The United Kingdom has recently reported increasing cases of humans dying of Mad Cow Disease. In humans, the disease is called Creutzfeldt-Jakob disease (CJD).
Creutzfeldt-Jakob Disease (CJD) is a devastating brain disorder that manifests in rapid progression, causing distinct alterations in brain tissue and severely impacting muscle coordination, cognitive abilities, and memory. With an estimated annual incidence of approximately 350 cases in the United States, CJD remains a significant concern in the field of neurology.
Abnormal proteins called prions accumulate in the brain, causing irreversible damage and impairing the normal functioning of neurons. This disruption in neural activity results in the characteristic symptoms of CJD, including difficulty with muscle coordination, confusion, memory loss, and changes in behavior.
One of the most distressing aspects of CJD is its rapid progression. Unlike other neurodegenerative disorders, this condition swiftly advances. Complete dementia usually occurs by the sixth month, and death follows quickly.
Do any of you old football fans remember Lyle Alzado? He was a 6'5" defensive end for the Oakland Raiders in the late 1980's. He was a monster who couldn't be stopped.
Alzado took human growth hormone injections in order to get even bigger. The problem was that the hormone was taken from cows, and many of them were infected with Mad Cow Disease.
Alzado contracted CJD and was dead within a year. I remember that he came on a talk show with his brand new bride. She had to do most of the talking because he was already losing it. They explained that there was no cure for these horrible prions. Despite being newly-wed, he was a dead man walking. It was pretty sad because she clearly loved him.
The thing about prions is that you can't kill them. They are not alive - not even in the sense of a virus. They are merely a protein that twists to the right rather than to the left. (Gross oversimplification.)
Apparently "twisting to the right" is what "all the cool kids are doing," so if a prion-infected brain cells rests next to a healthy cell, the healthy cell twists to the right too. Each time brain cells multiply, the infected cells touch even more healthy cells. Soon everyone gets Reefer Madness. (Haha! From an anti-marijuana movie in my school days.)
CJD is very difficult to treat, but if you catch it earlier enough, surgeons can carve out a huge chunk of your brain. You're left severely impaired, but at least you're alive (as saliva drips down my chin.)
The hospital has to throw away the surgical instruments because no amount of heating or chemical treatment can "kill" the prion. You can cook them at 600 degrees, but it doesn't help. The next patient that falls under that particular knife dies of CJD.
The use of human growth hormone continues to this day. My precious Cisca and I were considering using it on my shortest child, but she talked me out of it. "Let them experiment on someone else's child."
Three years later, we read that every child - about 250 of them - that got human growth hormones injections died. Yup. I know. I have a very smart and wonderful wife.
Anyway, a lot of old people are dying in Britain right now from what is originally diagnosed as Alzheimers, but which is in fact CJD.
If you are exposed to CJD as an adult, when your brain cells are growing more slowly, you can sometimes live with CDJ for decades. These folks in Britain who are being incorrectly diagnosed as Alzheimer's victims are actually victims of CJD. They probably got it from eating contaminated beef. Fortunately this is not terribly common.
You will recall that, almost 20 years ago, British cows were being fed bone meal made from cows who had collapsed from Mad Cow Disease.
