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.

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.