ArticleCerebellum (London, England)2012
Cognitive deficits in Machado-Joseph disease correlate with hypoperfusion of visual system areas.
Article in Cerebellum (London, England), 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 34 citations in OpenAlex.
- Altered static and dynamic spontaneous brain activity patterns in spinocerebellar ataxia type3 patients.Journal of neurology · 2025Article
- Cognitive, Emotional, and Other Non-motor Symptoms of Spinocerebellar Ataxias.Current neurology and neuroscience reports · 2024Review
- Cerebello-cerebral resting-state functional connectivity in spinocerebellar ataxia type 3.Human brain mapping · 2023Article
- The CCAS-scale in hereditary ataxias: helpful on the group level, particularly in SCA3, but limited in individual patients.Journal of neurology · 2022Article
- A resting-state fMRI pattern of spinocerebellar ataxia type 3 and comparison withNeuroImage. Clinical · 2022Article
- Altered cerebral blood flow in patients with spinocerebellar degeneration.Frontiers in neuroscience · 2022Article
- Diffusion Tensor Magnetic Resonance Imaging for Differentiating Multiple System Atrophy Cerebellar Type and Spinocerebellar Ataxia Type 3.Brain sciences · 2019Article
- Cognitive and Psychiatric Evaluation in SYNE1 Ataxia.Cerebellum (London, England) · 2019Article
- Intra- and Inter-Modular Connectivity Alterations in the Brain Structural Network of Spinocerebellar Ataxia Type 3.Entropy (Basel, Switzerland) · 2019Article
- Executive dysfunction in patients with spinocerebellar ataxia type 3.Journal of neurology · 2018Article
- The cerebral metabolic topography of spinocerebellar ataxia type 3.NeuroImage. Clinical · 2018Article
- Psychosis in Machado-Joseph Disease: Clinical Correlates, Pathophysiological Discussion, and Functional Brain Imaging. Expanding the Cerebellar Cognitive Affective Syndrome.Cerebellum (London, England) · 2016Article
- A longitudinal investigation into cognition and disease progression in spinocerebellar ataxia types 1, 2, 3, 6, and 7.Orphanet journal of rare diseases · 2016Article
- Consensus paper: radiological biomarkers of cerebellar diseases.Cerebellum (London, England) · 2015Article
- Neurophysiological studies and non-motor symptoms prior to ataxia in a patient with machado-joseph disease: trying to understand the natural history of brain degeneration.Cerebellum (London, England) · 2014Article
- Widespread neuronal damage and cognitive dysfunction in spinocerebellar ataxia type 3.Journal of neurology · 2013Article
- Association between spinocerebellar ataxias caused by glutamine expansion and psychiatric and neuropsychological signals - a literature review.American journal of neurodegenerative disease · 2013Article
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Authors and funding
12 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
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.
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