Friday, December 7, 2012

S. Korea confirms first death caused by Creutzfeldt-Jakob disease ?

S. Korea confirms first death caused by Creutzfeldt-Jakob disease


Publish By David K. Barger Updated 06/12/2012 8:41 pm in Health&Lifestyle / no comments



SEOUL, Nov. 29 – The first death in South Korea caused by neurodegenerative Creutzfeldt-Jakob disease was confirmed by health authorities on Tuesday.


The Center for Disorder Control (KCDC) announced that a 54-year- old woman was diagnosed with iatrogenic Creutzfeldt-Jakob disease (iCJD) after she died in July this year.


She is believed to have been infected during her brain surgery 23 years ago when she had dead brain tissue replaced with a graft of a dura mater product named Lyodura. The victim did not show symptoms until the occurrence of paralysis in her face and toe in June last year.


The KCDC said the transplanted dura mater originated from the body of a CJD patient. The center said it will track down potential patients who received similar transplants with Lyoduras around 1987.


Some 400 cases of iCDJ have been reported in 20 countries, 200 of which were caused after dura mater transplant operations. KCDC, meanwhile, stressed that there is no correlation between this case and the Variant Creutzfeldt-Jakob disease, which is often referred to as human mad cow disease.









> S. Korea confirms first death caused by Creutzfeldt-Jakob disease



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CONFUCIUS CONFUSED AGAIN ?




Thursday, December 8, 2011


S. Korea confirms second case of iatrogenic Creutzfeldt-Jakob disease 48-year-old man 2011/12/08 11:08 KST







Wednesday, November 30, 2011


First iCJD Death Confirmed in Korea






Development of Policy and Strategy for the Control of Creutzfeldt-Jakob Disease in Korea


Choi SI, Jeong BH, Kim YS.


Korean J Epidemiol


2005 Jun 27(1):81-89. Korean.




Total References:16 Cited Korean References:2 Times Cited:0





PURPOSE: Through the understanding of the current status of transmissible spongiform encephalopathy(TSE), this study was conducted to contribute to the development of policy and strategy for the control of TSE in Korea in order to keep Korea as a bovine spongiform encephalopathy(BSE)- and variant Creutzfeldt-Jakob disease(vCJD)-free country. BSE and vCJD cases have not been found in Korea.




During 2001-2004, the number of patients who have been diagnosed as a definite or probable CJD was 121, which are consisted of 62 male and 59 female(average age: 63 years old). The occurrence of the patients was 5-59 people per year until 2003 and has been gradually increasing due to the recent increase in the diagnostic rate rather than the increase of the incidence.




In 2004, the annual occurrence of sporadic CJD(sCJD) in Korea was 1 people per million, which is similar to the average occurrence rate of the world. Two cases of chronic wasting disease(CWD) in deer were found in Chungcheongbuk-do, one in August 2001 and one in October 2001. After that, 4 more CWD-affected deer have been reported in Kyungsangnam-do area in November 2004.




We have also examined the possibility that Korean CJD occurred as a result of dietary exposure to BSE. Fortunately, all of Korean CJD patients were not vCJD cases. However, if BSE occurs in Korea, there is a great potential for most of the Korean population to be easily infected with BSE due to their highly susceptible genotype to BSE infection as well as their traditional food habit. In 2003, the total number of people who left Korea was almost identical with the total number of people who entered Korea. However, we could not analyze the number of people who visited or stayed in the UK and Europe during 1980s~1990s, in which BSE was prevalent in Europe, because there was no statistical data available.


Affiliation:


Ilsong Institute of Life Science, Hallym University, Anyang. yskim@hallym.ac.kr Department of Microbiology, Hallym University College of Medicine, Chunchon.









J Korean Med Sci. 2010 July; 25(7): 1097–1100.


Published online 2010 June 16. doi: 10.3346/jkms.2010.25.7.1097


PMCID: PMC2890893 Familial Creutzfeldt-Jakob Disease with V180I Mutation


fCJD due to the V180I, as our patient's, is extremely rare, with all reported cases have come from Japan with one from France (11, 12), and two in the United states in a non-Asian (Geschwind et al. unpublished results) (13). To our knowledge this is the first reported case in South Korea.







Korean Med Sci. 1998 Jun;13(3):234-240. Published online 2009 June 22. Copyright © 1998 The Korean Academy of Medical Sciences



Molecular analysis of prion protein gene (PRNP) in Korean patients with Creutzfeldt-Jakob disease




B H Jeong, W K Ju, K Huh, E A Lee, I S Choi, J H Im, E K Choi and Y S Kim Institute of Environment & Life Science and Department of Microbiology, College of Medicine, Hallym University, Chunchon, Korea. This




Abstract



Creutzfeldt-Jakob disease (CJD), a relatively uncommon human dementia, is caused by an unconventional slow infectious agent. Several cases of CJD, clinically or histopathologically diagnosed, have been reported in Korea. In order to confirm the diagnosis of CJD and also differential diagnosis of sporadic and familial types of CJD in Korea, we studied two patients who had symptoms of CJD. The histopathological and immunohistochemical studies showed spongiform neurodegeneration and expression of abnormal isoform of prion protein (PrPSc) in astrocytes. Thus, these two patients were diagnosed CJD. To investigate whether these patients were sporadic or familial type of CJD, the molecular analyses of the prion protein gene (PRNP) were done by restriction fragment length polymorphism (RFLP) and DNA sequencing. In the cases of a healthy Korean and two CJD patients, no point mutation was detected in the known hot spots (178, 180, 200, 210, and 232) and they exhibited wild type PRNP sequences. We concluded that both patients have a sporadic type of CJD, but not familial type.








J Korean Med Sci. 1991 Sep;6(3):273-278. Published online 2009 May 26. Copyright © 1991 The Korean Academy of Medical Sciences



A case of Creutzfeldt-Jakob disease



Jae Kwan Cha, Myung Ho Kim, Suck Jun Oh and Eun Kyung Hong Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea. Abstract


Creutzfeldt-Jakob disease (CJD) is presumably caused by a slow infectious pathogen or prion. The principal clinical features of Creutzfeldt-Jakob disease are dementia, pyramidal and extrapyramidal symptoms and signs, cerebellar dysfunction, and myoclonus. The patient rapidly deteriorates, declines to a vegetative state, becomes comatous, and is ultimately dead within several months. The authors present a case of Creutzfeldt-Jakob disease, proved by clinical findings, typical serial EEG, and pathologic features.




Keywords: Creutzfeldt-Jakob disease.










SEE OTHER iCJD cases recently ;




6 December 2012


HONG KONG CHP closely monitors a probable case of sporadic Creutzfeldt-Jakob Disease


The Centre for Health Protection (CHP) of the Department of Health today (December 6) received a report from the health authority of Macao concerning a probable case of sporadic Creutzfeldt-Jakob Disease (CJD) affecting a 51-year-old female Macao resident who had undergone a brain operation in a private hospital in Hong Kong.


According to the health authority of Macao, the woman who developed progressive dementia with limbs spasm, unstable gait, muscle twitching and visual disturbance suggestive of CJD, has been admitted to a hospital in Macao on October 30. Her clinical diagnosis was sporadic CJD and the patient is now in severe condition.


Investigation by the Macao health authority revealed that the woman had received a brain surgery in a private hospital in Hong Kong on September 19.


Upon receipt of the report, the CHP acted in accordance with the international practice and advised the hospital to contact the patients who had undergone procedures using the same set of instruments as the patient since September 19.


According to the current understanding, the transmission risk is extremely low. The contact tracing is a precautionary measure.


A CHP spokesman said, "CJD is a rare disease that affects the brain and is thought to be caused by the build-up of an abnormal, transmissible protein called 'prion' in the brain."


The spokesman said surgical instruments used on the patient should have been sterilised but may not be enough to eradicate the prions which are the causing agents of the disease. Contact tracing conducted by the hospital is under way.


The clinical feature of CJD is characterised by dementia and difficulties in walking. Early symptoms include memory loss, unsteady gait and loss of co-ordination of limbs. These dementia-like symptoms will worsen and twitching of limbs and trunk will occur. Besides, visual disturbance, abnormal behaviour and seizures can occur. Most patients die within one to two years after onset of symptoms.


Since CJD is not transmitted through casual contact, isolation of patients is not necessary. To prevent the disease from spreading, tissue or organ transplant from any CJD patients or re-use of potentially contaminated surgical instruments should be avoided.


Ends/Thursday, December 6, 2012








Scientific Committee on Emerging and Zoonotic Diseases


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Why Americans, As Well as Koreans, Should Be Worried About Mad Cow Tainted USA Beef


By Terry S. Singeltary Sr.


May 15, 2008


Straight to the Source








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Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008


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Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis












full text with source references ;







Friday, November 23, 2012


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