A Case of Creutzfeldt-Jacob Disease(Heidenhain Variant) Confirmed by Pathology, Electron Microscopy and Immunohistochemistry
병리학적, 전자현미경 및 면역조직화학적으로 확진된 Creutzfeldt-Jacob(Heidenhain Variant) 병 1예
이, 은아; 조, 수진; 허, 균; 안, 영환; 주, 원규; 김, 용선
Taehan Sin'gyŏngkwa Hakhoe chi : sin'gyŏnghak, 16(6):891-894, 1998
Taehan Sin'gyŏngkwa Hakhoe chi : sin'gyŏnghak; Journal of the Korean Neurological Association : neurology
A previously healthy 54 year old man developed a catastrophic neurobehavioral
disorder, suggestive of initial occipital involvement and subsequent spreading to entire brain. Initial symptoms were mainly visual perceptive dysfunction such as
metamorphopsia, visual illusion and hallucination followed by visual agnosia and eventual cortical blindness. He also developed, within 3 months, a rapidly progressive severe dementia, leading to death in 7 months after the onset. Brain biopsy revealed spongiform change, neuronal loss and astrogliosis. Electron microscopy and Immunohistochemistry confirmed the diagnosis of Prion disease. Herewith, we report a case of Heidenhain variant of Creutzfeldt -Jacob Disease.
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