Published November 22, 2004
BEAUMONT, Texas – The family of a Beaumont, Texas, woman is waiting for test results to find out if she died from a form of an affliction connected to mad cow disease.
Burnell Baize, 71, died Oct. 16 of the rare Creutzfeldt-Jakob disease (search), which eats holes in the brain and always causes death, the Beaumont Enterprise reported Sunday.
There are two forms of the disease.
One type is called variant Creutzfeldt-Jakob disease and is linked to mad cow disease (search). It can be contracted by humans if they eat infected beef or nerve tissue, and possibly through blood transfusions.
The more common type of Creutzfeldt-Jakob disease, known as classic CJD, is responsible for about one in 10,000 U.S. deaths each year, and its cause is unknown 85 percent of the time.
Baize's family is wondering if she ate infected beef.
In the United States, there has been only one known case of variant CJD — a Florida woman who died in June after eating contaminated beef more than a decade ago in England.
The only confirmed U.S. case of mad cow disease was found last December in Washington state.
But on Thursday, Agriculture Department officials said a second case of mad-cow disease might have turned up.
Baize's family is worried.
"This is a scary, scary malady," said her son Gene Barnes, 53.
Baize began to suffer from dementia during the late summer, he said. In the last week of her life, she was in a coma.
Her brain was taken to the National Prion Disease Pathology Surveillance Center (search) in Ohio, where an autopsy will determine if she died of classic or variant CJD. The results are expected in about two weeks.
This year so far, two Texans have died of classic CJD, according to the Texas Department of State Health Services.
In the last eight years, there have been between one and 10 people to die of the illness each year, said spokesman Doug McBride.
Variant Creutzfeldt-Jakob disease, the human form of mad cow disease, so far has killed 100 people in Britain and elsewhere.
Both forms of CJD are believed to involve the unexplained mutation of proteins in the brain called prions.
Mad cow disease — known also as bovine spongiform encephalopathy (search), or BSE — eats holes in the brains of cattle. It sprang up in Britain in 1986 and spread through countries in Europe and Asia, prompting massive destruction of herds and decimating the European beef industry.