# Am I missing something?



## Huntnut (Jan 21, 2000)

OK,

I keep reading that there aren't any live animal tests for CWD...that you have to euthanize the animal and test it's brain tissue...

Then I read that the abnormal protein prions can be identified in urine and feces..

"Mr. Miskosky, writing in the current edition of Alberta Outdoorsmen, says scientific journals confirm that TSE prions, the mutant protein that causes CWD, have been found in urine long before the disease is detected in animals"

I guess my confusion comes from the term TSE prion (I thought this was sheep scrapies) causing CWD?

I thought they were independent of one another tse=sheep and cwd=deer.

Now TSE causes CWD?

So doesn't it stand to reason...that an animal that has the TSE prion in it's feces, definitely unquestionably has or will get CWD?

Is this like HIV being the prelude to AIDS?

And can't this prion detection in urine and feces be conducted while the animal is alive?

And if it can be detected in feces, wouldn't testing a vast statewide collection of wild spoor give researchers a clue on whether TSE is in the wild herd, and how far it has spread if it is?

I guess my biggest question is WHAT prion can they identify in spoor but not in live animals?

Major confused.....

Anybody?

Hunt


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## Tom Morang (Aug 14, 2001)

I don't know Huntnut. Maybe it's just that things take time and money to prove? Maybe this will shed a little more light on the subject, then again maybe not. 


Posted May 11, 2002 

DNR hunts for test for chronic wasting disease

Veterinarian envisions method easy for hunters 

By Ed Culhane 
Post-Crescent staff writer 

Researchers and entrepreneurs are racing against the clock to develop a test for chronic wasting disease in white-tailed deer that can be used by hundreds of thousands of hunters.

It is the number one priority sitting in my realm, currently, said Julie Langenberg, a DNR wildlife veterinarian and a member of the state's inter-agency science and disease team at the heart of the battle against the disease.

Currently, there is no method to test a live deer for the disease. Wildlife officials can test recently killed deer by removing a piece of the brain stem and sending the sample to the U.S. Department of Agriculture laboratory in Ames, Iowa. Wisconsin is currently without a lab certified to do the test.

Langenberg envisions a system in which hunters could cut off a piece of the deer's spleen, put it in an approved container and mail it to a lab, receiving the results within a week. The spleen is a large, easily recognizable organ that hunters remove when gutting an animal in the field.

The theory is that it should work, she said Friday. That is our goal, absolutely, to develop a system that can accommodate hundreds of thousands of hunters that is not going to cost the hunter very much and that is going to provide good, reliable results.

There is no evidence that eating meat from an infected animal causes any health problem for humans. However, scientists do not fully understand the means of transmission and so cannot provide an iron-clad guarantee. 

The disease itself is so terrible sponge-like holes form in the brain, gradually leading to dementia and death that some people have said they will not eat venison, no matter how slight the chances of infection.

It is a huge perception problem, said DNR deer researcher Keith McCaffery. It is probably safer to eat venison than the beef off a grocer's shelf.

Some local hunters share that view, said Don Rogalski of the Bow Hunters Shop in Black Creek.

One guy put it rather bluntly. He said he'd be damned if he would throw his good venison out and go to the store to get a chicken and get salmonella.

But others are frightened, and for wildlife officials, perception becomes reality. 

During a visit in Appleton this week, DNR Secretary Darrell Bazzell said finding a way to assure hunters that deer meat is safe is critical.

Hunters manage the deer herd for us, he said.

In current lab testing, technicians prepare the sample of brain stem on a microscopic slide and the sample is treated with a stain that will stick only to the prion associated with the disease, a mutated version of a naturally occurring protein. A pathologist then examines the slide and determines whether the disease is indicated.

It is the gold standard test,Langenberg said. It is highly accurate. But it is slow and it is costly. It involves a high level of expertise and it involves some expensive equipment.

A variety of other tests are being studied. Researchers are trying to determine whether a simple test for mad cow disease developed in England can be altered to detect CWD. Researchers in western states, where CWD has been present for decades, have developed experimental tests that show promise.

One area of research is seeking a way to detect the disease elsewhere in the animal, as in its tonsils, or in the spleen. The mutated prions are present not only in the brain but in other organs where metabolic activity is high.

If the system Langenberg envisions worked, volume would be a concern.

By the end of this summer, the Wisconsin Veterinary Diagnostic Laboratory in Madison will be able to test for CWD, Langenberg said. Other labs are seeking certification as well. 

This will help the fight tremendously, she said, since the DNR now needs to process thousands of samples from around the state. But these labs could never take samples from tens or hundreds of thousands of hunters.

Instead, Langenberg said, state officials are working with private labs that already do diagnostic testing of animals and even with entrepreneurs who recognize a need and an opportunity but have no expertise.

One obvious business problem is the seasonal nature of the hunt. A lab would receive few samples throughout the year, then be inundated with them during a few weeks in November.

This whole test development is going to happen in phases, she said. Where we will be in a year is light years from where we are now. 

Ed Culhane can be reached at 993-1000, ext. 216, or by e-mail at [email protected]


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## Fierkej (Dec 21, 2001)

Hi,
First, I will try to clear up the confusion between TSE, sheep disease and CWD. 

The disease in sheep is called Scrapie.
The disease in elk and deer is called Chronic Wasting Disease (CWD).
The disease in cows in Europe is called Bovine Spongiform Encephalopathy (BSE), also called Mad Cow Disease.
The disease in humans is called Creutzfeld-Jacob Disease (CJD). 

All of the above are in the category of *Transmissible Spongiform Encephalopathies (TSE's)* . They are all TSE's. They all cause similar symptoms in the animal, but they are separate diseases. Another name for TSE is "prion disease" as TSE's are all thought to be caused by prions. Prions (Proteinacious Infectious Particles) are normal proteins that have been changed into abnormal proteins, and cause damage to neurons which shows up as holes in the brain (a spongy appearance). 

It is _speculation_ that Scrapie might have spread from sheep to deer/elk causing CWD. 

I have not yet been able to find journal articles about detection of prions in feces and urine. That might have been for Scrapie, as the disease in sheep has been recognized longer and has been more extensively studied. 
The only acceptable tests for the detection of prion protein that causes CWD are immunohistochemistry tests (IHC) and the Western Blot Test. The Western Blot Test is done at the National Veterinary Services Lab in Ames, Iowa. I'm not sure, but IHC may be done at MSU.

Jean


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## Hamilton Reef (Jan 20, 2000)

Thank you Jean. You are most valuable through this difficult period defining the terms accurately and in a way we can understand to pass on.


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## Huntnut (Jan 21, 2000)

Yes, thank you Jean...

I find it interesting that the author mentions prion identification in spoor....as supported by some scientific journals....

I sure would be interested in which journals this was published!

If I knew that, I would run over to the University and read the studies!

If I read you correctly- cwd, bse, cjd, and scrapies are all TSE prions....

So it goes to figure anyway, that any TSE prion found in deer spoor makes the animal a CWD candidate.

Something about this statement from the author:

"scientific journals confirm that TSE prions, the mutant protein that causes CWD, have been found in urine long before the disease is detected in animals"

I'm not so sure he has his facts straight....and if he does, this is the first I have heard about TSE prion detection in spoor.

Interesting.

Hunt


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## Huntnut (Jan 21, 2000)

Something interesting I found....notice the date.....
I am researching the author...


Mad Cow Disease 
March 1, 2001 

Mad Deer and Mad Cows BSE, TSEs. CJD, nvCJD, Scrapie and Kuru 

Mark Konlee 

For the past few years, Americans have heard news on television and in newspapers of a fatal brain disease affecting cows living in Great Britain called Mad Cow Disease. Mad Cow Disease is known as Bovine Spongiform Encephalopathy or BSE. Over 180,000 cows in Great Britain were found to be infected with BSE and over 4 million have been killed and replaced with apparently clean imported animals. BSE is thought to have been passed on to persons who ate beef from the infected cows. 

There are now 94 persons in Great Britain that have been diagnosed with the bovine derived version of Creutfeldt-Jakob Disease (CJD) called (nvCJD). Im not sure what the nv stands for, but keeping track of all the names is a task in itself. Regular CJD kills about one person in every million each year in countries all around the planet, but the media is paying little attention to this problem and neither is anyone else. 

Medical researchers state that they dont know what causes CJD, but suspect either misfolded proteins called prions, a virus or some other pathogen. In reference to all forms of prion diseases in all species and animals, they are called Transmissible Spongiform Encephalopathies or TSEs. Today, little attention is being paid in the media to the epidemic of TSEs in deer, antelope, elk and other animals in the wild. 

When the disease first appeared in sheep 200 years ago, it was called Scrapie. Early in the last century, when it was found among cannibals living in New Guinea, it was called Kuru. The incubation time for the disease has been estimated by one source to be 5 to 10 years while another claimed 1 year to 30 years. 

A local pathologist told me that most persons diagnosed with the disease (CJD) die within one year and that the symptoms are indistinguishable from Alzheimers. With recent news of infected cows showing up in Germany, France and Switzerland, panic has swept throughout Europe. Sales of beef have plummeted and concern has spread around the globe. In January, France banned the sale of T bone steaks, because of their close proximity to potentially infected meat in the spinal cord. 

The origin of the problem in Great Britain is thought to have been caused by feeding parts of sheep infected with Scrapie to cows in their feed. The most infected part of any animal with Spongiform encephalopathy is the brain and spinal cord and all tissues that are near it. One article at the Center for Disease Controls website (www.cdc.gov) reported a change in processing animal parts in the 1980s may have rendered the feed meal less sterile. 

CJD (not beef related) is killing one person in every million each year.
Regular CJD kills about one person in every million each year, most over the age of 60, while the beef derived version called nvCJD affects people is all age groups including children. Regular CJD is now taking about 300 lives annually in the United States. While the media has reported deaths from Mad Cow Disease in England and the spread of the disease to France, Germany and Switzerland, it has not reported that people are dying from CJD in the United States each year - an average of 238 per yr. over the past 20 years.. 

Medical authorities seem less concerned about these cases because they are not apparently related to consumption of contaminated beef but the cause of regular CJD is reportedly unknown. However, the facts as I observe them are that regular CJD is an even greater threat than nvCJD and is coming from infected deer and other animals in the wild that are either undercooked or handled in an unsanitary way to cause cross-contamination with other foods like salads, bread, etc. 

Cattle infected with BSE have also been reported in Belgium, Denmark, Austria, Finland, Czech Republic, Greece, Ireland, Italy, Spain, Portugal, Bulgaria, Hungary, Poland, Sweden, Norway, Romania, Albania, Oman, Croatia and the Falkland Islands. Genetic predisposition in people Researchers have found that about 40% of the population has a genetic predisposition to develop the disease due to a gene called PRNP codon 129 M/M genotype, that confers a faster incubation time. All cases of nvCJD in England have thus far carried this gene also called the Met/Met gene. 

New York Times reports that the US Deer population has an Epidemic of Spongiform Encephalopathies (chronic wasting disease) 
While Spongiform Encephalopathy (Scrapie) was first observed in sheep about 200 years ago, other TSEs have affected other animals including deer, antelope, elk, mink and squirrels that live in the wild. In some states, estimates are that 10% or more of the deer population are infected with spongiform encephalopathy. 

On January 4, 2001, The New York Times on its front page reported the following: 

Although mad cow disease has not been detected in cattle in the United States, a related malady called chronic wasting disease is spreading rapidly among deer and elk herds, captive and wild, in six Western states and in Canada. In laboratory dish experiments, chronic wasting disease has been shown to infect human cells, in principle, hunters who ate infected deer or elk meat could have the disease and if they donate blood, could pass it on. 
Last month, a local women called to tell me that her brother who lives here in Wisconsin ate venison from a deer they killed this past fall. They had the deer examined to see if it had any diseases but didnt wait for the results of the examination to eat their first meal of venison. After having a few meals of deer meat, they got the bad news that the deer had chronic wasting or a brain infection of spongiform encephalopathy. Now, they are worried that they will get the disease. 

I told the reader that if they cooked the venison well that they need not worry, but to be wary of handling the infected meat and then make a salad without washing their hands in soap and water and preferably water with bleach in it. 

NOTE: Be Aware that knives and cutting boards not properly bleached and washed could spread the infection to other foods. There is a big risk factor is using the same cutting board for both meat and also bread and salads. Everyone should have two cutting boards with one used exclusively for meats and the 2nd one for other foods. The same goes for knives, and finally, hands touching raw meat must be washed in bleach water with soap added and then rinsed well under running water to avoid the risk of cross contamination. 

Research Letter in the Journal of the Am. Medical Assn. (Nov. 8, 2000) by Robert Gibbons et al from the Center for Disease Control reports 4,751 deaths from CJD in the United States from 1979 through 1998. 
That is an average of 238 deaths from CJD per year with a slow gradual increase in the number of cases reported by 1998 when they reached about 1 in every 779,000 people. The article also reported 10 cases in persons under 30 years of age. In the United States, the median age of deaths from CJD was 68. In England, the median age of deaths was 27.5 years of age. In the 10 cases in the US involving persons under 30 years of age, one was linked to the use of human growth hormone and nvCFD was ruled out in 2 other cases. No evaluation of the other 7 cases was mentioned in the article. Normally, CJD only affects people over 60 years old. After the age of 60, the odds of developing CJD increase to about one in every 160,000 persons. 

Medical experts state one in a million cases of CJD occurs each year 
It may be unknown, but it is not spontaneous. It is coming from somewhere. In my opinion, 99%+ of all the CJD cases in the United States and throughout the world are coming from undercooked animals killed in the wild (deer etc.) and from the mishandling of these raw meats in kitchens that results in cross-contamination. It could not be coming from the prions or we would have had a world wide epidemic long ago. The prions are not destroyed by cooking the meat, but the infectious agent is likely destroyed by boiling or proper cooking. CJD must be coming from a virus or other infectious agent co-mingled with the prions or a strain of mycoplasma that is destroyed by the cooking process. 

What causes spongiform encephalopathy? (prions, mycoplasma or virus?)
Scientists cannot agree on what actually causes the disease or how to treat it. The most common theory is the prion theory. A prion is a misfolded protein that is thought to be pathogenic and self replicating. The prion is not destroyed by heat as is a virus, mycoplasma or bacteria. It takes a very strong acid like formic acid or an alkali like sodium hydroxide to break it down. Prions have been observed in all cases of spongiform encephalopathies in different species. The prion theory contributes to the public panic but other researchers believe that the prions are the result of an underlying infection from a virus or mycoplasma. After evaluating available data from various sources, I no longer believe that the prion proteins are the infectious agents in any type of spongiform encephalopathy but that the real infectious agent is a virus or mycoplasma that co-mingles with the prions. 

More information on CJD and Mad Cow Disease can be found at the CDC website at www.cdc.gov. In the search box, type in either CJD or Mad Cow and you will turn up a long list of articles on the subject. 

The Food and Drug Adm (FDA) has taken a significant number of steps over the past few years to prevent the spread of BSE to the United States including banning the importation of glandulars and gelatin from countries where BSE outbreaks have occurred and in 1997 banning the feeding of animal parts including bonemeal to other animals except for cat and dog food. The problem I see is that they dont have enough inspectors to enforce the new regulations. By the way, I would not buy cat or dog food cereal that contains either lamb or beef parts. It is safer for your pet to buy a turkey or chicken based formula or if you want to feed beef parts to your cat or dog, used canned pet food only which would be cooked and therefore sterile. 

Researcher links Spiroplasma, a type of mycoplasma, to Spongiform Encephalopathies 
Frank Bastian MD, professor of pathology at the University of So Alabama College of Medicine, Mobile, AL, quoted in an article published in JAMA, Aug 14th, 1996, first reported his research that Spiroplasma, a type of mycoplasma, is the likely infectious agent that causes Transmissible Spongiform Encephalopathies (TSEs) in sheep and other animals. 

JAMA: Bastian, on the other hand, regards the prion theory as a red herring. the cause of transmissible spongiform encephalopathies (TSEs), he says is a conventional microorganism - a mollicute or, more specifically, a spiroplasma. The infection-related protein (prion) is produced by the host in response to the infection. 
Following the 8/14/96 JAMA article is a media article by Ed Gherman titled Spiroplasma and Transmissible Spongiform Encephalopathies. Bastian based his views on 20 years of research. He reported evidence of spiroplasma-like inclusion bodies seen in brain biopsies from patients with CJD. He reports that spiroplasma internal fibril proteins are identical morphologically to those seen in TSEs and that the spiroplasma proteins show cross reactivity with the TSE proteins and that spiroplasma, when injected into rodents, produced the brain disease. 

Bastian also stated that there are numerous reports of separating the prion protein from the infectivity. Finally, he reports that the immune system is involved and that in both naturally occurring TSEs and in experiments, there are autoantibodies and leukopenia. He said that the infectious agent, a spiroplasma, is dangerous because it can jump species. He said that we need to develop a test to find the presence of the spiroplasma, and that just looking for the prion proteins is not enough. 

The article by Ed Gherman, revised on June, 1998, reported the size of the transmissible agent (Spiroplasma) to be 42 nanometers (nm) in size. The molecular weight of the spiroplasma is 27,000 to 30,000 daltons. Spiroplasma affinity is for nerve and brain tissues. 

The research of Bastion indicates that an ultra filtering system that would remove particles of 42 nm or greater would be needed to completely remove the transmissible agent. A filtering system of 30 nm would certainly remove the transmissible agent. 

In my opinion, the infectious agent first sets up housekeeping in the intestines, probably in nerve tissues. Normally, friendly flora and healthy mucus membranes in the gut will prevent the infection from ever taking root. However, when mucosal immunity in the intestines and cell mediated immunity fail is when the infection can spread inside the body and eventually reach the brain. Since spiroplasma is a mycoplasma, an infectious agent that is between a virus and a bacteria, some wide spectrum antibiotics that have killed other mycoplasmas might also inactivate spiroplasma. For an immune defense, the CD8 Killer T cells would likely be the most effective defense internally. Maintaining a colony of friendly flora, acidophilus, bifidus and infection fighting strains like L Salivarius in the intestines would be a good natural defense against accidental exposure. 

My own analysis of the spread of CJD from the cannibals in New Guinea to the British experience with nvCJD to naturally occurring CJD cases worldwide indicate that bringing any food up to the boiling temperature of water will kill the infectious agent (spiroplasma or virus) even though the prions themselves are not destroyed. Once cooked, stomach acid breaks down the prions. The most likely cause of disease spread is through the handling of raw infected meats in an unsanitary manner (hands, knives, cutting boards etc.) that transfers the infectious agent to other foods.


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## Huntnut (Jan 21, 2000)

Did any of you catch this part?

"Last month, a local women called to tell me that her brother who lives here in Wisconsin ate venison from a deer they killed this past fall. They had the deer examined to see if it had any diseases but didnt wait for the results of the examination to eat their first meal of venison. After having a few meals of deer meat, they got the bad news that the deer had chronic wasting or a brain infection of spongiform encephalopathy. Now, they are worried that they will get the disease."

March, 2001 ??????


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