Executive and emotional dysfunction in Machado-Joseph disease.
Identifieur interne : 000900 ( Ncbi/Curation ); précédent : 000899; suivant : 000901Executive and emotional dysfunction in Machado-Joseph disease.
Auteurs : Tricia M. Zawacki [États-Unis] ; Janet Grace ; Joseph H. Friedman ; Lewis SudarskySource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2002.
English descriptors
- KwdEn :
- Adult, Attention, Brain (physiopathology), Cognition Disorders (diagnosis), Cognition Disorders (etiology), Female, Humans, Machado-Joseph Disease (complications), Machado-Joseph Disease (physiopathology), Male, Middle Aged, Mood Disorders (diagnosis), Mood Disorders (etiology), Neuropsychological Tests, Psychomotor Disorders (diagnosis), Psychomotor Disorders (etiology), Reaction Time, Severity of Illness Index, Speech Disorders (diagnosis), Speech Disorders (etiology), Verbal Behavior.
- 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.
DOI: 10.1002/mds.10033
PubMed: 12360550
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pubmed:12360550Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Executive and emotional dysfunction in Machado-Joseph disease.</title><author><name sortKey="Zawacki, Tricia M" sort="Zawacki, Tricia M" uniqKey="Zawacki T" first="Tricia M" last="Zawacki">Tricia M. Zawacki</name><affiliation wicri:level="1"><nlm:affiliation>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island 02903, USA. tricia_zawacki@brown.edu</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island 02903</wicri:regionArea><wicri:noRegion>Rhode Island 02903</wicri:noRegion></affiliation></author><author><name sortKey="Grace, Janet" sort="Grace, Janet" uniqKey="Grace J" first="Janet" last="Grace">Janet Grace</name></author><author><name sortKey="Friedman, Joseph H" sort="Friedman, Joseph H" uniqKey="Friedman J" first="Joseph H" last="Friedman">Joseph H. Friedman</name></author><author><name sortKey="Sudarsky, Lewis" sort="Sudarsky, Lewis" uniqKey="Sudarsky L" first="Lewis" last="Sudarsky">Lewis Sudarsky</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2002">2002</date><idno type="RBID">pubmed:12360550</idno><idno type="pmid">12360550</idno><idno type="doi">10.1002/mds.10033</idno><idno type="wicri:Area/PubMed/Corpus">003951</idno><idno type="wicri:Area/PubMed/Curation">003951</idno><idno type="wicri:Area/PubMed/Checkpoint">003A92</idno><idno type="wicri:Area/Ncbi/Merge">000900</idno><idno type="wicri:Area/Ncbi/Curation">000900</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Executive and emotional dysfunction in Machado-Joseph disease.</title><author><name sortKey="Zawacki, Tricia M" sort="Zawacki, Tricia M" uniqKey="Zawacki T" first="Tricia M" last="Zawacki">Tricia M. Zawacki</name><affiliation wicri:level="1"><nlm:affiliation>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island 02903, USA. tricia_zawacki@brown.edu</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Psychiatry and Human Behavior, Brown University School of Medicine, Providence, Rhode Island 02903</wicri:regionArea><wicri:noRegion>Rhode Island 02903</wicri:noRegion></affiliation></author><author><name sortKey="Grace, Janet" sort="Grace, Janet" uniqKey="Grace J" first="Janet" last="Grace">Janet Grace</name></author><author><name sortKey="Friedman, Joseph H" sort="Friedman, Joseph H" uniqKey="Friedman J" first="Joseph H" last="Friedman">Joseph H. Friedman</name></author><author><name sortKey="Sudarsky, Lewis" sort="Sudarsky, Lewis" uniqKey="Sudarsky L" first="Lewis" last="Sudarsky">Lewis Sudarsky</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="ISSN">0885-3185</idno><imprint><date when="2002" type="published">2002</date></imprint></series></biblStruct></sourceDesc></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></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></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.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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