| Comment Record|
Mr. Terry Singeltary ||
2003-01-16 16:33:04 |
CJD WATCH |
| Comments for FDA General |
1. General Comments
Docket: 02D-0073 - Guidance: Validation of Procedures for Processing of Human Tissues Intended for Transplantation
please be advised;
with the new findings from Collinge et al; that BSE transmission
to the 129-methionine genotype can lead to an alternate phenotype
which is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD, i only ponder how many of the sporadic CJDs in
the USA are tied to this alternate phenotype? these new findings
are very serious, and should have a major impact on the way
sporadic CJDs are now treated as opposed to the vCJD that
was thought to be the only TSE tied to ingesting beef, in the
medical/surgical arena. these new findings should have a major
impact on the way sporadic CJD is ignored, and should now be moved
to the forefront of research as with vCJD/nvCJD. the USA has many
TSEs, the USA lacks sufficient testing for TSEs in cattle, and the
USA still refuses to rapid TSE test USA cattle in sufficient
numbers to find, when the late Dr. Richard Marsh had proven
that mink had gone down with a TSE (TME), from being fed
on 95%+ downer cattle. the GAO has also warned the industry
and the FDA that the ruminant-to-ruminant feed ban has to
significantly improved if they expect to keep BSE/TSEs out
of USA cattle. Scrapie has increased significantly, and
CWD is spreading. all this should warrant CJD/TSEs in humans
in the USA to be made reportable on a National bases, immediately...
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD
Date: Thu, 28 Nov 2002 10:23:43 -0000
From: Asante, Emmanuel A
I have been asked by Professor Collinge to respond to your request. I am a Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have attached a pdf copy of the paper for your attention. Thank you for your interest in the paper.
In respect of your first question, the simple answer is, yes. As you will find in the paper, we have managed to associate the alternate phenotype to type 2 PrPSc, the commonest sporadic CJD.
It is too early to be able to claim any further sub-classification in
respect of Heidenhain variant CJD or Vicky Rimmer's version. It will take further studies, which are on-going, to establish if there are sub-types to our initial finding which we are now reporting. The main point of the paper is that, as well as leading to the expected new variant CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc.
I hope reading the paper will enlighten you more on the subject. If I can be of any further assistance please to not hesitate to ask. Best wishes.
Dr. Emmanuel A Asante
MRC Prion Unit & Neurogenetics Dept.
Imperial College School of Medicine (St. Mary's)
Norfolk Place, LONDON W2 1PG
Tel: +44 (0)20 7594 3794
Fax: +44 (0)20 7706 3272
PLEASE SEE FULL TEXT OF THIS ARTICLE;
ROUND TABLE ON BSE -- WASHINGTON -- 27-28 JUNE 1989
The summary does tend to give a particular slant to the epidemiology of
BSE which is not totally sound. It is a possibility that the agent of
BSE may be in the cattle population in a number of countries already
apart from the USA and that clinical cases are occurring on rare
occasions. It is also important to off the possibility of the
relationship between BSE and certain low-temperature rendering systems.
For that reason a number of other countries apart from the USA and
France are at risk and, in particular, the Netherlands, Denmark,
Germany and Belgium. For these reasons it would be wise to move to an
international ban on the feeding of ruminant protein to ruminants.
Clearly the summary also needs to refer to the incidence of BSE in the
UK and not solely to Great Britain. No doubt this has been tidied up
in your comments on the summary conclusions. It is a pity that more of
the comments put forward by Dr. Kimberlin have not been included in the
summary since his views on page 13 are succinct and valuable...
Is there a Scrapie-like disease in cattle ?
re-mink rancher 'Wisconsin' dead stock feeder using >95%
downer or dead dairy and a few horses...
Part of the Proceedings of an International Roundtable on Bovine
Spongiform Encephalopathy, Bethesda, Maryland, USA, June 27-28, 1989.
The possibility of infection with BSE in the United States, as defined
by studies on the disease in Great Britain, is judged to be low on the
basis of the following: (1) meat and bonemeals imported into the United
States from Great Britain between 1980 and 1988 were used mainly in
poultry, not ruminant feed; (2) the Scrapie Eradication Program had
reduced the prevalence of scrapie in the United States compared with
that in Great Britain; and (3) little, if any, rendered animal products
are used for protein supplements in cattle feed in the United States.
However, there is some evidence that there may already be a scrapie-like
disease in cattle in the United States. This evidence comes from
epidemiologic studies on an incident of transmissible mink
encephalopathy (TME) in Stetsonville, Wis, in 1985. This mink farmer
used no commercially available animal by-product mixtures in his feed,
but instead slaughtered all animals going into the mink diet, which
included mostly (>95%) downer dairy cows, a few horses, but never
sheep. To examine the possibility that cattle may have been the source
of this incident of TME, two 6-week-old Holstein bull calves were
inoculated intracerebrally with mink brain from the affected farm. The
bulls developed neurologic disease 18 and 19 months after inoculation.
Both brains had spongiform degeneration at necropsy and both were
transmissible back to mink by either intracerebral (incubation period of
4 months) or oral (incubation period of 7 months) inoculation
Whereas TME has been thought to be caused by feeding scrapie-infected
sheep to mink, this theory has no conclusive evidence. Experimental oral
inoculation of mink with several different sources of sheep scrapie has
never been successful, and an incubation period of less than 12 months
has never (sic) produced by intracerebral inoculation. Transmissible
mink encephalopathy can develop naturally by infection with incubation
periods of less than 12 months.
There is reason to believe that scrapie has not been transmitted in the
United States from sheep to cattle by rendered protein concentrates as
it was in Great Britain. However, some circumstantial evidence exists
that cattle may be a source of some TME infections. It is recommended
that we increase our surveillance for a BSE-like disease in American
cattle by encouraging state diagnostic laboratories to formalin-fix
specimens of midbrain and brain stem from bovine brains submitted for
rabies testing. If results of these tests are negative, these fixed
tissues can then be examined for evidence of spongiform degeneration of
the gray matter.
-Comments on bovine spongiform encephalopathy
J Am Vet Med Assoc 197 (4): (1990)
Letter to the Editor, Journal of the American Veterinary Medical
Association, August 15, 1990
In my article, Bovine spongiform encephalopathy in the United States
(JAVMA, May 15, 1990, p 1677), I stated that little, if any, rendered
animal products are used for protein supplements in cattle feed in the
United States. I have since learned that this is incorrect, because of
the recent trend of using less assimilated by-pass proteins in cattle
feed. A large amount of meat-and-bone meal is being fed to American
cattle, and this change in feeding practice has greatly increased the
risk of bovine spongiform encephalopathy (BSE) developing in the United
Epidemiologic studies on BSE in Great Britain have indicated that the
disease originated in cattle by exposure to the heat-resistant
transmissible agent in compounded feed containing rendered animal
protein. The most likely source of infection was assumed to be
meat-and-bone meal prepared from scrapie-infected sheep, but it is also
possible that a heretofore unrecognized scrapie-like infection of cattle
could have been spread in the same manner.
Because of concern for the possible development of BSE in the United
States, the American rendering industry discontinued the processing of
fallen and sick sheep last December. In my opinion, this was a prudent
policy, but one that will not prevent the possible transmission of BSE
from cattle to cattle. As emphasized in my article, there is some
evidence that BSE-like infection may already exist in American cattle.
The current practice of feeding meat-and-bone meal to cattle solidifies
the most important means to perpetuate and amplify the disease cycle.
In Great Britain, BSE has produced a great economic and emotional
burden. We must take all reasonable measures to prevent BSE from
developing in the United States. Therefore, the practice of using animal
protein in cattle feed should be discontinued as soon as possible.
Waiting until the first case of BSE is diagnosed in the United States
will certainly be closing the barn door after the horse is gone. With
a disease having a 3- to 6-year incubation period, thousands of animals
would be exposed before we recognize the problem and, if that happens,
we would be in for a decade of turmoil.
R. F. Marsh, DVM, PhD
Mule deer transmissions of CWD were by intracerebral inoculation
and compared with natural cases resulted in a more rapidly
progressive clinical disease with repeated episodes of synocopy
ending in coma. One control animal became affected, it is believed
through contamination of inoculam (?saline). Further CWD
transmissions were carried out by Dick Marsh into ferret, mink
and squirrel monkey. Transmission occurred in _all_ of these
species with the shortest incubation period in the ferret.
FULL TEXT OF GOA REPORT BELOW (takes a while to load)
2. Mad Cow Disease: Improvements in the Animal Feed Ban and Other
Regulatory Areas Would Strengthen U.S. Prevention Efforts. GAO-02-183,
Subject: SCRAPIE 'USA' ANNUAL REPORT (105 newly infected flocks 2002) & CWD IN USA
Date: Tue, 10 Dec 2002 08:17:17 -0600
From: Terry S. Singeltary Sr.
Date: Mon, 9 Dec 2002 21:21:10 -0600
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: Terry S. Singeltary Sr.
Subject: SCRAPIE 'USA' ANNUAL REPORT (105 newly infected flocks 2002) & CWD IN USA
As of September 30, 2002, there were 45 scrapie infected and source
flocks (figure 3). There were 105 newly infected flocks, reported in
FY2002 (figure 4). In addition, 379 scrapie cases were confirmed and
reported by the National Veterinary Services Laboratories (NVSL) in FY
2002 (figure 5) and (figure 6). Five cases of scrapie in goats were
reported in FY 2002 (figure 7), the last of which was confirmed in
August 2002. New infected and source flocks numbers and the number of
these flocks released in FY 2002 are depicted in chart 4. One hundred
(100) flocks which is 67 percent of the scrapie infected and source
flocks present in FY 2002 were released or put on clean-up plans in FY2002.
Slaughter Surveillance is currently in Phase II which is intended to
determine the prevalence of scrapie in the US culled sheep population.
Through September 2002 samples from 3,269 sheep were submitted to NVSL
for testing. Samples from a total of 6,795 sheep have been submitted
since the beginning of Phase II on April 1, 2002. Surveillance regions
are depicted in (figure 8).
During FY 2002 11,751 animals have been tested for scrapie which
includes: 2,711 regular necropsy cases, 1,343 third eyelid biopsies for
the test validation project, 546 third eyelid biopsies for the
regulatory program, and approximately 7,151 animals for Phase I & II of
SOSS (chart 5). Laboratory testing has been taking 10 - 11 days on
average with a range of 3 - 34 days.
Ear Tag Orders
During FY 2002 9.9 million plastic and 6.0 million metal tags were
distributed by APHIS (chart 6).
NEW SCRAPIE INFECTED AND SOURCE FLOCKS
DISTRIBUTION OF CHRONIC WASTING DISEASE THROUGHOUT THE STATES (as of
CWD USA surveillance
CJD Watch message board
Moms death from hvCJD
'MOMS AUTOPSY REPORT'