You wonder how many CJ cases were incorrectly diagnosed.


Idaho probes nine Creutzfeldt-Jakob cases
Higher-than-normal incidence of infectious brain disease puzzles
officials
Oct. 17, 2005

http://www.msnbc.msn.com/id/9725973/

BOISE, Idaho - From the moment Joan Kingsford first saw her husband
stagger in his welding shop, she wanted two things: His recovery and
to know what made him sick.

She got neither. Alvin Kingsford, 72, died recently of suspected
sporadic Creutzfeldt-Jakob disease, the 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 the permutation
dubbed 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 only strikes about one person in a million
each year, with an average of just 300 cases per year in the United
States, or just over 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 if 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 just short of
calling the Idaho cases a “cluster,” waiting for final test results
from the victims who got autopsies.

There is considerable confusion over two rare, degenerative brain
disorders that both go by the name Creutzfeldt-Jakob disease.
Patients who suffer from the variant Creutzfeldt-Jakob disease, which
is transmitted to humans through tainted beef, and those who suffer
from classic Creutzfeldt-Jakob disease, may have similar symptoms,
but there are key differences between the illnesses.

Fear of exposure
The best tool of investigators to pin down the diagnosis — the
autopsy — is sometimes hard to get, said Tom Shanahan with 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 Alvin’s body after his
brain cavity had been opened. They feared they would catch the rare
disease, Kingsford said.

Ultimately, she opted to skip the autopsy and have a traditional
funeral service.

“A week before he passed away, the funeral homes said they wouldn’t
take the blood out” if an autopsy was done on him, she said. “They
just put some embalming in him and told me I had to have a funeral in
three days.”

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.

Mortuary procedures — including embalming — can be done safely on
intact bodies of CJD victims as long as extra precautions are taken,
but the World Health Organization does not recommend embalming
patients who have had autopsies.

Larry Whitaker, a Beaverton, Ore.-based regional salesman for the
embalming chemical and equipment manufacturer Dodge Company, offers
workshops to his clients on safe handling of CJD-infected bodies.

“When the brain has been removed, it is an extraordinary risk,”
Whitaker said. “This is one time I think that cremation has to be
more than mildly considered.”

A member of the Mormon Church, Joan Kingsford’s church discourages
cremation. She was thrown into making a decision about her husband’s
remains much sooner than she expected.

“It was two and a half months before we knew what was wrong with him,
and by that time he was in the hospital,” she said. “I wish we could
have done the autopsy, because I think people need to know about
this.”

“We definitely have a problem in Idaho,” she added.