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Sporadic Creutzfeldt-Jakob disease in older people

Part of: Bespoke shallow archive Weizmann

Published online by Cambridge University Press:  11 December 2009

N Shah ,
A Agbobu ,
I Costello ,
A Beri  and
K Minhas
N Shah*
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
A Agbobu
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
I Costello
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
A Beri
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
K Minhas
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
*
Address for correspondence: Dr Nainal Shah, Department of Medicine for the Elderly, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Leicester LE1 5WW, UK. E-mail: [email protected]
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Summary

Prion diseases are rare degenerative disorders of the nervous system caused by abnormal accumulation and/or metabolism of prion proteins. They are invariably fatal. Creutzfeldt-Jakob disease (CJD) is the most common human prion disease and the sporadic form accounts for 85% of cases, whilst about 15% are genetic and 1% is iatrogenic. Variant CJD (vCJD) was mostly seen in the UK and France and mainly affected young people. Sporadic CJD (sCJD) occurs throughout the world with mean age of onset in the seventh decade. It typically presents with rapidly declining cognition that may get confused with other forms of dementia. Thus identification of sCJD in older people depends on a judicious awareness of the clinical features. Here we present a clinical, pathological, therapeutic and diagnostic review of sCJD.

Keywords

Creutzfeldt-Jakob syndromeprion diseasesspongiform encephalopathyolder people
Type
Neuropsychiatry of old age
Information
Reviews in Clinical Gerontology , Volume 19 , Issue 3 , August 2009 , pp. 207 - 215
Copyright
Copyright © Cambridge University Press 2009

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