Executive and emotional dysfunction in Machado‐Joseph disease
Identifieur interne : 000E38 ( Istex/Curation ); précédent : 000E37; suivant : 000E39Executive and emotional dysfunction in Machado‐Joseph disease
Auteurs : Tricia M. Zawacki [États-Unis] ; Janet Grace [États-Unis] ; Joseph H. Friedman [États-Unis] ; Lewis Sudarsky [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 2002-09.
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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.
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DOI: 10.1002/mds.10033
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Zawacki</name><affiliation wicri:level="1"><mods:affiliation>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island, USA</mods:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island</wicri:regionArea></affiliation></author><author><name sortKey="Grace, Janet" sort="Grace, Janet" uniqKey="Grace J" first="Janet" last="Grace">Janet Grace</name><affiliation wicri:level="1"><mods:affiliation>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island, USA</mods:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island</wicri:regionArea></affiliation></author><author><name sortKey="Friedman, Joseph H" sort="Friedman, Joseph H" uniqKey="Friedman J" first="Joseph H." last="Friedman">Joseph H. 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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>New York</pubPlace><date type="published" when="2002-09">2002-09</date><biblScope unit="vol">17</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="1004">1004</biblScope><biblScope unit="page" to="1010">1010</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">8B7B6D9E5A6B8F2947F556B30562D26EEE94EEBE</idno><idno type="DOI">10.1002/mds.10033</idno><idno type="ArticleID">MDS10033</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>ataxia</term><term>behavior</term><term>gait disorders</term><term>neuropsychology</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml: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.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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