BRAINS! BRAINS! BRAINS!

You guys heard of Creutzfeld-Jacob disease? You know, the human equivalent of mad cow disease, where ingestion of contaminated meat products is presumed to cause a rapid and invariably fatal dementia?

Well, yesterday they did a brain-only autopsy on someone who had it! Some little old guy from Oregon. They cut the skull open and took the brain out, so as to study it and stuff. In the autopsy suite! Where I work everyday! Needless to say, I was a bit concerned that by merely entering into the same room where, just the previous day, a brain with this horrible infection was jostled about, I was exposing myself to an astonishingly horrible fate. So I had to look into it a little bit.

As you may know, CJD, as well as mad cow disease (bovine spongiform encephalopathy), chronic wasting disease in deer and elk, and a few other rare entities, is caused by an infections particle known as a prion. "Prion" stands for PRoteinaceous Infectious Particle (or something else with an "on" at the end). It is an infectious protein that is able to change the conformation of normal, benign proteins in the brain. These proteins somehow become "deadly" and are then able to attack other normal proteins and make them nasty as well. This wreaks havock in the brain, causing widespread cell death and necrosis of cells. This causes microscopic holes to form in brain tissue, giving it a spong-like appearance, hence "spongiform."

What I find so fascinating about prions is that they are not living entities. They are not like bacteria which have certain requirements in order to be able to sustain life. They are kind of like alien, inanimate objects that are somehow incredibly destructive. But all they are is a chain of amino acids! Just one big molecule. How could that wreak so much havoc?? Very weird. And very creepy.

Sooo Andromeda Strain.

The theory is that you get CJD from ingesting meat contaminated with brains from an animal that had a prion disease. CJD has some links to deer and elk. Some folks in Europe have contracted mad cow disease in the same way. But apparently, and by that I mean HOPEFULLY, this disease is not transmissable. It is highly infectious, but not communicable. One cannot get it from being around a person with CJD, or from being around the brain of a person with CJD, unless one accidentally happens to eat a piece of that brain.

That was my funny joke today. "Dr. Nixon, Its ok if I *ate* some of that brain, right?"

There are no proven reports of anyone getting it from an autopsy or anything. So I am officially in the clear. You can all sleep soundly tonight. But Creutzfeld-Jacob has been dutifully added the list of Disease Not to Get.