Publicação:
Cerebellar Cognitive Affective Syndrome in Machado Joseph Disease: Core Clinical Features

dc.contributor.authorBraga-Neto, Pedro
dc.contributor.authorPedroso, Jose Luiz
dc.contributor.authorAlessi, Helena
dc.contributor.authorDutra, Livia Almeida
dc.contributor.authorFelicio, Andre Carvalho
dc.contributor.authorMinett, Thais
dc.contributor.authorWeisman, Patricia
dc.contributor.authorSantos-Galduroz, Ruth F. [UNESP]
dc.contributor.authorBertolucci, Paulo Henrique F.
dc.contributor.authorGabbai, Alberto Alain
dc.contributor.authorPovoas Barsottini, Orlando Graziani
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionHosp Israelita Albert Einstein
dc.contributor.institutionUniv Cambridge
dc.contributor.institutionUniversidade Federal do ABC (UFABC)
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.date.accessioned2014-05-20T13:56:02Z
dc.date.available2014-05-20T13:56:02Z
dc.date.issued2012-06-01
dc.description.abstractThe cerebellum is no longer considered a purely motor control device, and convincing evidence has demonstrated its relationship to cognitive and emotional neural circuits. The aims of the present study were to establish the core cognitive features in our patient population and to determine the presence of Cerebellar Cognitive Affective Syndrome (CCAS) in this group. We recruited 38 patients with spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD)-SCA3/MJD and 31 controls. Data on disease status were recorded (disease duration, age, age at onset, ataxia severity, and CAG repeat length). The severity of cerebellar symptoms was measured using the International Cooperative Ataxia Rating Scale and the Scale for the Assessment and Rating of Ataxia. The neuropsychological assessment consisted of the Mini-Mental State Examination, Clock Drawing Test, Wechsler Adult Intelligence Scale, Rey-Osterrieth Complex Figure, Wisconsin Card Sorting Test, Stroop Color-Word Test, Trail-Making Test, Verbal Paired Associates, and verbal fluency tests. All subjects were also submitted to the Hamilton Anxiety Scale and Beck Depression Inventory. After controlling for multiple comparisons, spatial span, picture completion, symbol search, Stroop Color-Word Test, phonemic verbal fluency, and Trail-Making Tests A and B were significantly more impaired in patients with SCA3/MJD than in controls. Executive and visuospatial functions are impaired in patients with SCA3/MJD, consistent with the symptoms reported in the CCAS. We speculate on a possible role in visual cortical processing degeneration and executive dysfunction in our patients as a model to explain their main cognitive deficit.en
dc.description.affiliationUniv Fed São Paulo, Dept Neurol & Neurosurg, BR-04039002 São Paulo, Brazil
dc.description.affiliationHosp Israelita Albert Einstein, Inst Israelita Ensino & Pesquisa Albert Einstein, São Paulo, Brazil
dc.description.affiliationUniv Fed São Paulo, Dept Prevent Med, BR-04039002 São Paulo, Brazil
dc.description.affiliationUniv Cambridge, Dept Publ Hlth & Primary Care, Cambridge, England
dc.description.affiliationUniversidade Federal do ABC (UFABC), Ctr Math Comp & Cognit, São Paulo, Brazil
dc.description.affiliationUNESP, Inst Biosci, Rio Claro, Brazil
dc.description.affiliationUnespUNESP, Inst Biosci, Rio Claro, Brazil
dc.description.sponsorshipFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.format.extent549-556
dc.identifierhttp://dx.doi.org/10.1007/s12311-011-0318-6
dc.identifier.citationCerebellum. New York: Springer, v. 11, n. 2, p. 549-556, 2012.
dc.identifier.doi10.1007/s12311-011-0318-6
dc.identifier.issn1473-4222
dc.identifier.urihttp://hdl.handle.net/11449/20040
dc.identifier.wosWOS:000304466100032
dc.language.isoeng
dc.publisherSpringer
dc.relation.ispartofCerebellum
dc.relation.ispartofjcr3.199
dc.relation.ispartofsjr1,705
dc.rights.accessRightsAcesso restrito
dc.sourceWeb of Science
dc.subjectSpinocerebellar ataxia type 3en
dc.subjectMachado-Joseph diseaseen
dc.subjectCognitive deficitsen
dc.subjectCerebellar Cognitive Affective Syndromeen
dc.titleCerebellar Cognitive Affective Syndrome in Machado Joseph Disease: Core Clinical Featuresen
dc.typeArtigo
dcterms.licensehttp://www.springer.com/open+access/authors+rights?SGWID=0-176704-12-683201-0
dcterms.rightsHolderSpringer
dspace.entity.typePublication
unesp.author.orcid0000-0001-7902-7502[9]
unesp.author.orcid0000-0001-9186-9243[1]
unesp.author.orcid0000-0001-6309-9077[4]
unesp.author.orcid0000-0002-0107-0831[11]

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