ArticleCerebellum & ataxias2017
Neural correlates of ataxia severity in spinocerebellar ataxia type 3/Machado-Joseph disease.
Article in Cerebellum & ataxias, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 2 of them syntheses that pooled it.
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Who cites it
13 citing papers in PubMed, 2 syntheses or guidelines pooled it, 30 citations in OpenAlex.
- Voxel-based meta-analysis of gray matter and white matter changes in patients with spinocerebellar ataxia type 3.Frontiers in neurology · 2023Pooled it
- Magnetic Resonance Imaging and Its Clinical Correlation in Spinocerebellar Ataxia Type 3: A Systematic Review.Frontiers in neuroscience · 2022Pooled it
- A Severity-Agnostic Atrophy Pattern in Spinocerebellar Ataxia Type 3: Volumetrics from ENIGMA-Ataxia.Movement disorders : official journal of the Movement Disorder Society · 2026Article
- Global and Regional Brain Grey and White Matter Morphometry Alterations in Type 1, 2, and 3 Spinocerebellar Ataxias (SCAs) Patients.Cerebellum (London, England) · 2024Article
- Altered large-scale individual-based morphological brain network in spinocerebellar ataxia type 3.CNS neuroscience & therapeutics · 2023Article
- Case report: Short-term efficacy and changes inFrontiers in neurology · 2023Article
- Brain MRI Volumetry Analysis in an Indonesian Family of SCA 3 Patients: A Case-Based Study.Frontiers in neurology · 2022Article
- Neuromelanin imaging analyses of the substantia nigra in patients with Machado-Joseph disease.Neuroradiology · 2020Article
- Cerebellum and cognition in Friedreich ataxia: a voxel-based morphometry and volumetric MRI study.Journal of neurology · 2020Article
- MR Imaging of SCA3/MJD.Frontiers in neuroscience · 2020Review
- Impaired Adaptive Motor Learning Is Correlated With Cerebellar Hemispheric Gray Matter Atrophy in Spinocerebellar Ataxia Patients: A Voxel-Based Morphometry Study.Frontiers in neurology · 2019Article
- Unique degeneration signatures in the cerebellar cortex for spinocerebellar ataxias 2, 3, and 7.NeuroImage. Clinical · 2018Article
- The cerebral metabolic topography of spinocerebellar ataxia type 3.NeuroImage. Clinical · 2018Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSpinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD) is an autosomal dominant inherited neurodegenerative disorder. Several post-mortem and imaging studies have shown cerebellar and brainstem atrophy. A number of studies have used volumetric regional information to investigate the relationship between neurodegeneration and the ataxia severity. However, regional analysis can obscure the specific location in which the degenerative process is affecting the brain tissue, which can be crucial for the development of new target treatments for this disease. Here we explored the relationship between the gray matter degeneration and the ataxia severity on a cohort of SCA3 patients using a voxel-wise approach.
methodsSeventeen patients with molecular diagnose of SCA3 and 17 matched healthy controls participated in this study. Magnetic resonance imaging (MRI) brain images were acquired and voxel-based morphometry was used to obtain the grey matter volume of each participant. Ataxia severity in the patient group was evaluated using the scale for the assessment and rating of ataxia (SARA).
resultsGroup comparison revealed significant atrophy in SCA3 including bilateral cerebellum, vermis, brainstem, and occipital cortex. Significant negative correlations between gray matter volume and SARA scores were found in the cerebellum and the cingulate gyrus.
conclusionsThese findings highlight the specific contribution of the cerebellum and the cingulate cortex to the ataxia deficits among the other regions showing neurodegeneration in SCA3 patients.
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