J Korean Neurol Assoc.  1996 Jun;14(2):662-668.

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

• Lee DK
• Do JK

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.

MeSH Terms

Adult
Ataxia
Creutzfeldt-Jakob Syndrome*
Dementia*
Diagnosis
Electroencephalography
Female
Humans
Middle Aged
Myoclonus
Startle Reaction