Mad Cow in US?

A college friend reports that one of his childhood friends is dying of Creutzfeld-Jakob Disease in Michigan. As I understand it, there are (at least) two forms of CJD - one form has been around for a long time, but another, called variant CJD, is more recent and has been linked to Bovine Spongiform Encephalopathy (BSE) or Mad Cow Disease.

So my simple question is: Are there any documented cases of variant CJD in humans in the US, and if so, where are they documented?

According to the CDC:

That page was last reviewd on 1/4/01.

My wife get’s Mad Cow Disease once a month. :smiley:

I’m caring for a man in my home who’s been diagnosed with Alzheimer’s, but I’d bet any amount of money that he really has vCJD. The neurologist disagrees with me (because my guy doesn’t have the classic jerking/ clonus) and he’s the doc, and either disease is absolutely horrible and incurable, so it makes no difference to my patient in the long run.

My guy is only 65: unusually young to have Alzheimer’s, much less die from it. Less than a year ago, he was still totally independent: able to work, drive, exercise, give himself insulin, etc. I’ve had him since February; in that time he’s become almost completely unable to talk, he can’t add or subtract, operate the TV remote control’s four buttons, read or write, or even find his bedroom or the bathroom most of the time. His behavior, when he’s not staring into space, is bizarre and often hostile. He thinks it’s 1992.

His physical decline is just as (or more) dramatic. Much of the time he can’t walk; he can almost never get out of a chair by himself or remain standing: his balance is completely gone. Often he can’t remain in a sitting position because his balance is so poor. He’s utterly incontinent of urine and feces. He’s begun to have some difficulty swallowing and feeding himself. He has severe hand tremors and a masklike facial expression (Parkinson’s-like).

From what I understand from my internet searches, the new variant CJD doesn’t cause the characteristic jerking of “regular” CJD or BSE. I’ve also read that 20% of people in nursing homes with diagnoses of Alzheimer’s really have CJD. (This was determined by doing autopsies/ brain biopsies.) If Qadgop calls me on this one, which he probably will because I’m just a stupid RN :stuck_out_tongue: I’ll be happy to find the cites.

I also had a home health patient last year who was diagnosed with CJD (not the new variant, though she had no jerking and also had the Parkinson’s-like symptoms my present patient has). She died within a few months of diagnosis; like my guy, she went from complete dependence to complete helplessness in a matter of months. She was also young (late 60’s). Her original dx was Alzheimer’s, which was later changed to Parkinson’s, then CJD.

She was raised on a cattle ranch. My 65 year old guy has spent many years on his daughter’s cattle ranch. Plus, we live in a city where the population of cattle is much larger than that of people.

Coincidence? I think not!

There have been no confirmed cases of vCJD or BSE (Mad cow disease) in the US. Or, I believe, anywhere in North America. It’s hard to diagnose humans because the disease is so similar to Alzheimer’s. And because it’s diagnosed by slicing the brain into pieces. But it’s a lot easier to check cows, and nothing’s been seen yet.

There’s a lot of hype about Mad Cow Disease. I have a hunch that rumors are being spread by animal rights extremists, but I’ll leave it at a hunch. If someone is demonstrating symptoms of vCJD, you can safely bet that it’s something else.

Besides Alzheimer’s Disease, there are several neurodegenerative disease that lead to dementia. The hallmark of these “other” diseases is that they affect motor function, with varying degrees of rigidity, spasticity, tremor, myoclonus, apraxia, etc. Examples include corticobasal degeneration, Pick’s Disease, Lewy-body Disease, various forms of leukencephalopathy and dys- demyelinating diseases, Parkinsonian "variants, such as Progressive Supranuclear Palsy, and, yes CJD and “variant” CJD…

The diseases that are of concern to this post are those presumably associated with prions…prionencephalopathy if you will.

I don’t care if the CDC says that there have been no confirmed cases of Mad Cow Disease in the US. I have had the “pleasure” of caring for at least three patients in the past 10 years who died from a rapidly progressive myoclonic dementia that was pathologically identical to CJD. The problem is that none of the patients had the classic risk factors: corneal transplants, treatment with cadaveric pituitary extracts, or invasive (needle) encephalography. One of the patients was a rancher from norther california, and all had had dental work (who hasn’t?).

Mad Cow Disease in the USA? You bet.

I live in the UK where we have had an incredibly large number of BSE cases compared to rest of the world. We monitor the non-old population in high detail to look for vCJD. We have had roughly 100 measured cases. There is no reason to suggest so far that the old are much more vunerable so you could estimate that the cases of vCJD is Britain so far is only a few hundred at max. In America there has been no widespread BSE problem what so ever, there have also been no documented vCJD cases and all non-old people going crazy must have had close scrutiny for vCJD. They have still had no recorded cases which would indicate that the number of old people with it must be small or zero aswell. Perhaps there are a few cases hidden in the oldish generation but because it has not been recorded in the young population there is nothing pointing to more than a tiny amount of cases at most. Of course in the future its likely that there will be North American victims but hopefully we will have a cure by then.

Dale, I’d like to counter your link from the American Cattle Feeder’s Association with this one which is a little more objective.

I don’t think so. My hunch is that the US cattle industry is enormous and well-funded. It would go to some lengths to squelch any talk of danger, whether the rumors are true or not. This is Amarillo, TX, after all: the place where the cattle barons sued Oprah Winfrey because it was against the law to say anything remotely negative about a food product.

But I’m not anti-cattle industry. I’m not a vegetarian. I’m just a nurse who is convinced that this guy I’m caring for does not have Alzheimer’s.

CJD is not reportable to the CDC, so we have absolutely no way of knowing how many people have it. It is not at all safe to bet that symptoms of CJD are caused by “something else”.


Similar, but not too similar. Once symptoms appear, CJD usually kills within one year and the physical symptoms are swiftly incapacitating. Unlike Alzheimer’s, CJD is infective: we already know it can be spread by cornea transplants, pituitary extracts like Human Growth Hormone, and certain surgical procedures, as Neurodoc said. We don’t know if we don’t know all the ways it’s spread. We don’t even clearly understand how prions work.

If you have brain surgery with instruments that were previously used on a person with CJD, you can contract CJD, even though the instruments were sterilized. How do prions escape destruction by sterilization? We have no clue, but since CJD apparently has a very long incubation period, how certain can you be that your neurosurgeon’s instruments have never touched a prion?

CJD can be differentiated from Alzheimer’s with a brain biopsy while the patient is alive, but generally this isn’t in the patient’s best interest. We could find out the prevalence of CJD by looking for it on autopsy.

If the cause of 80-85% of CJD cases is unknown, in my opinion, this is a cause for concern. I’d also like to know: what is the real incidence of CJD? Is this figure increasing?

If you’re not familiar with the “mad cowboy” site you might find it interesting. This is a 4th generation cattle farmer who gave up the homestead and became a vegetarian. His book and site are extremely well researched. Here’s a link to the vCJD page.

His book also mentions that the incubation period for symptoms to appear in humans is 10-30 years, so scores of people could already have it and we won’t know for some time.

The book is taking me a long time to get through; I keep having to put it down and do something to take my mind off the depressing and scary facts and images.

C.A. has touched on this question: