Creutzfeldt Jakob Disease - Mad Cow Disease

By - 2006-01-10 - Article viewed 15 times. Find more articles like this in our Disability Articles category.

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Before the mad cow disease epidemic in the mid-1990s, few people had heard of Creutzfeldt-Jakob disease (CJD).

That's because this invariably fatal, degenerative brain disorder has always been considered rare. Worldwide, doctors typically diagnose one case per million people each year, most commonly in older adults.

That changed a decade ago when an unusually large number of people in Great Britain developed what appeared to be CJD. The outbreak was alarming not only because so many people died of a presumably rare disease, but also because of their relatively young ages — the youngest victim was just 19. Even more disturbing was the way they appeared to have contracted the disease. All had eaten meat from cattle suspected of having bovine spongiform encephalopathy (BSE), the medical term for mad cow disease.

Scientists eventually concluded that the new ailment — named variant CJD (vCJD) — was a form of Creutzfeldt-Jakob disease resulting from exposure to BSE. Since then, a growing number of cases of vCJD have been linked to contaminated beef in Great Britain and in other countries, including Spain, Portugal, France and Germany. The risk, however, appears to be extremely low.

Although "classic" CJD doesn't appear to be connected to mad cow disease, it's similar to vCJD in many respects. Both are thought to occur when misshapen brain proteins (prions) attack brain cells, creating sponge-like holes in brain tissue. Both cause profound mental and physical deterioration, and both appear to have long incubation periods before signs and symptoms appear.

Once a person becomes sick, the course of the disease is swift. People usually die from complications of CJD just months after developing symptoms, and from vCJD within a year or so. No treatment exists for either type of CJD, and nothing slows the progression of the disease.