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Brain & Nervous System Health Center

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Regulators Debate U.S. Response to Mad Cow Disease


It is entirely possible that bacteria may be the cause of CJD, says Frank Bastian, MD, a professor of neuropathology at the University of South Alabama. Although prions appear in people with the disease, Bastian believes that prions are a product rather than a cause of the disease. There are bacteria that fit the criteria, he tells WebMD. Moreover, there is no actual proof that prions are the cause, Bastian says.

Bastian became interested in this explanation when he discovered during a brain biopsy a microorganism that reminded him of spiroplasma, he says. Unlike prions, spiroplasma contain both RNA and DNA, and in many ways fit a number of criteria for the type of agent that could cause an infectious disease such CJD, Bastian tells WebMD.

Prions may be an accurate marker, but without further research, there is a chance that these prion-based diagnostic tests may miss less virulent forms of the disease, Bastian says. "We simply have to do more research because we don't know the full spectrum, and we don't have a diagnostic test," he tells WebMD. "We could be looking at the tip of the iceberg."

But while scientists agree that there is no direct proof that prions cause CJD, the NIH still refuses to fund Bastian's research. "They just say it's all solved. It's the prion," he tells WebMD. Worst of all, he says, the NIH appears to be "throwing all their eggs into one basket when it would make more sense to spread out the research."

The NIH could not be reached for comment at the time this story was written. However, there is now overwhelming support for the prion theory, says Joseph Berger, MD, chair of neurology at the University of Kentucky.

"I don't think it's debatable," he tells WebMD. Prions are the only possible explanation for a disease that is both inherited and infectious, he says.

Cecile Sardo is Joseph Sardo's surviving wife. After Joseph died, Cecile helped found the Creutzfeldt-Jakob Disease Foundation to help other surviving family members get through the experience. For her, the important thing is that CJD finally is moving to the forefront of federal regulators' attention, she says.

"I remember the lonely feeling. You feel like an alien," she tells WebMD, referring to the lack of community support that existed when her husband died.

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