SynthesisJournal of neurology2021
Natural history of most common spinocerebellar ataxia: a systematic review and meta-analysis.
Synthesis in Journal of neurology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 2 of them syntheses that pooled it.
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
39 citing papers in PubMed, 2 syntheses or guidelines pooled it, 75 citations in OpenAlex.
- The Role of the Human Cerebellum for Learning from and Processing of External Feedback in Non-Motor Learning: A Systematic Review.Cerebellum (London, England) · 2024Pooled it
- Effects of physiotherapy on degenerative cerebellar ataxia: a systematic review and meta-analysis.Frontiers in neurology · 2024Pooled it
- Digital Balance Biomarkers and Interpretable Evaluation of Disease Severity in Spinocerebellar Ataxia Type 3.Annals of the New York Academy of Sciences · 2026Article
- Long-term clinical course and extracerebellar manifestations of hereditary spinocerebellar degeneration.Acta neurologica Belgica · 2026Article
- Early and Progressive Spinal Cord Atrophy in Spinocerebellar Ataxia Type 1.Movement disorders : official journal of the Movement Disorder Society · 2026Article
- Incorporating Patient Perspectives into a Composite Score for Measuring Disease Progression in Spinocerebellar Ataxia (SCA).Neurology and therapy · 2026Article
- Quantitative Assessment of Upper Limb Ataxia Using a Virtual Reality-Based Evaluation System.Annals of clinical and translational neurology · 2026Article
- Long-Term Effects of Annual Intensive Rehabilitation in Patients with Hereditary Pure Cerebellar Ataxia: A 7-year Follow-up Study.Cerebellum (London, England) · 2025Article
- Capture of Longitudinal Change in Real-Life Walking in Cerebellar Ataxia Increases Patient Relevance and Effect Size.Movement disorders : official journal of the Movement Disorder Society · 2025Article
- Longitudinal Changes in Patient- and Clinical-Reported Outcomes in Early Spinocerebellar Ataxia Types 1, 2, 3, and 6 from the IDEA Study.Movement disorders clinical practice · 2025Article
- Scale for the assessment and rating of ataxia: a live e-version.Journal of neurology · 2025Article
- Longitudinal description of health-related quality of life and depressive symptoms in polyQ spinocerebellar ataxia patients.Journal of neurology · 2025Article
- Fatigue Limits Motor and Cognitive Improvements after High-intensity Exercise Prior to Balance Training over Telehealth in People with Spinocerebellar Ataxia.International journal of telerehabilitation · 2025Article
- Differences in the Impact of Intensive Rehabilitation on Hereditary Ataxias and the Cerebellar Subtype of Multiple System Atrophy.Cerebellum (London, England) · 2024Article
- Psychometric Validation of the Modified Functional Scale for the Assessment and Rating of Ataxia (f-SARA) in Patients With Spinocerebellar Ataxia.Cerebellum (London, England) · 2024Article
- Content Validity of the Modified Functional Scale for the Assessment and Rating of Ataxia (f-SARA) Instrument in Spinocerebellar Ataxia.Cerebellum (London, England) · 2024Article
- Factors Influencing Health-Related Quality of Life of Patients with Spinocerebellar Ataxia.Cerebellum (London, England) · 2024Article
- SARA captures disparate progression and responsiveness in spinocerebellar ataxias.Journal of neurology · 2024Article
- Phenotypical, genotypical and pathological characterization of the moonwalker mouse, a model of ataxia.Neurobiology of disease · 2024Article
- Health-Related Quality of Life in Patients with Spinocerebellar Ataxia: a Validation Study of the EQ-5D-3L.Cerebellum (London, England) · 2024Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSpinocerebellar ataxias (SCAs) are rare dominantly inherited neurodegenerative disorders that lead to severe disability and premature death.
objectiveTo better characterize the natural history of the most common SCAs, SCA1, SCA2, SCA3 and SCA6, we performed a meta-analysis of literature to determine disease progression, provide data for sample-sizes calculations for interventional trials and study the impact of geographical locations and study follow-up on disease progression.
methodsA systematic literature search from MEDLINE and EMBASE databases for longitudinal natural history studies of SCA patients was conducted. Studies using the Scale for the Assessment and Rating Ataxia (SARA) as outcome measure were considered. Random-effect (RE) meta-analysis was applied to estimate pooled disease progression.
resultsSix studies with 1215 SCA patients enrolled between 2005 and 2016 were finally selected. Annual pooled SARA score increase was 1.83 (1.46-2.20) in patients with SCA1, 1.40 (1.19-1.61) in patients with SCA2, 1.41 (0.97-1.84) in patients with SCA3, and 0.81 (0.66-0.97) in patients with SCA6. For patients with SCA3, disease progression was faster in studies located in Asia and Europe than in the US. Two-arm interventional trials of 1-year duration to achieve 80% power and α level of 5% would require 92 patients per group with SCA1, 97 with SCA2, 115 with SCA3, and 430 with SCA6 to detect a 50% reduction in disease progression.
conclusionThis meta-analysis provides quantitative data on the progression of the most common spinocerebellar ataxias based on patient numbers that exceed those of previous studies and confirms that disease progression is faster in SCA1, intermediate in SCA2 and SCA3 and slower in SCA6, with similar rates of disease progression in SCA2 and SCA3 between different populations, suggesting a possibility of international collaborative studies. Nevertheless, individual-patient data meta-analysis is needed to better understand the risk factors that influence disease progression and improve patient stratification in interventional trials.
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