CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES HIGHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES
CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES
HIGHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES
* THE UKBSEMADCOWnvCJD only theory continues to unravel $$$
* more dpCJD 'diagnosis pending creutzfeldt jakob disease' as 'dpCJD', what
ever that is suppose to mean. how many more years, decades, are we going to have
to flounder for them to establish another name for the same disease ?
...2007
* CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES
HIGRHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES
* 6 Includes 25 cases in which the diagnosis is pending, and 20
inconclusive cases; ???
* 7 Includes 15 (14 from 2016) cases with type determination pending in
which the diagnosis of vCJD has been excluded. ???
* CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES
HIGHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES
National Prion Disease Pathology Surveillance Center Cases Examined1
(July 29, 2016)
Year
Total
Referrals2
Prion Disease Sporadic Familial Iatrogenic vCJD
1996 & earlier 57 36 31 4 1 0
1997 113 68 59 9 0 0
1998 89 53 45 7 1 0
1999 121 73 65 7 1 0
2000 144 101 89 12 0 0
2001 210 118 110 8 0 0
2002 242 144 125 17 2 0
2003 259 161 138 21 2 0
2004 313 179 161 17 0 13
2005 327 179 157 21 1 0
2006 369 182 162 17 1 24
2007 369 206 187 19 0 0
2008 387 223 207 16 0 0
2009 399 233 212 20 1 0
2010 402 246 218 28 0 0
2011 394 240 216 24 0 0
2012 409 241 218 23 0 0
2013 416 257 222 34 1 0
2014 357 211 188 21 1 15
2015 396 260 239 20 0 0
2016 190 113 88 11 0 0
TOTAL 59636 35247 31378 3569 12 4
1 Listed based on the year of death or, if not available, on year of
referral;
2 Cases with suspected prion disease for which brain tissue and/or blood
(in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and in Saudi Arabia in
the other;
5 Disease possibly acquired in a Middle Eastern or Eastern European
country;
*** 6 Includes 25 cases in which the diagnosis is pending, and 20
inconclusive cases;
*** 7 Includes 15 (14 from 2016) cases with type determination pending in
which the diagnosis of vCJD has been excluded.
*** 8 The sporadic cases include 3442 cases of sporadic Creutzfeldt-Jakob
disease (sCJD), ***56 cases of Variably Protease-Sensitive Prionopathy (VPSPr)
and 26 cases of sporadic Fatal Insomnia (sFI).
* 6 Includes 25 cases in which the diagnosis is pending, and 20
inconclusive cases; ???
* 7 Includes 15 (14 from 2016) cases with type determination pending in
which the diagnosis of vCJD has been excluded. ???
*** 8 The sporadic cases include 3442 cases of sporadic Creutzfeldt-Jakob
disease (sCJD), ***56 cases of Variably Protease-Sensitive Prionopathy (VPSPr)
and 26 cases of sporadic Fatal Insomnia (sFI). ???
Sunday, November 23, 2014
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in
June 2014 confirmed as USA case NOT European
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas
Updated: October 7, 2014
CDC and the Texas Department of State Health Services (DSHS) have completed
the investigation of the recently reported fourth vCJD case in the United
States. It confirmed that the case was in a US citizen born outside the Americas
and indicated that the patient's exposure to the BSE/vCJD agent most likely
occurred before he moved to the United States; the patient had resided in
Kuwait, Russia and Lebanon. The completed investigation did not support the
patient's having had extended travel to European countries, including the United
Kingdom, or travel to Saudi Arabia. The specific overseas country where this
patient’s infection occurred is less clear largely because the investigation did
not definitely link him to a country where other known vCJD cases likely had
been infected.
>>>the patient had resided in Kuwait, Russia and Lebanon.
>>>The completed investigation did not support the patient's
having had extended travel to European countries, including the United Kingdom,
or travel to Saudi Arabia.
NOW we all know why the state of Texas or the CDC did not want to report
this case, because it was a home grown case of nvCJD right here in Texas?...tss
Monday, June 02, 2014
Confirmed Variant CJD Case in Texas
Sunday, November 23, 2014
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in
June 2014 confirmed as USA case NOT European
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas
Sunday, November 23, 2014
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in
June 2014 confirmed as USA case NOT European
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas
Monday, November 3, 2014
USA CJD TSE PRION UNIT, TEXAS, SURVEILLANCE UPDATE NOVEMBER 2014
National Prion Disease Pathology Surveillance Center Cases Examined1
(October 7, 2014)
***6 Includes 11 cases in which the diagnosis is pending, and 19
inconclusive cases;
***7 Includes 12 (11 from 2014) cases with type determination pending in
which the diagnosis of vCJD has been excluded.
***The sporadic cases include 2660 cases of sporadic Creutzfeldt-Jakob
disease (sCJD),
***50 cases of Variably Protease-Sensitive Prionopathy (VPSPr)
***and 21 cases of sporadic Fatal Insomnia (sFI).
Tuesday, June 1, 2010
USA cases of dpCJD rising with 24 cases so far in 2010
USA cases of dpCJD rising with 24 cases so far in 2010
please be aware, i have termed this strange strain of 'diagnosis pending
creutzfeldt jakob disease' as 'dpCJD', what ever that is suppose to mean. how
many more years, decades, are we going to have to flounder for them to establish
another name for the same disease ?
Thursday, October 22, 2015
*** Former Ag Secretary Ann Veneman talks women in agriculture and we talk
mad cow disease USDA and what really happened those mad cows in Texas ***
USA CJD GETTING YOUNGER AND YOUNGER...WHY?
Sunday, August 21, 2016
Creutzfeldt-Jakob disease CJD TSE Prion USA getting younger and younger,
spontaneous sporadic, zoonosis, or iatrogenic?
August 16th, 2016 - 11:49 am
Inaugural Benefactory Festival in Scranton honors Julie Judge
Features
Proceeds go to TEDxYouth@Scranton
By Matt Mattei - Click for more information on Matt mmattei@timesleader.com
- @TLArts - 570-991-6651 More Articles By: Matt Mattei
Then Judge fell ill.
She became afflicted with Creutzfeldt-Jakob disease, a fatal brain protein
disorder known as a prion disease.
“It affects the brain and it affects it quickly,” Kenny Hill said. “She was
lively at one point, and then things started getting bad and getting bad quick.”
I spoke with the reporter this AM, Mr. Mattei, and he said Julie Judge was
52 or 53...another young victim of CJD in the USA, in Pennsylvania, where CWD in
cervid is well established. what about friendly fire or iatrogenic there from
???
Tuesday, May 03, 2016 Wednesday, May 11, 2016
PENNSYLVANIA TWELVE MORE CASES OF CWD FOUND: STATE GEARS UP FOR ADDITIONAL
CONTROL MEASURES
A Maine mother of four has died of the rare brain disorder
Creutzfeldt-Jakob disease.
Sandra Tucker Kennedy, 38, died March 3 of sporadic CJD, a degenerative
brain disease that affects only one in 1 million people worldwide per year, and
about 300 people per year in the U.S.
Kennedy, who lived in Kennebunk and worked as a nurse at Maine Medical
Center, leaves behind husband Jake Kennedy and their four children: son Tucker,
9, twin boys Asher and Gunner, 5, and daughter Skyler, 2.
Thursday, April 14, 2016
Arizona 22 year old diagnosed with Creutzfeldt Jakob Disease CJD
Thursday, January 15, 2015
41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE
Prion: Case Report
Saturday, January 17, 2015
*** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed
with the extremely rare Creutzfeldt-Jakob disease
P-121
Unusually young prion disease cases in the United States, 1979-2014
Ryan A. Maddox1, Marissa K. Person1, Arialdi M. Minino2, Janis E. Blevins3,
Lawrence B. Schonberqer 1, Ermias D. Belay1
1 National Center for Emerging and Zoonotic Infectious Diseases, Centers
for Disease Control and Prevention (CDC), USA;
2 National Center for Health Statistics, Centers for Disease Control and
Prevention (CDC), USA
3 National Prion Disease Pathology Surveillance Center (NPDPSC), Case
Western Reserve University, USA
Introduction: Creutzfeldt-Jakob disease (CJD) occurs with a frequency of
about 1.0-1.5 cases per million population per year in the United States. Most
cases occur sporadically in older adults; the disease is rare in persons <30 1979-2014.="" a="" age.="" diagnosis="" died="" disease="" div="" during="" for="" in="" information="" of="" patients="" prion="" reviewed="" states="" the="" united="" we="" who="" with="" years="" young="">
Methods: Prion disease decedents aged <30 1979-2014="" and="" at="" cause-of-death="" center.="" center="" contains="" data="" database="" disease="" div="" for="" from="" genetic="" identified="" laboratory="" medical="" multiple="" national="" neuropathological="" of="" pathology="" performed="" possible="" prion="" records="" results="" reviewed.="" surveillance="" testing="" the="" us="" were="" whenever="" which="" years="">
Results: During the 36-year period, a total of 23 decedents aged <30 1.6="" 129="" 16="" 1980s="" 1="" 22="" 2="" 3="" 4="" 6="" 7="" a="" an="" and="" annual="" associated="" at="" available="" average="" be="" billion.="" brand="" by="" cases="" cjd="" classified="" codon="" could="" decedent="" decedents="" diagnosis="" died="" disease="" div="" dura="" early="" either="" familial="" fatal="" for="" four="" further="" g54s="" genetic="" gerstmann-straussler-scheinker="" grafts.="" grafts="" growth="" had="" heterozygous.="" hgh="" homozygous="" hormone="" human="" iatrogenic="" identified="" in="" incidence="" included="" index="" infected="" insomnia.="" insomnia="" international="" linked="" lyodura="" making="" mater="" methionine="" mutation.="" of="" old="" or="" outbreaks="" overseas="" per="" pituitary-derived="" prion="" results="" seven="" sporadic="" states="" syndrome="" testing="" the="" these="" to="" type.="" type="" united="" unknown="" unlikely.="" valine="" variant="" vcjd="" was="" were="" with="" years="" young="" youngest="">
Conclusion: In contrast to prion disease cases among older adults, only
about one third of the unusually young cases were sporadic; over 40% had an
exogenous source of infection. Given the rarity of young prion disease cases and
the subset that have served as important initial signals for controllable
outbreaks, the continued identification and full workup of cases aged <30 div="" is="" recommended.="" years="">
- 239-
P-133 Suspected prion disease cases referred to the National Prion Disease
Pathology Surveillance Center, United States
Ermias D Belay1, Elizabeth J Harker1, Ryan A Maddox1, Janis E Blevins2,
Jiri G Safar2, Pierluigi Gambetti2, Lawrence B Schonberqer2
1Centers for Disease Control and Prevention, Atlanta, Georgia, United
States;
2National Prion Disease Pathology Surveillance Center, Case Western Reserve
University, Cleveland, Ohio, United States
Introduction: During 1996-1997, the US Centers for Disease Control and
Prevention, in collaboration with the American Association of Neuropathologists,
established the National Prion Disease Pathology Surveillance Center (NPDPSC) at
Case Western Reserve University in Cleveland, Ohio. This center provides prion
disease diagnostic services to US physicians and state and local health
departments.
Methods: Cases with brain tissue specimens referred to NPDPSC through 2014
were identified from the NPDPSC database and described according to their
demographics and diagnoses. Cases referred to the center from 2005- 2014 were
further described by test results obtained.
Results: Out of 5,369 suspected prion disease cases referred to NPDPSC
through 2014, 3,144 (58.6%) were diagnosed as having a prion disease. The
remaining cases were found to not have a prion disease based on negative
neuropathologic findings (n=2205) or were inconclusive (n=20). The majority of
the 3,144 prion disease cases were sporadic (89.3%), while 10.2% were familial,
0.3% were iatrogenic, and 0.1 % were variant CJD. The majority of cases (87.1 %)
were _>55 years of age. There was not a significant difference in the
percentages of male (51.8%) and female (48.2%) cases diagnosed with prion
disease at NPDPSC (p=0.06. As expected, the most cases were referred from states
with large populations; 44.0% of cases were referred from the 7 most populous
states where approximately this proportion of the US population resides. From
2005-2014, 2,213 cases were diagnosed with prion disease; all cases had either a
positive Western Blot (92.5%) or immunohistochemistry (98.4%) or both (90.9%).
Of the cases with cerebrospinal fluid (CSF) test results available during the
10-year period, 1,103 of 1,460 (75.5%) had a positive 14-3-3 and 1,141 of 1,300
(87.8%) had elevated total Tau protein. More sensitive tests such as RT QulC are
being validated.
Conclusion: The NPDPSC is a valuable resource for prion disease diagnostic
services, especially given that neuropathologic testing is required for disease
confirmation. Notably, a substantial percentage (41.1 %) of suspected prion
disease cases referred to the center were found to be negative for prion
disease. The large collection of brain tissues is critical for development and
validation of new human prion diagnostic and differentiation methods and further
studies advancing our understanding of prion diseases and other
neurodegenerative conditions.
- 251 -
PRION2016 TOKYO
Monday, August 22, 2016
Epidemiological characteristics of human prion diseases
snip...
CJD surveillance systems have some unique features compared with other
public health surveillance systems. One is that due to a lack of approved
biomarkers for CJDs, specific types of clinical and laboratory approaches are
critical to effectively diagnose and monitor CJDs. The other is that CJD
surveillance systems are primarily dependent on reports from physicians,
especially neurologists and neuropathologists in regional hospitals and medical
centers where first visit for most patients. Thus, the experiences of these
specialists directly determine the quality of the collected specimens, such as
accurate identify the clinical manifestations of CJD or supply the appropriate
specimens to CJD surveillance center. Both aspects seriously impact the
sensitivity of CJD surveillance.
snip...
Risk.21: Thirty-Year Review of Prion Disease Surveillance in the United
States
Robert C. Holman,1,† Ryan A. Maddox,1 Arianne M. Folkema,1 Arialdi M.
Minino,2 Teresa A. Hammett,1 Kenneth D. Kochanek,2 James J. Sejvar,1 Ermias D.
Belay1 and Lawrence B. Schonberger,1
1CDC; Atlanta, GA USA; 2CDC; Hyattsville, MD USA†Presenting author; Email:
rholman@cdc.gov
Background. With the emergence of bovine spongiform encephalopathy/variant
Creutzfeldt-Jakob disease (vCJD) in the UK, the Centers for Disease Control and
Prevention began utilizing national mortality data with additional surveillance
mechanisms to monitor US occurrences of human prion disease.
Objectives. To review US prion disease surveillance data.
Methods. We analyzed national mortality data for prion disease deaths (a
surrogate for CJD incidence) among US residents for the 30 year period,
1979–2008, augmenting and extending these data through 2010 with information
from other surveillance mechanisms (e.g., national neuropathology surveillance).
We calculated age-adjusted and age-specific death rates per million persons;
race-specific rates used data available beginning 1981. We age-adjusted death
rates to the standard projected US 2000 population. www.landesbioscience.com
Prion 131
Results. A total of 7,615 deaths during 1979–2008 were identified for an
average annual age-adjusted rate of 0.98 cases per million persons. The highest
rate (1.15) was observed in 1997; the highest number of reported cases was in
2008 (348). By race, the rate (1.06) among whites, who constituted 95% of the
cases, was significantly higher than among blacks (0.40), Asian/Pacific
Islanders (0.63) and American Indians/Alaska Natives (0.42). The rate (4.0)
among persons ≥55 years old was strikingly higher than the rate (0.14) among
persons <55 2004-2006="" 2010="" 21="" 30="" age="" among="" arabia.="" as="" cell="" deaths="" decedent="" decedents="" deficiency="" disease.="" div="" during="" factor="" hereditary="" identified="" in="" infected="" ix="" likely="" none="" of="" old.="" only="" or="" period="" previously="" reported="" residents="" saudi="" sickle="" thalassemia="" the="" three="" through="" uk="" us="" vcjd="" viii="" was="" were="" who="" with="" year="" years.="" years="" youngest="">
Conclusion. The annual age-adjusted US CJD death rates remained relatively
stable over several decades although the most recent, complete, annual data show
the highest number of cases. The absence of CJD in persons <20 100="" absence="" age="" an="" and="" any="" at="" blood-related="" blood="" cell="" cjd="" current="" deserve="" despite="" differences="" disease="" div="" during="" estimated="" further="" hemophilia="" if="" in="" investigation.="" is="" likely="" low.="" magnitude="" many="" more="" of="" or="" period="" persons="" plus="" population="" products="" racial="" rates="" received="" recipients="" remained="" risk="" sickle="" such="" suggests="" surveillance="" thalassemia="" the="" times="" transfusion="" transmissions="" very="" who="" with="" years="" youngsters="">
Risk.27: Creutzfeldt-Jakob Disease Among Hispanics in the United States,
1997–2008
Ryan A. Maddox,1,† Robert C. Holman,1 Arianne M. Folkema,1 Arialdi M.
Minino,2 Teresa A. Hammett,1 Lawrence B. Schonberger1 and Ermias D. Belay1
1National Center for Zoonotic and Emerging Infectious Diseases; Centers for
Disease Control and Prevention; Atlanta, GA USA; 2National Center for Health
Statistics; Centers for Disease Control and Prevention; Hyattsville, MD
USA†Presenting author; Email: rmaddox@cdc.gov
Introduction. At 16% of the US population, Hispanics make up the largest
ethnic or racial minority in the country, and this proportion is expected to
increase in the coming decades. The occurrence of Creutzfeldt-Jakob disease
(CJD) among Americans of Hispanic ethnicity has not been widely
investigated.
Methods. Hispanic CJD decedents of any age were identified from the US
national multiple cause-of-death data and other sources for 1997–2008. Relevant
portions of medical records and results from neuropathologic and genetic testing
for Hispanic CJD decedents <55 age="" and="" as="" available.="" div="" obtained="" of="" reviewed="" were="" years="">
Results. During 1997–2008, 160 CJD decedents were identified as being of
Hispanic ethnicity, for an average annual age-adjusted incidence of 0.65 per
million population, an incidence significantly lower than that for non-Hispanics
(RR =0.6; 95% CI 0.5–0.7). While 27 states reported at least one Hispanic
decedent during the time period, almost half (47.5%) of the decedents were
residents of California or Texas, the states with the highest Hispanic
populations. A majority (55.0%) of the decedents were females, but the average
annual age-adjusted incidence was slightly higher for males, although the
difference was not significant. The median age at death was 64 years (range
36-93 years). Thirty-three Hispanic CJD decedents (20.6%) were <55 0.0001="" 0.17="" 12.1="" 21="" 33="" age-specific="" age="" among="" and="" annual="" available="" average="" cases="" cjd="" compared="" confirmation="" decedent="" decedents="" div="" familial="" fatal="" for="" group="" had="" hispanic="" hispanics="" however="" in="" incidence="" including="" insomnia.="" lower="" medical="" neuropathologic="" neuropathology="" non-hispanic="" non-hispanics="" of="" one="" or="" p="" records="" reports="" respectively="" review.="" significantly="" sporadic="" ten="" that="" the="" these="" three="" to="" was="" with="" years="" young="">
Conclusions. Between 1997 and 2008, the reported CJD incidence among
Hispanics in the US was significantly lower than that for non-Hispanics. This
lower incidence may be at least partly due to underreporting of Hispanic
ethnicity relative to surveys and censuses, and further study is warranted.
Analyses of brain tissue remain important, especially considering that
approximately half of the young Hispanic decedents with information available
lacked CJD confirmation.
Risk.26: Sex Effect in Prion Diseases
Corinne Loeillet,1,† Pierre-Yves Boelle,2 Catherine Lemaire-Vieille,1
Philippe Naquet,3 Pierre Chambon,4 Marie-France Cesbron-Delauw,1 Alain-Jacques
Valleron,2 Jean Gagnon1 and Jean-Yves Cesbron1
1CNRS LAPM 5163–Université Joseph Fourier; Grenoble, France; 2INSERM U
707–2 Université Pierre et Marie Curie–Paris 6; Paris, France; 3Centre
d’Immunologie de Marseille-Luminy, INSERM-CNRS; Marseille, France; 4Institut de
Génétique et de Biologie Moléculaire et Cellulaire, Université Louis Pasteur de
Strasbourg; Strasbourg, France†Presenting author; Email:
corinne.loeuillet@ujf-grenoble.fr
Despite large exposure to BSE in the UK, less than 180 patients had
developed clinical vCJD by October 2009. This figure was closely anticipated in
2001, thanks to an epidemiological model whose main assumptions was that the
risk of acquiring vCJD was exponentially decreasing during childhood, which was
consistent with the age distribution of vCJD. Further investigation of the
models showed that this decrease of risk during childhood could not be explained
by the age variation of meat consumption, and was likely a consequence of an age
dependent susceptibility to the disease. The more likely explanation for this
strong age-susceptibility relationship during childhood is hormonal.
In this context, we investigated if there was a sex difference in human
vCJD cases, and we used a mouse model to test a first hypothesis on the possible
role of sexual hormones on the risk of prion diseases.
In the 167 vCJD cases reported in the UK as of January 2009, age at onset
was significantly lower in women (two years) than in men after stratification on
birth cohort. In C57/BL6N mice infected with ME-7 scrapie strain, incubation was
shorter in females than in males. The incubation period increased in castrated
male mice after intraperitoneal infection, but not after intracerebral
inoculation. We also observed that androgen receptor deficient mice the
incubation period of prion disease also increased after intraperitoneal
inoculation. In contrast, in ovariectomised or estrogen receptor a defective
female mice, no effect was observed on the incubation period of mouse prion
disease.
These results show that androgens influence the prion diseases incubation
period in a peripheral site.1
References
1. Loeuillet C, Boelle PY, Lemaire-Vieille C, Baldazza M, Naquet P, Chambon
P, et al. Sex effect in mouse and human prion disease. J Infect Dis 2010;
202:648-54.
Risk.49: Creutzfeldt-Jakob Disease in Canada, 1998–2009
Zheng Wang,1,† Gerard Jansen,1, 2 Elina Olsen,1 Stacy Sabourin,1 Rolande
D’Amour,1 Tim Connolly,1 Jennifer Kruse,1 Neil Cashman3 and Michael
Coulthart1
1The Canadian Creutzfeldt-Jakob Disease Surveillance System; Public Health
Agency of Canada; Ottawa, ON Canada; 2Department of Pathology; Ottawa Hospital;
Ottawa, ON Canada; 3Brain Research Centre; University of British Columbia;
Vancouver, BC Canada†Presenting author; Email: zheng.wang@phac-aspc.gc.ca
Background. Creutzfeldt-Jakob Disease (CJD) is a fatal, transmissible
neurodegenerative disease with sporadic, genetic and acquired forms. In 1998,
Canada launched comprehensive national CJD surveillance to assess the
characteristics of CJD in Canada and its risks to the health of Canadians. This
study describes the broad characteristics of CJD in Canada from 1998–2009.
Methods. Case ascertainment was based on internationally accepted criteria.
Demographic information and risk-factor data were collected by standardized
questionnaire and medical chart review. Poisson regression, descriptive
analysis, and case investigation were employed.
Results. A total of 453 CJD deaths in Canadian residents were registered
from 1998–2009. Four hundred and fifteen (92%) were sporadic (sCJD), 33 (7%)
were genetic and five (1%) were acquired. Average annual sCJD mortality was 1.1
per million population, increasing gradually from 0.9 in 1999 to 1.4 in 2009 (P
= 0.27). All provinces saw average annual mortalities ranging from 1.0 to 1.5 (P
= 0.85), except three territories where population is small (~25,000 to
~45,000), sCJD occurred equally in both genders at 1.1. sCJD was rare under 50
years of age with only 11 cases identified (2.7%). Mortality increased after 50
and peaked at 8 per million in the 70–74 age group. Median age at death was 69
and median duration of illness was 4 months. Genetic TSE accounted for 33
deaths: 19 were GSS (P102L: 5, D202N: 2, P105T: 2, Q217R:1, A117V: 1, unknown
mutation: 8); 13 were familial CJD (E200K: 9, D178N: 2, V203I: 1, V189I:1); one
was FFI (D178N). Median age for genetic TSE was 59 and median duration of
illness was 27 months. For the five acquired cases of CJD, four were associated
with dura mater procedures (3 Lyodura, 1 Tutoplast) and were identified from
1998–2003 in patients aged 14–59. Investigation indicated the infections
possibly occurred from 1981–1992 with incubation times from 10–16 years. One
biochemically and neuropathologically confirmed variant CJD death occurred in
2002 in a person under 40 years old, likely acquired overseas.
Discussion and Conclusion. Characteristics of CJD in Canada are consistent
with those observed in other countries. The increase in sCJD mortality can be at
least partly attributed to increased awareness of CJD among referring
clinicians. The finding of four dura matter associated CJD cases and one
imported vCJD case in Canada demonstrate risks to Canadians from acquired CJD
exist. Continued surveillance for iatrogenic risks and novel forms of CJD is
warranted.
HD.23: Creutzfeldt-Jakob disease among American Indians and Alaska natives
in the United States, 1983–2009
Robert C. Holman, Ryan A. Maddox, Arianne M. Folkema, Arialdi M. Minino,
Ermias D. Belay and Lawrence B. Schonberger CDC; Atlanta, GA, USA
Background/Introduction. Creutzfeldt-Jakob disease (CJD) occurrence among
American Indians and Alaska Natives (AI/ ANs) is of special interest, in part
because of the high prevalence of hunting and venison consumption in this
population. Such behaviors could place AI/ANs at increased risk of prion disease
if chronic wasting disease (CWD) were found to transmit to humans.
Materials and Methods. Death records with CJD as anylisted cause of death
for US residents identified from the national multiple cause-of-death data and
other surveillance mechanisms for 1983 through 2009 were analyzed, and incidence
was calculated by race. Available death certificates and medical records were
collected and examined for AI/AN decedents.
Results. During 1983 through 2009, 15 decedents with CJD as a cause of
death were reported as AI/AN race. The average annual age-adjusted CJD incidence
for AI/ANs was 0.39 per 1,000,000 persons. The rate for whites (1.07) was higher
compared with that for AI/ANs (RR = 2.9, 95% CI = 1.8–4.9) and blacks were
similar (0.41; RR = 1.1, 95% CI = 0.7–1.9). The median age at death was 65 y
(range 39–85 y), similar to those for whites and blacks (68 and 66 y,
respectively); four (27%) AI/ AN decedents were younger than 55 y of age. Most
of the AI/AN decedents were males (60%). Decedents were reported from 13 states;
none resided in the states with the longest known presence of CWD, Colorado,
Wyoming, and Nebraska.
Conclusion. The reported CJD incidence for AI/ANs appears lower than that
for whites and similar to that for blacks, although the CJD incidence for AI/ANs
is likely underestimated due to racial misclassification of AI/ANs. Continued
monitoring of CJD occurrence in this population is important as CWD spreads into
new areas.
P.204: Creutzfeldt-Jakob disease in the aging United States
population
Ryan A Maddox,1 Marissa K Person,1 Arialdi M Minino,2 Janis E Blevins,3
Lawrence B Schonberger,1 and Ermias D Belay1
1National Center for Emerging and Zoonotic Infectious Diseases; Centers for
Disease Control and Prevention (CDC); Atlanta, GA USA; 2National Center for
Health Statistics, CDC; Hyattsville, MD USA; 3National Prion Disease Pathology
Surveillance Center (NPDPSC); Case Western Reserve University; Cleveland, OH
USA
Introduction. Creutzfeldt-Jakob disease (CJD) predominantly occurs among
older individuals. To describe the possible impact of changing demographics in
the US population on the occurrence of CJD, we reviewed data from the US census
and from national prion disease surveillance.
Methods. Prion disease decedents were identified from the US national
multiple cause-of-death data and the National Prion Disease Pathology
Surveillance Center database for 2008-2010. Incidence rates were calculated for
decedents ≥65 years and then applied to US census population estimates for 2030
to obtain projections of the number of CJD deaths in that year, assuming no
advances in treatment or prevention of these diseases.
Results. US census data projects that ≥65-year-olds will increase from
13.1% of the population in 2010 to 20.3% in 2030. The CJD incidence rates for
2008-2010 among decedents in the 65-74, 75-84, and 85+ year age groups were, in
cases per million population, 6.5, 7.2, and 3.1, respectively. Applying these
incidence rates to US census projections, in 2030 there may be 461 CJD decedents
≥65 years of age in the United States, an increase of more than 200 cases
compared to the 2008-2010 average for this age group. Of the 461 cases, 251 are
projected to be aged 65-74 years, 182 to be aged 75-84 years, and 28 to be aged
85 years or older.
Conclusions. Unless effective treatments for CJD are developed, the aging
population in the United States will likely result in an increase in CJD cases
due to its higher incidence among older adults. The increase in cases could
impact infection control policies and health care costs, among other
factors.
HD.15: Prion disease among Asians and Pacific Islanders in the United
States, 2003–2009
Ryan A. Maddox,1 Robert C. Holman,1 Arialdi M. Minino,2 Janis E. Blevins,3
Lawrence B. Schonberger1 and Ermias D. Belay1
1National Center for Emerging and Zoonotic Infectious Diseases; Centers for
Disease Control and Prevention; Atlanta, GA USA; 2National Center for Health
Statistics; Centers for Disease Control and Prevention; Hyattsville, MD USA;
3National Prion Disease Pathology Surveillan ce Center (NPDPSC); Case Western
Reserve University; Cleveland, OH USA Introduction. Asians and Pacific Islanders
(APIs) comprise approximately 5% of the United States population. The occurrence
of Creutzfeldt-Jakob disease (CJD) and other prion diseases among APIs in the
United States has not been widely investigated.
Materials and Methods. API prion disease decedents were identified from the
United States national multiple cause-of-death data and the National Prion
Disease Pathology Surveillance Center database for 2003–2009. Relevant portions
of medical records and results from neuropathologic and genetic testing for API
prion disease decedents were obtained and reviewed, as available.
Results. During 2003–2009, 60 API prion disease decedents were identified;
34 of these decedents had additional race information available, with 15 API
decedents classified as Filipino (44%), 13 as Japanese (38%), 5 as Chinese (15%)
and 1 as Hawaiian (3%). The average annual age-adjusted incidence was 0.7 per
million population, significantly lower than that for whites (p < 0.001).
Over half (55%) of the API deaths occurred in California or Hawaii, the states
with the highest API populations. The median age at death was 66.5 y (range
40–87 y), similar to that for whites (68 y). Neuropathology reports and/ or
medical records were available for 24 of the decedents; for 20 cases with a
reported disease onset date, the median duration of illness was 4.5 mo (range
1–66 mo). Twenty of 22 decedents (91%) had sporadic CJD, while the remaining 2
decedents (9%) had familial CJD. All 20 decedents with genetic testing results
available were methionine homozygous at codon 129.
Conclusion. For 2003–2009, the reported prion disease incidence among APIs
in the United States was significantly lower than that for whites.
Underreporting of API race may contribute at least partly to this lower
incidence, but genetic factors may influence prion disease susceptibility as
well. Because the API race is heterogeneous, further study of prion diseases
among specific API ethnic groups is warranted.
*** USA sporadic CJD MAD COW DISEASE HAS HUGE PROBLEM Video
Jeff Schwan, sporadic cjd, clustering, and BSE aka mad cow type disease, is
there a link ? *video*
*** Human Mad Cow Video
1997-11-10: Panorama - The british disease *video*
Sunday, September 6, 2009
MAD COW USA 1997 *video*
*** Scrapie Video
http://zoomify.uzh.ch:8080/zoomify/videos/video-011/video-011.html
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis
*video*
Human Prion Diseases in the United States Robert C. Holman ,
Ermias D. Belay, Krista Y. Christensen, Ryan A. Maddox, Arialdi M. Minino,
Arianne M. Folkema, Dana L. Haberling, Teresa A. Hammett, Kenneth D. Kochanek,
James J. Sejvar, Lawrence B. Schonberger
PLOS
Published: January 1, 2010 • http://dx.doi.org/10.1371/journal.pone.0008521
re-Human Prion Diseases in the United States Posted by flounder on 01 Jan 2010
at 18:11 GMT I kindly disagree with your synopsis for the following reasons ;
snip...
RE: re-Human Prion Diseases in the United States part 2
flounder replied to flounder on 02 Jan 2010 at 21:26 GMT
I would kindly like to add to my initial concerns, something I brought up
years ago, and I believe that still hold true today, more so even than when I
first stated these concerns in 2003 ;
routine passive mortality CJD surveillance USA ?
THIS has been proven not to be very useful in the U.K.;
THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)
snip...
One reason for this was the _inaccuracy_ in coding of cases correctly
certified as CJD Coding is carried out by staff who are not medically qualified
and it is not surprising that coding errors occur in the processing of large
numbers of certificates. In 1982, 12,000 certificates per week were processed at
the office of population censuses and surveys by 15 coders and 6 checkers
(Alderson et al., 1983). The occurrence of both inter- and intra-observer coding
errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH
certification and coding discovered in this study _support_ the introduction of
a more accurate system of death certificates and a more detailed and specific
coding system...
snip...
Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R.
Will
snip...
IDENTIFICATION OF CASES
Cases of CJD may be identified from death certificates, but this alone is
unlikely to provide adequate monitoring. ERRORS are made in certification and
diagnosis; in the Oxford study death certificates were obtained on a series of
known confirmed cases and CJD was mentioned in only 66% of certificates. In
another series of 175 certified cases, 42 patients were judged not to have
suffered from CJD after examination of case notes (7)...
full text;
http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to
other species will invariably present pathology typical of a scrapie-like
disease.
snip...
http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
Confucius is confused again? how in 1996 and earlier can the 28 sporadic
CJD victims and the one-in-a-million there from, how can it still be one in a
million in 2008, with the sporadic CJD count rising to 205, still be
one-in-a-million? and the years in-between, steady rise just about every year,
and it still be only one-in-a-million, year after year after years? I suppose
just more of that fuzzy math, which you can see here;
Please see my complete comment to this synopsis here ;
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010
No competing interests declared.
see full text with references;
The Pathological Protein:
Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases
Philip Yam
*** ''Answering critics like Terry Singeltary, who feels that the US
undercounts CJD, Schonberger _conceded_ that the current surveillance system has
errors but stated that most of the errors will be confined to the older
population'' ***
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14,
2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1
reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD)
has been stable since 1985. These estimates, however, are based only on reported
cases, and do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number of
persons with a diagnosis of Alzheimer disease in fact may have CJD, although
only a small number of these patients receive the postmortem examination
necessary to make this diagnosis. Furthermore, only a few states have made CJD
reportable. Human and animal transmissible spongiform encephalopathies should be
reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
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?
Sent: Monday, January 08,2001 3:03 PM
TO: freas@CBS5055530.CBER.FDA.GOV
FDA CJD BSE TSE Prion Scientific Advisors and Consultants Staff January
2001 Meeting Singeltary Submission
2001 FDA CJD TSE Prion Singeltary Submission
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
2001 FDA CJD TSE Prion Singeltary Submission TSEAC
Tuesday, July 12, 2016
Chronic Wasting Disease CWD, Scrapie, Bovine Spongiform Encephalopathy BSE,
TSE, Prion Zoonosis Science History
see history of NIH may destroy human brain collection
Thursday, June 9, 2016
Advisory Committee; Transmissible Spongiform Encephalopathies Advisory
Committee; Termination
Saturday, December 12, 2015
CREUTZFELDT JAKOB DISEASE CJD TSE PRION REPORT DECEMBER 14, 2015
Sunday, December 14, 2014
ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD
strains, TSE prion aka Mad Cow Disease United States of America Update December
14, 2014 Report
Saturday, November 09, 2013
Surveillance for creutzfeldt-Jakob disease in Australia: update to December
2012
sporadic CJD, 11 year old victim, 2 year clinical course to date ???
Saturday, March 23, 2013
CJD Incidents Panel to be disbanded
Thursday, February 21, 2013
National Prion Disease Pathology Surveillance Center Cases Examined January
16, 2013
16 YEAR OLD SPORADIC FFI ?
Monday, January 14, 2013
Gambetti et al USA Prion Unit change another highly suspect USA mad cow
victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes
along with this BSe
Monday, December 31, 2012
Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State,
2006–2011-2012
Tuesday, December 25, 2012
CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25,
2012
Tuesday, June 26, 2012
Creutzfeldt Jakob Disease Human TSE report update North America, Canada,
Mexico, and USDA PRION UNIT as of May 18, 2012
type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the
rise in Canada and the USA
Wednesday, June 13, 2012
MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION
DISEASE SOME WITH POSSIBLE nvCJD
PRICE OF TSE PRION POKER GOES UP $
*** The discovery of previously unrecognized prion diseases in both humans
and animals (i.e., Nor98 in small ruminants) demonstrates that the range of
prion diseases might be wider than expected and raises crucial questions about
the epidemiology and strain properties of these new forms. We are investigating
this latter issue by molecular and biological comparison of VPSPr, GSS and
Nor98.
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion
poker goes up again $
OR-10: Variably protease-sensitive prionopathy is transmissible in bank
voles
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1
Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan
Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome,
Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna,
Italy; 3Case Western Reserve University; Cleveland, OH USA
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently
described “sporadic”neurodegenerative disease involving prion protein
aggregation, which has clinical similarities with non-Alzheimer dementias, such
as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in
Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the
prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is
the electrophoretic pattern of PrPSc after digestion with proteinase K (PK).
After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern
similar to that described in GSS cases. The clinical and pathological features
of VPSPr raised the question of the correct classification of VPSPr among prion
diseases or other forms of neurodegenerative disorders. Here we report
preliminary data on the transmissibility and pathological features of VPSPr
cases in bank voles.
Materials and Methods. Seven VPSPr cases were inoculated in two genetic
lines of bank voles, carrying either methionine or isoleucine at codon 109 of
the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases
selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical
diagnosis in voles was confirmed by brain pathological assessment and western
blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission
in BvM109. Overall, 3 voles were positive with survival time between 290 and 588
d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form
of the typical PrP27–30, which was indistinguishable to that previously observed
in BvM109 inoculated with sCJDMM1 cases.
In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until
now. Overall, 5 voles were positive with survival time between 281 and 596
d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like
PrPSc electrophoretic pattern, characterized by low molecular weight PrPres.
These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative
with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus
and the N-terminus. Second passages are in progress from these first successful
transmissions.
Conclusions. Preliminary results from transmission studies in bank voles
strongly support the notion that VPSPr is a transmissible prion disease.
Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of
voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.
The discovery of previously unrecognized prion diseases in both humans and
animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion
diseases might be wider than expected and raises crucial questions about the
epidemiology and strain properties of these new forms. We are investigating this
latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
Wednesday, March 28, 2012
*** VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion
poker goes up again $
Case report Sporadic fatal insomnia in a young woman: A diagnostic
challenge: Case Report
Karen M Moody , Lawrence B Schonberger , Ryan A Maddox , Wen-Quan Zou ,
Laura Cracco and Ignazio Cali
BMC Neurology 2011, 11:136doi:10.1186/1471-2377-11-136
Published:
31 October 2011
Abstract (provisional)
Background
Sporadic fatal insomnia (sFI) and fatal familial insomnia (FFI) are rare
human prion diseases.
Case presentation
We report a case of a 33-year-old female who died of a prion disease for
whom the diagnosis of sFI or FFI was not considered clinically. Following death
of this patient, an interview with a close family member indicated the patient's
illness included a major change in her sleep pattern, corroborating the reported
autopsy diagnosis of sFI. Genetic tests identified no prion protein (PrP) gene
mutation, but neuropathological examination and molecular study showed
protease-resistant PrP (PrPres) in several brain regions and severe atrophy of
the anterior-ventral and medial-dorsal thalamic nuclei similar to that described
in FFI.
Conclusions
In patients with suspected prion disease, a characteristic change in sleep
pattern can be an important clinical clue for identifying sFI or FFI;
polysomnography (PSG), genetic analysis, and nuclear imaging may aid in
diagnosis.
snip...
Case presentation
Clinical findings In February 2007, the Centers for Disease Control and
Prevention (CDC) and the National Prion Disease Pathology Surveillance Center
(NPDPSC) notified the Texas Department of State Health Services (DSHS) of a
32-year-old woman with an 18-month history of progressive neurological symptoms
suggestive of CJD. (Table 1) Based on the medical record and her neurologist,
her illness began in August 2005 with attention deficits and progressive memory
loss. In June 2006, she demonstrated anisocoria and bizarre behavior, including
talking incoherently to herself, and she was then referred to psychiatry. On a
mini-mental state examination, she scored abnormally low in the measure of
attention and calculation and she had reduced ability to repeat the names of
three unrelated objects [14]. Later in 2006 she was described as being in
constant motion, having unfocused hand gestures, and continued difficulty with
ambulation. She was reported as alert, but confused, sad, and having difficulty
with her thought process. Physicians caring for the case patient discussed the
possibility of several diagnoses such as viral encephalopathy, paranoid
schizophrenia, and subacute sclerosing panencephalitis, yet the overall etiology
remained unclear. By February 2007, the patient was unable to ambulate and
became bed-bound. She continued to demonstrate bizarre behavior, inability to
follow commands, and unintelligible speech. The patient expired in June 2007, 22
months after the onset of illness.
Over the course of her illness, she had EEGs, magnetic resonance imaging
(MRI) studies, and cerebrospinal fluid (CSF) tests. The EEG study performed in
July 2006 showed generalized slowing with bilateral periodic lateralized
epileptiform discharges. A second EEG performed two to three weeks later was
unsuccessful due to excessive movements of the patient. In April 2006, an MRI
study was negative for intracranial abnormalities. Another MRI study was
completed in February 2007 and it showed supratentorial parenchymal atrophy with
no other acute intracranial findings. CSF studies performed in March 2007 were
normal, including the amount of the 14-3-3 protein determined.
Because of the age of the patient and the potential for variant or
iatrogenic CJD, in July 2007 an investigator from the DSHS (KMM) interviewed a
family member to obtain additional information about the patient’s travel
history, past medical history, and the symptoms of the present illness. The
patient had a history of travel outside the continental United States to Puerto
Rico during 1995-96 where she had lived approximately one year. Her surgical
history included two back surgeries for internal disc disruption and
degenerative disc disease. An anterior lumbar discectomy with interbody fusion
at L4-5 was performed in November 2000 utilizing cadaver donated bone and in
August 2001 another fusion was performed at L5-S1 utilizing autologous bone. The
donor of cadaver bone was pre-screened minimizing the possibility of iatrogenic
transmission. There was no familial history of progressive neurological disease
or dementia-like illness. The family member also confirmed the clinical history
including the onset in August 2005 of progressive memory loss and, in February
2006, bizarre behavior that included the patient’s sitting in a chair for hours
making noises that progressively got louder.
Following preliminary autopsy results, the NPDPSC requested the DSHS
re-interview the family to ask specifically about the patient’s pattern of
sleep. When questioned about insomnia, the family member recalled that the
patient had experienced disturbed sleep at the time of her disease onset. The
family member also reported that the patient’s sleep pattern progressively
deteriorated throughout her illness. Some nights, for example, the patient did
not sleep. On other nights when she did appear to be sleeping, her sleep was
intermittent. During nights that the patient did not sleep, she would roam the
house at all hours, unable to calm down. By August of 2006, four hours was the
maximum amount of sleep the patient would get in one stretch and at times she
would go two to three days without sleep. Medications were prescribed to help
her sleep but they were not beneficial.
Genetic analysis Sequencing of the PrP gene open reading frame revealed
methionine homozygosity at codon 129, with no pathogenic mutation.
snip...
see full text ;
===================
Clinical findings In February 2007, the Centers for Disease Control and
Prevention (CDC) and the National Prion Disease Pathology Surveillance Center
(NPDPSC) notified the Texas Department of State Health Services (DSHS) of a
32-year-old woman with an 18-month history of progressive neurological symptoms
suggestive of CJD. (Table 1) Based on the medical record and her neurologist,
her illness began in August 2005 with attention deficits and progressive memory
loss. In June 2006, she demonstrated anisocoria and bizarre behavior, including
talking incoherently to herself, and she was then referred to psychiatry.
=====================
AND THAT MY FRIENDS, IS HOW YOU EXPLAIN SOMETHING AWAY INTO NOTHING. IT'S
THE USDA ET AL MAD COW WAY $$$
how many times have we seen this happen? time and time again.
sporadic FFI or nvCJD Texas style ???
Sunday, July 11, 2010
CJD or prion disease 2 CASES McLennan County Texas population 230,213 both
cases in their 40s
2 mysterious cases of disease in McLennan County a rarity, but no cause for
alarm
By Cindy V. Culp Tribune-Herald staff writer
Friday July 9, 2010
Two likely cases of a mysterious, fatal brain disorder have been reported
in McLennan County — a statistical anomaly considering that only one in 1
million people worldwide is affected by the condition in any given year.
Adding to the peculiarity is that the noncontagious disorder belongs to the
same family as Creutzfeldt-Jakob disease.
One of its forms is believed to be triggered by people eating meat from
cattle infected with mad cow disease.
As frightening as that might sound, officials said residents shouldn’t be
alarmed.
One of the local cases definitely is not the type associated with mad cow
disease, and there is no evidence the other one is, either. More importantly,
the disorder cannot be transmitted from person to person, officials said.
“To have potentially two cases this close together is statistically
unusual,” said Dr. Farley Verner, an infectious disease specialist who advises
the Waco-McLennan County Public Health District. “But because of the type of
disorder it is, and because of what we know about how it develops, it’s not a
worrisome coincidence. It’s just a coincidence.”
Because of privacy laws, health officials can release only limited details
about the local cases. Both were reported in May.
The first case involved a 49-year-old man from McGregor, Hammad Akram, the
health district’s epidemiologist, said. The man has since died.
Initial results from an autopsy show he had some type of human prion
disease, a family of diseases involving an abnormal protein.
Creutzfeldt-Jakob disease, or CJD, is the most common type of human prion
disease. The autopsy ruled out CJD, however, Akram said.
The second case involves a Waco woman in her late 40s, Akram said. Her
symptoms point to CJD, but since the only way to confirm the disease is to study
brain tissue after death, that diagnosis is not confirmed, he said.
No apparent link
There is no apparent link between the two local victims, Akram said.
Prion disease usually occurs in people older than age 60.
Doctors give patients a “working diagnosis” of human prion disease based on
certain symptoms, combined with results from a blood test, Farley said.
The symptoms are similar to those of other neurological conditions:
confusion, difficulty remembering recent events, loss of feeling in certain body
parts, balance problems, difficulty walking and muscle jerks and spasms.
If a physician rules out other causes for such symptoms, a blood test can
be done that indicates whether the person has a genetic mutation associated with
human prion disease. The test cannot confirm it, but positive results make the
diagnosis more likely, Verner said.
The name of the disease category comes from a protein called a prion.
People have normal prions, which are concentrated in the brain. But in some
instances, there is abnormal prion protein, which causes normal prions to be
converted to abnormal form.
That destroys brain tissue and is eventually fatal. The process can take
years, but most people die within three months to a year of having symptoms.
There are three main categories of human prion disease — sporadic, familial
and acquired.
Sporadic cases start spontaneously, without a clearly identifiable cause.
They account for about 85 percent of all human prion disease, according to the
National Prion Disease Pathology Surveillance Center.
Familial cases are inherited and are caused by a defect in the prion
protein gene, the center said.
Acquired cases are transmitted by infection, which can occur if a person
receives a transplant infected with prion disease or undergoes surgery where
contaminated instruments are used, according to the center.
Another avenue of infection is when someone eats contaminated beef, the
center said. That’s where the connection to mad cow disease comes in.
Only three cases linked to contaminated beef have been found in the United
States, according to health officials. In all three cases, the victims are
thought to have been infected while living overseas.
In Texas, about 120 people died from human prion disease between 2000-08,
according to state data.
Last year, there were 19 probable or confirmed cases of sporadic CJD and
two familial CJD cases statewide.
McLennan County has not had any human prion disease cases in the past
decade, according to state records. Verner said he can only recall two or three
cases in the 25 years he has been here.
cculp@wacotrib.com
757-5744
> "It’s just a coincidence.”
r i g h t. $$$
cjd = one-in-a-million ???
McLennan County, Texas population 2008 230,213
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN
AND SPINAL CORD MATTER
>>> Up until about 6 years ago, the pt worked at Tyson foods where
she worked on the assembly line, slaughtering cattle and preparing them for
packaging. She was exposed to brain and spinal cord matter when she would
euthanize the cattle. <<<
>>> Up until about 6 years ago, the pt worked at Tyson foods where
she worked on the assembly line, slaughtering cattle and preparing them for
packaging. She was exposed to brain and spinal cord matter when she would
euthanize the cattle. <<<
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN
AND SPINAL CORD MATTER
Creutzfeldt-Jakob Disease Surveillance in Texas
Sunday, July 11, 2010
CJD or prion disease 2 CASES McLennan County Texas population 230,213 both
cases in their 40s
Tuesday, August 03, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein
Monday, August 9, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein or just more PRIONBALONEY ?
snip...see full text ;
Monday, September 26, 2011
Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS,
gCJD, hvCJD, sCJD, TSE, PRION, update 2011
Monday, June 27, 2011
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive
Prionopathy and Gerstmann-Sträussler-Scheinker Disease
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease
update July 10, 2008 Friday, June 20, 2008
Sunday, August 10, 2008
A New Prionopathy OR more of the same old BSe and sporadic CJD
Sunday, August 24, 2008
Sporadic Fatal Insomnia with Unusual Biochemical and Neuropathological
Findings
O.K. let's compare some recent cases of this prionpathy in other countries
besides Gambetti's first 10 recently, that he claims is a spontaneous event,
from a genetic disorder, that is not genetic, but sporadic, that is related to
no animal TSE in North America, or the world. ...
> but the possibility that a small proportion of human cases so far
classified as "sporadic" CJD are of zoonotic origin could not be excluded.
> Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases
constitute an unforeseen first threat that could sharply modify the European
approach to prion diseases.
>Recently, however, so-called atypical forms of prion diseases have
been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and
BSE-L. These maladies resemble sporadic or genetic human prion diseases and
might be their animal equivalents. > This hypothesis also raises the
significant public health question of possible epidemiological links between
these diseases and their counterparts in humans.
>2.66 Dr Fahey also told the committee that in the last two years a
link has been established between forms of atypical CJD and atypical BSE. Dr
Fahey said that: They now believe that those atypical BSEs overseas are in fact
causing sporadic Creutzfeldt->Jakob disease. They were not sure if it was due
to mad sheep disease or a different form.
>The pathology features of Nor98 in the cerebellum of the affected
sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in
humans. >Intriguingly, these conclusions suggest that some pathological
features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.
>These observations support the view that a truly infectious TSE agent,
unrecognized until recently, infects sheep and goat flocks and may have
important implications in terms of scrapie control and public health.
>These findings raise some interrogation on the concept of TSE strain
and on the origin of the diversity of the TSE agents and could have consequences
on field TSE control measures.
>In summary, we have transmitted one atypical form of BSE (BASE) to a
cynomolgus macaque monkey that had a shorter incubation period than monkeys
infected with classical BSE, with distinctive clinical, neuropathological, and
>biochemical features; and have shown that the molecular biological
signature resembled that seen in a comparatively uncommon subtype of sporadic
CJD.
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far ***but the
possibility that a small proportion of human cases so far classified as
"sporadic" CJD are of zoonotic origin could not be excluded. Moreover,
transmission experiments to non-human primates suggest that some TSE agents in
addition to Classical BSE prions in cattle (namely L-type Atypical BSE,
Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic
wasting disease (CWD) agents) might have zoonotic potential.
snip...
PRION 2016 CONFERENCE TOKYO ZOONOSIS BSE, CWD, SCRAPIE, HUMAN TSE PRION
UPDATE
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a.
Vincent Beringue c. Patricia Aguilar a,
Natalia Fernandez-Borges a. and Alba Marin-Moreno a
"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos,
Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT.
Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas.
France
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated
bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD)
disease in human. To date, BSE agent is the only recognized zoonotic prion.
Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that
have been circulating for centuries in farmed ruminants there is no apparent
epidemiological link between exposure to ruminant products and the occurrence of
other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD).
However, the zoonotic potential of the diversity of circulating TSE agents has
never been systematically assessed. The major issue in experimental assessment
of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the
biological phenomenon that limits TSE agents’ propagation from a species to
another. In the last decade, mice genetically engineered to express normal forms
of the human prion protein has proved essential in studying human prions
pathogenesis and modeling the capacity of TSEs to cross the human species
barrier.
To assess the zoonotic potential of prions circulating in farmed ruminants,
we study their transmission ability in transgenic mice expressing human PrPC
(HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC
(129Met or 129Val) are used to determine the role of the Met129Val dimorphism in
susceptibility/resistance to the different agents.
These transmission experiments confirm the ability of BSE prions to
propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be
susceptible to BSE in sheep or goat to a greater degree than the BSE agent in
cattle and that these agents can convey molecular properties and
neuropathological indistinguishable from vCJD. However homozygous 129V mice are
resistant to all tested BSE derived prions independently of the originating
species suggesting a higher transmission barrier for 129V-PrP variant.
Transmission data also revealed that several scrapie prions propagate in
HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the
efficiency of transmission at primary passage was low, subsequent passages
resulted in a highly virulent prion disease in both Met129 and Val129 mice.
Transmission of the different scrapie isolates in these mice leads to the
emergence of prion strain phenotypes that showed similar characteristics to
those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie
prions have a zoonotic potential and raise new questions about the possible link
between animal and human prions.
*** Transmission data also revealed that several scrapie prions propagate
in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the
efficiency of transmission at primary passage was low, subsequent passages
resulted in a highly virulent prion disease in both Met129 and Val129 mice.
Transmission of the different scrapie isolates in these mice leads to the
emergence of prion strain phenotypes that showed similar characteristics to
those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie
prions have a zoonotic potential and raise new questions about the possible link
between animal and human prions.
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Transmission of scrapie prions to primate after an extended silent
incubation period
Authors
item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire,
Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item
Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item
Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron,
Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item
Deslys, Jean-Philippe -
Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal
Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015
Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E.,
Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C.,
Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J.
2015. Transmission of scrapie prions to primate after an extended silent
incubation period. Scientific Reports. 5:11573.
Interpretive Summary: The transmissible spongiform encephalopathies (also
called prion diseases) are fatal neurodegenerative diseases that affect animals
and humans. The agent of prion diseases is a misfolded form of the prion protein
that is resistant to breakdown by the host cells. Since all mammals express
prion protein on the surface of various cells such as neurons, all mammals are,
in theory, capable of replicating prion diseases. One example of a prion
disease, bovine spongiform encephalopathy (BSE; also called mad cow disease),
has been shown to infect cattle, sheep, exotic undulates, cats, non-human
primates, and humans when the new host is exposed to feeds or foods contaminated
with the disease agent. The purpose of this study was to test whether non-human
primates (cynomologous macaque) are susceptible to the agent of sheep scrapie.
After an incubation period of approximately 10 years a macaque developed
progressive clinical signs suggestive of neurologic disease. Upon postmortem
examination and microscopic examination of tissues, there was a widespread
distribution of lesions consistent with a transmissible spongiform
encephalopathy. This information will have a scientific impact since it is the
first study that demonstrates the transmission of scrapie to a non-human primate
with a close genetic relationship to humans. This information is especially
useful to regulatory officials and those involved with risk assessment of the
potential transmission of animal prion diseases to humans. Technical Abstract:
Classical bovine spongiform encephalopathy (c-BSE) is an animal prion disease
that also causes variant Creutzfeldt-Jakob disease in humans. Over the past
decades, c-BSE's zoonotic potential has been the driving force in establishing
extensive protective measures for animal and human health.
*** In complement to the recent demonstration that humanized mice are
susceptible to scrapie, we report here the first observation of direct
transmission of a natural classical scrapie isolate to a macaque after a 10-year
incubation period. Neuropathologic examination revealed all of the features of a
prion disease: spongiform change, neuronal loss, and accumulation of PrPres
throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of
scrapie to humans, at a time when protective measures for human and animal
health are being dismantled and reduced as c-BSE is considered controlled and
being eradicated.
*** Our results underscore the importance of precautionary and protective
measures and the necessity for long-term experimental transmission studies to
assess the zoonotic potential of other animal prion strains.
Transmission of scrapie prions to primate after an extended silent
incubation period
Emmanuel E. Comoy , Jacqueline Mikol , Sophie Luccantoni-Freire , Evelyne
Correia , Nathalie Lescoutra-Etchegaray , Valérie Durand , Capucine Dehen ,
Olivier Andreoletti , Cristina Casalone , Juergen A. Richt , Justin J. Greenlee
, Thierry Baron , Sylvie L. Benestad , Paul Brown & Jean-Philippe Deslys
Scientific Reports 5, Article number: 11573 (2015) doi:10.1038/srep11573
Download Citation Epidemiology Neurological manifestations Prion diseases
Received:16 February 2015Accepted:28 May 2015
***Moreover, sporadic disease has never been observed in breeding colonies
or primate research laboratories, most notably among hundreds of animals over
several decades of study at the National Institutes of Health25, and in nearly
twenty older animals continuously housed in our own facility.***
Tuesday, December 16, 2014
Evidence for zoonotic potential of ovine scrapie prions
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves
Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle
Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia
Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1,
Affiliations Contributions Corresponding author Journal name: Nature
Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821
Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014
Article tools Citation Reprints Rights & permissions Article metrics
Abstract
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie
prions remains unknown. Mice genetically engineered to overexpress the human
prion protein (tgHu) have emerged as highly relevant models for gauging the
capacity of prions to transmit to humans. These models can propagate human
prions without any apparent transmission barrier and have been used used to
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie
prions transmit to several tgHu mice models with an efficiency comparable to
that of cattle BSE. The serial transmission of different scrapie isolates in
these mice led to the propagation of prions that are phenotypically identical to
those causing sporadic CJD (sCJD) in humans. These results demonstrate that
scrapie prions have a zoonotic potential and raise new questions about the
possible link between animal and human prions.
snip...
Do our transmission results in tgHu imply that sheep scrapie is the cause
of sCJD cases in humans? This question challenges well-established dogma that
sCJD is a spontaneous disorder unrelated to animal prion disease. In our
opinion, our data on their own do not unequivocally establish a causative link
between natural exposure to sheep scrapie and the subsequent appearance of sCJD
in humans. However, our studies clearly point out the need to re-consider this
possibility. Clarification on this topic will be aided by informed and modern
epidemiological studies to up-date previous analysis that was performed at the
end of the last century3, 4. The value of such an approach is highlighted by the
implementation in the year 2000 of large-scale active animal TSE surveillance
programs around the world that provided an informed epidemiological-based view
of the occurrence and geographical spread of prion disease in small ruminant
populations51. The fact that both Australia and New-Zealand, two countries that
had been considered for more than 50 years as TSE-free territories, were finally
identified positive for atypical scrapie in their sheep flocks provides an
example of how prion dogma can be reversed52. However, the incubation period for
prion disease in humans after exposure to prions via the peripheral route, such
as in iatrogenic CJD transmission and Kuru, can exceed several decades53, 54. In
this context, it will be a challenge to combine epidemiological data collected
contemporarily in animal populations and humans to investigate the existence of
a causative link between prion disease occurrence in these different hosts.
Furthermore, it is crucial to bear in mind that sporadic sCJD in humans is a
rare disease (1–2 individuals per million of the population per year) and that
scrapie has been circulating in small ruminants populations used for food
purposes for centuries. Consequently, it is our opinion that even if a causative
link was established between sheep scrapie exposure and the occurrence of
certain sCJD cases, it would be wrong to consider small ruminant TSE agents as a
new major threat for public health. Despite this, it remains clear that our data
provide a new impetus to establish the true zoonotic potential of sheep scrapie
prions.
Subject terms: Biological sciences• Medical research At a glance
Thursday, August 04, 2016
MEETING ON THE FEASIBILITY OF CARRYING OUT EPIDEMIOLOGICAL STUDIES ON
CREUTZFELDT JAKOB DISEASE 1978 THE SCRAPIE FILES IN CONFIDENCE CONFIDENTIAL SCJD
SEE CONFIDENTIAL SCRAPIE FILES ;
BE SURE TO SEE THIS NEXT ONE WITH FIGURES...TSS
STUDIES ON CREUTZFELDT-JAKOB DISEASE
i enclose a list of ICD categories showing the numbers of deaths attributed
to each (as underlying cause) in England and Hales in 1975.
ICD NO...Number of Certificates examined
xxxxx...18...15 mentioned C-J
xxxxx...122...1 mentioned C-J with dimentia, 24 mentioned Alzheimer’s
disease, 1 mentioned Pick’s disease.
xxxxx...22...4 mentioned Myoclonic epilepsy
xxxxx...384...none mentioned Corticostrionigral degeneration
xxxxx...2...none mentioned Corticostrionigral degeneration
snip...
1979
SILENCE ON CJD AND SCRAPIE
1980
SILENCE ON CJD AND SCRAPIE
*** 1981 NOVEMBER
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were
exposed to the infectious agents only by their nonforced consumption of known
infectious tissues. The asymptomatic incubation period in the one monkey exposed
to the virus of kuru was 36 months; that in the two monkeys exposed to the virus
of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the
two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively.
Careful physical examination of the buccal cavities of all of the monkeys failed
to reveal signs or oral lesions. One additional monkey similarly exposed to kuru
has remained asymptomatic during the 39 months that it has been under
observation.
snip...
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie
by natural feeding to squirrel monkeys that we have reported provides further
grounds for concern that scrapie-infected meat may occasionally give rise in
humans to Creutzfeldt-Jakob disease.
PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
12/10/76
AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note CHAIRMAN: PROFESSOR PETER WILDY
snip...
A The Present Position with respect to Scrapie A] The Problem Scrapie is a
natural disease of sheep and goats. It is a slow and inexorably progressive
degenerative disorder of the nervous system and it ia fatal. It is enzootic in
the United Kingdom but not in all countries. The field problem has been reviewed
by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in
Britain for a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during the five
years 1971-1975. A further inestimable loss arises from the closure of certain
export markets, in particular those of the United States, to British sheep. It
is clear that scrapie in sheep is important commercially and for that reason
alone effective measures to control it should be devised as quickly as possible.
Recently the question has again been brought up as to whether scrapie is
transmissible to man. This has followed reports that the disease has been
transmitted to primates.
One particularly lurid speculation (Gajdusek 1977) conjectures that the
agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible
encephalopathy of mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit scrapie-blood
line and scrapie-exposed sheep and goats to be processed for human or animal
food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by
the finding that some strains of scrapie produce lesions identical to the once
which characterise the human dementias" Whether true or not. the hypothesis that
these agents might be transmissible to man raises two considerations. First, the
safety of laboratory personnel requires prompt attention. Second, action such as
the "scorched meat" policy of USDA makes the solution of the acrapie problem
urgent if the sheep industry is not to suffer grievously.
snip...
76/10.12/4.6
*** 1976 Scrapie Research USDA worried about Scrapie and sCJD in man...tss
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972);
doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological
Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey
(Macaca fascicularis) with an incubation period of more than 5 yr from the time
of intracerebral inoculation of scrapie-infected mouse brain. The animal
developed a chronic central nervous system degeneration, with ataxia, tremor and
myoclonus with associated severe scrapie-like pathology of intensive astroglial
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of
grey matter. The strain of scrapie virus used was the eighth passage in Swiss
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton,
Berkshire).
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972);
doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological
Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey
(Macaca fascicularis) with an incubation period of more than 5 yr from the time
of intracerebral inoculation of scrapie-infected mouse brain. The animal
developed a chronic central nervous system degeneration, with ataxia, tremor and
myoclonus with associated severe scrapie-like pathology of intensive astroglial
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of
grey matter. The strain of scrapie virus used was the eighth passage in Swiss
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton,
Berkshire).
why do we not want to do TSE transmission studies on chimpanzees $
IN CONFIDENCE
TRANSMISSION TO CHIMPANZEES
snip...
5. A positive result from a chimpanzee challenged severely would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.
snip...
R. BRADLEY
full text ;
RB3.20
IN CONFIDENCE
TRANSMISSION TO CHIMPANZEE
1. Kuru and CJD have been successfully transmitted to chimpanzees but
scrapie and TME have not.
2. We cannot say that scrapie will not transmit to chimpanzees. There are
several scrapie strains and I am not aware that all have been tried (that would
have to be from mouse passaged material). Nor has a wide enough range of field
isolates subsequently strain typed in mice been inoculated by the appropriate
routes (i/c, i/p and i v);
3. I believe the proposed experiment to determine transmissibility, if
conducted, would only show the susceptibility or resistance of the chimpanzee to
infection/disease by the routes used and the result could not be interpreted for
the predictability of the susceptibility for man. Proposals for prolonged oral
exposure of chimpanzees to milk from cattle were suggested a long while ago and
rejected.
4. In view of Dr Gibbs‘ probable use of Chimpazees Mr Wells‘ comments
(enclosed) are pertinent. I have yet to receive a direct communication from Dr
Schellekers but before any collaboration or provision of material we should
identify the Gibbs' proposals and objectives.
5. A positive result from a chimpanzee challenged severely would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.
A negative result would take a lifetime to determine but that would be a
shorter period than might be available for human exposure and it would still not
answer the question regarding mans' susceptibility. In the meantime no doubt the
negativity would be used defensively. It would however be counterproductive if
the experiment finally became positive- We may learn more about public reactions
following next Monday‘s meeting. CVO (+ Mr. Wells’ comments)
Dr. T W A Little
Dr. B J Shreeve
R Bradley September 1990
90/9.23/1/1
re-Evidence for human transmission of amyloid-β pathology and cerebral
amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26
April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated
online 11 September 2015 Erratum (October, 2015)
snip...see full Singeltary Nature comment here;
Self-Propagative Replication of Ab Oligomers Suggests Potential
Transmissibility in Alzheimer Disease
*** Singeltary comment PLoS
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion
disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
SWISS MEDICAL WEEKLY
Alzheimer-type brain pathology may be transmitted by grafts of dura mater
26/01/2016 Singeltary comment ;
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
TOKYO
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X
Monday, May 02, 2016
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
2015
O.05: Transmission of prions to primates after extended silent incubation
periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni,
Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys
Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies
reputed to be transmissible under field conditions since decades. The
transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that
an animal PD might be zoonotic under appropriate conditions. Contrarily, in the
absence of obvious (epidemiological or experimental) elements supporting a
transmission or genetic predispositions, PD, like the other proteinopathies, are
reputed to occur spontaneously (atpical animal prion strains, sporadic CJD
summing 80% of human prion cases). Non-human primate models provided the first
evidences supporting the transmissibiity of human prion strains and the zoonotic
potential of BSE. Among them, cynomolgus macaques brought major information for
BSE risk assessment for human health (Chen, 2014), according to their
phylogenetic proximity to humans and extended lifetime. We used this model to
assess the zoonotic potential of other animal PD from bovine, ovine and cervid
origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical
scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD,
albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked
in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases. We will present an
updated panorama of our different transmission studies and discuss the
implications of such extended incubation periods on risk assessment of animal PD
for human health.
===============
***thus questioning the origin of human sporadic cases***
===============
***our findings suggest that possible transmission risk of H-type BSE to
sheep and human. Bioassay will be required to determine whether the PMCA
products are infectious to these animals.
==============
PRION 2016 TOKYO
Zoonotic Potential of CWD Prions: An Update
Ignazio Cali1, Liuting Qing1, Jue Yuan1, Shenghai Huang2, Diane Kofskey1,3,
Nicholas Maurer1, Debbie McKenzie4, Jiri Safar1,3,5, Wenquan Zou1,3,5,6,
Pierluigi Gambetti1, Qingzhong Kong1,5,6
1Department of Pathology, 3National Prion Disease Pathology Surveillance
Center, 5Department of Neurology, 6National Center for Regenerative Medicine,
Case Western Reserve University, Cleveland, OH 44106, USA.
4Department of Biological Sciences and Center for Prions and Protein
Folding Diseases, University of Alberta, Edmonton, Alberta, Canada,
2Encore Health Resources, 1331 Lamar St, Houston, TX 77010
Chronic wasting disease (CWD) is a widespread and highly transmissible
prion disease in free-ranging and captive cervid species in North America. The
zoonotic potential of CWD prions is a serious public health concern, but the
susceptibility of human CNS and peripheral organs to CWD prions remains largely
unresolved. We reported earlier that peripheral and CNS infections were detected
in transgenic mice expressing human PrP129M or PrP129V. Here we will present an
update on this project, including evidence for strain dependence and influence
of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of
experimental human CWD prions.
PRION 2016 TOKYO
In Conjunction with Asia Pacific Prion Symposium 2016
PRION 2016 Tokyo
Prion 2016
Prion 2016
Purchase options Price * Issue Purchase USD 198.00
Cervid to human prion transmission
Kong, Qingzhong
Case Western Reserve University, Cleveland, OH, United States
Abstract
Prion disease is transmissible and invariably fatal. Chronic wasting
disease (CWD) is the prion disease affecting deer, elk and moose, and it is a
widespread and expanding epidemic affecting 22 US States and 2 Canadian
provinces so far. CWD poses the most serious zoonotic prion transmission risks
in North America because of huge venison consumption (>6 million deer/elk
hunted and consumed annually in the USA alone), significant prion infectivity in
muscles and other tissues/fluids from CWD-affected cervids, and usually high
levels of individual exposure to CWD resulting from consumption of the affected
animal among often just family and friends. However, we still do not know
whether CWD prions can infect humans in the brain or peripheral tissues or
whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no
essays to reliably detect CWD infection in humans. We hypothesize that:
(1) The classic CWD prion strain can infect humans at low levels in the
brain and peripheral lymphoid tissues;
(2) The cervid-to-human transmission barrier is dependent on the cervid
prion strain and influenced by the host (human) prion protein (PrP) primary
sequence;
(3) Reliable essays can be established to detect CWD infection in
humans;and
(4) CWD transmission to humans has already occurred. We will test these
hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in
vitro approaches.
Aim 1 will prove that the classical CWD strain may infect humans in brain
or peripheral lymphoid tissues at low levels by conducting systemic bioassays in
a set of "humanized" Tg mouse lines expressing common human PrP variants using a
number of CWD isolates at varying doses and routes. Experimental "human CWD"
samples will also be generated for Aim 3.
Aim 2 will test the hypothesis that the cervid-to-human prion transmission
barrier is dependent on prion strain and influenced by the host (human) PrP
sequence by examining and comparing the transmission efficiency and phenotypes
of several atypical/unusual CWD isolates/strains as well as a few prion strains
from other species that have adapted to cervid PrP sequence, utilizing the same
panel of humanized Tg mouse lines as in Aim 1.
Aim 3 will establish reliable essays for detection and surveillance of CWD
infection in humans by examining in details the clinical, pathological,
biochemical and in vitro seeding properties of existing and future experimental
"human CWD" samples generated from Aims 1-2 and compare them with those of
common sporadic human Creutzfeldt-Jakob disease (sCJD) prions.
Aim 4 will attempt to detect clinical CWD-affected human cases by examining
a significant number of brain samples from prion-affected human subjects in the
USA and Canada who have consumed venison from CWD-endemic areas utilizing the
criteria and essays established in Aim 3. The findings from this proposal will
greatly advance our understandings on the potential and characteristics of
cervid prion transmission in humans, establish reliable essays for CWD zoonosis
and potentially discover the first case(s) of CWD infection in humans.
Public Health Relevance There are significant and increasing human exposure
to cervid prions because chronic wasting disease (CWD, a widespread and highly
infectious prion disease among deer and elk in North America) continues
spreading and consumption of venison remains popular, but our understanding on
cervid-to-human prion transmission is still very limited, raising public health
concerns. This proposal aims to define the zoonotic risks of cervid prions and
set up and apply essays to detect CWD zoonosis using mouse models and in vitro
methods. The findings will greatly expand our knowledge on the potentials and
characteristics of cervid prion transmission in humans, establish reliable
essays for such infections and may discover the first case(s) of CWD infection
in humans.
Funding Agency Agency National Institute of Health (NIH)
Institute National Institute of Neurological Disorders and Stroke (NINDS)
Type Research Project (R01)
Project # 1R01NS088604-01A1
Application # 9037884
Study Section Cellular and Molecular Biology of Neurodegeneration Study
Section (CMND)
Program Officer Wong, May
Project Start 2015-09-30
Project End 2019-07-31
Budget Start 2015-09-30
Budget End 2016-07-31
Support Year 1
Fiscal Year 2015
Total Cost $337,507
Indirect Cost $118,756
Institution
Name Case Western Reserve University
Department Pathology
Type Schools of Medicine
DUNS # 077758407
City Cleveland
State OH
Country United States
Zip Code 44106
===========================================================
We hypothesize that:
(1) The classic CWD prion strain can infect humans at low levels in the
brain and peripheral lymphoid tissues;
(2) The cervid-to-human transmission barrier is dependent on the cervid
prion strain and influenced by the host (human) prion protein (PrP) primary
sequence;
(3) Reliable essays can be established to detect CWD infection in
humans;and
(4) *** CWD transmission to humans has already occurred. *** We will test
these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary
in vitro approaches.
============================================================
Key Molecular Mechanisms of TSEs
Zabel, Mark D.
Colorado State University-Fort Collins, Fort Collins, CO, United States
Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs),
are fatal neurodegenerative diseases affecting humans, cervids, bovids, and
ovids. The absolute requirement of PrPC expression to generate prion diseases
and the lack of instructional nucleic acid define prions as unique infectious
agents. Prions exhibit species-specific tropism, inferring that unique prion
strains exist that preferentially infct certain host species and confront
transmission barriers to heterologous host species. However, transmission
barriers are not absolute. Scientific consensus agrees that the sheep TSE
scrapie probably breached the transmission barrier to cattle causing bovine
spongiform encephalopathy that subsequently breached the human transmission
barrier and likely caused several hundred deaths by a new-variant form of the
human TSE Creutzfeldt-Jakob disease in the UK and Europe. The impact to human
health, emotion and economies can still be felt in areas like farming, blood and
organ donations and the threat of a latent TSE epidemic. This precedent raises
the real possibility of other TSEs, like chronic wasting disease of cervids,
overcoming similar human transmission barriers. A groundbreaking discovery made
last year revealed that mice infected with heterologous prion strains facing
significant transmission barriers replicated prions far more readily in spleens
than brains6. Furthermore, these splenic prions exhibited weakened transmission
barriers and expanded host ranges compared to neurogenic prions. These data
question conventional wisdom of avoiding neural tissue to avoid prion
xenotransmission, when more promiscuous prions may lurk in extraneural tissues.
Data derived from work previously funded by NIH demonstrate that Complement
receptors CD21/35 bind prions and high density PrPC and differentially impact
prion disease depending on the prion isolate or strain used. Recent advances in
live animal and whole organ imaging have led us to generate preliminary data to
support novel, innovative approaches to assessing prion capture and transport.
We plan to test our unifying hypothesis for this proposal that CD21/35 control
the processes of peripheral prion capture, transport, strain selection and
xenotransmission in the following specific aims. 1. Assess the role of CD21/35
in splenic prion strain selection and host range expansion. 2. Determine whether
CD21/35 and C1q differentially bind distinct prion strains 3. Monitor the
effects of CD21/35 on prion trafficking in real time and space 4. Assess the
role of CD21/35 in incunabular prion trafficking
Public Health Relevance Transmissible spongiform encephalopathies, or prion
diseases, are devastating illnesses that greatly impact public health,
agriculture and wildlife in North America and around the world. The impact to
human health, emotion and economies can still be felt in areas like farming,
blood and organ donations and the threat of a latent TSE epidemic. This
precedent raises the real possibility of other TSEs, like chronic wasting
disease (CWD) of cervids, overcoming similar human transmission barriers. Early
this year Canada reported its first case of BSE in over a decade audits first
case of CWD in farmed elk in three years, underscoring the need for continued
vigilance and research. Identifying mechanisms of transmission and zoonoses
remains an extremely important and intense area of research that will benefit
human and other animal populations.
Funding Agency Agency National Institute of Health (NIH)
Institute National Institute of Allergy and Infectious Diseases (NIAID)
Type High Priority, Short Term Project Award (R56)
Project # 1R56AI122273-01A1
Application # 9211114
Study Section Cellular and Molecular Biology of Neurodegeneration Study
Section (CMND)
Program Officer Beisel, Christopher E
Project Start 2016-02-16
Project End 2017-01-31
Budget Start 2016-02-16
Budget End 2017-01-31
Support Year 1
Fiscal Year 2016
Total Cost
Indirect Cost Institution Name Colorado State University-Fort Collins
Department Microbiology/Immun/Virology
Type Schools of Veterinary Medicine
DUNS # 785979618 City Fort Collins
State CO
Country United States
Zip Code 80523
PMCA Detection of CWD Infection in Cervid and Non-Cervid Species
Hoover, Edward Arthur
Colorado State University-Fort Collins, Fort Collins, CO, United States
Abstract Chronic wasting disease (CWD) of deer and elk is an emerging highly
transmissible prion disease now recognized in 18 States, 2 Canadian provinces,
and Korea. We have shown that Infected deer harbor and shed high levels of
infectious prions in saliva, blood, urine, and feces, and in the tissues
generating those body fluids and excreta, thereby leading to facile transmission
by direct contact and environmental contamination. We have also shown that CWD
can infect some non-cervid species, thus the potential risk CWD represents to
domestic animal species and to humans remains unknown. Whether prions borne in
blood, saliva, nasal fluids, milk, or excreta are generated or modified in the
proximate peripheral tissue sites, may differ in subtle ways from those
generated in brain, or may be adapted for mucosal infection remain open
questions. The increasing parallels in the pathogenesis between prion diseases
and human neurodegenerative conditions, such as Alzheimer's and Parkinson's
diseases, add relevance to CWD as a transmissible protein misfolding disease.
The overall goal of this work is to elucidate the process of CWD prion
transmission from mucosal secretory and excretory tissue sites by addressing
these questions: (a) What are the kinetics and magnitude of CWD prion shedding
post-exposure? (b) Are excreted prions biochemically distinct, or not, from
those in the CNS? (c) Are peripheral epithelial or CNS tissues, or both, the
source of excreted prions? and (d) Are excreted prions adapted for horizontal
transmission via natural/trans-mucosal routes? The specific aims of this
proposal are: (1) To determine the onset and consistency of CWD prion shedding
in deer and cervidized mice; (2); To compare the biochemical and biophysical
properties of excretory vs. CNS prions; (3) To determine the capacity of
peripheral tissues to support replication of CWD prions; (4) To determine the
protease- sensitive infectious fraction of excreted vs. CNS prions; and (5) To
compare the mucosal infectivity of excretory vs. CNS prions. Understanding the
mechanisms that enable efficient prion dissemination and shedding will help
elucidate how horizontally transmissible prions evolve and succeed, and is the
basis of this proposal. Understanding how infectious misfolded proteins (prions)
are generated, trafficked, shed, and transmitted will aid in preventing,
treating, and managing the risks associated with these agents and the diseases
they cause.
Public Health Relevance Chronic wasting disease (CWD) of deer and elk is an
emergent highly transmissible prion disease now recognized throughout the USA as
well as in Canada and Korea. We have shown that infected deer harbor and shed
high levels of infectious prions in saliva, blood, urine, and feces thereby
leading to transmission by direct contact and environmental contamination. In
that our studies have also shown that CWD can infect some non-cervid species,
the potential risk CWD may represents to domestic animal species and humans
remains unknown. The increasing parallels in the development of major human
neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, and
prion diseases add relevance to CWD as a model of a transmissible protein
misfolding disease. Understanding how infectious misfolded proteins (prions) are
generated and transmitted will aid in interrupting, treating, and managing the
risks associated with these agents and the diseases they cause.
Funding Agency Agency National Institute of Health (NIH)
Institute National Institute of Neurological Disorders and Stroke (NINDS)
Type Research Project (R01)
Project # 4R01NS061902-07
Application # 9010980
Study Section Cellular and Molecular Biology of Neurodegeneration Study
Section (CMND)
Program Officer Wong, May Project Start 2009-09-30
Project End 2018-02-28
Budget Start 2016-03-01
Budget End 2017-02-28
Support Year 7
Fiscal Year 2016
Total Cost $409,868
Indirect Cost $134,234 Institution Name Colorado State University-Fort
Collins
Department Microbiology/Immun/Virology
Type Schools of Veterinary Medicine
DUNS # 785979618 City Fort Collins
State CO
Country United States
Zip Code 80523
LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL
THE WRONG PLACES $$$
*** These results would seem to suggest that CWD does indeed have zoonotic
potential, at least as judged by the compatibility of CWD prions and their human
PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests
that if zoonotic CWD occurred, it would most likely effect those of the PRNP
codon 129-MM genotype and that the PrPres type would be similar to that found in
the most common subtype of sCJD (MM1).***
PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS
*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE ***
O18
Zoonotic Potential of CWD Prions
Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1,
Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve
University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy,
3Encore Health Resources, Houston, Texas, USA
*** These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.
==================
***These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.***
==================
P.105: RT-QuIC models trans-species prion transmission
Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover
Prion Research Center; Colorado State University; Fort Collins, CO USA
Conversely, FSE maintained sufficient BSE characteristics to more
efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was
competent for conversion by CWD and fCWD.
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.
================
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.***
================
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
*** chronic wasting disease, there was no absolute barrier to conversion of
the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay
when seeded with CWD, resembles that found in the most common human prion
disease, namely sCJD of the MM1 subtype.
*** These results would seem to suggest that CWD does indeed have zoonotic
potential, at least as judged by the compatibility of CWD prions and their human
PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests
that if zoonotic CWD occurred, it would most likely effect those of the PRNP
codon 129-MM genotype and that the PrPres type would be similar to that found in
the most common subtype of sCJD (MM1).***
*** The potential impact of prion diseases on human health was greatly
magnified by the recognition that interspecies transfer of BSE to humans by beef
ingestion resulted in vCJD. While changes in animal feed constituents and
slaughter practices appear to have curtailed vCJD, there is concern that CWD of
free-ranging deer and elk in the U.S. might also cross the species barrier.
Thus, consuming venison could be a source of human prion disease. Whether BSE
and CWD represent interspecies scrapie transfer or are newly arisen prion
diseases is unknown. Therefore, the possibility of transmission of prion disease
through other food animals cannot be ruled out. There is evidence that vCJD can
be transmitted through blood transfusion. There is likely a pool of unknown size
of asymptomatic individuals infected with vCJD, and there may be asymptomatic
individuals infected with the CWD equivalent. These circumstances represent a
potential threat to blood, blood products, and plasma supplies.
***********CJD REPORT 1994 increased risk for consumption of veal and
venison and lamb***********
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL
REPORT AUGUST 1994
Consumption of venison and veal was much less widespread among both cases
and controls. For both of these meats there was evidence of a trend with
increasing frequency of consumption being associated with increasing risk of
CJD. (not nvCJD, but sporadic CJD...tss)
These associations were largely unchanged when attention was restricted to
pairs with data obtained from relatives. ...
Table 9 presents the results of an analysis of these data.
There is STRONG evidence of an association between ‘’regular’’ veal eating
and risk of CJD (p = .0.01).
Individuals reported to eat veal on average at least once a year appear to
be at 13 TIMES THE RISK of individuals who have never eaten veal.
There is, however, a very wide confidence interval around this estimate.
There is no strong evidence that eating veal less than once per year is
associated with increased risk of CJD (p = 0.51).
The association between venison eating and risk of CJD shows similar
pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK
OF CJD (p = 0.04).
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY
OF LAMB EATING (p = 0.02).
The evidence for such an association between beef eating and CJD is weaker
(p = 0.14). When only controls for whom a relative was interviewed are included,
this evidence becomes a little STRONGER (p = 0.08).
snip...
It was found that when veal was included in the model with another
exposure, the association between veal and CJD remained statistically
significant (p = < 0.05 for all exposures), while the other exposures ceased
to be statistically significant (p = > 0.05).
snip...
In conclusion, an analysis of dietary histories revealed statistical
associations between various meats/animal products and INCREASED RISK OF CJD.
When some account was taken of possible confounding, the association between
VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS
STATISTICALLY. ...
snip...
In the study in the USA, a range of foodstuffs were associated with an
increased risk of CJD, including liver consumption which was associated with an
apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3
studies in relation to this particular dietary factor, the risk of liver
consumption became non-significant with an odds ratio of 1.2 (PERSONAL
COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
snip...see full report ;
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge
Spencers Lane BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
Thank you for your recent letter concerning the publication of the third
annual report from the CJD Surveillance Unit. I am sorry that you are
dissatisfied with the way in which this report was published.
The Surveillance Unit is a completely independant outside body and the
Department of Health is committed to publishing their reports as soon as they
become available. In the circumstances it is not the practice to circulate the
report for comment since the findings of the report would not be amended. In
future we can ensure that the British Deer Farmers Association receives a copy
of the report in advance of publication.
The Chief Medical Officer has undertaken to keep the public fully informed
of the results of any research in respect of CJD. This report was entirely the
work of the unit and was produced completely independantly of the the
Department.
The statistical results reqarding the consumption of venison was put into
perspective in the body of the report and was not mentioned at all in the press
release. Media attention regarding this report was low key but gave a realistic
presentation of the statistical findings of the Unit. This approach to
publication was successful in that consumption of venison was highlighted only
once by the media ie. in the News at one television proqramme.
I believe that a further statement about the report, or indeed statistical
links between CJD and consumption of venison, would increase, and quite possibly
give damaging credence, to the whole issue. From the low key media reports of
which I am aware it seems unlikely that venison consumption will suffer
adversely, if at all.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
Monday, May 02, 2016
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD
*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent
*** Here we report that a human prion strain that had adopted the cervid
prion protein (PrP) sequence through passage in cervidized transgenic mice
efficiently infected transgenic mice expressing human PrP,
*** indicating that the species barrier from cervid to humans is prion
strain-dependent and humans can be vulnerable to novel cervid prion strains.
PPo2-27:
Generation of a Novel form of Human PrPSc by Inter-species Transmission of
Cervid Prions
*** Our findings suggest that CWD prions have the capability to infect
humans, and that this ability depends on CWD strain adaptation, implying that
the risk for human health progressively increases with the spread of CWD among
cervids.
PPo2-7:
Biochemical and Biophysical Characterization of Different CWD Isolates
*** The data presented here substantiate and expand previous reports on the
existence of different CWD strains.
Envt.07:
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free
Ranging White-Tailed Deer Infected with Chronic Wasting Disease
***The presence and seeding activity of PrPTSE in skeletal muscle from
CWD-infected cervids suggests prevention of such tissue in the human diet as a
precautionary measure for food safety, pending on further clarification of
whether CWD may be transmissible to humans.
>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO
CONVERSION OF THE HUMAN PRION PROTEIN<<<
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
Wednesday, January 01, 2014
Molecular Barriers to Zoonotic Transmission of Prions
*** chronic wasting disease, there was no absolute barrier to conversion of
the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay
when seeded with CWD, resembles that found in the most common human prion
disease, namely sCJD of the MM1 subtype.
Monday, January 13, 2014
Prions in Variably Protease-Sensitive Prionopathy: An Update Pathogens 2013
Pathogens 2013, 2, 457-471; doi:10.3390/pathogens2030457
Monday, November 3, 2014
The prion protein protease sensitivity, stability and seeding activity in
variably protease sensitive prionopathy brain tissue suggests molecular overlaps
with sporadic Creutzfeldt-Jakob disease
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what it ???
Tuesday, November 04, 2014
The pathological and molecular but not clinical phenotypes are maintained
after second passage of experimental atypical bovine spongiform encephalopathy
in cattle
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and
VPSPr PRIONPATHY
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
Tuesday, May 26, 2015
Minimise transmission risk of CJD and vCJD in healthcare settings
Last updated 15 May 2015
Saturday, February 21, 2015
Design of Endoscopic Retrograde Cholangiopancreatography (ERCP)
Duodenoscopes May Impede Effective Cleaning: FDA Safety Communication
Thursday, January 22, 2015
*** Transmission properties of atypical Creutzfeldt-Jakob disease: a clue
to disease etiology? ***
==================================
Tuesday, August 4, 2015
*** FDA U.S. Measures to Protect Against BSE ***
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics
of BSE in Canada Singeltary reply ;
Monday, November 3, 2014
*** now, from all the consumption and exposure above, now think iatrogenic
cjd tse prion at a hospital near you, what if?
Thursday, August 13, 2015
Iatrogenic CJD due to pituitary-derived growth hormone with genetically
determined incubation times of up to 40 years
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of
Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD
20892.
Stereotactic multicontact electrodes used to probe the cerebral cortex of a
middle aged woman with progressive dementia were previously implicated in the
accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger
patients. The diagnoses of CJD have been confirmed for all three cases. More
than two years after their last use in humans, after three cleanings and
repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were
implanted in the cortex of a chimpanzee. Eighteen months later the animal became
ill with CJD. This finding serves to re-emphasise the potential danger posed by
reuse of instruments contaminated with the agents of spongiform
encephalopathies, even after scrupulous attempts to clean them.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract
Thursday, August 04, 2016
MEETING ON THE FEASIBILITY OF CARRYING OUT EPIDEMIOLOGICAL STUDIES ON
CREUTZFELDT JAKOB DISEASE 1978 THE SCRAPIE FILES IN CONFIDENCE CONFIDENTIAL SCJD
Monday, May 02, 2016
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
TOKYO
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what it ???
Monday, November 3, 2014
The prion protein protease sensitivity, stability and seeding activity in
variably protease sensitive prionopathy brain tissue suggests molecular overlaps
with sporadic Creutzfeldt-Jakob disease
Sunday, August 09, 2009
*** CJD...Straight talk with...James Ironside...and...Terry Singeltary...
2009 ***
Tuesday, August 18, 2009
*** BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009 ***
early days BSE and nvCJD UK
5.195 Among occupational groups exposed to BSE, farmers remain unusual in
having such an excess over the incidence of CJD for the population as a whole.
No cases of CJD have been reported amount veterinarians exposed to BSE. Four
people in the meat industry (butchers, abattoirs, rendering plants, etc) have
been reported to have vCJD.386 The present evidence has been accepted by some as
reassuring in that such occupations may not pose as serious a risk as might have
been expected.
This was not simply another farmer but the third farmer......
suspect case of CJD in a farmer who has had a case of BSE in his beef
suckler herd.
http://web.archive.org/web/20030331213802/http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf
cover-up of 4th farm worker ???
http://web.archive.org/web/20030516083454/http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf
http://web.archive.org/web/20030330175323/http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf
CONFIRMATION OF CJD IN FOURTH FARMER
now story changes from;
SEAC concluded that, if the fourth case were confirmed, it would be
worrying, especially as all four farmers with CJD would have had BSE cases on
their farms.
to;
This is not unexpected...
was another farmer expected?
http://web.archive.org/web/20030728074919/http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf
4th farmer, and 1st teenager
2. snip...
Over a 5 year period, which is the time period on which the advice from
Professor Smith and Dr. Gore was based, and assuming a population of 120,000
dairy farm workers, and an annual incidence of 1 per million cases of CJD in the
general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an
individual in the general population to develop CJD. Using the actual current
annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5
TIMES.
3. You will recall that the advice provided by Professor Smith in 1993 and
by Dr. Gore this month used the sub-population of dairy farm workers who had had
a case of BSE on their farms - 63,000, which is approximately half the number of
dairy farm workers - as a denominator. If the above sums are repeated using this
denominator population, taking an annual incidence in the general population of
1 per million the observed rate in this sub-population is 10 TIMES, and taking
an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE''
SCENARIO) than that in the general population...
http://web.archive.org/web/20030516181226/http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf
CJD FARMERS WIFE 1989
20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19
year old died from sCJD in France in 1985. There is no evidence of an iatrogenic
cause for those cases....
http://web.archive.org/web/20030330212925/http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf
THE COVER UP OF MAD COW DISEASE IN FARMERS, FARMERS WIVES, AND VICKY
RIMMER, THE DAY MAD COW SCIENCE CHANGED $$$
Monday, May 19, 2008
*** SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND
ABATTOIRS ***
DOES ANYONE BESIDES ME SEE A PATTERN YET ???
Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic
CJD, whatever the hell that is. and there have been 16 year old die from
sporadic CJD in the USA as well.
SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly
are expendable, pets and kids are not.
Science was dictated by 'big buisness' after the Vickey Rimmer case with
the ukbsenvcjd only myth.
and there have been 16 year old die from sporadic CJD in the USA as
well.
snip...
I have interviewed Mrs Rimmer at my constituency surgery
IF there is nothing to hide, why is there so much SECRECY? WHY is the
Government and other Bodies trying to stop any CHANCE OF PEOPLE CONNECTING THE
TWO DISEASES. The B.S.E. problem is obvious, but if the correct measures are
taken, surely the problem could be contained, however, as it stands the lack of
investigation and interest of the possibility of B.S.E. and C.J.D. being linked
is open for speculation and surely someone has to account for peoples lives! WHY
is so much trouble being taken to convice people that B.S.E. and C.J.D. are not
linked? Guilty Conscience perhaps ? - or cover up?
HOUSE OF COMMONS
FROM BARRY JONES, M.P.
22 FEBRUARY 1994
http://web.archive.org/web/20040526010710/http://www.bseinquiry.gov.uk/files/yb/1994/02/22009001.pdf
Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.
(now story changes that biopsy shows she does not have CJD...tss)
http://web.archive.org/web/20030511045541/http://www.bseinquiry.gov.uk/files/yb/1994/06/06004001.pdf
now story changes to ;
Advice
7. The Parliamentary Secretary is invited to note the recent statements
made on __________ and the present position which remains that CJD cannot be
confirmed, in this case at this stage.
http://web.archive.org/web/20030510165315/http://www.bseinquiry.gov.uk/files/yb/1994/06/08004001.pdf
3. The Medical Director at ___________________ Hospital advised the
Department on 6 June that the results of ___________________ brain biopsy had
been received and that it showed NO EVIDENCE OF CJD. ______________ Hospital
subsequently issued a statement to the press to this effect and this was
publicised widely in the press (doc 1). News coverage which followed suggested
that the statement made by ________________ Hospital had been misleading (doc
2). Enquires have been made of the Medical Director at _______________ Hospital
who has CONFIRMED THAT THE STATEMENT ISSUED BY THE HOSPITAL WAS ISSUED IN ERROR.
The facts are that two pathology reports on the same piece of brain tissue were
recieved. The first report indicated that CJD was unlikely, The second report
indicated that CJD was possible, PERHAPS EVEN LIKELY, but that no definitive
diagnosis could be made before a post mortem was undertaken.
http://web.archive.org/web/20030511023028/http://www.bseinquiry.gov.uk/files/yb/1994/06/08006001.pdf
MAD COW MEAL DESTROYED MY DAUGHTERS LIFE
A TEENAGE GIRL may have caught the human form of MAD COW DISEASE by eating
a contaminated burger it was claimed last night.
VICKY RIMMER, 16, has the killer Creutzfeldt-Jakob disease (CJD).
http://web.archive.org/web/20030510205841/http://www.bseinquiry.gov.uk/files/yb/1994/01/25007001.pdf
GIVE ME BACK MY LIFE
THEY BEGGED ME TO HUSH IT UP – GRAN’S AGONY
http://web.archive.org/web/20040521224258/http://www.bseinquiry.gov.uk/files/yb/1994/01/25008001.pdf
HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''
http://web.archive.org/web/20031025182000/http://www.bseinquiry.gov.uk/files/yb/1994/01/25009001.pdf
WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S
BODY
http://web.archive.org/web/20030513071650/http://www.bseinquiry.gov.uk/files/yb/1994/01/25010001.pdf
Wednesday, October 09, 2013
*** WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY,
£41,078,281 in compensation ***
Friday, October 23, 2015
*** CJD FOUNDATION CREUTZFELDT JAKOB DISEASE TSE PRION QUESTIONNAIRE UPDATE
OCTOBER 2015 ***
DEATH CERTIFICATES, CJD, AND CODING ERRORS
routine passive mortality CJD surveillance USA ?
THIS has been proven not to be very useful in the U.K.;
THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)
snip...
One reason for this was the _inaccuracy_ in coding of cases correctly
certified as CJD Coding is carried out by staff who are not medically qualified
and it is not surprising that coding errors occur in the processing of large
numbers of certificates. In 1982, 12,000 certificates per week were processed at
the office of population censuses and surveys by 15 coders and 6 checkers
(Alderson et al., 1983). The occurrence of both inter- and intra-observer coding
errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH
certification and coding discovered in this study _support_ the introduction of
a more accurate system of death certificates and a more detailed and specific
coding system...
snip...
Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R.
Will
snip...
IDENTIFICATION OF CASES
Cases of CJD may be identified from death certificates, but this alone is
unlikely to provide adequate monitoring. ERRORS are made in certification and
diagnosis; in the Oxford study death certificates were obtained on a series of
known confirmed cases and CJD was mentioned in only 66% of certificates. In
another series of 175 certified cases, 42 patients were judged not to have
suffered from CJD after examination of case notes (7)...
full text;
http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf
2001 Deepthroat to Singeltary
The most frightening thing I have read all day is the report of Gambetti's
finding of a new strain of sporadic cjd in young people.........Dear God, what
in the name of all that is holy is that!!! If the US has different strains of
scrapie.....why???? than the UK...then would the same mechanisms that make
different strains of scrapie here make different strains of BSE...if the
patterns are different in sheep and mice for scrapie.....could not the BSE be
different in the cattle, in the mink, in the humans.......I really think the
slides or tissues and everything from these young people with the new strain of
sporadic cjd should be put up to be analyzed by many, many experts in
cjd........bse.....scrapie Scrape the damn slide and put it into
mice.....wait.....chop up the mouse brain and and spinal cord........put into
some more mice.....dammit amplify the thing and start the damned
research.....This is NOT rocket science...we need to use what we know and get
off our butts and move....the whining about how long everything takes.....well
it takes a whole lot longer if you whine for a year and then start the
research!!! Not sure where I read this but it was a recent press release or
something like that: I thought I would fall out of my chair when I read about
how there was no worry about infectivity from a histopath slide or tissues
because they are preserved in formic acid, or formalin or formaldehyde.....for
God's sake........ Ask any pathologist in the UK what the brain tissues in the
formalin looks like after a year.......it is a big fat sponge...the agent
continues to eat the brain ......you can't make slides anymore because the agent
has never stopped........and the old slides that are stained with Hemolysin and
Eosin......they get holier and holier and degenerate and continue...what you
looked at 6 months ago is not there........Gambetti better be photographing
every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come
of it and there is not a damned thing anyone can do about it. Don't even hint at
it as it will be denied and laughed at.......... USDA is gonna do as little as
possible until there is actually a human case in the USA of the nvcjd........if
you want to move this thing along and shake the earth....then we gotta get the
victims families to make sure whoever is doing the autopsy is credible,
trustworthy, and a saint with the courage of Joan of Arc........I am not
kidding!!!! so, unless we get a human death from EXACTLY the same form with
EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any
action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to
international travel, international food, etc. etc. etc. etc. etc. They will go
so far as to find out if a sex partner had ever traveled to the UK/europe, etc.
etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth.
They have all the cards, all the money, and are willing to threaten and carry
out those threats....and this may be their biggest downfall...
Thanks as always for your help.
(Recently had a very startling revelation from a rather senior person in
government here..........knocked me out of my chair........you must keep
pushing. If I was a power person....I would be demanding that there be a least a
million bovine tested as soon as possible and agressively seeking this disease.
The big players are coming out of the woodwork as there is money to be made!!!
In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the
burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously....BSE will
NEVER be found in the US! As for the BSE conference call...I think you did a
great service to freedom of information and making some people feign
integrity...I find it scary to see that most of the "experts" are employed by
the federal government or are supported on the "teat" of federal funds. A scary
picture! I hope there is a confidential panel organized by the new government to
really investigate this thing.
You need to watch your back........but keep picking at them.......like a
buzzard to the bone...you just may get to the truth!!! (You probably have more
support than you know. Too many people are afraid to show you or let anyone else
know. I have heard a few things myself... you ask the questions that everyone
else is too afraid to ask.)
==============end...TSS=============
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
Saturday, April 16, 2016
APHIS [Docket No. APHIS-2016-0029] Secretary's Advisory Committee on Animal
Health; Meeting May 2, 2016, and June 16, 2016 Singeltary Submission
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle
Over the next 8-10 weeks, approximately 40% of all the adult mink on the
farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or
dead dairy cattle...
In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - APRIL-MAY 1989 - G A H Wells
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr. A Thiermann showed the picture in the
''Independent'' with cattle being incinerated and thought this was a fanatical
incident to be avoided in the US at all costs. ...
”The occurrence of CWD must be viewed against the contest of the locations
in which it occurred. It was an incidental and unwelcome complication of the
respective wildlife research programmes. Despite it’s subsequent recognition as
a new disease of cervids, therefore justifying direct investigation, no specific
research funding was forthcoming. The USDA veiwed it as a wildlife problem and
consequently not their province!” ...page 26.
Monday, May 09, 2016
A comparison of classical and H-type bovine spongiform encephalopathy
associated with E211K prion protein polymorphism in wild type and EK211 cattle
following intracranial inoculation
Tuesday, June 07, 2016
*** Comparison of two US sheep scrapie isolates supports identification as
separate strains ***
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Thursday, August 18, 2016
*** PROCEEDINGS ONE HUNDRED AND Nineteenth ANNUAL MEETING of the USAHA BSE,
CWD, SCRAPIE, PORCINE TSE PRION October 22 28, 2015 ***
Tuesday, August 9, 2016
*** Concurrence with OIE Risk Designations for Bovine Spongiform
Encephalopathy [Docket No. APHIS-2015-0055]
Saturday, July 23, 2016
*** BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING,
AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016
Tuesday, July 26, 2016
*** Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY
2016
Saturday, July 16, 2016
*** Importation of Sheep, Goats, and Certain Other Ruminants [Docket No.
APHIS-2009-0095]RIN 0579-AD10
WITH great disgust and concern, I report to you that the OIE, USDA, APHIS,
are working to further legalize the trading of Transmissible Spongiform
Encephalopathy TSE Pion disease around the globe.
THIS is absolutely insane. it’s USDA INC.
Monday, June 20, 2016
*** Specified Risk Materials SRMs BSE TSE Prion Program ***
TSS
posted by Terry S. Singeltary Sr. at
4:23 PM
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