ArticleMovement disorders clinical practice2025
Cognitive Changes in Pre-ataxic Spinocerebellar Ataxias: A Scoping Review.
Article in Movement disorders clinical practice, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
3 citing papers in PubMed.
- SCA27B in Brazil: frequency, phenotype and genotype-phenotype correlations.Journal of neurology · 2026Article
- Targeting Cognitive Dysfunction in Spinocerebellar Ataxia Type 2 Through Digital Cognitive Training.Cerebellum (London, England) · 2026Article
- Cognitive Deficits in Spinocerebellar Ataxia Type 2: A Comparative Analysis of Pre-ataxic and Ataxic Stages.Cerebellum (London, England) · 2025Article
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAlthough traditionally recognized for motor impairment, evidence suggests that cognitive deficits may emerge before ataxia onset in autosomal dominant spinocerebellar ataxias (SCA), particularly in nucleotide repeat expansion SCAs (NRE-SCAs). However, the nature and extent of these early cognitive changes and cognition disorders remain unclear.
objectiveThis scoping review maps existing evidence on cognitive alterations in pre-ataxic NRE-SCAs, focusing on affected cognitive domains, assessment tools, and early biomarkers.
methodsA comprehensive search in PubMed, EMBASE, and CINAHL was performed in accordance with Joanna Briggs Institute guidelines. Studies were included if they assessed cognitive function in genetically confirmed pre-ataxic NRE-SCA individuals using neuropsychological tests, imaging, or biomarkers. Data extraction comprised study design, cognitive domains assessed, and key findings.
resultsThirteen studies met inclusion criteria, examining pre-ataxic individuals with SCA1, SCA2, SCA3, and SCA36. Executive dysfunction, particularly in cognitive flexibility, inhibitory control, and working memory, was the most frequent finding, assessed using the Trail Making Test, Stroop Test, and phonemic fluency tasks. Processing speed deficits were also commonly reported. Pre-ataxic SCA2 exhibited the most consistent impairments, whereas findings in pre-ataxic SCA1 and pre-ataxic SCA3 were variable. Neuroimaging studies revealed early cerebellar microstructural changes linked to cognitive dysfunction.
conclusionCognitive impairments may precede motor symptoms in NRE-SCAs, particularly SCA2. However, methodological heterogeneity and small sample sizes limit definitive conclusions. Standardized assessments and longitudinal studies are needed to clarify cognitive decline trajectories and their potential as early biomarkers.
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