One Case Report of Creutzfeldt-Jakob Disease

J Korean Neurol Assoc > Volume 3(1); 1985 > Article

Journal of the Korean Neurological Association 1985;3(1): 109-115.

Creutzfeldt-Jakob disease로 사료되는 1예

정대수, 박규현, 김상욱, 정영인, 변원탄, 김명정

메리놀병원 신경과. 부산대학교 정신과.

One Case Report of Creutzfeldt-Jakob Disease

Dae-Soo Jeong, M.D.Myung-Jung Kim, M.D., Won-Tan Byun, M.D., Young-In Jung, M.D. ; Sang-Wook Kim, M.D., Kyue-Hyun Park

Department of Neurology, Merinol Hospital, Department of Psychiatry, Busan National University

Abstract

The original article was reported by Creutzfeld in 1920, and Jakob in 1921 respectively. CJD is an encephalopathy with worldwide distribution caused by an unidentified unconventional virus. The principal clinical features of CJD are dementia, upper and in some instances, lower motor neuron disorders; basal ganglionic and cerebellar dysfunction; and myoclonus including a heightened startle reaction. The disease is rapidly progressive, leading in several months to stupor, coma, and death. Pathologic changes are widespread, involving the cerebral cortex, basal ganglia, cerebellum, pyramidal tracts, and lower motor neruons of the brain stem and/or spinal cord. This case was 51 years old Korean male, who presented progressive dementia, myoclonus, heightened startle reaction, pyramidal, extrapyramidal symptoms, decerebrated rigidity, and died about 4 months after onset. We diagnosed this case as CJD with typical serial EEG pattern and clinical feature.