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J Neurol Neurosurg Psychiatry 2005;76:330-336 doi:10.1136/jnnp.2003.030320
  • Paper

The neuropsychology of variant CJD: a comparative study with inherited and sporadic forms of prion disease

  1. R J Cordery1,2,
  2. K Alner3,
  3. L Cipolotti3,
  4. M Ron4,
  5. A Kennedy5,6,
  6. J Collinge5,6,
  7. M N Rossor1,2
  1. 1Dementia Research Group, Department of Neurodegenerative Disease, Institute of Neurology, University College London, London, UK
  2. 2Division of Neuroscience and Psychological Medicine, Faculty of Medicine, Imperial College of Science, Technology and Medicine, London, UK
  3. 3Department of Neuropsychology, The National Hospital for Neurology and Neurosurgery, London, UK
  4. 4Department of Neuropsychiatry, Institute of Neurology, University College London, London, UK
  5. 5MRC Prion Unit, Department of Neurodegenerative Diseases, Institute of Neurology, University College London, London, UK
  6. 6National Prion Clinic, St Mary’s Hospital London, London, UK
  1. Correspondence to:
 Professor Martin Rossor
 Dementia Research Group, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK; mrossordementia.ion.ucl.ac.uk
  • Received 13 October 2003
  • Accepted 11 July 2004
  • Revised 3 June 2004

Abstract

Objective: To assess cognitive function in variant Creutzfeldt-Jakob disease (vCJD). We describe the neuropsychological profiles of 10 cases and compare these data with cross sectional data obtained from patients with histologically confirmed sporadic CJD and cases with inherited prion disease with confirmed mutations in the prion protein gene.

Methods: Patients referred to the Specialist Cognitive Disorders Clinic at the National Hospital for Neurology and Neurosurgery and the National Prion Clinic at St Mary’s Hospital, London for further investigation of suspected CJD were recruited into the study. The neuropsychological test battery evaluated general intelligence, visual and verbal memory, nominal skills, literacy skills, visual perception and visuospatial functions, and visuospatial and executive function.

Results: The results indicate that moderate to severe cognitive decline is a characteristic feature of vCJD. Specifically, verbal and visual memory impairments and executive dysfunction were pervasive in all disease groups. Nominal skills were impaired in variant and sporadic CJD, significantly so when compared with the inherited prion disease group. Perceptual impairment was less frequent in the vCJD group than in the sporadic and inherited groups.

Conclusion: This study confirms the occurrence of generalised cognitive decline in patients with vCJD. Although decline in cognitive function ultimately affects all domains, there is a suggestion that some components of visual perception may be spared in vCJD. The results also suggest that nominal function may be preserved in some cases with inherited prion disease.

Footnotes

  • This study formed part of project funded by a grant from the Medical Research Council of Great Britain.

  • Competing interests: none declared

  • Martin Rossor assumed editorship of the Journal of Neurology, Neurosurgery and Psychiatry after submission of this manuscript and has had no involvement in the review process

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