My understanding is that the symptoms don’t show up until you’re elderly, and then you die quickly. So you may have 40 years of no symptoms, then have severe neurological issues and die within a month.
I know they tried to control the disease, but is it possible that millions of people have the disease, they just aren’t showing symptoms anytime soon?
What would an upper range for how many potential people are infected be?
Maybe
From Wiki on Variant Creutzfeldt–Jakob disease, the human version of bovine spongiform encephalopathy.
“As of 2012 about 170 cases of vCJD have been recorded in the United Kingdom, and 50 cases in the rest of the world. The disease has become less common since 2000. The typical age of onset is less than 30 years old.”
However,
"They have also proposed that individuals having contracted CJD in the early 1990s represent a distinct genetic subpopulation, with unusually short incubation periods for bovine spongiform encephalopathy (BSE). This means that there may be many more people with vCJD with longer incubation periods, which may surface many years later."
And then there is this,
"In 1997, a number of people from Kentucky developed vCJD. It was discovered that all had consumed squirrel brains…
So it is not the usual course for symptoms to show up in the elderly but it is possible and we will see.
How long it takes from exposure to being symptomatic varies. Genetics is often involved.
During the UK outbreak they found several young people that were sick with it. Genetic testing revealed a predisposition to faster development of symptoms.
So, it’s not just an “old people” thing.
And even for them they still take a while to die. “Within a month” is flat out wrong.
Not directly related to mad cow but I thought it was interesting there are people who received Human Growth Hormone donated from the pituitaries of cadavers from the 1950’s - 1980’s that were infected with CJD that developed the disease decades later.
I thought that CJD is a hereditary genetic disease like Lewy Body Dementia, Parkinson’s and Huntington’s Disease. It just happens to some people. Whereas V-CJD - with a V - is the one that is acquired from eating diseased meat. That’s a big difference. Do the figures above distinguish between the two?
People can also develop CJD because they carry a mutation of the gene that codes for the prion protein (PRNP). This occurs in only 5–10% of all CJD cases. In sporadic cases the misfolding of the prion protein is a process which is hypothesized to occur as a result of the effects of aging on cellular machinery, explaining why the disease often appears later in life.[4][15] An EU study determined that "87% of cases were sporadic, 8% genetic, 5% iatrogenic and less than 1% variant.*
RioRico, CJD affects about 1 miliion people a year, not one in a million.
I see around 225 cases of vCJD since 2012 per Wiki.
It does just happen to some people, those are the sporadic cases. Supposedly 8% are thought to be genetic.
You are correct, I am wrong. I read that 3 times after I thought I noticed the discrepancy. I even found your quote. I then cut and pasted my quote here in this reply. That’s when I noticed I has mis-read it despite looking at it specifically. Time for me to get my prions checked.
My apologies.
ETA you can see my my mistake in the first reply WHICH I THOUGHT I DIDN"T POST!
Are CJD and vCJD similar diseases with similar effects, even if they arise from different causes?
Back when Mad Cow Disease was much in the news, I read an article that described the symptoms. It is truly ghastly and gruesome how it affects the brain, and how the victims suffer during the time it takes for them to slowly die.
According to a study done in 2013, 1 in 2,000 people in the UK may be infected with abnormal prions, so about 31,000 would be vulnerable to developing vCJD. Of known cases of vCJD, 177 of 229 occurred in the UK, so assuming a similar ratio there might be 9,000 carriers in the rest of the world, for a total estimate of 40,000.
There’s a prototype blood test available now, if that’s accurate we might get better figures for carriers, but still have no idea how many of them will develop the disease.