Creutzfeldt-Jakob Disease Appearing as Paranoid Psychosis
To the Editor: Creutzfeldt-Jakob disease, also known as subacute spongiform encephalopathy, is a prion disease that is characterized by rapidly progressive dementia, often accompanied by myoclonus and other signs of central nervous system dysfunction. Psychiatric disturbances may precede neurologic deficits (1–3), which make the diagnosis of Creutzfeldt-Jakob disease elusive. We describe a patient with Creutzfeldt-Jakob disease who had three psychiatric evaluations before neurologic consultations.
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.
The early psychiatric symptoms of Creutzfeldt-Jakob disease previously described in the literature include depression, affective lability, sleep disturbance, personality change, and memory impairment (1–3). Psychosis, although infrequent, may be present; auditory hallucinations and delusional ideas coexist with fluctuations in the level of consciousness (2). The diagnosis of Creutzfeldt-Jakob disease is aided by an EEG. A characteristic pattern of repetitive sharp waves or slow spikes followed by synchronous triphasic sharp waves is seen in 70% of the patients (2). A CT scan and magnetic resonance imaging usually show cerebral atrophy (2), and a CSF analysis for detection of protein 14-3-3 can confirm the clinical and encephalographic diagnosis. There has been an increase in the prevalence of Creutzfeldt-Jakob disease in European countries in recent decades (1). Our case report highlights the importance of considering Creutzfeldt-Jakob disease in the differential diagnosis of delirium once subtle neurologic deficits are identified in conjunction with psychiatric disturbances.
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