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.
[email protected]
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:
[email protected]
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
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.
[email protected]
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:
[email protected]
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