Regulators Debate U.S. Response to Mad Cow Disease
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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
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
"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