Impairment of executive cognitive functioning in males with fragile X‐associated tremor/ataxia syndrome
Identifieur interne : 003D23 ( Main/Merge ); précédent : 003D22; suivant : 003D24Impairment of executive cognitive functioning in males with fragile X‐associated tremor/ataxia syndrome
Auteurs : Jim Grigsby [États-Unis] ; Angela G. Brega [États-Unis] ; Maureen A. Leehey [États-Unis] ; Glenn K. Goodrich [États-Unis] ; Sébastien Jacquemont [Suisse] ; Danuta Z. Loesch [Australie] ; Jennifer B. Cogswell [États-Unis] ; Jennifer Epstein [États-Unis] ; Rebecca Wilson [États-Unis] ; Tristan Jardini [États-Unis] ; Emma Gould [Australie] ; Rachael E. Bennett [États-Unis] ; David Hessl [États-Unis] ; Susannah Cohen [États-Unis] ; Kylee Cook [États-Unis] ; Flora Tassone [États-Unis] ; Paul J. Hagerman [États-Unis] ; Randi J. Hagerman [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 2007-04-15.
English descriptors
- KwdEn :
- Adult, Aged, Cognition Disorders (diagnosis), Cognition Disorders (genetics), Cognition Disorders (psychology), DNA Mutational Analysis, Diagnosis, Differential, Fragile X Mental Retardation Protein (genetics), Fragile X Syndrome (diagnosis), Fragile X Syndrome (genetics), Fragile X Syndrome (psychology), Humans, Intelligence (genetics), Male, Middle Aged, Neuropsychological Tests, Problem Solving, Promoter Regions, Genetic, Spinocerebellar Degenerations (diagnosis), Spinocerebellar Degenerations (genetics), Spinocerebellar Degenerations (psychology), Tremor (diagnosis), Tremor (genetics), Tremor (psychology), Trinucleotide Repeats, cognition, executive cognitive function., fragile X, premutation, trinucleotide repeats.
- MESH :
- chemical , genetics : Fragile X Mental Retardation Protein.
- diagnosis : Cognition Disorders, Fragile X Syndrome, Spinocerebellar Degenerations, Tremor.
- genetics : Cognition Disorders, Fragile X Syndrome, Intelligence, Spinocerebellar Degenerations, Tremor.
- psychology : Cognition Disorders, Fragile X Syndrome, Spinocerebellar Degenerations, Tremor.
- Adult, Aged, DNA Mutational Analysis, Diagnosis, Differential, Humans, Male, Middle Aged, Neuropsychological Tests, Problem Solving, Promoter Regions, Genetic, Trinucleotide Repeats.
Abstract
The fragile X‐associated tremor/ataxia syndrome (FXTAS) is a recently identified phenotype associated with trinucleotide repeat expansions in the premutation range of the fragile X mental retardation 1 (FMR1) gene. In addition to progressive gait ataxia, action tremor, peripheral neuropathy, and parkinsonism, FXTAS involves impaired cognition. Our preliminary research suggests that executive cognitive functioning (ECF) is especially affected. In this study, a brief neuropsychological exam was administered to 33 men with FXTAS and 27 healthy controls. Compared with controls, individuals with FXTAS showed statistically significant impairments on measures from the Wechsler Adult Intelligence Scale, third edition (WAIS‐III; verbal IQ, performance [nonverbal] IQ, verbal comprehension, perceptual organization, and processing speed). FXTAS subjects scored significantly lower on three of four measures of ECF and on two tests of information processing speed. The results provide evidence that FXTAS involves impairment of general intellectual functioning, with marked impairment of executive cognitive abilities. The pattern of cognitive performance is somewhat similar to that observed in the frontal variant of frontotemporal dementia and several of the spinocerebellar ataxias, but differs from the deficits observed in dementia of the Alzheimer type. © 2007 Movement Disorder Society
Url:
DOI: 10.1002/mds.21359
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Loesch</name><affiliation wicri:level="1"><country xml:lang="fr">Australie</country><wicri:regionArea>School of Psychological Science, La Trobe University, Bundoora</wicri:regionArea><wicri:noRegion>Bundoora</wicri:noRegion></affiliation></author><author><name sortKey="Cogswell, Jennifer B" sort="Cogswell, Jennifer B" uniqKey="Cogswell J" first="Jennifer B." last="Cogswell">Jennifer B. Cogswell</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Epstein, Jennifer" sort="Epstein, Jennifer" uniqKey="Epstein J" first="Jennifer" last="Epstein">Jennifer Epstein</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Child Development Unit, The Children's Hospital, Denver, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Wilson, Rebecca" sort="Wilson, Rebecca" uniqKey="Wilson R" first="Rebecca" last="Wilson">Rebecca Wilson</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Child Development Unit, The Children's Hospital, Denver, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Jardini, Tristan" sort="Jardini, Tristan" uniqKey="Jardini T" first="Tristan" last="Jardini">Tristan Jardini</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Gould, Emma" sort="Gould, Emma" uniqKey="Gould E" first="Emma" last="Gould">Emma Gould</name><affiliation wicri:level="1"><country xml:lang="fr">Australie</country><wicri:regionArea>School of Psychological Science, La Trobe University, Bundoora</wicri:regionArea><wicri:noRegion>Bundoora</wicri:noRegion></affiliation></author><author><name sortKey="Bennett, Rachael E" sort="Bennett, Rachael E" uniqKey="Bennett R" first="Rachael E." last="Bennett">Rachael E. Bennett</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Hessl, David" sort="Hessl, David" uniqKey="Hessl D" first="David" last="Hessl">David Hessl</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Psychiatry and Behavioral Sciences, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Cohen, Susannah" sort="Cohen, Susannah" uniqKey="Cohen S" first="Susannah" last="Cohen">Susannah Cohen</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Cook, Kylee" sort="Cook, Kylee" uniqKey="Cook K" first="Kylee" last="Cook">Kylee Cook</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Tassone, Flora" sort="Tassone, Flora" uniqKey="Tassone F" first="Flora" last="Tassone">Flora Tassone</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Biochemistry and Molecular Medicine, University of California, Davis School of Medicine, Davis, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Hagerman, Paul J" sort="Hagerman, Paul J" uniqKey="Hagerman P" first="Paul J." last="Hagerman">Paul J. Hagerman</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Biochemistry and Molecular Medicine, University of California, Davis School of Medicine, Davis, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Hagerman, Randi J" sort="Hagerman, Randi J" uniqKey="Hagerman R" first="Randi J." last="Hagerman">Randi J. Hagerman</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Pediatrics, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</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>Hoboken</pubPlace><date type="published" when="2007-04-15">2007-04-15</date><biblScope unit="vol">22</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="645">645</biblScope><biblScope unit="page" to="650">650</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">A46E654CBC5F85EA27E8E9FAA7565AE272E7BEC1</idno><idno type="DOI">10.1002/mds.21359</idno><idno type="ArticleID">MDS21359</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Cognition Disorders (diagnosis)</term><term>Cognition Disorders (genetics)</term><term>Cognition Disorders (psychology)</term><term>DNA Mutational Analysis</term><term>Diagnosis, Differential</term><term>Fragile X Mental Retardation Protein (genetics)</term><term>Fragile X Syndrome (diagnosis)</term><term>Fragile X Syndrome (genetics)</term><term>Fragile X Syndrome (psychology)</term><term>Humans</term><term>Intelligence (genetics)</term><term>Male</term><term>Middle Aged</term><term>Neuropsychological Tests</term><term>Problem Solving</term><term>Promoter Regions, Genetic</term><term>Spinocerebellar Degenerations (diagnosis)</term><term>Spinocerebellar Degenerations (genetics)</term><term>Spinocerebellar Degenerations (psychology)</term><term>Tremor (diagnosis)</term><term>Tremor (genetics)</term><term>Tremor (psychology)</term><term>Trinucleotide Repeats</term><term>cognition</term><term>executive cognitive function.</term><term>fragile X</term><term>premutation</term><term>trinucleotide repeats</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Fragile X Mental Retardation Protein</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cognition Disorders</term><term>Fragile X Syndrome</term><term>Spinocerebellar Degenerations</term><term>Tremor</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Cognition Disorders</term><term>Fragile X Syndrome</term><term>Intelligence</term><term>Spinocerebellar Degenerations</term><term>Tremor</term></keywords><keywords scheme="MESH" qualifier="psychology" xml:lang="en"><term>Cognition Disorders</term><term>Fragile X Syndrome</term><term>Spinocerebellar Degenerations</term><term>Tremor</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>DNA Mutational Analysis</term><term>Diagnosis, Differential</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Neuropsychological Tests</term><term>Problem Solving</term><term>Promoter Regions, Genetic</term><term>Trinucleotide Repeats</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The fragile X‐associated tremor/ataxia syndrome (FXTAS) is a recently identified phenotype associated with trinucleotide repeat expansions in the premutation range of the fragile X mental retardation 1 (FMR1) gene. In addition to progressive gait ataxia, action tremor, peripheral neuropathy, and parkinsonism, FXTAS involves impaired cognition. Our preliminary research suggests that executive cognitive functioning (ECF) is especially affected. In this study, a brief neuropsychological exam was administered to 33 men with FXTAS and 27 healthy controls. Compared with controls, individuals with FXTAS showed statistically significant impairments on measures from the Wechsler Adult Intelligence Scale, third edition (WAIS‐III; verbal IQ, performance [nonverbal] IQ, verbal comprehension, perceptual organization, and processing speed). FXTAS subjects scored significantly lower on three of four measures of ECF and on two tests of information processing speed. The results provide evidence that FXTAS involves impairment of general intellectual functioning, with marked impairment of executive cognitive abilities. The pattern of cognitive performance is somewhat similar to that observed in the frontal variant of frontotemporal dementia and several of the spinocerebellar ataxias, but differs from the deficits observed in dementia of the Alzheimer type. © 2007 Movement Disorder Society</div></front></TEI><double doi="10.1002/mds.21359"><ISTEX><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Impairment of executive cognitive functioning in males with fragile X‐associated tremor/ataxia syndrome</title><author><name sortKey="Grigsby, Jim" sort="Grigsby, Jim" uniqKey="Grigsby J" first="Jim" last="Grigsby">Jim Grigsby</name></author><author><name sortKey="Brega, Angela G" sort="Brega, Angela G" uniqKey="Brega A" first="Angela G." last="Brega">Angela G. Brega</name></author><author><name sortKey="Leehey, Maureen A" sort="Leehey, Maureen A" uniqKey="Leehey M" first="Maureen A." last="Leehey">Maureen A. Leehey</name></author><author><name sortKey="Goodrich, Glenn K" sort="Goodrich, Glenn K" uniqKey="Goodrich G" first="Glenn K." last="Goodrich">Glenn K. Goodrich</name></author><author><name sortKey="Jacquemont, Sebastien" sort="Jacquemont, Sebastien" uniqKey="Jacquemont S" first="Sébastien" last="Jacquemont">Sébastien Jacquemont</name></author><author><name sortKey="Loesch, Danuta Z" sort="Loesch, Danuta Z" uniqKey="Loesch D" first="Danuta Z." last="Loesch">Danuta Z. Loesch</name></author><author><name sortKey="Cogswell, Jennifer B" sort="Cogswell, Jennifer B" uniqKey="Cogswell J" first="Jennifer B." last="Cogswell">Jennifer B. Cogswell</name></author><author><name sortKey="Epstein, Jennifer" sort="Epstein, Jennifer" uniqKey="Epstein J" first="Jennifer" last="Epstein">Jennifer Epstein</name></author><author><name sortKey="Wilson, Rebecca" sort="Wilson, Rebecca" uniqKey="Wilson R" first="Rebecca" last="Wilson">Rebecca Wilson</name></author><author><name sortKey="Jardini, Tristan" sort="Jardini, Tristan" uniqKey="Jardini T" first="Tristan" last="Jardini">Tristan Jardini</name></author><author><name sortKey="Gould, Emma" sort="Gould, Emma" uniqKey="Gould E" first="Emma" last="Gould">Emma Gould</name></author><author><name sortKey="Bennett, Rachael E" sort="Bennett, Rachael E" uniqKey="Bennett R" first="Rachael E." last="Bennett">Rachael E. Bennett</name></author><author><name sortKey="Hessl, David" sort="Hessl, David" uniqKey="Hessl D" first="David" last="Hessl">David Hessl</name></author><author><name sortKey="Cohen, Susannah" sort="Cohen, Susannah" uniqKey="Cohen S" first="Susannah" last="Cohen">Susannah Cohen</name></author><author><name sortKey="Cook, Kylee" sort="Cook, Kylee" uniqKey="Cook K" first="Kylee" last="Cook">Kylee Cook</name></author><author><name sortKey="Tassone, Flora" sort="Tassone, Flora" uniqKey="Tassone F" first="Flora" last="Tassone">Flora Tassone</name></author><author><name sortKey="Hagerman, Paul J" sort="Hagerman, Paul J" uniqKey="Hagerman P" first="Paul J." last="Hagerman">Paul J. Hagerman</name></author><author><name sortKey="Hagerman, Randi J" sort="Hagerman, Randi J" uniqKey="Hagerman R" first="Randi J." last="Hagerman">Randi J. Hagerman</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:A46E654CBC5F85EA27E8E9FAA7565AE272E7BEC1</idno><date when="2007" year="2007">2007</date><idno type="doi">10.1002/mds.21359</idno><idno type="url">https://api.istex.fr/document/A46E654CBC5F85EA27E8E9FAA7565AE272E7BEC1/fulltext/pdf</idno><idno type="wicri:Area/Istex/Corpus">001296</idno><idno type="wicri:Area/Istex/Curation">001296</idno><idno type="wicri:Area/Istex/Checkpoint">001909</idno><idno type="wicri:doubleKey">0885-3185:2007:Grigsby J:impairment:of:executive</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Impairment of executive cognitive functioning in males with fragile X‐associated tremor/ataxia syndrome</title><author><name sortKey="Grigsby, Jim" sort="Grigsby, Jim" uniqKey="Grigsby J" first="Jim" last="Grigsby">Jim Grigsby</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Brega, Angela G" sort="Brega, Angela G" uniqKey="Brega A" first="Angela G." last="Brega">Angela G. Brega</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Leehey, Maureen A" sort="Leehey, Maureen A" uniqKey="Leehey M" first="Maureen A." last="Leehey">Maureen A. Leehey</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neurology, University of Colorado at Denver and Health Sciences Center, Denver, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Goodrich, Glenn K" sort="Goodrich, Glenn K" uniqKey="Goodrich G" first="Glenn K." last="Goodrich">Glenn K. Goodrich</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Jacquemont, Sebastien" sort="Jacquemont, Sebastien" uniqKey="Jacquemont S" first="Sébastien" last="Jacquemont">Sébastien Jacquemont</name><affiliation wicri:level="3"><country xml:lang="fr">Suisse</country><wicri:regionArea>Department of Medical Genetics, Centre Hospitalier Universitaire Vaudois, Lausanne</wicri:regionArea><placeName><settlement type="city">Lausanne</settlement><region nuts="3" type="region">Canton de Vaud</region></placeName></affiliation></author><author><name sortKey="Loesch, Danuta Z" sort="Loesch, Danuta Z" uniqKey="Loesch D" first="Danuta Z." last="Loesch">Danuta Z. Loesch</name><affiliation wicri:level="1"><country xml:lang="fr">Australie</country><wicri:regionArea>School of Psychological Science, La Trobe University, Bundoora</wicri:regionArea><wicri:noRegion>Bundoora</wicri:noRegion></affiliation></author><author><name sortKey="Cogswell, Jennifer B" sort="Cogswell, Jennifer B" uniqKey="Cogswell J" first="Jennifer B." last="Cogswell">Jennifer B. Cogswell</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Epstein, Jennifer" sort="Epstein, Jennifer" uniqKey="Epstein J" first="Jennifer" last="Epstein">Jennifer Epstein</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Child Development Unit, The Children's Hospital, Denver, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Wilson, Rebecca" sort="Wilson, Rebecca" uniqKey="Wilson R" first="Rebecca" last="Wilson">Rebecca Wilson</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Child Development Unit, The Children's Hospital, Denver, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Jardini, Tristan" sort="Jardini, Tristan" uniqKey="Jardini T" first="Tristan" last="Jardini">Tristan Jardini</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Gould, Emma" sort="Gould, Emma" uniqKey="Gould E" first="Emma" last="Gould">Emma Gould</name><affiliation wicri:level="1"><country xml:lang="fr">Australie</country><wicri:regionArea>School of Psychological Science, La Trobe University, Bundoora</wicri:regionArea><wicri:noRegion>Bundoora</wicri:noRegion></affiliation></author><author><name sortKey="Bennett, Rachael E" sort="Bennett, Rachael E" uniqKey="Bennett R" first="Rachael E." last="Bennett">Rachael E. Bennett</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Colorado</wicri:regionArea><placeName><region type="state">Colorado</region></placeName></affiliation></author><author><name sortKey="Hessl, David" sort="Hessl, David" uniqKey="Hessl D" first="David" last="Hessl">David Hessl</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Psychiatry and Behavioral Sciences, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Cohen, Susannah" sort="Cohen, Susannah" uniqKey="Cohen S" first="Susannah" last="Cohen">Susannah Cohen</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Cook, Kylee" sort="Cook, Kylee" uniqKey="Cook K" first="Kylee" last="Cook">Kylee Cook</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Tassone, Flora" sort="Tassone, Flora" uniqKey="Tassone F" first="Flora" last="Tassone">Flora Tassone</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Biochemistry and Molecular Medicine, University of California, Davis School of Medicine, Davis, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Hagerman, Paul J" sort="Hagerman, Paul J" uniqKey="Hagerman P" first="Paul J." last="Hagerman">Paul J. Hagerman</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Biochemistry and Molecular Medicine, University of California, Davis School of Medicine, Davis, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Hagerman, Randi J" sort="Hagerman, Randi J" uniqKey="Hagerman R" first="Randi J." last="Hagerman">Randi J. Hagerman</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>M.I.N.D Institute, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Pediatrics, University of California, Davis Medical Center, Sacramento, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</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>Hoboken</pubPlace><date type="published" when="2007-04-15">2007-04-15</date><biblScope unit="vol">22</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="645">645</biblScope><biblScope unit="page" to="650">650</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">A46E654CBC5F85EA27E8E9FAA7565AE272E7BEC1</idno><idno type="DOI">10.1002/mds.21359</idno><idno type="ArticleID">MDS21359</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>cognition</term><term>executive cognitive function.</term><term>fragile X</term><term>premutation</term><term>trinucleotide repeats</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The fragile X‐associated tremor/ataxia syndrome (FXTAS) is a recently identified phenotype associated with trinucleotide repeat expansions in the premutation range of the fragile X mental retardation 1 (FMR1) gene. In addition to progressive gait ataxia, action tremor, peripheral neuropathy, and parkinsonism, FXTAS involves impaired cognition. Our preliminary research suggests that executive cognitive functioning (ECF) is especially affected. In this study, a brief neuropsychological exam was administered to 33 men with FXTAS and 27 healthy controls. Compared with controls, individuals with FXTAS showed statistically significant impairments on measures from the Wechsler Adult Intelligence Scale, third edition (WAIS‐III; verbal IQ, performance [nonverbal] IQ, verbal comprehension, perceptual organization, and processing speed). FXTAS subjects scored significantly lower on three of four measures of ECF and on two tests of information processing speed. The results provide evidence that FXTAS involves impairment of general intellectual functioning, with marked impairment of executive cognitive abilities. The pattern of cognitive performance is somewhat similar to that observed in the frontal variant of frontotemporal dementia and several of the spinocerebellar ataxias, but differs from the deficits observed in dementia of the Alzheimer type. © 2007 Movement Disorder Society</div></front></TEI></ISTEX><PubMed><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Impairment of executive cognitive functioning in males with fragile X-associated tremor/ataxia syndrome.</title><author><name sortKey="Grigsby, Jim" sort="Grigsby, Jim" uniqKey="Grigsby J" first="Jim" last="Grigsby">Jim Grigsby</name><affiliation wicri:level="1"><nlm:affiliation>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Co 80045-5701, USA. jim.grigsby@uchsc.edu</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Co 80045-5701</wicri:regionArea><wicri:noRegion>Co 80045-5701</wicri:noRegion></affiliation></author><author><name sortKey="Brega, Angela G" sort="Brega, Angela G" uniqKey="Brega A" first="Angela G" last="Brega">Angela G. Brega</name></author><author><name sortKey="Leehey, Maureen A" sort="Leehey, Maureen A" uniqKey="Leehey M" first="Maureen A" last="Leehey">Maureen A. Leehey</name></author><author><name sortKey="Goodrich, Glenn K" sort="Goodrich, Glenn K" uniqKey="Goodrich G" first="Glenn K" last="Goodrich">Glenn K. Goodrich</name></author><author><name sortKey="Jacquemont, Sebastien" sort="Jacquemont, Sebastien" uniqKey="Jacquemont S" first="Sébastien" last="Jacquemont">Sébastien Jacquemont</name></author><author><name sortKey="Loesch, Danuta Z" sort="Loesch, Danuta Z" uniqKey="Loesch D" first="Danuta Z" last="Loesch">Danuta Z. Loesch</name></author><author><name sortKey="Cogswell, Jennifer B" sort="Cogswell, Jennifer B" uniqKey="Cogswell J" first="Jennifer B" last="Cogswell">Jennifer B. Cogswell</name></author><author><name sortKey="Epstein, Jennifer" sort="Epstein, Jennifer" uniqKey="Epstein J" first="Jennifer" last="Epstein">Jennifer Epstein</name></author><author><name sortKey="Wilson, Rebecca" sort="Wilson, Rebecca" uniqKey="Wilson R" first="Rebecca" last="Wilson">Rebecca Wilson</name></author><author><name sortKey="Jardini, Tristan" sort="Jardini, Tristan" uniqKey="Jardini T" first="Tristan" last="Jardini">Tristan Jardini</name></author><author><name sortKey="Gould, Emma" sort="Gould, Emma" uniqKey="Gould E" first="Emma" last="Gould">Emma Gould</name></author><author><name sortKey="Bennett, Rachael E" sort="Bennett, Rachael E" uniqKey="Bennett R" first="Rachael E" last="Bennett">Rachael E. Bennett</name></author><author><name sortKey="Hessl, David" sort="Hessl, David" uniqKey="Hessl D" first="David" last="Hessl">David Hessl</name></author><author><name sortKey="Cohen, Susannah" sort="Cohen, Susannah" uniqKey="Cohen S" first="Susannah" last="Cohen">Susannah Cohen</name></author><author><name sortKey="Cook, Kylee" sort="Cook, Kylee" uniqKey="Cook K" first="Kylee" last="Cook">Kylee Cook</name></author><author><name sortKey="Tassone, Flora" sort="Tassone, Flora" uniqKey="Tassone F" first="Flora" last="Tassone">Flora Tassone</name></author><author><name sortKey="Hagerman, Paul J" sort="Hagerman, Paul J" uniqKey="Hagerman P" first="Paul J" last="Hagerman">Paul J. Hagerman</name></author><author><name sortKey="Hagerman, Randi J" sort="Hagerman, Randi J" uniqKey="Hagerman R" first="Randi J" last="Hagerman">Randi J. Hagerman</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2007">2007</date><idno type="doi">10.1002/mds.21359</idno><idno type="RBID">pubmed:17266074</idno><idno type="pmid">17266074</idno><idno type="wicri:Area/PubMed/Corpus">002864</idno><idno type="wicri:Area/PubMed/Curation">002864</idno><idno type="wicri:Area/PubMed/Checkpoint">002773</idno><idno type="wicri:Area/Ncbi/Merge">001A75</idno><idno type="wicri:Area/Ncbi/Curation">001A75</idno><idno type="wicri:Area/Ncbi/Checkpoint">001A75</idno><idno type="wicri:doubleKey">0885-3185:2007:Grigsby J:impairment:of:executive</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Impairment of executive cognitive functioning in males with fragile X-associated tremor/ataxia syndrome.</title><author><name sortKey="Grigsby, Jim" sort="Grigsby, Jim" uniqKey="Grigsby J" first="Jim" last="Grigsby">Jim Grigsby</name><affiliation wicri:level="1"><nlm:affiliation>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Co 80045-5701, USA. jim.grigsby@uchsc.edu</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Co 80045-5701</wicri:regionArea><wicri:noRegion>Co 80045-5701</wicri:noRegion></affiliation></author><author><name sortKey="Brega, Angela G" sort="Brega, Angela G" uniqKey="Brega A" first="Angela G" last="Brega">Angela G. Brega</name></author><author><name sortKey="Leehey, Maureen A" sort="Leehey, Maureen A" uniqKey="Leehey M" first="Maureen A" last="Leehey">Maureen A. Leehey</name></author><author><name sortKey="Goodrich, Glenn K" sort="Goodrich, Glenn K" uniqKey="Goodrich G" first="Glenn K" last="Goodrich">Glenn K. Goodrich</name></author><author><name sortKey="Jacquemont, Sebastien" sort="Jacquemont, Sebastien" uniqKey="Jacquemont S" first="Sébastien" last="Jacquemont">Sébastien Jacquemont</name></author><author><name sortKey="Loesch, Danuta Z" sort="Loesch, Danuta Z" uniqKey="Loesch D" first="Danuta Z" last="Loesch">Danuta Z. Loesch</name></author><author><name sortKey="Cogswell, Jennifer B" sort="Cogswell, Jennifer B" uniqKey="Cogswell J" first="Jennifer B" last="Cogswell">Jennifer B. Cogswell</name></author><author><name sortKey="Epstein, Jennifer" sort="Epstein, Jennifer" uniqKey="Epstein J" first="Jennifer" last="Epstein">Jennifer Epstein</name></author><author><name sortKey="Wilson, Rebecca" sort="Wilson, Rebecca" uniqKey="Wilson R" first="Rebecca" last="Wilson">Rebecca Wilson</name></author><author><name sortKey="Jardini, Tristan" sort="Jardini, Tristan" uniqKey="Jardini T" first="Tristan" last="Jardini">Tristan Jardini</name></author><author><name sortKey="Gould, Emma" sort="Gould, Emma" uniqKey="Gould E" first="Emma" last="Gould">Emma Gould</name></author><author><name sortKey="Bennett, Rachael E" sort="Bennett, Rachael E" uniqKey="Bennett R" first="Rachael E" last="Bennett">Rachael E. Bennett</name></author><author><name sortKey="Hessl, David" sort="Hessl, David" uniqKey="Hessl D" first="David" last="Hessl">David Hessl</name></author><author><name sortKey="Cohen, Susannah" sort="Cohen, Susannah" uniqKey="Cohen S" first="Susannah" last="Cohen">Susannah Cohen</name></author><author><name sortKey="Cook, Kylee" sort="Cook, Kylee" uniqKey="Cook K" first="Kylee" last="Cook">Kylee Cook</name></author><author><name sortKey="Tassone, Flora" sort="Tassone, Flora" uniqKey="Tassone F" first="Flora" last="Tassone">Flora Tassone</name></author><author><name sortKey="Hagerman, Paul J" sort="Hagerman, Paul J" uniqKey="Hagerman P" first="Paul J" last="Hagerman">Paul J. Hagerman</name></author><author><name sortKey="Hagerman, Randi J" sort="Hagerman, Randi J" uniqKey="Hagerman R" first="Randi J" last="Hagerman">Randi J. Hagerman</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="2007" type="published">2007</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Cognition Disorders (diagnosis)</term><term>Cognition Disorders (genetics)</term><term>Cognition Disorders (psychology)</term><term>DNA Mutational Analysis</term><term>Diagnosis, Differential</term><term>Fragile X Mental Retardation Protein (genetics)</term><term>Fragile X Syndrome (diagnosis)</term><term>Fragile X Syndrome (genetics)</term><term>Fragile X Syndrome (psychology)</term><term>Humans</term><term>Intelligence (genetics)</term><term>Male</term><term>Middle Aged</term><term>Neuropsychological Tests</term><term>Problem Solving</term><term>Promoter Regions, Genetic</term><term>Spinocerebellar Degenerations (diagnosis)</term><term>Spinocerebellar Degenerations (genetics)</term><term>Spinocerebellar Degenerations (psychology)</term><term>Tremor (diagnosis)</term><term>Tremor (genetics)</term><term>Tremor (psychology)</term><term>Trinucleotide Repeats</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Fragile X Mental Retardation Protein</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cognition Disorders</term><term>Fragile X Syndrome</term><term>Spinocerebellar Degenerations</term><term>Tremor</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Cognition Disorders</term><term>Fragile X Syndrome</term><term>Intelligence</term><term>Spinocerebellar Degenerations</term><term>Tremor</term></keywords><keywords scheme="MESH" qualifier="psychology" xml:lang="en"><term>Cognition Disorders</term><term>Fragile X Syndrome</term><term>Spinocerebellar Degenerations</term><term>Tremor</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>DNA Mutational Analysis</term><term>Diagnosis, Differential</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Neuropsychological Tests</term><term>Problem Solving</term><term>Promoter Regions, Genetic</term><term>Trinucleotide Repeats</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The fragile X-associated tremor/ataxia syndrome (FXTAS) is a recently identified phenotype associated with trinucleotide repeat expansions in the premutation range of the fragile X mental retardation 1 (FMR1) gene. In addition to progressive gait ataxia, action tremor, peripheral neuropathy, and parkinsonism, FXTAS involves impaired cognition. Our preliminary research suggests that executive cognitive functioning (ECF) is especially affected. In this study, a brief neuropsychological exam was administered to 33 men with FXTAS and 27 healthy controls. Compared with controls, individuals with FXTAS showed statistically significant impairments on measures from the Wechsler Adult Intelligence Scale, third edition (WAIS-III; verbal IQ, performance [nonverbal] IQ, verbal comprehension, perceptual organization, and processing speed). FXTAS subjects scored significantly lower on three of four measures of ECF and on two tests of information processing speed. The results provide evidence that FXTAS involves impairment of general intellectual functioning, with marked impairment of executive cognitive abilities. The pattern of cognitive performance is somewhat similar to that observed in the frontal variant of frontotemporal dementia and several of the spinocerebellar ataxias, but differs from the deficits observed in dementia of the Alzheimer type.</div></front></TEI></PubMed></double></record>Pour manipuler ce document sous Unix (Dilib)
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