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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한치매학회 Dementia and Neurocognitive Disorders(대한치매학회지) Dementia and Neurocognitive Disorders(대한치매학회지) 제11권 제2호
- 발행연도
- 2012.1
- 수록면
- 74 - 77 (4page)
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Creutzfeldt-Jakob disease (CJD) is a degenerative neurological disorder that is incurable and invariably fatal. It is characterized by rapidly progressive dementia presenting with memory loss, personality changes and hallucinations. The symptoms of CJD are caused by progressive death of neurons in the central nervous system, which is associated with build-up of the abnormal prion proteins forming amyloids. In human, CJD can be acquired genetically through a mutation of the gene encoding for the prion protein (PRNP). This occurs in only 5-10% of all CJD cases. We report a 64-year old woman with CJD carrying a V180I mutation that features late onset, rapid progression, no periodic sharp wave complexes on electroencephalography, and cortical signal change and edema in bilateral frontotemporoparietal lobes and basal ganglia on MRI.
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참고문헌 (12)
참고문헌 신청
Chasseigneaux S / 2006 / V180I mutation of the prion protein gene associated with atypical PrPSc glycosylation / Neurosci Lett 408 : 165 ~ 169
Heinemann U / 2008 / Novel PRNP mutation in a patient with a slow progressive dementia syndrome / Med Sci Monit 14 : 41 ~ 43
Jin K / 2004 / Clinical features of Creutzfeldt-Jakob disease with V180I mutation / Neurology 62 : 502 ~ 505
Kong QK / 2004 / Inherited Prion Diseases, In Prion Biology and Disease. 2nd ed / Cold Spring Harbor Laboratory Press : 673 ~ 776
Nixon R / 2000 / The PRNP-V180I mutation is associated with abnormally glycosylated PrPCJD and Intracellular PrP accumulations. Presented at XIVth International Congress of
Heinemann U / 2008 / Novel PRNP mutation in a patient with a slow progressive dementia syndrome / Med Sci Monit 14 : 41 ~ 43
Jin K / 2004 / Clinical features of Creutzfeldt-Jakob disease with V180I mutation / Neurology 62 : 502 ~ 505
Kong QK / 2004 / Inherited Prion Diseases, In Prion Biology and Disease. 2nd ed / Cold Spring Harbor Laboratory Press : 673 ~ 776
Nixon R / 2000 / The PRNP-V180I mutation is associated with abnormally glycosylated PrPCJD and Intracellular PrP accumulations. Presented at XIVth International Congress of
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