Dr. Paul Brown talks about the varient CJD case of a woman currently living in Florida

April 19, 2002 All Things Considered (8:00 PM ET) - NPR  by Liane Hansen

The Centers for Disease Control and the Florida Department of Health are investigating what may be a new case of variant Creutzfeldt-Jakob disease, the human form of mad cow disease. A 22-year-old British woman now living in Florida is showing symptoms of the illness, which is thought to be caused by eating beef from contaminated cattle. Joining us is Dr. Paul Brown, senior investigator at the National Institutes of Health in Bethesda.

First, Dr. Brown, can the disease actually be confirmed at this point? Dr. PAUL BROWN (Senior Investigator, National Institutes of Health): Yes, to make a very short answer. Short of an autopsy, this kind of disease is not categorized as a definite variant CJD. But from everything I know, there is no doubt that this is a case.

HANSEN: How would this case of variant CJD have developed?

Dr. BROWN: Oh, almost certainly it began in Great Britain where the patient had lived for the first 12 to 13 years of her life, during a period of which the British population was at greatest risk, mainly the 1980s.

HANSEN: What does this case perhaps tell you then about the incubation period?

Dr. BROWN: Well, if I could tell you that the patient never revisited England after she came here, I'd be able to give you a very precise answer. But the fact is she did revisit England many times, so I can't be absolutely sure. But you would suppose that continuing residence for the first 13 years of her life would have been the period when she acquired the disease. And if that is the case, in her case, at least, the minimum incubation period would be about 10 years. Now that corresponds very nicely with what we know from other patients in the United Kingdom where it is looking more and more as though the average incubation period is somewhere between 10 and 15 years.

HANSEN: Do you think you will be seeing more cases over the years that have been in incubation up until now?

Dr. BROWN: Yes, I do. The initial estimates were up in the hundreds of thousands. And the most recent predictions are several hundred cases total. Now we're already up above 100, so I think in the years to come, particularly in the United Kingdom, there will be continuing cases and it probably will start to peter out, in my opinion anyway, in the next two or three years so that you'll find yourself on the down slope of the outbreak.

HANSEN: What's the prognosis?

Dr. BROWN: For an individual patient?

HANSEN: Mm-hmm. What's the prognosis for this woman in Florida?

Dr. BROWN: Oh, the prognosis for all forms of CJD is dreadful. It's uniformly fatal.

HANSEN: No promising treatments?

Dr. BROWN: I think not, although there's been some noises in the past year. I think in the next year or two we're going to see a number of therapeutic trials of drugs that seem to work in tissue cultures. But you have to bear in mind that very frequently you have very nice results outside the body in test tubes or tissue cultures, and then when you treat a live, whole animal or a human being, you find the situation much more complicated.

HANSEN: Do you know more now than you did 10 years ago?

Dr. BROWN: Oh, I think we know a lot more now than we did 10 years ago. We know a little bit more than we knew a year ago. And to the question as to what the nature of this infectious agent is, there is still some argument about whether or not it's due to a still-unidentified environmental pathogen, like a peculiar virus. But the longer time goes on without identifying an environmental agent in spite of everybody's best efforts, the greater the probability is that it is not really a virus at all but as one has heard, a protein within the body which changes its shape and becomes able to multiply itself, a very peculiar and novel mechanism in biology.

HANSEN: Dr. Brown, thank you very much.

Dr. BROWN: You're welcome.

HANSEN: Dr. Paul Brown is senior investigator at the National Institutes of Health in Bethesda, Maryland.

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