ArticleCerebellum (London, England)2020
Cerebellar Cognitive Affective Syndrome in Costa da Morte Ataxia (SCA36).
Article in Cerebellum (London, England), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers, 1 of them a synthesis that pooled it.
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Who cites it
6 citing papers in PubMed, 1 synthesis or guideline pooled it, 11 citations in OpenAlex.
- Cognition in cerebellar disorders: What's in the profile? A systematic review and meta-analysis.Journal of neurology · 2025Pooled it
- Cognitive Changes in Pre-ataxic Spinocerebellar Ataxias: A Scoping Review.Movement disorders clinical practice · 2025Article
- Cognitive Deficits in Spinocerebellar Ataxia Type 2: A Comparative Analysis of Pre-ataxic and Ataxic Stages.Cerebellum (London, England) · 2025Article
- The Cerebellar Neuropsychiatric Rating Scale in the Spinocerebellar Ataxias.Cerebellum (London, England) · 2025Article
- Spinocerebellar Ataxia 36 is a Frequent Cause of Hereditary Ataxia in Eastern Spain.Movement disorders clinical practice · 2023Article
- Identification of the Largest SCA36 Pedigree in Asia: with Multimodel Neuroimaging Evaluation for the First Time.Cerebellum (London, England) · 2022Article
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
Abstract
SCA36 is an autosomal dominant spinocerebellar ataxia (SCA) affecting many families from Costa da Morte, a northwestern region of Spain. It is caused by an intronic GGCCTG repeat expansion in NOP56. In order to characterize the cognitive and affective manifestations of this cerebellar disease, a group of 30 SCA36 mutation carriers (11 preataxic and 19 ataxic patients) were assessed with a comprehensive battery of standardized tests. Phonological verbal fluency - but not semantic fluency - was already mildly impaired in preataxic subjects. In ataxic patients, both phonological and semantic fluencies were significantly below normal. Depression, while more frequent and prominent in ataxic patients, was also often present in the preataxic stage. This is the first systematic study supporting the presence of a mild cerebellar cognitive and affective syndrome in SCA36. Routine evaluation of cognitive and emotional spheres in SCA36 patients as well as asymptomatic mutation carriers should allow early detection and timely therapeutic intervention.
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