Prions
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Deer in three Wyoming counties infected with chronic wasting disease
Chronic wasting disease: deer-to-cattle shown
Worry over CWD hazards
Canada reports CWD in mule deer on game farms
Dr. Steven Dealler on CWD risks
Concerned rancher writes in about deer feeding habits
To eat or not to eat is the hunter's question
Scrapie in New Mexican bighorns -- make that scabies?
The US testing program for BSE
The Brain Eater ....tv special from NOVA
Narang reporting older cases of nvCJD
WHO experts say mad cow'' epidemic possible
Veef-on_bone enforcement bite

Deer in three Wyoming counties infected with CWD

Sat, 07 Feb 1998   Casper Star Tribune  and AP
The news here is that (1) CWD seems to be spreading out of its epicenter near Ft. Collins and (2) it can be transmitted intra-cerebrally back to cattle. CWD is a transmissible spongiform encephalopathy affecting mule deer, white-tailed deer, and elk and involves the prion protein in these animals.

Local authorities seem to be saying it is safe for hunters to eat the dorsal root ganglia of a cwd deer (porterhouse venison). This Web site strongly and emphatically disagrees -- we say hunters should not touch or eat any part of any wild ungulate from these Wyoming and Colorado counties until the situation is clarified. After passage through different hosts, the infectivity becomes completely unpredictable. For all anyone knows at this point, CWD could be worse than BSE in terms of transmissibility to humans. Who is measuring the infectious titre before the hunter eats the animal? Nobody. A lethal infectious dementia is not worth risking in our opinion.-- webmaster

RAWLINS, Wyo. - An infection related to mad cow disease has been found in about 1 percent of deer in three southern Wyoming counties, state veterinary officials said.

Dr. Beth Williams, a veterinary pathologist at the state Veterinary Lab in Laramie, said about 1 percent of deer tested this year in Laramie, Platte and Goshen counties appear to have chronic wasting disease.

Wildlife biologist Mark Zornes said the disease has been found in about 6 percent of deer in northeastern Colorado, but "we're not finding it nearly as prevalent here." He said the disease is active in the wild only in a small area of Wyoming and Colorado.

Williams said chronic wasting disease is not considered a human health hazard, but she advised hunters against eating the spinal cords or brains of deer or elk because the organism that causes the disease concentrates in those organs. [Is it ok to eat the dorsal root ganglia of 'porterhouse venison' and post-synaptic neuromuscular junctions ? -- webmaster]

The wasting disease is related to bovine spongiform encephalopathy, which infected many cows in Britain last year. No case of the bovine disease has been found in the United States.

Williams said chronic wasting disease can infect cattle only if infected tissue is injected directly into the brains of livestock. [Feeding experiments depend critically on the amount used and time of incubation. Animals may be asymptomatic yet have very high infectious titre of agent. -- webmaster]

Zornes said it takes up to two years for chronic wasting disease to show symptoms in mule deer, white-tailed deer and elk. Infected animals lose weight and coordination and drink unusually high amounts of water. Once symptoms begin to show, it usually dies within a few weeks, Zornes said.

Janice M. Miller responds:

National Aniimal Disease Center USDA ARS 13 Feb 98 Listserve
"I'm not sure where the misunderstanding originated but I thought everyone should know that CWD has NOT been successfully transmitted to cattle. Intracerebral inoculations of calves with CWD inoculum (mule deer origin), which was provided by Dr. Williams, were done just a few months ago at the National Aniimal Disease Center (USDA-ARS) by Dr. Randall Cutlip.

Of course it will be some time before any results are forthcoming. Experimental exposures of cattle by feeding and contact were being planned by Dr. Williams at Laramie and her collaborators at Colorado State. I believe those trials are underway but it would surely be too early for any conclusions to be reached."

Testing for BSE in the US

12 Feb 98 Listserve correspondence
The USDA APHIS Veterinary Services maintains an excellent Web page concerning the status of the BSE surveillance program in the US. As of February 28, 1997, APHIS diagnostic laboratories examined a total of 5,427 bovine brain submissions and found no evidence of BSE in the US. A map of where these animals came from is also provided.

The questions with the program have always been how to select cows for testing (downer cows, with neurological symptoms, made sense) and how to test these animals for strains of bovine TSE other than, and in addition to, the British BSE strain of bovine TSE. The latter question arises because TSEs are highly variable in their symptoms in all species studied. If one looks only for the British strain, one might show that this strain is very rare or non-existent in the US. But what about strains such as the one studied by Dr. Richard Marsh in the Stetsonville, Wisconsin outbreak? Would the cow selection method and diagnostic procedures designed to detect British BSE also detect all other transmissible bovine encephalopathies?

The just-released abstract below is a little curious but not unreasonable on the semantics side. The authors describe "cattle that had died of encephalopathy after experimental inoculation with brain from scrapie-affected sheep" but did not call it BSE. Presumably cattle inoculated from healthy control sheep did not die of encephalopathy and nothing happened with second passage either.

Is it then a bovine TSE but a dramatically different strain from UK? Or just a lethal, infectious encephalopathy but not necessarily spongiform (TE). Regardless, it is evidently still transmissible, still lethal, at least cow-to-cow. That wouldn't be much of an improvement over UK BSE. It might not cause nvCJD but what would rule out some component of sporadic CJD?

The article seems to be saying three things, that UK BSE probably did not arise from this particular strain of scrapie, that bovine TSE or TE symptoms can take on a dramatically different cast depending on origin, and that the USDA monitoring program is quite possibly mis-focused. The goal is not trying to keep BSE out of US meat but rather every TSE-related infectious encephalopathy that could cross a species barrier (this includes BSE).

Second passage of a US scrapie agent in cattle.

J Comp Pathol 1997 Oct;117(3):271-275
Cutlip RC, Miller JM, Lehmkuhl HD
Scrapie and bovine spongiform encephalopathy are similar chronic neurodegenerative diseases of sheep and cattle. An earlier study showed that, on first passage in cattle, a US scrapie agent caused an encephalopathy that was distinct from bovine spongiform encephalopathy (BSE).

The present report describes a second passage in cattle, carried out because diseases caused by the spongiform encephalopathy agents often change in character with additional passages in abnormal hosts. For this work, young calves were inoculated intracerebrally with a pooled suspension of brain from cattle that had died of encephalopathy after experimental inoculation with brain from scrapie-affected sheep.

The second passage disease was essentially identical with the first passage disease, as judged by clinical signs, histopathological findings and distribution of "prion protein scrapie" (PrPsc). This represents additional evidence to suggest that the US sheep scrapie agent tested is incapable of causing BSE in cattle.

Listserve question on 11 Feb 1998 from Linda Irene Zambenini

I'm not sure how many dairy plus beef cows are in the US. [101 million total], however, there is an excellent USDA BSE surveillance map. I was shocked to note on this map that of the total 5,621 of brains analyzed, 1,406 were in Kentucky. Only 88 in Wisconsin.

Since when did KY become such a big beef and dairy state?! I e-mailed them last week about this disproportionate excess in KY and have not heard a word back yet. Anyone out there know what's going on ? That is 1/4 of the total for the entire US. Think this has anything to do with cluster of CJD patients in KY that were thought to have been infected by eating squirrel brain?

Response

12 Feb 98 webmaster opinion 
The great thing about the cows from Kentucky and the reason that they may have been so heavily weighted in the BSE study is because they were suspected of having rabies as their neurological problem, if you follow me. This gets the head count up while keeping the risk of finding BSE low. The last thing you would want to include is a lot of very old downer dairy cows from, say, Stetsonville Wisconsin.

Because of all the bat caves, Kentucky is reportedly a high rabies state. I was told cattle rabies is mainly from the high bat population but there may be issues with foxes and raccoons, that it is not uncommon in cattle in the Southwest either but does not causing a human health problem as the virus is very heat-sensitive, downer cattle are pulled out of the human and pet food supply, etc.

Question:

If there are only 250 cases a year of CJD in the US, and if this rate has been steady for decades and the same worldwide, so what if a low level of bovine TSE exists in the US, it isn't causing a problem for anybody?

Response

Liz Armstrong of  CJD Voice (advocacy group of affected families)  11 Feb 98
The problem with CJD in the United States is that all but a few states do NOT report this disease. After many calls to state health departments, speaking with the disease reporting units, I discovered that most of them had no idea what CJD was and after they looked it up in the ICD-9 book, they replied, it is of unknown origin, with no treatment or cure and doesn't have to be reported. They refer to this as a class 5 disease. Which by the way, is the same class AIDS started out with. CJD Voice is growing by the day. We have been adding at least one new member per day at the least! The incidence of CJD is increased, the tracking methods just don't pick it up!

I do have some statistics on the real rate of CJD in the US. The CDC maintains that CJD attacks approx. 250 persons per year. PUH!!! More than 100 individuals that I am PERSONALLY aware of not only received blatant misdiagnosis but had to educate a neurologist as to what the symptoms were for CJD.

Without an efficient surviellance program in the US and the lack of information forwarded to the medical communities regarding CJD NO ONE can even guess as to how many individuals ARE dying from CJD. I do not mean to imply that US neurologists are lacking in education by any means, I blame this situation on the CDC.

Finally some states [such as Texas: 903-675-5626 -- reporter Keith Mitchner] are making CJD a reportable disease but it will take all states reporting acurate diagnosis of CJD before anyone can get an accurate figure. YOU tell me how an incidence of 250 per year could be accurate when there are so many misdiagnosed cases.... and those are only the cases I am FAMILIAR with!!!!

Mary Fishler-Fisk adds:

Question: Actual number of CJD cases is not important in such case: rate of variation is. Since you maintain that a lot of cases of CJD are misdiagnosed in the US, it's only logical to say that they were misdiagnosed in the past as well.

Response: How, pray tell, can you know if a number is increasing or decreasing if you have no base number to go from?

There is no way to know if there were 250 cases prior to all the controversy over BSE and nvCJD or if there were 1000, or 10,000 or 100,000. If the disease is as easy to misdiagnose as it appears to EVERYONE in CJDVoice who has PERSONALLY EXPERIENCED the level of knowledge and surveillance in the US medical community, then the original figures of 1 per million are way out of whack.

Sometime, somewhere, someone has to start taking reasonable surveillance seriously and establish a base line. It is only once that baseline is established that ANYONE can state whether or not there is an increase/decrease or no change in the number of CJD cases in the US.

Chronic Wasting Disease, Deer & Elk - Canada: Surveillance

Feb. 5/98  ProMED
Two Canadian provinces, at least, have initiated surveys for Chronic Wasting Disease in wild deer. Alberta began a survey last year and Saskatchewan is in the collection phase of its first survey year. British Columbia has made some preliminary plans for such a survey.

A great many wild deer brains have been examined over the past 30 years by Canadian veterinary pathologists, both wildlife disease specialists and general veterinary diagnosticians familiar with the lesions of scrapie, the equivalent disease in domestic sheep. No lesions typical of CWD have been detected except as reported previously for a small number of captive mule deer and one captive elk. [Sensitive modern methods of detection, such as prion immuno-assay, were very unlikely to have been used -- that and the epidemic in the south is why they are ramping up the monitoring. -- webmaster] Thus, there is some background evidence that the disease does not exist in wild cervids in Canada at a prevalence detectable under current conditions of surveillance. Further surveillance is justified, however. Health Canada recognizes its responsibility with respect to public safety and zoonotic diseases and is expanding its activity in this area.

We are indeed fortunate to have Dr. Harvey Artsob and his group and can look forward to expanded activity in zoonotic diseases when that group is relocated to its new facility in Winnipeg.

Contacts regarding Health Canada's program are:

Dr. Artsob or Dr. Robert Clarke
Frederick A. Leighton, Co-Director
Headquarters Office Canadian Cooperative Wildlife Health Centre
University of Saskatchewan Saskatoon, SK S7N 5B4 
1-800- 567-2033 (Canada only) or 306-966-5099 (toll)

Correspondence with Canadian authorities

Fri, 13 Feb 1998
Webmaster asks: Dr. Leighton at U. Saskatchewan in Saskatoon has been most helpful in chasing down wire story and internet stories of chronic wasting disease in Canada, which turns out to be early pre-emptive monitoring with negative results, sensitive methods being used via Dr. Williams' lab at U.Wyoming.

One basic worry that we have in the US is whether CWD is arising de novo on game farms due to use of rendered animal feed (non-British, unrecognized strain of bovine TSE?) or whether all the CWD is traceable back to the original Wyoming/Colorado provenance or whether other States or Provinces could have their own unrelated foci.

For the Toronto zoo mule deer and the Alberta elk with CWD, I am inclined to rule out zoo- and Alberta derived-feed given the incubation times and US background of these animals. The elk could only be tracked back to South Dakota, not Colorado/Wyoming unlike the mule deer as I understand it.

My questions:

What is fed to captive deer and elk at these game farms?
what Canadian laws and regulations apply amd are there compliance visits? 
how many of these game farms/animals are there?
what rules apply to trans-shipment  (especially back and forth across borders)?
what sort of records are kept on individual animals?
is there a necropsy program on 'downer' animals?
l Dr. Ted Leighton responds: Most of your questions are more appropriate for me to answer than for Dr. Clarke. Health Canada has a broadening program in zoonotic disease surveillance but much of it is currently under development and re-definition. Most of your questions are about CWD in wildlife and in game farmed animals.

Trace-back to origin for the one CWD-positive elk detected in Canada was not possible beyond the last farm of origin prior to importation. This is becaise of a lack of an animal identification and movement registration program for game farmed elk in the USA at the time. There has not been a case of CWD in mule deer on Canadian game farms. The case was at the Toronto zoo. It is thought that the disease originated from the Colorado-Wyoming focus through importation from a captive herd subsequently found to have the disease.

The surveillance programs of Alberta and Saskatchewan are making use of the expertise at the Veterinary Laboratory of the State of Wyoming. A battery of tests are run on each sample: histopathology of specific brain nuclei, immunohistochemistry for altered (scrapie) prion protein and western blot analysis. In Alberta, thus far, about 100 animals have been tested and all have been negative. No samples have yet been tested from Saskatchewan, but collection of samples for processing and shipment to Wyoming is underway.

No one but the game farmers knows what game farmed animals are fed. For information about Canadian laws regarding animals feeds, you should contact the Canadian Food Inspection Agency. A senior person in our region is Dr. Byrnne Rothwell. He could direct you to the most appropriate source of information.

Dr. Byrnne Rothwell writes that he, Dr. George Luterbach, Dr. Whiting, worked together with Dr. Broughton on the single case diagnosed in Saskatchewan, January of l996. Dr. Luterbach was the spokesperson, and will be best able to give you complete answers.

Rancher writes in about deer feeding realities

LifeSaveIn opinion Tue, 27 Jan 1998
"We, among my ranching friends here, have already shared reports of wasting disease seen and killing deer on our properties here [in Utah]. And as an old dairy rancher this should be no surprise.

Deer always scavenged my feeder bins when snow restricted their own foraging; so deer have been eating animal renderings in the "cattle" feed all along (including not only cow, sheep [scrapie] and all other animal "bypass", but also themselves in highway kills and deer and elk dressings from hunter processings commercially done) .

Also D.N.R. (Division of Natural Resources), State Fish and Game will also on occasions feed donated or purchased (animal fat/protein enriched) meal directly to snowbound, or feed and cold stressed deer and elk.

So reverse logic clearly dictates that if the deer and elk, which live free and longer than most cattle, are expressing and dying with spongiform encephalopathy while eating the shared renderings normally meant for cattle, and also associating with them; then as with all other animal pathogens, reason mandates that TSE "prions" are also shared; but since cattle raised for food do not live long enough for visible symptoms to appear, or if any health failure symptoms do begin to appear, the animal is quickly "processed" for product or rendering before gross disease expression renders the animal absolutely unsalable, so BSE or "cattle" wasting disease must never be noted or recorded.

And lets be economically real here, if it is ever suspected, no rancher or feeder would lay himself open to having his entire herd culled for BSE at a potential loss several hundreds to near a thousand dollars per unit animal, and neither would the livestock dependant industries, including pharmaceuticals, cosmetics, soaps and beyond, and the bureaucracies payed for and serving them all.

But any honest veterinary biologist knows this one is the clincher on TSE and BSE, and that other neurologically damaged biological animal in the picture - man - and his CJD!

Hunter writes in

Colorado correspondent  16 Feb 98
The 2/14/98 Denver Post had an article on CWD. The interesting thing is that a hunter who thought he was to have been notified within 3 weeks if his deer was positive for CWD was notified after 6 weeks, and the deer meat was ground up with lots of other deer meat and turned to sausage. I know that when a guy gets his animals, he tastes some of it right quick.

To eat or not to eat is the hunter's question

By Charlie Meyers  Denver Post Outdoor Editor Feb. 3, 1998
Whether Oprah Winfrey is guilty of defaming beef or, indeed, whether such a thing is actually possible, has provided the nation's media and comedians - not always the same - a gold mine of material.

But the debate about beef or, more to the point, Colorado's deer and elk, as a threat to human health is no joking matter. Whether it is called mad cow disease in Great Britain or wasting disease by Colorado biologists, the family of pathogens called transmissible spongiform encephalopathy poses a serious, and suddenly highly visible, concern. British researchers linked the bovine form of the disease with an extremely rare, yet fatal, disease in humans, touching off the current furor.

Colorado game managers have known of wasting disease for several decades and recently detected an unusual problem with deer in the area north and west of Fort Collins and along the South Platte River in the eastern plains.

Termed wasting disease for its degenerative traits, the malady belongs to the TSE class of diseases along with mad cow disease and scrapie, which infects sheep. Until officials in Great Britain began slaughtering more than a million beef cattle and before Oprah inserted her very high profile into the matter, nobody paid much attention.

This general apathy persisted despite the efforts of the Pure Food Campaign, a Washington, D.C.-based nonprofit public interest group dealing with food safety. Ron Cummins, the group's national director, flatly accuses the U.S. Department of Agriculture of a coverup to protect the nation's beef industry, particularly from a loss of exports to jittery overseas markets.

Cummins further suspects that TSE, in its various forms, may actually be the culprit in deaths typically diagnosed as Alzheimer's. He cites two studies showing that 5 and 13 percent of Alzheimer's deaths actually involved TSE, which literally eats holes in the brain. Autopsies are not routinely performed in deaths attributed to Alzheimer's.

Cummins also believes the Colorado Division of Wildlife also hasn't taken the matter seriously enough.

"At the height of the mad cow epidemic in Britain, only 1-2 percent of the cattle were infected. Compare that to 6 percent of the mule deer in the target area in Colorado and this is very, very serious,'' Cummins said. "I think it is prudent to say not to eat the animals until we know what's going on.''
Actually, the figure for deer tested in 1997 was 4 percent, down two points from the previous year. Only 1 percent of elk tested positive for wasting disease in 1996 and only one animal tested positive in 1997. This caused Division of Wildlife officials to totally remove elk from the requirement that hunters submit the heads of animals killed in 20 separate game management units, a requisite that still holds for deer. For the 1998 season, deer hunters in units 7, 8, 9, 18, 19, 20, 28, 29, 37, 371, 87, 90, 91, 92, 93, 94, 95, 96, 191 and 951 must submit heads to the division within five days of the kill. Details on where to deposit the deer heads will be listed in the regulations brochure available in March.

DOW further warns hunters against eating any animal that appears sick, to wear rubber gloves when field dressing game, minimize handling brain or spinal tissues and avoid eating brains or nerve tissue.

Cummins believe these measures don't go far enough. This conflicts directly with the belief of John Pape, an epidemiologist with the Colorado Department of Health. "There is no indication that chronic wasting disease is a threat to human health,'' declared Pape, who did concur with the DOW precautions. DOW officials point out that neither scrapie, which has been a study subject for two centuries, or wasting disease, have been linked to human illness. Although officials are certain animals west of the Continental Divide remain uninfected, they'll continue to conduct tests across the entire state.

Chronic wasting disease: deer-to-cattle link worry

Jan. 24 /98 AP MARTHA BELLISLE
ESTES PARK, Colo. -- A story about chronic wasting disease affecting wildlife in the Rocky Mountains on the border of Colorado and Wyoming, one of only two spots in the world, the story says, where the disease has appeared.

The U.S. National Institutes of Health is investigating because mad cow disease, similar to the chronic wasting disease that has struck mule deer and elk, has been linked to a brain-wasting malady in humans.

Byron Caughey, biochemist at the NIH's Rocky Mountain Laboratories in Hamilton, Mont., was quoted as saying, "We know there's a link between the diseases, not a causal link necessarily, but they're the same kind of disease. Of great interest is whether we have to worry about it being transmitted into other animal hosts, whether cattle or humans."

The story cites Todd Malmsbury, spokesman for the Colorado Division of Wildlife in Denver, as saying that about 6 per cent of mule deer in the area of northern Colorado and southern Wyoming suffer from chronic wasting disease, or CWD, and there are an estimated 550,000 mule deer, a brown, white-tailed species with big ears in all of Colorado.

The story notes that wildlife officials require hunters to turn in the heads of deer or elk they've killed. If the brains test positive, the hunters are advised to dump the meat. Malmsbury was cited as saying that 40 deer turned in during the past hunting season tested positive.

Mike Miller, a state wildlife veterinarian was cited as saying the NIH along with federal and state agencies have launched three studies to determine its transmissibility to cattle.

In Iowa, researchers are injecting brain material of infected wildlife into the brains of cattle. At the University of Wyoming, infected brain material is being given to cattle orally.

In addition, researchers are investigating whether the disease can be spread through contact, letting cattle live with deer herds that have contained infected animals.

Mad Cow-Like Disease Found in Deer

 AP Online  Sat, Jan 24, 1998  By MARTHA BELLISLE
ESTES PARK, Colo. (AP) -- The emaciated mule deer stares blankly into space. Then, stumbling in small circles, it falls over dead, another victim of chronic wasting disease. It is a grim sight for wildlife officials working in the Rocky Mountains on the border of Colorado and Wyoming, one of only two spots in the world where the disease has appeared.

For health officials, a frightening question must be answered: Will this terrible illness cross over to the human population? The National Institutes of Health is investigating because mad cow disease, similar to the chronic wasting disease that has struck mule deer and elk, has been linked to a brain-wasting malady in humans -- Creutzfeldt-Jakob disease -- that has killed 20 people in Europe.

"We know there's a link between the diseases, not a causal link necessarily, but they're the same kind of disease," said Byron Caughey, biochemist at the NIH's Rocky Mountain Laboratories in Hamilton, Mont. "Of great interest is whether we have to worry about it being transmitted into other animal hosts, whether cattle or humans," he said.
...

No one has become sick from carrying the wasting disease -- but wildlife officials require hunters to turn in the heads of deer or elk they've killed. If the brains test positive, the hunters are advised to dump the meat. Forty deer turned in during the past hunting season tested positive, Malmsbury said.

While the NIH [NIAID] is interested in whether the wasting disease can jump to humans, federal and state agencies have launched three studies to determine its transmissibility to cattle, said Mike Miller, a state wildlife veterinarian.

In Iowa, researchers are injecting brain material of infected wildlife into the brains of cattle. At the University of Wyoming, infected brain material is being given to cattle orally. [The first experiment gave transmission, the second has not -- webmaster].

In addition, researchers are investigating whether the disease can be spread through contact, letting cattle live with deer herds that have contained infected animals. Many scientists believe cattle in Britain became infected with mad cow disease by eating parts of sheep with a similar ailment, a practice outlawed in the United States, he said. Mad cow disease was first diagnosed in 1986. No case of mad cow disease has been reported in the United States.

Spongiform encephalopathy in free-ranging mule deer , white-tailed deer and Rocky Mountain elk in northcentral Colorado.

J Wildl Dis 1997 Jan;33(1):1-6
Spraker TR, Miller MW, Williams ES, Getzy DM, Adrian WJ, Schoonveld GG,
Spowart RA, O'Rourke KI, Miller JM, Merz PA
Between March 1981 and June 1995, a neurological disease characterized histologically by spongiform encephalopathy was diagnosed in 49 free-ranging cervids from northcentral Colorado (USA). Mule deer (Odocoileus hemionus) were the primary species affected and accounted for 41 (84%) of the 49 cases, but six Rocky Mountain elk (Cervus elaphus nelsoni) and two white-tailed deer (Odocoileus virginianus) were also affected.

Clinical signs included emaciation, excessive salivation, behavioral changes, ataxia, and weakness. Emaciation with total loss of subcutaneous and abdominal adipose tissue and serous atrophy of remaining fat depots were the only consistent gross findings. Spongiform encephalopathy characterized by microcavitation of gray matter, intraneuronal vacuolation and neuronal degeneration was observed microscopically in all cases. Scrapie-associated prion protein or an antigenically indistinguishable protein was demonstrated in brains from 26 affected animals, 10 using an immunohistochemical staining procedure, nine using electron microscopy, and seven using Western blot.

Clinical signs, gross and microscopic lesions and ancillary test findings in affected deer and elk were indistinguishable from those reported in chronic wasting disease of captive cervids. Prevalence estimates, transmissibility, host range, distribution, origins, and management implications of spongiform encephalopathy in free-ranging deer and elk remain undetermined.

NOVA TV show: 'Brain Eater'

12 Feb 98 webmaster
A transcript of the show is available online or the tape may be ordered for $20.00 from NOVA at 1-800-255-9424. NOVA has an excellent page on mad cow show.

Review: the show was quite good

The Nova show featured David Bolton re-enacting the discovering of Griffiths paper, a rare image of Griffiths himself, Ironside re-enacting the chain-mail glove and diving bell autopsy, Merz in macabre eyeshadow re-enacting the discovery of SAF, Suzette Priola wielding a noisy pipette, a less-chipper-than-in-real-life Linda Detweiler herding cows, Marsh and sadly affected mink, Zeidler re-living home visits to nvCJD families, Collinge fretting over the 18 million at-risk met-met people in the UK, the famous vignette of Oprah and her worried audience, a still of Prusiner, Brown and the TSE buried for 3 years in garden soil, and so on. The part I caught was protein-only though viral controversy was mentioned.

Paul Brown, frequently represented by activists here as a wimp on BSE, gave a solid presentation, saying bovine TSE in the US was likely low but not zero but not high either and that the US would have to rethink its position if nvCJD really takes off in the UK. Brown also said there was no absolutely evidence for scrapie-to-humans. Unfortunately, absence of evidence is not evidence of absence -- the studies to date are aenemic.

The show, while discussing the US feed ban, pointed out the loopholes still in it, for example cow blood can still be fed back to cows. Muscle and blood were said to be ok but this was qualified as not definitive. Much of the show seemed a year or so old as there were just 10 victims.

Somewhere they got some good outside advice on this. NOVA got a lot of stuff more or less right; the show would be satisfactory for a classroom. It was balanced without pulling punches. The spooky music and the steven king voice-over put an ominous spin on just about everything, from the sheep dog to the young people eating burgers.

The one bonehead thing I saw was not the producer's fault: someone gave them a molecular model of cross-beta fibril that was straight, not coiled. I had to go over to the Architecture School to get the software to do this right, it amounts to nested spiral staircases. I posted something in line with the diffraction data at a while back. The conversion animation of normal by rogue was well-handled though the beta sheet hydrogen-bonding was a stretch.

Narang reporting older cases of nvCJD

Wed, 11 Feb 1998 Listserve
Dr Harash Narang gave a lecture to the 95th meeting of the British Neuropathological Society in London, 7/8/9th January 1998. This is the published abstract:
Over 8 years, cinical histories, brains and urine samples of 33 CJD cases aged between 18 and 84, were examined. Nineteen of these were between 18 and 62 years with leading clinical features such as difficulties in balancing, ataxia with awkward placement of the feet and a swaying gait similar to that seen in BSE cattle. Histopathological examination of brains in these cases revealed spongiform changes in the caudate nuclei and the thalamus with extensive involvement of the cerebellum. Four of these 19 cases were classified by Surveillance Unit as "new variant". Histopathological changes and distribution of PrP plaques in the remaining 15 cases were identical in all respects with those classified as "new variant", the only difference being age: all 15 were over the age of 47. There is now agreement that at least those under 41 were exposed to the BSE strain of the agent. The results of these findings, however, suggest that all age groups can be infected with the BSE strain of the agent."
A representative from the Edinburgh Surveillence Unit present, asked to comment admitted they also had found an older victim of NV CJD. The clinical details of the cases presented at the British Neuropathological meeting are described in detail in the book "Death on the Menu" IBSN 0-9530764-1-5 are available from most bookshops.

As Dr Narang examined samples of brain from only 33 victims of CJD cases from England, however during this period at least 400 to 500 people have died and confirmed suffering from CJD.

The Edinburgh Surveillence Unit refuses to count victims they have not carried out the PM Test themselves.

WHO: large human epidemic possible

 Reuters North America Wed, Feb 11, 1998
GENEVA - Medical experts meeting at the World Health Organization concluded Wednesday that the risk of an epidemic of "mad cow" disease in humans over the next 10-15 years could not be dismissed. But one U.S. specialist who attended the gathering told a news conference that the chances of a major outbreak of the disease -- which has so far affected 23 people in Britain -- were remote, adding: "I don't think it will happen."

The three-day meeting at the United Nations agency was called to look at developments in the search for possible treatments of humans infected by "mad cow," who suffer a fatal brain-wasting ailment that doctors consider a new version of the previously known Creutzfeldt-Jakob disease or CJD. A statement read to the news conference by WHO neurologist Martin Zeidler said the gathering "noted that the possibility of a significant epidemic of new- variant CJD occurring within the next 10-15 years cannot be dismissed...." For this reason, the experts agreed, "the early identification of an effective therapy is of paramount importance," the statement added.

Researchers believe that new variant CJD began because humans who ate meat from animals infected with a similar new cattle disease, bovine spongiform encephalopathy or BSE. The identification of several cases in Britain led to a European Union ban, largely still in force, on exports of British beef and cattle by-products, and recently to a British government ban on sale of beef-on-the-bone. This week's Geneva meeting was part of a program of world-wide surveillance of the two diseases launched by the WHO two years ago.

So far no cure for CJD has been found, although several therapies have been tried, according to the WHO.

Dr. Paul Brown of the National Institute of Neurological Disorders in Bethesda, Maryland, who has been heavily involved in research on CJD, told the news conference the number of cases identified in Britain indicated there would be no great surge. "I personally would have thought that in the UK (Britain) by this time if new variant was going to be a huge problem, we already would have had a clue with an explosion of new cases. "That has not happened, but the statisticians tell us we still have to wait a year or two to be sure. We may be dealing with a non-problem," Brown said.

Beef-on-bone law takes a first bite

February 13 1998 Times BY MICHAEL HORNSBY AGRICULTURE
A SCOTTISH hotelier has become the first person in Britain to be prosecuted for breaching the Government's beef-on-the-bone ban imposed two months ago because of a remote risk of infection with "mad cow" disease.

Jim Sutherland, owner of The Lodge, Carfraemill, 20 miles south of Edinburgh, said yesterday he had been summoned to appear before Selkirk Sheriff Court on March 10. He faces a possible fine of 5,000 or six months in prison, or both. The case follows a dinner which Mr Sutherland held at his hotel on December 22 in deliberate defiance of the ban. Some 170 people, mostly local farmers, sat down to a free meal of prime Aberdeen Angus on the bone and donated 1,700 to the Marie Curie Cancer Care charity.

The dinner was interrupted by two health inspectors from the Scottish Borders Council. "Even though I paid for the meal myself ... it appears that the Food Safety Act, under which the ban is enforced, defines 'sale' very widely to include 'supply'," Mr Sutherland said.

The case will be closely watched in England and Wales where there has been a marked reluctance to enforce the ban. Last night Jeff Rooker, a junior Agriculture Minister, said farmers could eat T-bone steak and other similar cuts if it came from cattle slaughtered on their own land.

Dealler on CWD risks

Jan 98 Dealler Web site
Survey for chronic wasting disease in Rocky Mnt mule deer shows 6 per cent positive.Apparently wildlife officials require that people shooting mule deer must deliver the head of the animal to them for diagnosis and this is how they know the percentage with the disease. The research is being done at the NIH at Hamilton, Montana.

If the test proves positive, the hunter is advised to dump the rest of the carcass. Already they have started further research: inoculating the CWDD into cattle, and feeding it to them, and just letting the infected deer live in contact with cattle to see if the disease is transferred.

This percentage is simply so high that a similar type of epidemic might be underway as has taken place with BSE.

Up until recently CWDD in the wild was hardly reported at all and now may be prevalent in both mule deer and elk from Colorado and Wyoming. As many people are aware, the carcasses of deer hit by cars are taken for rendering, and various foods are left in the winter by local people for the animals. The worry is simply that a disease may be present and has become recycled in the same way as in BSE. It is also not clear how the result for the brain test will be given to the hunter quick enough for the carcass to either dumped or kept; it would need to be kept frozen at an abattoir and it is not clear that many of the hunters do this.

Scrapie in New Mexican bighorns -- make that scabies

15 Feb 98 New Mexican correspondent
"This was in the newspapers. They were capturing the sheep, housing them at the zoo, and when they could get "clear" animals, these were set free in a new environment. Bighorns are an endangered species.

I spoke with the vet at the Rio Grand Zoo. He says it was scabies, not scrapie, in these animals."

This would account for the confusion and persistent rumors. To see skin scrapings of scabies in a severe infestation with numerous eggs and mites present in the comified layer, click here. Scabies is caused by a microscopic insect, or mite (known as Sarcoptes scabiei). The mite tunnels or burrows into the skin, usually just below the surface, and then keeps burrowing through the subsurface leaving behind eggs, feces, and dead body parts. Although there may not be any skin reaction at first, once you become sensitized you will have an allergic reaction to the mites and their leavings which consists mainly of a rash with an intense itch. The burrows are visible on the skin surface as looped-thread-like lines, but often they are scratched away. The burrows are usually found in warm, moist parts of the body. [Adapted] Cattle and sheep get scabies and are dipped in organophosphates to get rid of them. Scabies is a reportable disease in some states and p[remises may be quarantined. It is similar to mange.

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