A Case of Creutzfeldt-Jacob Disease(Heidenhain Variant) Confirmed by Pathology, Electron Microscopy and Immunohistochemistry

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.