Moretti Rita, Torre Paola, Antonello Rodolfo M, Cattaruzza Tatiana, Cazzato Giuseppe
Department of Internal Medicine and Clinical Neurology, University of Trieste, Trieste, Italy.
Dement Geriatr Cogn Disord. 2005;20(2-3):158-62. doi: 10.1159/000087299. Epub 2005 Aug 3.
Corticobasal degeneration (CBD) is a rare disorder, which normally includes a combination of neurobehavioural features, movement disorders and other manifestations. It is now recognized that CBD patients usually present with two phenotypes: the lateralized phenotype and the dementia phenotype. The aim of our work was to determine the nature and the progression of cognitive and behavioural impairment in 10 lateralized CBD patients. In our patients, the most salient aspects of cognitive impairment were: an evident alteration of rapid alternating operative strategies, associated with the evident impairment of set shifting, of executive operations, of operative and sequential procedure and of implementation of judgement and abstract reasoning. The self-activation of retrieval processes is partly preserved in CBD. As all the other types of subcortical impairments, even CBD encompasses both cognitive impairment as well as a wide range of behaviour disturbances, such as progressive alterations of sleep, depression, and of anxiety (with a remarkable incidence of somatic pain). This suggests that in addition to neuropsychological assessment, quantification of the personality behaviour disorder is important for standardizing the diagnosis of subcortical vascular dementia and distinguishing it from any other dementias.
皮质基底节变性（CBD）是一种罕见的疾病，通常包括神经行为特征、运动障碍和其他表现的组合。现在人们认识到，CBD患者通常表现出两种表型：偏侧化表型和痴呆表型。我们研究的目的是确定10例偏侧化CBD患者认知和行为障碍的性质及进展。在我们的患者中，认知障碍最突出的方面是：快速交替操作策略明显改变，伴有定势转换、执行功能、操作及顺序性程序以及判断和抽象推理实施的明显受损。在CBD中，检索过程的自我激活部分得以保留。与所有其他类型的皮质下损伤一样，CBD也包括认知障碍以及广泛的行为障碍，如睡眠逐渐改变、抑郁和焦虑（躯体疼痛发生率显著）。这表明，除了神经心理学评估外，量化人格行为障碍对于规范皮质下血管性痴呆的诊断并将其与其他痴呆区分开来很重要。
