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Sunday, September 12, 2010

PRION DISEASE UPDATE 2010 (08) / New Strain Found


A ProMED-mail post

ProMED-mail is a program of the

International Society for Infectious Diseases

[With the continuing decline of the number of cases in the human population

of variant Creutzfeldt-Jakob disease -- abbreviated previously as vCJD or

CJD (new var.) in ProMED-mail -- it has been decided to broaden the scope

of the occasional ProMED-mail updates to include other prion-related

diseases. In addition to vCJD, data on other forms of CJD: sporadic,

iatrogenic, familial, and GSS (Gerstmann-Straussler-Scheinker disease) are

included also since they may have some relevance to the incidence and

etiology of vCJD. - Mod.CP]

In this update:

[1] UK: National CJD Surveillance Unit -- monthly statistics as of Mon 6

Sep 2010 -- no new vCJD cases

[2] France: Institut de Veille Sanitaire -- monthly statistics as of Wed 1

Sep 2010 -- no new vCJD cases

[3] USA - National Prion Disease Pathology Surveillance Center -- Sat 31

Aug 2010

[4] New sporadic prion disease


[1] UK: National CJD Surveillance Unit -- monthly statistics as of Mon 6

Sep 2010 -- no new vCJD cases

Date: Mon 6 Sep 2010

Source: UK National CJD Surveillance Unit, monthly statistics [edited]

The number of deaths due to definite or probable vCJD cases remains 169. A

total of 4 definite/probable patients are still alive so that the total

number of definite or probable vCJD cases is still 173. So far, 2

fatalities have been recorded in 2010.

Although 3 new deaths due to vCJD were recorded in 2009 and 2 deaths so far

in 2010, the overall picture is still consistent with the view that the

vCJD outbreak in the UK is in decline, albeit now with a pronounced tail.

The 1st cases were observed in 1995, and the peak number of deaths was 28

in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in

2004, 5 in 2005, 5 in 2006, 5 in 2007, one in 2008, 3 in 2009, and now 2 so

far in 2010.

Totals for all types of CJD cases in the UK so far in the year 2010


During the 1st 8 months of 2010, there have been 95 referrals, 29 fatal

cases of sporadic CJD, 2 fatal cases of vCJD, 2 cases of iatrogenic CJD,

and single cases of familial CJD and GSS.


communicated by:



[2] France: Institut de Veille Sanitaire -- monthly statistics as of 1 Sep

2010 -- no new vCJD cases

Date: Wed 1 Sep 2010

Source: IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees [in

French, trans. & summ. Mod.CP, edited]

During the 1st 8 months of 2010, there were 1090 referrals, 47 confirmed

cases of sporadic CJD, one case of familial CJD, and no cases of iatrogenic

CJD or vCJD.

A total of 25 cases of confirmed or probable vCJD have now been recorded in

France since records began in 1992. There was 1 case in 1996, 1 in 2000, 1

in 2001, 3 in 2002, 2 in 2004, 6 in 2005, 6 in 2006, 3 in 2007, 2 in 2009,

and none so far in 2010.

The 25 confirmed cases comprise 13 females and 12 males. All 25 are now

deceased. Their median age is 37 (between 19 and 58). 7 were resident in

the Ile-de-France and 18 in the provinces. All the identified cases have

been Met-Met homozygotes. No risk factor has been identified. One of the 25

had made frequent visits to the United Kingdom.


communicated by:



[3] USA - NPDPSC 31 Aug 2010 -- no indigenous vCJD cases

Date: Sat 31 Aug 2010

Source: US National Prion Disease Pathology Surveillance Center [edited]

During the 8 month period 1 Jan 2010 to 31 Aug 2010, there were 204

referrals, 124 of whom were classified as prion disease, comprising 85

cases of sporadic CJD, 20 of familial CJD, and no cases of iatrogenic CJD

or vCJD.

Since 1996 when records began there have been 3702 referrals, of which 2177

were classified as prion disease, comprising 1834 cases of sporadic CJD,

315 of familial CJD, 4 of iatrogenic CJD, and 3 of vCJD. The 3 cases of

VCJD, 2 were considered to have been contracted in in the United Kingdom

and one Saudi Arabia. The prion disease category includes 21 (19 in 2010)

cases with type determination pending in which vCJD has been excluded.

[There is a striking difference in the frequency of familial (genetic) CJD

in the three countries. In the UK familial CJD accounts for about 5 per

cent of all cases of CJD, in France it is about 2.4 to 5 per cent, whereas

in the USA it is 16 per cent. The relevant demographic and/or genetic

factors merit investigation. - Mod.CP]


communicated by:



[4] New sporadic prion disease

Date: Fri 13 Aug 2010

Source: Science Daily [edited]

New sporadic prion protein disease:

variably protease-sensitive prionopathy shares genotype characteristics

with Creutzfeldt-Jakob


A new sporadic prion protein disease has been discovered. Variably

protease-sensitive prionopathy (VPSPr), as it has been named, is the 2nd

type of complete sporadic disease to be identified since Creutzfeldt-Jakob

disease (CJD) was reported in the 1920s. The landmark finding from the

National Prion Disease Pathology Surveillance Center at Case Western

Reserve University is published in the August [2010] issue of Annals of

Neurology [see abstract below].

Normally, the human prion protein gene comes in 3 types due to its

capability to encode prion proteins that contain only the amino acid

methionine, commonly identified as M, both methionine and valine, commonly

identified as V, or only for the amino acid valine at position 129.

Therefore, when it comes to the prion protein gene unaffected people can be

identified as 129MM, 129MV or 129VV. Sporadic CJD (sCJD), which is the most

common human prion disease, can affect patients who have any one of the 3

types of the prion protein gene.

In 2008, Pierluigi Gambetti and Wen-Quan Zou, with collaborators, reported

the discovery of this novel disease, which affected patients who exhibit

only one of the 3 types of the prion protein gene. In this follow-up study,

they discovered that all 3 genetic groups can be affected also by this

novel disease which now joins sCJD in displaying this feature. However,

VPSPr is associated with an abnormal prion protein that exhibits

characteristics very different from those of sCJD, as well as other prion

diseases, suggesting that it may be caused by a different mechanism,

perhaps more akin to other neurodegenerative diseases, such as Alzheimer's

disease. This finding may exemplify, for the 1st time, the possibility that

the prion protein affects the brain with different mechanisms.

While examining cases received at the National Prion Disease Pathology

Surveillance Center where he is the director, Dr Gambetti observed that a

subset of cases had clinical and pathological features quite different from

those of all known types of human prion diseases. Further, after being

tested for prion proteins via the Western blot [technique] -- the gold

standard of prion disease diagnosis -- the cases were negative. Dr Gambetti

then collaborated with Dr Zou, associate director at the center, to solve

the riddle of a disease that exhibited some features of a prion disease in

histopathological examination but was negative using the standard Western

blot test.

Dr Zou's lab performed a full characterization of the disease and

discovered that the VPSPr-associated abnormal prion protein formed a

ladder-like electrophoretic profile on Western blotting. "When I obtained

the 1st Western blot result of these cases with a different antibody

against prions, I was surprised that these cases consistently exhibited

this particular profile; one that I had never seen in my more than 10 years

of work on human prion diseases," Dr Zou, assistant professor of pathology

at Case Western Reserve School of Medicine, recalls. This ladder-like

profile is quite distinctive and very different from the profile of common

prion diseases. "Discovery of this unique type of prion provides solid

evidence that this novel disease may possess a pathogenesis that is

different from that of the major prion diseases currently known," Dr Zou adds.

Despite extensive research, a relatively large group of neurodegenerative

diseases associated with dementia remain undefined. Before being discovered

and characterized, VPSPr was one of the undefined dementing diseases. The

discovery of VPSPr is chipping away at that group. In the 2 years since its

discovery, more than 30 cases have been reported.

"If, as the current evidence indicates, the VPSPr mechanism of affecting

the brain is different from that of other sporadic prion diseases, such as

sCJD, the discovery of VPSPr would also provide the 1st example that the

prion protein may spontaneously damage the brain with different

mechanisms," concludes Dr Gambetti, professor of pathology at Case Western

Reserve School of Medicine. "This might apply to other dementing illnesses

as well, and has implications for the strategies that need to be followed

to attain a cure."

Drs Gambetti and Zou, along with their extensive research team, plan to

further characterize the abnormal prion protein associated with VPSPr as

well as other important features of the protein, such as the disease's

propensity for transmission upon inoculation and its replication in test

tubes. These features in VPSPr will be compared with those of sCJD to

obtain a complete picture of how the abnormal prion protein attacks the

brain in these 2 diseases.


communicated by:

ProMED-mail rapporteur Mary Marshall

[The following is the reference for the paper discussed above followed by

the authors' abstract:

Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, et al. Variably

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

protein. Ann Neurol. 2010 Aug;68(2):162-72.


Department of Pathology, National Prion Disease Pathology Surveillance

Center, Case Western Reserve University, Cleveland, Ohio.



Objective: The objective of the study is to report 2 new genotypic forms of

protease-sensitive prionopathy (PSPr), a novel prion disease described in

2008, in 11 subjects all homozygous for valine at codon 129 of the prion

protein (PrP) gene (129VV). The 2 new PSPr forms affect individuals who are

either homozygous for methionine (129MM) or heterozygous for

methionine/valine (129MV).

Methods: A total of 15 affected subjects with 129MM, 129MV, and 129VV

underwent comparative evaluation at the National Prion Disease Pathology

Surveillance Center for clinical, histopathologic, immunohistochemical,

genotypical, and PrP characteristics.

Results: Disease duration (between 22 and 45 months) was significantly

different in the 129VV and 129MV subjects. Most other phenotypic features

along with the PrP electrophoretic profile were similar but distinguishable

in the 3 129 genotypes. A major difference laid in the sensitivity to

protease digestion of the disease-associated PrP, which was high in 129VV

but much lower, or altogether lacking, in 129MV and 129MM. This difference

prompted the substitution of the original designation with "variably

protease- sensitive prionopathy" (VPSPr). None of the subjects had

mutations in the PrP gene coding region.

Interpretation: Because all 3 129 genotypes are involved, and are

associated with distinguishable phenotypes, VPSPr becomes the 2nd sporadic

prion protein disease with this feature after Creutzfeldt-Jakob disease,

originally reported in 1920. However, the characteristics of the abnormal

prion protein suggest that VPSPr is different from typical prion diseases,

and perhaps more akin to subtypes of Gerstmann-Straussler-Scheinker disease.

VPSPr is not linked to eating infected meat. However, like CJD, the new

condition happens sporadically. It was 1st identified because of the

fast-advancing form of dementia seen in those affected. They were also

unable to speak or move. But tests for CJD proved negative. Further

molecular examination as described above has shown VPSPr was a prion

disease, but one which looked very different to those already known. - Mod.CP]

[see also:

Prion disease update 2010 (07) 20100809.2720

Prion disease update 2010 (06) 20100706.2248

Prion disease update 2010 (05) 20100507.1488

Prion disease update 2010 (04) 20100405.1091

Prion disease update 2010 (03) 20100304.0709

Prion disease update 2010 (02) 20100205.0386

Prion disease update 2010 20100107.0076



Prion disease update 2009 (10) 20091103.3784

vCJD - Italy: susp. 20091024.3671

Prion disease update 2009 (09) 20091005.3461

Prion disease update 2009 (08) 20090908.3170

Prion disease update 2009 (07) 20090806.2783

Prion disease update 2009 (06) 20090706.2433

Prion disease update 2009 (05) 20090602.2054

Prion disease update 2009 (04) 20090406.1337

vCJD, 5th death - Spain (Cantabria) 20090307.0953

Prion disease update 2009 (03) 20090305.0918

Prion disease update 2009 (02) 20090202.0463

Prion disease update 2009 (01) 20090108.0076



Prion disease update 2008 (14): new vCJD wave imminent? 20081218.3980

Prion disease update 2008 (13) 20081201.3780

Prion disease update 2008 (12) 20081103.345

Prion disease update 2008 (11) 20081006.3159

vCJD, mother & son - Spain: (Leon) 20080926.3051

Prion disease update 2008 (10) 20080902.2742

vCJD - Spain: susp. 20080410.1311

Prion disease update 2008 (05) 20080408.1285

Prion disease update 2008 (01): correction 20080104.0046

Prion disease update 2008 (01) 20080102.0014



Prion disease update 2007 (08) 20071205.3923

Prion disease update 2007 (07) 20071105.3602

Prion disease update 2007 (06) 20071003.3269

Prion disease update 2007 (05) 20070901.2879

Prion disease update 2007 (04) 20070806.2560

Prion disease update 2007 (03) 20070702.2112

Prion disease update 2007 (02) 20070604.1812

Prion disease update 2007 20070514.1542

CJD (new var.) update 2007 (05) 20070403.1130

CJD (new var.) update 2007 (04) 20070305.0780

CJD (new var.) update 2007 (03) 20070205.0455

CJD (new var.) update 2007 (02): South Korea, susp 20070115.0199



CJD (new var.), blood transfusion risk 20061208.3468

CJD, transmission risk - Canada (ON) 20061207.3457

CJD (new var.) update 2006 (12) 20061205.3431

CJD (new var.) update 2006 (11) 20061106.3190

CJD (new var.) update 2006 (10) 20061002.2820

CJD (new var.) - Netherlands: 2nd case 20060623.1741

CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432

CJD (new var.) update 2006 (02) 20060206.0386

CJD (new var.) update 2006 20060111.0101



CJD (new var.) update 2005 (12) 20051209.3547

CJD (new var.) update 2005 (11) 20051108.3270

CJD (new var.) update 2005 (10) 20051006.2916

CJD (new var.) update 2005 (02) 20050211.0467

CJD (new var.) - UK: update 2005 (01) 20050111.0095]




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