A Case of Acute Onset Progressive Dementia Suggestive of a Creutzfeldt-Jakob disease

J Korean Neurol Assoc > Volume 14(3); 1996 > Article

Journal of the Korean Neurological Association 1996;14(3): 662-668.

Creutzfeldt-Jakob 병으로 추정되는 급성진행성 치매 1례

이동국, 도진국

대구 효성가톨릭대학교 의과대학 신경과학교실

A Case of Acute Onset Progressive Dementia Suggestive of a Creutzfeldt-Jakob disease

Dong Kuck Lee, M.D., Jin Kuk Do, M.D.

Department of Neurology, Catholic University of Taegu-Hyosung School of Medicine

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare dementing illness that usually affects older adults and is characterized by a rapidly progressive dementia, ataxia, and myoclonus. The disease is due to an unusual transmissible protein called a prion. The diagnosis is often suspected on the basis of electroencephalographic (EEG) and clinical findings. Our case was 62-year-old woman, who presented acute onset rapid progressive dementia, myoclonus, heightened startle reaction, extrapyramidal symptoms, and died about 2.5 months after onset of disease. We diagnosed this case as CJD with typical EEG pattern and clinical features.