New variant Creutzfeldt-Jakob disease: psychiatric features

An apparently new variant of Creutzfeldt-Jakob disease (CJD), new variant CJD (nvCJD), was identified in the UK in 1996. There have now been 21 cases of nvCJD in the UK and one in France. Psychiatric symptoms are prominent in the initial presentation in these cases.

Cases of nvCJD are identified mainly by direct referral from neurologists and neuropathologists. Detailed clinical information was obtained by review of case notes and interviewing patients' relatives. We report the psychiatric features of the first 14 cases on nvCJD in the UK. Psychiatric notes were examined in all 13 of these cases who were seen by a psychiatrist.

Eight cases were women. All 14 cases had early psychiatric features and in nine, the first symptom was psychiatric. 13 cases were seen by a psychiatrist and the majority were diagnosed as suffering from depression or depression secondary to organic disease. Two cases suffered from first-rank symptoms suggestive of psychotic illness and transient delusions and auditory or visual hallucinations occurred in the majority. All cases were referred to a neurologist as the illness evolved and neurological signs developed.

Psychiatric symptoms are a consistent early clinical feature in nvCJD. Analysis of the psychiatric symptoms does not suggest specific features that readily allow distinction from more common psychiatric disorders, although the occurrence of associated persistent sensory symptoms may raise the possibility of this diagnosis. Neurological signs, including ataxia, involuntary movements and cognitive impairment developed in all cases and the evolution of increasing neurological deficits is likely to remain critical to the clinical diagnosis of nvCJD.