Thursday, August 05, 2010

Surveillance of Creutzfeldt-Jakob disease in Australia: 2010 update

Surveillance of Creutzfeldt-Jakob disease in Australia: 2010 update

Genevieve M Klug, Alison Boyd, Amelia McGlade, Christiane Stehmann, Colin L Masters, Steven J Collins Abstract Surveillance of all human prion diseases in Australia has been the responsibility of the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) on behalf of the Australian Government Department of Health and Ageing since the Registry’s inception in October 1993. The ANCJDR was established in response to the identification of 4 CJD deaths in recipients of human-derived pituitary hormone. The initial brief was to perform focused surveillance for any further iatrogenic cases of CJD; however the scope of surveillance was soon expanded to include all cases of CJD occurring in Australia both prospectively and retrospectively to 1970. The activities of the ANCJDR have evolved from: routine surveillance responsibilities to detailed epidemiological analysis at both national and international levels; expert advice in relation to, and management of, infection control issues; and the provision of a number of tests to aid the diagnosis and classification of CJD in suspect cases. In this brief report, surveillance outcomes are examined with the inclusion of figures from the reporting period of 1 April 2009 to 31 March 2010 and the diagnostic services offered by the ANCJDR are outlined to provide a greater insight into this aspect of the Registry. Commun Dis Intell 2010;34(2):96–101.

Keywords: Australian National Creutzfeldt-Jakob Disease Registry, CJD, human-derived pituitary hormone treatment, transmissible spongiform encephalopathies

Table 2: Transmissible spongiform encephalopathy (TSE) deaths and mortality rate, by state or territory

State or territory

TSE cases by year of death Total



Mean age-adjusted

mortality rate


00 01 02 03 04 05 06 07 08 09 10* 00–09† 93–09†

ACT 1 1 1 2 5 1.4 1.3

NSW 12 9 7 7 11 10 11 10 5 8 90 1.3 1.2

NT 2 1 3 1.0 0.9

Qld 7 3 3 3 7 2 4 2 31 0.8 1.0

SA 2 1 2 1 3 4 2 15 0.9 1.2

Tas 2 1 2 5 0.9 0.7

Vic 9 10 5 9 5 11 9 6 12 3 1 80 1.5 1.4

WA 2 1 2 3 2 4 4 6 4 28 1.3 1.5

Australia 32 23 20 23 21 26 37 28 31 15 1 257 1.2 1.2

* Provisional result for year to 31 March 2010.

† Includes all deaths occurring between the complete years 1 January 1993 or 1 January 2000 and 31 December 2009.$FILE/cdi3402b.pdf

Interestingly, when you have cases of cjd increasing yearly, it's supposedly due to better surveillance etc. yet here we see, cjd _decreased_ in 2009, and only a few bumps up and down since 2000, pretty much holding the same, and this is with _better_ surveillance. so, really, the myth that sCJD increases due to better surveillance, is just that, a myth. sporadic cjd and nvCJD increased with countries with BSE, and as the feed ban was put into place and enforcement took hold, BSE cases dropped, along with nvCJD cases and sporadic CJD cases in BSE countries. interesting is it not. However, I wonder how this would play out over the next decade or so, if Australia starts importing products from BSE mad cow Countries ??? something to ponder for sure, down under. ...TSS

Friday, November 06, 2009

Surveillance of Creutzfeldt-Jakob disease in Australia: 2009 update

Thursday, July 08, 2010

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

Thursday, July 08, 2010


Saturday, July 17, 2010

Variant Creutzfeldt-Jakob disease Ironside JW., Haemophilia. 2010 Jul;16 Suppl 5:175-80


Tuesday, June 1, 2010

USA cases of dpCJD rising with 24 cases so far in 2010

Wednesday, June 16, 2010

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties

Friday, November 30, 2007


Wednesday, July 28, 2010

re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010

Wednesday, July 28, 2010

Atypical prion proteins and IBNC in cattle DEFRA project code SE1796 FOIA Final report

Tuesday, August 03, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

SO, cows can transmit BSE to humans as nvCJD in every country but the USA, but here in the USA, it's a new prionpathy that is genetic, but yet sporadic, because of no related mutation, not related to cows, even though one of the USA mad cows has the same type genetics, and even though Kong et al said that same type cow h-BSE is transmissible to humans. interesting isn't it $$$

SOUND SCIENCE and public health, should NOT be based on 'may not's, and 'may's'. BUT that is how the O.I.E. and the U.S.D.A. have operated for years and years. The whole mad cow debackle was based on 'may's' and 'may not's', and look where it has gotten us. The O.I.E. and the U.S.D.A. systematically changed T.S.E. science to meet their needs, i.e. TRADE, TRADE, TRADE $$$ HOW in the world, or the better question might be WHY would you put the cart before the horse so to speak with human and animal life, on a disease that we know is 100% fatal once exposed and clininal. THE theory of no transmission of typical scrapie to humans, this theory and any evidence there from is very thin to say the least. Let's look at some sound science on atypical Nor-98 Scrapie, shall we ; [Although atypical scrapie is not yet ruled out, it is important to realize this is a type of scrapie that thus far has only tended to appear as a sporadic condition in older animals. Currently it has not been shown to follow the same genetic tendencies for propagation as the usual scrapie. However, the atypical phenotypic appearance has been shown to be preserved on experimental passage. Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries, as Australia may join that list. It is likely that this case will be sent to the UK for definitive conformation. [Ref: M Simmons, T Konold, L Thurston, et al. BMC Veterinary Research 2010, 6:14 [provisional abstract available at ]

"Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]

"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."

Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG],F2400_P1001_PUB_MAIL_ID:1000,81729

Saturday, June 5, 2010

Research Project: Transmissible Spongiform Encephalopathies: Identification of atypical scrapie in Canadian sheep

Sunday, April 18, 2010


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

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