The Insidious Prion Hits First As A Sleep Disorder

By James Donahue

Ever since researcher Stanley Prusiner proved the existence of the prion and linked it to various spongiform encephalopathies in 1997, medical science has been carefully studying this strange microbial assailant and its effects on the animal kingdom.

While this bug shows no sign of being a living micro-organism, is now blamed for Creutzfeldt-Jacob disease, its new variant and kuru in humans, Mad Cow Disease in cattle, scrapie in sheep and wasting disease in deer, elk and other wild creatures of that ilk. It has been our contention that all of these various diseases are the same thing . . a slow and fatal assault on the central nervous system for which there is no cure..

Thanks to the astute observation of writer D.T. Max, whose book Portobello was recently reviewed in The Observer, we now have an early symptom of this disease . . . at least for humans. Studying the work of Carleton Gajdusek, who lived with the natives of New Guinea and linked the disease kuru with the gruesome practice by the natives of eating the flesh of dead relatives, Max noted that the disease triggers extreme insomnia among its victims.

Even though he was awarded the 1976 Nobel Prize for medicine, Gajdusek's work was largely overlooked after it was revealed through his personal diaries that Gajdusek also was a paedophile who also recorded his sexual exploits with the native children. Thus after receiving highest honors in science, the man was charged by US authorities for child molestation and sentenced to 19 months in jail.

Yet it was Gajdusek who concluded that the custom of eating the flesh of dead relatives, as a matter of respect, was permitting a then unknown agent to pass from the dead to the living. The symptoms of kuru were described as a fatal form of extreme insomnia, known among the New Guinea natives as The Curse.

Now thanks to the work of Prusiner and others who followed him, we know that this unknown agent is the prion, a misfolded protein whose presence triggers a biochemical avalanche in the brain.

The effects are horrible in humans, and we must assume they are just as terrifying for cattle, sheep, deer and any of the other animals that become infected. Victims begin to manufacture more prions in their own bodies, which attack the sleep centers of the brain. As the disease advances, victims slowly proceed through what the Observer writer identified as a "sweaty, hollow-eyed demented death."

The emergence of a rash of Mad Cow Disease cases in the UK in the 1990s, and an outbreak of several human cases of Creutzfeldt-Jacob variant disease, hit the beef market and still is having its effect around the world. There is a general belief that the prion can be passed from one species to another simply by eating infected meat.

Another problem appears to be that cooking the meat does not kill the prion. Some scientists insist that the prion is not a living organism, yet the fact that it can multiply once it enters the body suggests otherwise. Also the disease is sometimes has a very slow onset lasting as long as 30 years, while at other times it might occur within months of contact. To date, science knows very little about this strange assailant.

There are no known cures for this disease, or methods of determining if the prion exists in the meat sent to market. Thus all meat eaters stand at risk today of death from a prion assault.