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You have heard lots about emerging viral diseases like Ebola, COVID-19 and just recently monkeypox. I bet you have heard too about emerging bacterial nasties that are so resistant to antibiotics, like MRSA and multi-drug resistant gram negatives. Have you heard about emerging prion diseases?

A deer-hunting friend of mine stopped me the other day. He had read my essay, “Emerging Infectious Diseases and Climate Change.” He wanted to know about the thing in white-tailed deer called chronic wasting disease (CWD), concerned that it might be a threat to him. He was even wondering if he should give up hunting. I told him that it was great he knew enough to ask, but that he really did not need to be worried. At least not here in New York state. Not yet. Nor should he be worried about prion diseases in general. But yes, CWD is slowly emerging, although maybe not because of climate change.

Then he asked another question: what in the heck are prions anyway? I told him it was complicated, and I would have to write an essay. Here it is.

In medical school in 1970, I took a job performing autopsies. It wasn’t that it was pleasant work or that I had a morbid streak or that it paid a lot of money. Actually, I did it to learn more about the body and how diseases impact it. This story is about one of my autopsies.

A 50-year-old previously perfectly healthy woman died with relentlessly progressive brain deterioration over a period of a year. No neurologist had been able to diagnose it. She came to autopsy. The brain looked surprisingly normal until I looked at the microscopic sections. Then I saw that the tissue was full of small holes, and many brain cells were just plain gone. That made the diagnosis: it was Creutzfeld-Jakob (C-J) disease, a rare entity that kills by creating a “spongiform encephalopathy.”

Not much was known about the entity at the time. It was rare and hadn’t stimulated much investigation. But some 15 years later it crashed into the news and into research laboratories when “mad cow disease,” more formally called bovine spongiform encephalopathy (BSE), was found to be killing some cows in Great Britain, and was also suspected of also killing several people. Horrifyingly, research did indeed show that the agent could be transferred from infected cows to those who ate the wrong steaks. And the disease with which people died was identical to C-J.

But I am ahead of myself. In the 1960s, a physician named Danial Gajdusek was researching another human spongiform encephalopathy called kuru. Kuru had been known to be transmitted from someone who died of it to someone who ate that person. Yes, it was a disease of cannibals. It was prevalent in the South Fore tribe in New Guinea. They practiced the ritual eating of their dead relatives as a way of preserving tribal memory. Grandma’s brain was thought to be the best part. Ironic, no?

Anyway, Gadjusek took some of grandma’s infected brain tissue, ground it up and injected it into a chimpanzee. After a long incubation time, the chimp developed kuru and died. Brain tissue from that chimp was then injected into another chimp and — same thing. So Gajdusek proved that kuru was caused by an infectious agent. Furthermore, he showed that the agent was so small it could pass through the finest filters, filters able to trap infectious agents like bacteria or funguses or parasites. So the agent was initially thought to be a tiny virus. It was labeled a “slow virus” because the disease it caused took a long time to develop. Further research has shown that the agent is not a virus at all. It is a prion, an abnormal protein. More about that shortly.

Here’s the thing about the autopsy: while doing it, I slipped and cut myself with a scalpel. Which meant my cut was contaminated with the lady’s blood. Studying what was known about C-J at the time, and reading Gajdusek’s work on the closely related disease kuru, I realized I might have injected myself with a slow viral agent. Which meant my brain ... .

I agonized with worry every time I forgot somebody’s name, figuring damn, here it comes. Well, I didn’t get it. I never was good at names anyway … which is getting worse … and I can’t remember some other things too. Hmm. Probably just age.

They are abnormal proteins. Proteins are large molecules made of amino acids in a kind of string. That string naturally folds back and forth upon itself in a very specific way because of mini electric forces between atoms of the amino acids. The folding pattern is vital to what they do, and proteins are both the fundamental structural elements of cells and critical actors of cell function.

Think of a glove for the right hand. Turn it inside out, it becomes a left handed glove. Like a glove, a protein can sometimes be turned inside out. That probably happens spontaneously from time to time in a cell. When it does, the protein won’t work, but usually it’s no big deal. Turn a certain type of protein, called a PrP, inside out though, it won’t work, but, for some reason not yet understood, it will develop the ability to replicate itself. That is what a prion is, a mis-folded PrP which has become self-replicating.

A lot of PrP exists in the brains of mammals. When a PrP flips to become a prion and starts to self-replicate, clumps of the stuff slowly accumulate in pockets. That puts pressure on neurons, the brain cells, to the point that they vanish. The brain tissue then looks like a sponge under the microscope, with way too few neurons. Of course the victim sinks into progressive brain dysfunction and ultimately brain death.

Several spongiform encephalopathies are known. Most occur in non-human mammals: BSE in cattle, scrapie in sheep and goats, feline spongiform encephalopathy in cats, and CWD in members of the deer family. A few occur in humans: C-J disease, kuru, Gerstmann-Straussler-Scheinker syndrome, and a fatal familial insomnia. All of these spongiform encephalopathies create the clinical picture of brain deterioration in whatever animal. There is no treatment for any of them.

The diseases can occur out of the blue, called sporadic cases. Sometimes they are passed down in families. Mostly, prion diseases can’t be spread from one species to another. But once in a while, as with mad cow disease, one can.

Also called zombie deer disease, CWD was first described in 1965 in Colorado. Now found in 29 states, it seems to be slowly spreading, probably through bodily fluid exchange among deer. In 2005, a single deer died with it in New York’s southern tier. The New York State Department of Environmental Conservation does quite a bit of testing of our deer population looking for it. No other cases have yet been reported. But in 2021, a case was found 5 miles from our border in Pennsylvania. That is what prompted my friend’s question.

In contrast to mad cow disease, CWD has not been found to infect humans. However, monkeys fed infected deer meat can be infected. The Center for Disease Control and Prevention is therefore not perfectly reassuring. They say: “there is no strong evidence for the occurrence of CWD in people, and it is not known if people can get infected with CWD prions.”

So to my hunter friend, I say: be aware, we may see CWD in New York soon. And for heaven sake (and for my sake should you choose to give me some meat), don’t shoot a deer that is staggering around looking confused!

Our bodies are so beautifully equipped to protect us from the vast variety of diseases out there, it is just amazing. If we live wisely and well, we can expect a good long life. Then we can enjoy the beauty of super moons, like the one last week (which was called the “buck moon!), can be utterly dumbfounded by the dazzling pictures of the universe from the Webb telescope, and can watch in awe as our grandchildren grow. Yes, there are the unexpected lightning strikes, like the case of C-J disease I may have been spared. But they are rare. We shouldn’t live in fear of them. What we should do is treat our bodies most kindly, like the great gifts they are. Same for our environment.

No, my hunter friend, don’t worry about CWD. Concentrate on living wisely and well. Concentrate on loving neighbors. Concentrate on leaving the world a better place than when you entered it.

Richard Leach, M.D., is a retired internist, infectious disease consultant and travel and tropical medicine specialist. He practiced in Glens Falls for 35 years, also serving as Glens Falls Hospital’s infection control officer and hospital epidemiologist.

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