Mariën Peter, Baillieux Hanne, De Smet Hyo Jung, Engelborghs Sebastiaan, Wilssens Ineke, Paquier Philippe, De Deyn Peter P
Department of Linguistics, Vrije Universiteit Brussel, Brussels, Belgium.
Cortex. 2009 Apr;45(4):527-36. doi: 10.1016/j.cortex.2007.12.010. Epub 2008 Mar 2.
The cerebellar cognitive affective syndrome (CCAS) is a neurobehavioral syndrome that may develop after congenital and acquired cerebellar lesions. The syndrome consists of deficits in executive functioning, spatial cognition, visual-spatial memory and language and also involves personality and behavioral changes. We describe a 58-year-old right-handed man who in addition to affective disturbances presented with a unique combination of cognitive and linguistic deficits following an ischemic infarction in the vascular territory of the right superior cerebellar artery (SCA). Neurocognitive and neurolinguistic examinations were performed in the acute phase (10 days post-onset) and lesion phase (four weeks post-onset) of the stroke. A Tc-99m-ECD SPECT study was performed five weeks after the stroke. Acute phase data revealed a generalized cognitive decline and mild transcortical sensory aphasia. In the lesion phase, the neurobehavioral tableau was dominated by executive dysfunctions, disrupted divided attention, disturbed visual-spatial organization and behavioral abnormalities. Neurolinguistic investigations disclosed visual dyslexia and surface dysgraphia. Reading of words and visual lexical decision tasks of words and nonwords were severely defective and predominantly characterized by visual errors. In addition, writing irregular and ambiguous words resulted in regularization errors (phonologically plausible errors based on phoneme-grapheme correspondence rules). In the absence of any structural damage in the supratentorial brain regions, a quantified SPECT study showed a relative hypoperfusion in the right cerebellar hemisphere and the left medial frontal lobe. CCAS is for the first time reported in association with visual dyslexia and surface dysgraphia. We hypothesize that the cognitive and linguistic deficits might result from functional disruption of the cerebellar-encephalic pathways, connecting the cerebellum to the frontal supratentorial areas which subserve attentional and planning processes. This phenomenon of crossed cerebellar-cerebral diaschisis is supported by SPECT findings revealing a hypoperfusion in the anatomoclinically suspected brain regions. The constellation of cognitive, linguistic and behavioral symptoms adds new evidence to the multifaceted area of cerebellar neurocognition and demonstrates that the cerebellum might play a crucial role in cognitive, linguistic, and affective processing.
小脑认知情感综合征（CCAS）是一种神经行为综合征，可在先天性和后天性小脑病变后出现。该综合征包括执行功能、空间认知、视觉空间记忆和语言方面的缺陷，还涉及人格和行为改变。我们描述了一名58岁的右利手男性，在右侧小脑上动脉（SCA）血管区域发生缺血性梗死后，除情感障碍外，还出现了认知和语言缺陷的独特组合。在中风的急性期（发病后10天）和病变期（发病后四周）进行了神经认知和神经语言学检查。中风后五周进行了Tc-99m-ECD SPECT研究。急性期数据显示普遍认知下降和轻度经皮质感觉性失语。在病变期，神经行为表现以执行功能障碍、注意力分散、视觉空间组织紊乱和行为异常为主。神经语言学研究发现了视觉性阅读障碍和表层书写障碍。单词阅读以及单词和非单词的视觉词汇判断任务严重受损，主要表现为视觉错误。此外，书写不规则和模糊的单词会导致规则化错误（基于音素-字素对应规则的语音上合理的错误）。在幕上脑区没有任何结构损伤的情况下，一项定量SPECT研究显示右侧小脑半球和左侧内侧额叶相对灌注不足。首次报道CCAS与视觉性阅读障碍和表层书写障碍相关。我们推测，认知和语言缺陷可能是由于连接小脑与额叶幕上区域的小脑-脑通路功能中断所致，这些区域参与注意力和计划过程。SPECT结果显示在解剖学上怀疑的脑区灌注不足，支持了交叉性小脑-大脑失联络现象。认知、语言和行为症状的组合为小脑神经认知的多方面领域增添了新证据，并表明小脑可能在认知、语言和情感处理中起关键作用。
