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Brain Advance Access published online on August 22, 2003
Brain, doi:10.1093/brain/awg279
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© 2003 Oxford University Press

Article

The neuropsychological profile associated with variant Creutzfeldt-Jakob disease

N. Kapur 1*, P. Abbott 2, A. Lowman 3, and R. G. Will 3

1 Wessex Neurological Centre, Southampton General Hospital, Southampton, UK; Department of Psychology, University of Southampton, Southampton, UK
2 Wessex Neurological Centre, Southampton General Hospital, Southampton, UK
3 National CJD Surveillance Unit, Edinburgh, UK

* Corresponding author. E-mail: n.kapur{at}soton.ac.uk.

Received 31 March 2003 ; revised 23 June 2003 ; accepted 26 June 2003

Abstract

We report the neuropsychological profile associated with variant Creutzfeldt-Jakob disease (vCJD). A retrospective study was carried out of data from neuropsychological reports prepared on 24 patients with vCJD. While there was some variability in neuropsychological profiles, the overall pattern was one of a combined cortical and subcortical dementia, with impaired performance being particularly prominent on tests of memory, executive function, speed of attention, and visuoperceptual reasoning. Across 16 cases where Wechsler Adult Intelligence Scale-Revised intelligence quotient (IQ) scores were available, this profile was in part reflected by an invariably low performance IQ (<90 in all patients). All patients who received tests of verbal fluency, digit-symbol substitution and faces recognition memory showed deficits on these tests. Basic vocabulary, digit span and verbal reasoning skills were relatively preserved in most patients. In four cases who underwent more detailed cognitive testing, additional observations were made of relatively intact long-term autobiographical memory and faces perception. Cognitive impairment may represent one of the earliest features of vCJD and it is possible that, at least in some cases, neuropsychological deficits precede the onset of psychiatric or neurological symptoms. Our findings may help in the early detection and management of patients with vCJD.

Keywords: variant Creutzfeldt-Jakob disease; vCJD; prion disease; dementia
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