Neurology Unit, "A. Perrino" Hospital, Brindisi (BR), Italy.
Department of Basic Medical Sciences, Neurosciences and Sense Organs, "Aldo Moro" University of Bari, Bari, Italy.
Cerebellum. 2020 Feb;19(1):40-47. doi: 10.1007/s12311-019-01079-5.
The clinical spectrum of spinocerebellar ataxia type 2 includes motor manifestations and cognitive disturbances in executive functions, memory, and visuoconstructive skills. The relationships between severity of motor disturbances and altered cognition are poorly known. In this study, we assessed patients with spinocerebellar ataxia type 2 and age- and sex-matched healthy control subjects by a test battery including the Mini-mental State Examination, the Wisconsin Card Sorting test, and the Wechsler Memory Scale-Revised. The correlation between severity of motor ataxia (as assessed by a validated and widely used severity scale, the SARA scale, and by an objective automated computerized system of gait analysis) and altered cognition was then evaluated by Spearman correlation analysis. Patients performed worse than healthy controls in almost all administered neuropsychological tests. Nevertheless, only global intellectual abilities and executive functions significantly correlated with the overall severity of ataxia as assessed by the SARA scale, and impaired executive functions alone correlated with performance on several spatio-temporal gait analysis parameters. Our findings would probably suggest a prominent influence of executive functions on motor abilities in patients with spinocerebellar ataxia type 2 and raise the possibility that cognitive pharmaceutical or rehabilitative interventions may be of benefit in the management of motor problems in these patients.
小脑性共济失调 2 型的临床特征包括运动表现以及执行功能、记忆和视空间构建技能方面的认知障碍。运动障碍的严重程度与认知改变之间的关系尚未明确。在这项研究中，我们使用包括 Mini-mental State Examination、威斯康星卡片分类测试和韦氏记忆量表修订版在内的测试组合，对小脑性共济失调 2 型患者和年龄及性别匹配的健康对照组进行了评估。然后，我们通过 Spearman 相关分析评估了运动共济失调严重程度（通过经验证和广泛使用的严重程度量表 SARA 量表和客观的自动步态分析计算机系统进行评估）与认知改变之间的相关性。患者在几乎所有进行的神经心理学测试中表现均差于健康对照组。然而，只有总体智力和执行功能与 SARA 量表评估的整体共济失调严重程度显著相关，而执行功能障碍仅与多个时空步态分析参数的表现相关。我们的研究结果可能表明执行功能对小脑性共济失调 2 型患者运动能力有重要影响，并提示认知药物或康复干预可能有益于这些患者运动问题的管理。
