Cognitive Neuroscience Section, NINDS, NIH, Bethesda, MD 20892-1440, USA.
Dement Geriatr Cogn Disord. 2010;29(1):88-96. doi: 10.1159/000255141. Epub 2010 Feb 11.
Patients with syndromes of the frontotemporal dementia spectrum are frequently unaware of their behavioral changes.
Seventy patients with a clinical diagnosis of behavioral variant frontotemporal dementia (bv-FTD, n = 27), aphasic variant frontotemporal dementia (a-FTD, n = 12) and corticobasal syndrome (CBS, n = 31) participated in the study. Anosognosia for behavioral disturbances was measured as discrepancy between caregiver's and patient's ratings on the Frontal Systems Behavior Scale for present and premorbid behavioral symptoms. Voxel-based morphometry analysis of MRI data was performed to explore the association between anosognosia and gray matter loss.
Although behavioral symptoms were reported in all the groups, the comparison between present and premorbid anosognosia revealed that bv-FTD patients not only underestimated their present behavioral disturbances compared to their caregivers, but also overestimated their premorbid behavioral disturbances. Across all groups, the degree of anosognosia for present behavioral impairment correlated with gray matter atrophy in a posterior region of the right superior temporal sulcus (adjacent to the temporoparietal junction).
These results confirm the role of the right temporoparietal cortex in the genesis of anosognosia and suggest that, in clinical syndromes of the frontotemporal dementia spectrum, anosognosia is associated with the dysfunction of temporoparietal mechanisms of self versus others knowledge.
额颞叶痴呆谱系患者常常对其行为变化不自知。
本研究纳入 70 名临床诊断为行为变异型额颞叶痴呆(bv-FTD,n=27)、失语变异型额颞叶痴呆(a-FTD,n=12)和皮质基底节综合征(CBS,n=31)的患者。采用额叶系统行为量表(Frontal Systems Behavior Scale),对患者和照料者的评分进行比较,以评估行为障碍的自知力。将 MRI 数据进行基于体素的形态学分析,以探索自知力与灰质丢失之间的关联。
尽管所有组均报告存在行为症状,但目前与发病前自知力的比较显示,bv-FTD 患者不仅对目前的行为障碍低估程度较照料者更大,而且对发病前的行为障碍高估程度也更大。在所有组中,目前行为损害自知力的程度与右侧上颞叶后区(与颞顶联合区相邻)的灰质萎缩程度相关。
这些结果证实了右颞顶联合区在自知力产生中的作用,并表明在额颞叶痴呆谱系的临床综合征中,自知力与自我与他人知识的颞顶机制功能障碍有关。