Scientists, Doctors Reveal
New Findings About "Mad Cow"

LONDON — The London Sunday Telegraph reports that a baby has been born with the human form of so-called "mad cow" disease.

The newspaper reports this month that four doctors who examined the 11 month-old girl, whose mother died of new variant Creutzfeldt-Jakob disease (vCJD) earlier this year, believe the baby also has the symptoms.

They say they believe the baby contracted the disease in the womb.

The baby, who was seven months old when her mother died, has brain damage and suffers from fits and convulsions.

A final analysis on whether the child has vCJD could only be made with a post mortem examination if the child dies. If the child's disease is confirmed, it would be the first known case of vCJD being transmitted from a mother to her baby.

Bovine spongiform encephalopathy, or "mad cow" disease, first broke out in British herds in 1986 and peaked in 1992. In 1996, scientists identified vCJD and suggested it could be contracted by eating contaminated beef.

In a related story, Reuters news service is reporting that Australia is expected to ban thousands of potential donors from giving blood if they have had extended visits to Britain, after research showed that the human form of BSE can be transmitted through blood transfusions.

The Australian Red Cross said the ban would likely affect 25,000 to 30,000 of the agency's established donors. About four percent of Australia's 19 million people are regular blood donors.

The ban, aimed at donors who have lived in Britain for more than six months, is similar to bans imposed recently in the United States, New Zealand and Canada.

It follows news this month of Scottish research that showed mad cow disease and its human equivalent can be transmitted through blood transfusions.

Australian health officials are debating whether the ban should apply only to those who ate British beef products during their visit, or to anyone who had spent at least six months in Britain between 1980 and 1996.

The ban could eventually be lifted if research shows the disease cannot be transmitted through blood transfusions or a test is found to screen for vCJD.

Reporting in a recent issue of British medical journal The Lancet, scientists at the Institute for Animal Health in Edinburgh said they fed a breed of British sheep a sample of brain from cattle infected with BSE.

Sheep were chosen as they harbor the infectious agent in tissues other than the brain and spinal cord, as do humans.

Blood was taken from 19 infected sheep before any symptoms were apparent, and was transfused into healthy sheep imported from New Zealand.

After 610 days, one of the transfused sheep began to show signs of the disease. All the others are currently healthy though most are at an earlier stage after transfusion than the affected sheep.

While acknowledging that their study still had several years to run and that so far only one animal had become sick, the scientists said this early finding was "sufficiently important" to announce now.

"This report suggests that blood donated by vCJD-infected human beings may represent a risk of spread of vCJD infection among the population of the UK," they said.

It should now be possible to identify which cells are infected and develop a diagnostic test for vCJD based on a blood sample, they added.

That would also help to test the effectiveness of a two year-old British policy which seeks to eliminate the likeliest source of vCJD transmission by stripping out white blood cells from blood donations.

As its name suggests, vCJD is a form of Creutzfeldt-Jakob disease, for which there is no evidence of a risk of transmission by blood transfusion. However, vCJD "has a different pathogenesis (from CJD) and could represent

different risks," the scientists cautioned.

Meanwhile, researchers from France's National Centre of Scientific Research reported that vCJD may be promoted by normal prions as well as abnormal ones.

Three types of non-mutant prions play a role in a complex "cascade" of enzyme signals that govern brain-cell functions, they said in work published in the U.S. weekly Science.

This discovery could ultimately throw up possible treatments for vCJD by designing drugs that block or alter the signaling process.

Britain is the seat of the BSE epidemic and the vCJD crisis that has followed in its wake. As of August, the total number of identified British cases with vCJD stood at 79.

People with vCJD suffer jerky movements, forgetfulness, dementia and finally death. There is no known cure or vaccine.