The Little Dog Laughed

So who wants to know about Mad Cow Disease? Probably nobody but me, but I find the issue fascinating in a horrific sort of way. Bovine Spongiform Encephalopathy (BSE) is a progressive neurodegenerative disorder and does not live in a pineapple under the sea. It attacks the cow’s central nervous system, destroying motor skills and eventually degrading autonomic nervous functions to the point of death. The disease is thought to be caused by an oddly shaped protein called a prion. Prions, while not living creatures, can be transmitted from cow to cow under certain circumstances, significantly increasing the price of a Big Mac.

BSE is a member of a family of diseases caused by prions which in humans includes Classic Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann-Straussler-Scheinker Syndrome (GSSS), Fatal Familial Insomnia (FFI) and Kuru. The differences between these diseases are largely a function of the source of the pathogen, with some, such as CJD and FFI being purely genetic in nature; that is, the source of the prion is actually a genetic mutation in the host itself, and others being the result of the introduction of the pathogen into the host from the environment, such as vCJD and Kuru. Gerstmann-Straussler-Scheinker Syndrome is actually thought to be a combination of environmental prions and a genetic abnormality which makes the host more susceptible to the neurodegenerative effects.

The prion is a truly sinister agent of destruction due to its banal nature and its nearly supernatural endurance. Consisting of nothing but a protein strand, the prion, by a very complex process which I shall not even attempt to explain, uses its chemical characteristics to modify the shape of vulnerable protein strands in the host. It is immune to heat, radiation, antibiotics, prayer and homeopathic remedies. It contains no nucleic acid and is therefore outside of the definition of “living”, but it perverts the body’s own life sustaining processes to perpetuate itself. It essentially “reproduces” by turning your brain into a useless waxy plaque riddled with holes. It does this by using itself as a template and twisting the “target” host proteins into shapes that mimic its own.

Now one might ask, what’s the big deal with the shape of a protein strand? Well, apparently it is quite a big deal; a matter of life and death big deal. Let’s take your comfortable leather easy-chair parked in front of your 52 inch HD plasma TV, for example. If you flip that chair upside down, it is still the same chair, still has the same chemical components and is still the same color and weighs the same; however, it is useless as a chair. It now pokes wires in your ass and smells like warm cheese. In the same way, the protein strands are basically the same, but can no longer perform the functions for which your body made them, resulting in the slow destruction of the structure of your brain and spinal column and the loss of everything that makes you what you are. There is even one incredibly extremely rare form of prion disease which only attacks the thalamus, which among other things regulates the process of sleep. Its victims literally die of insomnia, but not before descending into months of panic, paranoia, hallucinations and dementia. Sort of like a presidential campaign.

What really freaks me out about these prions is their Invasion-of-the-Body-Snatchers-like stealth and inevitability. You will never know you have been infected by prions until you begin manifesting symptoms of neural decay, which can show up years after initial exposure. There is no treatment of any kind available; even things like pain killers that could normally be used to ease the distress of your demise are rendered less effective by the structural damage to the brain. The diseases generally progress from loss of motor coordination to confusion, memory loss, seizures, voting Republican, blindness, paralysis and death. Of course, this is the course we all generally eventually follow, even without prion disease, but it usually happens much later and much quicker.

I guess when you get right down to it, these brain-wasting diseases are so unnerving (no pun intended) because they strike at the root of our humanity. You can die of a stroke or cancer or the flu and still be who you always were right to the end, but prions deform your very essence by depriving you of yourself. Memory, judgment, personality and moral perspective are slowly and inexorably disassembled by the accelerating cascade failure of protein conversion and all that ultimately remains prior to death is a sad, dysfunctional parody of a human. Whether the prions have succeeded in erasing the person’s existence, or have simply moved it along its path, is impossible to say.