From: TSS ()
Subject: UNUSUAL CASES OF SPONGIFORM ENCEPHALOPATHY IN CATTLE
Date: May 3, 2007 at 8:50 am PST
UNUSUAL CASES OF SPONGIFORM ENCEPHALOPATHY IN CATTLE
1. To consider the implications of emerging scientific data suggesting
the existence of other strains of spongiform encephalopathy in
2. In the past few years, surveillance of cattle for Bovine Spongiform
Encephalopathy (BSE) in a number of countries has identified
animals with phenotypes of abnormal prion protein (PrPSc) that
differ from that usually associated with BSE. Most of these
animals were over the age of eight years old. Neuropathological
investigations of some of these animals revealed differences in the
deposition and distribution of PrPSc compared with those expected
for BSE. Inoculations of mice have demonstrated the disease to
be transmissible. These findings suggest that cattle may be
susceptible to another strain, or strains, of naturally occurring
transmissible spongiform encephalopathy (TSE).
3. At SEAC 81, SEAC considered the findings of a research paper1
suggesting that a second spongiform encephalopathy exists in
cattle with a molecular signature similar to that of a subtype of
sporadic CJD (sCJD). SEAC agreed that without information on
the transmissibility, it was premature at that time to conclude this
was a new strain of BSE.
4. At SEAC 93, SEAC noted reports that western blot (WB) profiles of
two cases of BSE in USA cattle were similar to a small number of
unusual cases of BSE found in France. A study2 of the French
1 Casalone et al. Identification of a second bovine amyloidotic spongiform encephalopathy:
molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A.
2004 Mar 2;101(9):3065-70.
2 Baron et al. Transmission of new bovine prion to mice. Emerg Infect Dis. 2006
© SEAC 2007
cases had shown the condition to be transmissible to mice by
intracerebral inoculation, with the neuropathological phenotype
maintained on transmission. SEAC noted that claims had been
made about the existence of further cases in cattle, that are distinct
from classical BSE in other countries. It was noted that no study
had examined the tissue distribution of PrPSc or infectivity in such
5. At SEAC 96 the committee agreed to consider available
information on unusual cases of BSE (see SEAC paper 97/1), as
they will be described for the purposes of this paper, at SEAC 97.
6. This paper summarises the published information of unusual BSE
cases. At SEAC 97, the committee will also receive presentations
from a number of invited experts. A list of questions based on the
committee’s discussion at SEAC 96 are provided at the end of the
paper to focus discussions. A SEAC position statement will be
19. Brown et al11 discuss data on unusual BSE cases and the
possibility that their origin may be sporadic and possibly may
explain the occurrence of sCJD. The authors conclude that such a
link is not currently supported by epidemiological evidence. Details
of unusual BSE cases from Canada, Denmark, Belgium, The
Netherlands, and Sweden that have not been published in the
peer-review literature are included in this article.
WHAT DID DID 16 YEAR OLD VICKEY RIMMER HAVE ??? SPORADIC CJD
WHAT DID ALL THOSE FARMERS AND THERE WIVES HAVE ??? SPORADIC CJD
POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW
LIKELY TO ATRACT MEDIA ATTENTION
CONFIRMED CJD IN FARMER WITH BSE COW
line to take, sporadic CJD
SECOND CASE CJD IN DAIRY FARMER
CJD IN AN INDIVIDUAL OCCUPATIONALLY EXPOSED TO BSE
ii. on page 2 the sentence ''He had drunk pooled milk from the herd which included that from the affected animal'' will mislead the uninformed. It needs to be made clear that milk from a cow which is suspected to be affected with BSE cannot be drunk or added to the bulk milk produced by the rest of the herd.
iii. in the final paragraph I suggest that the phrase ''and a causal link with BSE is at most conjectural'' BE DELETED: the first paragraph of the sentence would then stand as a clear statement that the CJD case was likely to have been a CHANCE PHENOMENON.
''DH is aware of a second case of CJD in a dairy farmer who has had BSE in his herd. We cannot comment on the details of the case, but we know of nothing to suggest this is anthing other than a sporadic case of CJD. .........
The numbers concerned are very small, and it is not possible to draw any conclusions from such small numbers. This issue is being considered by the Government's expert advisers....
THE FARMER IS THOUGHT TO HAVE HAD AT LEAST TWO CASES OF BSE IN HIS HERD, which were diagnosed in 1992. The farmer is reported to have asssisted in calving and to have drunk milk from his herd. This does not suggest that this is anything other than a sporadic case of CJD. ...
CONFIRMED CASE OF CJD IN DAIRY FARMER
3. Neither Dr Will nor the CJD surveillance unit intend to disclose the existence of this case or make any comment at present unless it attracts media attention.
HUMAN CASE DETAILS CONFIDENTIAL
6. CJD IN FARMERS
The second annual report on CJD surveillance in the UK, which is about to be published, gives occupational history details of 29 definite and probable CJD cases recorded in people who had a history of employment at any time in particular occupational groups of potential significance for the occurrence of the disease. The 29 cases were amongst 95 diagnosed over a 3 year period: the other 66 cases did not fall into such occupational groups.
These relevant details are:-
ANIMAL LABORATORY 1
PHARMACEUTICAL LABORATORY 0
RESEARCH LABORATORY 0
FARMERS/VETERINARY SURGEONS 7
INVOLVING DIRECT CONTACT WITH ANIMAL
OR CARCASES 5
OCCUPATION INVOLVING ANIMAL PRODUCTS 9
snip... full text ;
POLICY IN CONFIDENCE
1. The article in the Daily Mail of 12 August again raises the question of a CAUSATIVE LINK BETWEEN BSE AND CJD. This follows the death of a second farmer from CJD...
I am, however, concerned about how DH and MAFF would respont to public concern generated if there are further CJD cases among farmers.
4. Unwelcome, though it maybe to the Tyrrell Committee, I think they must be asked at their next meeting to give further thought to what they might advise the Department and MAFF if ANOTHER FARMER (or TWO) DEVELOPS CJD. OR, if a butcher or abattoir worker develops the disease.
5. Although the Committee were given plenty of advance warning about the second farmer, they may NOT BE SO FORTUNATE NEXT TIME ROUND. Some Contingency planning on the Committee's response to a further case of CJD in a farmer seems essential. At the same time the Committee should consider if there is SPECIAL RISK TO FARMERS, FOR EXAMPLE THEIR HISTORICAL HABIT OF CHEWING CATTLE NUTS, that might be implicated. .....(oh my GOD...tss)
Ministers will note from this that experts are of the view, that there is unlikely to be a direct link between the cases of BSE, and the occurance of CJD in the farmer.
(NOTE CJD increasing over 3 years. ...TSS)
'AGE AT ONSET' is therefore likely to be a reflection of particulary aetiological factors, about which, for sporadic CJD at least, much is yet unknown. IT has therefore been suggested that examination of the f/d i/p of other groups with TSE's, and comparison with that of CJD subsets might help to elucidate aetiological mechanisms for sporadic CJD in particular; i.e. ALMOST A REVERSAL OF THE ORIGINAL UNDERTAKING.
OCCUPATIONAL EXPOSURE TO BSE AND CJD
2. The Tyrrell Committee met on 7 October and the significance of the two cases of CJD reported in dairy farmers who had BSE-affected animals on their farms was discussed at some length, AS WERE THE IMPLICATIONS OF A THIRD (OR FORTH) similar case.
3. The Committee were unable to identify any possible risk factors over and above those that they had already considered, both in general and with particular of TASTING THE FEED does continue but there was no consensus about the value of advising farmers to discontinue this practice. Feed currently in use does not pose a risk because of the ruminant-ruminant feed ban.
STRAIN CHARACTERISATION OF THE CREUTZFELDT-JAKOB DISEASE AGENT BY TRANSMISSION TO MICE
In view of the CONCERN that exposue to BSE OR SCRAPIE MAY POSE A RISK TO HUMANS, it is proposed investigate the relationship between sporadic creutzfeldt-jakob disease.....
3. While Committee may have no leads to pursue on why farmers might be at increased risk, I hope they understand the urgency with which they will need to respond if or when a THIRD FARMER DEVELOPS CJD.
CJD FARMERS WIFE 1989
cover-up of 4th farm worker ???
CONFIRMATION OF CJD IN FOURTH FARMER
now story changes from;
SEAC concluded that, if the fourth case were confirmed, it would be
worrying, especially as all four farmers with CJD would have had BSE
cases on their farms.
This is not unexpected...
was another farmer expected?
4th farmer, and 1st teenager
Over a 5 year period, which is the time period on which the advice
from Professor Smith and Dr. Gore was based, and assuming a
population of 120,000 dairy farm workers, and an annual incidence
of 1 per million cases of CJD in the general population, a
DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN
an individual in the general population to develop CJD. Using the
actual current annual incidence of CJD in the UK of 0.7 per
million, this figure becomes 7.5 TIMES.
3. You will recall that the advice provided by Professor Smith in
1993 and by Dr. Gore this month used the sub-population of dairy
farm workers who had had a case of BSE on their farms -
63,000, which is approximately half the number of dairy farm
workers - as a denominator. If the above sums are repeated using
this denominator population, taking an annual incidence in the general
population of 1 per million the observed rate in this sub-population
is 10 TIMES, and taking an annual incidence of 0.7 per million,
IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than
that in the general population...
CJD YOUNG PEOPLE
in the USA, a 16 year old in 1978;
ALSO IN USA;
(20 year old died from sCJD in USA in 1980 and a 16 year
old in 1981. see second url below)
in France, a 19 year old in 1982;
in Canada, a 14 year old of UK origin in 1988;
in Poland, cases in people aged 19, 23, and 27 were identified in
a retrospective study (published 1991), having been originally
misdiagnosed with a viral encephalitis;
Creutzfeldt's first patient in 1923 was aged 23.
20 year old died from sCJD in USA in 1980 and a 16 year
old in 1981. A 19 year old died from sCJD in
France in 1985. There is no evidence of an iatrogenic
cause for those cases....
USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN
18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.
64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.
Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.
3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.
***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than
classical BSE in humans.***
6:30 Close of Day One
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...
There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
BRITISH MEDICAL JOURNAL
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al  have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518