Structural cerebellar correlates of cognitive functions in spinocerebellar ataxia type 2
Structural cerebellar correlates of cognitive functions in spinocerebellar ataxia type 2
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DOI：
10.1007/s00415-018-8738-6
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发表时间：
2018-03-01
影响因子：
6
通讯作者：
Leggio, M.
中科院分区：
文献类型：
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作者：
Olivito, G.;Lupo, M.;Leggio, M.
脊髓小脑共济失调 2 型 (SCA2) 是一种累及小脑的常染色体显性神经退行性疾病，其特征是典型的运动综合征。此外，认知障碍的存在现已被广泛认为是 SCA2 的一个特征。鉴于小脑和相关大脑区域之间的广泛联系，可以合理地假设与 SCA2 相关的小脑神经变性可能会影响大脑皮层的小脑调节，从而导致功能障碍。本研究的目的是调查并定量绘制 SCA2 神经变性引起的小脑灰质 (GM) 萎缩模式，并将其与患者的认知表现相关联。使用基于体素的形态测量法提取小脑 GM 图，并在 SCA2 患者 (n = 9) 和对照组 (n = 33) 之间进行比较。此外，还评估了小脑 GM 萎缩与患者神经心理学评分之间的关系。发现患者的特定小脑 GM 区域受到影响。此外，认知后小叶（VI、Crus I、Crus II、VIIB、IX）的 GM 损失与视觉空间、言语记忆和执行任务相关，而与运动前小叶（V）和后小叶（VIIIA、VIIIB）的额外相关性被发现与涉及运动和计划部分的任务相关。我们的结果提供了证据，表明 SCA2 神经退行性过程影响小脑皮质，并且不同小脑亚区域的 MRI 萎缩指数可能解释了在患者中观察到的认知症状的特异性，这是小脑-大脑失调的结果。
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum and characterized by a typical motor syndrome. In addition, the presence of cognitive impairment is now widely acknowledged as a feature of SCA2. Given the extensive connections between the cerebellum and associative cerebral areas, it is reasonable to hypothesize that cerebellar neurodegeneration associated with SCA2 may impact on the cerebellar modulation of the cerebral cortex, thus resulting in functional impairment. The aim of the present study was to investigate and quantitatively map the pattern of cerebellar gray matter (GM) atrophy due to SCA2 neurodegeneration and to correlate that with patients' cognitive performances. Cerebellar GM maps were extracted and compared between SCA2 patients (n = 9) and controls (n = 33) by using voxel-based morphometry. Furthermore, the relationship between cerebellar GM atrophy and neuropsychological scores of the patients was assessed. Specific cerebellar GM regions were found to be affected in patients. Additionally, GM loss in cognitive posterior lobules (VI, Crus I, Crus II, VIIB, IX) correlated with visuospatial, verbal memory and executive tasks, while additional correlations with motor anterior (V) and posterior (VIIIA, VIIIB) lobules were found for the tasks engaging motor and planning components. Our results provide evidence that the SCA2 neurodegenerative process affects the cerebellar cortex and that MRI indices of atrophy in different cerebellar subregions may account for the specificity of cognitive symptomatology observed in patients, as result of a cerebello-cerebral dysregulation.