ReviewBrain sciences2017
Cognitive Changes in the Spinocerebellar Ataxias Due to Expanded Polyglutamine Tracts: A Survey of the Literature.
Review in Brain sciences, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers, 4 of them syntheses that pooled it.
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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
31 citing papers in PubMed, 4 syntheses or guidelines pooled it, 42 citations in OpenAlex.
- Cognition in cerebellar disorders: What's in the profile? A systematic review and meta-analysis.Journal of neurology · 2025Pooled it
- Voxel-based meta-analysis of gray matter and white matter changes in patients with spinocerebellar ataxia type 3.Frontiers in neurology · 2023Pooled it
- Neurocognitive Changes in Spinocerebellar Ataxia Type 3: A Systematic Review with a Narrative Design.Cerebellum (London, England) · 2022Pooled 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
- Abnormal Local Brain Activity in SCA3 Patients with Cognitive Impairment: A Resting-State Functional MRI Study.Cerebellum (London, England) · 2026Article
- Spinocerebellar Ataxia Type 12: Spectrums of Movement Disorders and Clinical Features.Neuro-degenerative diseases · 2026Review
- Translational Relevance of SCA1 Models for the Development of Therapies for Spinocerebellar Ataxia Type 1.Biomedicines · 2025Review
- Cognitive Changes in Pre-ataxic Spinocerebellar Ataxias: A Scoping Review.Movement disorders clinical practice · 2025Article
- The cognitive cerebellum: a hub for motor, emotional, and executive control.Arquivos de neuro-psiquiatria · 2025Review
- Cognitive Deficits in Spinocerebellar Ataxia Type 2: A Comparative Analysis of Pre-ataxic and Ataxic Stages.Cerebellum (London, England) · 2025Article
- Prevalence, Severity, and Progression of Cerebellar Cognitive-Affective Syndrome in Patients With Spinocerebellar Ataxias.Neurology · 2025Article
- Investigating ITM2B-associated ataxia in a Taiwanese cerebellar ataxia cohort.Annals of clinical and translational neurology · 2025Article
- Subtypes of cognitive impairment in cerebellar disease identified by cross-diagnostic cluster-analysis: results from a German multicenter study.Journal of neurology · 2024Article
- The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale in Spinocerebellar Ataxias.Cerebellum (London, England) · 2024Article
- Cognitive, Emotional, and Other Non-motor Symptoms of Spinocerebellar Ataxias.Current neurology and neuroscience reports · 2024Review
- Altered large-scale individual-based morphological brain network in spinocerebellar ataxia type 3.CNS neuroscience & therapeutics · 2023Article
- Neurophysiology of cerebellar ataxias and gait disorders.Clinical neurophysiology practice · 2023Review
- Neurocognitive and cerebellar function in ADHD, autism and spinocerebellar ataxia.Frontiers in systems neuroscience · 2023Review
- Cognitive Decline Is Closely Associated with Ataxia Severity in Spinocerebellar Ataxia Type 2: a Validation Study of the Schmahmann Syndrome Scale.Cerebellum (London, England) · 2022Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The dominantly-inherited ataxias characterised by expanded polyglutamine tracts-spinocere bellar ataxias (SCAs) 1, 2, 3, 6, 7, 17, dentatorubral pallidoluysian atrophy (DRPLA) and, in part, SCA 8-have all been shown to result in various degrees of cognitive impairment. We survey the literature on the cognitive consequences of each disorder, attempting correlation with their published neuropathological, magnetic resonance imaging (MRI) and clinical features. We suggest several psychometric instruments for assessment of executive function, whose results are unlikely to be confounded by visual, articulatory or upper limb motor difficulties. Finally, and with acknowledgement of the inadequacies of the literature to date, we advance a tentative classification of these disorders into three groups, based on the reported severity of their cognitive impairments, and correlated with their neuropathological topography and MRI findings: group 1-SCAs 6 and 8-mild dysexecutive syndrome based on disruption of cerebello-cortical circuitry; group 2-SCAs 1, 2, 3, and 7-more extensive deficits based largely on disruption of striatocortical in addition to cerebello-cerebral circuitry; and group 3-SCA 17 and DRPLA-in which cognitive impairment severe enough to cause a dementia syndrome is a frequent feature.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.