AR-News: CJD

[Ronda Roaring]

Date: Sun, 14 Mar 2004 17:25:17 -0500 (EST)
From: ProMED-mail 

Subject: PRO/AH/EDR> CJD (new var.) - UK: update 2004 (03)

CJD (NEW VAR.) - UK: UPDATE 2004 (03)
*************************************
A ProMED-mail post

ProMED-mail is a program of the
International Society for Infectious Diseases



[1]
Date: Sun 14 Mar 2004
From: ProMED-mail 

Source: UK Department of Health, Monthly CJD Statistics, 2004/0084, Mon 1
Mar 2004 [edited]
es/fs/en?CONTENT_ID=4074900&chk=553JlD>

[Posting of these statistics has been delayed by extensive reorganisation
of the UK Department of Health web-site. - Mod.CP]


Monthly Creutzfeldt-Jakob Disease Statistics; As of Mon 1 Mar 2004
- --------------------------------------------------
The Department of Health issued on Mon 1 Mar 2004 the latest information
about the numbers of known cases of Creutzfeldt-Jakob disease. This
includes cases of variant Creutzfeldt-Jakob disease [abbreviated in
ProMED-mail as vCJD or CJD (new var.)] -- the form of the disease thought
to be linked to bovine spongiform encephalopathy (BSE). The position is as
follows:

Definite and probable CJD cases in the UK:

Referrals of suspect CJD/ Deaths of definite and probable CJD

Year/ Referrals/ Sporadic/ Iatrogenic/ Familial/ GSS/ vCJD/ Total deaths

1990/ 53/ 28/ 5/ 0/ 0/ -/ 33
1991/ 75/ 32/ 1/ 3/ 0/ -/ 36
1992/ 96/ 45/ 2/ 5/ 1/ -/ 53
1993/ 78/ 37/ 4/ 3/ 2/ -/ 46
1994/ 118/ 53/ 1/ 4/ 3/ -/ 61
1995/ 87/ 35/ 4/ 2/ 3/ 3/ 47
1996/ 134/ 40/ 4/ 2/ 4/ 10/ 60
1997/ 161/ 60/ 6/ 4/ 1/ 10/ 81
1998/ 154/ 63/ 3/ 4/ 1/ 18/ 89
1999/ 170/ 62/ 6/ 2/ 0/ 15/ 85
2000/ 178/ 50/ 1/ 2/ 1/ 28/ 82
2001/ 179/ 57/ 3/ 3/ 2/ 20/ 85
2002/ 163/ 72/ 0/ 4/ 1/ 17/ 94
2003/ 153/ 63/ 5/ 2/ 2/ 18/ 90
2004*/ 16/ 5/ 0/ 0/ 1/ 0/ 0/ 6

Total/1815/702/45/40/22/139/948

(*Provisional as of Mon 1 Mar 2004)

Summary of vCJD cases

Deaths:
Deaths from definite vCJD (confirmed): 103
Deaths from probable vCJD (without neuropathological confirmation): 35
Deaths from probable vCJD (neuropathological confirmation pending): 1
Number of deaths from definite or probable vCJD (as above): 139

Alive:
Number of probable vCJD cases still alive: 7

Total number of definite or probable vCJD (dead and alive): 146

The precise definitions of the terms: Referrals, Deaths, Definite cases,
Probable vCJD cases, Sporadic, Probable sporadic, Iatrogenic, Familial,
GSS, variant CJD, and the case definitions can be found by accessing the
Department of Health web-site or by reference to a preceding ProMED-mail
post in this thread [CJD (new var.) - UK: update Mar 2002 20020305.3693]
The next monthly statistics will be published on Mon 5 Apr 2004.

- -- 
ProMED-mail



[Since the previous monthly statistics released by the Department of Health
on Mon 2 Feb 2004, the total number of definite or probable vCJD cases
(dead and alive) and the number of deaths remain unchanged, and the number
of deaths attributed to sporadic CJD has increased by 10.

In view of the experimental evidence from transmission experiments in mice
reported by Asante et al. (MRC Prion Unit, University College, London) in
the EMBO Journal, Vol. 21, No. 23, 6358-6368, 2002
, that:
"Some patients with a phenotype consistent with sporadic CJD may have a
disease arising from BSE exposure" (see: CJD, possible association with BSE
20021129.5921), the figures for sporadic CJD cases now have added
significance in relation to the course of the vCJD outbreak and will
continued to be presented here.

vCJD has been recognized to date only in individuals homozygous for
methionine at PRNP codon 129, and Asante et al. reported that transgenic
mice expressing human PrP methionine 129, inoculated with either bovine
spongiform encephalopathy (BSE) or variant CJD prions, may develop the
neuropathological and molecular phenotype of vCJD, consistent with these
diseases being caused by the same prion strain. However, BSE transmission
to these transgenic mice, in addition to producing a vCJD-like phenotype,
also resulted in a distinct molecular phenotype that was indistinguishable
from that of sporadic CJD with PrP\Sc type 2. These data suggest that more
than one BSE-derived prion strain might infect humans; it is therefore
possible that some patients with a phenotype consistent with sporadic CJD
may have a disease arising from BSE exposure. - Mod.CP]

******
[2]
Date: Mon 5 Jan 2004
From: ProMED-mail 

Source: Eurosurveillance Weekly, Volume 8 / Issue 6, Thu 5 Jan 2004 [edited]



Incidence of Variant CJD in the UK Shows Evidence of Having Peaked or
Reached a Plateau
- --------------------------------------------------
A total of 18 deaths from variant Creutzfeldt-Jakob disease (vCJD) were
reported in 2003 in the United Kingdom. The greatest number of deaths
reported in any one year was 28 in the year 2000. This fell to 20 in 2001
and 17 in 2002. At the end of 2003, the overall total number of deaths from
vCJD was 139 with a further 6 probable cases alive.

The quarterly analysis of vCJD incidence published on the CJD surveillance
unit website () shows that the incidence
of deaths since 1994 is consistent with an underlying trend that has either
reached a peak or a plateau. As was first noted in 2003 (refs. 1,2), the
data are not consistent with an underlying trend that is increasing
exponentially.

Quadratic and plateau models for vCJD deaths incidence trend are presented
as a figure in the original article. These 2 models fit the data equally
well. The quadratic model estimates that a peak in deaths occurred at the
end of 2000 and that the current incidence is 3.5 deaths per quarter. An
extrapolation of this model gives a prediction of 11 deaths for 2004 (95
percent prediction interval of 4 to 19). The plateau model estimates the
current incidence to be 4.9 deaths per quarter and predicts 19 deaths in
2004 (95 percent prediction interval 10 to 29).

The interpretation of these results requires caution, as highlighted by the
difference in the predictions from 2 models that fit the current data
equally well. There may be a long tail or more than one peak to the
epidemic. There is also the theoretical possibility of human-to-human
spread through procedures such as blood transfusions: one such potential
case was recently reported in the UK (ref. 3). Continued surveillance and
research into vCJD is therefore important to investigate these possibilities.

References:

(1) Andrews N, Molesworth A. Decline in the incidence of variant
Creutzfeldt-Jakob disease in the UK. Eurosurveillance Weekly 2003;
7(10):06/03/2003. ()

(2) Andrews N, Farrington C, Ward H, Cousens S, Smith P, Molesworth A, et
al. Deaths from variant Creutzfeldt-Jakob disease in the UK. Lancet 2003;
361: 751-2.

(3) House of Commons Statement by the Secretary of State for Health.
Development in vCJD. Department of Health (England), 17 Dec 2003.
()

[Byline: Nick Andrews , Health Protection Agency,
Communicable Disease Surveillance Centre, London, England]

- --
ProMED-mail



******
[3]
Date: Sat 7 Feb 2004
From: ProMED-mail 

Source: Lancet, 363, 422-28, 2004 [edited]
earch.28622.1>


Possible Risk of Variant CJD Linked to Endoscopic Procedures
- ---------------------------------------------------
[The following paper published recently in the Lancet is relevant to the
possible risk of transmission of variant Creutzfeldt-Jakob disease (vCJD)
by blood and tissue donations and surgical procedures. - Mod.CP]

Title: Tissue distribution of bovine spongiform encephalopathy agent in
primates after intravenous or oral infection.

Authors: C Herzog, N Sales, N Etchegaray, A Charbonnier, S Freire, D
Dormont, J-P Deslys and C I Lasmezas. (Commissariat a l'Energie Atomique,
Departement de Recherche Medicale, BP6, 92265 Fontenay-aux-Roses, Cedex,
France)

Abstract: The disease-associated form of prion protein (PrP/res) has been
noted in lymphoreticular tissues in patients with variant Creutzfeldt-Jakob
disease (vCJD). Thus, the disease could be transmitted iatrogenically by
surgery or use of blood products. We aimed to assess transmissibility of
the bovine spongiform encephalopathy (BSE) agent to primates by the
intravenous route and study its tissue distribution compared with infection
by the oral route.

Cynomolgus macaques were infected either intravenously or orally with brain
homogenates from first-passage animals with BSE. They were clinically
monitored for occurrence of neurological signs and killed humanely at the
terminal stage of the disease. Brain, lymphoreticular tissues, digestive
tract, and peripheral nerves were obtained and analysed by sandwich ELISA
and immunohistochemistry for quantitative and qualitative assessment of
their PrP/res content.

Incubation periods after intravenous transmission of BSE were much shorter
than after oral infection. We noted that PrP/res was present in
lymphoreticular tissues such as spleen and tonsils and in the entire gut
from the duodenum to the rectum. In the gut, PrP/res was present in Peyer's
patches and in the enteric nervous system and nerve fibres of intestinal
mucosa. Furthermore, PrP/res was found in locomotor peripheral nerves and
the autonomic nervous system. Amount of PrP/res ranged from 0.02 percent to
more than 10 percent of that recorded in brain. Distribution of PrP/res was
similar in animals infected by the intravenous or oral route.

Our findings suggest that the possible risk of vCJD linked to endoscopic
procedures might be currently underestimated. Human iatrogenic vCJD cases
infected intravenously raise the same public-health concerns as primary
cases and need the same precautionary measures with respect to blood and
tissue donations and surgical procedures.


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