ArticleJournal of neurology2022
The CCAS-scale in hereditary ataxias: helpful on the group level, particularly in SCA3, but limited in individual patients.
Article in Journal of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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22 citing papers in PubMed, 31 citations in OpenAlex.
- Remote Online-Based Application of the Cerebellar Cognitive Affective Syndrome Scale.Cerebellum (London, England) · 2026Article
- Cerebellar Cognitive Affective Syndrome in Spinocerebellar Ataxia Type 6.Cerebellum (London, England) · 2026Article
- The Cerebellar Cognitive-Affective Syndrome Scale Reveals Consistent, Early, and Progressive Neuropsychological Deficits in Autosomal-Recessive Spastic Ataxia of Charlevoix-Saguenay: A Large International Cross-Sectional Study.Movement disorders : official journal of the Movement Disorder Society · 2026Article
- Cerebellar clinical syndromes: the triad and rating scales.Journal of neurology · 2026Review
- The Medication Patterns of Spinocerebellar Ataxia Type 3 Mutation Carriers Enrolled in the ESMI Cohort.CNS drugs · 2026Article
- Two-year decline in performance on the Cerebellar Cognitive Affective Syndrome Scale in spinocerebellar ataxias.Journal of neurology · 2025Article
- Prevalence, Severity, and Progression of Cerebellar Cognitive-Affective Syndrome in Patients With Spinocerebellar Ataxias.Neurology · 2025Article
- Impaired reinforcement learning and coding of prediction errors in patients with cerebellar degeneration - a study with EEG and voxel-based morphometry.Cognitive, affective & behavioral neuroscience · 2025Article
- The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale: Updates and Insights.Cerebellum (London, England) · 2025Article
- Optimizing selectivity of the Cerebellar Cognitive Affective Syndrome Scale by use of correction formulas, and validation of its German version.Journal of neurology · 2025Article
- Psychomotor and non-motor correlates of cognition in spinocerebellar ataxias Types 1, 2, 3, and 6.Brain communications · 2025Article
- Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10.PloS one · 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 Role of Verbal Fluency in the Cerebellar Cognitive Affective Syndrome Scale in Friedreich Ataxia.Cerebellum (London, England) · 2024Article
- The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale in Spinocerebellar Ataxias.Cerebellum (London, England) · 2024Article
- A Comparative Study on Cognitive Assessment in Cerebellar and Supratentorial Stroke.Brain sciences · 2024Article
- Consensus Recommendations for Clinical Outcome Assessments and Registry Development in Ataxias: Ataxia Global Initiative (AGI) Working Group Expert Guidance.Cerebellum (London, England) · 2024Review
- Cognitive impairment associated with cerebellar volume loss in spinocerebellar ataxia type 3.Journal of neurology · 2024Article
- Cerebellar Degeneration Impairs Strategy Discovery but Not Strategy Recall.Cerebellum (London, England) · 2023Article
- Cognitive-Affective Functions of the Cerebellum.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2023Article
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Authors and funding
18 authors at 8 institutions in 2 countries.
Funding
Abstract
backgroundA brief bedside test has recently been introduced by Hoche et al. (Brain, 2018) to screen for the Cerebellar Cognitive Affective Syndrome (CCAS) in patients with cerebellar disease.
objectiveThis multicenter study tested the ability of the CCAS-Scale to diagnose CCAS in individual patients with common forms of hereditary ataxia.
methodsA German version of the CCAS-Scale was applied in 30 SCA3, 14 SCA6 and 20 FRDA patients, and 64 healthy participants matched for age, sex, and level of education. Based on original cut-off values, the number of failed test items was assessed, and CCAS was considered possible (one failed item), probable (two failed items) or definite (three failed items). In addition a total sum raw score was calculated.
resultsOn a group level, failed items were significantly higher and total sum scores were significantly lower in SCA3 patients compared to matched controls. SCA6 and FRDA patients performed numerically below controls, but respective group differences failed to reach significance. The ability of the CCAS-Scale to diagnose CCAS in individual patients was limited to severe cases failing three or more items. Milder cases failing one or two items showed a great overlap with the performance of controls exhibiting a substantial number of false-positive test results. The word fluency test items differentiated best between patients and controls.
conclusionsAs a group, SCA3 patients performed below the level of SCA6 and FRDA patients, possibly reflecting additional cerebral involvement. Moreover, the application of the CCAS-Scale in its present form results in a high number of false-positive test results, that is identifying controls as patients, reducing its usefulness as a screening tool for CCAS in individual patients.
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