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From: TSS ()
Subject: State officials inspect deaths possibly from rare disease CJD
Date: June 3, 2007 at 8:00 am PST

Posted on Sun, Jun. 03, 2007

State officials inspect deaths possibly from rare disease

By Michael Schroeder
The Journal Gazette

P. Jacquay

A rare, one-in-a-million degenerative brain disorder appears to have claimed the lives of four area victims in roughly five months.

Most recently, a man in his 70s from Steuben County died from the disorder, called Creutzfeldt-Jakob Disease, or CJD, according to his death certificate. Like the three other victims, he died in Allen County.

Dr. Deborah McMahan, Allen County health commissioner, did not confirm the identities of the deceased or their places of residence but did say that four deaths were suspected to have been caused by CJD. After the third death, McMahan contacted the state health department and asked that a call be made to the Centers for Disease Control and Prevention.

Northeast Indiana area hospitals serve about 1 million people, McMahan estimated. Based on that sample and national averages, one area death from CJD might be expected per year – not four in less than half that time.

Classic CJD – which appears to have caused the four area deaths – is not related to “mad cow” disease and is distinct from “variant CJD.” Usually sporadic in nature with no known cause, classic CJD leads to rapid brain deterioration and ultimately death. Although in exceedingly rare cases it may be acquired through exposure to brain tissue and spinal cord fluid from infected patients, CJD is not contagious through casual contact.

Pam Jacquay, 53, of New Haven lost her husband, John, 53, to the disease in March. She recalls the toll it took on his mental faculties and still wonders how her husband was taken from her so quickly.

On a Christmastime vacation he seemed perfectly fine, but within weeks he couldn’t drive, and within months he’d forgotten how to do even the simplest tasks. Jacquay dutifully helped her husband to shave and dress and do all the other things he forgot how to do as the disease ran its course, cutting spongelike lesions in his brain.

“One minute he could do something and the next minute it made no sense to him. … In the last week of his life he lost any ability to communicate with us at all.”

Jacquay took solace in the fact that her husband was not in pain. But the expectations of many good years to come made her husband’s death that much harder to bear. They were expecting to celebrate their 35th anniversary later this year.

“This just wasn’t the way it was supposed to happen,” Jacquay said.

She hopes increased awareness will lead to treatment for what is now an incurable disease. And she hopes health officials continue to investigate the increased incidence locally.

At this point, autopsies of two victims are planned, as testing of brain tissue is considered the only definitive way to determine whether a person had CJD.

Health officials with the state and the Centers for Disease Control and Prevention are withholding assessment of the situation pending further test results, which are expected to take two to three months.

John Jacquay is not among the victims who will be autopsied. But based on his symptoms – which included muscle spasms and hallucinations – and a neurologist’s diagnosis before his death, Pam Jacquay says she is sure her husband died from CJD.

However, Dr. Bernardino Ghetti, director of the Indiana Alzheimer Center and professor of neuropathology at the Indiana University School of Medicine, says a proper diagnosis can be made only with an autopsy and brain testing.

“We should look clearly at pathological diagnoses before making any assumptions, especially because the last two studies are still in progress,” Ghetti said. “If there are four (cases), which we still need to prove, then this would be an unusual number.”

Annually, fewer than 300 cases of CJD are reported in the U.S., according to the CDC. That equates to about one case per 1 million Americans, though there is some speculation about whether the true number is higher because the disease is difficult to diagnose.

Although most cases are sporadic, 5 percent to 15 percent of CJD cases result from inherited mutations of the prion protein gene. Jacquay said her husband’s case was deemed to be sporadic.

In either case, CJD “is rapidly progressive and always fatal,” according to the CDC. “Infection with this disease leads to death usually within 1 year of onset of illness.”

Unlike certain highly contagious conditions such as TB, the disorder is not considered reportable in Indiana.

Still, state epidemiologist Bob Teclaw says Indiana keeps an eye on cases by tracking death certificates. For now, he’s not drawing any conclusions from the deaths in northeast Indiana.

At this point, “we’re in the wait-and-see mode,” Teclaw said.

mschroeder@jg.net


http://www.fortwayne.com/mld/fortwayne/news/local/17318901.htm


they been in a wait and see mode for 25 years or more, what are they waiting on $$$


see history of cjd questionnaire

http://brain.hastypastry.net/forums/showthread.php?t=2408


USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.


snip...

64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.


snip...

http://www.seac.gov.uk/minutes/95.pdf


3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve
University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than
classical BSE in humans.***


6:30 Close of Day One


http://www.healthtech.com/2007/tse/day1.asp


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


http://www.cjdsurveillance.com/resources-casereport.html

There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535

BRITISH MEDICAL JOURNAL


BMJ


vCJD in the USA * BSE in U.S.
15 November 1999


http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406


BMJ


U.S. Scientist should be concerned with a CJD epidemic in the U.S., as
well...
2 January 2000


http://www.bmj.com/cgi/eletters/320/7226/8/b#6117

JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.


Volume 3, Issue 8, August 2003, Page 463


“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD)
and have been searching for answers ever since. What I have found is that we
have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem.”
............................


http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf


TSS



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