J Korean Neurol Assoc.  1985 Jun;3(1):109-115.

One Case Report of Creutzfeldt-Jakob Disease

• Jeong DS
• Kim MJ
• Byun WT
• Jung YI
• Kim SW
• Park KH

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.

MeSH Terms

Basal Ganglia
Brain Stem
Cerebellar Diseases
Cerebellum
Cerebral Cortex
Coma
Creutzfeldt-Jakob Syndrome*
Dementia
Electroencephalography
Ganglion Cysts
Humans
Male
Middle Aged
Motor Neurons
Myoclonus
Pyramidal Tracts
Spinal Cord
Startle Reaction
Stupor