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From: TSS ()
Subject: The neuropsychology of variant CJD: a comparative study with inherited and sporadic forms of prion disease
Date: February 17, 2005 at 9:35 am PST

-------- Original Message --------
Subject: The neuropsychology of variant CJD: a comparative study with inherited and sporadic forms of prion disease
Date: Wed, 16 Feb 2005 21:11:13 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################

Journal of Neurology Neurosurgery and Psychiatry 2005;76:330-336
© 2005 BMJ Publishing Group Ltd

------------------------------------------------------------------------


PAPER


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

R J Cordery1,2, K Alner3, L Cipolotti3, M Ron4, A Kennedy5,6, J
Collinge5,6 and M N Rossor1,2

1 Dementia Research Group, Department of Neurodegenerative Disease,
Institute of Neurology, University College London, London, UK
2 Division of Neuroscience and Psychological Medicine, Faculty of
Medicine, Imperial College of Science, Technology and Medicine, London, UK
3 Department of Neuropsychology, The National Hospital for Neurology and
Neurosurgery, London, UK
4 Department of Neuropsychiatry, Institute of Neurology, University
College London, London, UK
5 MRC Prion Unit, Department of Neurodegenerative Diseases, Institute of
Neurology, University College London, London, UK
6 National Prion Clinic, St Marys Hospital London, London, UK

Correspondence to:
Professor Martin Rossor
Dementia Research Group, The National Hospital for Neurology and
Neurosurgery, Queen Square, London WC1N 3BG, UK;
mrossor@dementia.ion.ucl.ac.uk

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 Marys 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.

------------------------------------------------------------------------

Abbreviations: BSE, bovine spongiform encephalopathy; CJD,
Creutzfeldt-Jakob disease; FFI, familial fatal insomnia; FIQ, full IQ;
GSS, Gerstmann-Sträussler-Scheinker disease; IQ, intelligence quotient;
NART, National Adult Reading Test; PIQ, performance IQ; vCJD, variant
Creutzfeldt-Jakob disease; VIQ, verbal IQ; WAIS-R, Wechsler Adult
Intelligence Scale-Revised

Keywords: cognition; Creutzfeldt-Jakob; neuropsychology; prion

http://jnnp.bmjjournals.com/cgi/content/abstract/76/3/330?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1108609455773_4700&stored_search=&FIRSTINDEX=0&volume=76&issue=3&journalcode=jnnp

TSS

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