Intro To BSE
What Is It?
History - Snowball Style
How'd This Mess Get Started?
Gimme Grass Baby

Prion? Whazat?
A Rose By Any Other Name
TSEs in Animals
TSEs in People
How Do You Get CJD
Meet the First Infant With CJD

Across the Border
Stronger Than Bear's Breath
How Much Is Too Much
CJD In Canada
CJD - Not in My Town! Not!
Kept Like a Mushroom


The case of Janet and Amanda establishes that the disease can be passed from mother to child. John Collinge, the Medical Research Council's professor in charge of prion research, stated: "It was something that was always on the cards. In sheep scrapie, a similar prion disease, the disease passes from ewes to their lambs. There is good evidence that in cattle about one in 10 infected animals transmits the disease to a calf. The prion that causes BSE is identical to the one found in humans with nvCJD, so it is logical that there would be a risk of nvCJD jumping from mothers to children."18

Backing up Collinge's statement, Johns Hopkins University findings show that BSE can move vertically, meaning from cow-to-calf, about 5-10% of the time.19 The same has been shown true to sheep with scrapie passing vertically from ewes to their lambs, and horizontally from lamb to lamb by vaccine injections. If this occurs in cattle and sheep, why not in other animals? Though the occurrence is low, we know it can occur in people.


In many illnesses, biological differences between species prevent diseases that originate in one kind of animal from infecting another. This is the "species barrier."

The assumption that TSEs could not cross species was a huge error. In 1990, Max, a Siamese became ill with the feline version of BSE and later that year, scientists showed the disease could be transmitted orally to mice fairly easily.20

"When a spongiform encephalopathy crosses a species barrier, a frightening phenomenon can take place. As the disease passes among members of the new species, it can become stronger and more virulent, and its incubation time can shrink. Then this new type of disease may be able to infect other species that were not previously susceptible."21

"Mad cow" has been found in herds of deer, elk and sheep. Various forms of TSEs have shown up in 24 animals so far including puma, cheetah, oryx, ocelot, sheep, free-ranging and captive mule deer, white tailed deer and elk, guinea pigs, eland, marmoset, cattle, squirrel, bison, lion, tiger, goat, lemur, mink, ankole, cat, ostrich, squirrel monkey and antelope.22 NOTE: This does NOT include all subspecies.


What do vampires and TSEs have in common? Both are nearly immortal. TSEs resist alcohol, boiling, freezing, domestic bleach, formaldehyde and hospital detergents. In an interview with Dr. Paul Brown, he told Nova/PBS: "We thought it would be interesting to see what would happen if we buried some of these agents, and so I ground up some scrapie brain and mixed it with soil, put it in a flower pot, enclosed it in a cage, and used my own garden as a burial site -- right here. And what we found was that a good deal of the infectivity remained in the soil after three years. We exposed it to temperatures that turned it to ash, and it did not entirely kill the agent, and so every known pathogen of man would have been destroyed by this process, and this was not."23


Good question as the answer is still frustrating scientists. It seems some people have a higher tolerance for infective prions than do others. Further complicating risk assessment is the issue of cumulative effect. Can you ingest smaller doses of diseased food over longer periods of time and it have the same effect as eating a massive quantity on one or two occasions?

They do know that feeding a cow as little as 1/4 teaspoon of BSE will kill that animal. But since BSE is not the strain found in people, how much has to be consumed to cross the specie barrier? Unfortunately, scientists don't have the answer to either question.


Cases from
April '98 - May '00

Canada's CJD-Surveillance System was launched in April 1998. Within six months, doctors had reported 85 cases. The majority of illnesses have been occurred in Ontario, British Columbia and Quebec following the areas of highest population.

Of these TSEs, three victims were diagnosed with GSS, an inherited form, and in three cases people contracted CJD in a medical setting where dura mater was involved. No cases of vCJD have developed.

Though CJD became a reportable disease January 1, 2000, only the location where the case occurred is identified, not the patient.

Source: Laboratory Centre for Disease Control, Health Canada


Officially, the occurrence of CJD is one case per million people annually. This would mean there are 286 new victims every year just in the US, 6200 new cases globally.

The graphic shows where the victim lived at the time of death. This does not necessarily indicate where the disease was contracted.

For example, someone living in California may have gone hunting in southern Wyoming where there is a high incidence of CWD and ate contaminated venison from that state.

In Moo Madness - Part 2 we'll look at the "cow connection", what's being done in Canada, the US and Australia to control the spread of Mad Cow, examine the chances of Mad Cow getting in your food and look at whether we are being told the whole truth.


Stan and I wanted to include more specifics on the occurrence of CJD in Australia, but like so many other instances in that country, the government is NOT forthcoming with information. Complicating matters is the fact that in Australia CJD is
not a reportable disease.

The occurrence of CJD is seen the world over. There are no exceptions in the statistic of 1 in every million with come down with CJD. Considering Australia has 19,750,000+ residents, 19 - 20 new cases per year of CJD would be a fair assessment.

Time and again, we've been frustrated at the lack of communication between this country's government and its taxpayers. We PAY the government for this information and it is their duty and obligation to dispense it. Canada and the United States are absolutely top shelf when it comes to divulging data, even it if's not good news. At least they keep the public informed.

The Australian government states there is a BSE surveillance program checking bovines, but there are no specifics on how many cattle are checked yearly or by what method. We have the uncomfortable feeling that a great deal of complacency is setting in and that's exactly what got the UK in a pile of trouble.

Since CJD is not reportable, "accurate case ascertainment is largely dependent on voluntary reporting by medical practitioners."25 Mailouts are sent to neurologists and pathologists twice a year in an effort to prompt notification of recent or prospective cases of CJD. The only other way to ferret out this data is to search the ~130,000 annual Australian death certificates. Lance Sander, the scientist who is quoted in this paragraph, also wrote that in view of possible blood donations coming from CJD-tainted people, "the time has now come for enhanced surveillance in Australia of all human TSEs."26 Gee, ya think? Good on you, Lance for speaking out! You're apt to be the lone voice in the wilderness. . .

With affection,
Holly and Stan
Seismo and Taco

Taco: (crunch... crunch... pause...) "Say, Seis, these sure are munchy dry food pellets The Parents have been feeding us... I wonder if they've got any of that BSE stuff in them..."
Seismo: (snorf... snorf... gobble... gobble...) "hmmmm... dunno,... say, do you want the rest in your bowl?..."
Taco: "Sheesh!, Seis, you've got a one-track mind... er.r..r... stomach!"
Seismo: (burp...) "Hmmm, BSE?.... BSE?.... yup... there's plenty of that in these dry food pellets for us canines. Ain't it great!?"
Taco: (dropping a chunk back in her bowl) "Seismo!... What are you saying!!!"
Seismo: "Well, let's see.... BSE stands for Bones, Steak and Everything, doesn't it..."
Taco: "Right..., Seis.... you big lummox.... it's that 'everything' you'd better worry about!
Seismo: "Taco, we're going vegetarian... well, at least until they find a cure for that stuff...."

Stan and Holly Deyo
P.O. Box 7711, Pueblo West, CO 81007

© Text and Graphics, 2001 Stan and Holly Deyo, except where otherwise credited


1BSE - The History; From New Scientiist; October 25, 2000; http://www.keysites.com/nsplus/insight/bse/history.html
2Prion Diseases Spreading Through Grains?; By Dr. Len Horowitz; The Idaho Observer; September 24, 1999; http://www.fortunecity.com/healthclub/cpr/349/prions.htm
3 Prion Diseases Spreading Through Grains?; By Dr. Len Horowitz; The Idaho Observer; September 24, 1999; http://www.fortunecity.com/healthclub/cpr/349/prions.htm
4Plain Truth From the Cattlerancher Who Won't Eat Meat; by Howard Lyman with Glen Merzer; http://www.madcowboy.com/
5Dormant But Deadly; September 9, 2000; http://www.smh.com.au/news/0009/09/text/review5.html
6NOVA #2505: The Brain Eater; February 10, 1998; http://www.pbs.org/wgbh/nova/transcripts/2505braineater.html
7Prion Diseases, November 2000; http://www-micro.msb.le.ac.uk/335/prions.html
8Humans Can Get Transmissible Encephalopathies; http://www.mad-cow.org/
9"Mad Cows", Englishmen And The Prion Hypothesis; Dorothy B. Preslar;
10Prion Diseases, November 2000; http://www-micro.msb.le.ac.uk/335/prions.html
11What Is A Prion?; http://www.sciam.com/askexpert/medicine/medicine14.html
12Prion Diseases, November 2000; http://www-micro.msb.le.ac.uk/335/prions.html
13 Surgery May Raise CJD Risk Small Study Links Operations, Mad Cow Disease
14 French Scientists May Face Charges Over CJD Outbreak; Michael Balter; Science 1993; 261:543
15 CJD Support Group Network Inc. http://www.cjdsupport.org.au/
16 Mad Cow and Human Prion Disease; May 18, 1998; Neuro News;
17 The Prion Diseases; by Stanley B. Prusiner; http://www.sciam.com/0896issue/prion.html
18 Can CJD Be Passed From Mother To Child?; Johnathan Leake; March 5, 2000; London Sunday Times; http://www.sunday-times.co.uk/
19Johns Hoplins University; http://www.jhu-prion.org/public/pub-ds-dca.shtml
20BSE - The History; From New Scientiist; October 25, 2000; http://www.keysites.com/nsplus/insight/bse/history.html
21NOVA #2505: The Brain Eater; February 10, 1998; http://www.pbs.org/wgbh/nova/transcripts/2505braineater.html
22Zoo Prion Disease: Review of Scientific Literature
23NOVA #2505: The Brain Eater; February 10, 1998; http://www.pbs.org/wgbh/nova/transcripts/2505braineater.html
24New Rules To Cut BSE Risk; January 1, 2001;
25Communicable Diseases - Australia National Centre for Disease Control/Communicable Diseases Network Australia New Zealand Australian Department of Health and Aged Care; Communicable Diseases Intelligence; Lance Sanders; Principal Scientist, Surveillance and Management Section, Population Health Division, Department of Health and Aged Care, Canberra, ACT. Vol 24 No 9; http://www.health.gov.au:80/hfs/pubhlth/cdi/cdi2409/cdi2409a.htm

The Prion Diseases, Scientific American; August 1996; Stanley B. Prusiner; http://www.sciam.com/0896issue/prion.html
The Transmissible Spongiform Encephalopathies (Prion Diseases); http://www.jhu-prion.org/definition/
Prion Diseases, November 2000; http://www-micro.msb.le.ac.uk/335/prions.html
The Creutzfeldt-Jakob Foundation; http://cjdfoundation.org/CJDInfo.html