ARKANSAS SPORTSMAN: Mystery contributes to chronic wasting disease fears

Mystery is the most frightening thing about chronic wasting disease.

Its threat to human health is unknown. There's no cure or remedy, and it seems to have come nowhere.

Chronic wasting disease is not a classical disease caused by a virus or bacterium, but by a radical protein that alters the shape of other proteins in a way that leaves holes in the brain of a victim until it causes death.

CWD is a version that affects deer, elk and moose. Mad cow disease (bovine spongiform encephalopathy) affects cattle. Scrapie affects sheep. There are at least three versions that affect humans. There's even a feline version.

Collectively, they are known as transmissible spongiform encephalopathies, or TSEs. Their history is a fascinating mixture of politics, marketing, suffering and greed.

The oldest known TSE is Fatal Familial Insomnia. It is hereditary and was for many years documented in only one Italian family. Its first known victim was a Venetian doctor in 1765. Victims first notice symptoms in their mid 50s, when one day they begin sweating profusely. Their pupils constrict to the size of pinpricks, and they lose the ability to sleep. Death from fatal familial insomnia is said to be horrific. Edgar Allen Poe mused on it in his poem, The Assignation.

Kuru is another form that exists among the Fore tribe of New Guinea and spreads through cannibalism.

Creutzfeldt-Jakob Disease is the best known of the human variants, and the one that gave the prion theory mainstream acceptance.

Dr. Stanley Prusiner, a neurologist and biochemist, won the Nobel Prize in 1997 for isolating radical proteins as the cause of CJD, a human variant that in 2005 killed Truman Ball, a well-known Little Rock realtor. CJD is sporadic. It follows no lineage, but occurs with scattered irregularity.

Like all researchers in fringe disciplines, Prusiner struggled to obtain grant money in the early days of his mission. He sensed the need for a simple, catchy buzzword to etch his concepts into public consciousness. He combined and juxtaposed "infectious protein" to create a new word, "prion." Like the word "evolution," it contemporized a timeless condition and captivated the commercial media.

To his great fortune, Prusiner's revelations coincided with an outbreak of mad cow disease in Europe. That helped open the floodgate of research money and publicity. To the consternation of his peers, most of it flowed to Prusiner.

TSE outbreaks wreak economic and political havoc. A scrapie outbreak in the 1700s nearly wrecked the English mutton industry, and by extension, the Spanish merino wool industry.

In the 1990s, the British government was so cowed by the potential collapse of its beef and dairy industries that it dithered far longer than it should have in preventing BSE-infected carcasses from entering the food supply. Some 200,000 BSE-infected cows and as many as 1.6 million symptomatic cows entered the human food chain in a six-year period.

Despite clinical proof to the contrary, Britain's government insisted that there was no human risk in eating mad cow infected beef. When it finally acknowledged the obvious, the government ordered the slaughter of 3.3 million cows. The European Union banned the import of English beef, and the total economic impact in British pounds was estimated in the billions.

The epidemic is widely believed to have been caused by cows ingesting scrapie infected protein that was included in food supplements. That is also one of the ways that chronic wasting disease spread across the United States.

Prusiner's prion theory was and still is heretical to classical epidemiology and still faces fierce, if dwindling, resistance. Nevertheless, Prusiner is the standard bearer, and the majority of the scientific community follows his lead.

The enigmatic Prusiner has proffered other controversial theories. One is that prions might be responsible for Alzheimer's Disease and Parkinson's Disease. He has also posited that prions are the same regardless of species, but that they might be radicalized by species-specific genetic triggers.

Supporting this theory is the conventional belief that some deer, for example, are genetically susceptible to CWD, but that no deer are genetically resistant to CWD.

No cure or remedy is in sight for any TSE. Even if one becomes available, it will not be practical, practicable or remotely cost-effective to administer it to a wild, free-ranging deer population on a county-wide scale, let alone a national scale.

CWD is with us for good, and if it is a human health risk, we will, regrettably, learn it the hard way.

Sports on 04/08/2018

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