Department of Rehabilitation Science, Sunnybrook Health Sciences Centre, University of Toronto, Ontario, Canada.
Cortex. 2011 Apr;47(4):460-72. doi: 10.1016/j.cortex.2010.04.010. Epub 2010 May 11.
Corticobasal syndrome (CBS) is a progressive neurodegenerative disorder with asymmetric presentation and course characterized by degeneration of basal ganglia and cortical structures. Limb apraxia is a commonly observed deficit in CBS. Few studies have examined comprehensively the nature of deficits in limb apraxia. The goal of our study was to investigate the severity of deficits in various conceptual and gesture production task modalities. CBS patients were divided in two groups based on the side of brain that was initially affected by the disease. Ten patients with right hemisphere presentation (RHP) and seven with left hemisphere presentation (LHP) were included. The results showed that while selective conceptual tasks deficits were present in both groups, the overall picture suggests preserved conceptual representations of tools and actions in CBS patients with either LHP or RHP. Both groups were impaired relative to controls on gesture production tasks. Performance on transitive gestures was more severely affected in both groups than intransitive gestures. Imitation was more severely affected than pantomime, suggesting deficits in visuomotor transformations. The addition of verbal cuing during concurrent imitation affected only the LHP patients, rendering them more impaired relative to controls in their imitation with verbal cuing as opposed to their imitation only performance. Imitation of non-representational gestures was least accurate and intransitive gestures were most accurate. Patients were more severely impaired relative to controls when holding the object and when they were shown pictures of tools to pantomime.
皮质基底节综合征(CBS)是一种进行性神经退行性疾病,具有非对称表现和病程,其特征为基底节和皮质结构的退化。肢体运动不能症是 CBS 中常见的缺陷。少数研究全面检查了肢体运动不能症的缺陷性质。我们的研究目的是研究各种概念和手势产生任务模式中缺陷的严重程度。根据疾病最初影响大脑的一侧,将 CBS 患者分为两组。纳入了 10 名右脑表现型(RHP)患者和 7 名左脑表现型(LHP)患者。结果表明,虽然两组均存在选择性概念任务缺陷,但总体情况表明,LHP 或 RHP 的 CBS 患者对工具和动作的概念表示保持不变。与对照组相比,两组在手势产生任务中均受损。两组的及物手势表现均比不及物手势更严重受损。模仿比模仿更严重受损,表明在视动转换中存在缺陷。在同时进行的模仿过程中添加口头提示仅影响左脑表现型患者,使他们在有口头提示的模仿中相对于对照组更受损,而在仅模仿表现中则不受损。非代表性手势的模仿最不准确,不及物手势最准确。当患者拿着物体或观看工具的图片进行模仿时,与对照组相比,他们的表现更差。