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

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 31 December 2013. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU,

A briefing on a recently-identified human prion disease Variably Protease-Sensitive Prionopathy is also presented below.

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 (table 1).

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 genetic 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 (as at 31 December 2013)


'At risk' Group
Identified as 'at risk'
Number notified as being 'at risk'
Asymptomatic infections [b]
All Alive
Recipients of blood from who later developed vCJD

Blood donors to who later developed vCJD

Other recipients of blood components from these donors
32 [c]
19 [c]

Plasma product recipients (non-bleeding disorders) who received UK sourced plasmsa products 1980-2001

Certain surgical contacts of patients diagnosed witih CJD
129 [d]
113 [e]

Highly transfused patients

Total for 'at risk' groups where PHE holds data
315 [f]
261 [f]

Patients with bleeding disorders who received UK-sourced plasma products 1980-2001 [a]
National information incomplete
National information incomplete

Recipients of human-derived growth hormone [a]

Total for all 'at risk' groups [a]
At least
At least 1765


Variably Protease-Sensitive Prionopathy
Professor James W Ironside and Dr Mark W Head

The National CJD Research and Surveillance Unit, University of Edinburgh.
Variably protease-sensitive prionopathy (VPSPr) is the most recently identified human prion disease, first described in the USA by Gambetti et al. in 2008 as “a novel human disease with abnormal prion protein sensitive to protease” [1]. Since then, similar cases have been identified in other countries; the National CJD Research and Surveillance Unit has identified nine cases in the UK, three of which have been identified retrospectively and the others prospectively from samples and data collected since 1991 [2-6]. Other candidate cases are currently under investigation.
Patients with VPSPr have no identified risk factors for acquired human prion disease and no associated mutations in the prion protein gene (PRNP) coding sequence have been found. In the original description a proportion of the patients had family histories of ill-defined dementia, but this has not been a feature in more recently identified cases [1,2,6]. VPSPr affects patients in the same age range as sporadic Creutzfeldt-Jakob disease (sCJD), occurring mostly in patients over the age of 60. The clinical features are more varied than in sCJD and include movement abnormalities, cognitive decline and unsteadiness while walking. The clinical illness is longer than for sCJD; most patients survive for over a year before succumbing to the illness. Diagnostic clinical criteria are therefore difficult to establish, and further work is required on this topic since this disease is likely to be under-ascertained [2,6].
Like sCJD, VPSPr occurs in all genetic groups defined by the polymorphism at codon 129 in the PRNP gene, ie MM, MV and MV. Unlike sCJD, there is a preponderance of the codon 129-V haplotype. VPSPr has distinctive neuropathological features, the most typical of which are microplaques that occur in a target-like arrangement and are particularly common in the cerebellum. These microplaques show differential staining with a panel of different anti-PrP antibodies, allowing a distinction from both the common sCJD VV2 and the rare sCJD VV1 subtypes [1,2,5,6]. The most distinctive and defining feature of VPSPr is the biochemistry of the abnormal prion protein in the brain, which is only poorly resistant to proteolytic digestion, yielding a low abundance, truncated 8kDa (approx) band in Western blot assays [1]. This fragment is often accompanied by a faint ladder of bands extending into the 18-30kDa range [1,2]. Some cases of VPSPr also show a sCJD-like pattern on Western blot analysis for abnormal prion protein, often in the cerebellum, suggesting molecular overlaps between VPSPr and sCJD [6,7].
Further work is required to fully establish the epidemiology, clinical and pathological diagnostic criteria and transmission characteristics of VPSPr. The Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy (ACDP TSE) Subgroup concluded that until further research can demonstrate how transmissible VPSPr may be, it would be advisable to add this novel form of human prion disease to the infection control guidance for CJD and other related disorders.

1. Gambetti P, Dong, Yuan J, et al. A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 2008; 63: 697-708.

2. Zou WQ, Puoti G, Xiao X, et al. Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein. Ann Neurol 2010; 68: 162-72.
3. Head MW, Knight R, Zeidler M, et al. A case of protease sensitive prionopathy in a patient in the United Kingdom. Neuropathol Appl Neurobiol 2009; 35: 628-32.

4. Jansen C, Head MW, van Gool WA, et al. The first case of protease-sensitive prionopathy (PSPr) in The Netherlands: a patient with an unusual GSS-like clinical phenotype. J Neurol Neurosurg Psychiatry 2010; 81: 1052-5.

5. Head MW, Lowrie S, Chohan G, et al. Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, a synclein and Aß pathology. Acta Neuropathol 2010: 120: 821-3.

6. Head MW, Yull HM, Ritchie DL, et al. Variably protease-sensitive prionopathy in the UK: a retrospective review 1991-2008. Brain 2013; 136: 1102-15.

7. Rodriguez-Martinez AB, de Munain AL, Ferrer I, et al. Coexistence of protease sensitive and resistant prion protein in 129 VV homozygous sporadic Creutzfeldt-Jakob disease. J Med Case Rep 2012; 6: 348



Wednesday, March 28, 2012




OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles


Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA


 Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.


 Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.


 Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.


 In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.


 Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.


 The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.



 Wednesday, March 28, 2012





 *** 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





 Friday, August 16, 2013

*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates


Volume 4 Number 7 Published on: 19 February 2010


Emerging infections/CJD Creutzfeldt-Jakob disease (CJD) biannual update (2010/1)



Saturday, March 23, 2013


$$$ CJD Incidents Panel to be disbanded $$$



ALL iatrogenic cjd is, is sporadic cjd, until route and source is documented, confirmed, and put in the academic and public domain, which very seldom happens. that's why 85%+ of all human TSE prion disease is sporadic CJD, they like to keep it that way$$$ ...just saying$$$

I suppose one of the most disturbing studies I have ever read, was the one of Gibbs et al, way back, with electrodes that caused CJD, again, and again.

I am not posting this to scare folks, so be it if it does, but I am posting this for you to see what you are dealing with. ...this study still amazes me. read it more than once.

please see ;

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.

PMID: 8006664 [PubMed - indexed for MEDLINE]

New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

Detection of protease-resistant cervid prion protein in water from a CWD-endemic area

A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing

Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals


Survival and Limited Spread of TSE Infectivity after Burial

*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

Saturday, November 16, 2013

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

Infect Control Hosp Epidemiol.


Tuesday, May 28, 2013

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

Tuesday, February 11, 2014

Novant Health Forsyth Medical Center Information on potential CJD exposure

Wednesday, January 15, 2014




Wednesday, November 27, 2013


NHS failed to sterilise surgical instruments contaminated with 'mad cow' disease



Friday, January 10, 2014


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



Thursday, January 23, 2014


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



Saturday, November 16, 2013


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


Infect Control Hosp Epidemiol.



Thursday, November 14, 2013


Prion diseases in humans: Oral and dental implications



Saturday, November 2, 2013


Recommendation of the Swiss Expert Committee for Biosafety on the classification of activities using prion genes and prion protein January 2013



Thursday, January 16, 2014


The Anspach Effort, Inc. RECALL FDA Blackmax motor had been used in a case where the patient was diagnosed with Creutzfeldt-Jacob Disease (CJD) MARYLAND HOSTPITAL



WHAT about the sporadic CJD TSE proteins ?


WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$


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 ***



Sunday, October 13, 2013


*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012



Sunday, January 19, 2014


National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014



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)



Tuesday, September 24, 2013


NORDION (US), INC., AND BIOAXONE BIOSCIENCES, INC., Settles $90M Mad Cow TSE prion Contamination Suit Cethrin(R)


Case 0:12-cv-60739-RNS Document 1 Entered on FLSD Docket 04/26/2012 Page 1 of 15



Tuesday, February 11, 2014

Novant Health Forsyth Medical Center Information on potential CJD exposure

Thursday, September 05, 2013


Possible Patient Exposure to Creutzfeldt-Jakob Disease Announced New Hampshire DHHS


Press Release



Friday, July 19, 2013


Beaumont Hospital in Dublin assessing patients for CJD



Tuesday, July 31, 2012


11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital



Thursday, August 02, 2012


CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients



Saturday, February 12, 2011


Another Pathologists dies from CJD, another potential occupational death ?


another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???



Monday, April 15, 2013


Dr. Stephen B. Thacker Director Centers for Disease Control and Prevention′s Office of Science, Epidemiology and Laboratory Services (OSELS) dies from Creutzfeldt Jakob Disease CJD



Thursday, April 12, 2012


Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010


Eurosurveillance, Volume 17, Issue 15, 12 April 2012


Research articles



Wednesday, February 16, 2011





Sunday, December 12, 2010


EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010



Sunday, April 18, 2010





Sunday, February 2, 2014


The Presence of Disease-Associated Prion Protein in Skeletal Muscle of Cattle Infected with Classical Bovine Spongiform Encephalopathy


NOTE Pathology



Monday, February 10, 2014


Enhanced Virulence of Sheep-Passaged Bovine Spongiform Encephalopathy Agent Is Revealed by Decreased Polymorphism Barriers in Prion Protein Conversion Studies



Wednesday, December 11, 2013


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



Wednesday, April 24, 2013

Chimpanzees Released After 30 Years Of Testing, Brace Yourself For Smiles




Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

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