A Case of Creutzfeldt-Jacob Disease( Heidenhain Variant) confirmed by pathology, electron microscopy and immunohistochemistry

J Korean Neurol Assoc > Volume 16(6); 1998 > Article

Journal of the Korean Neurological Association 1998;16(6): 891-894.

병리학적, 전자현미경 및 면역조직화학적으로 확진된 Creutzfeldt-Jacob(Heidenhain Variant) 병 1례

이은아, 조수진. 허균. 안영환*. 주원규**. 김용선**

아주대학교 의과대학 신경과학, 신경외과학 교실*. 한림대학교 생명과학연구소**

A Case of Creutzfeldt-Jacob Disease( Heidenhain Variant) confirmed by pathology, electron microscopy and immunohistochemistry

Eun Ah Lee, M.D.,Soo Jin Jo, M.D., Kyoon Huh, M.D., Young Hwan Ahn*, M.D. Won Kyu Joo**, M.D., Young Sun Kim**, M.D., Ph.D.,

Department of Neurology, and Neurosurgery*, Ajou University School of Medicine and Institute of Environment Life Science, Hallym University**

Abstract

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. Key Words: Creutzfeldt-Jacob Disease, Heidenhain Variant, Spongiform encephalopathy, Dementia