2 0 2 1 Volume 45
Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2020
Christiane Stehmann, Matteo Senesi, Shannon Sarros, Amelia McGlade, Victoria Lewis, Marion Simpson, Genevieve Klug, Catriona McLean, Colin L Masters, Steven Collins
Annual Report
Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2020 Christiane Stehmann, Matteo Senesi, Shannon Sarros, Amelia McGlade, Victoria Lewis, Marion Simpson, Genevieve Klug, Catriona McLean, Colin L Masters, Steven Collins
Abstract
Nationwide surveillance of Creutzfeldt-Jakob disease and other human prion diseases is performed by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR). National surveillance encompasses the period since 1 January 1970, with prospective surveillance occurring from 1 October 1993. Over this prospective surveillance period, considerable developments have occurred in pre-mortem diagnostics; in the delineation of new disease subtypes; and in a heightened awareness of prion diseases in healthcare settings. Surveillance practices of the ANCJDR have evolved and adapted accordingly. This report summarises the activities of the ANCJDR during 2020. Since the ANCJDR began offering diagnostic cerebrospinal fluid (CSF) 14-3-3 protein testing in Australia in September 1997, the annual number of referrals has steadily increased. In 2020, 510 domestic CSF specimens were referred for 14-3-3 protein testing and 85 persons with suspected human prion disease were formally added to the national register. As of 31 December 2020, just over half (44 cases) of the 85 suspect case notifications remain classified as ‘incomplete’; 27 cases were excluded through either detailed clinical follow-up (9 cases) or neuropathological examination (18 cases); 18 cases were classified as ‘definite’ and eleven as ‘probable’ prion disease. For 2020, sixty percent of all suspected human-prion-disease-related deaths in Australia underwent neuropathological examination. No cases of variant or iatrogenic CJD were identified.
The SARS-CoV-2 pandemic did not affect prion disease surveillance outcomes in Australia.
Keywords: Creutzfeldt-Jakob disease, prion disease, transmissible spongiform encephalopathy, disease surveillance
snip...
Discussion
In 2020, the number of suspected prion disease referrals and confirmed cases broadly matched the long-term average (1997–2019). Australia continued to be free of vCJD and no further cases of iCJD were detected. By state and territory, the numbers of suspected case referrals showed generally only modest fluctuations during 2020 compared to the previous year; fluctuations seen in 2020 are within previouslyobserved ranges.
Long-term national surveillance units report differing annual prion disease mortality rates, ranging from 0.24 to 4.56 per million population.4,5 Higher rates of human prion disease over short time frames have also been recognised and investigated in various global settings with inconclusive outcomes.8 The underlying basis for fluctuations and differences in national mortality rates is uncertain, although variation in case ascertainment is one potentially contributing factor.6
Spatio-temporal clustering of CJD has previously been recognised in New South Wales and Victoria.9,10 Detailed epidemiological assessment by the ANCJDR did not disclose any likely horizontal transmission event, but instead uncovered a heightened intensity of surveillance.9
This more intense level of surveillance was reflected by the significantly higher rates of referrals of suspect prion disease cases for evaluation and diagnostic testing to the ANCJDR, as well as higher neuropathological examination rates in suspected patients.6,9,10 Monitoring of the geographical distribution of suspected case referrals and confirmed cases remains an important facet of ANCJDR national surveillance.
An overall increase in sporadic CJD cases has also been observed in Australia and is most likely due to a combination of an ageing population, improved case ascertainment and diagnostic methodologies, and greater awareness of prion disease in the healthcare sector.5,11 The gradual but notable increase in the incidence of sCJD, but not that of genetic CJD, has also been reported in other countries with longstanding prion disease surveillance and supports the notion that it is a result of the globally ageing population.5,11
Ascertainment mechanisms in 2020 were unchanged compared to recent years, with the majority of initial referrals coming through requests for diagnostic CSF 14-3-3 protein testing. Some proactive ascertainment mechanisms (such as state health department and tertiary hospital mortality data base searches) have ceased, while other case detection methods have increased. The number of CSF referrals to the ANCJDR for diagnostic (14-3-3 protein) testing remained high for 2020. A 20% increase in diagnostic test referrals coincided with the introduction of CSF total-tau protein estimation in 2017. Estimation of total-tau protein in CSF is NATA/ILAC accredited and complementary to 14-3-3 protein testing to support a pre-mortem diagnosis of sporadic CJD. The identification of misfolded prion protein in CSF by RT-QuIC continues to be developed by the ANCJDR as a diagnostic test and is currently selectively performed for cases after discussion with clinicians. The addition of CSF total-tau protein estimation to 14-3-3 protein detection as a biomarker for the pre-mortem evaluation of suspected sCJD offers modestly enhanced diagnostic capacity while the ANCJDR completes imminent transition to clearly superior protein amplification techniques such as RT-QuIC.
The proportion of post-mortems being performed in suspect prion disease cases remains high and aligns with the long-term mean brain autopsy percentage of approximately 63% (of suspected case deaths) between 1993 and 2019. This contrasts with the findings of an Australian healthcare setting survey where the national hospital post-mortem rate was 12% in 2002–2003;12 more recently, a major Australian tertiary centre audit of hospital autopsy data has described an autopsy rate of 6.6% in 2011–2013.13
The high suspected prion-disease-related postmortem rate underpins the high and consistent number of confirmed Australian human prion disease cases recorded over the more recent prospective surveillance time period, and provides confident understanding of the cause of death in suspected cases ultimately determined as non-prion disease.
A recent study by the ANCJDR of prion disease in Aboriginal and Torres Strait Islander people has confirmed that sporadic CJD occurs in Aboriginal and Torres Strait Islander people throughout Australia with a phenotype and incidence rate equivalent to non-Indigenous Australians, supporting the adequacy of national human prion disease surveillance.14 No vCJD or further iCJD cases were confirmed in Australia during 2020. The most recent human cadaveric pituitary gonadotrophin-related CJD death occurred in 1991, while the most recent Lyodura-related CJD death occurred in 2000. In 2020, the USA and UK both reported one case of iatrogenic CJD associated with pituitary growth hormone. Germany and Italy reported one case of iCJD each, associated with dura mater transplants.
Since vCJD was first reported in 1996, a total of 232 patients, from 12 countries, have been identified with this disease. The most recent vCJD case died in France in 2019. A recent vCJD case (death occurred in 2016) from the UK was the first to be reported as methioninevaline heterozygous at codon 129 of the PRNP gene;15 all cases previously had been methionine homozygous. The patient was 36 years old when he presented with psychiatric symptoms prior to onset of neurological features that included cognitive decline, ataxia and myoclonus, dying after an illness of 20 months. CSF 14-3-3 and RT-QuIC were negative. Brain magnetic resonance imaging (MRI) revealed features more typical of sporadic CJD (bilateral high signal in basal ganglia) without any posterior thalamic high signal (‘pulvinar sign’). The patient did not meet the epidemiologic diagnostic surveillance criteria for ‘probable’ or ‘possible’ vCJD, although fulfilled criteria for ‘probable’ sporadic CJD; neuropathology, including western blot glycotyping, was typical of vCJD. It remains uncertain whether this case marks the start of a second wave of vCJD affecting those heterozygous for methionine-valine at codon 129.
This case also underscores the importance of performing suspect CJD brain autopsy examinations and the benefits of maintaining highlevel surveillance within Australia. Although the horizontal transmission of amyloid beta (Aβ) peptides associated with Alzheimer’s disease through contaminated pituitary hormone treatments and dura mater grafts is essentially proven,16-18 it remains contentious, but possible, that such inadvertent inoculation can also eventuate in a disease phenotype.19 Prion protein and Aβ protein share similar properties, such as prion-like mechanisms of template-directed protein propagation, as well as inter-cellular spread of the misfolded protein isoforms and formation of larger fibrils. The long incubation periods of these proteins can result in clinical and pathological evidence of disease becoming apparent only decades after inoculation. Further studies are required to resolve this important issue and the risk of Aβ peptide transmission during routine surgical procedures. History of neurosurgery, embolization procedures with the use of dura in infant or youth age and any other therapeutic procedures involving the use of dura mater or cadaver-derived pituitary hormones should be searched for in patients who develop early-onset and cerebral amyloid angiopathy (CAA).
Acknowledgements
The ANCJDR wishes to thank families, as well as medical practitioners and associated staff, for their generous support of Australian CJD surveillance. The ANCJDR also thanks Associate Professor Handan Wand, Professor Matthew Law and Professor John Kaldor (The Kirby Centre; formerly the National Centre in HIV Epidemiology and Clinical Research at the University of New South Wales) for their expert ad hoc epidemiological and statistical support, as well as the CJD Support Group Network for their assistance in surveillance activities. Author details
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
Background
Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.
Methods
Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.
Results
I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.
Conclusions
There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.
end...tss
Ann N Y Acad Sci. 1982;396:131-43.
Alzheimer's disease and transmissible virus dementia (Creutzfeldt-Jakob disease).
Brown P, Salazar AM, Gibbs CJ Jr, Gajdusek DC.
Abstract
Ample justification exists on clinical, pathologic, and biologic grounds for considering a similar pathogenesis for AD and the spongiform virus encephalopathies. However, the crux of the comparison rests squarely on results of attempts to transmit AD to experimental animals, and these results have not as yet validated a common etiology. Investigations of the biologic similarities between AD and the spongiform virus encephalopathies proceed in several laboratories, and our own observation of inoculated animals will be continued in the hope that incubation periods for AD may be even longer than those of CJD.
https://nyaspubs.onlinelibrary.wiley.com/doi/abs/10.1111/j.1749-6632.1982.tb26849.x
http://onlinelibrary.wiley.com/doi/10.1111/j.1749-6632.1982.tb26849.x/abstract
Background
Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.
Methods
Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.
Results
I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.
Conclusions
There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.
end...tss
Ann N Y Acad Sci. 1982;396:131-43.
Alzheimer's disease and transmissible virus dementia (Creutzfeldt-Jakob disease).
Brown P, Salazar AM, Gibbs CJ Jr, Gajdusek DC.
Abstract
Ample justification exists on clinical, pathologic, and biologic grounds for considering a similar pathogenesis for AD and the spongiform virus encephalopathies. However, the crux of the comparison rests squarely on results of attempts to transmit AD to experimental animals, and these results have not as yet validated a common etiology. Investigations of the biologic similarities between AD and the spongiform virus encephalopathies proceed in several laboratories, and our own observation of inoculated animals will be continued in the hope that incubation periods for AD may be even longer than those of CJD.
https://nyaspubs.onlinelibrary.wiley.com/doi/abs/10.1111/j.1749-6632.1982.tb26849.x
http://onlinelibrary.wiley.com/doi/10.1111/j.1749-6632.1982.tb26849.x/abstract
CJD1/9 0185 Ref: 1M51A
IN STRICT CONFIDENCE
Dr McGovern From: Dr A Wight Date: 5 January 1993 Copies: Dr Metters Dr Skinner Dr Pickles Dr Morris Mr Murray
TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES
1. CMO will wish to be aware that a meeting was held at DH yesterday, 4 January, to discuss the above findings. It was chaired by Professor Murray (Chairman of the MRC Co-ordinating Committee on Research in the Spongiform Encephalopathies in Man), and attended by relevant experts in the fields of Neurology, Neuropathology, molecular biology, amyloid biochemistry, and the spongiform encephalopathies, and by representatives of the MRC and AFRC. 2. Briefly, the meeting agreed that:
i) Dr Ridley et als findings of experimental induction of p amyloid in primates were valid, interesting and a significant advance in the understanding of neurodegenerative disorders;
ii) there were no immediate implications for the public health, and no further safeguards were thought to be necessary at present; and
iii) additional research was desirable, both epidemiological and at the molecular level. Possible avenues are being followed up by DH and the MRC, but the details will require further discussion. 93/01.05/4.1
IN STRICT CONFIDENCE
Dr McGovern From: Dr A Wight Date: 5 January 1993 Copies: Dr Metters Dr Skinner Dr Pickles Dr Morris Mr Murray
TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES
1. CMO will wish to be aware that a meeting was held at DH yesterday, 4 January, to discuss the above findings. It was chaired by Professor Murray (Chairman of the MRC Co-ordinating Committee on Research in the Spongiform Encephalopathies in Man), and attended by relevant experts in the fields of Neurology, Neuropathology, molecular biology, amyloid biochemistry, and the spongiform encephalopathies, and by representatives of the MRC and AFRC. 2. Briefly, the meeting agreed that:
i) Dr Ridley et als findings of experimental induction of p amyloid in primates were valid, interesting and a significant advance in the understanding of neurodegenerative disorders;
ii) there were no immediate implications for the public health, and no further safeguards were thought to be necessary at present; and
iii) additional research was desirable, both epidemiological and at the molecular level. Possible avenues are being followed up by DH and the MRC, but the details will require further discussion. 93/01.05/4.1
http://web.archive.org/web/20090506012455/http://www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf
BSE101/1 0136
IN CONFIDENCE
5 NOV 1992 CMO From: Dr J S Metters DCMO 4 November 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
1. Thank you for showing me Diana Dunstan's letter. I am glad that MRC have recognized the public sensitivity of these findings and intend to report them in their proper context. This hopefully will avoid misunderstanding and possible distortion by the media to portray the results as having more greater significance than the findings so far justify.
2. Using a highly unusual route of transmission (intra-cerebral injection) the researchers have demonstrated the transmission of a pathological process from two cases one of severe Alzheimer's disease the other of Gerstmann-Straussler disease to marmosets. However they have not demonstrated the transmission of either clinical condition as the "animals were behaving normally when killed'. As the report emphasizes the unanswered question is whether the disease condition would have revealed itself if the marmosets had lived longer. They are planning further research to see if the conditions, as opposed to the partial pathological process, is transmissible. What are the implications for public health?
3. The route of transmission is very specific and in the natural state of things highly unusual. However it could be argued that the results reveal a potential risk, in that brain tissue from these two patients has been shown to transmit a pathological process. Should therefore brain tissue from such cases be regarded as potentially infective? Pathologists, morticians, neuro surgeons and those assisting at neuro surgical procedures and others coming into contact with "raw" human brain tissue could in theory be at risk. However, on a priori grounds given the highly specific route of transmission in these experiments that risk must be negligible if the usual precautions for handling brain tissue are observed.
92/11.4/1-1 BSE101/1 0137
4. The other dimension to consider is the public reaction. To some extent the GSS case demonstrates little more than the transmission of BSE to a pig by intra-cerebral injection. If other prion diseases can be transmitted in this way it is little surprise that some pathological findings observed in GSS were also transmissible to a marmoset. But the transmission of features of Alzheimer's pathology is a different matter, given the much greater frequency of this disease and raises the unanswered question whether some cases are the result of a transmissible prion. The only tenable public line will be that "more research is required" before that hypothesis could be evaluated. The possibility on a transmissible prion remains open. In the meantime MRC needs carefully to consider the range and sequence of studies needed to follow through from the preliminary observations in these two cases. Not a particularly comfortable message, but until we know more about the causation of Alzheimer's disease the total reassurance is not practical.
JS METTERS Room 509 Richmond House Pager No: 081-884 3344 Callsign: DOH 832 121/YdeS 92/11.4/1.2
http://web.archive.org/web/20090506032549/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf
BSE101/1 0136
IN CONFIDENCE
CMO
From: Dr J S Metters DCMO
4 November 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
IN CONFIDENCE
CMO
From: Dr J S Metters DCMO
4 November 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
http://web.archive.org/web/20090506032549/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf
CJD1/9 0185
Ref: 1M51A
IN STRICT CONFIDENCE
From: Dr. A Wight Date: 5 January 1993
Copies:
Dr Metters Dr Skinner Dr Pickles Dr Morris Mr Murray
TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES
http://web.archive.org/web/20090506032549/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf
Tuesday, November 26, 2013
Transmission of multiple system atrophy prions to transgenic mice
‘’Our results provide compelling evidence that α-synuclein aggregates formed in the brains of MSA patients are transmissible and, as such, are prions.’’
http://www.pnas.org/content/110/48/19555.abstract.html
Transmission of multiple system atrophy prions to transgenic mice
‘’Our results provide compelling evidence that α-synuclein aggregates formed in the brains of MSA patients are transmissible and, as such, are prions.’’
http://www.pnas.org/content/110/48/19555.abstract.html
Transmission of a neurodegenerative disorder from humans to mice
The findings suggest that the α-synuclein deposits that form in the brains of patients with MSA behave like prions and are transmissible under certain circumstances, according to the authors. — N.Z.
α-Synuclein deposits in the brainstems of inoculated mice.
https://www.pnas.org/content/pnas/110/48/19175.full.pdf
kind regards, terry
The findings suggest that the α-synuclein deposits that form in the brains of patients with MSA behave like prions and are transmissible under certain circumstances, according to the authors. — N.Z.
α-Synuclein deposits in the brainstems of inoculated mice.
https://www.pnas.org/content/pnas/110/48/19175.full.pdf
kind regards, terry
No competing interests declared.
Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease
*** Singeltary comment PLoS ***
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
IN CONFIDENCE
5 NOVEMBER 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication.
There are also results to be made available shortly
(1) concerning a farmer with CJD who had BSE animals,
(2) on the possible transmissibility of Alzheimer’s and
(3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]
re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)
Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
>>> The only tenable public line will be that "more research is required’’ <<<
>>> possibility on a transmissible prion remains open<<<
O.K., so it’s about 23 years later, so somebody please tell me, when is "more research is required’’ enough time for evaluation ?
Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)
snip...see full Singeltary Nature comment here;
Alzheimer's disease
let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease...
Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Singeltary Comment at very bottom of this Nature publishing;
re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
I would kindly like to comment on the Nature Paper, the Lancet reply, and the newspaper articles.
First, I applaud Nature, the Scientist and Authors of the Nature paper, for bringing this important finding to the attention of the public domain, and the media for printing said findings.
Secondly, it seems once again, politics is getting in the way possibly of more important Transmissible Spongiform Encephalopathy TSE Prion scientific findings. findings that could have great implications for human health, and great implications for the medical surgical arena. but apparently, the government peer review process, of the peer review science, tries to intervene again to water down said disturbing findings.
where have we all heard this before? it's been well documented via the BSE Inquiry. have they not learned a lesson from the last time?
we have seen this time and time again in England (and other Country's) with the BSE mad cow TSE Prion debacle.
That 'anonymous' Lancet editorial was disgraceful. The editor, Dick Horton is not a scientist.
The pituitary cadavers were very likely elderly and among them some were on their way to CJD or Alzheimer's. Not a bit unusual. Then the recipients ?
who got pooled extracts injected from thousands of cadavers ? were 100% certain to have been injected with both seeds. No surprise that they got both diseases going after thirty year incubations.
That the UK has a "system in place to assist science journalists" to squash embargoed science reports they find 'alarming' is pathetic.
Sounds like the journalists had it right in the first place: 'Alzheimer's may be a transmissible infection' in The Independent to 'You can catch Alzheimer's' in The Daily Mirror or 'Alzheimer's bombshell' in The Daily Express
if not for the journalist, the layperson would not know about these important findings.
where would we be today with sound science, from where we were 30 years ago, if not for the cloak of secrecy and save the industry at all cost mentality?
when you have a peer review system for science, from which a government constantly circumvents, then you have a problem with science, and humans die.
to date, as far as documented body bag count, with all TSE prion named to date, that count is still relatively low (one was too many in my case, Mom hvCJD), however that changes drastically once the TSE Prion link is made with Alzheimer's, the price of poker goes up drastically.
so, who makes that final decision, and how many more decades do we have to wait?
the iatrogenic mode of transmission of TSE prion, the many routes there from, load factor, threshold from said load factor to sub-clinical disease, to clinical disease, to death, much time is there to spread a TSE Prion to anywhere, but whom, by whom, and when, do we make that final decision to do something about it globally? how many documented body bags does it take? how many more decades do we wait? how many names can we make up for one disease, TSE prion?
Professor Collinge et al, and others, have had troubles in the past with the Government meddling in scientific findings, that might in some way involve industry, never mind human and or animal health.
FOR any government to continue to circumvent science for monetary gain, fear factor, or any reason, shame, shame on you.
in my opinion, it's one of the reasons we are at where we are at to date, with regards to the TSE Prion disease science i.e. money, industry, politics, then comes science, in that order.
greed, corporate, lobbyist there from, and government, must be removed from the peer review process of sound science, it's bad enough having them in the pharmaceutical aspect of healthcare policy making, in my opinion.
my mother died from confirmed hvCJD, and her brother (my uncle) Alzheimer's of some type (no autopsy?). just made a promise, never forget, and never let them forget, before I do.
I kindly wish to remind the public of the past, and a possible future we all hopes never happens again. ...
[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. There are also results to be made available shortly (1) concerning a farmer with CJD who had BSE animals, (2) on the possible transmissibility of Alzheimer's and (3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]
Singeltary Comment at very bottom of this Nature publishing;
THURSDAY, FEBRUARY 7, 2019
In Alzheimer's Mice, Decades-Old Human Cadaveric Pituitary Growth Hormone Samples Can Transmit and Seed Amyloid-Beta Pathology
TUESDAY, OCTOBER 6, 2020
Potential human transmission of amyloid β pathology: surveillance and risks
LANCET
VOLUME 19, ISSUE 10, P872-878, OCTOBER 01, 2020
Wednesday, December 16, 2020
Expanding spectrum of prion diseases Prusiner et al
SATURDAY, JANUARY 12, 2019
Australia Creutzfeldt-Jakob disease surveillance: update to December 2016 Published December 17, 2018
SATURDAY, FEBRUARY 07, 2015
Annual report Creutzfeldt-Jakob disease surveillance in Australia, 2013
MONDAY, FEBRUARY 24, 2014
UPDATE ON CREUTZFELDT–JAKOB DISEASE Masters et al Australia
Saturday, November 09, 2013
Surveillance for creutzfeldt-Jakob disease in Australia: update to December 2012
Tuesday, March 16, 2010
COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef
FRIDAY, 5 FEBRUARY 2010 AUSTRALIA
COMMONWEALTH OF AUSTRALIA
Proof Committee Hansard
RRA&T 2 Senate Friday, 5 February 2010
RURAL AND REGIONAL AFFAIRS AND TRANSPORT
[9.03 am]
BELLINGER, Mr Brad, Chairman, Australian Beef Association CARTER, Mr John Edward, Director, Australian Beef Association CHAIR—Welcome. Would you like to make an opening statement? Mr Bellinger—Thank you. The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He states, and rightfully so: You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heidenhain variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. He goes on to state:
snip...see full text 110 pages ;
new url link;
Australia Atypical scrapie 5 cases diagnosed in 2016
RW Cook,a* J Bingham,bAS Besier,cCL Bayley,dM Hawes,ePL Shearer,fM Yamada,bJ Bergfeld,bDT Williamsband DJ Middletonb
Background
Since its initial detection in Norway in 1998, atypical scrapie (‘atypical/Nor98 scrapie’) has been reported in sheep in the majority of European countries (including in regions free of classical scrapie) and in the Falkland Islands, the USA, Canada,New Zealand and Australia.
Case series
The diagnosis in Australia of atypical scrapie in four Merino and one Merino-cross sheep showing clinical signs of neurological disease was based on the detection of grey matter neuropil vacuolation (spongiform change) in the brain (particularly inthe molecular layer of the cerebellar cortex) and associated abnormal prion protein (PrPSc) deposition in both grey and white matter. Changes were minimal in the caudal brainstem, the predilection site for lesions of classical scrapie.
Conclusion
The distinctive lesion profile of atypical scrapie in these five sheep highlights the diagnostic importance of routine histological evaluation of the cerebellum for evidence of neuropil vacuolation and associated PrPSc deposition in adult sheep with suspected neurological disease.
Keywords atypical scrapie; prion disease; sheep; transmissible spongiform encephalopathy
Abbreviations ANZSDP, Australian and New Zealand StandardDiagnostic Procedure; CNS, central nervous system; DMNV, dorsalmotor nucleus of the vagus nerve; H&E, haematoxylin and eosin;IHC, immunohistochemistry; NTSESP, National TSE SurveillanceProgram; PrPSc, abnormal prion protein isomer; TSE, transmissiblespongiform encephalopathy.
Aust Vet J 2016;94:452–455 doi:http://10.1111/avj.12529
THURSDAY, OCTOBER 7, 2010
Australia first documented case of atypical scrapie confirmed
Wednesday, July 28, 2021
France issues moratorium on prion research after fatal brain disease strikes two lab workers
Thursday, July 29, 2021
TSE PRION OCCUPATIONAL EXPOSURE VIA ANIMAL OR HUMAN, iatrogenic transmission, nvCJD or sCJD, what if?
Terry S. Singeltary Sr.