of recipient.

References

1. Purdue University. Mad cow disease, News and Public Affairs. 2003. www.agriculture.purdue.edu/agcomm/agcom/news/backgrd/disease_home.htm

2. Reuters Health. Theories abound for mad cow disease in Canada. May 22, 2003; www.laurushealth.com/HealthNews/reuters/NewsStory0522200341.htm

3. Belay ED, Schonberger LB. Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Clin Lab Med. 2002; 22: 849-62

4. Bons N, et al.,Natural and experimental oral infection of nonhuman primates by bovine spongiform encephalopathy agents. PNAS 1999; 96: 4046- 51.

5. Sy M-S, et al., Human prion diseases. Med Clin N Am 2002; 86: 551- 71.

6. Berger JR., Creutzfeldt-Jacob Disease and eating squirrel brains. Lancet 1997; 350: 642.

7. Kamin M, Patten BM. Creutzfeldt-Jacob Disease: possible transmission to humans by consumption of wild animal brains. Am J Med 1984: 76: 142-5.

8. Belay ED et al., Creutzfeldt-Jacob Disease in usually young patients who consumed venison. Arch Neurol 2001; 58: 1673-8