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MAD COW DISEASE
On December 23,the United States
Department of Agriculture announced a presumptive positive case of Bovine Spongiform Encephalopathy (BSE) in a Holstein cow from Washington State. On December 9, the animal was observed to be nonambulatory (a "downer") prior to slaughter. Accordingly, as part of  USDA's targeted surveillance program for BSE, samples were taken from the animal and immediately sent to USDA's National Veterinary Services Laboratories (NVSL) in Ames, Iowa, for testing.

Since the initial reporting, USDA officials have confirmed that this cow did suffer from BSE.   USDA has coordinated with Washington State officials to put a hold order on the farm restricting the movement of the animals (both premises). USDA also immediately made the appropriate notifications and confirmations under the plan. This included notifying the U.S.Food and Drug Administration and, while there is no evidence that this situation is related to an intentional introduction of the disease, the U.S. Department of Homeland Security. In addition, as discussed above, traceback and traceforwardinvestigations from the
animal have begun.

The USDA will be taking all other
necessary steps. USDA has also asked for assistance from Canada's
animal health officials who recently conducted a similar investigation following the detection of BSE in a cow in Alberta this past summer.

As part of USDA's response to this situation, officials will provide
regular briefings to update the media on the status of the investigation and related efforts. Please see USDA's website at

www.usda.gov

for information on these
briefings and other pertinent information.
What is BSE or Mad Cow Disease

BSE is a degenerative neurological disease caused by an aberrant protein called a prion. It is in the family of disease--all caused by prion--referred to as transmissible spongiform encephalopathies, or TSEs. TSEs include scrapie in sheep and goats, chronic wasting disease (CWD) in deer and elk, and Creutzfeldt-Jakob disease, or CJD, in humans.

What are the risks to the U.S. food supply as a result of this detection?

Despite this finding, USDA remains confident in the safety of the U.S. food supply. The risk to human health from BSE is extremely low. As is standard practice for downer animals identified prior to slaughter, the animal's brain, spinal cord, and other related products were removed and sent to a rendering facility. These so-called "specified risk materials" present the greatest risk of carrying the BSE agent and have not entered U.S. food supply channels. The scientific community believes that there is no evidence to demonstrate that muscle cuts or whole muscle meats that come from animals infected with BSE are at risk of harboring the causative agent of the disease.

How does BSE affect humans?

A fatal TSE affecting humans, variant Creutzfeldt-Jakob Disease (vCJD), is believed to be caused by eating neural tissue, such as brain and spinal cord, from BSE-affected cattle. For this reason, USDA requires that all nervous system materials be removed from downer cattle identified at U.S. slaughter facilities. These specified risk materials are removed, sent to rendering facilities, and do not enter U.S. food supply channels. USDA believes this practice effectively safeguards U.S. public health from vCJD.

It is important to clarify the differences between variant CJD and another form of the disease, referred to as classic CJD. Classic CJD occurs each year at a rate of 1 to 2 cases per 1 million people throughout the world, including in the United States and other countries where BSE has never occurred. It is not linked to the consumption of neural tissue from BSE-affected cattle--both vegetarians and meat eaters have died from classic CJD.

According to the U.S. Centers for Disease Control and Prevention (CDC), no cases of variant CJD have been identified in the United States, the form of the disease linked to eating neural tissue from BSE-affected cattle.

Is BSE a foodborne hazard in the United States?

Strong evidence indicates that BSE has been transmitted to humans primarily in the United Kingdom, causing a variant form of Creutzfeldt-Jakob disease (vCJD). In the United Kingdom, where over 1 million cattle may have been infected with BSE, a substantial species barrier appears to protect humans from widespread illness. As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide; of these, 143 cases had occurred in the United Kingdom. The risk to human health from BSE in the United States is extremely low.


Is there any monitoring of the incidence of Creutzfeldt-Jakob disease in the United States?

Yes. The possibility that BSE can spread to humans has focused increased attention on the desirability of enhancing national surveillance for Creutzfeldt-Jakob disease (CJD) in the United States.

The Centers for Disease Control and Prevention (CDC) monitors the trends and current incidence of CJD in the United States by analyzing death certificate information from U.S. multiple cause-of-death data, compiled by the National Center for Health Statistics, CDC. A summary of these data was published in the Journal of the American Medical Association on November 8, 2000 (Volume 284, No. 18, pp. 2322-3). and in Clinics of Laboratory Medicine in December 2002 (Volume 22, pp. 849-62).

The average annual CJD death rate in the United States has remained relatively stable at about one case per million population per year. In addition, CJD deaths in persons aged <30 years in the United States remain extremely rare (<1 case per 100 million per year). In contrast, in the United Kingdom, over half of the patients who died with variant CJD were in this young age group.

Is there a meat recall associated with the detection?

According to USDA, Verns Moses Lake Meats, a Moses Lake, WA, establishment, is voluntarily recalling approximately 10,410 pounds of raw beef that may have been exposed to tissues from the animal in question containing the infectious agent that causes BSE.

The beef subject to this recall (20 carcasses) was produced on December 9. It was then shipped to Midway Meats of Centralia, WA and several establishments where it was further processed. These establishments are Willamette Valley Meat Co., Portland, OR and Interstate Meat Dist., Inc., Clackamas, OR.

The Food Safety Inspection Service (FSIS) has Enforcement, Investigation and Analysis Officers (EIAO) at the three facilities and they are identifying and verifying the distribution of the product.

FSIS is continuing its investigation to ensure that all distribution of the beef products is correctly identified.

What is the designation of the recall?

FSIS' designation of this recall as Class II is due to the extremely low likelihood that the beef being recalled contains the infectious agent that causes BSE.

According to scientific evidence, the tissues of highest infectivity are the brain, spinal cord, and distal ileum, which were removed from the rest of the animal's carcass at slaughter. Therefore, the meat produced were cuts that would not be expected to be infected or have an adverse public health impact, but are being recalled out of an abundance of caution.

Is there a number consumers can call with questions about meat products?

Consumers with other food safety questions can phone the toll--ree USDA Meat and Poultry Hotline at 1-888-MPHotline. The hotline is available in English and Spanish and can be reached from 10 a.m. to 4 p.m. (Eastern Time), Monday through Friday. Recorded food safety messages are available 24 hours a day.
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What is the variant form of CJD that the experts in the United Kingdom believe might be
related to the BSE outbreak
in cattle?


In contrast to the classic form of CJD, the variant form in the United Kingdom predominantly affects younger persons (median age at death around 29 years) and has atypical clinical features. These atypical features include prominent psychiatric or sensory symptoms at the time of clinical presentation or early in the course of the illness, delayed onset of neurologic abnormalities, duration of illness of at least 6 months, and a diffusely abnormal non-diagnostic electroencephalogram.

The characteristic neuropathologic profile of variant CJD includes, in both the cerebellum and cerebrum, numerous kuru-type amyloid plaques surrounded by vacuoles and prion protein (PrP) accumulation at high concentration indicated by immunohistochemical analysis.

Recently published data indicate that the epidemic of variant CJD in the United Kingdom may have already reached a peak.
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