BAD MOO - Calif. testing potential therapy for disease in humans

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California researchers testing potential therapy for mad cow-related disease in humans

By Paul Elias, Associated Press, 8/13/2001 22:20

SAN FRANCISCO (AP) Researchers said Monday that they will soon start human testing to see if drugs used to treat malaria and schizophrenia also fight a brain-wasting illness similar to mad cow disease.

Researchers at the University of California, San Francisco, have been given federal approval to enroll about three dozen severely ill patients in a study by the end of the year. Only patients given less than a year to live will be included.

Doctors will give them doses of the malaria drug quinacrine and the schizophrenia drug chlorpromazine. Both drugs have shown promise in mouse cells infected with prions, abnormally shaped proteins that cause variants of Creutzfeldt-Jakob disease.

The research is being led by Dr. Stanley Prusiner, who won a Nobel Prize in 1997 for discovering prions.

Two women one British, one American believed to have forms of the disease have already been given the malaria drug by the university under a Food and Drug Administration policy that allows for experimental therapies on terminally ill patients.

The family of the British patient said she showed significant improvement. The researchers were more cautious, stressing that the women aren't part of the study and that no conclusions can be made about their reaction to the drug.

''We don't know that this compound will work in people,'' said Dr. Fred Cohen, a prion researcher at UCSF.

Research rarely moves so quickly from testing on animal cells to human subjects, but this development warrants the speed, said George Carlson, a prion expert at the McLaughlin Research Institute in Bozeman, Mont.

''This is something that can be tested right away. That's the exciting part,'' Carlson said.

There are several types of the disease.

The one that has drawn the most attention recently is called new variant Creutzfeldt-Jakob disease. It appears to be contracted by eating meat tainted by mad cow disease. Another version occurs spontaneously for unknown reasons in one of every million people.

In both variations, prions cause deep lesions and sponge-like holes in brain tissue. There is no known cure.

No instances of new variant CJD have been discovered in the United States. But 105 people in Europe have been diagnosed in the last five years, and thousands of cows have been slaughtered. Europeans fear the disease, which is infectious and incubates for years without symptoms, could reach epidemic proportions unless a cure is found.

In the mouse cell experiments, the two drugs were found to inhibit the conversion of normal prions into the disease-causing, abnormal form. The results were published in Tuesday's Proceedings of the National Academy of Sciences.

UCSF researchers decided to experiment with existing drugs rather than try to create a new therapy. Because the drugs already have FDA approval, positive results from the upcoming test could quickly lead to treatment.

''This immediately puts us at the head of the queue,'' Cohen said.

One of the two women to receive the malaria drug so far is Jane Forber, a 20-year-old from Britain. Her family contacted the university after she came down with what doctors believe is new variant CJD. Relatives told British media that the drug treatment helped her walk and talk again, abilities she lost as the disease progressed.

However, the treatment seemed to have no effect on the other patient, an American with a form of Creutzfeldt-Jakob not blamed on contaminated beef. The university would not identify her.

Governments around the world have gone to extraordinary measures to try to contain the spread of mad cow disease in cattle. The U.S. Agriculture Department has barred imports of beef or live cows and sheep from countries with outbreaks.

Associated Press Writer Lauran Neergaard in Washington, D.C., contributed to this story.

On the Net:

Disease background:

http://www.cdc.gov/ncidod/diseases/cjd/cjd.htm

http://www.who.int/inf-fs/en/fact180.html

-- Anonymous, August 14, 2001


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