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From: TSS ()
Subject: Re: On the Question of Sporadic or Atypical Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease
Date: December 8, 2006 at 8:12 am PST

In Reply to: On the Question of Sporadic or Atypical Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease posted by TSS on November 7, 2006 at 1:57 pm:

New Mad-Cow Variant Is Suspected
February 17, 2004 The Wall Street Journal by ANTONIO REGALADO

Italian scientists have discovered that cattle may harbor a new form of mad-cow disease.
The research by scientists at the University of Verona, in Italy, builds on earlier work that raised the possibility of unusual cases of the fatal brain-wasting disease, formally known as bovine spongiform encephalopathy. The findings pose new questions about the risk of consuming tainted meat.

"If you eat cattle with this new strain, you could get a disease in humans," said Salvatore Monaco, a biologist who made the finding with colleague Gianluigi Zanusso.

Dr. Monaco said he found that the brains of certain infected animals looked similar to human brains destroyed by Creutzfeldt-Jakob disease, a neurological disorder that is believed to arise spontaneously and hadn't previously been linked to tainted meat. The human form of mad-cow disease is called variant Creutzfeldt-Jakob disease, which manifests itself differently. Now the question is whether the new form of BSE could be responsible for other Creutzfeldt-Jakob cases.

About 150 cases of variant Creutzfeldt-Jakob disease -- nearly all of them in Britain -- have been linked to eating infected cattle. But the majority of cases of Creutzfeldt-Jakob, which strikes about one in a million people each year, are termed "sporadic" because they don't have any known cause.

Consumer groups are likely to leap at the Italian finding, published in the Proceedings of the National Academy of Science. Some activists have long contended that humans are contracting mad-cow disease in the U.S., contrary to what public-health officials say.

"I have been waiting for this," said Terry S. Singeltary, a Bacliff, Texas, resident whose mother died in 1997 of Creutzfeldt-Jakob disease. Mr. Singeltary and others suspect that some cases of sporadic Creutzfeldt-Jakob are caused by something in the environment, perhaps contaminated meat.

BSE is believed to be caused by misshapen proteins called prions that can be spread by consumption of contaminated tissue. The U.S. found its first case of the cattle disease in December, after a Holstein in Washington state tested positive for the condition.

The Italian findings give some support to the theory that BSE could be responsible for some cases of Creutzfeldt-Jakob disease, Dr. Monaco said. The Italian group studied the brains of eight cattle that had tested positive for BSE using standard tests. Six of the animals' brains showed the usual signs, but the brains of two animals showed unusual deposits of prions. The distinctive patterns of deposits may indicate a new strain of mad-cow disease, which the Italian scientists have dubbed BASE, for bovine amyloidotic spongiform encephalopathy.

Linda Detweiler, a former senior veterinarian for the U.S. Department of Agriculture, said the finding isn't a complete surprise. Studies in the U.K., France and Japan have hinted that some cattle may have been suffering from atypical cases of mad-cow disease.

A related disease in sheep, called scrapie, is known to come in many different strains.

Dr. Detweiler said more research will be needed to confirm whether there is in fact a new strain of BSE. One possibility is that the disease simply looks different in the specific cattle breeds in the Italian study. "I think there are still a lot of questions," Dr. Detweiler said.

Stanley Prusiner, a leading researcher at the University of California, San Francisco, who is the discoverer of the prion and who reviewed the Italian report, has said the possibility of unusual BSE cases is an argument in favor of more comprehensive testing of cattle destined for human consumption.

Currently, the Agriculture Department tests only a tiny fraction of cattle for mad-cow disease. Its policy is to target animals that aren't able to walk, known as downer animals, based on the theory that those are most likely to have BSE.

But Dr. Monaco said his findings argue strongly for more testing in the U.S. The two animals found to have the new strain were 11 and 15 years old, "weren't acting abnormal, and were apparently free of neurological problems," Dr. Monaco said.

In Europe, all cattle slaughtered that are more than 30 months of age are tested for BSE. In Japan, all cattle turned into food are tested for the disease.

Scientists first discovered that BSE could affect humans in 1996, when doctors in Britain -- where mad-cow disease was ravaging herds -- found young people succumbing to Creutzfeldt-Jakob disease, which mainly strikes older people. Further research showed the disease, which remains extremely rare, was being caused by the same strain of prion as BSE -- the pattern of damage to the brain was similar in humans and cattle, as were the molecular signatures of the prions.

The existence of a second strain of BSE could mean some people are contracting it, but that the cases aren't being diagnosed properly.

Write to Antonio Regalado at antonio.regalado@wsj.com


http://online.wsj.com/public/us


tss



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