USA cases of dpCJD rising with 24 cases so far in 2010
please be aware, i have termed this strange strain of 'diagnosis pending creutzfeldt jakob disease' as 'dpCJD', what ever that is suppose to mean. how many more years, decades, are we going to have to flounder for them to establish another name for the same disease ?
5 Includes 28 cases in which the diagnosis is pending, and 17 inconclusive cases;
6 Includes 28 (24 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded
Let's review some history ;
IDIOPATHIC BRAIN STEM NEURONAL CHROMATOLYSIS AND HIPPOCAMPAL SCLEROSIS
(VET REC 1992, M Jeffrey, J W Wilesmith p359-362)
1. The disease title is comlex and invites the press to coin a new one (such as BSE II, MAD COW II, or Son of BSE). It is suggested for now that it is called a brain disorder or brain dgeneration (BD).
BD - THE LINE TO TAKE
2. Disease clinically similar to BSE but pathologically distinct. BD is NOT a form of BSE.
20 October 1992
Martin Jeffrey observes that there is a strong similarity between the histopathological picture in BBD and that produced by ME7 strain of Scrapie in mice.
7. There have to date been 42 cases of BBD out of a total of 2,598 brains submitted for BSE diagnosis in Scotland. The incidence of the condition appears to be 1 in 10,000 of beef suckler cows.
W L GARDNER VHS
23 October 1992
''THE LINE TO TAKE'' ON IBNC $$$ 1995 $$$
page 9 of 14 ;30. The Committee noted that the results were unusual. the questioned whether there could be coincidental BSE infection or contamination with scrapie. Dr. Tyrell noted that the feeling of the committee was that this did not represent a new agent but it was important to be prepared to say something publicly about these findings. A suggested line to take was that these were scientifically unpublishable results but in line with the policy of openness they would be made publicly available and further work done to test their validity. Since the BSE precautions were applied to IBNC cases, human health was protected. Further investigations should be carried out on isolations from brains of IBNC cases with removal of the brain and subsequent handling under strict conditions to avoid the risk of any contamination.31. Mr. Bradley informed the Committee that the CVO had informed the CMO about the IBNC results and the transmission from retina and he, like the Committee was satisfied that the controls already in place or proposed were adequate. ...
snip... see full text
this information had been proved to be incorrect for a number of reasons...
Saturday, February 28, 2009
NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS
"All of the 15 cattle tested showed that the brains had abnormally accumulated PrP"
Wednesday, October 08, 2008
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
''LINE TO TAKE"
6. Trouble has been brewing for some time. Dr Collinge is lobbying hard, and threatening to go to the media, claiming Dr Will is blocking his research...
9. ...There are also results to be made available shortly (1) concerning a farmer with CJD who had BSE animals, (2) on the possible transmissibility of Alzheimers and (3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest. ...
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
Thursday, November 05, 2009
Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
Sunday, August 10, 2008
A New Prionopathy OR more of the same old BSe and sporadic CJD
Monday, April 5, 2010
UPDATE - CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER
Monday, March 29, 2010
Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER
Thursday, May 27, 2010
CDC - INQUIRY ABOUT vCJD DEATH OF SLAUGHTER HOUSE WORKER IRMA LINDA ANDABLO
Archive Number 20100405.1091 Published Date 05-APR-2010
Subject PRO/AH/EDR> Prion disease update 1010 (04)
[Terry S. Singeltary Sr. has added the following comment:
"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.
The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"
Questions linger in U.S. CJD cases
Published: Oct. 21, 2005 at 9:49 PM
By STEVE MITCHELL, Senior Medical Correspondent
WASHINGTON, Oct. 21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical test widely regarded as the only way to determine precisely the nature of his disease, United Press International has learned.
The case of Patrick Hicks, who died last November from his condition, has remained murky from the beginning. Dr. Ron Bailey, of Riverside, Calif., the man's neurologist, had suspected the 49-year-old Hicks of having contracted variant Creutzfeldt Jakob disease -- a fatal, brain-wasting illness humans can contract from eating beef products contaminated with the mad cow pathogen -- and both he and the family wanted an autopsy conducted to determine if Hicks had succumbed to the disorder.
Bailey became concerned that Hicks might have contracted vCJD because he initially had exhibited psychiatric symptoms, his illness appears to have lasted for more than one year and he showed normal brain-wave patterns via EEGs until the late stages -- all consistent with the disease. In addition, Hicks's relatively young age raised concerns, because nearly all of the more than 150 cases of vCJD detected worldwide have occurred in people under age 55.
The first hint of oddness began when, according to both Hicks's brother and mother, a team of six doctors, who they suspect were with the Centers for Disease Control and Prevention in Atlanta, visited Patrick last October while he was still alive and under care at Loma Linda University Medical Center in Loma Linda, Calif.
They said they were asked to leave when the doctors arrived to examine Patrick.
CDC officials would not confirm to UPI whether they had investigated the case, but the agency's policy does require examining all suspected cases of vCJD in anyone under 55.
The family also said Loma Linda refused to released Hicks's medical records to them.
The oddities continued after Hicks's death. Bailey found it almost impossible to get an autopsy conducted on Hicks, the only way to determine conclusively whether he had variant or sporadic CJD -- a version of the disease not related to mad cow. One county coroner's office referred him to another and both refused to conduct the procedure, he said.
Then, the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio -- which was established by the CDC to investigate potential vCJD cases in the United States -- dispatched a mobile autopsy company called 1-800-Autopsy, but the company failed to follow the center's protocol and did not collect frozen sections of brain, which are required for tests to determine whether the disease is vCJD or sCJD. Instead, the autopsy company fixed the entire brain in formalin.
The NPDPSC, however, considers the collection of frozen brain tissue essential to distinguishing vCJD from other forms of CJD.
"Only frozen brain tissue examination definitely confirms or excludes the diagnosis of prion disease and provides the information to identify the type of prion disease," the center's Web site says. Prions are abnormal proteins thought to play a role in causing vCJD and sCJD.
The problem raised enough concern that both Bailey and Hicks's family sought a second opinion.
Experts had told them that animal-injection studies could be done with formalin-fixed tissue, so the family arranged to have a sample of Patrick's brain sent to Dr. Jean Jacques Hauw at the Laboratoire De Neuropathologie at the Groupe Hospitalier Pitie-Salpetriere in Paris, who they thought had agreed to do the studies.
The NPDPSC, however, delayed sending the sample to France for two months after the family's request last March. During the delay, Pierluigi Gambetti, the NPDPSC's director, sent a letter to Hicks's wife.
"We can definitely rule out the diagnosis of variant CJD," the letter stated.
Gambetti's strong conclusion sounded strange to Bailey, because the NPDPSC had not conducted further tests since January, when they had said vCJD was unlikely but that they were unable to rule it out entirely.
After examining the brain tissue, Hauw's team told the family the disease was consistent with sCJD, but to date they have not explained why they did not conduct the animal-injection studies -- the family's reason for sending samples of his brain to France.
Asked the reasons for not following the family's wishes and conducting the animal studies, Hauw told UPI, "I cannot answer your question," citing French regulations that prohibited him from providing information about a specific patient.
He did say, however, that "animal injection is not needed for the routine diagnosis of Creutzfeldt-Jakob disease and its various variants, at least in France and in the United Kingdom."
That may be true, but it remains unclear why he accepted the case in the first place, knowing that is what the family wanted.
Moreover, this was not a "routine diagnosis." If Hicks suffered from vCJD, he potentially would have been the first person in the United States to have acquired the disease domestically, a development with significant domestic and international ramifications.
In addition, other experts, such as Dr. Laura Manuelidis, section chief of surgery in the neuropathology department at Yale University, have said the only way to know conclusively whether the disease is due to sCJD or vCJD is through animal-injection studies.
"From what I gather, the result was merely rubber stamped," Bailey told UPI. "I guess we will never really know for sure."
The handling of the case is noteworthy, because the NPDPSC currently is investigating nine potential sCJD cases in Idaho. Experts suspect some of those cases could be vCJD.
Bailey and some patient advocates said they are now skeptical of the NPDPSC's behavior.
"How could my experience with the Hicks case ... and the interaction with NPDPSC not lessen my confidence?" Bailey asked. "I anticipate that all of the Idaho cluster of CJD patients will turn out to have sCJD. I cannot for a minute see their results indicating anything but this. After all, if any patient were to have vCJD, it would have been Patrick Hicks. The results of NPDPSC are not definitive in excluding Hicks as not having vCJD. There certainly will always be that question in my mind."
Terry Singletary, a patient advocate whose mother died of a form of the disease called Heidenhain variant, told UPI he likewise had lost confidence in the NPDPSC.
"I do not trust them," Singletary said. "It's all going to be sporadic. This is the way they want it. They do not want to find out all the routes and sources of this agent."
Both vCJD and mad cow disease are politically sensitive issues because they can impact international trade. Dozens of nations closed their borders to American beef after a lone U.S. cow tested positive for the disease in 2003, resulting in more than $4.7 billion in losses for the industry, and the U.S. Department of Agriculture delayed doing confirmatory tests for seven months on what turned out to be a second case of mad cow.
The NPDPSC did not respond to UPI's phone call requesting comment about the Idaho cases. The CDC referred UPI to Idaho officials.
Of the nine Idaho cases, three people have tested positive for a CJD-like illness, but officials are conducting further tests to determine whether the disease is sCJD. Two others tested negative and four were buried without autopsies.
The cases could just be a statistical fluke, but the state averages about 1.2 sCJD cases per year and has never had more than three in a single year. The disease is rare and generally is thought to occur at the rate of one case per million people.
Several CJD clusters in other states have far exceeded that rate, however. These included:
--southern New Jersey (2000-2003),
--Lehigh, Pa. (1986-90),
--Allentown, Pa. (1989-92),
--Tampa, Fla. (1996-97),
--Oregon (2001-02), and
--Nassau County, N.Y. (1999-2000).
Some of the clusters involved as many as 18 deaths, and ranged from a rate of four to eight cases per million people.
A group of J.P. Morgan analysts issued an advisory last year on the impact the clusters could have on the beef industry, and said that some of the cases could be due to vCJD.
"The existence of clusters raises the question of 'contamination' or 'infection,' and also raises the hypothesis that rather than cases of sCJD, these might have been cases of vCJD," the advisory said. "Given that sCJD occurs randomly in one out of 1 million cases, it is a statistical rarity to find an sCJD cluster -- let alone six."
If that assessment is accurate, another cluster in Idaho would be even more unlikely.
Another possibility is some of the Idaho cases could be due to chronic wasting disease, which is similar to mad cow disease and currently is epidemic among deer and elk in several states, including Idaho's neighbors Wyoming and Utah.
No human cases of CWD have ever been confirmed, but the disease has been shown to infect human cells in a lab dish. Also, a team of researchers led by Jason Bartz of Creighton University in Omaha, Neb., report in the November issue of the Journal of Virology they had experimentally transmitted CWD to squirrel monkeys --the first reported transmission of CWD to primates.
If CWD is capable of infecting humans, it is unknown whether the resulting disease would resemble sCJD, vCJD or a novel disorder. If the disease looks like sCJD, cases could be going undetected or misdiagnosed.
NIH may destroy human brain collection
By Steve Mitchell Medical Correspondent
Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.
see full history of the day they almost destroyed all donated brains samples from CJD victims ;
3.56 A further difference in the transmission properties of the two diseases was the pattern of disease caused in the brains of experimental animals. Mice inoculated with scrapie material from geographically and temporally distinct sources were found to have variable brain lesions, whereas mice inoculated with BSE material similarly derived from different sources all had very similar patterns of disease. 30 These results showed that, unlike scrapie, only one strain of BSE was present in the inocula derived from different sources. As the current hypothesis suggested that scrapie had transmitted to cattle at a number of geographically separate sites, it might have been expected that several strains of BSE would have been evident, given that over 20 strains of scrapie were known. Since 1996, strain-typing studies in mice have shown that the lesion profile produced by BSE is different to all known scrapie strains. 31
3.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.
3.58 There are several possible reasons why the experiment was not performed in the UK. It had been recommended by Sir Richard Southwood (Chairman of the Working Party on Bovine Spongiform Encephalopathy) in his letter to the Permanent Secretary of MAFF, Mr (now Sir) Derek Andrews, on 21 June 1988, 35 though it was not specifically recommended in the Working Party Report or indeed in the Tyrrell Committee Report (details of the Southwood Working Party and the Tyrell Committee can be found in vol. 4: The Southwood Working Party, 1988-89 and vol. 11: Scientists after Southwood respectively). The direct inoculation of scrapie into calves was given low priority, because of its high cost and because it was known that it had already taken place in the USA. 36 It was also felt that the results of such an experiment would be hard to interpret. While a negative result would be informative, a positive result would need to demonstrate that when scrapie was transmitted to cattle, the disease which developed in cattle was the same as BSE. 37 Given the large number of strains of scrapie and the possibility that BSE was one of them, it would be necessary to transmit every scrapie strain to cattle separately, to test the hypothesis properly. Such an experiment would be expensive. Secondly, as measures to control the epidemic took hold, the need for the experiment from the policy viewpoint was not considered so urgent. It was felt that the results would be mainly of academic interest. 38
3.59 Nevertheless, from the first demonstration of transmissibility of BSE in 1988, the possibility of differences in the transmission properties of BSE and scrapie was clear. Scrapie was transmissible to hamsters, but by 1988 attempts to transmit BSE to hamsters had failed. Subsequent findings increased that possibility.
Sunday, April 18, 2010
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010
If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010 ***FINAL***
my comments to PLosone here ;
Friday, February 05, 2010
New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review
Thursday, May 06, 2010
Sporadic Creutzfeldt-Jakob disease mimicking nonconvulsive status epilepticus
Sunday, February 14, 2010
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
Wednesday, February 24, 2010
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 14th
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