Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction.
Identifieur interne : 000392 ( PubMed/Corpus ); précédent : 000391; suivant : 000393Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction.
Auteurs : Christopher A. Ross ; Alex Pantelyat ; Jane Kogan ; Jason BrandtSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2014.
English descriptors
- KwdEn :
- MESH :
- complications : Huntington Disease.
- etiology : Cognition Disorders, Movement Disorders.
- Activities of Daily Living, Disabled Persons, Humans, Neuropsychological Tests, Severity of Illness Index.
Abstract
The clinical syndrome of Huntington's disease (HD) is notable for a triad of motor, cognitive, and emotional features. All HD patients eventually become occupationally disabled; however, the factors that render HD patients unable to maintain employment have not been extensively studied. This review begins by discussing the clinical triad of HD, highlighting the distinction in the motor disorder between involuntary movements, such as chorea, and voluntary movement impairment, with the latter contributing more to functional disability. Cognitive disorder clearly contributes to disability, though the relative contribution compared to motor is difficult to unravel, especially because many of the tests used to asses "cognition" have a strong motor component. The role of emotional changes in disability needs more study. The literature on contributions to functional disability, driving impairment, and nursing home placement is reviewed. Relevant experience is presented from the long-standing JHU HD observational study on motor versus cognitive onset, as well as on cognitive and motor features at the time when individuals discontinued working. Finally, we briefly review government policies in several countries on disability determination. We interpret the data from our own studies and from the literature to indicate that there is usually a close relationship between cognitive and motor dysfunction, and that it is critical to take both into consideration in determining disability. © 2014 International Parkinson and Movement Disorder Society.
DOI: 10.1002/mds.26012
PubMed: 25216368
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pubmed:25216368Le document en format XML
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<author><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A" last="Ross">Christopher A. Ross</name>
<affiliation><nlm:affiliation>Division of Neurobiology, Department of Psychiatry, Departments of Neurology, Neuroscience, and Pharmacology, and Program in Cellular and Molecular Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.</nlm:affiliation>
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<author><name sortKey="Pantelyat, Alex" sort="Pantelyat, Alex" uniqKey="Pantelyat A" first="Alex" last="Pantelyat">Alex Pantelyat</name>
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<author><name sortKey="Kogan, Jane" sort="Kogan, Jane" uniqKey="Kogan J" first="Jane" last="Kogan">Jane Kogan</name>
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<author><name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name>
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<author><name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Activities of Daily Living</term>
<term>Cognition Disorders (etiology)</term>
<term>Disabled Persons</term>
<term>Humans</term>
<term>Huntington Disease (complications)</term>
<term>Movement Disorders (etiology)</term>
<term>Neuropsychological Tests</term>
<term>Severity of Illness Index</term>
</keywords>
<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Huntington Disease</term>
</keywords>
<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Cognition Disorders</term>
<term>Movement Disorders</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Activities of Daily Living</term>
<term>Disabled Persons</term>
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<front><div type="abstract" xml:lang="en">The clinical syndrome of Huntington's disease (HD) is notable for a triad of motor, cognitive, and emotional features. All HD patients eventually become occupationally disabled; however, the factors that render HD patients unable to maintain employment have not been extensively studied. This review begins by discussing the clinical triad of HD, highlighting the distinction in the motor disorder between involuntary movements, such as chorea, and voluntary movement impairment, with the latter contributing more to functional disability. Cognitive disorder clearly contributes to disability, though the relative contribution compared to motor is difficult to unravel, especially because many of the tests used to asses "cognition" have a strong motor component. The role of emotional changes in disability needs more study. The literature on contributions to functional disability, driving impairment, and nursing home placement is reviewed. Relevant experience is presented from the long-standing JHU HD observational study on motor versus cognitive onset, as well as on cognitive and motor features at the time when individuals discontinued working. Finally, we briefly review government policies in several countries on disability determination. We interpret the data from our own studies and from the literature to indicate that there is usually a close relationship between cognitive and motor dysfunction, and that it is critical to take both into consideration in determining disability. © 2014 International Parkinson and Movement Disorder Society.</div>
</front>
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<Abstract><AbstractText>The clinical syndrome of Huntington's disease (HD) is notable for a triad of motor, cognitive, and emotional features. All HD patients eventually become occupationally disabled; however, the factors that render HD patients unable to maintain employment have not been extensively studied. This review begins by discussing the clinical triad of HD, highlighting the distinction in the motor disorder between involuntary movements, such as chorea, and voluntary movement impairment, with the latter contributing more to functional disability. Cognitive disorder clearly contributes to disability, though the relative contribution compared to motor is difficult to unravel, especially because many of the tests used to asses "cognition" have a strong motor component. The role of emotional changes in disability needs more study. The literature on contributions to functional disability, driving impairment, and nursing home placement is reviewed. Relevant experience is presented from the long-standing JHU HD observational study on motor versus cognitive onset, as well as on cognitive and motor features at the time when individuals discontinued working. Finally, we briefly review government policies in several countries on disability determination. We interpret the data from our own studies and from the literature to indicate that there is usually a close relationship between cognitive and motor dysfunction, and that it is critical to take both into consideration in determining disability. © 2014 International Parkinson and Movement Disorder Society.</AbstractText>
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<ForeName>Christopher A</ForeName>
<Initials>CA</Initials>
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