It seems to me that thre's a striking lack of curiosity in the media about Americans who have Creutzfeldt-Jakob Disease (CJD), even after the recent discovery of mad cow disease in this country, and even though Maxine Postal, a legislator in Suffolk County, Long Island -- not far from New York City, the media capital of the world -- left office because she had CJD just before the diseased cow was discovered, and subsequently died on New Year's Day.

I realize, of course, that what's called "classic CJD" isn't exactly what you have if mad-cow disease crosses species and gets to you -- what you have then, according to experts, is variant CJD (vCJD). This AP story and this Seattle Times story explain the differences. Here's AP:

The classic version typically strikes people in their 60s and 70s; one-half the patients die by age 68, he said. The variant kind is a young person's disease, with one-half its victims dying by age 28.

Symptoms also tend to play out differently in the two kinds of disease. In variant CJD, the early symptoms tend to be mostly psychiatric problems, like depression, withdrawal and anxiety and patients often complain of pain or numbness or a pins-and-needles sensation. In classic CJD, early symptoms are usually neurologic, like trouble standing or walking properly, involuntary jerking and speech abnormalities, along with an erosion of mental abilities.

Eventually, patients become unable to move or speak and they typically enter a coma before death.

One other difference is classic CJD kills much faster. The variant form takes about 14 months from the first symptoms to kill one-half its victims. The classic form takes just six months and kills about 90 per cent within a year.

Remember that last sentence: The classic form takes just six months and kills about 90 per cent within a year. Now read this, from a story in the Amityville Record on Christmas Day, just as Maxine Postal was leaving office (and a week before she died):

Postal, who was reelected in November, has been battling a undiagnosed illness for almost two years, and had undergone tests to rule out everything from Parkinson’s Disease, which had afflicted her parents, to Lou Gehrig’s disease. Two weeks ago, her doctors determined she had Creutzfeldt-Jakob disease, a rare fatal brain disorder which causes a rapid, progressive dementia and associated neuromuscular disturbances. (emphasis mine)

So Postal reportedly lived for two years with a disease that kills half of its victims in six months and 90% in a year. Curious?

But surely it's unlikely that we have undetected cases of beef-borne (v)CJD in the U.S., right? After all, as The Seattle Times notes,

The United States has a surveillance system designed to pinpoint suspicious cases of CJD so that alarms would be raised if the variant form were to enter the country. Only one variant case has been found in the United States: a woman who lives in Florida but grew up in England during the peak of mad-cow disease there.

Well, yes. But ...

But the system isn't foolproof. Half the states — including Washington — do not require doctors to report cases of CJD. In fact, in Washington, only about one-third of the 102 patients who died from CJD in the past 25 years were autopsied.

That's a big concern, said Shu Chen, a protein expert at the National Prion Disease Pathology Surveillance Center in Cleveland, because an autopsy is the only way to definitively diagnose CJD or to distinguish between the classic and the variant form. "Without an autopsy, you don't know for sure; it could be a strange-looking case of early-onset Alzheimer's. Or an Alzheimer's case could really be CJD," he said.

Washington [State] is working on ramping up its surveillance, said Jo Hofmann, a state epidemiologist for communicable diseases. The state Department of Health will soon send a letter to neurologists, neuropathologists, medical examiners and local health departments requesting that doctors report any suspected cases of CJD to the state and encourage families to have the bodies autopsied. The prion-surveillance center, funded by the CDC, will pay for the autopsies.

So there you are: The reporting system is full of holes, holes that are only now being (partially) plugged -- and, apparently, not as the result of a national policy that requires (or even offers) a government-funded autopsy in the case of any death that might be CJD or vCJD.

(Incidentally, if you want to know about that Florida woman whose vCJD was caused by British beef, CNN has her story here. It's heartbreaking.)

I'm not a doctor, but it seems to me that we don't know nearly enough about these diseases -- a conclusion I come to when I read in the AP story that

The cause of classic CJD is unknown 85 per cent of the time. The remaining cases are either caused by inheriting a genetic mutation or acquired through medical procedures that used contaminated equipment or tissues.

And is this (also from the AP story) worrisome?

U.S. officials said American beef is safe despite the latest mad cow diagnosis, noting the rogue proteins called prions that cause both mad cow disease and variant CJD aren't found in cow muscle tissue, the source of roasts, steaks and other beef cuts. (Prions are found in the brain, spinal cord and small intestine and on Tuesday the U.S. government announced new rules for keeping these parts out of the food supply).

But last year, California scientists reported mice exposed to prions did accumulate them in muscle. They called for a major effort to look for prions in muscle of infected livestock.

Dr. Ermias Belay of the CDC and Dr. Richard Johnson of the Johns Hopkins School of Medicine say it isn't worrisome:

Belay cautioned the lab result doesn't necessarily apply to cattle. What's more, he said, other researchers have found muscle tissue from infected cows couldn't spread disease when put into other animals.

"That's pretty reassuring," Johnson said.

Still, I think we need to take this a hell of a lot more seriously than we're taking it now.