ArticleJournal of neurology2021
The cerebellar cognitive affective syndrome scale reveals early neuropsychological deficits in SCA3 patients.
Article 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 34 papers, 1 of them a synthesis that pooled it.
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Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Prevalence of Cerebellar Cognitive Affective Syndrome in Patients with Cerebellar Disorders: A Systematic Review and Meta-Analysis.Cerebellum (London, England) · 2026Pooled it
- 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
- Spatial perspective taking is impaired in spinocerebellar ataxias and Friedreich ataxia.Scientific reports · 2025Article
- The cerebellum and psychiatric disorders: unraveling its role in mental health.Arquivos de neuro-psiquiatria · 2025Review
- The Italian Standardization of the Cerebellar Cognitive Affective/Schmahmann Syndrome Scale: Cognitive Profiling in a Healthy, Heterogeneous Population.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
- Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10.PloS one · 2025Article
- Psychomotor and non-motor correlates of cognition in spinocerebellar ataxias Types 1, 2, 3, and 6.Brain communications · 2025Article
- Feasibility of repetitive transcranial magnetic stimulation on non-motor symptoms of spinocerebellar ataxia type 3: a secondary analysis of a randomized clinical trial.Frontiers in 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
- Neuropsychiatric Symptoms in Rhombencephalosynapsis: A Clinical Report.Cerebellum (London, England) · 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
- Assessment of the reliability, responsiveness, and meaningfulness of the scale for the assessment and rating of ataxia (SARA) for lysosomal storage disorders.Journal of neurology · 2024Article
- A combination of chlorzoxazone and folic acid improves recognition memory, anxiety and depression in SCA3-84Q mice.Human molecular genetics · 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 Complaints and Their Impact on Daily Life in Patients with Degenerative Cerebellar Disorders.Cerebellum (London, England) · 2024Article
- New Insights into the Neuropsychological Profile and Intellectual Quotient Variability in Joubert Syndrome Compared to Other Congenital Cerebellar Malformations.Cerebellum (London, England) · 2024Article
Corrections and comments
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Authors and funding
4 authors.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundThe cerebellar cognitive affective syndrome scale (CCAS-S) was recently developed to detect specific neuropsychological deficits in patients with cerebellar diseases in an expedited manner.
objectivesTo evaluate the discriminative ability of the CCAS-S in an etiologically homogeneous cohort of spinocerebellar ataxia type 3 (SCA3) patients and to examine relationships between cognitive deficits and motor symptom severity.
methodsThe CCAS-S was administered to twenty mildly to moderately affected SCA3 patients and eighteen healthy controls matched for age, sex, and educational level. Disease severity was measured by the Scale for the Assessment and Rating of Ataxia (SARA), Inventory of Non-Ataxia Signs (INAS), 8 m walk test, nine-hole peg test (9HPT), and Patient Health Questionnaire-9 (PHQ-9).
resultsSCA3 patients had a lower total CCAS-S score (p < 0.001) and higher number of failed tests (p = 0.006) than healthy controls. Patients displayed impairments in semantic fluency, phonemic fluency, category switching, cube drawing, and affect regulation. Total CCAS-S score showed high discriminative ability (area under the curve [AUC]: 0.96) and was associated with disease duration, SARA score, walking speed, and dominant hand 9HPT performance. No correlations were observed with INAS count, repeat length, and PHQ-9 score. Discriminative capacity of the number of failed tests was moderate (AUC: 0.76).
conclusionEssentially all SCA3 patients exhibited some form of cognitive impairment. The CCAS-S differentiates SCA3 patients from healthy controls, detects neuropsychological deficits early in the disease course, and correlates with relevant ataxia severity measures.
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