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Published in: Current Neurology and Neuroscience Reports 3/2024

25-01-2024 | Spinocerebellar Ataxia | Review

Cognitive, Emotional, and Other Non-motor Symptoms of Spinocerebellar Ataxias

Authors: Chi-Ying R. Lin, Sheng-Han Kuo, Puneet Opal

Published in: Current Neurology and Neuroscience Reports | Issue 3/2024

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Abstract

Purpose of Review

Spinocerebellar ataxias (SCAs) are autosomal dominant degenerative syndromes that present with ataxia and brain stem abnormalities. This review describes the cognitive and behavioral symptoms of SCAs in the context of recent knowledge of the role of the cerebellum in higher intellectual function.

Recent Findings

Recent studies suggest that patients with spinocerebellar ataxia can display cognitive deficits even early in the disease. These have been given the term cerebellar cognitive affective syndrome (CCAS). CCAS can be tracked using newly developed rating scales. In addition, patients with spinocerebellar ataxia also display impulsive and compulsive behavior, depression, anxiety, fatigue, and sleep disturbances.

Summary

This review stresses the importance of recognizing non-motor symptoms in SCAs. There is a pressing need for novel therapeutic interventions to address these symptoms given their deleterious impact on patients’ quality of life.
Literature
19.
go back to reference •• GPD A, van Dun K, Adamaszek M, et al. The cerebellar cognitive affective/Schmahmann syndrome: a task force paper. Cerebellum. 2020;19(1):102–25. https://doi.org/10.1007/s12311-019-01068-8. This consensus paper summarizes a constellation of important symptoms and neuroimaigng findings in the cerebellar cognitive affective syndrome.CrossRef •• GPD A, van Dun K, Adamaszek M, et al. The cerebellar cognitive affective/Schmahmann syndrome: a task force paper. Cerebellum. 2020;19(1):102–25. https://​doi.​org/​10.​1007/​s12311-019-01068-8. This consensus paper summarizes a constellation of important symptoms and neuroimaigng findings in the cerebellar cognitive affective syndrome.CrossRef
21.
go back to reference Kano M, Watanabe M. Chapter 4 - Cerebellar circuits. In: Rubenstein J, Rakic P, Chen B, Kwan KY, editors. Neural Circuit and Cognitive Development (Second Edition). Academic Press; 2020. p. 79–102.CrossRef Kano M, Watanabe M. Chapter 4 - Cerebellar circuits. In: Rubenstein J, Rakic P, Chen B, Kwan KY, editors. Neural Circuit and Cognitive Development (Second Edition). Academic Press; 2020. p. 79–102.CrossRef
49.
go back to reference •• Amokrane N, Viswanathan A, Freedman S, et al. Impulsivity in cerebellar ataxias: testing the cerebellar reward hypothesis in humans. Mov Disord. 2020;35(8):1491–3. https://doi.org/10.1002/mds.28121. This article reveals the human evidence of domain-specific impulsive and compulsive symptoms in individuals with dysfunctional cerebellum, suggesting that an intact cerebellum is important for normal reward system.CrossRefPubMedPubMedCentral •• Amokrane N, Viswanathan A, Freedman S, et al. Impulsivity in cerebellar ataxias: testing the cerebellar reward hypothesis in humans. Mov Disord. 2020;35(8):1491–3. https://​doi.​org/​10.​1002/​mds.​28121. This article reveals the human evidence of domain-specific impulsive and compulsive symptoms in individuals with dysfunctional cerebellum, suggesting that an intact cerebellum is important for normal reward system.CrossRefPubMedPubMedCentral
50.
52.
go back to reference • Lin CR, Amokrane N, Chen S, et al. Cerebellar impulsivity-compulsivity assessment scale. Ann Clin Transl Neurol. 2022; https://doi.org/10.1002/acn3.51698. The authors developed a novel rating scale to assess ICBs in cerebellar ataxia and broaden our understanding of the cerebellum-related cognitive and behavioral symptoms. • Lin CR, Amokrane N, Chen S, et al. Cerebellar impulsivity-compulsivity assessment scale. Ann Clin Transl Neurol. 2022; https://​doi.​org/​10.​1002/​acn3.​51698. The authors developed a novel rating scale to assess ICBs in cerebellar ataxia and broaden our understanding of the cerebellum-related cognitive and behavioral symptoms.
54.
go back to reference Ma J, Wu C, Lei J, Zhang X. Cognitive impairments in patients with spinocerebellar ataxia types 1, 2 and 3 are positively correlated to the clinical severity of ataxia symptoms. Int J Clin Exp Med. 2014;7(12):5765–71.PubMedPubMedCentral Ma J, Wu C, Lei J, Zhang X. Cognitive impairments in patients with spinocerebellar ataxia types 1, 2 and 3 are positively correlated to the clinical severity of ataxia symptoms. Int J Clin Exp Med. 2014;7(12):5765–71.PubMedPubMedCentral
Metadata
Title
Cognitive, Emotional, and Other Non-motor Symptoms of Spinocerebellar Ataxias
Authors
Chi-Ying R. Lin
Sheng-Han Kuo
Puneet Opal
Publication date
25-01-2024
Publisher
Springer US
Published in
Current Neurology and Neuroscience Reports / Issue 3/2024
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-024-01331-4