A Case of Creutzfeldt-Jacob Disease / 신경정신의학

ABSTRACT

We report a case of Creutzfeldt-Jacob disease and review the relevant literatures. This 56-year-old male patient was admitted to our hospital with a history of rapidly progressing cognitive dysfunction, visual disturbance, gait disturbance, weakness, involuntary movement of lower extremities, and the symptoms of delirium. On mental status examination, he showed psychomotor retardation, speech disturbance, perseveration, disorientation, impairement of short-term and long-term memory, and inability of abstract thinking. He had a rapid downhill course, along with shakiness of trunk, rigidity of the limbs, myoclonus, confusion, and finally, inability to speak or move on the bedside. On electroencephalogram(EEG), continuous multifocal slow delta activities were found in all leads, especially in the right frontal areas. Brain biopsy showed, spongiosis with neuronal loss and gliosis noted at the cerebral cortex. These findings were compatible with Creutzfeldt-jacob disease.