PRO/AH/EDR> Prion disease update 2012

isfidVariant Creutzfeldt-Jakob disease
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In this update:

[1] UK CJD surveillance as of Mon 11 Jun 2012
[2] 26th case of vCJD (France)
[3] CJD cluster (Spain)
[4] RT-QuIC diagnostic test trial

[1] UK CJD surveillance as of Mon 11 Jun 2012
Date: Mon 11 Jun 2012
Source: UK National CJD Surveillance Unit, monthly statistics
variant Creutzfeldt-Jakob disease (vCJD) statistics
The number of deaths due to definite or probable vCJD as of Mon 11 Jun
2012 remains 176. No definite/probable patient remains alive, so the
total number of definite or probable vCJD cases (dead and alive)
remains 176.

The overall picture remains 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, 3 in
2010, 5 in 2011, and none so far in 2012.

Totals for all types of CJD cases in the UK in 2012
During 2012 so far [as of 11 Jun 2012], there have been 46 referrals,
28 fatal cases of sporadic CJD, none of GSS
[Gerstmann-Straussler-Scheinker disease], 2 cases of familial CJD, no
cases of vCJD, and no cases of iatrogenic CJD.

Since records began in 1990, there have been 2933 referrals, 1335
fatal cases of sporadic CJD, 176 cases of vCJD, 96 cases of familial
CJD, 68 cases of iatrogenic CJD, and 45 cases of GSS.

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[A ProMED-mail HealthMap for the UK can be found at

[2] 26th case of vCJD (France)
Date: Mon 11 Jun 2012
Soure: TFI News [in French, trans. & edited]
A new case of variant Creutzfeldt-Jakob disease (vCJD), the human form
of bovine spongiform encephalopathy (BSE), has been identified in
France, bringing the total number of cases to 26 since 1996, and the
1st new case for 3 years.

vCJD, the human form of bovine spongiform encephalopathy (BSE), is
believed to be related to the ingestion of contaminated beef products,
such as brain. Of these 26 probable or confirmed cases of vCJD in
France (12 men and 14 women), all except the most recent case have
died. The 25 deaths occurred in 1996 (1 case), 2000 (1 case), 2001 (1
case), 2002 (3 cases), 2004 (2 cases) 2005 (6 cases) in 2006 (6
cases), in 2007 (3 cases) and 2009 (2 cases). The median age at death
or diagnosis was 37 years (ranging from 19 to 58 years). Of these 26
victims, 8 patients lived in the Ile-de-France and 18 in the
provinces. One victim had regularly resided in the UK during a 10 year
period since 1987. In Britain, where the epidemic of the so-called mad
cow disease 1st appeared, there has been a total of 176 fatal cases
according to the most recent figures as of 11 Jun 2012 (see above).

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[For those interested, the Table of the Global Total of the Number and
Distribution of Human vCJD Cases has been updated to include this new
case: <>. – Mod.CP]

[A ProMED-mail HealthMap for France can be found at

[3] CJD cluster (Spain)
Date: Mon 21 May 2012
From: Terry Singeltary <>
Creutzfeldt-Jakob disease cluster in the health area of Meixoeiro
(M J Moreno, et al. Acta Neurologica Scandinavica, early view (online

Galicia is the Spanish region in which most bovine spongiform
encephalopathy cases have been registered. Meixoeiro Hospital is
included in the Galician Health Service (SERGAS). The aim of the study
was to analyze the clinical and epidemiological characteristics of
Creutzfeldt-Jakob disease (CJD) in the health area of Meixoeiro
Hospital and to identify possible specific risk factors to the general

All incident cases of CJD were identified in the health area of
Meixoeiro Hospital (187 877 inhabitants) over a 14 year period,
1997-2010, and classified according to WHO diagnostic criteria. We
obtained clinical details and epidemiological information on all
cases. Crude and age-specific incidence rates were calculated. A
review of surgical or invasive medical procedures was undertaken.

We diagnosed 12 patients with CJD, 10 sporadic CJD (sCJD), and 2
genetic CJD (gCJD). No iatrogenic or variant CJD was detected.
According to Poisson distribution, 3.9 CJD cases would be expected for
our area over the 14 years researched. The average yearly mortality
rate from CJD was 4.6 cases per million (3.8 from sCJD and 0.8 from
gCJD). Eight patients (67 per cent) underwent at least one surgical or
invasive medical procedure. 16 of 27 (59 per cent) of these procedures
were undertaken in Meixoeiro Hospital.

The incidence of CJD in the health area of Meixoeiro Hospital is 3
times higher than expected. The hypothesis that at least some cases of
sCJD are apparently because of covert transmission or zoonosis events
should not be formally refuted and might explain the high rate found.

Terry S Singeltary Sr

[These figures may be no more than a statistical anomaly, but they
deserve attention and further investigation and similar analysis
extension to other locations. – Mod.CP]

[A ProMED-mail HealthMap for Spain can be found at

[4] RT-QuIC diagnostic test trial
Date: 15 Mar 2012
From: Terry Singeltary <>
RT-QuIC analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob
(Lynne I McGuire, et al. Annals of Neurology online, DOI:


Current cerebrospinal fluid (CSF) tests for sporadic Creutzfeldt-Jakob
disease (sCJD) are based on the detection of surrogate markers of
neuronal damage such as CSF 14-3-3 which are not specific for sCJD. A
number of prion protein conversion assays have been developed,
including real-time quaking induced conversion (RT-QuIC). The
objective of this study is to investigate whether CSF RT-QuIC analysis
could be used as a diagnostic test in sCJD.

An exploratory study was undertaken which analysed 108 CSF samples
from patients with neuropathologically confirmed sCJD or from control
patients. Of the 108 CSF samples, 55 were from sCJD patients (30
female, 25 male, aged 31-84 years; 62.1 plus-minus 13.5 years) and 53
were from control patients (26 female, 27 male, aged 43-84 years; 67.8
plus-minus 10.4 years). A confirmatory group of 118 patients were
subsequently examined which consisted of 67 cases of
neuropathologically confirmed sCJD (33 female, 34 male, aged 39-82
years; 67.5 plus-minus 9.0 years) and 51 control cases (26 female, 25
male, aged 36-87 years; 63.5 plus-minus 11.6 years).

The exploratory study showed that RT-QuIC analysis had a sensitivity
of 91 per cent and a specificity of 98 per cent for the diagnosis of
sCJD. These results were confirmed in the confirmatory study which
showed that CSF RT-QuIC analysis had a sensitivity and specificity of
87 per cent and 100 per cent respectively.

This study shows that CSF RT-QuIC analysis has the potential to be a
more specific diagnostic test for sCJD than current CSF tests.

Terry S Singeltary Sr

Sobre snmv