This study sought evidence for a specific cerebellar contribution to cognition by characterising the cognitive phenotype of Spinocerebellar Ataxia Type 6 (SCA-6); an autosomal dominant genetic disease which causes a highly specific late-onset cerebellar degeneration. A comprehensive neuropsychological assessment was administered to 27 patients with genetically confirmed SCA-6. General intellectual ability, memory and executive function were examined using internationally standardised tests (Wechsler Adult Intelligence Scale-III, Wechsler Memory Scale-III, Delis and Kaplan Executive Function System, Brixton Spatial Anticipation test). The patient group showed no evidence of intellectual or memory decline. However, tests of executive function involving skills of cognitive flexibility, inhibition of response and verbal reasoning and abstraction demonstrated significant impairment at the group level with large effect sizes. The results demonstrate an executive deficit due to SCA-6 that can be conceptualised as parallel to the motor difficulties suffered by these patients: the data support a role for the cerebellum in the regulation and coordination of cognitive, as well as motor processes that is relevant to individual performance.

中文翻译：

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小脑对脊髓小脑共济失调 6 型认知的贡献。

本研究通过描述脊髓小脑共济失调 6 型 (SCA-6) 的认知表型来寻找小脑对认知的特定贡献的证据；一种常染色体显性遗传病，可导致高度特异性的迟发性小脑变性。对 27 名经基因证实的 SCA-6 患者进行了全面的神经心理学评估。使用国际标准化测试（韦氏成人智力量表-III、韦氏记忆量表-III、熟食店和卡普兰执行功能系统、布里克斯顿空间预测测试）检查一般智力能力、记忆力和执行功能。患者组没有显示智力或记忆力下降的证据。然而，涉及认知灵活性技能的执行功能测试，抑制反应和语言推理和抽象在群体水平上表现出显着的损害，影响大小很大。结果表明，由于 SCA-6 导致的执行缺陷可以被概念化为与这些患者遭受的运动困难平行：数据支持小脑在认知调节和协调中的作用，以及相关的运动过程到个人表现。