MovDisordV3, PascalFrancis, Corpus, bibRecord, 002625

Executive and emotional dysfunction in Machado-Joseph disease

Identifieur interne : 002625 ( PascalFrancis/Corpus ); précédent : 002624; suivant : 002626

Executive and emotional dysfunction in Machado-Joseph disease

Auteurs : Tricia M. Zawacki ; Janet Grace ; Joseph H. Friedman ; Lewis Sudarsky

Source :

Movement disorders [ 0885-3185 ] ; 2002.

RBID : Pascal:02-0584490

Descripteurs français

Pascal (Inist)
- Hérédodégénérescence spinocérébelleuse, Trouble émotion, Test neuropsychologique, Psychométrie, Exploration, Homme, Machado-Joseph maladie.

English descriptors

KwdEn :
- Emotional disorder, Exploration, Human, Neuropsychological test, Psychometrics, Spinocerebellar heredodegeneration.

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.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

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A11	`03`	`1`		`@1 FRIEDMAN (Joseph H.)`
A11	`04`	`1`		`@1 SUDARSKY (Lewis)`
A14	`01`			`@1 Department of Psychiatry and Human Behavior, Brown University School of Medicine @2 Providence, Rhode Island @3 USA @Z 1 aut. @Z 2 aut.`
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C01	`01`		`ENG`	@0 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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C03	`07`	`X`	`FRE`	`@0 Machado-Joseph maladie @2 NM @4 INC @5 86`
C07	`01`	`X`	`FRE`	`@0 Système nerveux pathologie @5 37`
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C07	`01`	`X`	`SPA`	`@0 Sistema nervioso patología @5 37`
C07	`02`	`X`	`FRE`	`@0 Système nerveux central pathologie @5 38`
C07	`02`	`X`	`ENG`	`@0 Central nervous system disease @5 38`
C07	`02`	`X`	`SPA`	`@0 Sistema nervosio central patología @5 38`
C07	`03`	`X`	`FRE`	`@0 Moelle épinière pathologie @5 39`
C07	`03`	`X`	`ENG`	`@0 Spinal cord disease @5 39`
C07	`03`	`X`	`SPA`	`@0 Médula espinal patología @5 39`
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Format Inist (serveur)

NO :	PASCAL 02-0584490 INIST
ET :	Executive and emotional dysfunction in Machado-Joseph disease
AU :	ZAWACKI (Tricia M.); GRACE (Janet); FRIEDMAN (Joseph H.); SUDARSKY (Lewis)
AF :	Department of Psychiatry and Human Behavior, Brown University School of Medicine/Providence, Rhode Island/Etats-Unis (1 aut., 2 aut.); Department of Clinical Neurosciences, Brown University School of Medicine/Providence, Rhode Island/Etats-Unis (3 aut.); Department of Neurology, Brigham and Women's Hospital/Boston, Massachusetts/Etats-Unis (4 aut.)
DT :	Publication en série; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2002; Vol. 17; No. 5; Pp. 1004-1010; Bibl. 42 ref.
LA :	Anglais
EA :	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.
CC :	002B17G
FD :	Hérédodégénérescence spinocérébelleuse; Trouble émotion; Test neuropsychologique; Psychométrie; Exploration; Homme; Machado-Joseph maladie
FG :	Système nerveux pathologie; Système nerveux central pathologie; Moelle épinière pathologie; Encéphale pathologie; Maladie dégénérative; Maladie héréditaire
ED :	Spinocerebellar heredodegeneration; Emotional disorder; Neuropsychological test; Psychometrics; Exploration; Human
EG :	Nervous system diseases; Central nervous system disease; Spinal cord disease; Cerebral disorder; Degenerative disease; Genetic disease
SD :	Heredodegeneración espinocerebelosa; Trastorno emocíon; Prueba neuropsichologica; Psicometría; Exploración; Hombre
LO :	INIST-20953.354000105152380170
ID :	02-0584490

Links to Exploration step

Pascal:02-0584490

Le document en format XML

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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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<ET>Executive and emotional dysfunction in Machado-Joseph disease</ET>
<AU>ZAWACKI (Tricia M.); GRACE (Janet); FRIEDMAN (Joseph H.); SUDARSKY (Lewis)</AU>
<AF>Department of Psychiatry and Human Behavior, Brown University School of Medicine/Providence, Rhode Island/Etats-Unis (1 aut., 2 aut.); Department of Clinical Neurosciences, Brown University School of Medicine/Providence, Rhode Island/Etats-Unis (3 aut.); Department of Neurology, Brigham and Women's Hospital/Boston, Massachusetts/Etats-Unis (4 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2002; Vol. 17; No. 5; Pp. 1004-1010; Bibl. 42 ref.</SO>
<LA>Anglais</LA>
<EA>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.</EA>
<CC>002B17G</CC>
<FD>Hérédodégénérescence spinocérébelleuse; Trouble émotion; Test neuropsychologique; Psychométrie; Exploration; Homme; Machado-Joseph maladie</FD>
<FG>Système nerveux pathologie; Système nerveux central pathologie; Moelle épinière pathologie; Encéphale pathologie; Maladie dégénérative; Maladie héréditaire</FG>
<ED>Spinocerebellar heredodegeneration; Emotional disorder; Neuropsychological test; Psychometrics; Exploration; Human</ED>
<EG>Nervous system diseases; Central nervous system disease; Spinal cord disease; Cerebral disorder; Degenerative disease; Genetic disease</EG>
<SD>Heredodegeneración espinocerebelosa; Trastorno emocíon; Prueba neuropsichologica; Psicometría; Exploración; Hombre</SD>
<LO>INIST-20953.354000105152380170</LO>
<ID>02-0584490</ID>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Executive and emotional dysfunction in Machado-Joseph disease

Executive and emotional dysfunction in Machado-Joseph disease

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