Human Prion Diseases

SangYun Kim; Hae-Kwan Cheong; Seong Soo An

doi:10.5124/jkma.2008.51.12.1125

J Korean Med Assoc > Volume 51(12); 2008 > Article

Kim, Cheong, and An: Human Prion Diseases

Continuing Education Column

Journal of the Korean Medical Association

Journal of the Korean Medical Association 2008; 51(12): 1125-1138.

Published online: December 31, 2008

DOI: http://dx.doi.org/10.5124/jkma.2008.51.12.1125

Human Prion Diseases

SangYun Kim, MD¹, Hae-Kwan Cheong, MD², Seong Soo An, PhD³

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

Abstract

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.

Prion; Creutzfeldt - Jakob disease; Variant Creutzfeldt - Jakob disease; Transmissible spongiform encephalopathy

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Figure 1

Relationship of animal and human prion diseases

Figure 2

Diffusion weighted images (lower line) of sporadic Creutzfeldt-Jakob disease show asymmetrical high signal intensities along the gyrus, which were not so clear in T2W images (upper line)

Figure 3

Periodic sharp waves at about 1.5/sec; diffuse, bilateral, slightly asymmetrical: and maximal over the anterior portions of the brain

Figure 4

A Pulvinar sign (arrows) of variant Creutzfeldt-Jakob disease (MR flair image) (from internet)

Table 1

Comparison between sporadic and variant Creutzfeldt-Jakob disease