Wednesday, September 10, 2014

Creutzfeldt-Jakob disease (CJD) biannual update (August 2014), with updated guidance on decontamination of gastrointestinal endoscopy equipment

Research and analysis

Creutzfeldt-Jakob disease (CJD) biannual update (August 2014), with updated guidance on decontamination of gastrointestinal endoscopy equipment

Published 8 August 2014

This six-monthly report provides an update on the enhanced surveillance of potential iatrogenic (healthcare-acquired) exposures to Creutzfeldt-Jakob Disease (CJD). The data is correct as at 30 June 2014. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU).
Following the enhanced surveillance report, an update on the guidance for the decontamination of equipment for gastrointestinal endoscopy is presented.

1. Monitoring of patients ‘at increased risk’ of CJD

Individuals who have been identified as at increased risk of CJD as a consequence of their medical care are informed of their exposure and asked to follow public health precautions to avoid potentially transmitting the infection to others. They are also followed-up to help determine the risks of CJD transmission to patients through different routes and to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.
Public health follow-up activities include clinical monitoring, general practitioner (GP) updates, and post mortem investigations to determine whether asymptomatic individuals in these groups have been infected with the CJD agent. Some individuals also provide blood or tissue specimens for research purposes. A number of different organisations are involved in these activities: Public Health England (formerly the Health Protection Agency), Health Protection Scotland (HPS), UCL Institute of Child Health/Great Ormond Street Hospital (ICH), NHS Blood and Transplant (NHSBT), National CJD Research and Surveillance Unit (NCJDRSU), National Prion Clinic (NPC), and the UK Haemophilia Centre Doctors’ Organisation (UKHCDO).
The PHE CJD Section coordinates the collation of data on individuals identified as at increased risk of CJD, and who have been informed of this. These individuals are followed up through public health monitoring and research activities by different organisations.
The PHE CJD Section currently holds data on the following groups of ‘at risk’ patients:
  • recipients of blood components from donors who subsequently developed vCJD
  • blood donors to individuals who later developed vCJD
  • other recipients of blood components from these blood donors
  • recipients of certain plasma products between 1990 and 2001 (non-bleeding disorder patients)
  • certain surgical contacts of patients diagnosed with CJD
  • highly transfused recipients.
Data on the following risk groups are not held by PHE, but are held by other organisations:
  • bleeding disorder patients who received plasma products between 1990 and 2001 (UKHCDO)
  • recipients of human derived growth hormone before 1985 (ICH)
  • patients who could have received a dura mater graft before August 1992 (data not currently collected)
  • people who have been treated with gonadotrophin sourced from humans before 1973 (data not currently collected)
  • family risk of inherent prion disease (NPC).
The data from the UKHCDO are likely to be an underestimate of the true number of ‘at risk’ patients with bleeding disorders who received UK-sourced clotting factors, as there was incomplete reporting of identified ‘at risk’ patients by haemophilia centres to the UKHCDO database. Notified ‘at risk’ patients are given the option of removing their details from the UKHCDO database, and are then removed from the ‘at risk’ totals.
The data on ‘at risk’ patients who received human-derived human growth hormone held by the ICH is a slight underestimate of the total as a small number of these patients are not included in the ICH follow-up.
Table 1. Summary of all ‘at risk’ groups on which data are collected (data correct as at 30 June 2014)
At risk Group Identified as ‘at risk’ Number notified as being ‘at risk’   Cases Asymptomatic infections [b]
    All Alive    
Recipients of blood from donors who later developed vCJD 67 27 14 3 1
Blood donors to individuals who later developed vCJD 112 108 104
Other recipients of blood components from these donors 34 32 [c] 19 [c]
Plasma product recipients (non-bleeding disorders) who received UK sourced plasma products 1980-2001 11 10 4
Certain surgical contacts of patients diagnosed with CJD 196 161 [d] 140 [e]
Highly transfused patients 11 10 5
Total for ‘at risk’ groups where PHE holds data 431 348 [f] 286 [f] 3 1
Patients with bleeding disorders who received UK-sourced plasma products 1980-2001 [a] 3,977 National information incomplete National information incomplete 1
Recipients of human-derived growth hormone [a] 1,883 1,883 1,503 75
Total for all ‘at risk’ groups [a] 6,291 At least 2,231 At least 1,789 78 2
           
a. These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and a small number of patients have opted out of the central UKHCDO database. A small number of ‘at risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of ‘at risk’ growth hormone recipients have been notified. There is no central record of who has been informed.
b. An asymptomatic infection is when an individual does not exhibit any of the signs and symptoms of CJD in life but abnormal prion protein indicative of CJD infection has been found in tissue obtained from them. In these cases the abnormal prion protein was identified at post mortem.
c. One patient was notified by proxy.
d. Five of these notified by proxy.
e. Three of these were notified by proxy.
f. Includes patients notified by proxy.

2. Updated guidance on decontamination of equipment for gastrointestinal endoscopy

The British Society of Gastroenterology have updated their guidance on decontamination of equipment for gastrointestinal endoscopy [1]. The update includes revised advice for management and decontamination of endoscopes after they have been used for procedures on individuals at risk of variant CJD. This aligns with Department of Health guidance, CFPP 01-06, published in April 2013 [2], which recommended that: There is no longer a requirement to quarantine endoscopes following an “invasive” procedure on patients at risk of vCJD (with very few exceptions); a single quality assured decontamination cycle following recommended guidelines is considered sufficient, but the endoscope should be decontaminated separately from others with a single-use disinfectant; routine traceability data should be available to demonstrate thorough reprocessing; ‘Single use’ accessories should always be used in preference to reusable accessories.
The ACDP TSE specific guidance on endoscopy, Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex F [3], which builds on the recommendations from the BSG guidance has been updated.

2.1 References

  1. SG Guidance on Decontamination of Equipment for Gastrointestinal Endoscopy (2014).
  2. Management and decontamination of flexible endoscopes (CFPP 01-06) (2013): Policy and Management.
  3. Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex F (2014).

see steady rise in sporadic CJD cases, from 33 cases in 1990, to 115 cases in 2013...and please NOTE ;

 

‘’1 There are in addition a total of 9 cases of VPSPr (death in 1997(1 case), 2004(1), 2006(1), 2008(2), 2012(4)) not included in the above figures.’’

 

CREUTZFELDT-JAKOB DISEASE IN THE UK (By Calendar Year)

 

REFERRALS OF SUSPECT CJD

 

DEATHS OF DEFINITE AND PROBABLE CJD

 

Year

 

Referrals

 

Year

 

Sporadic1

 

Iatrogenic

 

Genetic2

 

vCJD

 

Total Deaths

 

1990 [53]† 1990 28 5 0 - 33

 

1991 75 1991 31 1 4 - 36

 

1992 96 1992 45 2 6 - 53

 

1993 79 1993 36 4 7 - 47

 

1994 119 1994 54 1 8 - 63

 

1995 87 1995 35 4 5 3 47

 

1996 133 1996 40 4 6 10 60

 

1997 163 1997 59 6 6 10 81

 

1998 155 1998 64 3 5 18 90

 

1999 170 1999 62 6 2 15 85

 

2000 178 2000 50 1 3 28 82

 

2001 179 2001 58 4 6 20 88

 

2002 164 2002 73 0 5 17 95

 

2003 162 2003 79 5 6 18 108

 

2004 114 2004 50 2 6 9 67

 

2005 124 2005 67 4 13 5 89

 

2006 112 2006 68 1 9 5 83

 

2007 119 2007 64 2 10 5 81

 

2008 150 2008 86 5 5 2 98

 

2009 153 2009 80 2 8 3 93

 

2010 150 2010 85 3 7 3 98

 

2011 158 2011 90 4 14 5 113

 

2012 127 2012 93 5 11 0 109

 

2013 149 2013 104 2 8 1 115

 

2014* 76 2014 51 1 5 0 57

 

Total Referrals 3245 Total Deaths 1552 77 165 177 1971

 

† Referral figure for 1990 is from 1 May onwards * As at 1st September 2014

 

Summary of vCJD cases

 

Deaths

 

Deaths from definite vCJD (confirmed): 122

 

Deaths from probable vCJD (without neuropathological confirmation): 55

 

Deaths from probable vCJD (neuropathological confirmation pending): 0

 

Number of deaths from definite or probable vCJD (as above): 177

 

Alive

 

Number of definite/probable vCJD cases still alive: 0

 

Total number of definite or probable vCJD (dead and alive): 177

 

1 There are in addition a total of 9 cases of VPSPr (death in 1997(1 case), 2004(1), 2006(1), 2008(2), 2012(4)) not included in the above figures.

 

2 includes all genetic prion disease, including GSS.

 


 

archive of annual reports

 


 


 

Annex F – Endoscopy

 

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex F

 

Published: September 2004

 

Revised and updated: January 2014

 

Annex F

 

ENDOSCOPY

 

Summary of advice

 

Annex F provides the definitive UK guidance on decontamination of flexible endoscopes for TSE infection prevention and control.

 

The specific recommendations in this guidance are complementary to national guidance on all aspects of endoscope decontamination such as Choice Framework for local Policy and Procedures 01-06 and the British Society of Gastroenterology (BSG) Guidelines for Decontamination of Equipment for Gastrointestinal Endoscopy.

 

Annex F provides specific advice for the management of instruments used in all types of endoscopic procedures. This advice differs depending on the type of CJD that a patient has been diagnosed with, or for which symptoms are being investigated, and for those who are asymptomatic but for whom an increased risk of developing disease has been identified.

 

Paragraphs F4 to F27 set out the guidance for each circumstance in detail, while summary advice is provided in table F1 and table F2a.

 

Endoscopes currently in quarantine

 

Advice is given below regarding endoscopes that have been held in quarantine following previous use on patients who are at increased risk of vCJD.

 

Endoscopes that have been placed into quarantine on or after 1 January 2010, assuming not used to treat one of the patient categories described at paragraphs F20 to F23 should be reviewed as follows:

 

1) Was the endoscope properly decontaminated using a validated process prior to quarantine?

 

2) Is there tracking to demonstrate the above?

 

3) Has the scope been stored properly whilst in quarantine (in a drying cabinet or at least positioned vertically, not coiled up in a case)?

 

If all the above are met, the endoscope can be returned to use. If the endoscope has been out of use for more than a few months it is recommended that it is returned to the manufacturer for service and a check of handling characteristics before returning to use.

 

Previous revision date: December 2012

 

Changes new to this edition:

 

Date

 

Change

 

Notes

 

January 2014

 

Slightly modified text to reflect the wording of Table F1 and F2a

 

This change affects paragraphs: F8, F17, F18, F22 and F26

 

January 2014

 

Changes to table F1 and F2a to align with policy

 

January 2014

 

Changes to notes of table F2a to align with policy

 

January 2014

 

Change of terminology from “infection control” to “infection prevention and control”

 

Changed throughout the document as appropriate

 

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex F

 


 

Annex J - Assessment to be carried out before surgery and/or endoscopy to identify patients with, or at increased risk of, CJD or vCJD

 

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J

 

Published: 31 July 2006

 

Revised and updated: January 2014

 

ANNEX J

 

Assessment to be carried out before surgery and/or endoscopy to identify patients with, or at increased risk of, CJD or vCJD

 

Summary of advice (revised January 2014)

 

Annex J provides a clear and pragmatic way of assessing CJD and vCJD risk prior to surgery or endoscopy. Certain groups of patients have been informed that they are at increased risk of CJD or vCJD.

 

*** Therefore it is recommended that all patients about to undergo any surgery or endoscopy should be asked if they have ever been notified as at increased risk of CJD or vCJD. This recommendation is outlined in paragraphs J1 and J2.

 

In addition, patients undergoing surgery or neuro-endoscopy which may involve contact with tissues of potentially high level TSE infectivity (“high risk tissues”) should, through a set of detailed questions, be assessed for their possible CJD/vCJD risk exposure. These questions are outlined in Table J1 and paragraphs J3 to J6.

 

Previous revision date: January 2013

 

Changes new to this edition:

 

Date

 

Change

 

Notes

 

January 2014

 

Alignment of the list of people considered at increased risk of vCJD with that contained in Part 4.

 

This change affects paragraph J14.

 

January 2014

 

Change of terminology from “infection control” to “infection prevention and control”

 

Changed throughout the document as appropriate

 


 

These annexes have table at the start setting out the changes that have been included in the new version.

 

FAQs – a new question has been added regarding blood transfusion

 

My patient has a history of blood transfusion. Should my patient be considered at increased risk of CJD and/or vCJD?

 


 

Wednesday, January 15, 2014

 

*** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014

 


 

Thursday, January 23, 2014

 

Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]

 


 

Monday, January 13, 2014

 

*** Prions in Variably Protease-Sensitive Prionopathy: An Update Pathogens 2013 Pathogens 2013, 2, 457-471; doi:10.3390/pathogens2030457

 


 

Friday, January 10, 2014

 

*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

 


 

 *** 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 $

 


 

Saturday, August 14, 2010

 

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

 


 

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

 


 

VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $

 

OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS TRANSMISSIBLE IN BANK VOLES Nonno

 


 

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.

 

SOURCE PRION2012

 


 

I believe it was Gambetti et al that coined this term sporadic FFI, from some conspicuous sub-type of sporadic CJD possibly?

 

seems they could not tie it to a true FFI by diagnostic standards to date, so it was then termed a sFFI, confusing matters even worse. ...

 

A subtype of sporadic prion disease mimicking fatal familial insomnia

 


 

THIS seems to raise more questions than answers, confusing the TSEs even worse.

 

WHAT is sporadic CJD, and how many sub-types and atypical strains, phenotypes etc. will there be, arising from nothing. a spontaneous happening of sorts???

 

i think not. ...tss

 


 

Wednesday, October 27, 2010

 

A novel variant of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report

 

snip...

 

Genetic findings

 

No mutations were found in the open reading frame after sequencing the prion protein gene (PRNP). A heterozygosis methionine valine (MV) was observed in codon 129.

 

snip...

 


 

Thursday, July 10, 2008

 

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008

 

Although several subjects had family histories of dementia, no mutations were found in the PrP gene open reading frame.

 


 

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

 


 

Here we go folks. AS predicted. THIS JUST OUT !

 

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 ;

 


 


 

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. ...

 


 

Wednesday, October 27, 2010

 

A novel variant of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report

 


 

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

 


 

====================================

 

The familial mutations, Gajdusek proposed, lowered the barrier to such accidental conversion. "Thus," he wrote in 1996, "with these mutations, this ordinarily rare event becomes a ... dominant inherited trait." But Weissmann's qualification still remained to be refuted: the mutations might simply allow easier entry to a lurking virus. ...page 202 Deadly Feast

 

===================================

 

something to think about for sure.

 

but i interpret this as (1st not the gold standard, just my opinion;-), as because of certain gene mutations, one or a family, would be more susceptible to the many different strains of TSE, and the many different proven routes and sources, (which will cause different symptoms, different incubation periods from onset of clinical symptoms to death, different parts of the brain infected, etc.). in other words, it's NOT the gene mutation that CAUSES the disease, but the fact that it makes you more SUSCEPTIBLE, to the TSEs from the surrounding environment, and PLUS accumulation, i think this plays a critical role. maybe there is a one dose scenario, but i think there is more of the 'accumulators' that go clinical, than the 'one dose'. and what is the threshold to sub-clinical to clinical ?

 

anyway, just pondering out loud here.

 

also, for anyone interested, there are some studies with links to follow here ;

 


 

Sunday, August 11, 2013

 

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

 

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***

 


 

Tuesday, May 28, 2013

 

Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance

 


 

Saturday, November 16, 2013

 

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

 

Infect Control Hosp Epidemiol.

 


 

Wednesday, December 11, 2013

 

*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

 


 


 

Tuesday, March 5, 2013

 

Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)

 

FDA believes current regulation protects the public from BSE but reopens comment period due to new studies

 


 

what’s the big secret about the age and history of this poor gentleman ???

 

MAD COW COVER UP USA, THE EVIDENCE MOUNTS $$$

 

Monday, June 02, 2014

 

Confirmed Human BSE aka mad cow Variant CJD vCJD or nvCJD Case in Texas

 


 

Thursday, January 17, 2013

 

TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)

 


 

Wednesday, July 23, 2014

 

After the storm? UK blood safety and the risk of variant Creutzfeldt-Jakob Disease

 


 

Thursday, July 24, 2014

 

Government must do more to reduce risk of vCJD infection

 


 

Friday, February 14, 2014

 

Creutzfeldt-Jakob disease (CJD) biannual update (February 2014), with briefing on novel human prion disease National CJD Research and Surveillance Unit NCJDRSU

 


 

 U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001

 


 


 

Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014

 

Transmissible Spongiform Encephalopathy TSE Prion Disease have now been discovered in a wide verity of species across North America. typical C-BSE, atypical L-type BASE BSE, atypical H-type BSE, atypical H-G BSE, of the bovine, typical and atypical Scrapie strains, in sheep and goats, with atypical Nor-98 Scrapie spreading coast to coast in about 5 years. Chronic Wasting Disease CWD in cervid is slowly spreading without any stopping it in Canada and the USA and now has mutated into many different strains. Transmissible Mink Encephalopathy TME outbreaks. These Transmissible Spongiform Encephalopathy TSE Prion Disease have been silently mutating and spreading in different species in North America for decades.

 

The USDA, FDA, et al have assured us of a robust Triple BSE TSE prion Firewall, of which we now know without a doubt, that it was nothing but ink on paper. Since the 1997 mad cow feed ban in the USA, literally tons and tons of banned mad cow feed has been put out into commerce, never to return, as late as December of 2013, serious, serious breaches in the FDA mad cow feed ban have been documented. The 2004 enhanced BSE surveillance program was so flawed, that one of the top TSE prion Scientist for the CDC, Dr. Paul Brown stated ; Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

 

see ;

 


 

The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month.

 

nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment.

 

sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?

 

Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end

 

REFERENCES

 

Sunday, June 29, 2014

 

Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014

 


 

Tuesday, August 12, 2014

 

MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014

 


 

Monday, July 28, 2014

 

Mitigating the Risk of Transmission and Environmental Contamination of Transmissible Spongiform Encephalopathies 2013 Annual Report

 


 

Thursday, October 25, 2012

 

Current limitations about the cleaning of luminal endoscopes and TSE prion risk factors there from

 

Article in Press

 


 

Saturday, January 16, 2010

 

Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al

 

Evidence For CJD/TSE Transmission Via Endoscopes

 

From Terry S. Singletary, Sr flounder@wt.net 1-24-3

 

Terry S. Singeltary Sr., P.O. BOX 42, Bacliff, Texas 77518 USA

 


 

Professor Michael Farthing wrote:

 

Louise Send this to Bramble (author) for a comment before we post. Michael

 

snip...

 

Evidence For CJD/TSE Transmission Via Endoscopes

 

From Terry S. Singletary, Sr flounder@wt.net 1-24-3

 

I have researched human/animal TSEs now for over 5 years due to the death of my Mother from the Heidenhain Variant Creutzfeldt Jakob disease, one of six - known - variants of the infamous 'sporadic' CJD.

 

I did a little survey several years ago about CJD and ENDOSCOPY in 2001, and then went there again when another article was released recently. However, they seemed to only be concerned with the vCJD strain and risk from endoscopy equipment.

 

My concerns are if vCJD can be transmitted by blood, and there are now 6 variants of the infamous sporadic CJDs that they are documenting to date, how do they know that none of these 6 variants will not transmit the agent (prion) via blood?...especially since the sporadic CJDs are the only ones documented to date to transmit via the surgical arena and now that the CWD is spreading more and more, who knows about the cattle?

 

I would always read this study and it would bring me back to reality as to how serious/dangerous this agent is in the surgical/medical arena. You might want to read this short abstract from the late, great Dr. Gibbs twice, and let it really sink in. And please remember while reading some of these transmission studies, that most all, if not ALL these agents transmit freely to primates. Humans, of course, are primates.

 

Regarding claims that:

 

'Well, it has never been documented to transmit to humans."

 

There are two critical factors to think about:

 

A. CJD/TSEs in the USA are NOT reportable in most states and there is NO CJD/TSE questionnaire for most victims and their families, and the one they are now issuing asks absolutely nothing about route and source of the (prion) agent, only how the disease was diagnosed. Furthermore, the elderly are only very rarely autopsied, ie looking for Alzheimer's or 'FAST Alzheimer's' OR prion disease-related factors and phenomena, such as heart failure caused by disease.

 

B. It is unethical and against the law to do transmission studies of TSEs to humans, they are 100% FATAL.

 

I suggest you read these case studies about medical arena CJD transmission very carefully:

 

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

 

Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

 

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.

 


 

Tissue Infectivity and TSEs (brain = high / rectum = medium)

 

snip...see full text ;

 

Evidence For CJD TSE Transmission Via Endoscopes 1-24-3

 

re-Singeltary to Bramble et al

 

Evidence For CJD/TSE Transmission Via Endoscopes

 

From Terry S. Singletary, Sr flounder@wt.net 1-24-3

 


 

TSS

Wednesday, September 03, 2014

Coexistence of mixed phenotype Creutzfeldt-Jakob disease, Lewy body disease and argyrophilic grain disease plus histological features of possible Alzheimer's disease: A multi-protein disorder in an autopsy case

CASE REPORT

 

Coexistence of mixed phenotype Creutzfeldt-Jakob disease, Lewy body disease and argyrophilic grain disease plus histological features of possible Alzheimer's disease: A multi-protein disorder in an autopsy case

 

Iván Fernández-Vega1,2,*, Javier Ruiz-Ojeda3, Ramon A. Juste4, Maria Geijo4, Juan Jose Zarranz5, Jose Luis Sánchez Menoyo3, Ikerne Vicente-Etxenausia2, Jennifer Mediavilla-García2 and Isabel Guerra-Merino1,2

 

Article first published online: 3 SEP 2014

 

DOI: 10.1111/neup.12150

 

© 2014 Japanese Society of Neuropathology

 

Issue

 

Cover image for Vol. 34 Issue 4

 

Neuropathology

 

Early View (Online Version of Record published before inclusion in an issue)

 

Keywords:

 

argyrophilic grain disease; Creutzfeldt-Jakob disease; immunohistochemistry; Lewy body disease; neurodegeneration

 

 We report hereby an autopsy case of sporadic mixed phenotype CJD without hereditary burden and a long-term clinical course. An 80-year old man was diagnosed with mild cognitive impairment 27 months before death, caused by bronchopneumonia and severe respiratory impairment. During this time, the patient developed gradual mental deterioration, some sleeping problems and myoclonus. Other clinical manifestations were progressive gait problems, language deterioration, presence of primitive reflexes and irritability. In keeping with those symptoms, a rapidly evolving dementia was clinically suspected. Cerebrospinal fluid test for 14-3-3 protein was negative. However, an abnormal EEG and MRI at end-stage of disease were finally consistent with CJD. Post-mortem examination revealed a massive cortical neuronal loss with associated reactive astrocytosis, also evident in the white matter. Diffuse spongiform changes involving some basal ganglia, especially medial thalamus, some troncoencephalic nuclei, mainly inferior olivary nucleus and the molecular layer of the cerebellum were seen. Immunorreactive deposits for anti-prion protein antibody were present at different areas of the CNS. Additionally, Lewy bodies were observed at the brainstem and amygdala. Furthermore, argirophilic grains together with oligodendroglial coiled bodies and pre-tangle inclusions in the neurons from the limbic system containing hyperphosphorylated 4R tau were noted. To the best of our knowledge, this is the first case of CJD combined with Lewy body disease and argirophilic grain disease. Furthermore, we believe this case is an extremely rare combination of MM2-cortical-type and MM2-thalamic-type sporadic CJD (sCJD), which explains the broad spectrum of MM2-type sCJD findings and symptoms. Moreover, histological features of possible Alzheimer's disease were also reported.

 


 

 

Wednesday, August 27, 2014

 

Highly sensitive detection of small ruminant BSE within TSE mixes by serial Protein Misfolding Cyclic Amplification

 


 

 

TSE PRION URL SOURCE LINK BLOGS TSS

 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 


 

 

 

TSS

Monday, August 18, 2014

Bilateral Hearing Loss Heralding Sporadic Creutzfeldt-Jakob Disease: A Case Report and Literature Review

September 2014 - Volume 35 - Issue 8 - p 1327-1329 doi: 10.1097/MAO.0000000000000485 Sensorineural Hearing Loss & Tinnitus
 
Bilateral Hearing Loss Heralding Sporadic Creutzfeldt-Jakob Disease: A Case Report and Literature Review
 
Salazar, Richard*; Cerghet, Mirela†; Ramachandran, Virginia‡
 
Supplemental Author Material Collapse Box Abstract Objective
 
To report a case of sporadic Creutzfeldt-Jakob disease (CJD) with bilateral hearing loss at onset and literature review of the scarce cases of CJD with similar audiologic manifestations at presentation.
 
Case Report
 
A 67-yr-old man presented to the hospital for evaluation of rapid progression of behavioral decline, unsteady gait, and bilateral hearing loss. Three months before admission, he abruptly developed bilateral hypoacusis without associated tinnitus or vertigo. Shortly after, his family noted an ataxic gait and behavioral changes, for example, paranoid delusions. Initial workup, including a complete autoimmune panel and heavy metals, infectious, toxicology, and paraneoplastic panel (e.g., anti-Hu, anti-VGKC), was conducted. Electroencephalography revealed diffuse generalized slowing without periodic complexes. The presence of distortion product otoacoustic emissions bilaterally was consistent with normal cochlear function, suggesting a retrocochlear origin for symptoms of hearing loss. In the meantime, the patient developed startle myoclonus. The brain magnetic resonance imaging demonstrated asymmetric cortical ribbon along with T2 FLAIR signal hyperintensities of bilateral basal ganglia. Later on, the protein 14-3-3 in the cerebrospinal fluid came back positive, which supported the diagnosis of CJD. Only three cases of CJD with deafness at onset have been published: one sporadic, associated with symptoms suggestive of polyneuropathy; and the other two familial, with the E200K mutation. One presented with symptoms of polyneuropathy and the other with typical features.
 
Conclusion
 
This case illustrates the phenotypic variability of presentation of CJD in a patient with hearing loss as the initial manifestation. In patients with subacute bilateral hypoacusis and signs of dementia, the differential diagnosis of CJD must be taken into consideration.
 
Copyright © 2014, Otology & Neurotology, Inc.
 
 
 
SepOct2012 | Audiology Today 53
 
The Case of Encephalopathy Presenting as Hearing Loss
 
By Emily Na irn, Virginia Ramacha ndran, and Brad A. Stach
 
Case History A 67-year-old male was seen in the emergency department, accompanied by his family. The patient was unable to communicate and could not give his own case history information. It was reported that both the patient and his wife developed flulike symptoms about two months earlier. His wife recovered, but the patient reportedly woke up one morning shortly after contracting the illness and stated that he was having “difficulty hearing.” At this time he also complained of a headache at the back of his head and base of his neck. Over the course of the next two months he began to experience confusion, gait problems, slurred speech, weight loss, emotional lability, and eventually, hallucinations. The patient’s family noticed a distinct progressive decline in hearing ability during this time, with the patient talking loudly. Due to the severity and progression of symptoms, the patient’s daughter, who lives in the United States, flew to his home in Central America, escorted her father back to the United States, and brought him immediately to the emergency department. The treating physician reported that the patient was “deaf” and was speaking loudly. At this time, the patient was admitted with a working diagnosis of “encephalitis” (inflammation of the brain).
 
SNIP...
 
And the Diagnosis Is… Creutzfeldt-Jakob disease
 
worsening hearing loss were instrumental in shaping the initial diagnostic picture for the treating physicians. Hearing loss is characteristic of Susac’s syndrome but rare in CJD, and this knowledge initially led the team to lean toward the former diagnosis, which has a very different prognosis and treatment strategy. In the end, audiologic evaluation results were only able to rule out extensive peripheral hearing loss, and we were 56 Audiology Today | SepOct2012 CSI: Audi ology unable to confirm or rule out the existence of retrocochlear or cortical hearing loss. As we are well aware, the existence of peripheral sensorineural hearing loss in the older population is pervasive, so the presence of such in this patient would not be diagnostically useful. However, the absence of peripheral hearing loss, while not ruling out Susac’s syndrome, can be helpful in the diagnostic equation, because it has been shown that the hearing loss characteristic of this disorder is peripheral and not central (Rennebohm et al, 2010). This case is illustrative of the challenges inherent in diagnosing rare disorders in the face of abnormal presenting symptoms. The due diligence that ultimately resulted in the determination of CJD demonstrates the value that can be found in a multidisciplinary approach to diagnosis.
 
SEE FULL TEXT ;
 
 
Familial Creutzfeldt–Jakob disease with E200K mutation presenting with neurosensorial hypoacusis
 
Creutzfeldt–Jakob disease (CJD) is characterised by rapidly progressive dementia, myoclonus, ataxia, visual disturbances and motor dysfunction. Neuropathological examination shows diffuse spongiosis, neuronal loss, gliosis and a variable degree of amyloid plaque deposition composed of protease-resistant prionic protein (PrPRES) in several locations, including the brain stem. The most frequent clinical presentations are dementia, ataxia or visual symptoms. Most of the cases are sporadic. Only 10–15% are familial, and the most frequent point mutation is E200K. The course of disease, investigation results and neuropathology are similar to those of the sporadic form of CJD. The typical clinical presentation of E200K is a rapidly progressive dementia with myoclonus and pyramidal, cerebellar or extrapyramidal signs.1 We report a familial case with an unusual onset, with deafness and polyneuropathy.
 
A 53-year-old man presented with subacute progressive bilateral hypoacusis, with tinnitus in the left ear. He was a frequent diver and the symptoms were attributed to barotrauma. During the following months, his hypoacusis worsened and he progressively developed bilateral stocking-type paresthaesia and gait instability. On examination, he was alert and cooperative, although communication was mildly affected because of the hypoacusis. He showed emotional lability; his speech was slow but fluent, and he was partially disoriented in time. Extrinsic ocular motility, cranial nerves and muscular strength were normal. Lower limbs showed mild hypertonia, right extensor plantar response, stocking-type hypoaesthaesia and hypopallaesthaesia, and moderate gait ataxia. An audiometric examination showed bilateral neurosensorial hypoacusis, and nerve conduction studies showed a mixed axonal polyneuropathy. Computed tomography and magnetic resonance imaging of the brain were normal and the electroencephalography (EEG) showed non-specific changes.
 
These symptoms led to an initial suspicion of a paraneoplastic disorder, and an examination for malignant disease was started. At this moment, we learnt that the patient’s mother had died of neuropathologically confirmed CJD; hence we conducted a CSF 14-3-3-protein detection test, which was positive. Serial EEGs showed repeated non-specific changes. Brain stem auditory evoked potentials (BAEPs) could not be performed, owing to lack of patient collaboration.
 
During the following 2 weeks, myoclonus appeared and rapidly generalised, mental status deteriorated and progressive ataxia confined the patient to bed. He died of respiratory infection 10 months after onset of symptoms.
 
Neuropathological examination showed neuronal loss, microspongiosis and astroglial and microglial proliferation predominantly in the isocortex, entorhinal cortex, and hippocampal CA1 region, striatum, amygdala and cerebellar cortex. Punctate, synaptic-like deposits of PrPRES in the cerebral and cerebellar cortices were found, as well as scattered large PrPRES deposits in the granular layer of the cerebellum. The mesencephalon did not show spongiosis, but gliosis in colliculum and periaqueductal grey matter were detected.
 
Marked neuronal loss and gliosis in the vestibular and cochlear nuclei were observed, associated with PrPRES deposition (fig 1). Western blot of PrPRES showed a three-band pattern, with an unglycated fragment migrating at 21 kDa, corresponding to PrPRES type 1. Genetic sequencing of PrP showed the presence of the E200K mutation in heterozygosis. No insertions or deletions were found in the 51–91 region. The patient was heterozygotic for the M/V polymorphism at codon 129.
 
Only two cases of CJD with deafness at onset have been published: one sporadic, associated with symptoms suggestive of polyneuropathy,2 and the other familial, with the E200K mutation and typical features.3 Other cases have been reported as presenting with auditory agnosia or with cortical deafness, and early involvement of the acoustic pathway was already detected through demonstration of progressive BAEP deterioration in patients with CJD who did not present deafness in the course of the disease.
 
The first case was that of a 71-year-old woman who presented with a sudden change in hearing and aural fullness, and a vague feeling of imbalance.2 Hearing loss and gait instability worsened rapidly Audiometry showed bilateral neurosensorial hearing loss, and BAEPs were initially normal. She later developed signs of polyneuropathy and mental deterioration, left homonynous hemianopsia and decreased vibratory and pinprick sensation. The second case was that of a 46-year-old Italian woman with the E200K mutation, who had rapidly worsening hearing loss.3 Three weeks later she developed an unstable gait, and her condition rapidly progressed to bilateral deafness, ataxia, myoclonus, pyramidal and extrapyramidal dysfunction, and mental deterioration. She died 6 months after the onset of the disease. Magnetic resonance imaging scans showed high signal areas, mostly in the caudate and putamen, EEGs showed periodic sharp-wave complexes, and protein 14-3-3 was present in the cerebrospinal fluid. Audiometric investigation showed bilateral sensorineural hearing loss, and BAEP abnormalities from the beginning seemed to confirm early brain stem involvement. The course of the illness, clinical features and EEG recordings were similar to those of the sporadic form of CJD.
 
Accumulation of PrP in the brain stem has been found to be an early pathological event in sporadic CJD, but these deposits are not necessarily associated with clinical symptoms or neuronal loss, and the brain stem seems to remain relatively resistant to the pathological process of sporadic CJD.4 Neuropathological changes in brain stem structures have been described in sporadic and familial CJD, associated with atypical onset, with gaze disorders and with fatal familial insomnia. Unfortunately, necropsy was not possible in the two patients with early deafness, and to our knowledge specific involvement of cochlear and vestibular nuclei has not been reported previously
 
Western blot of PrP showed a type 1 pattern in our case. This is the pattern usually observed in sporadic CJD M/M homozygotic at codon 129, and it has also been described in patients with the E200K mutation associated with the allele 129M in the mutated chromosome.5 It is not known whether the glycation pattern of abnormal PrP has an influence on phenotype. In our patient also, who was M/V homozygotic, the codon 129 status of the mutated allele was not investigated. This case illustrates the phenotypic variability of presentation of CJD, and describes the specific involvement of brain stem auditive nuclei in a patient with hypoacusis as the initial manifestation, thereby reflecting early brain stem involvement.
 
 
Prions 2005, pp 31-40 Dura mater related Creutzfeldt-Jakob disease in Japan: Relationship between sites of grafts and clinical features
 
T. Sato, M. Masuda, Y. Utsumi, Y. Enomoto, M. Yamada, H. Mizusawa, T. Kitamoto
 
 Summary
 
A nationwide survey documented 117 cases of Creutzfeldt-Jakob disease (CJD) transmitted from cadaveric dura mater grafts in Japan to September 2004. Of these, 110 patients were identified to have received the same type of lyophilized cadaveric dura mater graft during the period between 1978 and 1991. Incubation period from grafting to onset of symptoms varied from 16 months to 23 years, with most patients developing neurological symptoms after 2 to 15 years. We conducted q retrospective review of the full medical records of 107 of dura-related CJD (dCJD) patients. Patients were divided into two groups by site of neurosurgical or orthopedic procedure (supratentorial vs. infratentorial). Hemiparesis or hemianopsia developed as an initial manifestation in 31.9% of 47 patients with supratentorial grafts but did not develop among any of the infratentorial group (p<0 .0001="" 25.0="" brainstem="" but="" conversely="" diplopia="" div="" facial="" group="" hearing="" in="" infratentorial="" ipsilateral="" loss="" not="" noted="" nystagmus="" of="" or="" p="" paresis="" paresthesia="" seen="" supratentorium="" symptoms="" the="" were="">
 
 
Hearing loss as the initial presentation of Creutzfeldt-Jakob disease
 
July 31, 2004 by Priya Krishna, MD; Carol Bauer, MD, FACS
 
Abstract
 
Creutzfeldt-Jakob disease is a rare type of spongiform encephalopathy. Affected patients present with constitutional symptoms, which progress to severe mental deterioration and movement disorders. Dizziness is the most common early otologic symptom. Few reports in the literature describe patients with Creutzfeldt-Jakob disease who present with sudden-onset hearing loss as their primary symptom for seeking treatment. This paper discusses one such patient and reviews the clinical presentation, treatment options, and relevant literature.
 
 
J Neurol Neurosurg Psychiatry 1994;57:872-873 doi:10.1136/jnnp.57.7.872 Research Article
 
Research Article:
 
A case of Creutzfeldt-Jakob disease presenting with cortical deafness. E Tobias, C Mann, I Bone, R de Silva, J Ironside J Neurol Neurosurg Psychiatry 1994;57:7 872-873doi:10.1136/jnnp.57.7.872
 
SNIP...
 
In conclusion, this pathologically verified case of Creutzfeldt-Jakob disease presented with cortical deafness. This is, to our knowledge, the first description of such a presentation, and emphasises the variety of cortical disturbances with which this disease can present...
 
 
Sunday, December 15, 2013
 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
 
 
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
 
 
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007
 
Date: March 21, 2007 at 2:27 pm PST
 
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II
 
PRODUCT
 
Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007
 
CODE
 
Cattle feed delivered between 01/12/2007 and 01/26/2007
 
RECALLING FIRM/MANUFACTURER
 
Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
 
Firm initiated recall is ongoing.
 
REASON
 
Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.
 
VOLUME OF PRODUCT IN COMMERCE
 
42,090 lbs.
 
DISTRIBUTION
 
WI
 
___________________________________
 
PRODUCT
 
Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007
 
CODE
 
The firm does not utilize a code - only shipping documentation with commodity and weights identified.
 
RECALLING FIRM/MANUFACTURER
 
Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.
 
REASON
 
Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.
 
VOLUME OF PRODUCT IN COMMERCE
 
9,997,976 lbs.
 
DISTRIBUTION
 
ID and NV
 
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
 
 
Friday, December 14, 2012
 
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012
 
snip...
 
==================================
 
In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. ***However, this recommendation is guidance and not a requirement by law.
 
=================================
 
Animals considered at high risk for CWD include:
 
1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and
 
2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.
 
Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.
 
The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.
 
Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.
 
There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.
 
snip...
 
36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).
 
The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE).
 
Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.
 
snip...
 
The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).
 
snip...
 
In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.
 
snip...
 
In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.
 
snip...
 
Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.
 
snip...
 
 
The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month.
 
nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment.
 
sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?
 
Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end
 
REFERENCES
 
Sunday, June 29, 2014
 
Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
 
 
Tuesday, August 12, 2014
 
*** MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014 ***
 
see history of record of either the biggest cover up of mad cow disease, or one of the biggest blunders of the mad cow debacle, just my opinion of the facts...tss
 
 
Monday, June 02, 2014
 
Confirmed Human BSE aka mad cow Variant CJD vCJD or nvCJD Case in Texas
 
 
Tuesday, April 01, 2014
 
*** Questions linger in U.S. CJD cases 2005, and still do in 2014 ***
 
 
Monday, August 18, 2014
 
*** Barriers to Creutzfeldt-Jakob Disease Autopsies, California and the USA
 
 
PLEASE REMEMBER ;
 
The Akron, Ohio-based CJD Foundation said the Center for Disease Control revised that number in October of 2004 to about one in 9,000 CJD cases per year in the population group age 55 and older.
 
HAVE YOU GOT YOUR CJD QUESTIONNAIRE ASKING REAL QUESTIONS PERTAINING TO ROUTE AND SOURCE OF THE TSE AGENT THAT KILLED YOUR LOVED ONE ???
 
if not, why not...
 
Friday, November 30, 2007
 
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
 
 
 
2000 Terry S. Singeltary Sr.
 
just speaking of human TSE's; "different strains (of same disease), different routes of infection (of same disease), different infectivity levels (dose rate) of the (same disease) = different symptoms, different lengths of illness from 1st onset of illness to death, (of the same disease) + different cultures = different geographical locations = different strains (of same disease)...TSS"
 
see full text ;
 
 
p.s. for anyone wanting to read the old links, you will have to change the links with lyman in it, change lyman to madcow, leave everything else the same, and then copy and paste that link in the link window and it should work. the vegsource site was the first site I started documented the history of the usda inc. mad cow follies. it was before the blogs, before social media, and all that stuff. thank you to Jeff Nelson for allowing me to document this history, and keeping it on files for everyone to witness. this was the second message board. there was a first with Howard Lyman and Oprah Winfrey, but after the first trial, and before the second trial, they took the first website down, no history of that board is available, but when it came back after the second trial was victorious as well, then it just became a board for me to post science and history of the TSE prion aka mad cow type disease too. ...
 
SEE TRIAL;
 
 
example ;
 
'v' CJD could it be vaccineCJD???
 
 
change to ;
 
 
YOU can see the history of the USDAinc MAD COW FOLLIES HERE. remember, I was young and dumb, my manors were not to write home to mom about, but I was persistent in wanting to find the truth. ...
 
 
 
 
 
 
 
 
 
 
 
 
 
TSS