Ataxia Unit, Cognitive/Behavioral Neurology Unit, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, 175 Cambridge Street, Boston, MA 02114, USA.
Cortex. 2010 Jul-Aug;46(7):831-44. doi: 10.1016/j.cortex.2009.11.008. Epub 2010 Jan 11.
Patients with cerebellar damage often present with the cerebellar motor syndrome of dysmetria, dysarthria and ataxia, yet cerebellar lesions can also result in the cerebellar cognitive affective syndrome (CCAS), including executive, visual spatial, and linguistic impairments, and affective dysregulation. We have hypothesized that there is topographic organization in the human cerebellum such that the anterior lobe and lobule VIII contain the representation of the sensorimotor cerebellum; lobules VI and VII of the posterior lobe comprise the cognitive cerebellum; and the posterior vermis is the anatomical substrate of the limbic cerebellum. Here we analyze anatomical, functional neuroimaging, and clinical data to test this hypothesis. We find converging lines of evidence supporting regional organization of motor, cognitive, and limbic behaviors in the cerebellum. The cerebellar motor syndrome results when lesions involve the anterior lobe and parts of lobule VI, interrupting cerebellar communication with cerebral and spinal motor systems. Cognitive impairments occur when posterior lobe lesions affect lobules VI and VII (including Crus I, Crus II, and lobule VIIB), disrupting cerebellar modulation of cognitive loops with cerebral association cortices. Neuropsychiatric disorders manifest when vermis lesions deprive cerebro-cerebellar-limbic loops of cerebellar input. We consider this functional topography to be a consequence of the differential arrangement of connections of the cerebellum with the spinal cord, brainstem, and cerebral hemispheres, reflecting cerebellar incorporation into the distributed neural circuits subserving movement, cognition, and emotion. These observations provide testable hypotheses for future investigations.
小脑损伤的患者常表现出运动协调障碍、构音障碍和共济失调的小脑运动综合征,但小脑病变也可导致小脑认知情感综合征(CCAS),包括执行功能、视觉空间和语言障碍以及情感调节障碍。我们假设人类小脑存在拓扑组织,使得前叶和VIII 小叶包含感觉运动小脑的代表;后叶的 VI 和 VII 小叶构成认知小脑;而后蚓部是边缘小脑的解剖学基础。在这里,我们分析解剖学、功能神经影像学和临床数据来检验这一假设。我们发现支持小脑运动、认知和边缘行为区域组织的证据越来越多。当病变涉及前叶和 VI 小叶的部分区域,中断小脑与大脑和脊髓运动系统的通信时,会出现小脑运动综合征。当后叶病变影响 VI 和 VII 小叶(包括 Crus I、Crus II 和 VIIB 小叶)时,会出现认知障碍,中断小脑对大脑联合皮质认知回路的调制。当蚓部病变使脑-小脑-边缘回路失去小脑输入时,就会出现神经精神障碍。我们认为这种功能拓扑结构是小脑与脊髓、脑干和大脑半球连接的差异排列的结果,反映了小脑整合到运动、认知和情感的分布式神经回路中。这些观察结果为未来的研究提供了可检验的假设。