Saturday, February 12, 2011

Another Pathologists dies from CJD, another potential occupational death ?

Another Pathologists dies from CJD, another potential occupational death ?

another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???

i will give you 3 guesses what the cdc et al will say, and the first two don't count. ...

sCJD...something from nothing...and that is sad.

our condolences go out to the family and friends of Dr. Anthony Manoukian. ...

with kindest regards,


is much less’ with death of Manoukian

May 21, 1952 — Feb. 6, 2011 February 11, 2011 - By LILA FUJIMOTO, Staff Writer Save

Dr. Anthony Manoukian, Maui County coroner's physician who was known as much for his generosity and humility as his expertise in death investigations, is being mourned by friends, colleagues and family members.

Many remember the island's first forensic-trained pathologist for his expert testimony on autopsies he conducted in connection with murders and other criminal cases in 2nd Circuit Court.

After being diagnosed in November with Creutzfeldt-Jakob disease, Manoukian died Sunday. He was 58.

Fellow pathologist and longtime friend Dr. Barry Shitamoto said Manoukian's loss will be felt keenly by Maui's medical community and beyond.

"Whoever Tony was friends with, they all felt like they were his best friend," Shitamoto said. "Tony had a knack for making people feel that way. He had many best friends."

In 1997, Manoukian served as chief of the medical staff at Maui Memorial Medical Center. He was a passionate supporter of the hospital, serving on various committees, Shitamoto said. And members of his family said Manoukian's favorite charity was the Maui Memorial Medical Center Foundation.

Shitamoto said Manoukian served as a teacher and mentor for high school students and police officers.

"Tony's influence on youth, students, new police officers (and) others was enormous," he said. "There are more than several students that are following their dreams in the forensic sciences due to Tony's mentorship. His professional legacy may live through these professionals and serve our Hawaii communities."

Another longtime friend, attorney Tony Takitani, said he met Manoukian more than a dozen years ago when, while he was working for a client, he had a conversation with him about an autopsy.

He said Manoukian was an "impressive guy," and he felt nervous talking with him.

But Takitani's uneasy feeling quickly evaporated when he found how personable Manoukian was.

"He was the nicest, easiest guy to talk to," he recalled. "He didn't talk down to anybody. He could explain things in a simple, clear way. ... You'd never guess he's one of the most capable professionals in America.

"So many people felt like he was their best friend," he said.

According to a biography provided by his family, Manoukian was born May 21, 1952, in Chicago, where he started a lifelong love affair with the Cubs. In 1960, the family moved to the San Francisco Bay area, and Manoukian eventually attended the University of California at Davis, earning a bachelor of science degree in zoology in 1974.

He received his graduate degree in public health from the University of Minnesota in 1983 and his medical degree in 1987 from St. George's University School of Medicine in Grenada, West Indies.

"Tony excelled in anatomy, becoming a teaching assistant and preparing tissue specimens that would be used to train future students," his family's website said.

His studies in Grenada were interrupted by the U.S. invasion of the island in October 1983. He was evacuated with other students but later returned to complete his medical degree.

Following a pathology residency at the Kaiser hospital in Honolulu and a one-year forensic pathology fellowship in Baltimore, Manoukian came to Maui in the early 1990s to work as one of the few forensic pathologists in the Pacific basin.

A doctor in general practice had been doing autopsies on Maui. But after the state Legislature passed a law requiring that autopsies be done by a pathologist, Manoukian became the first permanent resident forensic pathologist for Maui County. He also did autopsies on Kauai and the Big Island and in other parts of the Pacific.

As assistant to the medical examiner, Burt Freeland worked with Manoukian for 18 years, doing about 200 autopsies a year.

A few years ago, the two hiked to the top of a mountain in Waihee to perform an autopsy on a body that was too fragile to move, Freeland recalled. They had to make sure the man hadn't been shot or otherwise met with foul play. The man, a military deserter, had been camping in the area before running out of food and living off the land, Freeland said. He said an investigation determined that the man may have poisoned himself.

Manoukian worked with the Hawaii Disaster Medical Assistance Team and the Federal Disaster Mortuary Operational Response Team to provide assistance after disasters, including a tsunami in Samoa, Hurricane Katrina and the earthquake in Haiti.

Freeland, who is also part of the federal mortuary team, said volunteers in those situations sometimes must live in tents.

"You've got to be able to really rough it and get used to eating MREs when they don't have kitchen facilities," he said. "Even though you're not doing armed combat, it gets a little rough. But he and I both enjoyed serving our country that way."

Manoukian was deputy commander of Region 9 of the Federal Disaster Mortuary Operational Response Team.

"We were pretty close friends," Freeland said. "We had good times. He was a lot of fun.

"Anybody can become a great pathologist, but Dr. Manoukian wasn't known to be just in the morgue. He got out in the community and did a lot of things.

"I just remember him as a really happy guy, and all of a sudden he's gone. He'll be missed."

Acting Maui County Prosecuting Attorney John D. Kim said attorneys in the office worked closely with Manoukian on many cases.

"He was just the best," Kim said. "He knew everything, and he explained it so we could explain it in layman's terms. He was always there. He'd give you his direct line when nobody else would.

"It's a very big loss for the county."

Manoukian testified about his autopsy findings at many murder trials, including those of convicted double-murderer Daniel Kosi in 1999 and Michael Arlo Pavich in 2005 for strangling to death an 82-year-old Kihei man.

"He was able to speak for the deceased or the victims and tell their story," said First Deputy Prosecuting Attorney Robert Rivera, who handled many of the high-profile cases during Manoukian's tenure. "That's how we got a lot of the evidence.

"The thing that stood out about Dr. Manoukian was he explained it in a way that the layperson could understand."

Rivera said jurors he talked to after trials would mention how Manoukian's testimony was "extremely helpful and insightful."

"He captivated them," Rivera said. "It made it easier for them to come up with a decision.

"He had his quirkiness and his long hair. That just endeared him even more to the jury. He wasn't stuffy."

For a time, Manoukian was owner of Molina's bar in Wailuku. He purchased the business in 2000 before later selling it.

"He was a proud bar owner," said Rivera, who was a deputy corporation counsel when the liquor board reviewed the matter.

Rivera said he appreciated how Manoukian would make himself available to testify in cases, even when hearings were delayed and he would have to return to court more than once.

"Although he was the expert, he would come back," Rivera said. "He's one of the nicer persons."

When he headed the Maui police Traffic Section, retired Capt. Charles Hirata worked closely with Manoukian in the investigation of traffic deaths.

"I enjoyed his friendship both on and off the job," Hirata said. "He's one of the few that took the time to teach others as he went about his job. I pass on some of his lessons when I teach car seat safety. We actually learn a lot from the dead, which helps us to protect the living."

Manoukian was instrumental in planning a new forensic facility that will move the county morgue out of its cramped quarters, said police Assistant Chief Larry Hudson.

Manoukian was there for the blessing of the new facility Dec. 28.

"In dealing with him, he was humble," Hudson said. "He would talk to anybody. He didn't care about where you were in life. He treated everybody exactly the same.

"He was a super intelligent man who would listen, who was compassionate. He was just a likable guy.

"He was a good man. The community is much less because he's gone."

According to his family, beginning last autumn, Manoukian had difficulty speaking and needed to type his pathology reports by hand instead of dictating them. Later, he was diagnosed with Creutzfeldt-Jakob disease, a rapidly progressive and incurable neurological disorder.

Jerry Manoukian, an internal medicine physician in Mountain View, Calif., said family members don't know how his brother became infected with the illness, although it's possible he was exposed to it through his work.

The disease is rare, with about 300 cases reported in the United States each year, or about 1 in a million, he said.

In a day or two, family and friends plan to gather in California to have a barbecue and party in Manoukian's honor - no service, just friends and good food, his brother said.

"We'll mostly enjoy ourselves and remember him," he said. "It's very hard . . . It's very hard being without him."

Jerry Manoukian said he'd like to hold a similar event on Maui for family members to be with his brother's friends and colleagues here. But it was undecided when that might be.

Manoukian is survived by his wife, Downey, and brothers Pete, Jerry and Phil.

In a letter to the Maui County Council dated Thursday, Maui County Police Chief Gary Yabuta asked that Manoukian be recognized posthumously for commitment to his work in death investigations.

"What was further remarkable about the character of Dr. Manoukian was his ability to interact with every officer and employee of the Maui Police Department," Yabuta said. "He was a true friend to all of us.

"Through his professional findings, the families of the deceased were provided with personal closure. And, in the case of a criminal homicide, we were all given a sense of satisfaction that justice was served through the methodology and skills of Dr. Anthony Manoukian."

* Lila Fujimoto can be reached at City Editor Brian Perry contributed to this report.

Pathologist dies of suspected Creutzfeldt-Jakob (Mad cow) disease 29 March, 2009 03:33:00

Creutzfeldt-Jakob disease (mad cow) research pathologist Antonio Ruiz Villaescusa died Sat., March 28, from the disease. Colleagues suspect he may have contracted the disease from exposure to infected human tissue, according to the Barcelona Reporter newspaper.

Ruiz headed the department of pathology at the University Hospital in Madrid and was studying whether the disease is passed on to people who have been exposed to infected tissue.

The head of the pathology department at the Fundación Alcorcón, Dr. Radish, will perform an autopsy to clarify the cause of death and the final results will be announced in about a month, according to the Spanish news site.

Ruiz was recognized internationally for his study in the fields of neuropathology and anatomopatología, and devoted much of his professional life to the study of human transmissible spongiform encephalopathy.

Creutzfeldt-Jakob disease is a rare and invariably fatal brain disorder, according to the National Institute of Neurological Disorders and Stroke. There is currently no single diagnostic test for the disease. The only way to confirm a Creutzfeldt-Jakob disease diagnosis is by brain biopsy or autopsy.

e-mail: e-mail:

Dr. Alberto Rábano Gutiérrez. Fundación Hospital Alcorcón. Madrid

Monday, March 29, 2010



>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

Monday, March 29, 2010



>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas.

She left 6 Kids and a Husband.The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.- Physician Discharge Summary, Parkland Hospital, Dallas Texas Admit Date: 12/29/2009 Discharge Date: 1/20/2010 Attending Provider: Greenberg, Benjamin Morris; General Neurology Team: General Neurology Team Linda was a Hispanic female with no past medical history presents with 14 months of incresing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/blader incontinence. She was, in her usual state of health up until February, 2009, when her husbans notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA,although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers. The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically. Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.

Monday, March 29, 2010 Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas

Monday, February 7, 2011

FDA's Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???

Wednesday, February 2, 2011

Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay

Monday, January 17, 2011

Aerosols Transmit Prions to Immunocompetent and Immunodeficient Mice

Tuesday, December 14, 2010

Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J, UPDATE DECEMBER 2010

Thursday, September 02, 2010

NEUROSURGERY AND CREUTZFELDT-JAKOB DISEASE Health Law, Ethics, and Human Rights The Disclosure Dilemma

Thursday, July 08, 2010

Nosocomial transmission of sporadic Creutzfeldt–Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

Thursday, July 08, 2010


Wednesday, June 02, 2010

CJD Annex H UPDATE AFTER DEATH PRECAUTIONS Published: 2 June 2003 Updated: May 2010

Tuesday, May 11, 2010

Current risk of iatrogenic Creutzfeld–Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments

Tuesday, March 16, 2010

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Part 4 REVISED FEB. 2010

Saturday, January 16, 2010

Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al

Monday, July 20, 2009

Pre-surgical risk assessment for variant Creutzfeldt-Jakob disease (vCJD) risk in neurosurgery and eye surgery units

[2] UK: SEAC position statement on dentistry Date: Sat 30 Jun 2007 Source: Position Statement vCJD and Dentistry, Spongiform Encephalopathy Advisory Committee (SEAC) Update, June 2007 [edited]

Position Statement vCJD and Dentistry ------------------------------------- Issue ----- 1. The Department of Health (DH) asked SEAC to advise on the findings of preliminary research aimed at informing estimates of the risk of variant Creutzfeldt-Jakob Disease (vCJD) transmission via dentistry.

Background ---------- 2. Prions are more resistant than other types of infectious agents to the conventional cleaning and sterilization practices used to decontaminate dental instruments (1). Appreciable quantities of residual material may remain adherent to the surface after normal cleaning and sterilization (2). Therefore, if dental tissues are both infectious and susceptible to infection, the dental instruments are a potential mechanism for the secondary transmission of vCJD. Dentistry could be a particularly significant route of transmission for the population as a whole, due to the large number of routine procedures undertaken and also because dental patients have a normal life expectancy.

This is in contrast with other transmission routes, such as blood transfusion and neurosurgery, where procedures are often carried out in response to some life-threatening condition. Additionally, the ubiquity of dental procedures and the lack of central records on dental procedures means that should such transmission occur, then it would be difficult to detect and control.

3. No cases of vCJD transmission arising from dental procedures have been reported to date (3). Previous DH risk assessments (4,5) have focused on 2 possible mechanisms for the transfer of vCJD infectivity via dental instruments; accidental abrasion of the lingual tonsil and endodontic procedures that involve contact with dental pulp. In considering these assessments, SEAC agreed that the risk of transmission via accidental abrasion of the lingual tonsil appears very low. However, the risk of transmission via endodontic procedures may be higher and give rise to a self sustaining vCJD epidemic under circumstances where (i) dental pulp is infective, (ii) transmission via endodontic instruments is efficient and (iii) a large proportion of vCJD infections remain in a subclinical carrier state (SEAC 91, February 2006). In light of this, SEAC advised that restricting endodontic files and reamers to single use be considered (6). SEAC recommended reassessment of these issues as new data emerge.

New research ------------ 4. Preliminary, unpublished results of research from the Health Protection Agency, aimed at addressing some of the uncertainties in the risk assessments, were reviewed by SEAC (SEAC 97, May 2007). The prion agent used in these studies is closely related to the vCJD agent. This research, using a mouse model, shows that following inoculation of mouse-adapted bovine spongiform encephalopathy (BSE) directly into the gut, infectivity subsequently becomes widespread in tissues of the oral cavity, including dental pulp, salivary glands and gingiva, during the preclinical as well as clinical stage of disease.

5. It is not known how closely the level and distribution of infectivity in the oral cavity of infected mice reflects those of humans infected with vCJD, as there are no comparable data from oral tissues, in particular dental pulp and gingiva, from human subclinical or clinical vCJD cases (7). Although no abnormal prion protein was found in a study of human dental tissues, including dental pulp, salivary glands and gingiva from vCJD cases, the relationship between levels of infectivity and abnormal prion protein is unclear (8). Infectivity studies underway using the mouse model and oral tissues that are presently available from human vCJD cases will provide some comparable data. On the basis of what is currently known, there is no reason to suppose that the mouse is not a good model for humans in respect to the distribution of infectivity in oral tissues. Furthermore, the new data are consistent with published results from experiments using a hamster scrapie model (9).

6. A 2nd set of experiments using the same mouse model showed that non-invasive and transient contact between gingival tissue and fine dental files contaminated with mouse-adapted BSE brain homogenate transmits infection very efficiently. It is not known how efficient gingival transmission would be if dental files were contaminated with infectious oral tissues and then subsequently cleaned and sterilized, a situation which would more closely model human dental practice. Further studies using the mouse model that would be more representative of the human situation, comparing oral tissues with a range of doses of infectivity, cleaned and sterilized files and the kind of tissue contact with instruments that occurs during dentistry, should be considered.

7. SEAC considered that the experiments appear well designed and the conclusions justified and reliable, while recognizing that the research is incomplete and confirmatory experiments have yet to be completed. It is recommended that the research be completed, submitted for peer-review and widely disseminated as soon as possible so others can consider the implications. Nevertheless, these preliminary data increase the possibility that some oral tissues of humans infected with vCJD may potentially become infective during the preclinical stage of the disease. In addition, they increase the possibility that infection could potentially be transmitted not only via accidental abrasion of the lingual tonsil or endodontic procedures but a variety of routine dental procedures.

Implications for transmission risks ----------------------------------- 8. The new findings help refine assumptions made about the level of infectivity of dental pulp and the stage of incubation period when it becomes infective in the risk assessment of vCJD transmission from the reuse of endodontic files and reamers (10). For example, if one patient in 10 000 were to be carrying infection (equivalent to about 6000 people across the UK, the best current estimate (11), the data suggest that in the worst case scenario envisaged in the risk assessment, reuse of endodontic files and reamers might lead to up to 150 new infections per annum. It is not known how many of those infected would go on to develop clinical vCJD. In addition, transmission via the reuse of endodontic files and reamers could be sufficiently efficient to cause a self-sustaining vCJD epidemic arising via this route.

9. These results increase the importance of obtaining reliable estimates of vCJD infection prevalence. Data that will soon be available from the National Anonymous Tonsil Archive may help refine this assessment and provide evidence of the existence and extent of subclinical vCJD infection in tonsillectomy patients. Further data, such as from post mortem tissue or blood donations, will be required to assess prevalence in the general UK population (12).

10. Recent guidance issued by DH to dentists to ensure that endodontic files and reamers are treated as single use (13) is welcomed and should, as long as it is effectively and quickly implemented, prevent transmission and a self-sustaining epidemic arising via this route. However, the extent and monitoring of compliance with this guidance in private and National Health Service dental practice is unclear.

11. The new research also suggests that dental procedures involving contact with other oral tissues, including gingiva, may also be capable of transmitting vCJD. In the absence of a detailed risk assessment examining the potential for transmission via all dental procedures, it is not possible to come to firm conclusions about the implications of these findings for transmission of vCJD. However, given the potential for transmission by this route, serious consideration should be given to assessing the options for reducing transmission risks, such as improving decontamination procedures and practice or the implementation of single use instruments.

12. The size of the potential risk from interactions between the dental and other routes of secondary transmission, such as blood transfusion and hospital surgery, to increase the likelihood of a self-sustaining epidemic is unclear.

13. It is likely to be difficult to distinguish clinical vCJD cases arising from dietary exposure to BSE from secondary transmissions via dental procedures, should they arise, as a large proportion of the population is likely both to have consumed contaminated meat and undergone dentistry.

However, an analysis of dental procedures by patient age may provide an indication of the age group in which infections, if they occur, would be most likely to be observed. Should the incidence of clinical vCJD cases in this age group increase significantly, this may provide an indication that secondary transmission via dentistry is occurring. Investigation of the dental work for these cases may provide supporting data. There is no clear evidence, to date, based on surveillance or investigations of clinical vCJD cases, that any vCJD cases have been caused by dental procedures, but this possibility cannot be excluded.

Conclusions ----------- 14. Preliminary research findings suggest that the potential risk of transmission of vCJD via dental procedures may be greater than previously anticipated. Although this research is incomplete, uses an animal model exposed to relatively high doses of infectivity, and there are no data from infectivity studies on human oral tissues, these findings suggest an increased possibility that vCJD may be relatively efficiently transmitted via a range of dental procedures. Ongoing infectivity studies using human oral tissues and the other studies suggested here will enable more precise assessment of the risks of vCJD transmission through dental procedures.

15. Guidance was issued to dentists earlier this year [2007] recommending that endodontic files and reamers be treated as single use, which, provided this policy is adhered to, will remove any risk of a self-sustaining epidemic arising from reuse of these instruments. To minimize risk, it is critical that appropriate management and audit is in place, both for NHS and private dentistry.

16. It is also critical that a detailed and comprehensive assessment of the risks of all dental procedures be conducted as a matter of urgency. While taking into account the continuing scientific uncertainties, this will allow a more thorough consideration of the possible public health implications of vCJD transmission via dentistry and the identification of possible additional precautionary risk reduction measures. The assessment will require continued updating as more evidence becomes available on the transmissibility of vCJD by dental routes, and on the prevalence of infection within the population. A DH proposal to convene an expert group that includes dental professionals to expedite such an assessment is welcomed. Given the potential for transmission via dentistry, consideration should be given to the urgent assessment of new decontamination technologies which, if proven robust and effective, could significantly reduce transmission risks.

References ---------- (1) Smith et al. (2003) Prions and the oral cavity. J. Dent. Res. 82, 769-775. (2) Smith et al. (2005) Residual protein levels on reprocessed dental instruments. J. Hosp. Infect. 61, 237-241. (3) Everington et al. (2007) Dental treatment and risk of variant CJD - a case control study. Brit. Den. J. 202, 1-3. (4) Department of Health. (2003) Risk assessment for vCJD and dentistry. (5) Department of Health (2006) Dentistry and vCJD: the implications of a carrier-state for a self-sustaining epidemic. Unpublished. (6) SEAC (2006) Position statement on vCJD and endodontic dentistry . (7) Head et al. (2003) Investigation of PrPres in dental tissues in variant CJD. Br. Dent. J. 195, 339-343. (8) SEAC 90 reserved business minutes. (9) Ingrosso et al. (1999) Transmission of the 263K scrapie strain by the dental route. J. Gen. Virol. 80, 3043-3047. (10) Department of Health (2006) Dentistry and vCJD: the implications of a carrier-state for a self-sustaining epidemic. Unpublished. (11) Clarke & Ghani (2005) Projections of future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility R. J. Soc. Interface. 2, 19-31. (12) SEAC Epidemiology Subgroup (2006) position statement of the vCJD epidemic . (13) DH (2007) Precautionary advice given to dentists on re-use of instruments .

-- Communicated by Terry S. Singletary, Sr.


Monday, December 31, 2007

Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation

Subject: CJD: update for dental staff Date: November 12, 2006 at 3:25 pm PST

1: Dent Update. 2006 Oct;33(8):454-6, 458-60.

CJD: update for dental staff.

Friday, July 17, 2009

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009

Tuesday, August 12, 2008

Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)

Thursday, January 29, 2009

Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research

Wednesday, August 20, 2008 Tonometer disinfection practice in the United Kingdom: A national survey

Tuesday, August 12, 2008 Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)

Tuesday, May 04, 2010

Review of the Human Pituitary Trust Account and CJD Issue 20 January 2010

Monday, May 19, 2008


Friday, February 11, 2011

Creutzfeldt-Jakob disease (CJD) biannual update (2010/1) Emerging infections/CJD

Friday, February 19, 2010 Creutzfeldt-Jakob disease (CJD) biannual update (2010/1)

Monday, February 7, 2011

FDA's Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???

Saturday, January 29, 2011

Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to Cynomolgus Macaques, a Non-Human Primate

Jpn. J. Infect. Dis., 64 (1), 81-84, 2011

Thursday, February 10, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31

Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues


Wednesday, September 08, 2010


Tuesday, January 25, 2011

Generation of a new form of human PrPSc in vitro by inter-species transmission from cervids prions

Journal of Virology, September 2009, p. 9608-9610, Vol. 83, No. 18 0022-538X/09/$08.00+0 doi:10.1128/JVI.01127-09 Copyright © 2009, American Society for Microbiology. All Rights Reserved.

Prion Infectivity in Fat of Deer with Chronic Wasting Disease

Brent Race,# Kimberly Meade-White,# Richard Race, and Bruce Chesebro* Rocky Mountain Laboratories, 903 South 4th Street, Hamilton, Montana 59840

Received 2 June 2009/ Accepted 24 June 2009


Chronic wasting disease (CWD) is a neurodegenerative prion disease of cervids. Some animal prion diseases, such as bovine spongiform encephalopathy, can infect humans; however, human susceptibility to CWD is unknown. In ruminants, prion infectivity is found in central nervous system and lymphoid tissues, with smaller amounts in intestine and muscle. In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.


The highest risk of human contact with CWD might be through exposure to high-titer CNS tissue through accidental skin cuts or corneal contact at the time of harvest and butchering. However, the likelihood of a human consuming fat infected with a low titer of the CWD agent is much higher. It is impossible to remove all the fat present within muscle tissue, and fat consumption is inevitable when eating meat. Of additional concern is the fact that meat from an individual deer harvested by a hunter is typically consumed over multiple meals by the same group of people. These individuals would thus have multiple exposures to the CWD agent over time, which might increase the chance for transfer of infection.

In the Rocky Mountain region of North America, wild deer are subject to predation by wolves, coyotes, bears, and mountain lions. Although canines such as wolves and coyotes are not known to be susceptible to prion diseases, felines definitely are susceptible to BSE (9) and might also be infected by the CWD agent. Deer infected with the CWD agent are more likely to be killed by predators such as mountain lions (11). Peripheral tissues, including lymph nodes, muscle, and fat, which harbor prion infectivity are more accessible for consumption than CNS tissue, which has the highest level of infectivity late in disease. Therefore, infectivity in these peripheral tissues may be important in potential cross-species CWD transmissions in the wild.

The present finding of CWD infectivity in deer fat tissue raises the possibility that prion infectivity might also be found in fat tissue of other infected ruminants, such as sheep and cattle, whose fat and muscle tissues are more widely distributed in both the human and domestic-animal food chains. Although the infectivity in fat tissues is low compared to that in the CNS, there may be significant differences among species and between prion strains. Two fat samples from BSE agent-infected cattle were reported to be negative by bioassay in nontransgenic RIII mice (3, 6). However, RIII mice are 10,000-fold-less sensitive to BSE agent infection than transgenic mice expressing bovine PrP (4). It would be prudent to carry out additional infectivity assays on fat from BSE agent-infected cattle and scrapie agent-infected sheep using appropriate transgenic mice or homologous species to determine the risk from these sources.

Our results have far-reaching implications for human health, since they indicate that cervid PrPSc can trigger the conversion of human PrPC into PrPSc, suggesting that CWD might be infectious to humans. Interestingly our findings suggest that unstable strains from CWD affected animals might not be a problem for humans, but upon strain stabilization by successive passages in the wild, this disease might become progressively more transmissible to man.

Reference List


please see full text and many thanks to the Professor Soto and the other Authors of this study AND to The Journal Of Biological Chemistry for the free full text !!!


there are now two documented strains of CWD, and science is showing that indeed CWD could transmit to humans via transmission studies ;



Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2 Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary Research Institute, 4.Center for Prions and Protein Folding Diseases, 5 Department of Biological Sciences, University of Alberta, Edmonton AB, Canada T6G 2P5

The identification and characterization of prion strains is increasingly important for the diagnosis and biological definition of these infectious pathogens. Although well-established in scrapie and, more recently, in BSE, comparatively little is known about the possibility of prion strains in chronic wasting disease (CWD), a disease affecting free ranging and captive cervids, primarily in North America. We have identified prion protein variants in the white-tailed deer population and demonstrated that Prnp genotype affects the susceptibility/disease progression of white-tailed deer to CWD agent. The existence of cervid prion protein variants raises the likelihood of distinct CWD strains. Small rodent models are a useful means of identifying prion strains. We intracerebrally inoculated hamsters with brain homogenates and phosphotungstate concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD endemic area) and experimentally infected deer of known Prnp genotypes. These transmission studies resulted in clinical presentation in primary passage of concentrated CWD prions. Subclinical infection was established with the other primary passages based on the detection of PrPCWD in the brains of hamsters and the successful disease transmission upon second passage. Second and third passage data, when compared to transmission studies using different CWD inocula (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin white-tailed deer population is different than the strain(s) present in elk, mule-deer and white-tailed deer from the western United States endemic region.


Prion Transmission from Cervids to Humans is Strain-dependent

Qingzhong Kong, Shenghai Huang,*Fusong Chen, Michael Payne, Pierluigi Gambetti and Liuting Qing Department of Pathology; Case western Reserve University; Cleveland, OH USA *Current address: Nursing Informatics; Memorial Sloan-Kettering Cancer Center; New York, NY USA

Key words: CWD, strain, human transmission

Chronic wasting disease (CWD) is a widespread prion disease in cervids (deer and elk) in North America where significant human exposure to CWD is likely and zoonotic transmission of CWD is a concern. Current evidence indicates a strong barrier for transmission of the classical CWD strain to humans with the PrP-129MM genotype. A few recent reports suggest the presence of two or more CWD strains. What remain unknown is whether individuals with the PrP-129VV/MV genotypes are also resistant to the classical CWD strain and whether humans are resistant to all natural or adapted cervid prion strains. Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP, indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains. Preliminary results on CWD transmission in transgenic mice expressing human PrP-129V will also be discussed.

Acknowledgement Supported by NINDS NS052319 and NIA AG14359.


Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions

Marcelo A. Barria,1 Glenn C. Telling,2 Pierluigi Gambetti,3 James A. Mastrianni4 and Claudio Soto1 1Mitchell Center for Alzheimer's disease and related Brain disorders; Dept of Neurology; University of Texas Houston Medical School; Houston, TX USA; 2Dept of Microbiology, Immunology & Molecular Genetics and Neurology; Sanders Brown Center on Aging; University of Kentucky Medical Center; Lexington, KY USA; 3Institute of Pathology; Case western Reserve University; Cleveland, OH USA; 4Dept of Neurology; University of Chicago; Chicago, IL USA

Prion diseases are infectious neurodegenerative disorders affecting humans and animals that result from the conversion of normal prion protein (PrPC) into the misfolded and infectious prion (PrPSc). Chronic wasting disease (CWD) of cervids is a prion disorder of increasing prevalence within the United States that affects a large population of wild and captive deer and elk. CWD is highly contagious and its origin, mechanism of transmission and exact prevalence are currently unclear. The risk of transmission of CWD to humans is unknown. Defining that risk is of utmost importance, considering that people have been infected by animal prions, resulting in new fatal diseases. To study the possibility that human PrPC can be converted into the infectious form by CWD PrPSc we performed experiments using the Protein Misfolding Cyclic Amplification (PMCA) technique, which mimic in vitro the process of prion replication. Our results show that cervid PrPSc can induce the pathological conversion of human PrPC, but only after the CWD prion strain has been stabilized by successive passages in vitro or in vivo. Interestingly, this newly generated human PrPSc exhibits a distinct biochemical pattern that differs from any of the currently known forms of human PrPSc, indicating that it corresponds to a novel human prion strain. Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.


Biochemical and Biophysical Characterization of Different CWD Isolates

Martin L. Daus and Michael Beekes Robert Koch Institute; Berlin, Germany

Key words: CWD, strains, FT-IR, AFM

Chronic wasting disease (CWD) is one of three naturally occurring forms of prion disease. The other two are Creutzfeldt-Jakob disease in humans and scrapie in sheep. CWD is contagious and affects captive as well as free ranging cervids. As long as there is no definite answer of whether CWD can breach the species barrier to humans precautionary measures especially for the protection of consumers need to be considered. In principle, different strains of CWD may be associated with different risks of transmission to humans. Sophisticated strain differentiation as accomplished for other prion diseases has not yet been established for CWD. However, several different findings indicate that there exists more than one strain of CWD agent in cervids. We have analysed a set of CWD isolates from white-tailed deer and could detect at least two biochemically different forms of disease-associated prion protein PrPTSE. Limited proteolysis with different concentrations of proteinase K and/or after exposure of PrPTSE to different pH-values or concentrations of Guanidinium hydrochloride resulted in distinct isolate-specific digestion patterns. Our CWD isolates were also examined in protein misfolding cyclic amplification studies. This showed different conversion activities for those isolates that had displayed significantly different sensitivities to limited proteolysis by PK in the biochemical experiments described above. We further applied Fourier transform infrared spectroscopy in combination with atomic force microscopy. This confirmed structural differences in the PrPTSE of at least two disinct CWD isolates. The data presented here substantiate and expand previous reports on the existence of different CWD strains.


Wednesday, September 08, 2010


Friday, February 11, 2011

AN EPIDEMIOLOGIC CRITIQUE OF CREUTZFELDT-JAKOB DISEASE Vol. 2, 1980 Paul Brown vs Zohreh Davanipour and Scrapie



Friday, November 30, 2007


Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518