Executive and emotional dysfunction in Machado‐Joseph disease
Identifieur interne : 004465 ( Main/Curation ); précédent : 004464; suivant : 004466Executive 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.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Adult, Attention, Brain (physiopathology), Cognition Disorders (diagnosis), Cognition Disorders (etiology), Emotional disorder, Exploration, Female, Human, Humans, Machado-Joseph Disease (complications), Machado-Joseph Disease (physiopathology), Male, Middle Aged, Mood Disorders (diagnosis), Mood Disorders (etiology), Neuropsychological Tests, Neuropsychological test, Psychometrics, Psychomotor Disorders (diagnosis), Psychomotor Disorders (etiology), Reaction Time, Severity of Illness Index, Speech Disorders (diagnosis), Speech Disorders (etiology), Spinocerebellar heredodegeneration, Verbal Behavior, ataxia, behavior, gait disorders, neuropsychology.
- MESH :
- complications : Machado-Joseph Disease.
- diagnosis : Cognition Disorders, Mood Disorders, Psychomotor Disorders, Speech Disorders.
- etiology : Cognition Disorders, Mood Disorders, Psychomotor Disorders, Speech Disorders.
- physiopathology : Brain, Machado-Joseph Disease.
- Adult, Attention, Female, Humans, Male, Middle Aged, Neuropsychological Tests, Reaction Time, Severity of Illness Index, Verbal Behavior.
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
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<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.</div>
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<wicri:regionArea>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island</wicri:regionArea>
<placeName><region type="state">Rhode Island</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Friedman, Joseph H" sort="Friedman, Joseph H" uniqKey="Friedman J" first="Joseph H." last="Friedman">Joseph H. Friedman</name>
<affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Clinical Neurosciences, Brown University School of Medicine, Providence, Rhode Island</wicri:regionArea>
<placeName><region type="state">Rhode Island</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Sudarsky, Lewis" sort="Sudarsky, Lewis" uniqKey="Sudarsky L" first="Lewis" last="Sudarsky">Lewis Sudarsky</name>
<affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts</wicri:regionArea>
<placeName><region type="state">Massachusetts</region>
</placeName>
</affiliation>
</author>
</analytic>
<monogr></monogr>
<series><title level="j">Movement Disorders</title>
<title level="j" type="sub">Official Journal of the Movement Disorder Society</title>
<title level="j" type="abbrev">Mov. 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>Adult</term>
<term>Attention</term>
<term>Brain (physiopathology)</term>
<term>Cognition Disorders (diagnosis)</term>
<term>Cognition Disorders (etiology)</term>
<term>Female</term>
<term>Humans</term>
<term>Machado-Joseph Disease (complications)</term>
<term>Machado-Joseph Disease (physiopathology)</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Mood Disorders (diagnosis)</term>
<term>Mood Disorders (etiology)</term>
<term>Neuropsychological Tests</term>
<term>Psychomotor Disorders (diagnosis)</term>
<term>Psychomotor Disorders (etiology)</term>
<term>Reaction Time</term>
<term>Severity of Illness Index</term>
<term>Speech Disorders (diagnosis)</term>
<term>Speech Disorders (etiology)</term>
<term>Verbal Behavior</term>
<term>ataxia</term>
<term>behavior</term>
<term>gait disorders</term>
<term>neuropsychology</term>
</keywords>
<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Machado-Joseph Disease</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cognition Disorders</term>
<term>Mood Disorders</term>
<term>Psychomotor Disorders</term>
<term>Speech Disorders</term>
</keywords>
<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Cognition Disorders</term>
<term>Mood Disorders</term>
<term>Psychomotor Disorders</term>
<term>Speech Disorders</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Brain</term>
<term>Machado-Joseph Disease</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>Attention</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Neuropsychological Tests</term>
<term>Reaction Time</term>
<term>Severity of Illness Index</term>
<term>Verbal Behavior</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>
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