Creutzfeldt-Jakob Disease Appearing as Paranoid Psychosis

Mr. A was a 49-year-old man with no past history of psychoactive drug use or psychiatric disturbances. He developed the symptoms of disorientation, psychomotor agitation, auditory hallucinations, and paranoid delusions over a month, which prompted his family to arrange for two successive psychiatric consultations. Short trials of haloperidol and risperidone did not relieve his psychiatric symptoms. When Mr. A developed ataxia, he was taken to an emergency room and admitted to the hospital for a examination of his “altered mental status.” By this time, Mr. A was disoriented to time and place and inattentive; he had cogwheel rigidity and incoherent speech; and he appeared to respond to hallucinatory stimuli and voiced vague persecutory delusions. Antipsychotic drug therapy was discontinued, and a diagnosis of delirium was given. Once the psychiatric consultant noticed myoclonus, an EEG was recommended to rule out Creutzfeldt-Jakob disease. A neurologic consultation followed; a computerized tomographic (CT) scan showed diffuse cortical atrophy, and an EEG showed diffuse background slowing and periodic frontal sharp waves. An analysis of his CSF was positive for protein 14-3-3, which confirmed the diagnosis of Creutzfeldt-Jakob disease. Mr. A’s neurologic condition worsened over the next 2 months; he developed incontinence and the inability to swallow. A feeding tube was placed, and Mr. A was discharged to the custody of his family.