Behavioural abnormalities contribute to functional decline in Huntington’s disease

J M Hamilton; D P Salmon; J Corey-Bloom; A Gamst; J S Paulsen; S Jerkins; M W Jacobson; G Peavy

doi:10.1136/jnnp.74.1.120

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Behavioural abnormalities contribute to functional decline in Huntington’s disease

Journal article

Open access

Peer reviewed

Behavioural abnormalities contribute to functional decline in Huntington’s disease

J M Hamilton, D P Salmon, J Corey-Bloom, A Gamst, J S Paulsen, S Jerkins, M W Jacobson and G Peavy

Journal of neurology, neurosurgery and psychiatry, Vol.44(1), pp.1021-122

01/2003

DOI: 10.1136/jnnp.74.1.120

PMCID: PMC1738208

PMID: 12486282

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Abstract

The independent and relative contributions of motor, cognitive, and behavioural deficits to functional decline in patients with Huntington’s disease are examined. Twenty two patients with Huntington’s disease were assessed with rating scales for motor dysfunction, cognitive measures of executive functions, and behavioural measures of apathy, executive dysfunction, and disinhibition. Their functional status was assessed with informant based and clinician based ratings of activities of daily living (ADL). A composite apathy/executive dysfunction behavioural index was strongly related to decline in ADL independently and after controlling for motor and cognitive deficits. These results suggest that behavioural dysfunction contributes to functional decline in patients with Huntington’s disease and may impede their ability to utilise motor or cognitive skills that remain available in the early stages of the disease.

Burundi

Beck depression inventory

behavioural dysfunction

CANTAB

HD-ADL

ADL

frontal lobe personality scale

Cambridge neuropsychological test automated battery

total functional capacity scale

TFC

FLOPS

activities of daily living

Huntington’s disease activities of daily living scale

Huntington’s disease

UHDRS

unified Huntington’s disease rating scale

Details

Title: Subtitle: Behavioural abnormalities contribute to functional decline in Huntington’s disease
Creators: J M Hamilton - Department of Neurosciences, University of California, San Diego, California, USA
D P Salmon - Department of Neurosciences, University of California, San Diego, California, USA
J Corey-Bloom - Department of Neurosciences, University of California, San Diego, California, USA
A Gamst - Department of Family and Preventative Medicine, University of California, San Diego, California
J S Paulsen - Departments of Psychiatry and Neurology, The University of Iowa, Iowa City, Iowa, USA
S Jerkins - Department of Neurosciences, University of California, San Diego, California, USA
M W Jacobson - Department of Neurosciences, University of California, San Diego, California, USA
G Peavy - Department of Neurosciences, University of California, San Diego, California, USA
Resource Type: Journal article
Publication Details: Journal of neurology, neurosurgery and psychiatry, Vol.44(1), pp.1021-122
DOI: 10.1136/jnnp.74.1.120
PMID: 12486282
PMCID: PMC1738208
NLM abbreviation: J Neurol Neurosurg Psychiatry
ISSN: 0022-3050
eISSN: 1468-330X
Publisher: BMJ Publishing Group Ltd
Language: English
Date published: 01/2003
Academic Unit: Psychiatry; Psychological and Brain Sciences
Record Identifier: 9984083201702771

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