WASHINGTON, April 15 (UPI) -- On May 2, 1997, a cow showed up at a slaughter plant in Oriskany Falls, N.Y., that would spark concerns inside the U.S. Department of Agriculture as possibly being the first case of mad cow disease in the United States.
"We thought it was mad cow then," Masuo Doi, the U.S. Department of Agriculture veterinarian involved with the case, told United Press International.
Recent media reports have suggested the cow was not properly tested by the USDA and in fact may have been positive for the deadly disease. However, a two-year investigation by UPI has revealed the cow was tested multiple times, not only by the USDA, but also by a scientist at the National Institutes of Health, and in all cases the tests came back negative.
In addition, BSE experts have reviewed the USDA documents for UPI and all have agreed the cow was negative and have commended the agency's handling of the case.
Even Doi himself has now concluded the cow was negative.
In recent days, USDA officials, including Agriculture Secretary Mike Johanns, have denied allegations that the cow was not properly screened for BSE.
Doi and other USDA inspectors became concerned in 1997 when they first saw the cow and made a videotape of the animal, which was viewed by UPI. The tape shows a skinny, dehydrated Hereford cow with a hunched back that was very small for its age -- so small it initially was thought to be only 8 months or 9 months old -- but it turned out to be more than 2 years old. The cow constantly trembled and its limbs shook. It was wobbly and had difficulty walking and controlling its movement.
After the cow was slaughtered, the veterinarian sent a sample of brain tissue to the USDA's lab in Athens, Ga., and this set in motion a chain of events that would lead the agency to respond as if the animal really might have mad cow disease.
USDA documents obtained through the Freedom of Information Act and other sources, and interviews with officials involved, indicate the agency was particularly concerned about the case, referring to it as a "BSE suspect" and responding as if it was the first U.S. instance of the deadly disease.
"We reacted to it as if it were a real case," Kaye Wachsmuth, who served as deputy administrator for USDA's Office of Public Health and Science, told UPI.
The USDA prepared a statement about the first case, apparently intended for media calls, which was obtained by UPI. The agency did not include this document in FOIA documents. The statement said the cow was being tested for a variety of CNS disorders, but did not mention the possibility of mad cow disease.
However, the "talking points" and timeline attached to the statement mention the possibility of mad cow and note that in responding to the case the "USDA-BSE taskforce began two-day meeting in Riverdale, Md., to review and update BSE Emergency Action Plan."
The information was not made public, however, and only two small reports about the case appeared in local newspapers.
Doi said Karl Langheinrich, a pathologist at the lab, telephoned him and said, "Congratulations! You found the first case of BSE."
Langheinrich later denied he had said this, and instead acknowledged he may have said, "Congratulations, you found a good one," or something of that nature, he said. Langheinrich, who retired from the agency May 31, 1997, shortly after this case occurred, insisted he never examined the cow's tissue.
USDA documents indicate a pathologist named Scott Hafner -- not Langheinrich -- examined the brain tissue from the animal.
Initially, personnel at the Athens lab reported finding spongiform changes, or microscopic holes in the brain of the cow, which can indicate mad cow disease, but this later turned out to be a false alarm.
"We rushed somebody up on an airplane with specimens from the Athens lab up to the Ames, Iowa, lab," Wachsmuth said. The USDA's National Veterinary Services Laboratory in Ames specializes in conducting mad cow tests.
Linda Detwiler, a veterinarian in charge of the USDA's mad cow surveillance program at the time, and now retired from the agency, told UPI that as the suspicious brain sample was being forwarded to the Ames lab, agency officials tried to trace back the animal's origins. This is the same procedure they followed in the first and only confirmed case of mad cow in the United States in December 2003.
One of the intentions of a traceback is to determine an animal's feeding history, because the disease usually is transmitted through infected feed that contains remnants of cows that had BSE.
"They (USDA officials) were able to talk to the cow owner and get a good history of it," which showed it had not been fed any material that might have contained mad cow disease and helped rule out the possibility it was infected, Detwiler said.
Tests on the brain sample also came back negative for mad cow.
"It happened pretty quickly and they (lab personnel in Ames) were able to set up the test and say there's no PrP (mad cow pathogen) here," she said. In addition to running its "gold-standard" test, called immunohistochemistry, personnel at the Iowa lab ran another test called Western blot. Both showed no evidence of infection.
"They ran the whole gamut of tests just to make sure ... because of the spongiform changes," Detwiler said.
While all this was occurring, and unbeknownst to the USDA, Doi had secretly obtained a sample of the brain tissue and given it to Michael Hansen of Consumer's Union and Felicia Nestor, who was with the Government Accountability Project at the time.
They delivered it to Joe Gibbs, head of the National Institutes of Health's Laboratory for Central Nervous System Studies -- a now-defunct lab that conducted groundbreaking work on mad cow and similar disorders in humans. At first, Gibbs, who died in 2001, was reluctant to take the sample, but his curiosity was piqued after Hansen showed him the tape of the animal.
Upon seeing the cow's staggering behavior, Gibbs "walked straight from where we were watching the tape back to his office and dialed out to (the USDA Ames lab) and said, 'I want to see the brains of that animal,'" Hansen said. "They told him everything turned up negative."
Gibbs agreed to store the brain-tissue sample for them, but he would not test it until after August, Hansen said. "He ultimately tested it later, and it was negative," he said.
Bruce Johnson, a former scientist at the NIH who conducted the test on the sample, said he remembered the case.
"That was a negative cow," Johnson told UPI, noting he tested at least three different samples from the brain tissue. "It was clearly negative," he said.
Johnson is not sure if the samples contained the brain's obex, the area preferred for testing for BSE because it is where prions -- the pathogen thought to cause mad cow disease -- typically concentrate. But he said it was likely, because the cow exhibited advanced symptoms, that he would have detected prions in other regions of the brain if it was infected.
Other experts confirmed for UPI it was possible in this case to arrive at a solid conclusion the cow did not have BSE without having the obex.
Janice Miller, a former veterinary medical officer at Ames, was involved with the testing of the suspect animal.
Miller, who is now retired, told UPI: "Contrary to people's suggestion that they (the USDA) are very clandestine, they were very open about this suspect tissue and invited pathologists from the (Ames) lab" and Iowa State University to examine the specimens. So we went over to (Ames) and they had their own pathologist there, another pathologist (from the USDA's Food Safety and Inspection Service) and Iowa State University pathologists and we all sat around with ... microscopes and looked at the tissue."
She said the group "all concluded pretty quickly ... that was not the typical lesion associated with BSE."
Art Davis, chief pathologist at NVSL, thought the tissues showed evidence of a very rare condition called progressive ataxia, Miller said. He "suspected it was progressive ataxia and it was decided to send off that tissue to a true neuropathologist expert," she recalled. The tissue was sent to Dr. Alexander deLahunta at Cornell University, who confirmed it was progressive ataxia.
The testing group ruled out BSE because of the brain region where the damage was observed, Miller said.
"We recognized very quickly it involved the white matter and not the gray matter," she said. "BSE is usually in the gray matter, in a particular site in the brain called the obex, where BSE targets, and that's where the spongiform change occurs and that slide showed spongiform change ... in white matter," she explained.
The pathology report did not indicate the obex was tested for signs of prions, but other nearby portions of the brain were clearly marked as tested.
It is not uncommon for the obex not to be included in samples sent to labs for BSE testing, according to Elizabeth Mumford, a veterinarian and BSE expert at Safe Food Solutions in Bern, Switzerland, a company that provides advice on reducing mad cow risk to industry and governments.
"The submission of inappropriate samples is not surprising and happens everywhere, even in Switzerland at the beginning of the outbreak here," Mumford told UPI.
Despite the missing obex, experts showed no concern the cow might have been positive. Several mad cow experts reviewed the records from this case for UPI and all agreed the USDA acted appropriately and some commended the agency.
"I am very confident that the proper diagnosis has been made," one veterinary pathologist specializing in the diagnosis of neurological diseases and transmissible spongiform encephalopathies, of TSEs, told UPI. The source, who requested confidentiality, works at one of the top BSE testing laboratories in the world.
"The pathological description is very clear, very professional, and the fact that both Dr. deLahunta and Dr. Huxtable (well known veterinarian working in the field of neuropathology) have seen these slides and have come to the same conclusions is not far from being a proof to me," the pathologist said. "I think they did enough to rule out BSE in this case."
Stephen Dealler, a medical microbiologist at Lancaster Royal infirmary who has been researching BSE since it first began in the 1980s in the United Kingdom, told UPI, "I have had a look and what it shows to me so far is that they have tried quite hard and were extremely worried at the time ... but have got good reasons to say that no BSE was found."
Dealler jokingly said he was embarrassed he couldn't find anything to criticize the agency for since he had a reputation of being a bulldog on BSE.
Another BSE testing expert, who also requested anonymity, said, "It seems to me that USDA went above and beyond the book in dealing with this case, and legitimately ruled out BSE."
The expert added, "They handled it very well."
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Steve Mitchell is UPI's Medical Correspondent. E-mail: [email protected]
