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Brain disease's rarity at issue

April, 27 2002 Rocky Mountain News (Denver, CO) by Lou Kilzer
More young people may be dying of a rare and inevitably fatal disease than previously thought, according to researchers at the University of Michigan.

However, some consumer advocates say the research could point to another conclusion - that humans encountered a new type of this deadly disease class, called transmissible spongiform encephalopathy (TSE).

The most well-known TSE is "mad cow" disease, which has killed humans in Britain and Europe. In the United States, the TSE called chronic wasting disease has spread from elk and deer in Colorado and Wyoming to several other states. In a scientific abstract presented at a neurology convention in Denver, Dr. Norman Foster said two men, ages 26 and 28, came to the University of Michigan Hospital simultaneously suffering a human TSE called Creutzfeldt-Jakob disease (CJD).

CJD typically strikes about one in a million people in their 60s and 70s. It happens in those under 30 at a rate of one case per 270 million people per year. The odds of two cases happening simultaneously in the same place would approach one in 270 million squared.

Foster, a neurologist at the University of Michigan-Ann Arbor, said it is possible that the odds might be much lower than previously thought for CJD in the young, and that many cases may be misdiagnosed.

But he said the two men did not have some of the typical symptoms of CJD. Their brain waves were different and they lacked a diagnostic protein called 14-3-3 in spinal fluid. One was originally thought to have epilepsy.

It's possible that many cases diagnosed as viral encephalitis might be CJD, Foster suggested.

Dr. Ermias Belay of the Centers for Disease control said disease detectives were dispatched to Michigan because the CDC takes particular interest in CJD cases in young people.

In England, cows stricken with mad cow disease infected young people with what is called variant CJD, or vCJD. That announcement was made after 10 years of official denials that mad cow could spread to humans, and officials don't want to make a similar mistake in the United States.

The two Michigan men's brains were examined and found not to be infected with vCJD, said Foster. Also, the two had different strains of the protein - called a prion - that most scientists believe causes the disease.

Belay said the CDC investigators "did not discover any environmental cause" for CJD in the two Michigan men.

Dr. Michael Hansen of the Consumers Union, publisher of Consumer Reports, said the cases are a cause for alarm. "I think they should take this very, very seriously," Hansen said.

"There is some possibility that it (classical CJD) occurs more frequently in the young, but I would seriously doubt it," he said.

He said scientists should look hard at what the men ate or what drugs they took. He was particularly interested if either man had eaten venison, which may be infected with prions causing chronic wasting disease.

Both Michigan and CDC officials looked hard at the two men's backgrounds and found no history of venison eating. The men also lacked vCJD risk factors such as travel to Europe or receiving human growth hormone.

Foster dismisses Hansen's assertion that the two cases must be related. The fact that the prion strains were different "rules out the possibility of a common cause," he said.

Hansen said that if CWD prions have different strains, then their effect on humans could be different, as well.

TSEs can take years to develop into clinical symptoms, which include dementia and staggering.

Belay said investigation into the two Michigan cases is not complete.



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