Executive and emotional dysfunction in Machado-Joseph disease.
Identifieur interne : 003951 ( PubMed/Curation ); précédent : 003950; suivant : 003952Executive 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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<author><name sortKey="Zawacki, Tricia M" sort="Zawacki, Tricia M" uniqKey="Zawacki T" first="Tricia M" last="Zawacki">Tricia M. Zawacki</name>
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<author><name sortKey="Friedman, Joseph H" sort="Friedman, Joseph H" uniqKey="Friedman J" first="Joseph H" last="Friedman">Joseph H. Friedman</name>
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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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<Abstract><AbstractText>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.</AbstractText>
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