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From: TSS ()
Subject: Re: Suspected CJD numbers grow in Idaho, Eighth person dies after being diagnosed with CJD
Date: October 5, 2005 at 8:09 am PST

In Reply to: Suspected CJD numbers grow in Idaho, Eighth person dies after being diagnosed with CJD posted by TSS on October 5, 2005 at 7:35 am:

Biological Agents
Two Idaho women died of brain wasting disease
By David Liu Ph.D.
Oct 3, 2005, 19:06

The Associated Press (AP) reported on Oct. 1 that preliminary tests indicated two Idaho women died of the brain-wasting disease or Creutzfeldt-Jakob disease (CJD). It is still unknown whether the disease is the naturally occurring form (CJD) or the variant of Creutzfeldt-Jakob disease (vCJD) they acquired by eating tainted beef.

Idaho Department of Health and Welfare officials released the news Sept. 28 after notifying the families of the victims. "One of the victims was in her 60s and lived in Twin Falls County, and the other was identified by her family as 53-year-old Kathy Isenberg of St. Maries," according to the AP.

One case of Creutzfeldt-Jakob disease diagnosed in another Twin Falls County woman was reported earlier this year. More tests are needed to determine whether the disease occurred naturally or through consumption of tainted beef.

Idaho officials believed that the disease occurred naturally. The AP quoted Tom Shanahan, spokesman for the Idaho agency, as saying that 85 percent of cases are naturally occurring, 14 percent are transmitted from one generation to another in a family, and only less than 1 percent is the variant form that patients acquire through consumption of infected beef. Shanahan was further quoted by the AP as saying that, "there's never been a variant case acquired in the United States."

Officials believe that one out of a million people in the U.S. may acquire the naturally occurring CJD each year. People who die of the natural form of CJD are usually over the age of 60. Idaho has a population of 1.4 million. The number of CJD cases for 2005 in Idaho indicates that not all the cases may be the naturally occurring form. In other words, some people may acquire mad cow disease through eating tainted beef or other animal meat.

Itís officially admitted that only one case of variant Creutzfeldt-Jakob disease or vCJD was reported in 2002 in the State of Florida. Itís believed that the young woman acquired the disease when she traveled to England and ate tainted beef.

In the U.S., cow remains, the risky parts humans fear to eat such as brains and blood have been used in cow feed and are still being used in animal feed for chickens, pigs, turkeys and others. The by-products from these animals are used to feed cows.

The risk is that the infectious agent called prion can survive the whole process. Prion is extremely resistant to heat, ultraviolet light, ionizing radiation, normal sterilization processes, and common disinfectants that normally inactivate viruses and bacteria, according to the USDA. Critics say the practice potentially keeps the mad cow disease transmitted among animals, and as a result consumers, including those who eat chicken and pork, may be at risk of vCJD.

Unlike in Japan where every cow is subject to mad cow disease testing before entering the market, the U.S. only tests mad cow disease in a small percentage of the downers, who cannot walk into the slaughterhouse or die on the way. So far, two mad cows have been reported in the U.S. One was in the state of Washington, which was imported from Canada, and another in Texas. No one knows for sure how the cow acquired the disease.

Also, the protocol for mad cow disease testing does not seem effective enough. It took six months for scientists in the U.S. and U.K. to perform four tests to confirm the second U.S. case of mad cow disease.

Furthermore, it appears that states and local organizations are not allowed to voluntarily test mad cow disease in their cattle. Montana and California indicated their intention to test the disease in the cows in their states. The USDA says the states do not have the authority of conducting such a test.

The U.S. feed rule, the mad cow surveillance program, and the testing protocol may potentially leave the door open for the mad cow disease to transmit from animals to animals and humans. That is, there is a potential that people may eat tainted animal meat and get infected with vCJD, with or without knowing it.

The U.S. "select and test" approach is one of the reasons Japan hesitates to lift its ban on the U.S. beef. The Japanese Food Safety Commission claimed that if they adopted the U.S. surveillance program, they would have missed six cases of 18 cases of mad cow disease detected in Japan. They found that mad cow disease not only inflicts cows older than 30 months. Downers are usually already in an advanced stage of mad cow disease if they test positive.

According to People for the Ethical Treatment of Animals, "two studies on Alzheimer's victims showed that 5.5 percent and 13 percent, respectively, of patients who had been diagnosed with Alzheimer's had, in fact, been suffering from the human variant of mad cow disease, Creutzfeldt-Jakob Disease (CJD). The National Foundation for Infectious Diseases states that one out of every million people gets CJD, thereby giving the United States about 270 cases, but if even 1 percent of the 4 million Alzheimer's patients in the U.S. actually have CJD, we would have 40,000 cases, not 270. PETA is calling on the Centers for Disease Control and Prevention (CDC) to track CJD to determine whether we have an epidemic."

If this statement holds true, there might be 400 cases of vCJD among the 40,000 cases of CJD, because one percent of VJD cases can be vCJD, according to Idaho officials.

It is unknown whether the cases of CJD in Idaho can be classified as clusters. Clusters of CJD somewhat indicate the disease does not occur naturally. Idaho officials may leave the judgment to the Centers of Disease Control and Prevention. Yet, the Idaho incidence of Creutzfeldt-Jakob Disease is not a single event. Suspected clusters of CJD have been reported in some states: Pennsylvania in 1993, Florida in 1994, Oregon in 1996, New York in 1999-2000, Texas in 1996, and recently in New Jersey, according to a news story of United Press International (UPI) published on December 29, 2003. Except for some cases, all the cases have been classified as sporadic, meaning they occurred naturally, according to the UPI.

It remains to be confirmed whether the Idaho cases of CJD occurred naturally or acquired through consumption of tainted beef or other animal meats. Whether it proves to be CJD or vCJD, the risk of mad cow disease to humans seems to be real. The UPI reported that a study released in December, 2002 indicated "the mad cow pathogen can cause both sporadic CJD and the variant form." That is, even if the Idaho cases turn out to be CJD, that does not mean they were not caused by consumption of beef infected with mad cow disease.

Mad cow disease - bovine spongiform encephalopathy, CJD and vCJD are transmissible spongiform encephalopathy (TSE) that infects the brain and central nervous system of cows and humans. There is no cure or treatment for the brain-wasting disease.

Recent studies have found that infection of mad cow disease agent prion does not have to cause symptoms of mad cow disease, and researchers suggested that some cases of the infection may be mistakenly diagnosed with Alzheimerís. Up to recently, mad cow disease, vCJD, and Alzheimerís couldnít have been tested for sure unless an autopsy was performed on brain tissues.


1) Christopher Smith, Associated Press writer, 10-01-2005:
Tests show two women died of brain-wasting disease.

2) People for the Ethical Treatment of Animals, date unknown:
Mad Cow Disease: What the Meat Industries Don't Want You to Know.

3) Steve Mitchell- United Press International- 12-29-2003:
Mad Cow: Linked to thousands of CJD cases?

4) NIH/National Institute of Allergy and Infectious Diseases Ė 06-03-2005:
Variant prion protein causes infection but no symptoms.


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