Monday, August 22, 2016

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;

 


 

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...

 


 

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

 


 

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.

 


 

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.

 


 

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.

 


 

cover-up of 4th farm worker ???

 


 


 

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?

 


 

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...

 


 

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....

 


 

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

 


 

Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.

 

(now story changes that biopsy shows she does not have CJD...tss)

 


 

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.

 


 

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.

 


 

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).

 


 

GIVE ME BACK MY LIFE

 

THEY BEGGED ME TO HUSH IT UP – GRAN’S AGONY

 


 

HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''

 


 

WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S BODY

 


 

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;

 


 

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 ***

 


 


 


 


 

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