Executive and emotional dysfunction in Machado-Joseph disease
Identifieur interne : 002762 ( PascalFrancis/Checkpoint ); précédent : 002761; suivant : 002763Executive 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.
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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.
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Zawacki</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Department of Psychiatry and Human Behavior, Brown University School of Medicine</s1><s2>Providence, Rhode Island</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Rhode Island</region></placeName></affiliation></author><author><name sortKey="Grace, Janet" sort="Grace, Janet" uniqKey="Grace J" first="Janet" last="Grace">Janet Grace</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Department of Psychiatry and Human Behavior, Brown University School of Medicine</s1><s2>Providence, Rhode Island</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ></inist:fA14><country>États-Unis</country><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. 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Zawacki</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Department of Psychiatry and Human Behavior, Brown University School of Medicine</s1><s2>Providence, Rhode Island</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Rhode Island</region></placeName></affiliation></author><author><name sortKey="Grace, Janet" sort="Grace, Janet" uniqKey="Grace J" first="Janet" last="Grace">Janet Grace</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Department of Psychiatry and Human Behavior, Brown University School of Medicine</s1><s2>Providence, Rhode Island</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ></inist:fA14><country>États-Unis</country><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"><inist:fA14 i1="02"><s1>Department of Clinical Neurosciences, Brown University School of Medicine</s1><s2>Providence, Rhode Island</s2><s3>USA</s3><sZ>3 aut.</sZ></inist:fA14><country>États-Unis</country><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"><inist:fA14 i1="03"><s1>Department of Neurology, Brigham and Women's Hospital</s1><s2>Boston, Massachusetts</s2><s3>USA</s3><sZ>4 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">Massachusetts</region></placeName></affiliation></author></analytic><series><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno><imprint><date when="2002">2002</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Emotional disorder</term><term>Exploration</term><term>Human</term><term>Neuropsychological test</term><term>Psychometrics</term><term>Spinocerebellar heredodegeneration</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Hérédodégénérescence spinocérébelleuse</term><term>Trouble émotion</term><term>Test neuropsychologique</term><term>Psychométrie</term><term>Exploration</term><term>Homme</term><term>Machado-Joseph maladie</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</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><inist><standard h6="B"><pA><fA01 i1="01" i2="1"><s0>0885-3185</s0></fA01><fA03 i2="1"><s0>Mov. disord.</s0></fA03><fA05><s2>17</s2></fA05><fA06><s2>5</s2></fA06><fA08 i1="01" i2="1" l="ENG"><s1>Executive and emotional dysfunction in Machado-Joseph disease</s1></fA08><fA11 i1="01" i2="1"><s1>ZAWACKI (Tricia M.)</s1></fA11><fA11 i1="02" i2="1"><s1>GRACE (Janet)</s1></fA11><fA11 i1="03" i2="1"><s1>FRIEDMAN (Joseph H.)</s1></fA11><fA11 i1="04" i2="1"><s1>SUDARSKY (Lewis)</s1></fA11><fA14 i1="01"><s1>Department of Psychiatry and Human Behavior, Brown University School of Medicine</s1><s2>Providence, Rhode Island</s2><s3>USA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ></fA14><fA14 i1="02"><s1>Department of Clinical Neurosciences, Brown University School of Medicine</s1><s2>Providence, Rhode Island</s2><s3>USA</s3><sZ>3 aut.</sZ></fA14><fA14 i1="03"><s1>Department of Neurology, Brigham and Women's Hospital</s1><s2>Boston, Massachusetts</s2><s3>USA</s3><sZ>4 aut.</sZ></fA14><fA20><s1>1004-1010</s1></fA20><fA21><s1>2002</s1></fA21><fA23 i1="01"><s0>ENG</s0></fA23><fA43 i1="01"><s1>INIST</s1><s2>20953</s2><s5>354000105152380170</s5></fA43><fA44><s0>0000</s0><s1>© 2002 INIST-CNRS. All rights reserved.</s1></fA44><fA45><s0>42 ref.</s0></fA45><fA47 i1="01" i2="1"><s0>02-0584490</s0></fA47><fA60><s1>P</s1></fA60><fA61><s0>A</s0></fA61><fA64 i1="01" i2="1"><s0>Movement disorders</s0></fA64><fA66 i1="01"><s0>USA</s0></fA66><fC01 i1="01" l="ENG"><s0>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. 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Zawacki</name></region><name sortKey="Friedman, Joseph H" sort="Friedman, Joseph H" uniqKey="Friedman J" first="Joseph H." last="Friedman">Joseph H. Friedman</name><name sortKey="Grace, Janet" sort="Grace, Janet" uniqKey="Grace J" first="Janet" last="Grace">Janet Grace</name><name sortKey="Sudarsky, Lewis" sort="Sudarsky, Lewis" uniqKey="Sudarsky L" first="Lewis" last="Sudarsky">Lewis Sudarsky</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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