• Journal of the Korean neurological association
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• v.36 no.4
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• pp.345-349
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• 2018

Familial Creutzfeldt-Jakob Disease (fCJD) is characteristic with older age onset, relatively low occurrence rate, slower progression and lower possibility of developing myoclonus, cerebellar, pyramidal signs and visual disturbance compared with classical sporadic CJD. We report a case of 75-year-old male patient presented with sudden onset of right side weakness with Broca's aphasia who has been diagnosed with fCJD with V180I mutation. This case indicates that fCJD with V180I mutation can have stroke-like initial presentation.

• Proceedings of the KSMRM Conference
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• 2005.09a
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• pp.79-83
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• 2005

• Proceedings of the Microbiological Society of Korea Conference
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• 2009.05a
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• pp.144-144
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• 2009

• Journal of Sasang Constitutional Medicine
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• v.18 no.2
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• pp.139-147
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• 2006

1. Objectives Creutzfelt Jacob Disease is one of a group of neurodegenerative disorders causing spongiform encephalopathies due to a infection of prion or unconventional slow virus on central nerve system. The diagnosis of this disease is not easy and there is currently no cure. This article is to report our case about a female patient who was not diagnosed as CJD at the early period so that we treated her with Yangkyuksanhwa-tang(凉膈散火湯) and Jihwangbaekho-tang(地黃白虎湯). 2. Methods Magnetic resonance imaging(brain MRI), blood test and computer tomography were performed. The treatment for this patient was clinically based on Sasang Constitutional Medicine. 3. Results and Conclusions (1) Visual field defect, ataxia, myoclonus, sweating and dysuria were the main symptoms of the patient. (2) The pathological change in parenchyme was not revealed during the early periods by MR imaging. So the diagnosing CJD was not possible during the time in this case. (3) Jihwangbaekho-tang(地黃白虎湯) improved her myoclonus and sweating. Bur her mental disorder and the progress of the pathological change in the parenchyme was not able to be treated.

• 한국환경농학회:학술대회논문집
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• 2009.07a
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• pp.273-282
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• 2009

The transmissible spongiform encephalopathies (TSEs) disease group are fatal neurodegenerative disorders affecting a wide range of hosts. The group includes kuru and Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep and goats and Bovine spongiform encephalopathy (BSE) in cattle. The exact nature of the infectious agent involved in the transmission of these diseases remains controversial. However, a central event in their pathogenesis is the accumulation in infected tissues of an abnormal form of a host-encoded protein, the prion protein (PrP). Whereas the normal cellular protein is fully sensitive to protease ($PrP^{sen}$), the disease-associated prion protein ($PrP^d$) is only partly degraded ($PrP^{res}$), its amino-terminal end being removed. BSE was first reported in the mid-80s in the UK. Ten years later, a new form of human prion disease, variant CJD (vCJD) developed in the wake of the BSE epidemic, and there is now strong scientific evidence that vCJD was initiated by the exposure of humans to BSE-infected tissues, thus indicating a zoonotic disease. However, the ban on the feeding of animal-derived proteins to ruminants, and the apparent lack of vertical transmission of BSE, have led to a decline in the incidence of the disease within cattle herd and therefore, an assumed decreased risk for human contacting vCJD. The origin of the original case(s) of BSE still remains an enigma even though three hypotheses have been raised. Hypotheses are i) sheep- or goat-derived scrapie-infected tissues included in meat and bone meal fed to cattle, ii) a previously undetected sporadic or genetic bovine TSE contaminating cattle feed or iii) originating from a human TSE through animal feed contaminated with human remains. A host cellular membrane protein ($PrP^C$), which is abundant in central nervous system tissue, appear to be conformationally altered in the diseased host into a prion protein ($PrP^{Sc}$). This $PrP^{Sc}$ is detergent insoluble and partially protease-resistant ($PrP^{res}$). The term $PrP^{res}$ is normally used to describe the protein detected after protease treatment, in techniques such as Western immunoblotting, and enzyme-linked immunosorbant assay using fresh/frozen tissue. Immunohistochemistry may performed with formalin-fixed tissues. Also, clinical signs of the BSE are one of the major diagnostic indicators. Recently, atypical forms (known as H- and L-type) of BSE have appeared in several European countries, Japan, Canada and the United States. An unusual case was also reported in a miniature zebu. The atypical BSE fall into two groups based on the relative molecular mass (Mm) of the unglycosylated $PrP^{res}$ band relative to that of classical BSE, one of the higher Mm (H-type) and the other lower (L-type). Both types have been detected worldwide as rare cases in older animals, at a low prevalence consistent with the possibility of sporadic forms of prion diseases in cattle. This raises the unwelcome possibility that vCJD could increase in the human population. Now, active surveillance program against BSE is going on in Korea. In regional veterinary service lab, ELISA is applied to screen the BSE in slaughter and confirmatory tests by Western immunoblotting and immunohistochemisty are carried out if there are positive or suspect in the screening test. Also, the ruminant feed ban is rigorously enforced. Removal of specified risk materials such as brain and spinal cord from cattle is mandatory process at slaughter to prevent the infected material from entering the human food chain.

• Journal of the korean veterinary medical association
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• v.32 no.5
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• pp.285-316
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• 1996

소 해면형 뇌증(Bovine Spongiform Encephalpathy; BSE)이 처음 확인된 것은 1986년 11월이며, 영국에서의 최초발생을 시작으로 하여, 지난 10여년 동안 발생국가의 수는 다소 증가하고 있지만 전체적인 발생두수는 1993년을 정점으로하여 감소하는 추세를 보이고 있다. 아직까지도 확실한 병인체는 밝혀져 있지 않은 상태이며, 최초 확인당시 인체에의 감염 가능성은 없는 것으로 발표된 바 있으나, 지난 1996년 3월 20일, 영국정부 보건장관이 영구하원에서, 소 해면형 뇌증이 사람의 희귀한 뇌 질환인 크로이츠휄트-야곱병(CJD)의 새로운 스트레인(v-CJD)과 관련성이 있을 수 있다고 보고한 다음날, CNN 등 세계 각 매스컴을 통하여, '광우병 사람에게 전파될지도$\cdots$'(Mad cow illness may threaten humans)라는 제목으로 Top News로 보도되었으며, 수의학이나 의학적인 사실 확인의 여부를 떠나, 사회 경제적인 문제로 큰 놀라움과 많은 의문사항을 남겨둔 채, 국제적인 문제로 확대된 바있다. 본 편에서는, 소 해면형 뇌증 (BSE)과 관련, 영국의 광우병 파동이 일어난 지난 3월부터 지금까지 국내외 매스컴에 보도된 내용중 주요사항들을 스크랩하여 참고자료로 제공하고자 한다. 자료중에는 중복기사나 추측기사, 검증되지 아니한 오보, 지극히 개인적인 견해 등도 있을 수 있으며, 외신보도의 접수시간, 국내보도의 조간-석간, 지면구성, 수도권판과 지방판 등, 그리고 특히 병명이나 고유명사, 외래어 등의 전문용어 표기에 있어서 꼭 일치하지 아니하는 경우가 있을 수 있음을 참고로 하여야할 것이다.

• Journal of The Korean Association For Science Education
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• v.30 no.8
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• pp.933-952
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• 2010

This study aims to characterize debate on a socio-scientific issue in the Internet and to provide implications from a postmodernist perspective. This study concentrates on disentanglement of the complex relationship among society, economy, politics and science in an issue and characterization of the given text centering on its originality, the relationship between writer and reader, and the purpose of utterance. Sixty-six most read articles on a web message board were chosen and analyzed as a typical case of a socio-scientific issue in the internet. In them, five scientific disputes were identified: the cause of mad cow disease (MCD), specified risk material and the incubation period, the cause of new variant Creutzfeld-Jakob disease (vCJD), vulnerability of vCJD and the relation of Alzheimer and vCJD in American patients. Each argument is intertwined with social, economic and political problems such as its impact on the domestic beef market, feeding environment of imported cattle and the retaliation against denial of importation. With regard to originality, it is found that the originality of an author is weakened but communal through repetitive quotation of 'Peom', cutting and pasting, and engagement of readers with their comments. Furthermore, in order to close the gap between writer and reader, identity and personal narrative of the writers are often introduced into their writing. In terms of purpose of utterance, these are intended to deliver one's feelings or facilitate human behavior rather than inform through verification of a principle.

• Microbiology and Biotechnology Letters
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• v.43 no.2
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• pp.91-96
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• 2015

Prions are proteinaceous infectious particles that cause neurodegenerative diseases, such as scrapie in sheep, bovine spongiform encephalopathy in cattle and Creutzfeldt-Jakob disease (CJD) in humans. Although the detailed process, regarding the abnormal conversion of prion proteins (PrP), remains to be fully elucidated, a number of environmental factors appear to affect the formation of misfolded PrP, termed PrP^Sc. Because oceanic algae contain mycosporine-like amino acids (MAAs), which exhibit cellular defensive activities under a variety of stress conditions, we investigated the level of PrP^Sc in prion-infected neuroblastoma cells using mycosporine-glycine, porphyra-334 and shinorine. When judged by the level of protease-resistant PrP^Sc in western blots, porphyra-334 and shinorine increased the level of PrP^Sc in cells, but mycosporine-glycine did not. The current results indicate that the MAAs tested in this study enhance the formation of PrP^Sc.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.29 no.3
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• pp.262-267
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• 2007

Implantation of allografts has increased widely with not only the availability of many allogenic bone but also allogenic soft tissues. The aim of tissue banking is to provide surgeons with safe tissues compatible with their intended clinical application. The incidence of tissue transplant-transmitted infection is unknown and can only be inferred from prospective studies. The possibility of donor-to-recipient disease transmission through soft tissue transplantation can be considered by reviewing the risk associated with other transplanted hard tissues. Viral, bacterial, and fungal infections have been transmitted via transplantation of soft tissue allografts such as skin, cornea, dura, pericardium. fascia lata, and heart valves. Corneas have transmitted rabies, Creutzfeldt-Jakob disease (CJD), hepatitis B (HBV), cytomegalovirus (CMV), herpes simplex virus (HSV), bacteria, and fungi. Heart valves have been implicated in transmitting tuberculosis, hepatitis B. HIV-1 and CMV. CJD has been transmitted by dura and pericardium transplants. Skin has transmitted CMV, bacteria, and fungi. Cadaveric skin, pericardium, dura, and fascia lata have been used in dental patients with intra-oral soft tissue injuries and GBR. This study is review of the considering transmission of infectious disease in allogenic soft tissues and guidelines of reducing the risk. Prior to use, many tissues are exposed to antibiotics, disinfectants, and sterilants, which further reduce or remove the risk of transmitted disease. Because some soft tissue grafts cannot be subjected to sterilization steps, the risk of infectious disease transmission remains and thorough donor screening and testing is especially important.

• Journal of the korean veterinary medical association
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• v.34 no.4
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• pp.253-257
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• 1998

동물에서 발생되는 질병의 수는 헤아릴 수 없이 많으며, 병인체도 다양하다. 이러한 질병을 효과적으로 관리하기 위하여, 우리나라의 가축전염병예방법(개정법률 제 4,885호; 1995. 1. 5)에는 제1종 전염병 26종과 제 2종 전염병 28종, 총 54종을 법정가축전염병(法定家畜傳染病)으로 지정해 놓고 있다. 최근에 들어, 1996년 3월에 영국에서 소해면형뇌증 (일병 광우병, BSE)이 인체에서의 변형 크로이츠휄트-야곱병 (v-CJD)과 관련하여 세계적인 주목을 받음은 물론 유럽연합 (EU)을 중심으로한 쇠고기의 국제교역에 크게 영향을 미치고 있으며, 지난해(1997) 3월에는 대만에 구제역 (口蹄疫, FAD)이 발생하여, 여러 양돈장에 급속히 퍼져, 대만의 수출주력 산업인 양돈업의 붕괴를 가져왔을 뿐만 아니라 국가적인 경제위기를 초래하였으며, 연간 약 9조원의 경제적인 손실이 예상되고 있고 양돈경기 회복을 위하여는 막대한 자금(약 40조원)과 긴 세월(최소 4-5년)이 필요할것으로 생각되고 있다. 한편, 국제적으로는 아주 전통적인 질병이며 한동안 자취를 감추었던 광견병과 기종저가 개발하여 피해를 입히고 있으며, 아주 최근에 알려진 새로운 원충성 유산증을 일으키는 네오스포라증도 확인되고 있다. 본 편에서는, 최근 국내에서 특히 문제시 되고 있는 풍토병인 기종저(blackleg)에 대하여 그 발생실태와 임상진단, 방역대책 등에 관하여 고찰해 보고자 한다.