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Title: Cognitive Deficits in Machado-Joseph Disease Correlate with Hypoperfusion of Visual System Areas
Authors: Braga-Neto, Pedro [UNIFESP]
Dutra, Livia Almeida [UNIFESP]
Pedroso, Jose Luiz [UNIFESP]
Felicio, Andre C. [UNIFESP]
Alessi, Helena [UNIFESP]
Santos-Galduroz, Ruth F.
Bertolucci, Paulo Henrique F. [UNIFESP]
Castiglioni, Mario Luiz V. [UNIFESP]
Bressan, Rodrigo Affonseca [UNIFESP]
Jara de Garrido, Griselda Esther
Barsottini, Orlando Graziani Povoas [UNIFESP]
Jackowski, Andrea [UNIFESP]
Universidade Federal de São Paulo (UNIFESP)
Universidade Federal do ABC (UFABC)
Univ Estadual Paulista
Univ Western Australia
Keywords: Machado-Joseph disease
Spinocerebellar ataxia type 3
Cognitive deficits
Issue Date: 1-Dec-2012
Publisher: Springer
Citation: Cerebellum. New York: Springer, v. 11, n. 4, p. 1037-1044, 2012.
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.
ISSN: 1473-4222
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