Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
-------------------- BSE-L@LISTS.AEGEE.ORG --------------------
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle? Martin Jeffrey , Belinda Baquero-Perez , Stuart Martin , Linda Terry and Lorenzo Gonzalez
BMC Veterinary Research 2008, 4:38doi:10.1186/1746-6148-4-38
Published: 30 September 2008
Abstract (provisional) Background The epidemic form of Bovine Spongiform Encephalopathy (BSE) is generally considered to have been caused by a single prion strain but at least two strain variants of cattle prion disorders have recently been recognized. Idiopathic brainstem neuronal chromatolysis and hippocampal sclerosis (IBNC) is a rare neurological disease of adult cattle which was recognised in a sub-set of cattle submitted under the BSE Orders in which lesions of BSE were absent. Between the years of 1988 and 1991 IBNC occurred in Scotland with an incidence of ~7 cases per 100,000 beef suckler cows over the age of 6 years.
Results When brains of 15 IBNC cases were each tested by immunohistochemistry, all showed the abnormal presence of prion protein (PrP). Abnormal PrP labeling was also present in the retina of a single case available for examination. The pattern of PrP labeling in brain is distinct from that seen in other ruminant prion diseases. Brains of IBNC cattle do not reveal abnormal PrP isoforms when tested by the commercial BioRad or Idexx test kits and do not reveal PrPres when tested using stringent proteinase digestion methods. However, some biochemical evidence for weakly protease resistant isoforms of PrP can be detected when tissues are examined using mild proteinase digestion techniques.
Conclusions The study shows that a distinctive neurological disorder of cattle which has some clinical similarities to BSE, is associated with abnormal PrP accumulation in brain but the pathology and biochemistry of IBNC are distinct from BSE. The study is important either because it raises the possibility of a significant increase in the scope of prion disease or because it demonstrates that widespread and consistent PrP alterations may not be confined to prion diseases. Transmission experiments are needed to establish whether IBNC is a condition in which prion protein is abnormally regulated or is yet a further example of an infectious cattle prion disease.
http://www.biomedcentral.com/1746-6148/4/38/abstract
snip...
Discussion
This study shows that the novel condition of cattle previously identified as IBNC and recognized from within the BSE suspect submissions, abnormally expresses or accumulates PrP in brain and retina. However, this abnormal PrP is not composed of isoforms that are strongly resistant to protease digestion suggesting that it is not present in the form of large aggregates.
Immunohistochemical demonstration of PrP labelling and increased levels of PrP mRNA have previously been described in adult humans affected with acute vascular disorders, in infants with perinatal hypoxia and experimental infarction of rodents [9]. These findings are considered to represent upregulation of PrP expression which encompasses part of the oxidative stress response of neurons. We have also observed increased PrP in the cytoplasm of neurons undergoing ischaemic degeneration in a variety of sheep encephalopathies. Though ischaemic neuronal degeneration is not a feature of IBNC, nevertheless, the presence of PrP within the cytoplasm of some chromatolytic and degenerate neurons of IBNC affected cattle is consistent with the idea that stressed neurons may respond by increasing PrP expression.
Though present in only two cows, the pattern of PrP accumulation within the granule cell layer of the cerebellum is morphologically similar to that reported by several authors for Nor 98 types of the transmissible spongiform encephalopathy or prion disease of sheep [3, 10]. The PrP accumulation within the plexiform layers of the eye is similar to that of both natural scrapie [11] and of Nor 98 (MJ personal observations). However the majority type of PrP labelling that occurred in all IBNC cases was found within the neuropil, mainly in the rostral neuraxis and cerebrum, the nature of which is previously unreported in cattle or in any other prion disorder. This novel pattern of labelling appears to correspond to a rarefaction or fine microvacuolation of neuropil as seen on standard HE stained sections.
Pathological, biochemical and bioassay data all suggest that the epidemic form of cattle BSE is a single strain. However, recent large scale EU wide surveillance for BSE has led to the unexpected discovery of rare and hitherto unknown prion diseases of cattle. Small numbers of atypical forms of cattle prion diseases have now been recognized from several European countries, in the USA and Japan and can be distinguished by histological, molecular and transmission characteristics [12-14]. Bovine Amyloidotic Spongiform Encephalopathy (BASE) was the first of these novel cattle prion disorders to be recognized and was characterized by the presence of numerous small amyloid deposits of abnormal PrP. It was initially discovered in three aged Italian cattle [1] and has subsequently been transmitted to transgenic mice [13, 14]. A further variant of a cattle prion disease affecting cows between 8 and 15 years was initially recognized in France [2] and has also been transmitted to mice. BSE and these novel cattle prion diseases can be distinguished using biochemical and molecular methods and are now classified as C, H and L type isolates [12]. H type and L type (BASE) isolates are defined according to the higher and lower positions of the unglycosylated PrPres bands in Western blots, respectively, when compared to the position of the corresponding band in classical BSE (C type) isolates [12]. L type cases formerly classified as BASE, have a distinctive glycopattern in which monoglycoslyated PrPres predominates compared to BSE [12,14]. While IBNC cases
are on average older than BSE cases they occupy a similar age class of cattle to that of H and L type cattle prion diseases, but IBNC can be readily distinguished from H, L and C type cattle prion disease by morphologic pathology and by the absence of PrPres under stringent conditions of protease digestion.
Not all abnormal PrPres isoforms detected from brains of animals affected with prion disease are resistant to stringent protease digestion. The PrPres of two sheep of the ARR/ARR PrP genotype affected with a classical scrapie-like disease accumulated unusually protease sensitive isoforms of PrP [15]. Similarly, the transmissible prion disease of sheep known as Nor 98 and related conditions (often referred to as atypical scrapie) also have weakly protease resistant PrPres [3,16]. Nor 98 does not appear to transmit readily to other sheep under field conditions: it also does not transmit to conventional mice although it does readily transmit disease to one strain of transgenic mouse which substantially over-expresses the VRQ allele of sheep PrP [16,17]. The transgenic PG14 mouse also has PrPres which is even more readily digested than that found in Nor 98 but this prion protein disorder has not so far been successfully transmitted [18]. The biochemical analyses of limited numbers of IBNC cases clearly shows that highly aggregated forms of protease resistant PrP are not present in brain tissue. However, when the data from the ELISA and immunoblot tests using mild protease digestion are compared with that of normal control material it is possible that smaller aggregates of PrP molecules may be present.
Conclusions
The present results indicate that there are changes in PrP expression or accumulation in the neurodegenerative cattle disorder known as IBNC. The pathology and biochemistry of IBNC are quite distinct from that of other prion diseases of cattle and other species but the pathology does include grey matter spongiform changes. The transmissibility of this disorder is undetermined. These results are interesting as they show that either the range of prion diseases and associated pathology is still wider than previously thought or that substantial abnormalities of prion protein expression may be associated with brain lesions unconnected with classical prion diseases. Further biochemical and transmission studies are needed to determine which of these possibilities is correct.
http://www.biomedcentral.com/content/pdf/1746-6148-4-38.pdf
NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
http://www.pnas.org/cgi/content/abstract/0502296102v1
Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007
http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html
http://nor-98.blogspot.com/
SCRAPIE USA
http://scrapie-usa.blogspot.com/
Sunday, June 15, 2008
A descriptive study of the prevalence of atypical and classical scrapie in sheep in 20 European countries
Research article
http://nor-98.blogspot.com/2008/06/descriptive-study-of-prevalence-of.html
another question, just how long have these atypical BSE TSEs been around in the bovine ???
let's look at another case of atypical BSE in Germany way back in 1992 ;
Subject: atypical BSE reported in 1992 and conviently slaughterd and incinerated and then swept under rug for about 12 years Date: April 26, 2007 at 1:08 pm PST 1992
NEW BRAIN DISORDER
3. WHAT ABOUT REPORTS OF NEW FORM OF BSE?
THE VETERINARY RECORD HAS PUBLISHED AN ARTICLE ON A NEW BRAIN DISORDER OF CATTLE DISCOVERED THROUGH OUR CONTROL MEASURES FOR BSE. ALTHOUGH IT PRESENTS SIMILAR CLINICAL SIGNS TO BSE THERE ARE MAJOR DIFFERENCES IN THE HISTOPATHOLOGY AND INCUBATION PERIODS BETWEEN THE TWO. MUST EMPHASISE THAT THIS IS NOT BSE.
4. IS THIS NEW BRAIN DISORDER A THREAT?
WE DO NOT EVEN KNOW WHETHER THE AGENT OF THIS DISEASE IS TRANSMISSIBLE. IN ANY CASE, CASES SO FAR IDENTIFIED HAD SHOWN SIMILAR SYMPTOMS TO THOSE OF BSE, AND THEREFORE HAVE BEEN SLAUGHTERED AND INCINERATED, SO THAT IF A TRANSMISSIBLE AGENT WERE INVOLVED IT WOULD HAVE BEEN ELIMINATED. .......
http://www.bseinquiry.gov.uk/files/yb/1992/10/26001001.pdf
2. The Collinge/Will dispute appears to rumble on. Dr. Collinge had told Dr. Tyrrell that Dr. Will's response to his criticism about sharing material had been ''quite unacceptable'' (in spite of it's apparently conciliatory tone). Apparently Professor Allen was now going to try and arrange a meeting to resolve the dispute. No action here for MAFF, although Mr. Murray may be interested.
3. Dr. Tyrrell regretted that the Committee had not seen the article on BBD. However he felt that for the time being NO specific action was called for. The most important need was to consider the possibility that the condition might be transmissible. As we have discussed, I suggested that we might circulate a paper to the members of the committee giving our appreciation of this condition (and perhaps of other non-BSE neurological conditions that had been identified in negative cases) and of any necessary follow up action. IF any Committee member felt strongly about this, or if the issue CAME TO A HEAD, we would call an interim meeting. He was happy with this approach. I would be grateful if Mr. Maslin could, in discussion with CVL and veterinary colleagues draft such a note, which will presumably very largely follow what Mr. Bradley's briefing paper has already said, taking account of DOH comments, We can then clear a final version with DOH before circulating it to Committee members.
http://www.bseinquiry.gov.uk/files/yb/1992/10/29005001.pdf
IN CONFIDENCE
This is a highly competitive field and it really will be a pity if we allow many of the key findings to be published by overseas groups while we are unable to pursue our research findings because of this disagreement, which I hope we can make every effort to solve.
http://www.bseinquiry.gov.uk/files/yb/1992/10/26002001.pdf
COLLINGE THREATENS TO GO TO MEDIA
http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf
2. The discovery might indicate the existence of a different strain of BSE from that present in the general epidemic or an unusual response by an individual host.
3. If further atypical lesion distribution cases are revealed in this herd then implications of misdiagnosis of 'negative' cases in other herds may not be insignificant.
snip...
This minute is re-issued with a wider distribution. The information contained herein should NOT be disseminated further except on the basis of ''NEED TO KNOW''.
R Bradley
http://www.bseinquiry.gov.uk/files/yb/1993/02/17001001.pdf
IN CONFIDENCE
BSE ATYPICAL LESION DISTRIBUTION
http://www.bseinquiry.gov.uk/files/yb/1993/03/14001001.pdf
ALABAMA MAD COW CASE
http://www.aphis.usda.gov/newsroom/hot_issues/bse/downloads/EPI_Final5-2-06.pdf
http://www.cdc.gov/ncidod/dvrd/bse/news/alabama_cow_031506.htm
Texas BSE Investigation Final Epidemiology Report August 2005
http://www.aphis.usda.gov/newsroom/hot_issues/bse/downloads/bse_final_epi_report8-05.pdf
State-Federal Team Responds to Texas BSE Case
JUNE 30, 2005
(please note 7+ month delay in final confirmation so the BSE MRR policy could be set in stone first. $$$...tss)
http://www.tahc.state.tx.us/news/pr/2005/2005Jun30_BSE_Positive_Results.pdf
https://www.cidrap.umn.edu/cidrap/content/other/bse/news/june3005bse.html
SEE ATTEMPTED COVER-UP BEFORE THE END AROUND BY FONG ET AL OF THE O.I.G
The U.S. Department of Agriculture confirmed June 29 that genetic testing had verified bovine spongiform encephalopathy (mad cow disease) in a 12-year-old cow that was born and raised in a Texas beef cattle herd.
Subsequent epidemiological investigations resulted in the culling and testing of 67 adult animals from the index herd. Bio-Rad tests for BSE were conducted on all 67 animals by the National Veterinary Services Laboratory (NVSL) in Ames, Iowa. All tests were negative.
On July 12, Texas officials lifted the quarantine on the source herd. At press time, USDA's Animal and Plant Health Inspection Service was tracing animals of the same age that had left the ranch.
Timeline
The BSE-positive animal was a Brahman-cross cow born and raised in a single Texas herd. The location of the ranch was not disclosed.
On Nov. 11, 2004, the 12-year-old cow was taken to a Texas auction market. Because of its condition, the cow was sent to Champion Pet Foods in Waco, Texas. The company produces several blends of dog food, primarily for the greyhound industry.
On Nov. 15, the animal arrived dead at Champion. Under procedures established by USDA's intensive surveillance program, a sample was sent to the USDA-approved Texas Veterinary Medical Diagnostic Testing Laboratory (TVMDL) at Texas A&M University.
Between June 1, 2004, and June 1, 2005, TVMDL tested nearly 34,000 samples from Texas, New Mexico, Arkansas and Louisiana. They tested the sample from Champion on Nov. 19 using a Bio-Rad ELISA rapid test for BSE. Initial results were inconclusive.
Because of the inconclusive results, a representative from USDA took the entire carcass to TVMDL where it was incinerated. USDA's Animal and Plant Health Inspection Service (APHIS) began tracing the animal and herd.
The sample was then sent to the National Veterinary Services Laboratory for further testing. Two Immunohistochemistry (IHC) tests were conducted and both were negative for BSE. At that point APHIS stopped their trace.
USDA scientists also ran an additional, experimental IHC "rapid" tissue fixation test for academic purposes. This test has not been approved internationally.
Some abnormalities were noted in the experimental test, but because the two approved tests came back negative, the results were not reported beyond the laboratory.
Monitoring by OIG
USDA's Office of Inspector General (OIG) has been monitoring implementation of the BSE expanded surveillance program and evaluating the following:
* Effectiveness of the surveillance program;
* Performance of BSE laboratories in complying with policies and procedures for conducting tests and reporting results;
* Enforcement of the ban on specified risk materials in meat products;
* Controls to prevent central nervous system tissue in advanced meat recovery products;
* Ante mortem condemnation procedures; and
* Procedures for obtaining brain tissue samples from condemned cattle.
While reviewing voluminous records, OIG auditors noticed conflicting test results on one sample-rapid inconclusive, IHC negative, experimental reactive.
Sample retested
At the recommendation of the Inspector General, the sample was retested during the week of June 5 with a second confirmatory test, the Western Blot. The results were reactive.
USDA scientists then conducted an additional IHC confirmatory test, using different antibodies from the November 2004 test. On Friday, June 10, Secretary of Agriculture Mike Johanns publicly announced the results as a "weak positive."
On June 16 an official with USDA's National Veterinary Services Laboratory hand-carried samples for further testing to the Veterinary Laboratory Agency (VLA) in Weybridge, England. Since 1991, the VLA has been a BSE reference laboratory for the World Organization for Animal Health (OIE).
Experts from the Weybridge lab confirmed the accuracy of the results of USDA's November confirmatory IHC test, concurring that the case could not have been confirmed on the basis of this sample. They also examined the November experimental IHC test and interpreted the results to be positive.
Weybridge also conducted additional tests, including IHC, OIE-prescribed Western Blot, NaTTA Western Blot and Prionics Western Blot tests.
To better understand the conflicting results, USDA also conducted Bio-Rad and IDEXX rapid screening tests, IHC and OIE-prescribed Western Blot. USDA also used DNA sequencing to determine the prion protein gene sequence of the animal.
http://findarticles.com/p/articles/mi_qa5420/is_200508/ai_n21377094
Texas even had a 'secret' test that showed that mad cow positive; experimental IHC test results, because the test was not a validated procedure, and because the two approved IHC tests came back negative, the results were not considered to be of regulatory significance and therefore were not reported beyond the laboratory. . A Western blot test conducted the week of June 5, 2005, returned positive for BSE.
http://www.usda.gov/documents/vs_bse_ihctestvar.pdf
48 hr BSE confirmation turnaround took 7+ months to confirm this case, so the BSE MRR policy could be put into place. ...TSS
-------- Original Message --------
Subject: re-USDA's surveillance plan for BSE aka mad cow disease
Date: Mon, 02 May 2005 16:59:07 -0500
From: "Terry S. Singeltary Sr."
To: paffairs@oig.hhs.gov, HHSTips@oig.hhs.gov, contactOIG@hhsc.state.tx.us
Greetings Honorable Paul Feeney, Keith Arnold, and William Busbyet al at OIG, ...............
snip...
There will be several more emails of my research to follow. I respectfully request a full inquiry into the cover-up of TSEs in the United States of America over the past 30 years. I would be happy to testify...
Thank you, I am sincerely, Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 xxx xxx xxxx
snip... see full text ;
Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ? August 20, 2008
http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html
Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate
http://organicconsumers.org/forum/index.php?showtopic=1951
http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html
snip... FULL TEXT ;
http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0003017
"the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine." <<<
NOT to forget ;
Thursday, June 05, 2008
Review on the epidemiology and dynamics of BSE epidemics
Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article
snip...
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
snip...
Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
full text 18 pages ;
http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf
please see full text ;
http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
snip...
Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
snip...
http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html
Greetings BSE-L AND CJD-L,
I'm still here damn't. well, barely, a few photo's and news articles to explain my absence, i hope no one minds. the wife said if we survived riding out IKE, she would kill me later. we went from being an Island, to literally being in the surf for a while. IKE threw everything including the kitchen cabinets, and literally the kitchen sink from the garage apartment at us. the old homestead still stands. battered, bruised, and wet, we are very blessed. much to do, so until later. ...TSS
more photo's of this area destruction from IKE. ...
http://www.boston.com/bigpicture/2008/09/the_short_but_eventful_life_of.html?s_campaign=8315
Residents dig out from storm
By Chris Paschenko The Daily News Published September 16, 2008
BACLIFF - A wall of water not seen in generations of storms smashed through dozens of homes lining Galveston Bay, and residents spent Tuesday clearing debris and digging out the mud and the muck.
Terry Singeltary refused to evacuate his Bayshore Drive home in Bacliff, saying he feared looters more than Hurricane Ike.
"My grandparents bought this place in 1935, and I wound up with it," Singeltary said. "It made it through a storm in '48, and (hurricanes) Carla and Alicia. I've never seen the water this high."
The Singeltary home survived with water damage in his game room. Singeltary never needed his backup plan: a kayak or an axe stashed in the attic. ...snip
http://galvestondailynews.com/story.lasso?ewcd=276365f800b0da1c
216. vortfix 9:25 PM GMT on September 15, 2008
Galveston warns of 'downward spiral'
Mobile morgue en route to Galveston as search continues
Five dead, but thousands of homes still to be searched
Three unrelated deaths from apparent carbon monoxide poisoning from generator use have brought the local death toll from Hurricane Ike to at least 14, local officials said.
The Harris County Medical Examiner's office reports that three people, a 4-year-old boy and two men, ages 18 and 34, died when generators were being used without proper ventilation. The three are unrelated and their identities are not being released.
The storm and its fallout are also believed responsible for at least five deaths in Galveston County, among the hardest hit areas, and several others in Montogmery, Chambers and Walker counties from fires and fallen trees.
As rescue officials continued searching for victims across the coast, the storm also has resulted in mass closing at area school districts - some of which have suspended classes until further notice.
In addition to the Houston ISD - which announced today that schools likely will be closed for a week to 10 days because of lack of electricity and minor damage to some campuses - a number of other districts say they are postponing classes until further notice.
Those include the Pearland, Goose Creek, Hitchcock, and Pasadena school districts. Texas City schools will be closed until post-hurricane assessments are finished, and Episcopal High School in Houston is closed until Sept. 22.
School officials will confer with the Texas Education Agency to see how many school days missed because of the hurricane may need to be made up.
In other developments, oil company inspections of damage at the Houston Ship Channel refinery complex the nation's largest continued today as President Bush expressed concern about Hurricane Ike's impact on the nation's fuel supply.
Bush, who plans to visit Houston on Tuesday, told reporters he was concerned about "upward pressure" on prices proving a hardship to consumers.
The AAA auto club today said gasoline in Texas has increased an average 13 cents per gallon since Friday. Nationally, the cost has risen by 10 percent, on average.
ExxonMobil reported that its Baytown and Beaumont refinery suffered widespread, but not significant, damage from the hurricane. The world's largest oil company is importing gasoline from refineries in Europe, Asia and the Middle East to supplement supplies.
Shell Oil Co. today said flyover examinations of its offshore platforms or drilling rigs has revealed no major structural damage. Marathon Oil's Texas City refinery remains without electrical power.
Diamond Offshore Drilling today reported that its offshore rigs stayed in place during the hurricane, but at least one jack-up rig lost all its drilling equipment.
On the local level, many Houston-area service stations remained without power today. Those that do have electricity to run their pumps were the sites of blocks-long lines of customers growing desperate for fuel.
State Sen. Rodney Ellis said he is investigating, at Mayor Bill White's request, the possibility of bringing gasoline trucks into Houston neighborhoods.
Ellis expressed concerns about the safety of such a plan, however.
"We are no longer in the eye of the storm," he said, "but in the eye of the aftermath, which in many ways can be more devastating."
Meanwhile, an estimated 10,000 people flocked to a food distribution site at a north Houston church this morning as emergency officials opened six such centers around the Houston area and prepared to open 11 more by day's end.
The opening of the sites, where food, ice and bottled water are being distributed, apparently defused a potential conflict between the city and the Federal Emergency Management Agency.
In addition to FEMA supplies, the centers distributed 200,000 pounds of ice donated by H-E-B supermarkets.
The line of residents awaiting assistance stretched two blocks at the Greenspoint distribution point, the Harvest Time Church at 17770 Imperial Valley. Authorities said the first client arrived at the site at 3:30 a.m., hours before the church opened its doors.
The center briefly ran out of food a few hours after opening, but more trucks soon arrived and the food distribution resumed.
At Conroe's distribution point, the Grand Theatre, the process of handing out essentials seemingly went smoothly. Ray Alewin, 61, of Seven Coves waited in line only 10 minutes.
"Ice is the big commodity," he said. "If you get ice, you can make it."
In La Porte, cars began lining up at the distribution center, New Life Christian Fellowship, early today, stretching about a half-mile on Underwood, then an additional half-mile down Spencer Highway.
While most recipients were grateful, consternation arose when some cars containing multiple families finally arrived at the head of the line. Only then did they learn that each vehicle was limited to two bags of ice, one case of bottled water and a single 12-pack of ready-to-eat meals.
"The rules are in place to make sure everybody gets at least something," said church pastor Sean Mooney. "It's not the church's limit; it's the FEMA rule. They don't want people hoarding up and then taking it out and selling it. This makes the distribution more equal."
Homeland Security Secretary Michael Chertoff pledged on Sunday that his team was "working feverishly" to get the centers open, even as Mayor Bill White expressed concern over how long the process was taking.
FEMA officials responded that setting up the centers was contingent on assurances that workers would be on hand to staff them.
This morning, Houstonians Carlos Torres, 62, and his wife, Zenaida Carrizales, waited in the Greenspoint line for 90 minutes the thought of the wilting food in their powerless refrigerator foremost in their minds.
Torres said the couple had searched the city in vain for a bag of ice.
"Beggars can't be choosers," he said as he eyed the line snaking around the church. "I just hope they'll have enough for everyone."
Torres and his wife, and thousands of others standing in FEMA lines, had the blessing of waiting under sunny, azure skies. Thanks to a cold front that arrived Sunday, temperatures this morning were in the low 70s.
As FEMA sought to help residents, some survivors complained of delays when they called for help rebuilding their homes and lives are advised to call the agency 1-800-621-FEMA. When they do, several said, they get a recorded message and then, abruptly, a dial tone.
The recorded message says FEMA personnel are busy helping others, which is, no doubt, true. After advising callers to contact the agency via the Internet, it says: ``We apologize for this inconvenience. Your call will now be disconnected.''
``Why don't they let you hold on a little bit?,'' asked Roger Boneno of Sugar Land, a diabetic who needs ice to refrigerate his insulin.
Why not do as the computer help lines do - warn that it might be a long wait but advise the caller to hold on, Boneno wondered. ``They disconnect you. It's right away. It's immediate. They want you to use their Web site - which would be neat if I could,'' he said. Indeed, callers are advised twice in the short message to contact the agency via the Internet. ``But we don't have the Internet if we don't have power,'' Boneno said.
Terry Singeltary of Bacliff, whose home, along with his neighbors,' lost its rear walls to the storm, wondered Monday when help would arrive. ``I've seen two police cars since Thursday,'' said Singeltary, who is disabled by a neck injury. ``I've seen helicopters flying over, but nobody is coming in here. And I can't get ahold of FEMA. That's a joke. ``I did get ahold of my insurance people,'' he added. ``They've been pretty good so far.''
Although the Texas Gulf Coast is strewn with wreckage in Hurricane Ike's wake, there were a few bright signs today that life is haltingly, incrementally moving toward normal. Commercial flights resumed today at Bush Intercontinental Airport with the 5:56 a.m. arrival of a Continental flight from Seattle. Hobby Airport should begin commercial flights on Tuesday. The city's admonition to boil tap water before drinking it expired this afternoon. No evidence of contamination has been found. The Metropolitan Transit Authority resumed operation of some high-priority bus routes. Regular Houston garbage collection began today and Fort Bend County with the possible exception of Sugar Land is planning trash pickup Tuesday of trees, shrubs and other debris. The city's 311 service line this morning was back in operation, with at least 40 lines up and running. All freeways to downtown Houston were reported open this morning, although the Interstate 10 Smith Street exit and the Interstate 45 Milam Street exit to the city's center remained closed. In a city still largely shuttered, it was apparent which businesses had returned to life. Lines of customers stretched from front doors to around the block at a League City Wal-Mart, a Hobby Airport Shipley's Doughnuts and a Gulfgate H-E-B supermarket. A similar scene was apparent at service stations that had fuel and electricity to dispense it. That was the good news. Good news came today, too, from the federal government, which, the Associated Press reported, will offer a three-month moratorium on mortgage foreclosures that are insured by the Federal Housing Authority to people living in disaster areas. Additionally, federally guaranteed loans will be provided state and local governments for rehabilitate housing, economic development and infrastructure repair. Still, the situation for coastal residents in southeast Texas and western Louisiana remained bleak. The storm seemingly spared no one, with at least seven fatalities in Texas so far, floodwaters, fallen trees, and shortages of power, water and ice affecting every community from Galveston to Beaumont, Houston to Surfside, The Woodlands to Pearland. Ike also destroyed a number of production platforms and damaged pipelines in the Gulf of Mexico, exasperating a rebuilding effort that is expected to top $10 billion. The death toll includes at least five in the battered barrier island city of Galveston. With the hurricane transforming the popular destination into a debris-covered ghost town, Mayor Lyda Ann Thomas warned residents to stay away, possibly for weeks, until the most pressing services are restored. In Houston, Mayor Bill White on Sunday ordered a weeklong curfew from 9 p.m. to 6 a.m. because of the widespread damage and darkness. White today reported that Houston crime levels are below normal, although fires have been more frequent. "The level of vigilance has been high," he said. White said Houston residents whose homes are uninhabitable can find shelter at the George R. Brown Convention Center. He asked that the shelter cots be saved for those with severe hardship, not those merely made uncomfortable by power outages. For the first time this morning rescuers flew into the heart of the coastal devastation. State helicopters flew 115 rescuers into Bolivar Peninsula, across Bolivar Roads from Galveston Island's eastern tip, to find entire subdivisions obliterated. "They had a lot of devastation over there," task force leader Chuck Jones told the Associated Press. "It took a direct hit." Jones said his crew would attempt to reach hard-hit Galveston Island tonight, but was prepared to remain on Bolivar.
National death toll at least 30
At least 30 people have died as a result of the hurricane, but most of the fatalities have occurred elsewhere in the United States as the storm followed a northeasterly track across the nation. This morning, the remnants of the storm were near Rochester, N.Y., where they were generating winds of 30 mph. By this afternoon, the National Weather Service had dropped its regular postings regarding the storm's location. In the Houston area, deaths so far included a 72-year-old Galveston man who died in his truck after trying to flee the island late Friday. A woman died in her bed early Saturday when a tree fell on her home in Pinehurst. And a 4-year-old Houston boy and two adults in his home died of carbon monoxide poisoning from the generator his family was using for power. Despite repeated warnings from authorities regarding the hazards of improper use of gasoline-powered generators, storm victims continue to fall ill with carbon monoxide poisoning. Three members of a Sugar Land family this morning were hospitalized from exposure to the deadly gas. Meanwhile, the search-and-rescue effort was the largest in the state's history, with more than 50 helicopters, 1,500 searchers and teams from federal, state and local agencies. Authorities said that 1,984 people had been rescued, including 394 by air, as of Sunday afternoon. The state opened more than 250 shelters for more than 37,000 evacuees, and there were plans to evacuate up to 10,500 residents who had stayed in Galveston.
Setting priorities
With the area gripped by a massive power outage, millions of Houstonians got creative and, in some cases, cranky as they craved fresh coffee, cold air and their Sunday football fix.
Some took to their lawns to escape sauna-like heat, while others huddled in the homes of those lucky enough to have working lights, air conditioning and television. Grocery stores, pharmacies and a few restaurants with restored power or generators drew long lines of customers.
CenterPoint said it had restored power to 500,000 customers, but about 1.6 million were still without power. Entergy said 99 percent of their customers still were without power, and Texas New Mexico Power said 64 percent were without electricity.
This report was compiled by Chronicle reporters Allan Turner and Matt Stiles and includes contributions by reporters Bill Murphy, Mark Carreau, Rad Sallee, Lynn Cook, Carolyn Feibel, Mary Flood, Mike Glenn, Roma Khanna, Ericka Mellon, Bradley Olson, Liz Austin Peterson, Ruth Rendon, Bennett Roth, Dane Schiller and Jennifer Latson. The Associated Press also contributed
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
-------------------- BSE-L@LISTS.AEGEE.ORG --------------------
--------------------------------------------------------------------------------
--------------------------------------------------------------------------------
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle? Martin Jeffrey , Belinda Baquero-Perez , Stuart Martin , Linda Terry and Lorenzo Gonzalez
BMC Veterinary Research 2008, 4:38doi:10.1186/1746-6148-4-38
Published: 30 September 2008
Abstract (provisional) Background The epidemic form of Bovine Spongiform Encephalopathy (BSE) is generally considered to have been caused by a single prion strain but at least two strain variants of cattle prion disorders have recently been recognized. Idiopathic brainstem neuronal chromatolysis and hippocampal sclerosis (IBNC) is a rare neurological disease of adult cattle which was recognised in a sub-set of cattle submitted under the BSE Orders in which lesions of BSE were absent. Between the years of 1988 and 1991 IBNC occurred in Scotland with an incidence of ~7 cases per 100,000 beef suckler cows over the age of 6 years.
Results When brains of 15 IBNC cases were each tested by immunohistochemistry, all showed the abnormal presence of prion protein (PrP). Abnormal PrP labeling was also present in the retina of a single case available for examination. The pattern of PrP labeling in brain is distinct from that seen in other ruminant prion diseases. Brains of IBNC cattle do not reveal abnormal PrP isoforms when tested by the commercial BioRad or Idexx test kits and do not reveal PrPres when tested using stringent proteinase digestion methods. However, some biochemical evidence for weakly protease resistant isoforms of PrP can be detected when tissues are examined using mild proteinase digestion techniques.
Conclusions The study shows that a distinctive neurological disorder of cattle which has some clinical similarities to BSE, is associated with abnormal PrP accumulation in brain but the pathology and biochemistry of IBNC are distinct from BSE. The study is important either because it raises the possibility of a significant increase in the scope of prion disease or because it demonstrates that widespread and consistent PrP alterations may not be confined to prion diseases. Transmission experiments are needed to establish whether IBNC is a condition in which prion protein is abnormally regulated or is yet a further example of an infectious cattle prion disease.
http://www.biomedcentral.com/1746-6148/4/38/abstract
snip...
Discussion
This study shows that the novel condition of cattle previously identified as IBNC and recognized from within the BSE suspect submissions, abnormally expresses or accumulates PrP in brain and retina. However, this abnormal PrP is not composed of isoforms that are strongly resistant to protease digestion suggesting that it is not present in the form of large aggregates.
Immunohistochemical demonstration of PrP labelling and increased levels of PrP mRNA have previously been described in adult humans affected with acute vascular disorders, in infants with perinatal hypoxia and experimental infarction of rodents [9]. These findings are considered to represent upregulation of PrP expression which encompasses part of the oxidative stress response of neurons. We have also observed increased PrP in the cytoplasm of neurons undergoing ischaemic degeneration in a variety of sheep encephalopathies. Though ischaemic neuronal degeneration is not a feature of IBNC, nevertheless, the presence of PrP within the cytoplasm of some chromatolytic and degenerate neurons of IBNC affected cattle is consistent with the idea that stressed neurons may respond by increasing PrP expression.
Though present in only two cows, the pattern of PrP accumulation within the granule cell layer of the cerebellum is morphologically similar to that reported by several authors for Nor 98 types of the transmissible spongiform encephalopathy or prion disease of sheep [3, 10]. The PrP accumulation within the plexiform layers of the eye is similar to that of both natural scrapie [11] and of Nor 98 (MJ personal observations). However the majority type of PrP labelling that occurred in all IBNC cases was found within the neuropil, mainly in the rostral neuraxis and cerebrum, the nature of which is previously unreported in cattle or in any other prion disorder. This novel pattern of labelling appears to correspond to a rarefaction or fine microvacuolation of neuropil as seen on standard HE stained sections.
Pathological, biochemical and bioassay data all suggest that the epidemic form of cattle BSE is a single strain. However, recent large scale EU wide surveillance for BSE has led to the unexpected discovery of rare and hitherto unknown prion diseases of cattle. Small numbers of atypical forms of cattle prion diseases have now been recognized from several European countries, in the USA and Japan and can be distinguished by histological, molecular and transmission characteristics [12-14]. Bovine Amyloidotic Spongiform Encephalopathy (BASE) was the first of these novel cattle prion disorders to be recognized and was characterized by the presence of numerous small amyloid deposits of abnormal PrP. It was initially discovered in three aged Italian cattle [1] and has subsequently been transmitted to transgenic mice [13, 14]. A further variant of a cattle prion disease affecting cows between 8 and 15 years was initially recognized in France [2] and has also been transmitted to mice. BSE and these novel cattle prion diseases can be distinguished using biochemical and molecular methods and are now classified as C, H and L type isolates [12]. H type and L type (BASE) isolates are defined according to the higher and lower positions of the unglycosylated PrPres bands in Western blots, respectively, when compared to the position of the corresponding band in classical BSE (C type) isolates [12]. L type cases formerly classified as BASE, have a distinctive glycopattern in which monoglycoslyated PrPres predominates compared to BSE [12,14]. While IBNC cases
are on average older than BSE cases they occupy a similar age class of cattle to that of H and L type cattle prion diseases, but IBNC can be readily distinguished from H, L and C type cattle prion disease by morphologic pathology and by the absence of PrPres under stringent conditions of protease digestion.
Not all abnormal PrPres isoforms detected from brains of animals affected with prion disease are resistant to stringent protease digestion. The PrPres of two sheep of the ARR/ARR PrP genotype affected with a classical scrapie-like disease accumulated unusually protease sensitive isoforms of PrP [15]. Similarly, the transmissible prion disease of sheep known as Nor 98 and related conditions (often referred to as atypical scrapie) also have weakly protease resistant PrPres [3,16]. Nor 98 does not appear to transmit readily to other sheep under field conditions: it also does not transmit to conventional mice although it does readily transmit disease to one strain of transgenic mouse which substantially over-expresses the VRQ allele of sheep PrP [16,17]. The transgenic PG14 mouse also has PrPres which is even more readily digested than that found in Nor 98 but this prion protein disorder has not so far been successfully transmitted [18]. The biochemical analyses of limited numbers of IBNC cases clearly shows that highly aggregated forms of protease resistant PrP are not present in brain tissue. However, when the data from the ELISA and immunoblot tests using mild protease digestion are compared with that of normal control material it is possible that smaller aggregates of PrP molecules may be present.
Conclusions
The present results indicate that there are changes in PrP expression or accumulation in the neurodegenerative cattle disorder known as IBNC. The pathology and biochemistry of IBNC are quite distinct from that of other prion diseases of cattle and other species but the pathology does include grey matter spongiform changes. The transmissibility of this disorder is undetermined. These results are interesting as they show that either the range of prion diseases and associated pathology is still wider than previously thought or that substantial abnormalities of prion protein expression may be associated with brain lesions unconnected with classical prion diseases. Further biochemical and transmission studies are needed to determine which of these possibilities is correct.
http://www.biomedcentral.com/content/pdf/1746-6148-4-38.pdf
NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
http://www.pnas.org/cgi/content/abstract/0502296102v1
Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007
http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html
http://nor-98.blogspot.com/
SCRAPIE USA
http://scrapie-usa.blogspot.com/
Sunday, June 15, 2008
A descriptive study of the prevalence of atypical and classical scrapie in sheep in 20 European countries
Research article
http://nor-98.blogspot.com/2008/06/descriptive-study-of-prevalence-of.html
another question, just how long have these atypical BSE TSEs been around in the bovine ???
let's look at another case of atypical BSE in Germany way back in 1992 ;
Subject: atypical BSE reported in 1992 and conviently slaughterd and incinerated and then swept under rug for about 12 years Date: April 26, 2007 at 1:08 pm PST 1992
NEW BRAIN DISORDER
3. WHAT ABOUT REPORTS OF NEW FORM OF BSE?
THE VETERINARY RECORD HAS PUBLISHED AN ARTICLE ON A NEW BRAIN DISORDER OF CATTLE DISCOVERED THROUGH OUR CONTROL MEASURES FOR BSE. ALTHOUGH IT PRESENTS SIMILAR CLINICAL SIGNS TO BSE THERE ARE MAJOR DIFFERENCES IN THE HISTOPATHOLOGY AND INCUBATION PERIODS BETWEEN THE TWO. MUST EMPHASISE THAT THIS IS NOT BSE.
4. IS THIS NEW BRAIN DISORDER A THREAT?
WE DO NOT EVEN KNOW WHETHER THE AGENT OF THIS DISEASE IS TRANSMISSIBLE. IN ANY CASE, CASES SO FAR IDENTIFIED HAD SHOWN SIMILAR SYMPTOMS TO THOSE OF BSE, AND THEREFORE HAVE BEEN SLAUGHTERED AND INCINERATED, SO THAT IF A TRANSMISSIBLE AGENT WERE INVOLVED IT WOULD HAVE BEEN ELIMINATED. .......
http://www.bseinquiry.gov.uk/files/yb/1992/10/26001001.pdf
2. The Collinge/Will dispute appears to rumble on. Dr. Collinge had told Dr. Tyrrell that Dr. Will's response to his criticism about sharing material had been ''quite unacceptable'' (in spite of it's apparently conciliatory tone). Apparently Professor Allen was now going to try and arrange a meeting to resolve the dispute. No action here for MAFF, although Mr. Murray may be interested.
3. Dr. Tyrrell regretted that the Committee had not seen the article on BBD. However he felt that for the time being NO specific action was called for. The most important need was to consider the possibility that the condition might be transmissible. As we have discussed, I suggested that we might circulate a paper to the members of the committee giving our appreciation of this condition (and perhaps of other non-BSE neurological conditions that had been identified in negative cases) and of any necessary follow up action. IF any Committee member felt strongly about this, or if the issue CAME TO A HEAD, we would call an interim meeting. He was happy with this approach. I would be grateful if Mr. Maslin could, in discussion with CVL and veterinary colleagues draft such a note, which will presumably very largely follow what Mr. Bradley's briefing paper has already said, taking account of DOH comments, We can then clear a final version with DOH before circulating it to Committee members.
http://www.bseinquiry.gov.uk/files/yb/1992/10/29005001.pdf
IN CONFIDENCE
This is a highly competitive field and it really will be a pity if we allow many of the key findings to be published by overseas groups while we are unable to pursue our research findings because of this disagreement, which I hope we can make every effort to solve.
http://www.bseinquiry.gov.uk/files/yb/1992/10/26002001.pdf
COLLINGE THREATENS TO GO TO MEDIA
http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf
2. The discovery might indicate the existence of a different strain of BSE from that present in the general epidemic or an unusual response by an individual host.
3. If further atypical lesion distribution cases are revealed in this herd then implications of misdiagnosis of 'negative' cases in other herds may not be insignificant.
snip...
This minute is re-issued with a wider distribution. The information contained herein should NOT be disseminated further except on the basis of ''NEED TO KNOW''.
R Bradley
http://www.bseinquiry.gov.uk/files/yb/1993/02/17001001.pdf
IN CONFIDENCE
BSE ATYPICAL LESION DISTRIBUTION
http://www.bseinquiry.gov.uk/files/yb/1993/03/14001001.pdf
ALABAMA MAD COW CASE
http://www.aphis.usda.gov/newsroom/hot_issues/bse/downloads/EPI_Final5-2-06.pdf
http://www.cdc.gov/ncidod/dvrd/bse/news/alabama_cow_031506.htm
Texas BSE Investigation Final Epidemiology Report August 2005
http://www.aphis.usda.gov/newsroom/hot_issues/bse/downloads/bse_final_epi_report8-05.pdf
State-Federal Team Responds to Texas BSE Case
JUNE 30, 2005
(please note 7+ month delay in final confirmation so the BSE MRR policy could be set in stone first. $$$...tss)
http://www.tahc.state.tx.us/news/pr/2005/2005Jun30_BSE_Positive_Results.pdf
https://www.cidrap.umn.edu/cidrap/content/other/bse/news/june3005bse.html
SEE ATTEMPTED COVER-UP BEFORE THE END AROUND BY FONG ET AL OF THE O.I.G
The U.S. Department of Agriculture confirmed June 29 that genetic testing had verified bovine spongiform encephalopathy (mad cow disease) in a 12-year-old cow that was born and raised in a Texas beef cattle herd.
Subsequent epidemiological investigations resulted in the culling and testing of 67 adult animals from the index herd. Bio-Rad tests for BSE were conducted on all 67 animals by the National Veterinary Services Laboratory (NVSL) in Ames, Iowa. All tests were negative.
On July 12, Texas officials lifted the quarantine on the source herd. At press time, USDA's Animal and Plant Health Inspection Service was tracing animals of the same age that had left the ranch.
Timeline
The BSE-positive animal was a Brahman-cross cow born and raised in a single Texas herd. The location of the ranch was not disclosed.
On Nov. 11, 2004, the 12-year-old cow was taken to a Texas auction market. Because of its condition, the cow was sent to Champion Pet Foods in Waco, Texas. The company produces several blends of dog food, primarily for the greyhound industry.
On Nov. 15, the animal arrived dead at Champion. Under procedures established by USDA's intensive surveillance program, a sample was sent to the USDA-approved Texas Veterinary Medical Diagnostic Testing Laboratory (TVMDL) at Texas A&M University.
Between June 1, 2004, and June 1, 2005, TVMDL tested nearly 34,000 samples from Texas, New Mexico, Arkansas and Louisiana. They tested the sample from Champion on Nov. 19 using a Bio-Rad ELISA rapid test for BSE. Initial results were inconclusive.
Because of the inconclusive results, a representative from USDA took the entire carcass to TVMDL where it was incinerated. USDA's Animal and Plant Health Inspection Service (APHIS) began tracing the animal and herd.
The sample was then sent to the National Veterinary Services Laboratory for further testing. Two Immunohistochemistry (IHC) tests were conducted and both were negative for BSE. At that point APHIS stopped their trace.
USDA scientists also ran an additional, experimental IHC "rapid" tissue fixation test for academic purposes. This test has not been approved internationally.
Some abnormalities were noted in the experimental test, but because the two approved tests came back negative, the results were not reported beyond the laboratory.
Monitoring by OIG
USDA's Office of Inspector General (OIG) has been monitoring implementation of the BSE expanded surveillance program and evaluating the following:
* Effectiveness of the surveillance program;
* Performance of BSE laboratories in complying with policies and procedures for conducting tests and reporting results;
* Enforcement of the ban on specified risk materials in meat products;
* Controls to prevent central nervous system tissue in advanced meat recovery products;
* Ante mortem condemnation procedures; and
* Procedures for obtaining brain tissue samples from condemned cattle.
While reviewing voluminous records, OIG auditors noticed conflicting test results on one sample-rapid inconclusive, IHC negative, experimental reactive.
Sample retested
At the recommendation of the Inspector General, the sample was retested during the week of June 5 with a second confirmatory test, the Western Blot. The results were reactive.
USDA scientists then conducted an additional IHC confirmatory test, using different antibodies from the November 2004 test. On Friday, June 10, Secretary of Agriculture Mike Johanns publicly announced the results as a "weak positive."
On June 16 an official with USDA's National Veterinary Services Laboratory hand-carried samples for further testing to the Veterinary Laboratory Agency (VLA) in Weybridge, England. Since 1991, the VLA has been a BSE reference laboratory for the World Organization for Animal Health (OIE).
Experts from the Weybridge lab confirmed the accuracy of the results of USDA's November confirmatory IHC test, concurring that the case could not have been confirmed on the basis of this sample. They also examined the November experimental IHC test and interpreted the results to be positive.
Weybridge also conducted additional tests, including IHC, OIE-prescribed Western Blot, NaTTA Western Blot and Prionics Western Blot tests.
To better understand the conflicting results, USDA also conducted Bio-Rad and IDEXX rapid screening tests, IHC and OIE-prescribed Western Blot. USDA also used DNA sequencing to determine the prion protein gene sequence of the animal.
http://findarticles.com/p/articles/mi_qa5420/is_200508/ai_n21377094
Texas even had a 'secret' test that showed that mad cow positive; experimental IHC test results, because the test was not a validated procedure, and because the two approved IHC tests came back negative, the results were not considered to be of regulatory significance and therefore were not reported beyond the laboratory. . A Western blot test conducted the week of June 5, 2005, returned positive for BSE.
http://www.usda.gov/documents/vs_bse_ihctestvar.pdf
48 hr BSE confirmation turnaround took 7+ months to confirm this case, so the BSE MRR policy could be put into place. ...TSS
-------- Original Message --------
Subject: re-USDA's surveillance plan for BSE aka mad cow disease
Date: Mon, 02 May 2005 16:59:07 -0500
From: "Terry S. Singeltary Sr."
To: paffairs@oig.hhs.gov, HHSTips@oig.hhs.gov, contactOIG@hhsc.state.tx.us
Greetings Honorable Paul Feeney, Keith Arnold, and William Busbyet al at OIG, ...............
snip...
There will be several more emails of my research to follow. I respectfully request a full inquiry into the cover-up of TSEs in the United States of America over the past 30 years. I would be happy to testify...
Thank you, I am sincerely, Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 xxx xxx xxxx
snip... see full text ;
Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ? August 20, 2008
http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html
Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate
http://organicconsumers.org/forum/index.php?showtopic=1951
http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html
snip... FULL TEXT ;
http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0003017
"the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine." <<<
NOT to forget ;
Thursday, June 05, 2008
Review on the epidemiology and dynamics of BSE epidemics
Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article
snip...
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
snip...
Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
full text 18 pages ;
http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf
please see full text ;
http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
snip...
Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
snip...
http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html
Greetings BSE-L AND CJD-L,
I'm still here damn't. well, barely, a few photo's and news articles to explain my absence, i hope no one minds. the wife said if we survived riding out IKE, she would kill me later. we went from being an Island, to literally being in the surf for a while. IKE threw everything including the kitchen cabinets, and literally the kitchen sink from the garage apartment at us. the old homestead still stands. battered, bruised, and wet, we are very blessed. much to do, so until later. ...TSS
more photo's of this area destruction from IKE. ...
http://www.boston.com/bigpicture/2008/09/the_short_but_eventful_life_of.html?s_campaign=8315
Residents dig out from storm
By Chris Paschenko The Daily News Published September 16, 2008
BACLIFF - A wall of water not seen in generations of storms smashed through dozens of homes lining Galveston Bay, and residents spent Tuesday clearing debris and digging out the mud and the muck.
Terry Singeltary refused to evacuate his Bayshore Drive home in Bacliff, saying he feared looters more than Hurricane Ike.
"My grandparents bought this place in 1935, and I wound up with it," Singeltary said. "It made it through a storm in '48, and (hurricanes) Carla and Alicia. I've never seen the water this high."
The Singeltary home survived with water damage in his game room. Singeltary never needed his backup plan: a kayak or an axe stashed in the attic. ...snip
http://galvestondailynews.com/story.lasso?ewcd=276365f800b0da1c
216. vortfix 9:25 PM GMT on September 15, 2008
Galveston warns of 'downward spiral'
Mobile morgue en route to Galveston as search continues
Five dead, but thousands of homes still to be searched
Three unrelated deaths from apparent carbon monoxide poisoning from generator use have brought the local death toll from Hurricane Ike to at least 14, local officials said.
The Harris County Medical Examiner's office reports that three people, a 4-year-old boy and two men, ages 18 and 34, died when generators were being used without proper ventilation. The three are unrelated and their identities are not being released.
The storm and its fallout are also believed responsible for at least five deaths in Galveston County, among the hardest hit areas, and several others in Montogmery, Chambers and Walker counties from fires and fallen trees.
As rescue officials continued searching for victims across the coast, the storm also has resulted in mass closing at area school districts - some of which have suspended classes until further notice.
In addition to the Houston ISD - which announced today that schools likely will be closed for a week to 10 days because of lack of electricity and minor damage to some campuses - a number of other districts say they are postponing classes until further notice.
Those include the Pearland, Goose Creek, Hitchcock, and Pasadena school districts. Texas City schools will be closed until post-hurricane assessments are finished, and Episcopal High School in Houston is closed until Sept. 22.
School officials will confer with the Texas Education Agency to see how many school days missed because of the hurricane may need to be made up.
In other developments, oil company inspections of damage at the Houston Ship Channel refinery complex the nation's largest continued today as President Bush expressed concern about Hurricane Ike's impact on the nation's fuel supply.
Bush, who plans to visit Houston on Tuesday, told reporters he was concerned about "upward pressure" on prices proving a hardship to consumers.
The AAA auto club today said gasoline in Texas has increased an average 13 cents per gallon since Friday. Nationally, the cost has risen by 10 percent, on average.
ExxonMobil reported that its Baytown and Beaumont refinery suffered widespread, but not significant, damage from the hurricane. The world's largest oil company is importing gasoline from refineries in Europe, Asia and the Middle East to supplement supplies.
Shell Oil Co. today said flyover examinations of its offshore platforms or drilling rigs has revealed no major structural damage. Marathon Oil's Texas City refinery remains without electrical power.
Diamond Offshore Drilling today reported that its offshore rigs stayed in place during the hurricane, but at least one jack-up rig lost all its drilling equipment.
On the local level, many Houston-area service stations remained without power today. Those that do have electricity to run their pumps were the sites of blocks-long lines of customers growing desperate for fuel.
State Sen. Rodney Ellis said he is investigating, at Mayor Bill White's request, the possibility of bringing gasoline trucks into Houston neighborhoods.
Ellis expressed concerns about the safety of such a plan, however.
"We are no longer in the eye of the storm," he said, "but in the eye of the aftermath, which in many ways can be more devastating."
Meanwhile, an estimated 10,000 people flocked to a food distribution site at a north Houston church this morning as emergency officials opened six such centers around the Houston area and prepared to open 11 more by day's end.
The opening of the sites, where food, ice and bottled water are being distributed, apparently defused a potential conflict between the city and the Federal Emergency Management Agency.
In addition to FEMA supplies, the centers distributed 200,000 pounds of ice donated by H-E-B supermarkets.
The line of residents awaiting assistance stretched two blocks at the Greenspoint distribution point, the Harvest Time Church at 17770 Imperial Valley. Authorities said the first client arrived at the site at 3:30 a.m., hours before the church opened its doors.
The center briefly ran out of food a few hours after opening, but more trucks soon arrived and the food distribution resumed.
At Conroe's distribution point, the Grand Theatre, the process of handing out essentials seemingly went smoothly. Ray Alewin, 61, of Seven Coves waited in line only 10 minutes.
"Ice is the big commodity," he said. "If you get ice, you can make it."
In La Porte, cars began lining up at the distribution center, New Life Christian Fellowship, early today, stretching about a half-mile on Underwood, then an additional half-mile down Spencer Highway.
While most recipients were grateful, consternation arose when some cars containing multiple families finally arrived at the head of the line. Only then did they learn that each vehicle was limited to two bags of ice, one case of bottled water and a single 12-pack of ready-to-eat meals.
"The rules are in place to make sure everybody gets at least something," said church pastor Sean Mooney. "It's not the church's limit; it's the FEMA rule. They don't want people hoarding up and then taking it out and selling it. This makes the distribution more equal."
Homeland Security Secretary Michael Chertoff pledged on Sunday that his team was "working feverishly" to get the centers open, even as Mayor Bill White expressed concern over how long the process was taking.
FEMA officials responded that setting up the centers was contingent on assurances that workers would be on hand to staff them.
This morning, Houstonians Carlos Torres, 62, and his wife, Zenaida Carrizales, waited in the Greenspoint line for 90 minutes the thought of the wilting food in their powerless refrigerator foremost in their minds.
Torres said the couple had searched the city in vain for a bag of ice.
"Beggars can't be choosers," he said as he eyed the line snaking around the church. "I just hope they'll have enough for everyone."
Torres and his wife, and thousands of others standing in FEMA lines, had the blessing of waiting under sunny, azure skies. Thanks to a cold front that arrived Sunday, temperatures this morning were in the low 70s.
As FEMA sought to help residents, some survivors complained of delays when they called for help rebuilding their homes and lives are advised to call the agency 1-800-621-FEMA. When they do, several said, they get a recorded message and then, abruptly, a dial tone.
The recorded message says FEMA personnel are busy helping others, which is, no doubt, true. After advising callers to contact the agency via the Internet, it says: ``We apologize for this inconvenience. Your call will now be disconnected.''
``Why don't they let you hold on a little bit?,'' asked Roger Boneno of Sugar Land, a diabetic who needs ice to refrigerate his insulin.
Why not do as the computer help lines do - warn that it might be a long wait but advise the caller to hold on, Boneno wondered. ``They disconnect you. It's right away. It's immediate. They want you to use their Web site - which would be neat if I could,'' he said. Indeed, callers are advised twice in the short message to contact the agency via the Internet. ``But we don't have the Internet if we don't have power,'' Boneno said.
Terry Singeltary of Bacliff, whose home, along with his neighbors,' lost its rear walls to the storm, wondered Monday when help would arrive. ``I've seen two police cars since Thursday,'' said Singeltary, who is disabled by a neck injury. ``I've seen helicopters flying over, but nobody is coming in here. And I can't get ahold of FEMA. That's a joke. ``I did get ahold of my insurance people,'' he added. ``They've been pretty good so far.''
Although the Texas Gulf Coast is strewn with wreckage in Hurricane Ike's wake, there were a few bright signs today that life is haltingly, incrementally moving toward normal. Commercial flights resumed today at Bush Intercontinental Airport with the 5:56 a.m. arrival of a Continental flight from Seattle. Hobby Airport should begin commercial flights on Tuesday. The city's admonition to boil tap water before drinking it expired this afternoon. No evidence of contamination has been found. The Metropolitan Transit Authority resumed operation of some high-priority bus routes. Regular Houston garbage collection began today and Fort Bend County with the possible exception of Sugar Land is planning trash pickup Tuesday of trees, shrubs and other debris. The city's 311 service line this morning was back in operation, with at least 40 lines up and running. All freeways to downtown Houston were reported open this morning, although the Interstate 10 Smith Street exit and the Interstate 45 Milam Street exit to the city's center remained closed. In a city still largely shuttered, it was apparent which businesses had returned to life. Lines of customers stretched from front doors to around the block at a League City Wal-Mart, a Hobby Airport Shipley's Doughnuts and a Gulfgate H-E-B supermarket. A similar scene was apparent at service stations that had fuel and electricity to dispense it. That was the good news. Good news came today, too, from the federal government, which, the Associated Press reported, will offer a three-month moratorium on mortgage foreclosures that are insured by the Federal Housing Authority to people living in disaster areas. Additionally, federally guaranteed loans will be provided state and local governments for rehabilitate housing, economic development and infrastructure repair. Still, the situation for coastal residents in southeast Texas and western Louisiana remained bleak. The storm seemingly spared no one, with at least seven fatalities in Texas so far, floodwaters, fallen trees, and shortages of power, water and ice affecting every community from Galveston to Beaumont, Houston to Surfside, The Woodlands to Pearland. Ike also destroyed a number of production platforms and damaged pipelines in the Gulf of Mexico, exasperating a rebuilding effort that is expected to top $10 billion. The death toll includes at least five in the battered barrier island city of Galveston. With the hurricane transforming the popular destination into a debris-covered ghost town, Mayor Lyda Ann Thomas warned residents to stay away, possibly for weeks, until the most pressing services are restored. In Houston, Mayor Bill White on Sunday ordered a weeklong curfew from 9 p.m. to 6 a.m. because of the widespread damage and darkness. White today reported that Houston crime levels are below normal, although fires have been more frequent. "The level of vigilance has been high," he said. White said Houston residents whose homes are uninhabitable can find shelter at the George R. Brown Convention Center. He asked that the shelter cots be saved for those with severe hardship, not those merely made uncomfortable by power outages. For the first time this morning rescuers flew into the heart of the coastal devastation. State helicopters flew 115 rescuers into Bolivar Peninsula, across Bolivar Roads from Galveston Island's eastern tip, to find entire subdivisions obliterated. "They had a lot of devastation over there," task force leader Chuck Jones told the Associated Press. "It took a direct hit." Jones said his crew would attempt to reach hard-hit Galveston Island tonight, but was prepared to remain on Bolivar.
National death toll at least 30
At least 30 people have died as a result of the hurricane, but most of the fatalities have occurred elsewhere in the United States as the storm followed a northeasterly track across the nation. This morning, the remnants of the storm were near Rochester, N.Y., where they were generating winds of 30 mph. By this afternoon, the National Weather Service had dropped its regular postings regarding the storm's location. In the Houston area, deaths so far included a 72-year-old Galveston man who died in his truck after trying to flee the island late Friday. A woman died in her bed early Saturday when a tree fell on her home in Pinehurst. And a 4-year-old Houston boy and two adults in his home died of carbon monoxide poisoning from the generator his family was using for power. Despite repeated warnings from authorities regarding the hazards of improper use of gasoline-powered generators, storm victims continue to fall ill with carbon monoxide poisoning. Three members of a Sugar Land family this morning were hospitalized from exposure to the deadly gas. Meanwhile, the search-and-rescue effort was the largest in the state's history, with more than 50 helicopters, 1,500 searchers and teams from federal, state and local agencies. Authorities said that 1,984 people had been rescued, including 394 by air, as of Sunday afternoon. The state opened more than 250 shelters for more than 37,000 evacuees, and there were plans to evacuate up to 10,500 residents who had stayed in Galveston.
Setting priorities
With the area gripped by a massive power outage, millions of Houstonians got creative and, in some cases, cranky as they craved fresh coffee, cold air and their Sunday football fix.
Some took to their lawns to escape sauna-like heat, while others huddled in the homes of those lucky enough to have working lights, air conditioning and television. Grocery stores, pharmacies and a few restaurants with restored power or generators drew long lines of customers.
CenterPoint said it had restored power to 500,000 customers, but about 1.6 million were still without power. Entergy said 99 percent of their customers still were without power, and Texas New Mexico Power said 64 percent were without electricity.
This report was compiled by Chronicle reporters Allan Turner and Matt Stiles and includes contributions by reporters Bill Murphy, Mark Carreau, Rad Sallee, Lynn Cook, Carolyn Feibel, Mary Flood, Mike Glenn, Roma Khanna, Ericka Mellon, Bradley Olson, Liz Austin Peterson, Ruth Rendon, Bennett Roth, Dane Schiller and Jennifer Latson. The Associated Press also contributed
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
-------------------- BSE-L@LISTS.AEGEE.ORG --------------------
--------------------------------------------------------------------------------
--------------------------------------------------------------------------------
Labels: atypical bse, ATYPICAL SCRAPIE, SPORADIC CJD, USA
posted by Terry S. Singeltary Sr. at
8:54 AM
0 Comments:
Post a Comment
Subscribe to Post Comments [Atom]
<< Home