Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction.
Identifieur interne : 000621 ( PubMed/Checkpoint ); précédent : 000620; suivant : 000622Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction.
Auteurs : Christopher A. Ross [États-Unis] ; 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
Affiliations:
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Ross</name><affiliation wicri:level="2"><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><country xml:lang="fr">États-Unis</country><wicri:regionArea>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</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Pantelyat, Alex" sort="Pantelyat, Alex" uniqKey="Pantelyat A" first="Alex" last="Pantelyat">Alex Pantelyat</name></author><author><name sortKey="Kogan, Jane" sort="Kogan, Jane" uniqKey="Kogan J" first="Jane" last="Kogan">Jane Kogan</name></author><author><name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2014">2014</date><idno type="doi">10.1002/mds.26012</idno><idno type="RBID">pubmed:25216368</idno><idno type="pmid">25216368</idno><idno type="wicri:Area/PubMed/Corpus">000392</idno><idno type="wicri:Area/PubMed/Curation">000392</idno><idno type="wicri:Area/PubMed/Checkpoint">000621</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction.</title><author><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A" last="Ross">Christopher A. Ross</name><affiliation wicri:level="2"><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><country xml:lang="fr">États-Unis</country><wicri:regionArea>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</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Pantelyat, Alex" sort="Pantelyat, Alex" uniqKey="Pantelyat A" first="Alex" last="Pantelyat">Alex Pantelyat</name></author><author><name sortKey="Kogan, Jane" sort="Kogan, Jane" uniqKey="Kogan J" first="Jane" last="Kogan">Jane Kogan</name></author><author><name sortKey="Brandt, Jason" sort="Brandt, Jason" uniqKey="Brandt J" first="Jason" last="Brandt">Jason Brandt</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2014" type="published">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><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><term>Humans</term><term>Neuropsychological Tests</term><term>Severity of Illness Index</term></keywords></textClass></profileDesc></teiHeader><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></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">25216368</PMID><DateCreated><Year>2014</Year><Month>09</Month><Day>13</Day></DateCreated><DateCompleted><Year>2015</Year><Month>05</Month><Day>15</Day></DateCompleted><DateRevised><Year>2015</Year><Month>09</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Electronic">1531-8257</ISSN><JournalIssue CitedMedium="Internet"><Volume>29</Volume><Issue>11</Issue><PubDate><Year>2014</Year><Month>Sep</Month><Day>15</Day></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction.</ArticleTitle><Pagination><MedlinePgn>1351-8</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1002/mds.26012</ELocationID><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><CopyrightInformation>© 2014 International Parkinson and Movement Disorder Society.</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Ross</LastName><ForeName>Christopher A</ForeName><Initials>CA</Initials><AffiliationInfo><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.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Pantelyat</LastName><ForeName>Alex</ForeName><Initials>A</Initials></Author><Author ValidYN="Y"><LastName>Kogan</LastName><ForeName>Jane</ForeName><Initials>J</Initials></Author><Author ValidYN="Y"><LastName>Brandt</LastName><ForeName>Jason</ForeName><Initials>J</Initials></Author></AuthorList><Language>eng</Language><GrantList CompleteYN="Y"><Grant><GrantID>NS16375</GrantID><Acronym>NS</Acronym><Agency>NINDS NIH HHS</Agency><Country>United States</Country></Grant><Grant><GrantID>P50 NS016375</GrantID><Acronym>NS</Acronym><Agency>NINDS NIH HHS</Agency><Country>United States</Country></Grant></GrantList><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D052061">Research Support, N.I.H., Extramural</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Mov Disord</MedlineTA><NlmUniqueID>8610688</NlmUniqueID><ISSNLinking>0885-3185</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><CommentsCorrectionsList><CommentsCorrections RefType="Cites"><RefSource>Ann Neurol. 1986 Dec;20(6):727-31</RefSource><PMID Version="1">2949692</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Annu Rev Neurosci. 1986;9:357-81</RefSource><PMID Version="1">3085570</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>J Neurosci. 2008 Jul 9;28(28):7143-52</RefSource><PMID Version="1">18614684</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>J Neuropsychiatry Clin Neurosci. 2008 Fall;20(4):466-72</RefSource><PMID Version="1">19196932</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Mov Disord. 2009 Mar 15;24(4):574-8</RefSource><PMID Version="1">19097181</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Lancet Neurol. 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Version="1">2970248</PMID></CommentsCorrections></CommentsCorrectionsList><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000203">Activities of Daily Living</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D003072">Cognition Disorders</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000209">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="Y" UI="D006233">Disabled Persons</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006816">Huntington Disease</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000150">complications</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009069">Movement Disorders</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000209">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" 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Pantelyat</name></noCountry><country name="États-Unis"><region name="Maryland"><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A" last="Ross">Christopher A. 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