Thursday, September 02, 2010

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

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


The most vexing large-scale adverse events involve potential injuries that cannot be definitively diagnosed and have no treatment. A patient who underwent neurosurgery in Denver was found several weeks later at autopsy to have died of classic sporadic Creutzfeldt-Jakob disease. This diagnosis was not suspected at the time of surgery. Instruments used in this patient’s surgery were later used in surgery involving six other patients.17

Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy, a rare prion disease that causes rapid, fatal progression of neurologic symptoms. Iatrogenic transmission of Creutzfeldt-Jakob disease has been associated with neurosurgical procedures and corneal transplantation.18,56 The incubation period for surgical exposure to Creutzfeldt-Jakob disease ranges from 6 months to 20 years or more.17,57 Once symptoms appear, patients die within about 1 year.58 Creutzfeldt-Jakob disease is diagnosed by postmortem examination of the brain. Estimates of risk range from 1 in 100,000 to 1 in 1 million.58 Iatrogenic transmission is estimated to account for less than 1% of cases of transmissible spongiform encephalopathy.12 Prevention of surgical transmission of Creutzfeldt-Jakob disease is possible but cumbersome and costly. Although normal disinfection methods are ineffective against prions, available guidelines for infection control involve the sequestering, incinerating, or high-intensity sterilizing of neurosurgical instruments if transmissible spongiform encephalopathy is suspected.18,59

This event is among the most rare and challenging type of large-scale adverse event, since it is difficult to know whether any harm has occurred until decades after the exposure. Thus, a duty to tell the truth might be outweighed by a duty of nonmaleficence. The fear and worry that could accompany disclosure may constitute a greater and more permanent harm in the case of Creutzfeldt-Jakob disease than in other large-scale adverse events without any corresponding benefit, given the remote chance of transmission of this disease and the lack of diagnostic and treatment options.

The rationale for disclosure is also compelling. There is a professional duty to disclose because contracting the disease, even decades later, is a severe, deadly harm. Arguably, a patient has a right to this information. Should Creutzfeldt-Jakob disease develop in the patient, the harm is intensified by the sense of betrayal if the patient learns that providers have kept the risk of the development of a terminal, incurable disease a secret. If a potentially infected patient has subsequent neurosurgery, additional patients may be at risk for exposure, thereby amplifying harm. Disclosure would also allow patients to access testing and treatment that become available in the future,16 and it would demonstrate to the community that the hospital puts the interests of its patients first, even if the benefits to the patient are small and there is a risk of litigation.

Thus, when the institution has no testing or treatment to offer patients and the disclosure is associated with a risk of creating clinically significant long-term harm, a true ethical dilemma exists. Although, on balance, we believe that disclosure is often warranted, there is also greater ethical justification for nondisclosure in this case than in the other types of large-scale adverse events. The challenge of disclosing large-scale adverse events related to prion diseases is an important topic for further analysis.



Some large-scale adverse events are caused by deviations from standards of practice,31 but often they are not. Perhaps the most common large-scale adverse events involve insufficient disinfection of equipment. At the University of Washington Medical Center, one step in a six-step endoscope disinfection process failed. The faulty machine was running several minutes too fast. Two months later, when the malfunction was detected, the hospital corrected the flaw and identified approximately 600 patients who were exposed to incompletely cleaned endoscopes.11 Scientists could not calculate the increased risk posed by omitting one cleaning step, but they thought it was remote and indistinguishable from the baseline risk of contracting bloodborne pathogens from an endoscope (estimated to be 1 in 1.8 million).54,55 Despite the extremely low risk of infection, the hospital considered the rationale for disclosure to be compelling and sent letters to all affected patients. The event was reported in a front-page story in the Seattle Times.11

The hospital devoted considerable time and resources to developing and implementing a process for following up on the disclosure. It enlisted its organizational ethics consultants and patient relations department, set up a hotline to answer concerned patients’ questions, referred interested patients to physicians, and provided free follow-up testing. No cases of infection with bloodborne pathogens were identified and no lawsuits were filed.


see full text ;

Health Law, Ethics, and Human Rights The Disclosure Dilemma — Large-Scale Adverse Events

NEJM September 1, 2010 Topics: Medical Ethics Denise M. Dudzinski, Ph.D., Philip C. Hébert, M.D., Ph.D., Mary Beth Foglia, R.N., Ph.D., and Thomas H. Gallagher, M.D.

IN my opinion, there should be NO Disclosure Dilemma when it comes to a transmissible disease as with CJD. The ethical reasons of health discloser law should NOT have anything to do with an adverse event that may harm many more from further transmission from the 1st adverse event, from a disease that if risk factor is not validated, the risk from further infectivity of exposure from the friendly fire and or pass it forward modes of transmission and possible death is very real. IT should be _law_ to inform those others that are _adversely_ exposed, and possible death there from. Hiding behind confidentiality laws on any adverse events (negligence), just to hide this adverse event from the public and media should be illegal. IF not, and they go on to have other medical procedures in the years and or decades to come, how many more will become exposed, and possibly die there from ??? THEN who's responsibility will it be for those further infected ??? IF not reported, no one, and that's the whole purpose. kinda like the SSS of the USDA et al, i.e. shoot, shovel, and shut the hell up. ...just my opinion...TSS

Friday, August 13, 2010

Creutzfeldt-Jakob disease (CJD) biannual update 13 August 2010 UK Iatrogenic CJD Incidents Report

Thursday, August 12, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

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

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 Evidence For CJD/TSE Transmission Via Endoscopes

From Terry S. Singletary, Sr


Friday, November 20, 2009

SaBTO Advisory Committee on the Safety of Blood, Tissues and Organs Summary of the Eighth Meeting, 27 October 2009

Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

Friday, July 24, 2009

UW Hospital and Clinics Addresses Creutzfeldt-Jakob Disease Risk

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

Monday, August 17, 2009

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009

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)

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

Wednesday, July 28, 2010

re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

Wednesday, March 31, 2010

Atypical BSE in Cattle

Friday, August 27, 2010


Tuesday, August 03, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

Monday, August 9, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

Wednesday, August 18, 2010

Incidence of CJD Deaths Reported by CJD-SS in Canada as of July 31, 2010

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)


>>> 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. <<<

>>> 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. <<<


Thursday, July 08, 2010


Thursday, August 19, 2010


Thursday, August 19, 2010

SCRAPIE CANADA UPDATE Current as of 2010-07-31 The following table lists sheep flocks and/or goat herds confirmed to be infected with scrapie in Canada in 2010.

Current as of: 2010-07-31

Friday, May 14, 2010

Prion Strain Mutation Determined by Prion Protein Conformational Compatibility and Primary Structure

Published Online May 13, 2010 Science DOI: 10.1126/science.1187107 Science Express Index

Thursday, June 03, 2010

Prion Strain Mutation and Selection John Collinge MEDICINE



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.


Susceptibility of Cattle to First-passage Intracerebral Inoculation with Chronic Wasting Disease Agent from White-tailed Deer

A. N. Hamir1, J. M. Miller1, R. A. Kunkle1, S. M. Hall2 and J. A. Richt1 + Author Affiliations

1National Animal Disease Center, ARS, USDA, Ames, IA 2Pathobiology Laboratory, National Veterinary Services Laboratories, Ames, IA Dr. A. N. Hamir, National Animal Disease Center, ARS, USDA, 2300 Dayton Avenue, PO Box 70, Ames, IA 50010 (USA). E-mail: Abstract Fourteen, 3-month-old calves were intracerebrally inoculated with the agent of chronic wasting disease (CWD) from white-tailed deer (CWDwtd) to compare the clinical signs and neuropathologic findings with those of certain other transmissible spongiform encephalopathies (TSE, prion diseases) that have been shown to be experimentally transmissible to cattle (sheep scrapie, CWD of mule deer [CWDmd], bovine spongiform encephalopathy [BSE], and transmissible mink encephalopathy). Two uninoculated calves served as controls. Within 26 months postinoculation (MPI), 12 inoculated calves had lost considerable weight and eventually became recumbent. Of the 12 inoculated calves, 11 (92%) developed clinical signs. Although spongiform encephalopathy (SE) was not observed, abnormal prion protein (PrPd) was detected by immunohistochemistry (IHC) and Western blot (WB) in central nervous system tissues. The absence of SE with presence of PrPd has also been observed when other TSE agents (scrapie and CWDmd) were similarly inoculated into cattle. The IHC and WB findings suggest that the diagnostic techniques currently used to confirm BSE would detect CWDwtd in cattle, should it occur naturally. Also, the absence of SE and a distinctive IHC pattern of CWDwtd and CWDmd in cattle suggests that it should be possible to distinguish these conditions from other TSEs that have been experimentally transmitted to cattle.

second passage is even worse ;

Experimental Second Passage of Chronic Wasting Disease (CWDmule deer) Agent to Cattle

A. N. Hamir, R. A. Kunkle, J. M. Miller, J. J. Greenlee and J. A. Richt Agricultural Research Service, United States Department of Agriculture, National Animal Disease Center, 2300 Dayton Avenue, P.O. Box 70, Ames, IA 50010, USA


To compare clinicopathological findings in first and second passage chronic wasting disease (CWDmule deer) in cattle, six calves were inoculated intracerebrally with brain tissue derived froma first-passageCWD-affected calf in an earlier experiment. Two uninoculated calves served as controls. The inoculated animals began to lose both appetite and weight 10–12 months later, and five subsequently developed clinical signs of central nervous system (CNS) abnormality. By 16.5 months, all cattle had been subjected to euthanasia because of poor prognosis. None of the animals showed microscopical lesions of spongiform encephalopathy (SE) but PrPres was detected in their CNS tissues by immunohistochemistry (IHC) and rapid Western blot (WB) techniques. Thus, intracerebrally inoculated cattle not only amplified CWD PrPres from mule deer but also developed clinicalCNSsigns in the absence of SElesions.This situation has also been shown to occur in cattle inoculated with the scrapie agent. The study confirmed that the diagnostic techniques currently used for diagnosis of bovine spongiformencephalopathy (BSE) in theUS would detect CWDin cattle, should it occur naturally. Furthermore, it raised the possibility of distinguishing CWDfromBSE in cattle, due to the absence of neuropathological lesions and to a distinctive multifocal distribution of PrPres, as demonstrated by IHC which, in this study, appeared to be more sensitive than the WB technique. Published by Elsevier Ltd. Keywords: cattle; chronic wasting disease (CWD); deer; transmissible spongiform encephalopathy (TSE)


Discussion CWD, like all other TSEs, is characterized by a long incubation period, which in deer is seldom less than 18 months (Williams and Young, 1992). In an experimental study of cattle inoculated intracerebrally with CWD from mule deer (first passage), amplification of PrPres was demonstrated in only five of 13 (38%) cattle, after incubation periods that ranged from 23 to 63 months (Hamir et al., 2001a, 2005a). In contrast, all inoculated cattle in the present study were positive for PrPres within 16.5 months. This increased attack rate with shorter incubation periods probably indicates adaptation of the CWDmule deer agent to a new host.


The uniform susceptibility, relatively short incubation, and absence of microscopical lesions in cattle given CWD brain material passaged once through cattle resembled findings in cattle inoculated intracerebrally with the scrapie agent (Cutlip et al., 1997). In that experiment, 100% of cattle died 14–18 months after inoculation with material from the first cattle-passage of a US strain of the scrapie agent; none showed microscopical lesions and all were positive for PrPres.

Thursday, August 12, 2010

Seven main threats for the future linked to prions


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