| A Case of Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease. |
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Dong Hyun Kim, Sang Yun Kim, Young Soon Yang, Seong Hae Jeong, Ji Soo Kim, Jeong Min Hwang |
1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea. hjm@snu.ac.kr
2Department of Neurology, Seoul National University College of Medicine, Seoul, Korea.
3Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea.
4Department of Neurolology, Seoul National University Bundang Hospital, Seongnam, Korea. |
| 하이덴하인 변형 크로이츠펠트-야콥병 1예 |
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김동현1ㆍ김상윤2,4ㆍ양영순2,4ㆍ정성해2,4ㆍ김지수2,4ㆍ황정민1,3 |
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Department of Ophthalmology, Seoul National University College of Medicine1, Seoul, Korea / Department of Neurology, Seoul National University College of Medicine2, Seoul, Korea / Department of Ophthalmology, Seoul National University Bundang Hospital3, Seongnam, Korea / Department of Neurolology, Seoul National University Bundang Hospital4, Seongnam, Korea |
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| Abstract |
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. |
| Key Words:
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 |
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