Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Res Dev Disabil. 2013 Jul;34(7):2118-26. doi: 10.1016/j.ridd.2013.03.026. Epub 2013 Apr 30.
In Williams syndrome (WS) cerebellar measures were only indirectly related to behavioral outcomes. T1-weighted magnetic resonance images and neuropsychological data were acquired to investigate whether cerebellar vermis differences were present in 12 WS individuals compared with 13 chronological age-matched controls and whether WS cerebellar vermis measures were related to cognitive scores. In WS participants, we observed a significant increase in the volume of the posterior superior cerebellar vermis (lobules VI-VII) and an atypical ratio between width and height of the cerebellar vermis. Furthermore, we found an inverse correlation between cerebellar posterior vermis volume and scores on implicit learning, phonological fluency and the verbal short-term memory tasks. The present study supported a role for the posterior cerebellar vermis in higher cognitive processes and indicated that the cerebellar vermis abnormalities (enlargement) in WS individuals have an effect in worsening the cognitive performance in specific domains.
在威廉姆斯综合征（WS）中，小脑测量值仅与行为结果间接相关。采集 T1 加权磁共振图像和神经心理学数据，以研究 12 名 WS 个体与 13 名年龄匹配的对照组之间是否存在小脑蚓部差异，以及 WS 小脑蚓部测量值是否与认知评分相关。在 WS 参与者中，我们观察到后上小脑蚓部（VI-VII 叶）的体积显著增加，小脑蚓部的宽度和高度比呈现非典型状态。此外，我们发现小脑后蚓部体积与内隐学习、语音流畅性和言语短期记忆任务的评分呈负相关。本研究支持后小脑蚓部在高级认知过程中的作用，并表明 WS 个体小脑蚓部异常（增大）会对特定领域的认知表现产生负面影响。
