Wednesday, April 02, 2014

Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems

Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems
 
 
Atsushi Kobayashi, Yuichi Matsuura, Shirou Mohri and Tetsuyuki Kitamoto

 

Author Affiliations For all author emails, please log on.

 

Acta Neuropathologica Communications 2014, 2:32 doi:10.1186/2051-5960-2-32

 

Published: 31 March 2014 Abstract (provisional) Dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) can be divided into two subgroups that exhibit distinct clinical and neuropathological features, with the majority represented by a non-plaque-type of dCJD (np-dCJD) and the minority by a plaque-type of dCJD (p-dCJD). The two distinct phenotypes of dCJD had been considered to be unrelated to the genotype (methionine, M or valine, V) at polymorphic codon 129 of the PRNP gene or type (type 1 or type 2) of abnormal isoform of prion protein (PrPSc) in the brain, while these are major determinants of clinicopathological phenotypes of sporadic CJD (sCJD). The reason for the existence of two distinct subgroups in dCJD had remained elusive. Recent progress in research of the pathogenesis of dCJD has revealed that two distinct subgroups of dCJD are caused by infection with different PrPSc strains from sCJD, i.e., np-dCJD caused by infection with sCJD-MM1/MV1, and p-dCJD caused by infection with sCJD-VV2 or -MV2. These studies have also revealed previously unrecognized problems as follows: (i) the numbers of p-dCJD patients may increase in the future, (ii) the potential risks of secondary infection from dCJD, particularly from p-dCJD, may be considerable, and (iii) the effectiveness of the current PrPSc decontamination procedures against the PrPSc from p-dCJD is uncertain. To prevent secondary infection from p-dCJD, the establishment of effective decontamination procedures is an urgent issue. In this review, we summarize the past and future problems surrounding dCJD.

 

snip...

 

Concluding remarks Recent progress in the study of the pathogenesis of dCJD has revealed that the two distinct subgroups of dCJD are caused by infection with different PrPSc strains of sCJD, i.e., np-dCJD caused by M1 PrPSc from sCJD-MM1/MV1 and p-dCJD caused by Mi PrPSc and/or V2 PrPSc from sCJD-VV2, −MV2K, or -MV2K + C. Studies have also revealed previously unrecognized problems such as the considerable risks of secondary infection from dCJD, particularly from p-dCJD. To prevent secondary infection from p-dCJD, the effectiveness of the current decontamination procedures should be tested urgently using sensitive Mi PrPSc detection systems.

 


 


 

Saturday, April 20, 2013

 

Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan

 


 

Thursday, December 08, 2011

 

A case of Iatrogenic Creutzfeldt Jakob Disease (iCJD) in a patient who had received a German-manufactured human dura mater graft 23 years ago

 


 

 

[scroll down past article for my comments]

 

Subject: Creutzfeldt-Jakob disease: implications for gastroenterology & CJD 38 years after _diagnostic_ use of hGH (Iatrogenic CJDs & sporadic CJDs)

 

Date: Mon, 17 Jun 2002 16:46:46 –0700

 

From: "Terry S. Singeltary Sr."

 

Reply-To: Bovine Spongiform Encephalopathy To: BSE-L@uni-karlsruhe.de

 

snip...end

 

 


 

 

“Cases of vCJD peaked in 2000, leading some scientists to speculate that the disease has an incubation period of about a decade. Yet studies of different forms of CJD suggest that the incubation time of vCJD could be much longer, indicating that many people in Britain could be carrying the infection without symptoms.”

 


 

Monday, October 14, 2013

 

Researchers estimate one in 2,000 people in the UK carry variant CJD proteins

 


 

However, I think that the specific confusion there is that people talk about sporadic CJD occurring at 1 per million. That is not your individual risk. Your risk is 1 per million every year. Actually, it is nearer 2 per million per year of the population will develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 1 in 30,000. So that has not really changed. When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.

 


 

Cases of vCJD peaked in 2000, leading some scientists to speculate that the disease has an incubation period of about a decade. Yet studies of different forms of CJD suggest that the incubation time of vCJD could be much longer, indicating that many people in Britain could be carrying the infection without symptoms.

 


 

Tuesday, March 11, 2014

 

Science and Technology Committee Oral evidence: Blood, tissue and organ screening, HC 990 Wednesday 5 March 2014 SPORADIC CJD

 

Actually, it is nearer 2 per million per year of the population will develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 1 in 30,000. So that has not really changed. When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.

 


 
Wednesday, January 15, 2014

INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014
http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/infection-prevention-and-control-of-cjd.html

 

Sunday, March 09, 2014

 

A Creutzfeldt-Jakob Disease (CJD) Lookback Study: Assessing the Risk of Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease

 

FDA TSEAC CIRCUS AND TRAVELING ROAD SHOW FOR THE TSE PRION DISEASES

 


 

*** Because typical clinical signs of BSE cannot always be observed in nonambulatory disabled cattle, and because evidence has indicated these cattle are more likely to have BSE than apparently healthy cattle, FDA is designating material from nonambulatory disabled cattle as prohibited cattle materials.

 


 


 


 


 

Tuesday, April 01, 2014

 

Questions linger in U.S. CJD cases 2005, and still do in 2014

 


 

 

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