Posted on 10/18/2005 10:28:47 PM PDT by neverdem
Associated Press
BOISE, Idaho — From the moment Joan Kingsford first saw her husband stagger in his welding shop, she wanted two things: for him to recover and to know what made him sick.
She got neither. Alvin Kingsford, 72, died recently of suspected sporadic Creutzfeldt-Jakob disease (CJD), a fatal brain-wasting illness. The disease can be conclusively diagnosed only with an autopsy, which did not take place.
State and federal health officials are trying to get to the bottom of nine reported cases of suspected sporadic CJD in Idaho this year. Sporadic, or naturally occurring, CJD differs from so-called variant CJD, which is caused by eating mad-cow-tainted beef and has killed at least 180 people in the United Kingdom and continental Europe since the 1990s.
"One thing is very clear in Idaho: The number seems to be higher than the number reported in previous years," said Dr. Ermias Belay, a CJD expert with the federal Centers for Disease Control and Prevention. "So far, the investigations have not found any evidence of any exposure that might be common among the cases."
Normally, sporadic CJD strikes about one person in a million each year, with an average of 300 cases per year in the United States, with just more than one case a year in Idaho. Over the past two decades, the most cases reported in Idaho in a single year has been three.
Until this year.
Of the nine suspected cases reported so far in 2005, three tested positive for an infectious disease of the nervous system, though more tests are pending to determine whether the fatal illness was in fact sporadic CJD. Four apparent victims were buried without autopsies. Two suspected cases tested negative.
Still, federal and state health officials are stopping short of calling the Idaho cases a "cluster," waiting for final test results from the victims who got autopsies.
The best tool of investigators to pin down the diagnosis — the autopsy — is sometimes hard to get, said Tom Shanahan of the Idaho Department of Health and Welfare.
Pathologists are often reluctant to perform the procedures, the cost of an autopsy can be high and some families are reluctant to give their consent, officials say.
Joan Kingsford wanted an autopsy done on her husband, but no mortician in the area would agree to handle his body after his brain cavity had been opened. They feared they would catch the rare disease, Kingsford said.
CJD is transmitted through a malformed prion found primarily in the brain and spinal fluid of those infected, Belay said. Standard sterilization procedures don't eliminate the risk of infection; instead, equipment must be soaked in a chemical solution for more than an hour and then heated, according to the World Health Organization.
Spiroplasma as a candidate agent for the transmissible spongiform encephalopathies.
Spiroplasma-like inclusions in Creutzfeldt-Jakob disease.
The last link appears to be the first paper of F.O. Bastian associating Spiroplasma with spongiform encephalopathy. The "failure to detect spiroplasmas by serology and culture points to the absence of spiroplasmal involvement in spongiform encephalopathies," as claimed by J.M. Bove in Spiroplasmas: infectious agents of plants, arthropods and vertebrates is not proof of absence.
FReepmail me if you want on or off my health and science ping list.
P.S. For author searches on PubMed use surname space initial(s), e.g. Bastian FO, Bove JM, etc.
You do good research! Welcome to the wonderful world of infectious disease cover-ups.
You do good research! Welcome to the wonderful world of infectious disease cover-ups.
Theres one thing about CJD that has never been clear to me. This article makes it sound like there is a way to identify "variant CJD" (mad cow) as opposed to sporadic CJD. But does this involve actually testing for varieties of prions or is the judgment based only on circumstances?
Idahoping
My impression is that it is based on a history of presumed consumption of contaminated meat in folks who die relatively young with typical neurological symptoms. MRI or CT imaging studies with a sponge-like appearence are helpful. Autopsies are truly rare in the general population. When biopsies or autopsies are performed, they attempt to confirm with Western blot analysis using the monoclonal antibody 3F4 (1:50,000) for vCJD and the rabbit antiserum PrP 95-108 (1:10,000) for BSE (32, 33). Western blot analysis is the technique used for confirmation of a HIV/AIDS diagnosis.
I'm just a family practice doc with probably more than average experience in chemistry and biochemistry. Death certificates just state the opinions of the attending physicians that sign their name. Those opinions are typically based on medical history alone unless the physical examination is abnormal or the results of laboratory and imaging studies are available. When available, most of these studies can have equivocal results subject to varying interpretations and debate.
Disclaimer: Opinions posted on Free Republic are those of the individual posters and do not necessarily represent the opinion of Free Republic or its management. All materials posted herein are protected by copyright law and the exemption for fair use of copyrighted works.