Hostname: page-component-8448b6f56d-c4f8m Total loading time: 0 Render date: 2024-04-24T01:16:09.998Z Has data issue: false hasContentIssue false
  • English
  • Français

Variant Creutzfeldt-Jakob disease: Neuropsychological profile in an extended series of cases

Published online by Cambridge University Press:  01 September 2009

KARI HAWKINS ,
GURJIT CHOHAN ,
CHRISTOPHER KIPPS ,
ROBERT WILL  and
NARINDER KAPUR
KARI HAWKINS
Affiliation:
Neuropsychology Department, Addenbrooke’s Hospital, Cambridge
GURJIT CHOHAN
Affiliation:
CJD Surveillance Unit, Department of Neurology, University of Edinburgh
CHRISTOPHER KIPPS
Affiliation:
Wessex Neurological Centre, Southampton
ROBERT WILL
Affiliation:
CJD Surveillance Unit, Department of Neurology, University of Edinburgh
NARINDER KAPUR*
Affiliation:
Neuropsychology Department, Addenbrooke’s Hospital, Cambridge
*
*Correspondence and reprint requests to: Narinder Kapur, R3 Neurosciences, Box 83, Addenbrooke’s Hospital, Hills Road, Cambridge CB2 0QQ. E-mail: narinder.kapur@addenbrookes.nhs.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Neuropsychological data on an extended series of cases of variant Creutzfeldt-Jakob Disease (vCJD) are presented, complementing earlier findings from smaller sample studies of this condition. Distinct neuropsychological features in this extended series included relatively preserved verbal knowledge, immediate verbal memory span, and elementary visual processing. This sparing contrasted with ubiquitous impairment in every vCJD patient on timed tests of verbal fluency and digit-symbol substitution. There were also high rates of impairment on tests of memory, and of visuoperceptual and visuospatial reasoning. Our findings lend support to the view that distinctive neuropsychological features may be one of the diagnostic markers of the condition. (JINS, 2009, 15, 807–810.)

Keywords

DementiaPrionSpongiformSubcorticalFluencyMemory
Type
Brief Communications
Information
Journal of the International Neuropsychological Society , Volume 15 , Issue 5 , September 2009 , pp. 807 - 810
Copyright
Copyright © The International Neuropsychological Society 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

REFERENCES

Cordery, R.J., Alner, K., Cipolotti, L., Ron, M., Kennedy, A., Collinge, J., et al. (2005). The neuropsychology of variant CJD: A comparative study with inherited and sporadic forms of prion disease. Journal of Neurology, Neurosurgery, and Psychiatry, 76, 330–336.CrossRefGoogle ScholarPubMed
Cordery, R.J., Hall, M., Cipolotti, L., Al-Sarraj, S., O’Donovan, D.G., Davidson, L., et al. (2003). Early cognitive decline in Creutzfeldt-Jakob disease associated with human growth hormone treatment. Journal of Neurology, Neurosurgery, and Psychiatry, 74, 1412–1416.CrossRefGoogle ScholarPubMed
Geschwind, M.D., Tan, K.M., Lennon, V.A., Barajas, R.F. Jr, Haman, A., Klein, C.J., et al. (2008). Voltage-gated potassium channel autoimmunity mimicking Creutzfeldt-Jakob disease. Archives of Neurology, 65, 1341–1346.CrossRefGoogle ScholarPubMed
Kapur, N., Abbott, P., Lowman, A., & Will, R.G. (2003). The neuropsychological profile associated with variant Creutzfeldt-Jakob disease. Brain, 126, 2693–2702.CrossRefGoogle ScholarPubMed
Snowden, J.S., Mann, D.M., & Neary, D. (2002). Distinct neuropsychological characteristics in Creutzfeldt-Jakob disease. Journal of Neurology, Neurosurgery, and Psychiatry, 73, 686–694.CrossRefGoogle ScholarPubMed
Spencer, M.D, Knight, R.S., & Will, R.G. (2002). First hundred cases of variant Creutzfeldt-Jakob disease: Retrospective case note review of early psychiatric and neurological features. BMJ, 324, 1479–1482.CrossRefGoogle ScholarPubMed
Stone, R., Archer, J.S., & Kiernan, M. (2008). Wernicke’s encephalopathy mimicking variant Creutzfeldt-Jakob disease. Journal of Clinical Neuroscience, 15, 1308–1310.CrossRefGoogle ScholarPubMed
Will, R.G., Ironside, J.W., Zeidler, M., Cousens, S.N., Estibeiro, K., Alperovitch, A., et al. (1996). A new variant of Creutzfeldt-Jakob disease in the UK. Lancet, 347, 921–925.CrossRefGoogle ScholarPubMed
Will, R.G., Zeidler, M., Stewart, G.E., Macleod, M.A., Ironside, J.W., Cousens, S.N., et al. (2000). Diagnosis of new variant Creutzfeldt-Jakob disease. Annals of Neurology, 47, 575–582.3.0.CO;2-W>CrossRefGoogle ScholarPubMed