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

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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.

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Funding

Dr. Kuo received funding from the National Institutes of Health (NIH: R01NS118179, R01NS104423, R0NS1124854, R25NS070697) and the National Ataxia Foundation. Dr. Opal received funding from the NIH (R01NS082351, R01NS127204, R61NS127141, and U01NS104326) and the Giddan Foundation and the National Ataxia Foundation. Dr. Lin received funding from Baylor College of Medicine Junior Faculty Seed Award.

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Authors and Affiliations

  1. Department of Neurology, Baylor College of Medicine, Houston, TX, USA

    Chi-Ying R. Lin

  2. Department of Neurology and Initiative for Columbia Ataxia and Tremor, Columbia University Irving Medical Center, New York, NY, USA

    Sheng-Han Kuo

  3. Davee Department of Neurology and Department of Cell and Developmental Biology, Northwestern University Feinberg School of Medicine, 303 East Chicago Avenue, Chicago, IL, 60611, USA

    Puneet Opal

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  1. Chi-Ying R. Lin
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Lin, CY.R., Kuo, SH. & Opal, P. Cognitive, Emotional, and Other Non-motor Symptoms of Spinocerebellar Ataxias. Curr Neurol Neurosci Rep 24, 47–54 (2024). https://doi.org/10.1007/s11910-024-01331-4

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