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Neurocognitive Changes in Spinocerebellar Ataxia Type 3: A Systematic Review with a Narrative Design

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Abstract

Spinocerebellar ataxia type 3 (SCA3), the commonest dominantly inherited ataxia worldwide, is characterized by disruption in the cerebellar-cerebral and striatal-cortical networks. Findings on SCA3-associated cognitive impairments are mixed. The classification models, tests and scoring systems used, language, culture, ataxia severity, and depressive symptoms are all potential confounders in neuropsychological assessments and may have contributed to the heterogeneity of the neurocognitive profile of SCA3. We conducted a systematic review of studies evaluating neurocognitive function in SCA3 patients. Of 1304 articles identified, 15 articles met the eligibility criteria. All articles were of excellent quality according to the National Institutes of Health quality assessment tool for case–control studies. In line with the disrupted cerebellar-cerebral and striatal-cortical networks in SCA3, this systematic review found that the neurocognitive profile of SCA3 is characterized by a core impairment of executive function that affects processes such as nonverbal reasoning, executive aspects of language, and recall. Conversely, neurocognitive domains such as general intelligence, verbal reasoning, semantic aspect of language, attention/processing speed, recognition, and visuospatial perception and construction are relatively preserved. This review highlights the importance of evaluating neurocognitive function in SCA3 patients. Considering the negative impact of cognitive and affective impairment on quality of life, this review points to the profound impairments that existing or future treatments should prioritize.

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Funding

This review was funded by Dana Impak Perdana Grant (DIP-2019–007) received by NMI from Universiti Kebangsaan Malaysia.

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Authors and Affiliations

  1. Department of Medicine, UKM Medical Center, 56000, Kuala Lumpur, Malaysia

    Kah Hui Yap, Shahrul Azmin & Norlinah Mohamed Ibrahim

  2. Donders Institute for Brain, Cognition and Behavior, Radboud University, PO Box 9104, 6500 HE, Nijmegen, The Netherlands

    Roy P. C. Kessels

  3. Department of Medical Psychology, Radboud University Medical Center, PO Box 9101, 6500 HB, Nijmegen, The Netherlands

    Roy P. C. Kessels

  4. Vincent Van Gogh Institute for Psychiatry, Venray, The Netherlands

    Roy P. C. Kessels

  5. Department of Neurology, Donders Institute for Brain, Cognition and Behavior, Radboud University Medical Center, PO Box 9101, 6500 HB, Nijmegen, The Netherlands

    Bart van de Warrenburg

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  1. Kah Hui Yap
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  3. Shahrul Azmin
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Contributions

All authors contributed to the study conception and design. Funding acquisition was performed by Norlinah Mohamed Ibrahim and Shahrul Azmin; literature search was performed by Kah Hui Yap and Shahrul Azmin. The first draft of this review was written by Kah Hui Yap, and all authors critically revised the work. All authors read and approved the final version of the review.

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Correspondence to Norlinah Mohamed Ibrahim.

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Yap, K.H., Kessels, R.P.C., Azmin, S. et al. Neurocognitive Changes in Spinocerebellar Ataxia Type 3: A Systematic Review with a Narrative Design. Cerebellum 21, 314–327 (2022). https://doi.org/10.1007/s12311-021-01282-3

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