Creutzfeldt-Jakob Disease Presenting as Psychosis

Mr. A was a 31-year-old Caucasian man who was hospitalized for delusions of being controlled and persecutory, megalomanic auditory hallucinations, somatic tactile hallucinations, and visual hallucinations. He had no history of iatrogenic exposure, psychiatric disorders, or substance abuse disorders and no family history of psychiatric illness. The onset of the disorder was insidious. According to his family, Mr. A had had psychiatric symptoms for 9 months before the hospitalization: sleep disorders, auditory hallucinations, and delusional beliefs of persecution, and subsequently, social withdrawal, affective nonresponsivity, ideas of reference, physical transformation beliefs, and aggressive behaviors. His first EEG, performed 1 month before the hospitalization, was normal.

At the onset of the hospitalization, Mr. A had memory problems characterized by an impaired ability to learn new information and daytime wandering, verbal stereotypies, inappropriately smiling, and reduced speech. Haloperidol, 15 mg/day, was used over 3 weeks with no improvement. His memory impairment gradually worsened. Neurological symptoms, such as hyperreflexia and hypertonia, appeared 16 months after his first psychiatric symptoms. He never had myoclonus. His condition deteriorated subsequently, with ataxia, mutism, and incontinence. He died 22 months after appearance of the first psychiatric symptoms.

During hospitalization, repetition of the EEG showed nonspecific cortical abnormalities, without the periodic triphasic waves typical of sporadic Creutzfeldt-Jakob disease. A computerized tomographic scan showed diffuse cortical atrophy. The results of laboratory tests and CSF analysis were normal. The neuropathological findings at autopsy confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease, including frontal and temporal atrophy, extensive cortical spongiform changes, neuronal loss, and gliosis throughout the cerebral cortex.