하이덴하인 변형 크로이츠펠트-야콥병 1예

• 영문명: A Case of Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease
• 발행기관: 대한안과학회
• 저자명: 김동현 김상윤 양영순 정성해 김지수 황정민,Dong-Hyun Kim, M.D., Sang Yun Kim, M.D.,, Young Soon Yang, M.D.,, Seong-Hae Jeong, M.D.,, Ji Soo Kim, M.D.,, Jeong-Min Hwang, M.D.,
• 간행물 정보: 『대한안과학회지』Ophthalmological Society,volume49,number9, 1548~1552쪽, 전체 5쪽
• 주제분류: 의약학 > 기타의약학
• 파일형태: PDF
• 발행일자: 2008.09.15

영문 초록

Purpose: To report a case of the Heidenhain variant of sporadic Creutzfeldt-Jakob disease (CJD), predominantly characterized by visual impairment at onset. Case summary: History-taking, ophthalmologic examination, neurologic examination, cerebrospinal fluid examination including 14-3-3 protein analysis, and brain MRI were performed in a 48-year-old man with progressive visual loss and a visual field defect. These symptoms were accompanied by visual illusion and macropsia. Neurologic examination revealed relatively rapidly progressing cognitive impairment, ataxia, aphasia, and myoclonus. The 14-3-3 protein was detectable in otherwise normal CSF samples. The diffusion weighted brain MRI showed increased signal intensity in both occipital lobes, the basal ganglia, the temporal and frontal lobes. He was clinically diagnosed as having a Heidenhain variant of sporadic CJD. Conclusions: In a patient with a rapidly progressive visual loss, visual field defects, visual illusion, and neurologic abnormalities including progressive dementia, ataxia, aphasia, and myoclonus, the Heidenhain variant of CJD should be considered. Because prions, a cause of CJD, exhibit unusual resistance to conventional chemical and physical decontamination methods, it is necessary to have an appropriate management scheme to prevent the spread of infection. J Korean Ophthalmol Soc 2008;49(9):1548-1552

키워드

Diffusion weighted brain MRI, Heidenhain variant of sporadic creutzfeldt-jakob disease (CJD), Progressive visual loss and visual field defect, Visual illusion, 14-3-3 protein