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.
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
TSS
posted by Terry S. Singeltary Sr. at
11:57 AM
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