학회지

권호별 검색

  • HOME
  • 학회지
  • 학회지
  • 권호별 검색
Case Report
Dementia and Neurocognitive Disorders 2012: 11: 2: 74-77

전문보기

우울증을 초기증상으로 발현한 가족성 크로이츠펠트-야콥병
주재정·양영순·강진호·이선화 하상원·한정호·조은경·김두응
보훈공단 중앙보훈병원 신경과
A Case of Familial Creutzfeldt-Jacob Disease (V180I) Initially Presenting with Depression
JaeJeong Joo, M.D., YoungSoon Yang, M.D., Jin Ho Kang, M.D., Sun Hwa Lee, M.D., Sang Won Ha, M.D., Jung Ho Han, M.D., Eun Kyung Cho, M.D., Doo Eung Kim, M.D.
Department of Neurology, Veterans hospital, Seoul Medical Center, Seoul, Korea
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.
Key Words: Creutzfeldt-Jakob disease, Prion protein, V180I mutation
대한치매학회지 (Dementia and Neurocognitive Disorders)