Cognitive Deficits in Machado-Joseph Disease Correlate with Hypoperfusion of Visual System Areas

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dc.contributor.author Braga-Neto, Pedro [UNIFESP]
dc.contributor.author Dutra, Livia Almeida [UNIFESP]
dc.contributor.author Pedroso, Jose Luiz [UNIFESP]
dc.contributor.author Felicio, Andre C. [UNIFESP]
dc.contributor.author Alessi, Helena [UNIFESP]
dc.contributor.author Santos-Galduroz, Ruth F.
dc.contributor.author Bertolucci, Paulo Henrique F. [UNIFESP]
dc.contributor.author Castiglioni, Mario Luiz V. [UNIFESP]
dc.contributor.author Bressan, Rodrigo Affonseca [UNIFESP]
dc.contributor.author Jara de Garrido, Griselda Esther
dc.contributor.author Barsottini, Orlando Graziani Povoas [UNIFESP]
dc.contributor.author Jackowski, Andrea [UNIFESP]
dc.date.accessioned 2016-01-24T14:28:04Z
dc.date.available 2016-01-24T14:28:04Z
dc.date.issued 2012-12-01
dc.identifier http://dx.doi.org/10.1007/s12311-012-0354-x
dc.identifier.citation Cerebellum. New York: Springer, v. 11, n. 4, p. 1037-1044, 2012.
dc.identifier.issn 1473-4222
dc.identifier.uri http://repositorio.unifesp.br/handle/11600/35551
dc.description.abstract Cognitive and olfactory impairments have previously been demonstrated in patients with spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD)-SCA3/MJD. We investigated changes in regional cerebral blood flow (rCBF) using single-photon emission computed tomography (SPECT) imaging in a cohort of Brazilian patients with SCA3/MJD. the aim of the present study was to evaluate the correlation among rCBF, cognitive deficits, and olfactory dysfunction in SCA3/MJD. Twenty-nine genetically confirmed SCA3/MJD patients and 25 control subjects were enrolled in the study. the severity of cerebellar symptoms was measured using the International Cooperative Ataxia Rating Scale and the Scale for the Assessment and Rating of Ataxia. Psychiatric symptoms were evaluated by the Hamilton Anxiety Scale and Beck Depression Inventory. the neuropsychological assessment consisted of Spatial Span, Symbol Search, Picture Completion, the Stroop Color Word Test, Trail Making Test (TMT), and Phonemic Verbal Fluency. Subjects were also submitted to odor identification evaluation using the 16-item Sniffin' Sticks. SPECT was performed using ethyl cysteine dimer labeled with technetium-99m. SCA3/MJD patients showed reduced brain perfusion in the cerebellum, temporal, limbic, and occipital lobes compared to control subjects (pFDR < 0.001). A significant positive correlation was found between the Picture Completion test and perfusion of the left parahippocampal gyrus and basal ganglia in the patient group as well as a negative correlation between the TMT part A and bilateral thalamus perfusion. the visuospatial system is affected in patients with SCA3/MJD and may be responsible for the cognitive deficits seen in this disease. en
dc.description.sponsorship Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.description.sponsorship Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
dc.description.sponsorship Instituto Albert Einstein de Ensino e Pesquisa
dc.format.extent 1037-1044
dc.language.iso eng
dc.publisher Springer
dc.relation.ispartof Cerebellum
dc.rights Acesso restrito
dc.subject Machado-Joseph disease en
dc.subject Spinocerebellar ataxia type 3 en
dc.subject Cognition en
dc.subject Olfaction en
dc.subject Cognitive deficits en
dc.subject SPECT en
dc.title Cognitive Deficits in Machado-Joseph Disease Correlate with Hypoperfusion of Visual System Areas en
dc.type Artigo
dc.rights.license http://www.springer.com/open+access/authors+rights?SGWID=0-176704-12-683201-0
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.contributor.institution Universidade Federal do ABC (UFABC)
dc.contributor.institution Univ Estadual Paulista
dc.contributor.institution Univ Western Australia
dc.description.affiliation Universidade Federal de São Paulo, Ataxia Unit, Dept Neurol & Neurosurg, São Paulo, Brazil
dc.description.affiliation Universidade Federal de São Paulo, Behav Neurol Sect, Dept Neurol & Neurosurg, São Paulo, Brazil
dc.description.affiliation UFABC, Ctr Math Comp & Cognit, São Paulo, Brazil
dc.description.affiliation Univ Estadual Paulista, Inst Biosci, São Paulo, Brazil
dc.description.affiliation Universidade Federal de São Paulo, Nucl Med Sect, Dept Radiol, São Paulo, Brazil
dc.description.affiliation Universidade Federal de São Paulo, Lab Interdisciplinar Neurociencias Clin LiNC, Dept Psychiat, São Paulo, Brazil
dc.description.affiliation Univ Western Australia, Med Res Ctr, Western Australian Ctr Hlth & Ageing, Crawley, Australia
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Ataxia Unit, Dept Neurol & Neurosurg, São Paulo, Brazil
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Behav Neurol Sect, Dept Neurol & Neurosurg, São Paulo, Brazil
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Nucl Med Sect, Dept Radiol, São Paulo, Brazil
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Lab Interdisciplinar Neurociencias Clin LiNC, Dept Psychiat, São Paulo, Brazil
dc.identifier.doi 10.1007/s12311-012-0354-x
dc.description.source Web of Science
dc.identifier.wos WOS:000311249400023



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