ArticleCerebellum (London, England)2022
Cognitive Decline Is Closely Associated with Ataxia Severity in Spinocerebellar Ataxia Type 2: a Validation Study of the Schmahmann Syndrome Scale.
Article in Cerebellum (London, England), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 47 citations in OpenAlex.
- Remote Online-Based Application of the Cerebellar Cognitive Affective Syndrome Scale.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
- Validation of the SODA in Spinocerebellar Ataxia Type 2.Cerebellum (London, England) · 2026Article
- Cerebellar clinical syndromes: the triad and rating scales.Journal of neurology · 2026Review
- Two-year decline in performance on the Cerebellar Cognitive Affective Syndrome Scale in spinocerebellar ataxias.Journal of neurology · 2025Article
- Understanding cognitive features of cervical dystonia: application of the cerebellar cognitive affective syndrome scale (CCAS-S).Journal of neural transmission (Vienna, Austria : 1996) · 2025Article
- Prevalence, Severity, and Progression of Cerebellar Cognitive-Affective Syndrome in Patients With Spinocerebellar Ataxias.Neurology · 2025Article
- Spatial perspective taking is impaired in spinocerebellar ataxias and Friedreich ataxia.Scientific reports · 2025Article
- The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale: Updates and Insights.Cerebellum (London, England) · 2025Article
- The Cerebellar Neuropsychiatric Rating Scale in the Spinocerebellar Ataxias.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
- Depression and Associated Factors Among Patients with Spinocerebellar Ataxia.Medicina (Kaunas, Lithuania) · 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
- Reliability and validity study of the Chinese version of the Cerebellar Cognitive Affective Syndrome Scale in patients with cerebellar injury.Acta neurologica Belgica · 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
- Cognitive-Affective Functions of the Cerebellum.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2023Article
- Ferdinando Rossi Lecture: the Cerebellar Cognitive Affective Syndrome-Implications and Future Directions.Cerebellum (London, England) · 2023Review
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Authors and funding
11 authors at 4 institutions in 2 countries.
Funding
Abstract
The cerebellar cognitive affective syndrome scale (CCAS-S) was designed to detect specific cognitive dysfunctions in cerebellar patients but is scarcely validated in spinocerebellar ataxias (SCA). The objective of this study is to determine the usefulness of the CCAS-S in a Cuban cohort of SCA2 patients and the relationship of its scores with disease severity. The original scale underwent a forward and backward translation into Spanish language, followed by a pilot study to evaluate its comprehensibility. Reliability, discriminant, and convergent validity assessments were conducted in 64 SCA2 patients and 64 healthy controls matched for sex, age, and education. Fifty patients completed the Montreal Cognitive Assessment (MoCA) test. The CCAS-S showed an acceptable internal consistency (Cronbach's alpha = 0.74) while its total raw score and the number of failed tests showed excellent (ICC = 0.94) and good (ICC = 0.89) test-retest reliability, respectively. Based on original cut-offs, the sensitivity of CCAS-S to detect possible/probable/definite CCAS was notably high (100%/100%/91%), but specificities were low (6%/30/64%) because the decreased specificity observed in four items. CCAS-S performance was significantly influenced by ataxia severity in patients and by education in both groups. CCAS-S scores correlated with MoCA scores, but showed higher sensitivity than MoCA to detect cognitive impairments in patients. The CCAS-S is particularly useful to detect cognitive impairments in SCA2 but some transcultural and/or age and education-dependent adaptations could be necessary to improve its diagnostic properties. Furthermore, this scale confirmed the parallelism between cognitive and motor deficits in SCA2, giving better insights into the disease pathophysiology and identifying novel outcomes for clinical trials.
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