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flatfish
11-12-2006, 12:57 PM
##################### Bovine Spongiform Encephalopathy #####################

The Brain Killer: Part Three

Nov 10, 2006 04:42 PM CST

Bob Segall/13 Investigates

Researchers have made progress in understanding Creutzfeldt-Jakob Disease, but there is still debate about what causes it. Do victims catch the disease or does it spontaneously appear? Answering that question is essential to finding a cure.

Inside laboratories around the country, doctors and researchers are trying to learn more about CJD. What they've discovered so far is the disease is linked to proteins in the brain. Those proteins begin folding the wrong way and then start attacking healthy brain tissue.

"These are actually holes where the brain cells essentially have died away," said Dr. James Mastriani, describing the effects of the disease from his lab at the University of Chicago.

The question is, why? What causes the protein to mutate and go on the attack?

"There's no obvious source," Mastriani said. "That means it happens normally in the brain."

Mastriani and many other CJD specialists believe the whole process is a natural one - that a diseased protein (called a prion) in the brain simply occurs on its own.

"We don't think it came from anywhere," he explained. "We think it simply happens spontaneously in the brain. 99.9 percent of the scientific community feels that it's really related to this misfolded protein."

Dr. Laura Manuelidis disagrees. "Truth is not defined by popular vote," she said.

For nearly 30 years, Manuelidis has studied CJD at Yale Medical School. She says misfolded proteins are clearly present in the brains of CJD victims, but she also believes those problematic proteins don't simply appear by themselves.

Manuelidis enthusiastically insists something is missing: some still-unknown ingredient that triggers prions to become infectious.

"Nobody knows what the nature of the infectious agent itself is," Manuelidis said. "And I think there's a great deal of evidence which points to a virus, a small virus."

Manuelidis hasn't figured out what that virus is, but she continues to search. "There's a world out there of viruses we don't know anything about," she said. "It's still a mystery. I would like to solve the problem before I die."

For now, there's no treatment for CJD and there is no cure. Most CJD victims are in their 60s and 70s, but some are much younger. While the disease is considered rare, it is impossible to tell who is going to get it next.

"I've met many families that are in the same situation as I," said Denice Hinojosa, whose brother died of CJD in 2004 at the age of 32. "I never thought CJD would be a part of my vocabulary - nobody does."

Faces of CJD
See personal stories of CJD victims. (Click on the name to watch the video.)

Jack Bennett
Zane Mingus
Jean Burgette

CJD Information, Resources and Support Groups:

CJD Aware!

CJD Foundation

CJD Voice

National Prion Disease Pathology Surveillance Center

U.S. Centers for Disease Control and Prevention

CJD or Alzheimer's? - CJD Aware! is an organization founded by families of CJD victims. The group provides this brochure to help educate about the similarities and differences between CJD and Alzheimer's Disease.

See a map showing which states report CJD

To donate towards CJD research:

Donation and Research list

Learn about the CJD memorial quilt:

CJD Memorial Quilt - The daughter of one of the victims started this quilt to honor her mother and other CJD victims.

Back to Brain Killer main page



http://www.wthr.com/Global/story.asp?S=5663484&nav=menu188_2



The Brain Killer


Nov 10, 2006 04:45 PM CST

Brain killer 2







Jack Bennett



Zane Mingus



Jean Burgette






Part One - It's a disease that devours the human brain. There is no treatment. There is no cure. 13 Investigates shows you how the deadly disease is striking right here in Indiana, and no one knows where it's coming from.

Part Two - Government statistics claim CJD affects one in a million Americans. 13 Investigates reporter Bob Segall shows why those numbers may be seriously flawed - especially in Indiana - and why Alzheimer's patients should pay special attention to this disease.

Part Three - Researchers have made progress in understanding Creutzfeldt-Jakob Disease, but there is still debate about what causes it. Do victims catch the disease or does it spontaneously appear? Answering that question is essential to finding a cure.

Faces of CJD
See personal stories of CJD victims. (Click on the name to watch the video.)

Jack Bennett
Zane Mingus
Jean Burgette

CJD Information, Resources and Support Groups:

CJD Aware!

CJD Foundation

CJD Voice

National Prion Disease Pathology Surveillance Center

U.S. Centers for Disease Control and Prevention

CJD or Alzheimer's? - CJD Aware! is an organization founded by families of CJD victims. The group provides this brochure to help educate about the similarities and differences between CJD and Alzheimer's Disease.

See a map showing which states report CJD

To donate towards CJD research:

Donation and Research list

Learn more about the CJD Memorial Quilt:

CJD Memorial Quilt - The daughter of one of the victims started this quilt to honor her mother and other CJD victims.

http://www.wthr.com/global/Story.asp?s=5651761


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