The cerebellar cognitive affective syndrome
The cerebellar cognitive affective syndrome
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DOI：
10.1093/brain/121.4.561
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发表时间：
1998-04-01
期刊：
影响因子：
14.5
通讯作者：
Sherman, JC
中科院分区：
文献类型：
--
作者：
Schmahmann, JD;Sherman, JC
解剖学，生理学和功能性神经影像学研究表明，小脑参与组织高阶功能，但很少有临床相关病例的描述，解决这种可能性。我们对20例局限于小脑的疾病患者进行了神经系统检查、床旁精神状态休息、神经心理学研究和解剖神经影像学检查，并评估了神经和精神功能变化的性质和严重程度。行为变化在小脑后叶和小脑蚓部病变患者中临床上很突出，在某些情况下，它们是最明显的表现。这些变化的特点是：执行功能受损，如计划，设置转换，语言流畅性，抽象推理和工作记忆;空间认知困难，包括视觉空间组织和记忆;个性变化，情感迟钝或不受抑制和不适当的行为;和语言缺陷，包括语法缺失和韵律障碍。小脑前叶的病变只产生轻微的变化，在执行和视觉空间功能。我们把这种新定义的临床症状称为“小脑认知情感综合征”。这一系列的缺陷表明小脑对连接前额叶和后顶叶的神经回路的调节受到了破坏。上级颞叶和边缘皮质与小脑。
Anatomical, physiological and functional neuroimaging studies suggest that the cerebellum participates in the organization of higher order function, but there are very few descriptions of clinically relevant cases that address this possibility. We performed neurological examinations, bedside mental state rests, neuropsychological studies and anatomical neuroimaging on 20 patients with diseases confined to the cerebellum, and evaluated the nature and severity of the changes in neurological and mental function. Behavioural changes were clinically prominent in patients with lesions involving the posterior lobe of the cerebellum and the vermis, and in some cases they were the most noticeable aspects of the presentation. These changes were characterized by: impairment of executive functions such as planning, set shifting, verbal fluency, abstract reasoning and working memory; difficulties with spatial cognition including visual-spatial organization and memory; personality change with blunting of affect or disinhibited and inappropriate behaviour; and language deficits including agrammatism and dysprosodia. Lesions of the anterior lobe of the cerebellum produced only minor changes in executive and visual-spatial functions. We have called this newly defined clinical entity the 'cerebellar cognitive affective syndrome'. The constellation of deficits is suggestive of disruption of the cerebellar modulation of neural circuits that link prefrontal, posterior parietal. superior temporal and limbic cortices with the cerebellum.