Human Mad-Cow Disease

PRION DISEASE UPDATE 2010 (06)
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[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 5 Jul 2010 -- one new vCJD case
[2] France: Institut de Veille Sanitaire -- monthly statistics as of
1 Jul 2010 -- no new vCJD cases
[3] USA - not updated since 30 Apr 2010
[4] Validation of vCJD diagnostic criteria

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[1] UK: National CJD Surveillance Unit -- monthly statistics as of
Mon 5 Jul 2010 -- one new vCJD case
Date: Mon 5 Jul 2010
Source: UK National CJD Surveillance Unit, monthly statistics [edited]



The number of deaths due to definite or probable vCJD cases has
increased to 169. A total of 4 definite/probable patients are still
alive so that the total number of definite or probable vCJD cases is
now 173. So far, two 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
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During the 1st 6 months or 2010, there have been 67 referrals, 24
fatal cases of sporadic CJD, 2 fatal cases of vCJD, one case of
iatrogenic CJD, and none of familial CJD or GSS.

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[2] France: Institut de Veille Sanitaire -- monthly statistics as of
1 Jul 2010 -- no new vCJD cases
Date: Thu 1 Jul 2010
Source: IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees
[in French, trans. & summ. Mod.CP, edited]



During the 1st 6 months of 2010, there were 842 referrals, 35
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 has 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.

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[3] USA - not updated since 30 Apr 2010
Date: Fri 30 Apr 2010
Source: US National Prion Disease Pathology Surveillance Center [edited]



During the 4 month period 1 Jan 2010 to 30 Apr 2010, there were 116
referrals, of which 62 were classified as Prion disease, comprising
31 cases of sporadic CJD, 7 of familial CJD, and no cases of
iatrogenic CJD or vCJD. Since 1996 when records began there have been
3617 referrals, of which 2117 were classified as prion disease,
comprising 1781 cases of sporadic CJD, 301 of familial CJD, 4 of
iatrogenic CJD, and 3 of vCJD. The 3 cases of VCJD were considered to
have been contracted in the United Kingdom or Saudi Arabia. The prion
disease category includes 28 (24 in 2010) cases with type
determination pending in which vCJD has been excluded.

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[4] Validation of vCJD diagnostic criteria
Date: Wed 27 Jan 2010
Source: Ann Neurol. 2010 Jun;67(6):761-70. [edited]



Ref: Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease.
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[Author: Heath CA, Cooper SA, Murray K, Lowman A, Henry C, MacLeod
MA, Stewart GE, Zeidler M, MacKenzie JM, Ironside JW, Summers DM,
Knight RS, Will RG.
At: Department of Clinical Neurosciences, Western General Hospital,
Edinburgh, UK. craigheath@nhs.net]

Abstract
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Objective: Variant Creutzfeldt-Jakob disease (vCJD), a novel form of
human prion disease, was recognized in 1996. The disease affected a
younger cohort than sporadic CJD, and the early clinical course was
dominated by psychiatric and sensory symptoms. In an attempt to aid
diagnosis and establish standardization between surveillance
networks, diagnostic criteria were established. These were devised
from the features of a small number of cases and modified in 2000 as
the clinical phenotype was established. Since then, only minor
changes have been introduced; revalidation of the criteria in the
current format is overdue.

Methods: Included in this study are autopsy/cerebral biopsy-proven
cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU)
between 1995 and 2004 and suspect cases in which an alternative
diagnosis was identified following autopsy/cerebral biopsy.

Results: Over the 10-year period, 106 definite cases of vCJD and 45
pathologically confirmed "noncases" were identified from the archives
of the NCJDSU. The median age at onset of the cases was significantly
younger than that of the noncases (27 years [range, 12-74 years] vs
43 years [range, 10-64 years]), and the median duration of illness
was significantly shorter (14 months [range, 6-39 months] vs 22
months [range, 2-139 months]). The most commonly identified core
clinical feature in cases was dementia; persistent painful sensory
symptoms were the least frequent. Eighty-eight of 106 (83 percent)
vCJD cases were retrospectively classified as probable in life, 6
cases were classified as possible. Most cases were classified as
probable on the basis of core clinical features and brain magnetic
resonance imaging. To date, the diagnostic criteria remain 100
percent specific, with no autopsy/ cerebral biopsy-proven noncases
classified as probable in life.

Interpretation: This study confirms that the diagnostic criteria for
vCJD are sensitive and specific and provide a useful standard
framework for case classification in a surveillance setting.

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[These findings confirm the accuracy of the diagnosis of variant
Creutzfeldt-Jakob disease as a distinct disease entity. The epidemic
in the UK is now characterised by lengthening tail. - Mod.CP]

[see also:
Prion disease update 2010 (05) 2010 20100507.1488
Prion disease update 2010 (04) 2010 20100405.1091
Prion disease update 2010 (03) 20100304.0709
Prion disease update 2010 (02) 2010 20100205.0386
Prion disease update 2010 20100107.0076
2009
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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
2008
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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
2007
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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
2006
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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
2005
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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]
...................cp/ejp/lm

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