Document Directory

14 Jun 01 - CJD - Scientists make breakthrough in CJD detection
14 Jun 01 - CJD - Mad Cow fears may spur sale of non-bovine collagen
14 Jun 01 - CJD - Country faces first Mad Cow
14 Jun 01 - CJD - Europe 'has no BSE epidemic'
14 Jun 01 - CJD - BSE case is confirmed; exports cease
14 Jun 01 - CJD - Ministers blamed for food crises
14 Jun 01 - CJD - Major Breakthrough
14 Jun 01 - CJD - Serono devises method for early detection of BSE
14 Jun 01 - CJD - Live exports spreading BSE - expert
14 Jun 01 - CJD - Blood Test
14 Jun 01 - CJD - Looking for a faster way to detect Mad Cow disease
14 Jun 01 - CJD - BSE: Is It A Threat?
14 Jun 01 - CJD - Rogue protein 'could help cancer spread'
14 Jun 01 - CJD - Experts fear Mad Cow pandemic
10 Jun 01 - CJD - Mad Cow case in Hong Kong
10 Jun 01 - CJD - Suspected victim of `Mad Cow' disease in stable condition
10 Jun 01 - CJD - Quickening the Diagnosis of Mad Cow Disease
09 Jun 01 - CJD - Hong Kong patient has human form of Mad Cow disease, officials say
09 Jun 01 - CJD - HK Mad Cow disease scare
09 Jun 01 - CJD - HK woman may be Asia's first case of human Mad Cow disease
09 Jun 01 - CJD - BSE Results Nervously Awaited

14 Jun 01 - CJD - Scientists make breakthrough in CJD detection


PA News--Thursday 14 June 2001

A new discovery could make it far easier to detect the human form of Mad Cow disease.

At present, variant CJD can only be diagnosed by examining brain tissue either after death or from a surgically-removed sample.

The breakthrough by Swiss researchers could make it possible to recognise the disease from a blood sample.

It may also open up new avenues for identifying similar diseases in animals, such as BSE in cattle and scrapie in sheep.

The family of transmissible spongiform encephalopathy diseases which includes vCJD and BSE are thought to be caused by abnormal "rogue" prion proteins.

A prion that has changed shape to make it dangerous causes other prions it comes in contact with to "turn bad" in a chain reaction.

The discovery from Claudio Soto and colleagues at the Serono Pharmaceutical Research Institute in Geneva shows that even a minute amount of rogue prion protein can convert large amounts of normal prion.

Silvano Fumero, a senior executive at Serano, said: "The procedure mimics the replication of abnormal prion proteins in the body in 'fast forward' mode, compressing years of real-life time into a few hours in the laboratory.

"This is a major scientific breakthrough and has potential applications in improving tests for prion diseases, as well as identifying targets against which future drugs should be aimed."

The breakthrough could lead to the identification of abnormal prion proteins in human vCJD victims by testing spinal fluid or blood, say the researchers.

14 Jun 01 - CJD - Mad Cow fears may spur sale of non-bovine collagen


CNET---Thursday 14 June 2001

NEW YORK, June 13 (Reuters) - FibroGen Inc., a company that is developing synthetic gelatin and collagen, a tissue-binding agent used in skin grafts, thinks fears of Mad Cow disease will help sales boom as the public seeks non-bovine made products.

San Francisco-based FibroGen expects its genetically engineered products will be widely adopted as companies and hospitals seek to shield themselves from the perception that gelatin and collagen -- now derived from cows -- are not safe.

The small company is working to develop treatments for tissue disorders such as fibrosis and excessive scarring using collagen and gelatin, which is a collagen derivative that is used in pharmaceuticals, food and cosmetics. Collagen and gelatin are typically derived from cow tendons, bones and hides -- potential transmitters, some argue, of Bovine Spongiform Encephalopathy, also known as Mad Cow disease, or BSE.

"My guess is that within six or seven years we will have 20 to 30 percent of the overall collagen-gelatin market," said Jack Anthony, FibroGen's vice president of global business development.

If he's right, privately held FibroGen stands to make a good deal of money. The global market for unprocessed gelatin is worth as much as $4 billion, and the market for collagen is worth about $1 billion, according to company estimates.

FibroGen hopes to get a jump on the competition by using the BSE scare to raise its profile. FibroGen began developing its products in the early 1990s, before Mad Cow became an issue. However, Anthony said BSE is "the icing on the cake."

The company's Web site devotes several pages to BSE and raises the specter of drug shortages should regulatory authorities go so far as to ban bovine-derived products.

"There is a real problem with the TSE's (transmissible spongiform encephalopathies), the transmission risks from various commonly used products are unclear and there are few alternatives," the company says on its Web site. "Clearly, we have not heard the end of this story."


But FibroGen may be overestimating the extent to which customers are or will be concerned about Mad Cow. Collagenisis, a privately held non-bovine collagen producer in Beverly, Massachusetts, said BSE isn't an issue. The company makes human-based collagen from the skin of cadavers. Its collagen is valuable to plastic surgery patients who are allergic to bovine-based products and to the urology profession, which uses the company's collagen strips to support the bladders of patients suffering from incontinence.

Dale DeVore, chief scientific officer and president of research and development at Collagenisis, said none of the company's customers have ever raised concerns about BSE.

"That may be different in the future, especially if there were to be an occurrence of the disease in the United States or Canada," he said. "The situation is different in Europe, where we or another company could make big inroads into the bovine market."

In Europe, more than 100 cases of Creutzfeldt-Jakob disease, the human form of BSE, have been linked to the consumption of infected beef. But how the disease is transmitted is unknown, and there is no evidence it is communicated through products containing collagen or gelatin.


U.S. regulatory bodies are nonetheless urging manufacturers to be cautious. British beef has been banned and the U.S. Food and Drug Administration has warned vaccine producers and others to ensure their raw materials come from disease-free cows.

That's helped ferment interest in FibroGen's synthetic collagen and gelatin. The company is working with 60 pharmaceuticals and biotechnology companies to test the products, Anthony said.

"We have had a very strong demand from pharmaceutical and medical device companies based in Europe and now we are now seeing U.S. companies also coming forward and beginning to evaluate the materials," he said.

But drug companies are reluctant to enthuse publicly about FibroGen's products, in part because they fear raising doubts about the safety of their existing products.

"To some extent, as an industry, we have to affirm the science, which says the risk of contracting BSE through collagen or gelatin is infinitesimal," said Dr. Gillian Woollett, associate vice president for biologics and biotechnology at the Pharmaceutical Research and Manufacturers of America, an industry group. "However, if someone could come up with a cost-effective alternative to bovine-based products, of course the pharmaceuticals industry would be interested."

FibroGen reckons it's done just that. Anthony attributes the company's scientific advances to technology it licensed from Oulu University in Finland. The technology uses yeast and transgenic plants to duplicate the way the body produces its 21 different collagens.

FibroGen expects its products to be commercially launched within two years. As it moves towards commercialization the company will consider offering its shares to the public for the first time.

FibroGen is not without competition. Santa Barbara, California-based Inamed Corp., which has 90 percent of the market for bovine-based collagen, hopes to introduce a human collagen grown from the foreskins of infant boys by the end of this year.

Inamed's use of human collagen has less to do with customer fears of Mad Cow disease than of their irritation at having to take a skin allergy test with bovine collagen.

"Studies have shown that a relatively large percentage of people who seek collagen treatment, and are told they must take a skin test to see if they have an allergy to bovine, never return," said Inamed's chief financial officer, Michael Doty.

14 Jun 01 - CJD - Country faces first Mad Cow

By Kate Swoger

Prague Post--Thursday 14 June 2001

Officials fear alarm after discovery of domestic BSE case

Despite appeals for calm in the wake of the Czech Republic's first case of Mad Cow disease, the country is bracing itself for serious fallout, as neighboring nations impose bans on Czech beef.

Prime Minister Milos Zeman urged the public to avoid panic and continue eating beef, even as authorities prepared to slaughter dozens of cattle at the south Moravian farm where the animal was found.

All cattle a year older or younger than the infected cow will be tested for bovine spongiform encepholopathy (BSE) to determine the extent of the contamination. The cow belongs to a herd in Dusejov, 120 kilometers (75 miles) southeast of Prague.

"We believe that the likelihood [of other cases] is as small as it has been up until now," said Josef Duben, a spokesman for the State Veterinary Institute.

Tests conducted in Germany on June 8 confirmed BSE in a 5-year-old dairy cow from a herd of 371 in Dusejov. The discovery is expected to hurt the country's already flagging beef industry.

"There's going to be a panic. I know Czech customers," Radim Patek, marketing director at retail chain Julius Meinl, told Reuters.

Slovakia, Austria, Poland and Lithuania imposed bans on the import of Czech beef immediately after the case was confirmed. Hungary also imposed import restrictions.

Officials believe the Dusejov dairy cow was infected through contaminated feed, either a milk substitute given to calves or a solid mixture for mature animals.

Experts believe the feeding of animal products to cattle, natural herbivores, causes the brain-wasting disease. First discovered in British cattle in 1984, it has been linked to its human equivalent, a variant of Creutzfeldt-Jakob disease, which has killed 92 people.

The Dusejov case, which is also the first outside Western Europe, followed an April report by the European Community (EC), the European Union's executive branch, cautioning that BSE was probably present in Czech herds.

The report produced loud complaints from Prague. The government protested that while it had imported significant amounts of live cattle and meat and bone meal from countries where BSE had occurred, the use of animal-meal feed had been banned a decade ago, in then-Czechoslovakia.

But the EC was unwilling to rule out the risk of cross-contamination of cattle feed with meat and bone meal.

The European Union will continue permitting Czech beef imports, largely because strict controls were adopted as a result of the EC report.

-- Martina Sedlakova contributed to this report.

Kate Swoger may be reached at

14 Jun 01 - CJD - Europe 'has no BSE epidemic'

Consumer Affairs Correspondent Nicola Carslaw

BBC--Thursday 14 June 2001

The latest figures on BSE are promising, say experts

The latest results of BSE tests carried out on healthy cattle across the EU suggest there is no massive, hidden BSE epidemic in Europe.

The Food Standards Agency (FSA) figures show that in the first four months of this year only a tiny fraction of animals tested positive for the disease.

Tests for BSE in cattle destined for the food chain have been underway throughout the European Union since the beginning of the year.

Out of a total 1.7 million healthy animals tested, the figures show that by the end of April the number of positive cases reported were:

Spain 22 (from 73,859 tested)

France 21 (from 623,349)

Germany 13 (from 613,550)

Belgium (10 from 97,311)

Italy 7 (from 64,351)

Holland 2 (from 97,794)

Irish Republic 1 (from 71,699)

The figures, showing a total of 76 cases across the EU, are encouraging says the FSA.

'Low level' of cases

Agency chairman Sir John Krebs said: "BSE remains the food issue of most concern to consumers and they want to know what is being done to protect them.

"People have been worried about the spread of BSE in Europe but the EU programme of testing animals destined for the food chain shows a very low level of BSE cases. The figures indicate there is no hidden, massive epidemic of BSE in Europe."

However, Sir John warned against complacency.

"The Food Standards Agency continues with 100% checks on imported beef. We will not tolerate any breaches of BSE controls and we will examine any new evidence rigorously," he said.

Sir John said a new leaflet was being launched on Wednesday, together with the Consumers' Association.

New information leaflet

Titled 'BSE and Beef' it describes the BSE controls in place and also explains the various labels that can be found on beef on sale in the shops.

The Consumers' Association hopes the leaflets will be distributed to every butcher's shop and supermarket in the UK.

Its spokeswoman, Mona Patel, said they had been designed to address shoppers' concerns about the nature of the risk of BSE and provide sources of further information.

"It's important people have the chance to understand the issue so they can make choices about safety and the relative levels of risk from eating beef that's produced within the UK or elsewhere," she said.

The figures unveiled on Wednesday, coupled with the leaflet, may offer reassurance to those worried about the risk of beef and BSE.

Whether they will persuade any of those who have stopped eating beef to put it back on their shopping lists remains to be seen.

14 Jun 01 - CJD - BSE case is confirmed; exports cease

Vladimir Kuchar and wire reports

Prague Business Journal---Thursday 14 June 2001

State Veterinary Administration to run tests on all slaughtered animals older than 30 months

Czech cattle breeders, meat processors and veterinarians' worst nightmare came true last week when the first case of Mad Cow disease (BSE) was found in the Czech Republic.

Agriculture Minister Jan Fencl announced Friday that original suspicions had been confirmed by further tests. However, the Czechs have asked a German laboratory to double check their results with that verdict due later this week.

Hungary and Slovakia did not wait for further checks - Bratislava immediately announced a ban on Czech beef imports and transport of cattle across its territory from Sunday, and Budapest said no import licenses would be given for Czech beef from Monday.

Confirmation means the Czech Republic becomes the first country outside the EU (except for Switzerland) with an official presence of the disease.

Veterinarians detected the case in a Jihlava-based meat producer Kostelecke Uzeniny in South Moravia. The milk cow with BSE (Bovine Spongiform Encephalopathy) was bred on the farming cooperative at Dusejov, 120 km southeast of Prague. "We have no idea how the dangerous disease could have gotten into our farm, we have never fed our cattle with meat-and-bone meal, we have never bought cattle from abroad," the cooperative's head Zdenek Bartosik said.

Germany has been asked to verify the Czech tests because it has much more experience of BSE. The suspicious sample comes from a cow age 5 years and eight months. It is from a herd of 400 cattle, including 371 milk cows and 18 pregnant cows.

For the first time, the State Veterinary Administration (SVS) took the measure of banning the transport of animals from the farm and is preparing for further measures to be taken. Confirmation of BSE means the Jihlava herd, or at least those animals that were in contact with the affected animal during the period when it would have been contagious, will be slaughtered.

The SVS will now start checking all slaughtered animals older than 30 months, or a total of 170,000 animals. The Czech Republic has so far examined 10,656 samples of cattle brains with a negative result. "The checking of all slaughtered animals older than 30 months would cost Kc 280 million annually," said SVS spokesman Josef Duben.

The farm in Dusejov has around 1,500 cattle, of which 600 are milk cows. It supplies slaughter cattle mainly to Kostelecke Uzeniny and around 12-20 animals monthly to smaller, neighboring slaughterhouses.

Duben said there were two hypotheses of how the animal could have caught the disease. The most likely possibility is that the animal was infected by a milk substitute. Imported meat-and-bone meal, which could have "by accident" been mixed with the cattle feed in the feed mixing plant, is the less likely possibility, he said. The feeding of meat-and-bone meal to cattle has been banned here since 1991.

"It is a catastrophe," said Pavel Vacek from Veverska Bytiska, South Moravia. "We do not know what we can expect - only that our business will collapse because the sale of cattle will be held up or drop to a minimum."

14 Jun 01 - CJD - Ministers blamed for food crises

Staff Reporter

BBC--Thursday 14 June 2001

The lessons of BSE "have not yet been learnt"

One of the UK's leading food safety experts has blamed the government for the scale of such diseases as BSE, foot-and-mouth and e-coli.

Professor Hugh Pennington, of Aberdeen University, says the lessons of the outbreaks have still not been learned.

And he said that without "root and branch reform", another disaster was waiting to happen.

Speaking at BBC Radio 4's Food Programme awards on Wednesday, Professor Pennington criticised the use of scientific advice by policymakers.

He said the same criticisms had been made in Lord Phillips' report, published last October, into the BSE crisis.

"Have we learned the lessons of this and applied them so that another BSE-like catastrophe couldn't happen again? Not yet," he said.

He praised the setting-up of the Food Standards Agency, and the way it has been practising openness "with deliberate zeal".

But he criticised secrecy and infighting amongst government departments and civil servants.

Professor Hugh Pennington said his biggest worry was that government ministers were simply not capable of understanding the scientific advice they were given.

"It seems to be almost the rule that the scientific education of those destined to be permanent secretaries or government ministers must finish at puberty," he said.

"We haven't got the relationship between science and government right yet."

He said the scientific community had plenty of foreknowledge about the particular strain of foot-and-mouth which hit the UK in February.

'Lack of understanding'

"So we should have been prepared and able to raise our defences... without having to hire outside scientists in a hurry well after the outbreak had started," he said.

He said the Phillips inquiry into BSE had also identified a lack of understanding by government departments of what was going on on the ground.

Officials responsible for policy-making did not know what was happening in feed mills or in abattoirs, he said.

And that scenario had been repeated with the foot-and-mouth outbreak.

"This has happened again with Maff admitting that it was completely taken by surprise at the level and degree of sheep movement that was going on," he said.

"But it was its business to know".

He said the government must urgently address these problems, before more Food Onlineborne bugs arrive.

"Without root and branch reform in the way policymakers get and use scientific advice, there will be big trouble ahead," he said.

"At the end of the day nothing less than root-and-branch reform of the civil service will do.

"A good start would be some top jobs going to people who know more about science."

14 Jun 01 - CJD - Major Breakthrough


YAHOO--Thursday 14 June 2001

Major Breakthrough To Improve Detectability Of Mad Cow And Other Prion Diseases.

Claudio Soto have developed a new procedure that should greatly improve the sensitivity of current tests used to detect abnormal prion proteins.

These are believed to cause fatal neurodegenerative diseases such as Bovine Spongiform Encephalopathy (BSE) in cattle, scrapie in sheep and Creutzfeldt-Jakob disease (CJD and nvCJD) in humans.

A paper describing the research is published in the June 14 edition of Nature(1).

"The procedure mimics the replication of abnormal prion proteins in the body in 'fast forward' mode, compressing years of real-life time into a few hours in the laboratory", said Silvano Fumero, Senior Executive Vice President, Research & Pharmaceutical Development.

"This is a major scientific breakthrough and has potential applications in improving tests for prion diseases, as well as identifying targets against which future drugs could be aimed." Current tests are not used to detect BSE in dead animals under 30 months of age or those recently infected, and there are no tests available for live animals or humans.

The new procedure developed by the Serono researchers makes it possible to amplify, or multiply, abnormal prion proteins up to several hundred times within a day and therefore this enables a much more sensitive test for BSE and other prion diseases.

Protein Misfolding Cyclic Amplification The procedure is termed Protein Misfolding Cyclic Amplification (PMCA).

Minute samples of abnormal prion protein (PrPsc) were taken from the brains of scrapie infected hamsters, and mixed with large excess amounts of normal prion protein (PrPc) from disease-free hamsters.

This resulted in a rapid conversion of the normal prion protein into many aggregates of the abnormal prion protein.

The aggregates were then treated with ultrasound.

This cycle of amplification can be repeated many times within a day in a laboratory to produce quantities of the abnormal prion protein which are several hundred times greater than is currently available in the brain tissue of dead animals or humans.

Major scientific breakthroughs This publication involves a number of major scientific breakthroughs:

The work done by the Serono scientists marks the first time that the abnormal prion protein has been cultivated in vitro, with a greater efficiency than the conversion process that has been postulated to occur in vivo with prion diseases such as BSE and CJD.

As a result of this research, there is now a sensitive in-vitro model to help understand the underlying biology of prions, to identify other factors that may be responsible for the abnormal prion protein conversion, and to identify potential novel drug targets for prion diseases.

Further research Through their continued work in this field, Dr Soto's team are targeting further significant scientific advances:

Ongoing experiments suggest in vivo that the abnormal prion protein is infectious.

If this data is confirmed, it would strongly support the hypothesis for which Stanley Prusiner won the Nobel Prize in 1997.

The researchers are currently conducting confirmatory experiments that will be published in due course.

This procedure also suggests that the abnormal prion protein can be detected in the blood of animals.

Confirmatory experiments are on-going.

Significant potential applications This work has a number of significant potential applications in the diagnosis of protein misfolding diseases:

- The identification of abnormal prion proteins in the brains of dead cattle under 30 months of age or those recently infected with BSE, which currently are not tested due to the minute amounts of PrPsc at the earliest stage of the disease.

- The identification of abnormal prion protein in live cattle (symptomatic or pre-symptomatic with BSE), using tissues or biological fluids such as blood.

- The detection of CJD and nvCJD in humans (symptomatic or pre-symptomatic), using spinal fluid or blood.

This procedure may be applicable to the detection of other protein-misfolding diseases such as Alzheimer's.

Commercial implications for Serono

There are many stages involved in bringing this major scientific breakthrough into commercial applications.

Serono will explore the opportunities of licensing agreements for this procedure with leaders in the field of diagnostics.

These would cover both animal and human tests, using brain tissue, other tissue types and biological fluids such as blood.

Serono has already moved to patent the proprietary knowledge and processes resulting from Dr Soto's research.


The disease mechanism of prion proteins so-called "normal" prion proteins (PrPc), those with a normal molecular shape, are naturally occurring and are attached to the surface of many cell types in the body.

Their function is still unknown.

Prions become pathological when their shape changes.

Once they have taken on an abnormal shape, these "abnormal" prion proteins (PrPsc) have the ability to replicate themselves.

They convert normal prion proteins into abnormal prion proteins, which in turn convert other normal prion proteins, causing a slow but relentless chain reaction.

Over time, aggregates of these abnormal prion proteins form plaques in the brain, causing degeneration of tissue and neuronal death, which leads to the disease symptoms.

Current diagnostic tests for BSE At present, there are a number of companies making diagnostic tests for BSE, including Prionics (Switzerland), Bio-Rad (US, UK), Enfer Scientific (Ireland).

All of the current diagnostic tests are performed post mortem on brain tissue from infected cattle.

There are currently no tests available for live animals.

Current tests, which are performed on the brain tissue of dead animals, are not used to detect BSE in animals younger than 30 months of age or those recently infected, due to the low number of abnormal prion proteins in the early stage of the disease.

Efforts thus far have focused on improving the speed and sensitivity of the tests, but the limiting factor remains the inability to detect small amounts of the abnormal prion proteins in brain tissue samples.

Current diagnostic tests for nvCJD Clinical symptoms can be observed at a late stage in the disease.

But currently, the only definite confirmation of nvCJD is post mortem analysis of brain tissue.

A number of companies have made statements of strategic intent on developing blood screening tests for nvCJD patients, including Bio-Rad, Bayer, Roche and Ortho Johnson & Johnson.

Some of the statements in this press release are forward looking.

Such statements are inherently subject to known and unknown risks, uncertainties and other factors that may cause actual results, performance or achievements of Serono S.A.

and affiliates to be materially different from those expected or anticipated in the forward-looking statements.

Forward-looking statements are based on Serono's current expectations and assumptions, which may be affected by a number of factors, including those discussed in this press release and more fully described in Serono's Annual Report on Form 20-F filed with the US Securities and Exchange Commission on April 23, 2001.

These factors include any failure or delay in Serono's ability to develop new products, any failure to receive anticipated regulatory approvals, any problems in commercialising current products as a result of competition or other factors, our ability to obtain reimbursement coverage for our products, and government regulations limiting our ability to sell our products.

Serono has no responsibility to update the forward-looking statements contained in this press release to reflect events or circumstances occurring after the date of this press release.

About Serono Serono, headquartered in Geneva, Switzerland, is a global biotechnology leader.

The Company has six recombinant products on the market, Gonal-F(R), Luveris(R), Ovidrel(R), Rebif(R), Serostim(R) and Saizen(R).

In addition to being the world leader in reproductive health, Serono has strong market positions in neurology, metabolism and growth.

The Company's research programs are focused on growing these businesses and on establishing new therapeutic areas.

Currently, there are eleven molecules in development.

In 2000, Serono achieved world-wide revenues of US 1.240 billion, and a net income of US 301 million, making it the third largest biotech company in the world based on revenues.

The Company operates in 45 countries, and its products are sold in over 100 countries.

Bearer shares of Serono S.A., the holding company, are traded on the SWX Swiss Exchange (SEO) and its American Depositary Shares are traded on the New York Stock Exchange (SRA).

(1) Gabriela P.

Saborio, Bruno Permanne & Claudio Soto, "Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding"; Nature; June 14 2001; +1-411: 810-813.

UNS Contact: Serono International S.A., Geneva, Switzerland: Media Relations: Tel: +41-22-739-36-00 Fax: +41-22-739-30-85 Investor Relations: Tel: +41-22-739-3601 Fax: +41-22-739-30-22 Reuters: SEOZ.S/SRA.N Bloomberg: SEO SW/SRA US Serono, Inc., Norwell, MA Media Relations: Tel.

+1-781-681-2340 Fax: +1-781-982-1369 Noonan/Russo Communications London: Tel: +44-207 726 4452 Fax: +44-207-726-4453 Noonan/Russo Communications New York: Tel: +1-212-696-4455 Fax: +1-212-685-5348 Serono International, S.A., Media Relations, +41-22-739-36-00, or Investor Relations, +41-22-739-36-01; or Noonan-Russo Communications, +44-207-726-4452 (London), or +1-212-696-4455 (New York); or Serono, Inc.

Media Relations, +1-781-681-2340 Web site: ends dsl

14 Jun 01 - CJD - Serono devises method for early detection of BSE

By Patricia Reaney

YAHOO--Thursday 14 June 2001

LONDON, June 13 (Reuters) - Swiss biotechnology firm Serono SA said on Wednesday it has developed a new technique that will lead to earlier detection of Mad Cow disease and other animal and human brain disorders.

Mad Cow disease, or Bovine Spongiform Encephalopathy (BSE) and its human equivalent variant Creutzfeldt-Jakob disease (VCJD) are caused by abnormal brain proteins or prions.

The fatal degenerative brain disorders are diagnosed after death by an examination of brain tissue because levels of abnormal prions in other parts of the body are too low to detect.

Dr Claudio Soto and a team of scientists at the Serono Pharmaceutical Research Institute in Geneva have cultivated mutated prions in the laboratory for the first time and have devised a method to replicate them to high enough levels to detect the disease at an earlier stage, using existing tests.

The technique will improve understanding of prion diseases and may also help scientists to identify drug targets for vCJD and other brain disorders such as Alzheimer's and Parkinson's diseases.

"The procedure mimics the replication of abnormal prion proteins in the body in fast forward mode, compressing years of real-life time into a few hours in the laboratory," said Silvano Fumero, Serono's senior vice president of research and pharmaceutical development.

"This is a major scientific breakthrough and has potential applications in improving tests for prion diseases," he added.


In addition to improving the sensitivity of available tests, the technique that is described in the science journal Nature could form the basis of a blood or spinal fluid diagnostic test for live animals and humans.

There are currently no tests available for live or newly infected animals, or dead animals less than 30 months old.

Serono is seeking a patent on the technique, called Protein Misfolding Cyclic Amplification (PMCA), and is looking into collaborations or licensing agreements for animal and human applications.

"What we have done is one step before detection. We have amplified the material that can be then applied to any existing detection systems," Soto explained in a telephone interview.

"It boosts the amount of the material and therefore increases several hundred times the sensitivity," he added.

The Swiss research will also allow scientists to study normal prions in the laboratory to determine what causes them to mutate and force other normal proteins to change. Mutated prions form plaques in the brain which cause the disease symptoms and eventually death.

Scientists suspect the BSE epidemic was caused by feeding cattle meat and other byproducts from animals infected with BSE.

British researchers identified a new strain of the CJD in 1996 which they said was probably caused by eating meat infected with BSE.

So far there have been about 100 cases of vCJD in Britain, France and Ireland. Because of the long incubation period, which can be up to 30 years, scientists say it is impossible to predict how many people will be struck down by the disease. Estimates range from thousands to ten of thousands over the coming years.

14 Jun 01 - CJD - Live exports spreading BSE - expert

Staff Reporter

CNN--Thursday 14 June 2001

LONDON, England -- Exports of live animals contaminated with Mad Cow disease could be to blame for spreading the disease in eastern Europe.

Czech Republic authorities confirmed a positive test on a suspected case of Bovine Spongiform Encephalopathy (BSE) late last week.

"It will be a case exported not from the UK but from places like Germany, so it's the first caused by re-exports," Dr Stephen Dealler, a microbiologist who has worked on BSE since 1988, told Reuters news agency.

"What's happening is that we are seeing a potential indicator that the epidemic in Europe is greater than was expected, and it means the import of animals from particular places may actually become more and more complicated," he added.

Despite tough measures against meat-based feed introduced in 1996, the disease has spread further than governments first feared due to a muddle of live animal shipments and flawed feeding practices in Europe, the scientists said.

Britain first detected BSE in 1986 and has been blamed for spreading it to France, Germany and other European countries via exports of infected meat-based feed.

Scientists and the European Commission had warned eastern European countries they were likely to be at risk.

The results from the Czech case have been sent to Germany for verification.

UK scientists said other east European countries were likely to have the disease, linked in 1996 to the fatal human form, new variant Creutzfeldt-Jakob disease, which has claimed the lives of about 100 Britons.

"Cases should already be being picked up both in humans and in cattle (in eastern Europe)," Iain McGill, a veterinary surgeon who worked for the UK agriculture ministry at the height of Britain's crisis, said recently.

"The spread of meat-and-bone-meal (animal feed) and the transport of cattle has occurred from Europe into eastern Europe, so you would expect a progression of disease."

Dealler agreed, saying BSE was probably already widespread in the region.

"The Poles' statements that they have never got a case of BSE in their lives probably aren't true," he said.

Dealler said one infected cow could pass on the disease to 50 others and even if potentially risky parts of the animal were never used, the disease could hit two or more other cattle.

"Imagine you're doing the best you can and you're still infecting two other cows. That means the disease continues to spread and grow," Dealler said.

He said government inaction was to blame.

"What happened is they shouldn't have been so smug in assuming they didn't have any cases," he said of governments across Europe.

"What they could have done was to take on some quite severe action, but they didn't."

14 Jun 01 - CJD - Blood Test

Staff Reporter

New Scientist--Thursday 14 June 2001

Blood tests could soon pick up the human form of Mad Cow disease - before any symptoms appear

Blood tests could soon pick up the human form of Mad Cow disease - before any symptoms appear. Two groups of researchers have developed separate ways to detect low levels of the prion which causes variant CJD.

The startling advances could also lead to animal tests for BSE and scrapie and ways to screen blood donated for transfusions.

Small amounts of the prion that causes vCJD are thought to circulate in the blood, but current tests cannot detect it. Now Claudio Soto and his colleagues from the Serono Pharmaceutical Research Institute in Geneva have found a way to amplify the offending prion.

They do this by using small amounts of infectious protein to convert much larger amounts of the normal form.Until now, no one had managed to do this in the lab because newly converted prions form unreactive clumps.

Soto uses sound waves to break up the clumps after each round of conversion. When brain extracts from scrapie-infected hamsters were passed through five cycles of the treatment, the amount of converted prion was amplified over 30 times.

They also amplified prions from human brain samples, but have still to publish their results. "We are confident that with our method we'll be able to detect it [in blood]," Soto says.

Sticking power

A team at Oxford University and the Institute for Animal Health in Berkshire is also blazing a trail to a blood test. Oxford pathologist William James is using artificial strands of RNA called aptamers, which stick to molecules of particular shapes.

From an initial pool of 1014 molecules, James's team selected an RNA strand that is 100 times better at binding to infectious prions than to the normal protein. "It's the first time that anyone has found something that fits the disease form better than the normal form," James says.

James's team, together with American firm VITEX in Watertown, Massachusetts, hopes to produce a blood-testing kit that could screen donated blood samples to make sure they are safe, diagnose people with vCJD before symptoms arise, or screen livestock so that infectious animals can be removed from the food chain.

VITEX also plans to remove prions from infected blood samples by this method. Passing blood through huge columns containing immobilised aptamers would mop up any infectious molecules on the way through.

This could lead to a treatment for vCJD. "We know that aptamers inhibit the conversion process in vitro," says James. He believes it may be possible to design small molecules to do the same thing in the body. These could be used as drugs to slow down or halt the progression of the disease.

More at: Nature (vol 411, p 810)

14 Jun 01 - CJD - Looking for a faster way to detect Mad Cow disease

Joy Powell

Minneapolis-St. Paul Star Tribune---Thursday 14 June 2001

FONTENAY-AUX-ROSES, France - At a high-security laboratory near Paris, a U.S. veterinarian worked side by side with French scientists. Wearing white gowns and masks that covered their mouths and noses, they peered through plastic face shields at the deadly brew in front of them.

In a well on a tiny plate, a solution had turned bright yellow. That change signaled the presence of infectious prions - the warped brain proteins that cause Mad Cow disease.

The veterinarian, S. Mark Hall, was on a mission in Europe this spring to find a faster way to screen U.S. cattle for Bovine Spongiform Encephalopathy (BSE), commonly called Mad Cow disease.

Faster methods are needed because the U.S. Department of Agriculture (USDA) is stepping up testing of slaughtered cattle for BSE. Hall, who heads the pathology section at the National Veterinary Services Laboratories in Ames, Iowa, is leading the search for new methods.

BSE was first discovered in Britain in 1986. In the past two years, wider screenings of cattle have detected BSE in France, Germany, Ireland and other nations. The scare is leading the United States to double the number of slaughtered cattle that will be tested this year. About 5,000 sick cows will be targeted.

There's no indication that BSE is in the United States, which has taken measures to keep the nation free of the disease. But some experts say testing hasn't been widespread enough to detect the disease.

"We're working hard to make sure that we test an adequate number of cattle in this country," Hall said after his European trip.

The race is on because Mad Cow and the human form of the disease are always fatal. More than 100 people, mostly in Britain, have died of variant Creutzfeldt-Jakob disease. Creutzfeldt-Jakob is believed to come from eating tainted beef.

With an incubation period that could exceed 40 years in humans, nobody knows how many other people could develop the disease. Once symptoms appear, death follows, usually within months.

If BSE is diagnosed in the United States, the nation's $175 billion beef industry would be crippled and world trade balances disrupted. Elsewhere in the world, the disease has led to millions of cattle being destroyed and billions of dollars being spent.

"If you want to stop the crisis, you have to be able to give a guarantee that no dangerous cattle will enter into the food chain," said Jean-Philippe Deslys, a leading French scientist who developed a test that detects BSE prions.

Hall is a tall, bearded scientist with a Ph.D. in veterinary pathology from Oklahoma State University. He is an expert in pathology - the microscopic examination of tissues for lesions or abnormalities. He's also trained in testing methods based on chemistry.

Because of Hall's knowledge in both areas, his boss chose him to scout for new tests to be used in Ames, which is the nation's central diagnostics site for BSE and other diseases that could affect the U.S. economy.

Five weeks ago, Hall traveled from Iowa to France, Belgium, Austria, Germany and Switzerland to evaluate BSE testing methods.

On a sunny morning in early May, he arrived at France's Atomic Energy Commission in the Paris suburb of Fontenay-Aux-Roses. The massive fortress of white and gray buildings is a heavily guarded national defense facility.

Hall was escorted to the medical research laboratories, where he met with Deslys, the pioneer in prion research. Deslys told Hall about his data and the French test. Over the next two days, Hall was trained how to run the test.

He and other scientists weighed samples dissected from softball-size cattle brains. They treated the samples with an enzyme that stripped away normal prion proteins, leaving only the misshapen prions that eat away brain tissue and leave spongelike holes.

Using a special solution, the scientists concentrated the rogue prions in a well on a 3-by-5-inch plastic plate. They added an antibody that clings to prions, immobilizing them, and then added a second antibody. After more processing, the mixture glowed yellow.

"It's very impressive," Hall later said of the highly sensitive French test, which is marketed by a California-based company, Bio-Rad Laboratories.

He also evaluated other tests. Hall is mum on whether he favors any one test, saying that even the slightest nod from him could affect stock prices. Each test uses a different method, but all examine tissue for prions.

Prion-diagnosing tests are used after symptoms appear during late stages of the disease in humans, whose tonsils are tested, or on brain and spinal tissue taken from dead animals.

No one has yet developed a blood test that can detect the presence of the disease in its early stages. Around the world, companies are scrambling to develop one.

Hall's home base is a cramped diagnostics laboratory tucked into a strip mall in Ames. In leased space that once was an old welding shop, Hall runs a section that diagnoses prion-related diseases in animals.

USDA officials are asking Congress for up to $548 million to build a new, consolidated center. Until then, the pathologists and other scientists are scattered throughout Ames in dozens of buildings.

Pathologists examine brains from sheep, cows, deer and elk. They first "fix" the soft, pink brains for five days in a formulin solution that hardens them to a gray, rubbery consistency. Part of the brain stem, where prions typically concentrate, is removed and sliced as thin as onion skin. It's mounted on slides, stained and studied under a microscope.

The pathologists are hunting for holes in the brain tissue that accompany prion diseases, called transmissable spongiform encephalopathies (TSEs). BSE is one of them.

As their workload increases, American scientists are turning to Europe for a faster testing method in which ground brain samples are mixed in a solution, without the five-day "fixing" process or use of a microscope. The solution simply changes color if rogue prions are present.

Because European tests have been known to produce false positive results, they must be verified by "gold standard" tests now used in Ames, said Art Davis, chief of the pathobiology laboratory.

Though testing is being ramped up, the United States lags far behind European countries that are testing all slaughtered cows older than 30 months before they enter the food chain. Prions accumulate over time and are easier to detect in older animals. And older cows might have been fed contaminated feed before governments banned use of meat and bone meal as a protein supplement.

In 11 years - from 1990 through March 31 - the United States tested 12,341 slaughtered cattle for BSE.

France has tested more than 500,000 slaughtered cows older than 30 months since Jan. 1. Cows that are 30 months or older are incinerated if not tested to prevent them from getting into the food chain.

From July 1999 to December 2000, France tested a smaller number: 50,000 "downer" cows that couldn't walk or were considered emergency slaughters because they were sick. The incidence of BSE was 1.5 cases per 1,000 sick cows.

Rather than testing by age, the United States is targeting downer cows that can't walk at slaughterhouses and others exhibiting neurological problems.

14 Jun 01 - CJD - BSE: Is It A Threat?

by Tosha Sweitzer

Organic Consumers Association---Thursday 14 June 2001

Mad Cow Disease; it's a British problem. It will never show up in the United States. Or will it? This paper will explain what Bovine Spongiform Encephalopathy is and how it is spread, then illustrate why it is a threat to human lives in the United States. It will also explore other Transmissible Spongiform Encephalopathies, including Creutzfeldt-Jakob disease, Chronic Wasting Disease, and scrapie.

"Beef is one of the great unifying symbols of our culture. The Roast Beef of Old England is a fetish, a household god, which has suddenly been revealed as a Trojan horse for our destruction." --THE GUARDIAN

An Overview of Transmissible Spongiform Encephalopathies

Tracie McEwen reaches over to the dying man... As he moans softly, she strokes his arm and kisses his forehead. "It's OK. Doug, it's OK."

Tracie married Doug exactly four years ago. She marked their anniversary by pouring sparkling cider into cups, making a toast, and lovingly dropping some into Doug's mouth....

It started slowly. First, there was the memory loss and the inability to do simple math, then the light tremors. Eventually came violent seizures as well as unexplainable outbursts of emotion--hysterical laughter, sometimes followed by uncontrollable crying. By late January, he could no longer speak in sentences....

"This is the worst thing I have seen," [Tracie McEwen] says. "I wouldn't wish it on my worst enemy."

The above, reported by Mark Kennedy in the Ottawa Citizen, accurately describes death from Creutzfeldt-Jakob disease. At first, victims like Doug McEwen just seem to have memory problems. They often forget phone numbers or seem confused. Nobody thinks it's a big deal. Then their personalities suddenly change, they lose their balance, and their vision blurs. Finally they stop walking and swallowing and lose all bowel and bladder control. Most victims spend their last months on a feeding tube in a vegetative state.

Doug McEwen was a sportsman with a passion for deer hunting. He regularly ate deer meat. Two other deer hunters recently died from CJD: Jim Koepke, 39, of Nevada in February 1999, and Jay Dee Whitlock, from Oklahoma in April 2000. Despite the fact that doctors cannot confirm how these deer hunters developed CJD, no one can rule out the venison they regularly ate. In a January 1999 article in USA Today, Lawrence Schonberger of the Center for Disease Control said only five cases of classic CJD per billion people are reported annually worldwide for people aged 30 and under. These young hunters may have contracted CJD from eating the meat of deer infected with chronic wasting disease.

Chronic wasting disease, or CWD, is a neurological disorder found in elk and deer that causes brain damage, results in severe weight loss and is eventually fatal. It has been found in 5-15% of wild deer and elk in areas of Colorado and Wyoming. It has also been identified in animals in Nebraska, and in elk farms in the above-mentioned states, as well as Montana, Oklahoma, South Dakota, and Saskatchewan, Canada. Like Creutzfeldt-Jakob disease, chronic wasting disease is a form of Transmissible Spongiform Encephalopathy.

Transmissible Spongiform Encephalopathies, or TSEs, are neurological disorders that are caused not by viruses or bacteria, but by abnormally folded proteins called prions. Because prions are proteins, not foreign bodies, they are invisible to the immune system. They are also virtually indestructible; they can survive conditions that kill any known pathogen. Boiling, freezing, and soaking in harsh solvents like formaldehyde, carbolic acid, or chloroform have no effect on prions. They have survived temperatures of over 700F, and easily jump the species barrier.

Infectious prions are chemically identical to normal proteins; they have the same amino acids, they just have a different 3D structure. Enzymes can break down normal proteins, but they cannot break down prions, because of their unusual shape. Once a tiny amount of abnormal prions appears, they can convert normal proteins into prions. This leads to "clumping" of proteins, which causes tiny holes, called "vacuoles", in nervous tissue. The holes make it hard for electrical impulses to be transmitted between neurons.

Creutzfeldt-Jakob disease is a TSE that infects humans. German researchers discovered classic CJD, or sCJD, in the 1920's. sCJD is considered a biochemical fluke, a disease that strikes randomly and infrequently all over the world. It has a long incubation period, and strikes mainly older people. Most of the time it appears sporadically, striking one person in a million, typically around age 60.

Alison Williams, a twenty year old woman from North Wales, developed CJD in 1986. Her case led to the description of new variant Creutzfeldt-Jakob disease, or vCJD, a more virulent strain that can be spread through the consumption of infected animal protein.

The most well known form of TSE is Bovine Spongiform Encephalopathy, otherwise known as Mad Cow Disease. It is so named because the animals become agitated, shake with tremors, and kick violently when touched. BSE has spread across Europe like a plague, and has many concerned that it will spread even farther. But how did it all start?

History of Transmissible Spongiform Encephalopathies Scrapie, which infects sheep, was the first TSE discovered. Shepherds in Britain and Europe first described it in the 1730's. Like BSE, scrapie causes infected animals to grow ill tempered and wobbly. Then they experience seizures, paralysis, and blindness for the next three to six months until their deaths. So far, no one has contracted CJD from consuming tainted mutton, although scrapie is still common in sheep.

Dr Carleton Gajdusek, a virologist employed by the National Institutes of Health in Maryland, found a similar disorder in the Fore people of Papua New Guinea in the 1950s. The condition was called Kuru, and was thought to be a curse cast by sorcerers. The symptoms of Kuru were eerily similar to those of CJD. Gajdusek believed it to be an epidemic disease, because the appearance of this disease coincided with a change in eating habits of the women and children of the Fore people. They had been eating the prion-infected brains of dead relatives in a religious ritual.

Gajdusek examined living patients, and found no outward signs of infection, and nothing suspicious in their spinal fluid. His colleagues in Maryland found that their brains resembled those of CJD patients. Dr. William Hadlow, a scrapie expert, read one of the many reports that Gajdusek wrote about Kuru. Hadlow recognized many of the symptoms of Kuru victims, and noticed that the vacuoles in their brains were similar to those of sheep with scrapie. Hadlow wondered what would happen if he injected a healthy animal with brain tissue of a Kuru victim. Studies had shown that healthy animals developed scrapie when injected with a sheep's diseased brain tissue. So, Gajdusek injected chimps and monkeys with the ground brains of Fore tribeswomen, then repeated the experiment with brain tissue from an American CJD victim. He won a Nobel Prize when the results of his experiments proved that both Kuru and CJD were transmissible.

Just before Christmas in 1984, a vet inspected a cow at Pitsham Farm in South Downs, England. The cow was drooling, arching its back, waving its head, and threatening its peers. The vet named this unfamiliar disease Pitsham Farm syndrome. In 1986, pathologists discovered that Pitsham Farm syndrome was yet another variation on scrapie, Kuru and CJD--a Bovine Spongiform Encephalopathy, or BSE.

The Epidemic

BSE has spread at an alarming rate. Since its discovery in Britain, it has been found in Belgium, Ireland, Italy, France, Spain, Switzerland, Portugal, Holland, Denmark, Luxembourg, Liechtenstein, Germany, and Canada. In February of this year, Italy confirmed its fourth case of BSE in domestic cows. Denmark confirmed its forth domestic case of BSE recently, and officials from the Danish Veterinary and Food Administration said that they expect the figure of confirmed cases to reach 10 by the end of the year. BSE has killed nearly 200,000 cattle, and claimed 94 human lives so far.

How did BSE spread to epidemic proportions? [...]

One influence that caused BSE to spread to such a great magnitude was tainted feed. Infected cows were ground up and shipped around the world as animal feed. For several years after the disease first appeared at Pitsham Farm, the British government allowed cattle to be fed a gruel derived from meat by-products, which includes "downer" cows, considered unsafe for human consumption, and meat stripped from the spinal area of carcasses, as well as bones and inedible organs. This infected feed was fed to British cows until the government banned the use of beef by-products in any feed meant for ruminant animals.

Yet they continued to allow feed to be exported, and Asian nations alone bought nearly a million tons from 1988 to 1996. Fortunately, many countries have banned beef products imported from the United Kingdom. But it may be too late. Sumolya Kachanapangka, of Bangkok's Chulalongkorn University said, "It is probable that some of our cattle were fed with tainted feed that was imported into the country before the ban was imposed."

The government also allowed feed containing beef by-products to be fed to non-ruminant animals. They soon found it impossible to keep the possibly infected pig and chicken feed separate from cattle feed in factories and on farms. In a recent inspection, nine percent of feed mills were found to lack a system to prevent the mixing of cattle feed with feed made for other animals. The continued sale of feed made from the meat and bone meal of contaminated animals was the factor that is most responsible for BSE's rapid spread across Europe. [...]

Other ways that BSE spread was through Growth Hormone, organ transplants, and vaccines. "The thing that worries me is the immunization of the children," says Dr. Tom Pringle, a molecular biologist and the administrator of the Official Mad Cow Disease Home Page. "Every kid in the United States can't go to school without their shots... They're growing vaccines out of fetal-calf serum. Then you're injecting fOur Worldyear-old children--which is much worse than eating, 100,000 times more effective... Every schoolchild in the UK has already been immunized with vaccine made from serum from infected bovines."

There have been several documented cases of people getting CJD from corneas, brain matter grafts, and other organs transplanted from infected donors. Blood, blood products, bovine extracts and transplant organs are not screened for CJD in the United States, although around the world infected organ recipients, who developed symptoms sometimes decades after treatment, have been traced to infected donors. Blood meal and horticultural bone meal can be easily inhaled or enter the body through the eyes, a direct route to the brain. [...] Surgical instruments are at a high risk of transmitting the infection, because standard sterilization does not neutralize infectious prions.

In the early 1960's, endocrinologists discovered that human Growth Hormone may possibly help dwarves attain normal heights. Human cadavers were the only known source of hGH, which is produced in the pituitary gland. The National Pituitary Agency, started by the federal government in 1963 to gather pituitaries, distributed hGH to roughly 8,200 kids within the next two decades.

This continued until a frightening pattern emerged in 1984. Normally, sCJD is rare in people under 50. But patients who had received hGH injections were getting CJD in their 20s. By spring 1985 there were four such cases on record, and 27,000 of the world's children had received injections when the practice was stopped on April 20. No one knew how many pituitary donors had been silently incubating CJD, or how many recipients were now set to die from it. [...]

Growth Hormone recipients weren't the only people unknowingly exposed to CJD. In November 1999, scientists produced experimental evidence that blood can contain infectious prions. In April of the next year, the U.S. FDA and American Red Cross established a ban on blood donations from people who have spent six months or more in Britain between Jan. 1, 1980, and Dec. 31, 1996.

"We have to be very responsible and be extremely safety conscious and keep [blood] donations as safe as possible even at the risk of depleting the blood supply," said Rebecca Haley, a chief medical officer at the American Red Cross. She warns that Mad Cow disease could pose as great a threat to the blood supply as AIDS if precautions aren't taken.

The American Red Cross will soon broaden its ban to include blood from all Western Europe countries, and blood donations from anyone who has lived or traveled in Western Europe for at least three months.

The Reaction

The Mad Cow Disease craze has caused myriad extreme reactions among consumers, government officials, and expert scientists.

Unfortunately, many government officials are spreading the deception that the United States is completely free of Mad Cow disease. Susan Combs, Texas Commissioner of Agriculture and a rancher, assured investors of U.S. beef safeguards. "We've not fed ruminant feed to cattle in years...I don't think we're going to see BSE in this country. We have a firewall," said Combs. Apparently, she was not aware that until the end of January, Purina had fed ruminant feed to cattle in Texas.

"Banning imported meat from the countries would minimize the risk of contaminated meat that will enter the human food chain," said Dr. Eric Tayag. He insists that stringent measures put up by the Department of Agriculture have prevented the spread of the disease by prohibiting the entry of contaminated meat or livestock from countries reported to be having problems with BSE.

Many consumers don't buy into the government's misinformation, and are avoiding beef. A study by Gardenburger Inc. concluded that nearly 20 percent of Americans have already reduced their beef consumption or have stopped eating beef entirely. Six out of ten Americans polled say they are concerned that the disease may affect beef in the United States. Three-quarters of Americans say they would be likely to avoid or reduce their consumption of beef if the disease was even suspected in American cattle. Forty-two percent of Americans polled said they would be likely to try meat alternatives, such as soy or veggie burgers.

And experts agree with these consumers. House Speaker Feliciano Belmonte Jr. urged the public to temporarily refrain from eating beef until the government has a way to detect if meat products are contaminated with BSE. Belmonte said, "It would be better for our people to eat fish, vegetable, chicken or pork in the meantime."

"I don't think that any country can say they are 100% sure that they are free of BSE," says Ralph Blanchfield of the independent Institute of Food Science and Technology based in Britain.

The Future

Mounting evidence indicates that U.S. ranches already contain Mad Cows, and that the occurrence of vCJD in the U.S. is exponentially higher than the Centers for Disease Control has revealed.

CJD's tremendous complexity, long incubation period and similarity to Alzheimer's have probably obscured the leading edge of a deadly epidemic in the United States. CJD is so rare that few doctors even recognize the symptoms, so it's frequently misdiagnosed as Alzheimer's disease. Dr. Michael Hanson, chief scientist of Consumers Union, reviewed the situation, "A study at the University of Pittsburgh, in which autopsies were done on 54 demented patients diagnosed as having probable or possible Alzheimer's or some other dementia (but not CJD), found three cases (or 5.5%) of CJD among the 54 studied (Boller et al., 1989). A Yale study found that of 46 patients diagnosed with Alzheimer's, six (or 13%) were CJD at autopsy [...]. Since there are over two million cases of Alzheimer's disease currently in the United States, if even a small percentage of them turned out to be CJD, there could be a hidden CJD epidemic."

TSEs have been found in populations of American mink, deer, elk, sheep, goats, and squirrels. These TSEs were acquired through contaminated feed and maternal transmission, and probably from contaminated areas and through close proximity of animals to one another. Wild animals might also contract the disease by raiding contaminated feed meant for livestock. That means that these animals catch TSEs from eating feed meant for cattle and other livestock. Feed that contains prions. Since the cattle are eating the same food, it's only logical to conclude that they have also consumed prions.

How did infected feed end up in the United States? For three years after BSE was diagnosed in Britain, feed made of rendered animals was imported from Britain. In addition, over the past decade, 32 cows were shipped in from Britain that USDA officials can't account for.

"The sheep thing in the US is demonstrably worse," says Pringle. "They know they've got scrapie in thirty-nine states, that there are a lot of really infected flocks, and they know those flocks are being eaten by people, and there's no effort to keep scrapie out of the human food chain. They've gotten away with murder."

America's safeguards and surveillance efforts are far weaker than most people realize. Sure, officials have examined brain tissue from 12,000 "downer" cattle without finding any BSE. But they are ignoring the fact that not only "downer" cattle can have BSE; in reality infected cows may look healthy for five years.

Leading Food Onlinesafety advocates question the USDA's small test sample, methodology and motives. Dr. Pierluigi Gambetti says, "thirty-seven million animals are slaughtered a year for consumption and less than 1,000 are tested a year - it's too low," he said. "If you don't look, you don't find it. Our testing is not on the cutting edge,"

Dr. Pringle agrees. "The FDA is taking half-measured steps. It's a surveillance problem, it's weak. It's more than a matter of time before we get our first case." Germany seemed BSE-free when only downers were tested, says Marcus Doherr, an epidemiologist. "Now, with very intensive screening, they have found over 30 cases within two months."

Not only is the testing weak, but regulations are as well. Dr. Stephen Sundlop of the FDA said that at least 700 of 5,000 feed handlers inspected were not in compliance with regulations aimed at keeping U.S. cattle feed free of bone and meat meal from dead cattle, and inspections of about 5,000 more have yet to be completed. The General Accounting Office found that 20 percent of the 1,700 U.S. companies handling both restricted and unrestricted feed "did not have a system... to prevent commingling and cross contamination."

Says Dr. Hanson on the regulations concerning the feeding of beef by-products back to cattle, "All they said is that you've got to label it 'Do not feed to cattle and other ruminants.' Farmers can walk in a feed store and still buy it. Nobody asks 'Are you feeding it to cattle or pigs?'.... The way the rule is written, you can take scrapie-infested sheep, CWD-infested deer and BSE-infested animals and legally put that in animal feed and give it to pigs, chickens--anything but ruminants, as long as it's labeled. That's outrageous."

Britain utilized the existing U.S. standard from 1988 until 1996. There was so much cross-contamination that an estimated 60,000 cattle were contaminated as a consequence. Why do Americans believe that this technique will be any more successful here? If more stringent measures are not taken, Americans will soon find out exactly why neurogeneticist John Collinge, a member of the British government's Spongiform Encephalopathy Advisory Committee has warned of "a disaster of Biblical proportions."

14 Jun 01 - CJD - Rogue protein 'could help cancer spread'


PA News--Thursday 14 June 2001

Academics say cancers may be helped to spread around the body by a rogue protein similar to the one thought to cause Mad Cow disease.

In diseases such as BSE and its human version, variant CJD, proteins called prions in nerve tissue alter shape and become dangerous. Other prions they come into contact with are then dangerously modified in a chain reaction.

Scientists have now found a protein in the blood of cancer patients that acts like a prion. The cancer-related protein, called tNOX, has a prion-like form which can resist both heat and enzymes called proteases which would normally break it down.

Experiments showed that it appeared to be capable of altering other proteins so they acquire the same resistant properties, New Scientist magazine reported.

James Morre, from Purdue University in Indiana, who led the research, found that tNOX was most concentrated in the blood of cancer patients whose disease had spread. He suspects the protein might help cancers invade different parts of the body.

Mike Scott, a prion researcher at the University of California in San Francisco, said: "We always tend to think of cancer as a genetic disease, caused by mutations. But there is this distinct possibility that there might be some prion-like mechanism involved in at least some tumours."

Morre stressed that tNOX protein cannot "infect" people like a true BSE prion. When the protein was injected into healthy mice, the animals did not get cancer.

14 Jun 01 - CJD - Experts fear Mad Cow pandemic

Mark Kennedy

Ottawa Citizen---Thursday 14 June 2001

UN report draws parallels to early stages of AIDS explosion; Long incubation allows stealthy spread

PARIS -- Mad Cow disease has joined AIDS as a major public health challenge facing the world, an international conference was told yesterday.

And just as governments imposed safety measures -- even without scientific justification -- to prevent the spread of AIDS two decades ago, so too must governments take precautionary action now to limit the spread of the human form of Mad Cow disease.

The stark message was delivered to government officials and scientific experts at the launch of a fOur Worldday meeting hosted by United Nations agencies that fear the fatal neurological disorder could become a global epidemic.

"Despite high-level political posturing at the start of this (Mad Cow) epidemic among many, many countries there's no denial today that potentially infected live animals and potentially infected products have been distributed widely," said Dr. David Heymann, executive director of the communicable diseases program at the World Health Organization.

"And the challenge is to determine just how widely and to determine how best to help, especially the developing countries determine their risk and what they can do to prevent the spread."

Dr. Heymann told the conference there are many striking similarities between Mad Cow disease and AIDS, which exploded onto the world stage 20 years ago as a frightening disease that baffled scientists.

"Both have a very long incubation period, one which permitted the disease to be widely distributed before it was even recognized," said Dr. Heymann.

Other similarities include: there is no vaccine; they're both "universally fatal"; and at the outset of each disease, there was no diagnostic test to determine whether someone is infected (an AIDS blood test was eventually discovered, but one is still lacking for people with the human form of Mad Cow disease).

"Both of these diseases required policy decisions before the science was in place to really make these decisions based on science," said Dr. Heymann.

He said that just like AIDS in the early days, Mad Cow disease carries many unknowns: How easy is it for a human to get it from contaminated food and donated blood? How many will get it and die? Can it ever be cured?

And so while Dr. Heymann said it's too early to suggest the disease will become a mass killer like AIDS, it's also too soon to insist the disease has not established a "foothold" in many countries.

"Some may say it has not, but the public health implications are profound if they are wrong."

Conrad Brunk, a University of Waterloo professor and expert in risk communication, told the conference the worst thing regulators can do is tell people there is "zero risk" associated with a product. Governments often do that because they fear the public will panic if told there is an unknown risk connected with something.

"That's a recipe for disaster down the road," Mr. Brunk said in an interview, "because events will prove you wrong and you'll lose your credibility as a risk regulator."

That's exactly what happened in Europe, where government officials spent years wrongly assuring their citizens that Mad Cow disease would not emerge within their nations.

Dr. Samuel Jutzi, a senior official of the UN's Food and Agriculture Organization, told the conference "animal agriculture in Europe has never before been faced by a similar vote of distrust from the consumers and the society at large."

Moreover, he suggested that "free market forces and deregulation" have contributed to the heightened risks of Mad Cow disease crossing borders through trade in goods and animals.

"Market liberalization and globalization may indeed, in the absence of strict enforcement of biologically and ecologically justified regulation, contribute to worldwide spread of such risks," Dr. Jutzi said.

Mad Cow disease is thought to have started with a single British cow that experienced a genetic mutation and developed Bovine Spongiform Encephalopathy (BSE). The disease was spread to other cattle when the BSE-infected cow died and its remains were recycled in animal feed to other cows. When those cows died, they were also fed back to cows.

Eventually, a large proportion of the British herds became contaminated in the 1980s, and some people who ate the beef contracted the human form of the brain-wasting ailment, known as variant Creutzfeldt-Jakob disease (vCJD).

About 100 people, mostly Britons, have contracted vCJD, and estimates of the final toll range from hundreds to millions of victims. The most recent suspected victim is a 34-year-old woman in Hong Kong who travelled frequently to Britain in previous years and may have caught the disease there.

Still, what frightens public-health officials is that much of the tainted U.K. beef and beef products were exported throughout the world in the '80s and '90s.

Animal feed supplements, known as meat and bone meal (MBM), containing the ground-up remains of infected British cattle were exported to dozens of countries, where more humans will eat infected beef and be exposed to vCJD.

In Canada, a report prepared for the federal government last summer concluded that Mad Cow disease could already be silently incubating among cattle and unsuspecting humans in this country.

However, officials in Health Canada and the Canadian Food Inspection Agency say they are confident this country is "BSE-free." The Canadian cattle industry provides the same assurance.

Nonetheless, critics say Canada's existing safeguards contain loopholes. They say there should be mass testing of cattle to look for BSE. And they say a 1997 feed ban that prohibits the feeding of cow-based meat and bone meal back to cows doesn't go far enough. It should also be extended so the feed isn't given to other animals such as pigs and poultry, they say.

10 Jun 01 - CJD - Mad Cow case in Hong Kong

Gulf Daily News--Sunday 10 June 2001

Hong Kong: Health authorities have found the first suspected case of the human form of Mad Cow disease in Hong Kong.

After performing brain scans and tests, doctors believed a 34-year-old Chinese woman, who was not identified, has variant Creutzfeldt-Jakob (CJD) disease, the Hospital Authority said.

Her symptoms include progressive neurological disorder, involuntary limb movements and dementia, the authority said. She was admitted to a hospital last month and is in serious condition.

I must stress that it is still a suspected case at this stage and the experts are still investigating, Director of Health Margaret Chan said.

Neurologist Richard Kay, who is treating the woman, said she probably contracted the disease from eating beef in Britain, but doctors were awaiting a diagnosis from the National CJD Surveillance Centre in Edinburgh.

She can't walk and can't talk coherently, but she is still conscious, Kay said. The disease has a very long latent period before striking.

Chan stressed that the patient never donated blood a possible means of spreading the disease. Hong Kong banned beef imports from Britain in 1996.

CJD disease has killed about 80 Europeans since the mid-1990s, mostly in Britain.

10 Jun 01 - CJD - Suspected victim of `Mad Cow' disease in stable condition

Staff reporter, Hong Kong iMail

Hong Kong Mail--Sunday 10 June 2001

An SAR woman believed to be suffering from the human form of ``Mad Cow'' disease was in a ``stable'' condition yesterday.

The 34-year-old had displayed all the symptoms of variant Creutzfeldt-Jakob disease (vCJD) when she was admitted to Prince of Wales Hospital.

The case, believed to be the first in Asia, was detected last month, the Department of Health and the Hospital Authority said on Saturday.

Doctors believe the Chinese woman contracted the disease in Britain, where she lived from 1985 to 1992. For the next five years she lived in Hong Kong but returned to Britain several times.

In 1997, she returned to Britain and stayed there until earlier this year, when she returned to the SAR to seek treatment.

Officials said there was no need to isolate the patient because the disease was not spread through social contact.

The first case of vCJD was reported in 1996 and was linked to the ``Mad Cow'' epidemic in the UK.

10 Jun 01 - CJD - Quickening the Diagnosis of Mad Cow Disease

Laura DeFrancesco

The Scientist---Sunday 10 June 2001

Quickening the Diagnosis of Mad Cow Disease

New tests for prions shorten the time frame for detection

June 11, 2001 The Scientist (Volume 15[12]:22) by Laura DeFrancesco

Europeans have destroyed 4.5 million cows since 1996, the height of the epidemic in the United Kingdom, because they were believed to be at risk for Mad Cow disease (Bovine Spongiform Encephalopathy, or BSE).1 Necropsies, however, showed that only a few hundred thousand of them actually were infected.2 Had a diagnostic test for Mad Cow disease existed when this epidemic erupted, these numbers might have been different. But no such test did exist. The only available assay was a bioassay in which host animals are inoculated with material from suspected animals, and some number of months to years later, an answer appears.

Now basic research on prions, the infectious proteinaceous particles that cause Mad Cow disease and other cases of transmissible spongiform encephalopathy (TSEs), has caught up with the disease. The latest batch of assays validated for use by the European Commission cuts the time down from months to hours. And even faster, more sensitive tests, including one that purports to detect single prion aggregates,3 are on the horizon. Millions of tests for the BSE prion have now been sold throughout Europe, and as a result, wholesale slaughter of herds is no longer taking place. Instead, animals en route to slaughter for food use are tested for BSE prior to their entering the food chain--testing up to 100 percent of feed animals in Germany, for example.

Prions are found throughout virtually all species, and for the most part are innocuous. However, certain events, such as a mutation or exposure to infected material, causes a conformational change in the protein that makes it deadly and infective. This change, from an a-helical form (PrPc) to a -flat sheet (PrPsc), is all that distinguishes the normal prion from the abnormal, infective version. The identity of the two forms, in terms of amino acid composition and even post-translational modifications, has made detecting and quantifying abnormal prions a challenge.

The first real breakthrough in prion research came in 1997, when Michael Scott and colleagues from the Institute for Neurodegenerative Diseases (directed by Nobel laureate Stanley Prusiner) at the University of California, San Francisco, created a strain of transgenic (Tg) mice that overexpressed the bovine prion PrP protein.4 The wild mouse strain, without the extra gene, was relatively resistant to BSE. Adding the bovine gene resulted in the mice not only becoming sensitive, but much more so than the cows, the natural host. Tg mice die within a few months of infection, rather than the years it takes for the disease to appear in a cow. This was an important first step in designing experimental tools for studying BSE, and for determining the precise prion concentration in infected cows. According to Prusiner colleague Jiri Safar, the wild mice test underestimates the prion concentration by as much as 1,000-fold, which means the exposure of general population to BSE prions in the United Kingdom was probably much higher than previously thought.

Serious and exhaustive screening efforts, however, require even speedier tests, preferably ones that can test animals by the thousands. Currently, several immunodiagnostics tests, which can distinguish the normal from the abnormal prion, are filling this need. The European Commission has validated three such tests: a western blot assay developed by the Zurich, Switzerland-based Prionics AG; an ELISA assay made by Enfer Scientific Ltd. of Newbridge, Ireland; and a sandwich immunoassay developed by the French agency CEA and marketed by Bio-Rad of Hertfordshire, U.K., the most sensitive one to date.5

A Conformation-Dependent Assay

While western blotting and other immunodetection methods can detect abnormal prion protein, they are laborious, and only work on postmortem material. The hunt is on for assays that are sensitive enough to detect prions in central nervous system fluids and other biomaterials that could potentially be used in clinical diagnosis. To this end, Safar and Prusiner at UCSF are looking for antibodies that can directly distinguish normal from abnormal protein. Capitalizing on the fact that the normal and abnormal proteins, because of differences in conformation, have different epitopes exposed, they have isolated antibodies that recognize epitopes exposed in the normal prion, but are hidden in the abnormal. They found that with such antibodies, they cannot only quantify abnormal protein but also differentiate eight different prion strains, each with a particular and different conformation.6

Mad Cow disease, one of a group of degenerative diseases of the brain, has created not just scientific problems, but economic, ecological--think 4.5 million carcasses--and political ones as well. Designing surveillance programs present their own challenges, as farmers stand to lose their livelihood in the process. At the PittCon 2001 meeting in New Orleans this past March, Bruno Oesch of the University of Zurich described how the approaches to surveillance in different European countries has had an impact on disease reporting. In Switzerland, for example, every living thing is slaughtered on farms (including pets) where infected animals are found. Predictably, the number of reported cases is low. In contrast, in Portugal, where farmers are compensated more than 125 percent of the herd's value, BSE reporting was considerably higher.

In this country, the government has taken several measures to keep Mad Cow disease out; the most recent being the ban on blood donations from people who spent more than six months in the United Kingdom during the height of the epidemic. There are no reported cases of BSE in the United States, nor of new variant Creutzfeldt-Jakob disease (nvCJD), the human prion disease that has been shown conclusively to originate from tainted beef7 and has so far killed roughly 100 Britons.

U.S. Efforts Falling Short?

The European experience shows that wherever people have looked seriously, BSE has been found. But surveillance efforts in the United States seem modest compared to those in the European Union (EU); the U.S. Department of Agriculture tests but a fraction of animals each year--2,700 last year out of some 3.4 million (Mad Cow editor: 34 million?) cattle slaughtered for food during the same time period.8 And while the EU requires all countries to set up mandatory surveillance centers for CJD, so far in this country, it is being done on a voluntary basis.

Since the United States seems to be BSE-free, one might wonder why surveillance is necessary. Says Pierluigi Gambetti, director of the National Prion Disease Pathology Surveillance Center at Case Western Reserve University: "It is extremely important to know whether we have a vCJD or not. Other countries would resent it if the U.S. were careless. With communication and exchange of people and travel, this could put other countries at risk."

Gambetti's surveillance agency also acts as a repository of tissue that is available to researchers. He sees it as "an invaluable resource [without which] the U.S. would be put at a tremendous disadvantage in research on these diseases as compared to EU, which has been banking tissues for years." Furthermore, while BSE has not been found here, other TSEs have. Chronic wasting disease (CWD), which infects deer and elk, is endemic in Colorado, where millions of cattle are being raised. One widely held theory on the origins of the BSE epidemic in the United Kingdom proposes that it arose from scrapie-infected sheep material that got into cattle feed. If true, this would mean that prions can cross species barriers. It's only a small leap to suppose that the CWD prion could find its way into the food chain in the United States.

Although no one is ringing alarm bells yet, whether Mad Cow disease poses a serious threat to the United States is still largely unknown. Furthermore, while the BSE epidemic appears to be under control in the United Kingdom, where most of the Mad Cow cases have occurred, it's unclear where it will end in Europe and what the impact on people will be because the incubation period for Mad Cow is about five years. Some epidemiologists estimated that the cases of nvCJD in the United Kingdom could run into the hundreds of thousands, based on the numbers of people affected so far. Recent work showing that animals with different genetic backgrounds have varying incubation times suggest that an estimate based on the reported cases, all of which had the same genetic background, may be optimistic and that what has appeared to date might only be the tip of the iceberg.9 Laura DeFrancesco can be contacted at


1. P. Brown et al., "Bovine Spongiform Encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns," Emerging Infectious Diseases, 7[1]:6-16, January-February 2001.

2. Ministry of Agriculture, Fisheries, and Food BSE weekly cumulative statistics:

3. J. Bieschke et al., "Ultra-sensitive detection of pathological prion protein aggregates by dual-color scanning for intensely fluorescent targets," Proceedings of the National Academy of Sciences (PNAS), 97:5468-73, 2000.

4. M.R. Scott et al., "Identification of a prion protein epitope modulating transmission of Bovine Spongiform Encephalopathy to transgenic mice," PNAS, 94:14279-84, 1997.

5. "The Evaluation of Tests for the Diagnosis of Transmissible Spongiform Encephalopathy in Bovines," European Commission, Directorate General XXIV, Consumer Policy and Consumer Health Protection, July 8, 1999

6. J. Safar et al., "Eight prion strains have PrPSc molecules with different conformations," Nature Medicine, 4:1157-65, 1998.

7. M.E. Bruce et al., "Transmission to mice indicate that nvCJD is caused by the BSE agent," Nature, 389:498-501, 1997.

8. BSE Surveillance, U.S. Department of Agriculture, Animal and Plant Health Inspection Service.

9. S.E. Lloyd et al., "Identification of multiple quantitative trait loci linked to prion disease incubation period in mice," PNAS, 98:6279-83, May 22, 2001. Resources Ministry of Agriculture Fisheries and Food (MAFF)'s BSE site

09 Jun 01 - CJD - Hong Kong patient has human form of Mad Cow disease, officials say

By Helen Luk, Associated Press

Nando Times--Saturday 9 June 2001

HONG KONG (June 9, 2001 08:37 a.m. EDT) - Doctors suspect that a Chinese woman is suffering from the human form of Mad Cow disease in what would be the first case of the lethal brain-wasting sickness in Hong Kong, health officials said.

After performing brain scans and tests, doctors believed the unidentified 34-year-old woman has variant Creutzfeldt-Jakob disease, the Hospital Authority said.

Her symptoms include a progressive neurological disorder, involuntary limb movements and dementia, the hospital authority said. She was admitted to a hospital last month and is in serious condition.

Director of Health Margaret Chan said the government is seeking the views of British experts.

"I must stress that it is still a suspected case at this stage and the experts are still investigating," Chan said.

Neurologist Richard Kay, who is treating the woman, said she probably contracted the disease from eating beef in Britain, but doctors were awaiting a diagnosis from the National Creutzfeldt-Jakob disease Surveillance Center in Edinburgh, Scotland.

"She can't walk and can't talk coherently, but she is still conscious," Kay said.

Chan stressed that the patient never donated blood - a possible means of spreading the disease - during her stay in Hong Kong, and noted that Hong Kong banned beef imports from Britain in 1996.

First reported in 1996, variant Creutzfeldt-Jakob disease has killed about 80 Europeans since the mid-1990s, mostly in Britain.

People are believed to contract the illness by eating meat from cattle with Bovine Spongiform Encephalopathy, or Mad Cow disease. It usually manifests itself in humans as depression, memory loss and dementia as the brain turns "spongy."

The disease can only be confirmed by an examination of the brain after death.

According to the hospital authority, the patient lived in Hong Kong from 1992 to 1997, but had lived in Britain and traveled there several times. She returned to Britain in 1997 and returned to Hong Kong for treatment early this year.

Two unrelated livestock diseases - Mad Cow and foot-and-mouth - have prompted many Asian countries to ban meat imports from European countries, including Britain, France and Germany.

09 Jun 01 - CJD - HK Mad Cow disease scare

Staff Reporter

News Interactive---Saturday 9 June 2001

20:10 (AEST) THE first suspected case of the human form of Mad Cow disease in Hong Kong was announced by Health authorities today.

After performing brain scans and tests, doctors believed a 34-year-old Chinese woman has variant Creutzfeldt-Jakob disease, the Hospital Authority said.

Her symptoms include progressive neurological disorder, involuntary limb movements and dementia, the authority said.

She was admitted to a hospital last month and is in serious condition.

Director of Health Margaret Chan said the government is seeking the views of British experts.

"I must stress that it is still a suspected case at this stage and the experts are still investigating," Chan said.

Chan said the patient had never donated blood - a possible means of spreading the disease - during her stay in Hong Kong, and that Hong Kong banned beef imports from Britain in 1996.

First reported in 1996, variant Creutzfeldt-Jakob disease has killed about 80 Europeans since the mid-1990s, mostly in Britain.

People are believed to contract the illness by eating meat from cattle with Bovine Spongiform Encephalopathy, or Mad Cow disease. It usually manifests itself in humans as depression, memory loss and dementia as the brain turns "spongy."

The disease can only be unmistakably confirmed by an examination of the brain after death.

According to the authority, the patient lived in Hong Kong from 1992 to 1997 but travelled several times to Britain, where she had previously lived. She returned to Britain in 1997 and stayed there until she flew to Hong Kong for treatment early this year.

Two unrelated livestock diseases - Mad Cow and foot-and-mouth, have prompted many Asian countries to ban meat imports from European countries, including Britain, France and Germany, devastating the farming industry there.

09 Jun 01 - CJD - HK woman may be Asia's first case of human Mad Cow disease


YAHOO--Saturday 9 June 2001

A Hong Kong woman who lived in Britain for years may be Asia's first case of the fatal brain-wasting illness linked to Mad Cow disease, her doctor and health officials said Saturday.

The 34-year-old woman is believed to have contracted variant Creutzfeldt-Jakob disease (vCJD), the human form of Mad Cow disease, said Dr Donald Lyon of the Prince of Wales Hospital.

"It's the same form of disease which has been reported in the United Kingdom," said Lyon, adding that the woman was in a serious condition.

If the woman was confirmed as having vCJD, it would likely be the first time the disease has appeared in Asia, he said.

"I believe that if this case is confirmed it would be Asia's first case. Although, of course, this patient actually became unwell in the United Kingdom and came back," he said.

He said that she likely caught the disease in Britain by eating infected beef, and stressed vCJD was not spread through personal contact.

"So this lady, if she is confirmed, most likely acquired the disease in the United Kingdom and then came back," he said.

Scientists believe vCJD is caused by eating meat infected with Bovine Spongiform Encephalopathy (BSE). Only an autopsy can confirm if a patient was stricken with the classic or variant version of the disease.

The Hong Kong woman lived in Britain between 1985 and 1992, and then from 1997 until two months ago, when she was admitted to hospital here suffering from depression and memory loss.

Her doctor rejected an earlier news report she had contracted a new strain of the disease. "It's not a new form of vCJD," Lyon said.

VCJD was first detected in 1996 in Britain, where more than 100 cases have so far been reported. All but a handful have been fatal.

Hong Kong recorded some 18 cases of classic CJD between 1996 and 2000, according to press reports, and most of the sufferers have died.

The territory banned British beef over vCJD fears in 1996.

09 Jun 01 - CJD - BSE Results Nervously Awaited


Prague Business Journel---Saturday 9 June 2001

June 8 - Food retailers, cattle breeders and meat processors today are anxiously awaiting the result of tests which could confirm the first-ever occurrence of BSE in the Czech Republic, writes MfD.

If their fears are validated, officials will begin testing all cattle older than 30 months, at a cost of Kc 280 mln per year. Farmers say the occurrence of the disease would spell disaster for all those involved in the meat industry, with beef prices expected to plummet. Also, the Czech Republic would become the first EU candidate country to confirm the presence of BSE in its livestock. (MfD 1,2, LN 1, Pravo 1)