Movement Disorders (revue)

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Executive and emotional dysfunction in Machado‐Joseph disease

Identifieur interne : 000E38 ( Istex/Corpus ); précédent : 000E37; suivant : 000E39

Executive and emotional dysfunction in Machado‐Joseph disease

Auteurs : Tricia M. Zawacki ; Janet Grace ; Joseph H. Friedman ; Lewis Sudarsky

Source :

RBID : ISTEX:8B7B6D9E5A6B8F2947F556B30562D26EEE94EEBE

English descriptors

Abstract

Machado‐Joseph disease (MJD) is an autosomal dominant spinocerebellar ataxia. Few studies have examined the neuropsychological and neurobehavioral profiles of patients with MJD. In this study, six individuals with MJD were given a battery of neuropsychological tests. Relative impairments on timed verbal attention tasks and verbal fluency (Stroop, Oral Symbol Digit Modalities, and Controlled Oral Word Association Test) were found. Other executive impairments also were seen on the Wisconsin Card Sorting Test, independent of motor dysfunction severity. Moderate‐ to severe levels of depressive symptoms were endorsed by four of the six patients, and caregivers observed increased apathy in the patients. Impaired executive and emotional functioning in MJD does not appear to be related to ataxia severity. These patients did not meet the criteria for dementia. General cognitive abilities, language, list learning, story recall, and untimed tasks of attention were within normal limits. Impaired executive abilities and emotional functioning in MJD patients is consistent with disruption of frontal‐subcortical systems. © 2002 Movement Disorder Society.

Url:
DOI: 10.1002/mds.10033

Links to Exploration step

ISTEX:8B7B6D9E5A6B8F2947F556B30562D26EEE94EEBE

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<name type="personal">
<namePart type="given">Joseph H.</namePart>
<namePart type="family">Friedman</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Clinical Neurosciences, Brown University School of Medicine, Providence, Rhode Island, USA</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Lewis</namePart>
<namePart type="family">Sudarsky</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts, USA</affiliation>
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<dateIssued encoding="w3cdtf">2002-09</dateIssued>
<dateCaptured encoding="w3cdtf">2001-02-01</dateCaptured>
<dateValid encoding="w3cdtf">2001-08-04</dateValid>
<copyrightDate encoding="w3cdtf">2002</copyrightDate>
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<languageTerm type="code" authority="rfc3066">en</languageTerm>
<languageTerm type="code" authority="iso639-2b">eng</languageTerm>
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<abstract lang="en">Machado‐Joseph disease (MJD) is an autosomal dominant spinocerebellar ataxia. Few studies have examined the neuropsychological and neurobehavioral profiles of patients with MJD. In this study, six individuals with MJD were given a battery of neuropsychological tests. Relative impairments on timed verbal attention tasks and verbal fluency (Stroop, Oral Symbol Digit Modalities, and Controlled Oral Word Association Test) were found. Other executive impairments also were seen on the Wisconsin Card Sorting Test, independent of motor dysfunction severity. Moderate‐ to severe levels of depressive symptoms were endorsed by four of the six patients, and caregivers observed increased apathy in the patients. Impaired executive and emotional functioning in MJD does not appear to be related to ataxia severity. These patients did not meet the criteria for dementia. General cognitive abilities, language, list learning, story recall, and untimed tasks of attention were within normal limits. Impaired executive abilities and emotional functioning in MJD patients is consistent with disruption of frontal‐subcortical systems. © 2002 Movement Disorder Society.</abstract>
<subject lang="en">
<genre>Keywords</genre>
<topic>gait disorders</topic>
<topic>ataxia</topic>
<topic>neuropsychology</topic>
<topic>behavior</topic>
</subject>
<relatedItem type="host">
<titleInfo>
<title>Movement Disorders</title>
<subTitle>Official Journal of the Movement Disorder Society</subTitle>
</titleInfo>
<titleInfo type="abbreviated">
<title>Mov. Disord.</title>
</titleInfo>
<subject>
<genre>article category</genre>
<topic>Research Article</topic>
</subject>
<identifier type="ISSN">0885-3185</identifier>
<identifier type="eISSN">1531-8257</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8257</identifier>
<identifier type="PublisherID">MDS</identifier>
<part>
<date>2002</date>
<detail type="volume">
<caption>vol.</caption>
<number>17</number>
</detail>
<detail type="issue">
<caption>no.</caption>
<number>5</number>
</detail>
<extent unit="pages">
<start>1004</start>
<end>1010</end>
<total>7</total>
</extent>
</part>
</relatedItem>
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<identifier type="DOI">10.1002/mds.10033</identifier>
<identifier type="ArticleID">MDS10033</identifier>
<accessCondition type="use and reproduction" contentType="copyright">Copyright © 2002 Movement Disorders Society</accessCondition>
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<recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin>
<recordContentSource>WILEY</recordContentSource>
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