ArticleActa neurologica Scandinavica2021
In vivo microstructural white matter changes in early spinocerebellar ataxia 2.
Article in Acta neurologica Scandinavica, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 15 citations in OpenAlex.
- Novel Imaging Phenotype in Spinal Cerebellar Ataxia Type 8: Symmetrical White Matter Changes without Cerebellar Atrophy.Cerebellum (London, England) · 2026Article
- Article
- Bioinformatic Analyses of the Ataxin-2 Family Since Algae Emphasize Its Small Isoforms, Large Chimerisms, and the Importance of Human Exon 1B as Target of Therapies to Prevent Neurodegeneration.International journal of molecular sciences · 2026Article
- Repeat Expansions in a Chilean Cohort with Adult-Onset Cerebellar Ataxia.Cerebellum (London, England) · 2025Article
- Multiomics approach identifies SERPINB1 as candidate biomarker for spinocerebellar ataxia type 2.Scientific reports · 2025Article
- Nerve Fiber Bundle Damage in Spinocerebellar Degeneration on Diffusion Tensor Imaging.Current medical imaging · 2025Article
- Involvement of the Superior Cerebellar Peduncles in GAA-Neurology. Genetics · 2025Article
- Peak width of skeletonized mean diffusivity: a novel biomarker for white matter damage in spinocerebellar ataxia type 2.Neuroradiology · 2025Article
- A comprehensive review of iPS cell line-based disease modelling of the polyglutamine spinocerebellar ataxias 2 and 3: a focus on the research outcomes.Annals of medicine and surgery (2012) · 2024Review
- Neuroradiological findings in GAA-medRxiv : the preprint server for health sciences · 2024Article
- Progressive white matter degeneration in patients with spinocerebellar ataxia type 2.Neuroradiology · 2024Article
- Management of essential tremor deep brain stimulation-induced side effects.Frontiers in human neuroscience · 2024Review
- Myelinating Glia: Potential Therapeutic Targets in Polyglutamine Spinocerebellar Ataxias.Cells · 2023Review
- Brain structural abnormalities in the preclinical stage of Machado-Joseph disease/spinocerebellar ataxia type 3 (MJD/SCA3): evaluation by MRI morphometry, diffusion tensor imaging and neurite orientation dispersion and density imaging.Journal of neurology · 2022Article
- Cerebellar White Matter Abnormalities in Charcot-Marie-Tooth Disease: A Combined Volumetry and Diffusion Tensor Imaging Analysis.Journal of clinical medicine · 2021Article
- Cognitive impairment and its neuroimaging correlates in spinocerebellar ataxia 2.Parkinsonism & related disorders · 2021Article
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
objectiveWhite matter (WM) integrity of Spinocerebellar ataxia 2 (SCA2) is poorly understood, more so in the early stages of SCA2. In this study, we evaluated the microstructural integrity of the WM tracts with an emphasis on the nature of in vivo pathological involvement in early SCA2. MATERIALS AND
methodsWe evaluated the MRI images of 26 genetically proven SCA2 patients with disease duration <5 years and 24 age- and gender-matched healthy controls using tract-based spatial statistics (TBSS) to identify the WM tract changes and their clinico-genetic correlates (age at onset, duration of disease, ataxia severity and CAG repeat length) using standard methodology.
resultsThe mean age at onset and duration of disease were 28.7 ± 8.51 years and 3.5 ± 0.69 months, respectively. The mean CAG repeat length was 42.5 ± 4.6, and the ataxia severity score was 16.1 ± 4.9. Altered DTI scalars signifying degeneration was present in the bilateral anterior thalamic radiation (ATR), corticospinal tract (CST), inferior fronto-occipital fasciculus (IFOF), superior and inferior longitudinal fasciculus (SLF and ILF), uncinate fasciculus (UF), cingulum, corpus callosum (CC), forceps major and forceps minor (corrected p < .05). DTI scalars representing demyelination was seen in the superior cerebellar peduncle (SCP) and cerebellar WM. There was a significant correlation of SARA score with axial diffusivity of the bilateral cingulum, ATR, CST, forceps minor, IFOF, ILF, SLF and SCP on the right side (corrected p < .05).
conclusionExtensive WM involvement is present in early SCA2. The DTI scalars indicate degeneration and demyelination and may have clinical implications.
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