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J Korean Med Assoc > Volume 51(12); 2008 > Article
Journal of the Korean Medical Association 2008;51(12):1125-1138.
DOI: https://doi.org/10.5124/jkma.2008.51.12.1125   
Human Prion Diseases
Sang Yun Kim, Hae Kwan Cheong, Seong Soo An
1Department of Neurology, Clinical Neuroscience Center, Seoul National University Bundang Hospital, Korea. neuroksy@snu.ac.kr
2Department of Social and Preventive Medicine, Sungkyunkwan University College of Medicine, Korea. hkcheong@skku.edu
3Department of Bionanotechnology, KyungWon University Gachon Bionanotechnology Research Institute, Korea. seongaan@kyungwon.ac.kr
Transmissible Spongiform Encephalopathy (TSE) or prion diseases are fatal neurodegenerative diseases, which are caused by transmissible abnormal prion proteins, converting the endogenous normal prion in the body to the infectious abnormal prions. The most common form of human prion diseases is Creutzfeldt - Jakob disease (CJD). Most of CJD are sporadic with unknown cause. Some familial or iatrogenic CJDs are reported in many countries, but there have been no formally reported case in Korea. Variant CJD (vCJD) is a new form of human prion disease, which revealed differentiated clinical presentations and laboratory diagnostic results. vCJD was thought to be originated from eating the beefs or other parts of bovine spongiform encephalopathy (BSE) infected cattle. The unpredictable species barriers, the underestimated distribution of prion infected tissues, the variable clinical courses, and uncertain disease progressions of many prion diseases, all made the prion related risk assessment very difficult. Korea needs our own surveillance system for various prion diseases of human and animals and to make plans for the risk assessment of the various prion disease transmissions for the minimal spread by maximizing the research capacities.
Key Words: Prion, Creutzfeldt - Jakob disease, Variant Creutzfeldt - Jakob disease, Transmissible spongiform encephalopathy


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