Wednesday, November 30, 2011

First iCJD Death Confirmed in Korea

First iCJD Death Confirmed in Korea

Korea's Centers for Disease Control and Prevention, announced Tuesday, that a 54-year old woman died of Creutzfeldt-Jakob disease in November last year.

The incurable and invariably fatal brain disease has a long latent period and creates holes in the brain when an outbreak occurs.

Authorities believe the woman contracted the disease during brain surgery in 1987 when she received a transplanted membrane from overseas.

[Interview : Park Hye-kyung, Head of Infectious Diseases Surveillance
Centers for Disease Control and Prevention]

"The deceased patient went through a brain tumor surgery and received a transplant of a dura mater called 'Lyodura' that was imported from Germany.

Her CJD symptoms appeared twenty-three years later, in June 2010."

Korea's health ministry, however, said that, there is NO correlation, between the woman's case, and the variant Creutzfeldt-Jakob Disease, which is often referred to as, human mad cow disease.
Currently there are four known types of CJDs.

The most common form is called sCJD, or sporadic CJD, which accounts for nearly 90 percent of all outbreaks and its cause is known to be natural mutation.

There is also the familial type of CJD, which is believed to be genetic.

The vCJD, or variant Creutzfeldt-Jakob disease is transmitted from cattle with mad cow disease.

This woman's case was Korea's first case of iCJD, or iatrogenic Creutzfeldt-Jakob disease which is caused by transplants of infected human tissues.

Some 400 iCJD cases have been reported in 20 countries throughout the world.

Doctors say that iCJD cases such as this one are very rare because the German manufacturer who supplied the membrane to the woman halted production of dura mater in 1987. and many producers have since reinforced preventive measures.

The World Health Organization also banned production of membranes from tissue extracted from corpses in 1997.

[Interview : Kim Yun-joong, Neurology professor
Hallym Medical School]

"Most providers reinforced the inactivation of infectivity by raising the level of sodium hydroxide.
That is the reason why outbreaks rarely occured after 1987."

The Centers for Disease Control said that they will follow-up with other patients who went through similar surgeries in the '80s.. to trace whether there might be other cases.
Song Ji-sun, Arirang News.

NOV 30, 2011

Reporter :


Prediction for Potential Risk Factors Through the Association Study Between Epidemiological Data and SNPs of Prion Protein Gene in the Korean Population and Suspected CJD Patients

Suyeon Kim,† Sol Moe Lee, Jae Wook Hyeon, Bo-Yeong Choi, Chi-Kyeong Kim, Jun Sun Park and Young Ran Ju

National Institute of Health, Korea CDC; Cheongwongun, Chungcheongbukdo, Korea;†Presenting author; Email:

Cases of the suspected CJD patients and reports of probable CJD have been increased due to a social uneasiness for import permission of beef from western countries reported BSE outbreak since 2008 in Korea CDC. It has been hard to definite diagnosis them due to Korean funeral culture though reports of probable sporadic CJD and genetic CJD have been increased in Korea. First of all, we need to clear the characteristics of PRNP gene in Korean population and to analyze the association between the endemic environmental factors and SNPs of the gene to predict the underlying cause.

We sequenced up to 5kb of the genomic region including the promoter region, exon I and exon II to analyze the correlation between SNPs of 185 suspected patients and diagnostic factors, and between SNPs of PRNP gene of 296 normal population and 60 epidemiologic factors like medical and familial history and diet. General statistical analyses were carried out by using SPSS statistic 18 (SPSS Inc., NY). Their significance levels were determined by the chi-square test (Fisher’s exact test).

We identified 19 SNPs in normal group and 15 SNPs gene in suspected patients’ group in their promoter and exon II regions. Our statistic analyses demonstrated that between rs1799990 (+385A>G; 129MV), rs28933385 (+598A>G; 200EK) and patient factors in suspected patients’ group showed significantly. In normal population group, between rs2756271 (-14605A>G), rs73612131 (-13537A>T), -14409 (C>T), rs1800014 (+655A>G; 219EK), +695(T>G; 232MR) and +591 (C>T; 197NN) SNPs and several demographic and dietary factors like an intake frequency of beef or ham were significantly associated.

In this study, we could predict the potential risk factors through the association study between SNPs of PRNP gene and several epidemiological factors. Especially, significance level of some dietary habits might show higher than other factors. However, we cannot entirely decide them risk factors of genetic markers in prion disease without identification of environmental or other causes of definite CJD patients though we found de novo SNP and significant result of PRNP. We expect to elucidate clearly their association through the combination of our results with other clinical information including additional clues. ...

PRION 2011


The aim of this review was to examine the evidence for the secondary transmission of CJD/vCJD by invasive diagnostic, surgical or dental procedures. The relevant data derive from three sources:

• case reports

• case-control studies

• experimental studies of the transmission of prion disease to animals using surgical instruments or surrogates.

A systematic review was undertaken to identify all relevant studies. Details of the literature searches and inclusion criteria may be found in Appendix 2.

3.1 Case reports

By July 2000, only seven cases of CJD worldwide were known to have been transmitted by invasive diagnostic or surgical procedures which did not involve transplantation or grafting. Two cases were transmitted in 1974 by stereotactic EEG using probes cleaned by a method which would no longer be considered adequate. Symptomatic neurological disease developed after 20 months in one patient and after 16 months in the other.16

After sterilisation and storage in formaldehyde vapour for two years, the probes were implanted in the brain of a chimpanzee, who developed signs of encephalopathy 18 months after implantation.17 A further five cases have been attributed to neurosurgery (median incubation period 17 months, range 12-28).18 These presumably include the case infected by cranial surgery in 1965, with an incubation period of 26 months19 and three cases infected in the UK in the 1950s in which the incubation period was 18-24 months.20 No cases of CJD attributable to invasive diagnostic or surgical procedures without transplantation or grafting have been identified more recently than the 1970s.18

No cases of CJD have been securely attributed to dental procedures. However, in Japan two patients treated by the same dentist developed CJD, and it is possible that one or both were infected in this way.21

3.2 Case-control studies

Seven studies were identified which recorded histories of surgical and dental procedures in cases with definite or probable

CJD and in matched controls22,23,24,25,26,27,28 (for details, see Appendix 2). Two of these studies27,28 used substantially the same cases, but different controls. An eighth study, the EUROSURGYCJD study, is ongoing.29

Case-control studies are inevitably problematic as they depend on respondent recall of past events. Case-control studies of CJD are particularly problematic because the cases are not able to respond for themselves, and therefore past events are further filtered through the memory of another respondent (usually a close family member). Some of the included studies attempted to achieve comparability by collecting information on both cases and controls from similar respondents; others introduced a source of bias by using the controls themselves as respondents.


The validity of case-control studies also depends on the appropriate choice of controls. Some of the included studies used controls drawn from hospital settings. This may distort the results as such controls are likely to have higher than average exposure frequencies for surgical interventions.30,23,24,25,26,27,28

Ideally, therefore, only those studies would have been included which both drew their control group from the community and used similar respondents as a source of data for cases and controls. However, no studies were identified which met these criteria.

The best quality evidence available is therefore derived from five studies which used community controls who responded directly: these are the studies by Collins et al,22 Davanipour et al,23 Juan et al,25 Kondo and Kuroiwa,26 and Ward et al.28 The studies by Collins et al22 and Ward et al28 found that a history of any surgery was associated with a significantly increased risk of CJD (odds ratio 1.7-1.8), while Kondo and Kuroiwa found that any surgery in the five years preceding diagnosis was associated with a relative risk of developing CJD of 3.5 (p<0.01)26 (for details, see Appendix 2 Table 1).

see full report here ;

J Neurol Neurosurg Psychiatry 2002;72:792-793 doi:10.1136/jnnp.72.6.792

Short report

Creutzfeldt-Jakob disease 38 years after diagnostic use of human growth hormone

Monday, July 18, 2011

Impact of Being Placed at Risk of Creutzfeldt-Jakob Disease: A Qualitative Study of Blood Donors to Variant CJD Cases and Patients Potentially Surgically Exposed to CJD

Tuesday, March 29, 2011


Wednesday, June 29, 2011

TSEAC Meeting August 1, 2011 donor deferral ...

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

Tuesday, May 04, 2010

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

Saturday, January 26, 2008


09:00 - 26 January 2008

Sunday, November 21, 2010

Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates and potential Iatrogenic TSE there from

Monday, February 01, 2010

Import Alert 57-20 and 84-03 Human Dura Mater and risk factors there from due to Creutzfeldt Jakob Disease (CJD)

Saturday, June 25, 2011

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

"BSE-L in North America may have existed for decades"

Sunday, June 26, 2011

Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque

Wednesday, August 24, 2011

There Is No Safe Dose of Prions

Wednesday, August 24, 2011

All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD

Friday, September 2, 2011

Supreme Court clears ‘PD Notebook’ of distortion over USA MAD COW CONTROVERSY

Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease

Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011

(see video here) ;

Saturday, March 5, 2011




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