Sunday, May 03, 2015

Unusual Case of Sporadic Creutzfeldt–Jakob Disease Subtype VV1

Unusual Case of Sporadic Creutzfeldt–Jakob Disease Subtype VV1

 

 

To the Editor: Creutzfeldt-Jakob disease is characterized by a variety of symptoms, including rapidly progressive dementia, ataxia, and myoclonus.

 

Sporadic Creutzfeldt-Jakob disease (sCJD) has a median disease duration of 6 months.1

 

Seven different molecular subtypes of sCJD hae been identified (MM1, MM2-C, MM2T, MV1, MV2, VV1, and VV2), based on prion protein gene (PRNP) codon 129 polymorphism and the prion protein type 2.

 

VV1 is the rarest molecular subtype of sCJD (only about 1% of cases). To date, reported cases of sCJD VV1 have been characterized by young age at disease onset, long duration, and progressive dementia.

 

We describe a unique case of sCJD VV1 with older age at disease onset and shorter illness duration.

 
 

CASE REPORT

 

snip...

 
 
 


Free first page
 
 
 
 
 

Wednesday, April 08, 2015

Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 Are Identical in Transmission Properties
http://creutzfeldt-jakob-disease.blogspot.com/2015/04/sporadic-creutzfeldt-jakob-disease.html
 
Saturday, April 04, 2009
 
An unusually presenting case of sCJD—The VV1 subtype Volume 111, Issue 3, Pages 282-291 (April 2009)
 
An unusually presenting case of sCJD—The VV1 subtype
 
Kaloyan S. Taneva, Mimi Yilmab
 
Received 16 November 2007; received in revised form 4 September 2008; accepted 12 September 2008.
 
Abstract
 
Creutzfeldt–Jakob disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Typically CJD presents with a triad of rapidly progressive dementia, abnormal movements (e.g., myoclonus) and electroencephalographic (EEG) changes. Recently, CJD has been subdivided into subtypes based on host genetic polymorphisms and the characteristics of the pathological prion protein. Different subtypes likely have different clinical and laboratory presentations. We describe a case of sporadic CJD of the VV1 subtype. We describe our patient's clinical symptoms, time course, laboratory workup, structural and functional neuroimaging data, EEG data and CJD biomarkers. Our patient presented with clinical symptoms atypical for CJD. Because of that, her clinical symptoms were initially attributed to psychiatric reasons. After extensive clinical and laboratory investigation, we concluded that the patient probably had CJD. Postmortem neuropathological results confirmed this clinical hypothesis. We compare our patient's clinical, laboratory and neuroimaging data to the data on typical CJD as well as the data on the few CJD VV1 cases described in the literature. We discuss our case's relevance to the diagnosis of CJD.
 
Keywords: Creutzfeldt–Jakob disease, Dementia, Neuroimaging, Magnetic resonance imaging, Electroencephalography, Biomarkers, Prion diseases a Department of Psychiatry, Massachusetts General Hospital, 55 Fruit Street, Warren 1220/Blake 11, Boston, MA 02114, United States
 
b University of Connecticut Health Center, Farmington, CT, United States
 
Corresponding author. Tel.: +1 617 726 7511; fax: +1 617 724 9155.
 
PII: S0303-8467(08)00320-X
 
doi:10.1016/j.clineuro.2008.09.017
 
© 2008 Elsevier B.V. All rights reserved.
 
 
 
rare atypical strain of sporadic cjd ??? seems these rare strains are increasing ???
 
Wednesday, February 04, 2009
 
Creutzfeldt-Jacob disease presenting as severe depression: a case report
 
 
 
A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001-2004
 
 
 
Thursday, July 10, 2008
 
A New Prionopathy update July 10, 2008
 
snip...
 
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.
 
snip...
 
Sporadic creutzfeldt-jakob disease in two adolescents
 
 
 
see full text sporadic CJD the big lie;
 
snip...
 
IT seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ???
 
lets look at the full circle, to date ;
 
 
 
Sunday, August 10, 2008
 
A New Prionopathy OR more of the same old BSe and sporadic CJD
 
 
 
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
 
snip...
 
Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
 
snip...
 
 
 
Elsevier Editorial System(tm) for The Lancet Infectious Diseases Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary
 
Abstract:
 
TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007. snip...
 
see full text 31 pages ;
 
 
 
Friday, November 30, 2007
 
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
 
 
 
Saturday, April 04, 2009
 
An unusually presenting case of sCJD—The VV1 subtype
 
 
 
Tuesday, April 21, 2015
 
Transmissible Spongiform Encephalopathy Advisory Committee TSEAC MEETING SCHEDULED FOR June 1, 2015
 
 


 
tss

Wednesday, April 08, 2015

Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 Are Identical in Transmission Properties

Research Article

 

Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 Are Identical in Transmission Properties

 

Atsushi Kobayashi1,†, Yuichi Matsuura2, Toru Iwaki3, Yasushi Iwasaki4, Mari Yoshida4, Hitoshi Takahashi5, Shigeo Murayama6, Masaki Takao6, Shinsuke Kato7, Masahito Yamada8, Shirou Mohri1 and Tetsuyuki Kitamoto1,*

 

DOI: 10.1111/bpa.12264

 

This article is protected by copyright. All rights reserved.

 

Issue

 

Cover image for Vol. 25 Issue 2

 

Brain Pathology

 

Conflicts of interest: The authors declare that they have no conflict of interest

 

 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1111/bpa.12264

 

Abstract

 

Abstract

 

The genotype (methionine, M or valine, V) at polymorphic codon 129 of the PRNP gene and the type (1 or 2) of abnormal prion protein in the brain are the major determinants of the clinicopathological features of sporadic Creutzfeldt-Jakob disease (CJD), thus providing molecular basis for classification of sporadic CJD, e.g., MM1, MM2, MV1, MV2, VV1, or VV2. In addition to these “pure” cases, “mixed” cases presenting mixed neuropathological and biochemical features have also been recognized. The most frequently observed mixed form is the co-occurrence of MM1 and MM2, namely MM1+2. However, it has remained elusive whether MM1+2 could be a causative origin of dura mater graft-associated CJD (dCJD), one of the largest subgroups of iatrogenic CJD. To test this possibility, we performed transmission experiments of MM1+2 prions and a systematic neuropathological examination of dCJD patients in the present study. The transmission properties of the MM1+2 prions were identical to those of MM1 prions because MM2 prions lacked transmissibility. In addition, the neuropathological characteristics of MM2 were totally absent in dCJD patients examined. These results suggest that MM1+2 can be a causative origin of dCJD and causes neuropathological phenotype similar to that of MM1.

 


 

friendly fire, the pass it forward mode of the TSE prion aka mad cow type disease, i.e. iatrogenic TSE prion disease.

 

with the many different species in North America with a TSE prion disease, and then the others nobody tests for i.e. feline, canine, just for a few examples, and the exposure there from via consumption, casual contact, or the many other potential exposure possibilities, what about friendly fire there from ???

 

it would be nice to be able to start differentiating the many causes of the sporadic CJDs TSE prion disease, and I believe that to be iatrogenic.

 

all sporadic CJD Creutzfeldt Jakob Disease Transmissible Spongiform Encephalopathy TSE Prion disease means, from UNKNOWN CAUSE, simply meaning the route and source of the TSE prion agent is not known to date. there are many different strain or phenotype of the so called sporadic or spontaneous CJD.

 

it will be costly to the industry to differentiate the many potential causes and sources of sCJD. who will prevail, the industry or the consumer $$$

 

 

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...

 


 


 

Thursday, August 12, 2010

 

Seven main threats for the future linked to prions

 

First threat

 

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

 

*** 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.

 

Second threat

 

snip...

 


 

 2014

 

***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

 

***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.

 

*** Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

 

snip...

 


 


 


 

Tuesday, November 04, 2014

 

Towards an Age-Dependent Transmission Model of Acquired and Sporadic Creutzfeldt-Jakob Disease

 


 

Thursday, September 18, 2014

 

Development of Dose-Response Models of Creutzfeldt-Jakob Disease Infection in Nonhuman Primates for Assessing the Risk of Transfusion-Transmitted variant Creutzfeldt-Jakob Disease

 


 

Tuesday, December 30, 2014

 

TSEAC USA Reason For Recalls Blood products, collected from a donors considered to be at increased risk for Creutzfeldt-Jakob Disease (CJD), were distributed END OF YEAR REPORT 2014

 


 

Sunday, February 08, 2015

 

FDA SCIENCE BOARD TO THE FOOD AND DRUG ADMINISTRATION BOVINE HEPARIN BSE CJD TSE PRION Wednesday, June 4, 2014

 


 

Thursday, March 26, 2015

 

Variant CJD and blood transfusion: are there additional cases?

 

Vox Sanguinis (2014) 107, 220–225 ORIGINAL PAPER © 2014 International Society of Blood Transfusion DOI: 10.1111/vox.12161

 


 

Saturday, March 21, 2015

 

Canada and United States Creutzfeldt Jakob TSE Prion Disease Incidence Rates Increasing

 


 

Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease

 

Received July 24, 2014; Accepted September 16, 2014; Published November 3, 2014

 


 

Singeltary comment Iatrogenic Alzheimer’s, a TSE prion disease, what if ?

 


 

Friday, January 10, 2014

 

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

 


 


 

 some old history on Endoscopy equipment and CJD TSE Prion concerns ; 1999

 

Subject: CJD * Olympus Endoscope Date: Sun, 10 Oct 1999 16:41:49 –0500

 

From: "Terry S. Singeltary Sr."

 

Saturday, February 21, 2015

 

Design of Endoscopic Retrograde Cholangiopancreatography (ERCP) Duodenoscopes May Impede Effective Cleaning: FDA Safety Communication

 


 

From: Terry S. Singeltary Sr.

 

Sent: Tuesday, March 31, 2015 9:59 AM

 


 

Subject: re-Design of Endoscopic Retrograde Cholangiopancreatography (ERCP) Duodenoscopes May Impede Effective Cleaning: FDA Safety Communication

 

Greetings Miss Storms Ma’am, Olympus et al,

 

Thank you kindly for the reply.

 

Miss Storms stated;

 

>>>Dear Mr. Singeltary - thank you for contacting me and I will ask an Olympus representative to respond to your question. As duodenoscopes do not contact tissue at high risk associated with CJD, can you please advise on specifically what is your request. <<<

 

My request were stated in my previous email ;

 

>>>In reference to the latest incident of deaths from the use of these endoscopy equipment, what is Olympus recommendations, requirements, and guarantees, for disinfection against the CJD Transmissible Spongiform Encephalopathy TSE PRION type disease ?

 

Miss Storms, I was in contact with Olympus around 1999 about my concerns with endoscopy equipment and CJD TSE prion via iatrogenic mode. we did a crude questionnaire on CJD and endoscopy, and it was disturbing how many of our loved ones, had in the past, before dying from CJD, had some sort of endoscopy work done on them

 


 

and then recently, I read a letter from Olympus from January 6, 2012, and it stated ;

 

>>>At this time, there have been 6 reported cases of iatrogenic transmission of the CJD agent associated with contaminated medical equipment, all of which occurred prior to 1976 before the implementation of standard sterilization procedures. Of the six cases linked to the use of contaminated equipment, four were associated with neurosurgical instruments, and two with stereotactic EEG depth electrodes. In addition, there are no known cases of iatrogenic transmission of the CJD agent from endoscopy.<<< (see letter below for reference...tss)

 

Miss Storms, Olympus et al, this in my opinion, is a red herring, an oxymoron of sorts. all iatrogenic CJD is, is sporadic cjd, until the iatrogenic event is discovered, documented, and put in the public domain. this very seldom happens due to trace back efforts, and the very industry that refuses to do any trace back for CJD, or any TSE prion disease. so your (Olympus) assumptions on only 6 _reported_ cases of iatrogenic CJD, is just that, an assumption, gross negligence of _all_ the facts presented, or kindly left out.

 

I simply believe that by Olympus omitting all these facts, and not presenting them to the consumer of your products, in this case endoscopy equipment, you are misleading your consumers, and in my opinion of the science to date, Olympus is exposing people around the globe with the Transmissible Spongiform Encephalopathy TSE prion disease.

 

Miss Storms, Olympus et al stated ;

 

>>> As duodenoscopes do not contact tissue at high risk associated with CJD, ...snip...end <<<

 

BUT, duodenoscopes DO contact medium to low dose TSE prion infections tissues, if the person being scoped is silently harboring a CJD TSE prion, thus exposing everyone after that to the CJD TSE prion disease. I am very well aware of the old study by Gibbs et al, and those brain electrodes ;

 

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.

 


 

regardless of how many lives endoscopy equipment saves ever year, I strongly, strenuously, urge Olympus to warn their product users, and consumers, that there is NO KNOWN way to date, that will completely clean and decontaminate the endoscopy equipment from the CJD TSE prion disease, and they risk exposure to this agent when exposed to endoscopy equipment. anything less than that would be criminal in my opinion, with the documented science to date. these atypical strains of TSE prion disease are mutating, and as they mutate, they can, and have, become more virulent.

 

with the emergence of atypical strains of TSE prion in animals and humans, we cannot wait to prove a negative, while exposing millions waiting on that negative to come. the science to date, in my opinion, has shown us that this negative has not, and is not coming, thus, exposure, and friendly fire, i.e. iatrogenic, is the key to the 85%+ of all human TSE, i.e. sporadic CJD, and once the science is finally peeled all the way back, biomarkers of some sorts are discovered, and trace back of iatrogenic events are finally able to be documented and traced back to source, I believe that companies like Olympus, that chose to continue to expose millions, regardless of these facts, will be left for much litigation.

 

I strongly urge Olympus et al to state plainly these risk factors of their equipment to the CJD TSE prion like disease, for all to know and see, ASAP. ...

 

thank you, with kind regards, terry

 

OLYMPUS

 

January 6, 2012

 

RE: Reprocessing Olympus Flexible Endoscopes Exposed to CJD

 

Dear Healthcare Professional,

 

This letter is in response to your recent inquiry regarding the reprocessing of Olympus flexible endoscopes (medical and surgical) exposed to the proteinaceous infectious agent, or prion, that causes Creutzfeldt - Jakob disease (CJD). The CJD agent presents a unique infection control challenge because it exhibits an unusual resistance to conventional disinfection and sterilization methods. In an effort to provide our customers with information, Olympus has reviewed various recommendations for the reprocessing of medical devices exposed to the CJD agent. However, Olympus has not tested the efficacy of these proposed methods, and therefore cannot recommend these methods for reprocessing Olympus flexible endoscopes contaminated with the CJD agent. The following information is provided for your reference only and should not be considered a recommendation or endorsement for the reprocessing of Olympus flexible endoscopes contaminated with the CJD agent.

 

At this time, there have been 6 reported cases of iatrogenic transmission of the CJD agent associated with contaminated medical equipment, all of which occurred prior to 1976 before the implementation of standard sterilization procedures. Of the six cases linked to the use of contaminated equipment, four were associated with neurosurgical instruments, and two with stereotactic EEG depth electrodes. In addition, there are no known cases of iatrogenic transmission of the CJD agent from endoscopy.

 

A number of organizations have proposed guidelines for the reprocessing of medical instruments exposed to the CJD agent. The World Health Organization (WHO) has developed infection control guidelines for preventing iatrogenic transmission of the CJD agent. Proposed recommendations for the reprocessing of heat-sensitive medical instruments, such as endoscopes, exposed to the CJD agent include immersion in sodium hypochlorite (NaClO) or sodium hydroxide (NaOH) for one hour. NaOH is extremely corrosive to flexible endoscopes and is not recommended for use. However, Olympus has performed material compatibility testing and concluded that Olympus flexible endoscopes can withstand up to 25, one-hour immersions in sodium hypochlorite (NaClO, 2% available chlorine concentration). Excessive immersion beyond this may cause corrosion and water leaks.

 

OLYMPUS AMERICA INC. 3500 Corporate Parkway Center Valley, PA 18034 Telephone: (484) 896-5000 www.olympusamerica.com

 

It is important to note that Olympus has not tested the efficacy of immersion in NaCIO and makes no claims regarding the effectiveness of this procedure for reprocessing endoscopes contaminated with the CJD agent. Olympus only confirms the material compatibility of NaClO with Olympus flexible endoscopes.

 

To evaluate reprocessing any medical instrument exposed to the CJD agent, published guidance documents recommend that a comprehensive risk assessment be performed. The risk assessment is based upon three primary criteria: the CJD status of the patient (high or low risk), the potential infectivity of the tissue examined (high, low or no infectivity), and the classification of the medical device (critical vs. semi-critical). Olympus recommends that you consult with your infection control department and/or industry experts to determine a strategy that reduces or eliminates the risk of iatrogenic transmission of the CJD agent.

 

Olympus continues to monitor published studies and guidance regarding the destruction or inactivation of the agents responsible for transmissible spongiform encephalopathies, including variant CJD (vCJD), and will review its recommendations as additional information becomes available. Below is a list of useful references that provide guidance on reprocessing medical instruments exposed to the CJD agent. If you have any additional questions, please contact your local Olympus sales representative or the Olympus Technical Assistance Center at 1-800-848-9024 (United States).

 

Thank you.

 

Sincerely,

 

Mary Ann Drosnock, MS, CIC Scientist, Clinical Affairs maryann.drosnock@olympus.com OLYMPUS AMERICA INC. 3500 Corporate Parkway Center Valley, PA 18034 Telephone: (484) 896-5000 www.olympusamerica.com

 

Alvarado, CJ. APIC guideline for infection prevention and control in flexible endoscopy. Am J Infect Control 2000; 28: 138-155.

 

References

 

Centers for Disease Control and Prevention. Questions and Answers: Creutzfeldt-Jakob disease infection control practices, available at http://www.cdc.gov/ncidod/dvrd/cjd/qa_cjd_infection_control.htm Rutala, WA, and Weber DJ. Creutzfeldt-Jakob disease: recommendations for disinfection and sterilization. Clinical Infectious Diseases 2001: 32: 1348-1356. [accessed 6 January 2012] Rutala, WA, and Weber DJ. Managing the risk of nosocomial transmission of prion diseases. Current Opinion in Infectious Diseases 2002 Aug.15 (4): 421-5.

 

United Kingdom, Department of Health, Advisory Committee on Dangerous Pathogens and the Spongiform Encephalopathy Advisory Committee. Transmissible spongiform encephalopathy agents: safe working and the prevention of infection, Annex F: decontamination of endoscopes: available at

 


 

World Health Organization. WHO infection control guidelines for transmissible spongiform encephalopathies; available at [accessed 6 January 2012].

 


 


 

Research and analysis

 

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

 

Updated 13 February 2015

 

Contents 1.Monitoring of patients 'at increased risk' of CJD 2.Updated guidance on decontamination of equipment for gastrointestinal endoscopy .

 

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).

 

This report was published in Health Protection Report volume 8 issue 31.

 


 

Wednesday, September 10, 2014

 

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

 

Research and analysis

 


 

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

 


 

EFSA supporting publication 2014:EN-559 Any enquiries related to this output should be addressed to biohaz@efsa.europa.eu. The TSEi model is available upon request to biohaz@efsa.europa.eu. Suggested citation: Amie Adkin, Robin Simmons and Mark Arnold, 2014. TSE infectivity model (TSEi) in animal tissues: Bovine intestines and mesenteries. EFSA supporting publication 2014:EN-559, 74 pp. Available online: www.efsa.europa.eu/publications © European Food Safety Authority, 2014 EXTERNAL SCIENTIFIC REPORT TSE infectivity model (TSEi) in animal tissues: Bovine intestines and mesenteries1 Adkin A, Simons R, and Arnold M Animal Health and Veterinary Laboratories Agency (AHVLA), Weybridge, United Kingdom

 

ABSTRACT A stochastic quantitative risk assessment (QRA) has been developed to (1) compare the level of infectivity of different TSE agents in animal tissues, (2) estimate the impact of amendments to the list/age for the removal of SRM on residual TSE infectivity levels for a single infected animal and at the country level per year, and (3) estimate the impact of certain processing technologies on residual TSE infectivity in selected animal tissues or products. In this report the QRA is focused on bovine intestines and mesentery. The tissue types identified for quantitative modelling are: ileum, duodenum, jejunum, caecum, colon, mesenteric lymph nodes, mesenteric nerves and the celiac and mesenteric ganglion complex (CMGC). Of these tissues processed products include bovine intestines (duodenum, jejunum, caecum, and colon) used to produce sausage casings and the rendering of fats from mesentery tissues. The ileum is not processed for human consumption. This report describes the model approach taken together with the parameterization for each tissue type conceptually divided into five different components: surveillance, abattoir, SRM, processing, and infectivity. Both uncertainty and variability associated with input data have been included separately in the model where estimates are known. A baseline model has been completed using surveillance and demographic data from 2012. Two case studies are also provided, the retrospective analysis of the estimated amount of infectivity in the healthy slaughter and emergency slaughter streams by age at slaughter (2007-2011), and the amount of infectivity accumulating during a theoretical re-emergence of BSE. Results are provided based on the current parameterization and include associated quantifiable uncertainty and variability. When developing the risk assessment a number of assumptions were made which need to be considered when reviewing results.

 

© Animal Health and Veterinary Laboratories Agency (AHVLA), Weybridge, United Kingdom, 2014 KEY WORDS Bovine Spongiform Encephalopathies, BSE, risk assessment, mathematical model, bovine intestines, mesentery tissues DISCLAIMER The present document has been produced and adopted

 

snip...

 

10. Conclusions This report describes the model framework developed to compare the pattern of BSE infectivity in selected animal tissues (bovine intestines and mesenteries) for an individual infected animal. The model also estimates infectivity at the member state, or group of member states level by age at slaughter over one year, results provided in this report for the EU27, and includes the impact of certain processing technologies (sausage casing processing) on residual BSE infectivity. Case studies are provided estimating the annual infectivity historically and in the situation of a future re-emergence of disease. Results, where appropriate, are stratified by age at slaughter, infectivity by meter length (for bovine intestinal tissues), tissue type, and pre and post processing. ...see full text.

 


 

Cattle All ages • The tonsils, the intestines, from the duodenum to the rectum, and the mesentery;

 


 

4. Processes regarding the removal or inactivation of transmissible spongiform encephalopathy (TSE) agents (i.e., prions) from contaminated medical devices. Please note that as of the date of this guidance, FDA has not approved or cleared medical devices, including sterilizers, for the intended use of reducing the infectivity of TSE agents.

 


 

nothing on TSE Prions ;

 


 

Interpretation Our findings suggest that the possible risk of vCJD linked to endoscopic procedures might be currently underestimated. Human iatrogenic vCJD cases infected intravenously raise the same public-health concerns as primary cases and need the same precautionary measures with respect to blood and tissue donations and surgical procedures.

 

We noted that PrPres was present in lymphoreticular tissues such as spleen and tonsils and in the entire gut from the duodenum to the rectum.

 


 

March 26, 2015

 

URGENT SAFETY NOTIFICATION IMPORTANT UPDATED LABELING INFORMATION: NEW REPROCESSING INSTRUCTIONS FOR THE OLYMPUS TJF-Q180V DUODENOSCOPE

 

ATTENTION: Endoscopy Department, Risk Management and Reprocessing Units

 

Dear Health Care Professional:

 


 

Dr. Linda Detwiler

 


 

03-025IF 03-025IF-631 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-631 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8. Page 9. Page 10. Page 11. Page 12.

 

Intestine

 

The scenario describe above is essentially true for the intestine. Infectivity was readily detectable in the distal ileum of cattle infected with BSE. While certain additional sections of the intestine were tested with no infectivity identified, not every section of the intestine was included in the bioassays. Positive immunostaining for Prpres was identified along the length of the intestine providing evidence for the entire intestine to be considered as SRM per EU regulations. (personal communication Danny Matthews, UK, VLA). The International Advisory Committee appointed by Secretary Veneman also recommended that the SRM ban in the US be amended to the entire intestine from duodenum to rectum. I recommend that the USDA adjust the definition of SRM to include the entire intestine from the duodenum to the rectum .

 

snip...

 

see full text ;

 


 

03-025IF 03-025IF-634 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-634 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8.

 


 


 


 

Linda A. Detwiler, DVM

 

225 Hwy 35

 

Red Bank, New Jersey 07701

 

Phone: 732-741-2290

 

Cell: 732-580-9391

 

Fax: 732-741-7751

 

June 22, 2005

 

FSIS Docket Clerk

 

U.S. Department of Agriculture Food Safety and Inspection Service 300 12th Street, SW. Room 102 Cotton Annex Washington, DC 20250

 

RE: DEPARTMENT OF AGRICULTURE Food Safety and Inspection Service

 

9 CFR Parts 301, 309, 310, 311, 313, 318, 319 and 320

 

Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle; Meat Produced by Advanced Meat/Bone Separation Machinery and Meat Recovery (AMR) Systems;

 

Prohibition of the Use of Certain Stunning Devices Used To Immobilize Cattle During Slaughter; Bovine Spongiform Encephalopathy (BSE) Surveillance Program

 

Docket Number 03-025IF: Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Nonambulatory Disabled Cattle

 

I am writing to clarify a comment I submitted to the above mentioned docket on May 7, 2004. I had previously written that the entire length of the intestine should be excluded as SRM. I still hold this opinion and submit the same recommendation, however one of the reasons behind this opinion needs to be further clarified. I had misunderstood comments made by Dr. Danny Matthews in that immunostaining (of PrPbse) was not found throughout the entire length of the intestine. There was however immunostaining in the myenteric plexus of the distal ileum in both naturally infected and experimentally challenged cattle with BSE. (Terry et al.,2003) Given that the myenteric plexus exists throughout the intestine one cannot eliminate the possibility of infectivity being in other sections. In fact this was some of the thought behind the designation of the entire intestine as SRM in the EU:

 

In its opinion of 7-8 December 2000 (EC 2000), the SSC concluded that the entire bovine intestine is a risk issue and Commission Regulation (EC) No.

 

270/2002 (14th February 2002) ANNEX II designates “the entire intestines from the duodenum to the rectum and the mesentery of bovine animals of all ages;” as SRM. Also, in the SSC opinion of 28-29 JUNE 2001, Adipose tissue associated with the digestive tract of cattle, sheep and goats: an appreciation of possibleTSE risks (EC 2001) the view was expressed that for cattle, “due to the infectivity titre that could be theoretically reached in nervous tissues and in some parts of intestine, and due to the risk of contamination with intestine tissue….

 

The International Advisory Committee appointed by Secretary Veneman also recommended that the SRM ban in the US be amended to the entire intestine from duodenum to rectum.

 

Although certain additional sections of the intestine were tested with no infectivity identified, not every section of the intestine was included in the bioassays. In addition, the study involving immunostaining was also extremely limited in regard to the testing of tissues other than the distal ileum. Specifically, other sections of intestinal tissues (excluding the distal ileum work) were limited to those collected from 3 calves inoculated with BSE at a timeframe of 6 months post inoculation. Instead of assuming that the untested sections are devoid of infectivity, it is my belief that we should err on the side of caution when it comes to protecting public health. Hence I maintain my opinion that the entire intestine should be considered SRM.

 

This clarification is also intended for my comments submitted to the FDA’s ANPR.

 

Thank you for the opportunity to clarify my comments.

 

Linda A. Detwiler, DVM

 

REFERENCES

 

Terry, L. A.., Marsh, S., Ryder, S. J., Hawkins, S. A. C., Wells, G. H., and Spencer, Y. I. (2003) Detection of disease-specific PrP in the distal ileum of cattle exposed orally to the agent of bovine spongiform encephalopathy. Vet Rec., 152, 387-392 Wells G.A.H., Dawson M., Hawkins, S.A.C., Green R. B., Dexter I., Francis M. E., Simmons M. M., Austin A. R., & Horigan M. W. (1994) Infectivity in the ileum of cattle challenged orally with bovine spongiform encephalopathy. Vet. Rec., 135, 40-41. Wells G.A.H., Hawkins, S.A.C., Green R. B., Austin A. R., Dexter I., Spencer, Y. I., Chaplin, M. J., Stack, M. J., & Dawson, M. (1998) Preliminary observations on the pathogenesis of experimental bovine spongiform encephalopathy (BSE): an update. Vet. Rec., 142, 103-106.

 


 

snip...

 

see full text ;

 


 


 

WHO Tables on Tissue Infectivity Distribution in Transmissible Spongiform Encephalopathies Updated 2010

 

snip...

 

Table IB: Lower-infectivity tissues Humans Cattle Sheep & goats Elk & deer vCJD Other TSEs BSE Scrapie CWD Tissues Infectivity1 PrPTSE Infectivity1 PrPTSE Infectivity1 PrPTSE Infectivity1 PrPTSE Infectivity1 PrPTSE

 

Table IB: Lower-infectivity tissues

 

Alimentary tract5

 

Esophagus NT - NT - - NT [+] + NT +

 

Fore-stomach6

 

(ruminants only) NA NA NA NA - NT [+] + NT +

 

Stomach/

 

abomasum NT - NT - - NT [+] + NT +

 

Duodenum NT - NT - - - [+] + NT +

 

Jejunum7 NT + NT - - + [+] + NT NT

 

Ileum7 NT + NT - + + + + NT +

 

Appendix (-) + NT - NA NA NA NA NA NA

 

Colon/caecum7 NT + NT - - - + + NT +

 

Rectum [+] + NT NT NT NT NT + NT +

 

snip...

 


 

EFSA Journal 2014;12(7):3798

 

Suggested citation: European Food Safety Authority, 2014.

 

Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE.

 

EFSA Journal 2014;12(7):3798, 55 pp. doi:10.2903/j.efsa.2014.3798 Available online: www.efsa.europa.eu/efsajournal © European Food Safety Authority, 2014 SCIENTIFIC REPORT OF EFSA

 

Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE1 European Food Safety Authority2,3 European Food Safety Authority (EFSA), Parma, Italy

 

ABSTRACT

 

Information on the pathogenesis and tissue distribution of Atypical Bovine Spongiform Encephalopathy (BSE) in cattle through the study of field cases and experimental transmission studies is lacking. The latter are limited to transmission of Atypical BSE through intracerebral (i.c.) inoculation of cattle. All data currently available relate to the presence or absence of PrPSc, but do not quantify relative amounts of PrPSc or levels of infectivity. A laboratory protocol for further studies is recommended, to allow the assessment of the relative infectious titre, PrPSc accumulation and prion seeding activity in the tissues of cattle that developed H-BSE or L-BSE (using posterior brainstem as a reference). Tissues to be covered by those studies are categorised in three priorities, based on their inclusion in the list of specific risk material in cattle, on the presence of infectivity, or PrPSc presence, demonstrated in Atypical BSEs or other Transmissible Spongiform Encephalopathies (TSEs) in ruminants, and on the importance in terms of input into the food chain in the EU. The protocol provides details in terms of the minimum number of animals to be tested, processing and preparation of tissues, and methods to be used to identify abnormal PrP and quantify infectivity, also depending on the expected level of infectivity and amount of tissue available for analysis. It is recommended that, through the implementation of the protocol, information should also be obtained on the performance of currently validated rapid tests for TSE active surveillance in cattle/bioassay for detecting H-BSE and L-BSE agents.

 

© European Food Safety Authority, 2014

 

KEY WORDS

 

Atypical BSE, cattle, H-BSE, L-BSE, laboratory protocol, prion

 

1 On request from the European Commission, Question No EFSA-Q-2013-01015, approved on 11 July 2014.

 

2 Correspondence: biohaz@efsa.europa.eu

 

3 Acknowledgement: EFSA wishes to thank the members of the Working Group on Atypical BSE study protocol: Olivier Andreoletti, Anne Balkema-Buschmann, Vincent Béringue, Marion Simmons and Juan-Maria Torres for the preparatory work on this scientific output, the members of the EFSA Panel on Biological Hazards (BIOHAZ) for their endorsement of the scientific output, and EFSA staff members: Winy Messens and Pietro Stella for the support provided to this scientific output.

 

snip...

 

Atypical BSE study protocol

 

EFSA Journal 2014;12(7):3798 16

 

2.3. Additional information on material available

 

Data available from the literature relating to field case examinations, the EURL study and the other transmission studies outlined above (Section 2.1) have been collated to provide a comprehensive list of the tissue samples that have been collected and examined from natural and experimental cases of H-BSE and L-BSE. The full list is given in Appendix A and represents a wide range of tissues from both the central and peripheral nervous systems, the lymphoreticular system, the musculoskeletal system and the gastrointestinal tract, together with other principal edible organs. However, none of these studies was explicitly designed to address the issue of potential infectivity in the context of the current legislation on SRM, and so there are some SRM tissues that have not been collected within any of these studies, namely the duodenum, the jejunum and ileum (without Peyer’s patches), the caecum, the colon and the mesenteric fat. For these tissues there are therefore no data, and no possibility to create data without undertaking further experimental challenges. All of the experimental material is derived from animals that have been challenged intracerebrally. In total, data are available for 15 experimental H-BSE and 23 L-BSE cases, representing challenges with 4 donors respectively, while data on PrP distribution in naturally-occurring field cases have been published for only three L-BSE-affected cattle.

 

There are no data for field case H-BSE. Where data exist from both field cases and experimental animals (i.e. for L-BSE only), there is good agreement between the data with and abnormal PrP distribution, with the CNS and muscles both consistently affected. However, these data relate to the presence or absence of PrPSc, and do not attempt to quantify relative amounts of PrPSc or levels of infectivity. Overall, disease-related PrP has been reported in CNS tissues, peripheral ganglia and nerves, muscles (predominantly muscle spindles), adrenal glands and retina for both H-BSE and L-BSE. By contrast, no lymphoid tissues or gastrointestinal tissues have tested positive by IHC, WB or bioassay. Some tissues have been collected, but with no testing outcome explicitly reported.

 

Comparative data relating to C-BSE (see Appendix A, Table 12) are to a large extent fragmented, and have evolved over a long period as different analytical methods have been developed and applied. Many of the original infectivity data are based on conventional mouse bioassay rather than transgenic models so, although positive results are robust, negative results do not necessarily have the same sensitivity thresholds (see also EFSA BIOHAZ Panel (2014)). It must also be noted that data available for C-BSE are derived predominantly from field cases or oral challenge models. There are few data from i.c. challenge studies that can be directly compared with existing data for Atypical BSE. However, collective data indicate that C-BSE shares the same tissue distribution as the Atypical BSE cases, with PrPSc and/or infectivity detected in the central and peripheral nervous systems, including ganglia and nerves, and the muscle spindles in skeletal muscle. As in Atypical BSE, the adrenal glands and the retina are also affected.

 

Additionally, in C-BSE there is also evidence of involvement of the lymphoreticular system, particularly, but not exclusively, in those tissues associated with the gastrointestinal tract. The PrPSc distribution and relative levels of infectivity in the gastrointestinal tissues were presented in detail in a previous EFSA Opinion (EFSA BIOHAZ Panel, 2014). Nasal mucosa and bone marrow have also been shown to contain infectivity.

 

There are insufficient data at present to be clear about whether these apparent differences in the distribution of disease-specific markers reflect absolute differences between C-BSE and the H-BSE and L-BSE variants, whether they are a consequence of detection threshold limitations, or whether they are a consequence of the different routes of challenge. Atypical BSE study protocol

 

EFSA Journal 2014;12(7):3798 17

 

2.4. Concluding remarks Where data exist from both field cases and experimental animals (i.e. for L-BSE only), there is good agreement of the data with regard to abnormal PrP distribution. There are no data for field case H-BSE. All data currently available relate to the presence or absence of PrPSc, but do not quantify relative amounts of PrPSc or levels of infectivity. Disease-related PrP has been reported consistently in CNS tissues, peripheral ganglia and nerves, muscles (predominantly muscle spindles), adrenal glands and retina for both H-BSE and L-BSE. All of these tissues are also positive in C-BSE. By contrast with C-BSE, at this stage no lymphoid tissues or gastrointestinal tissues from H-BSE- and L-BSE-affected animals have tested positive for PrPSc presence (IHC, WB) or infectivity (bioassay). There are insufficient data at present to be clear about whether these apparent differences in the distribution of disease-specific markers reflect absolute differences between C-BSE and the H-BSE and L-BSE variants, whether they are a consequence of detection threshold limitations, or whether they are a consequence of the different

 


 

Research Article

 

Prion Protein Gene Variability in Spanish Goats. Inference through Susceptibility to Classical Scrapie Strains and Pathogenic Distribution of Peripheral PrPsc Cristina Acín ,

 

Inmaculada Martín-Burriel,

 

Eva Monleón,

 

Jaber Lyahyai,

 

José Luis Pitarch,

 

Carmen Serrano,

 

Marta Monzón,

 

Pilar Zaragoza,

 

Juan José Badiola

 

PLOS

 

Published: April 8, 2013 •DOI: 10.1371/journal.pone.0061118 Detection of PrPsc in peripheral organs.

 

snip...

 

PrPsc was detected in the tonsils, iliac, axillary, pre-scapular, submandibular, popliteal, and mammary lymph nodes of the six goats analyzed, but just in five in the mediastinal and mesenteric lymph nodes, ileal and jejunal Peyer patches, ileocaecal valve and spleen. The enteric nervous system was positive throughout the whole intestine, from duodenum to rectum, in the five animals in which PrPsc was also detected in all the areas of the CNS (see Table 1).

 


 

Sunday, November 23, 2014

 

*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June 2014 confirmed as USA case NOT European ***

 


 

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).

 


 

Thursday, January 15, 2015

 

41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE Prion: Case Report

 


 

Subject: *** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease aka mad cow type disease

 

what is CJD ? just ask USDA inc., and the OIE, they are still feeding the public and the media industry fed junk science that is 30 years old.

 

why doesn’t some of you try reading the facts, instead of rubber stamping everything the USDA inc says.

 

sporadic CJD has now been linked to BSE aka mad cow disease, Scrapie, and there is much concern now for CWD and risk factor for humans.

 

My sincere condolences to the family and friends of the House Speaker Becky Lockhart. I am deeply saddened hear this.

 

with that said, with great respect, I must ask each and every one of you Politicians that are so deeply saddened to hear of this needless death of the Honorable House Speaker Becky Lockhart, really, cry me a friggen river. I am seriously going to ask you all this...I have been diplomatic for about 17 years and it has got no where. people are still dying. so, are you all stupid or what??? how many more need to die ??? how much is global trade of beef and other meat products that are not tested for the TSE prion disease, how much and how many bodies is this market worth?

 

Saturday, January 17, 2015

 

*** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease

 


 

*** 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 ***

 


 

Tuesday, November 04, 2014

 

Towards an Age-Dependent Transmission Model of Acquired and Sporadic Creutzfeldt-Jakob Disease

 


 

Thursday, January 22, 2015

 

Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology?

 


 


 

Sunday, July 06, 2014

 

Dietary Risk Factors for Sporadic Creutzfeldt-Jakob Disease: A Confirmatory Case-Control Study

 

Conclusions—The a priori hypotheses were supported.

 

*Consumption of various meat products may be one method of transmission of the infectious agent for sCJD.

 


 

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

 


 


 

Friday, January 10, 2014

 

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

 


 


 

Sunday, February 08, 2015

 

FDA SCIENCE BOARD TO THE FOOD AND DRUG ADMINISTRATION BOVINE HEPARIN BSE CJD TSE PRION Wednesday, June 4, 2014

 


 

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

 


 

Wednesday, June 19, 2013

 

Spreading of tau pathology in Alzheimer's disease by cell-to-cell transmission

 


 

Wednesday, January 28, 2015

 

Another new prion disease: relationship with central and peripheral amyloidoses

 

here we go again...

 


 


 


 


 


 


 


 


 


 


 


 


 


 

Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease

 

Received July 24, 2014; Accepted September 16, 2014; Published November 3, 2014

 


 

Singeltary comment ;

 


 

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net

 


 

Sent: Monday, March 30, 2015 8:11 AM

 

To: Terry S. Singeltary Sr.

 

Subject: Re: Fw: Design of Endoscopic Retrograde Cholangiopancreatography (ERCP) Duodenoscopes May Impede Effective Cleaning: FDA Safety Communication

 

Dear Mr. Singeltary - thank you for contacting me and I will ask an Olympus representative to respond to your question. As duodenoscopes do not contact tissue at high risk associated with CJD, can you please advise on specifically what is your request.

 

Regards,

 

Laura Storms V.P., Regulatory/Clinical Affairs & Quality Assurance Olympus Corporation of the Americas 3500 Corporate Parkway Center Valley PA 18034 Phone (484) 896-5688 Cell (631) 871-1724 laura.storms@olympus.com

 

 From: "Terry S. Singeltary Sr." flounder9@verizon.net

 


 

Date: 03/27/2015 01:44 PM

 

Subject: Fw: Design of Endoscopic Retrograde Cholangiopancreatography (ERCP) Duodenoscopes May Impede Effective Cleaning: FDA Safety Communication

 

--------------------------------------------------------------------------------

 


 

In reference to the latest incident of deaths from the use of these endoscopy equipment, what is Olympus recommendations, requirements, and guarantees, for disinfection against the CJD Transmissible Spongiform Encephalopathy TSE PRION type disease ? thank you, kind regards, terry snip...

 

Saturday, December 13, 2014

 

*** Terry S. Singeltary Sr. Publications TSE prion disease Peer Review ***

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

 


 

some old history on Endoscopy equipment and CJD TSE Prion concerns ; 1999

 

Subject: CJD * Olympus Endoscope

 

Date: Sun, 10 Oct 1999 16:41:49 –0500

 

From: "Terry S. Singeltary Sr."

 

Saturday, February 21, 2015

 

Design of Endoscopic Retrograde Cholangiopancreatography (ERCP) Duodenoscopes May Impede Effective Cleaning: FDA Safety Communication

 


 

Sunday, April 5, 2015

 

*** Guidance for Industry Ensuring Safety of Animal Feed Maintained and Fed On-Farm Draft Guidance FDA-2014-D-1180 ***

 


 

 all iatrogenic CJD TSE prion disease is, is sporadic cjd TSE prion disease, until the iatrogenic event is discovered, documented, and put in the public domain. this very seldom happens due to trace back efforts, and the very industry that refuses to do any trace back for CJD, or any TSE prion disease. ...

 

kind regards, terry