Penn Frontotemporal Degeneration Center (FTDC), University of Pennsylvania, Philadelphia, PA, USA.
Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
J Alzheimers Dis. 2022;89(2):641-658. doi: 10.3233/JAD-215728.
An understudied variant of Alzheimer's disease (AD), the behavioral/dysexecutive variant of AD (bvAD), is associated with progressive personality, behavior, and/or executive dysfunction and frontal atrophy.
This study characterizes the neuropsychological and neuroanatomical features associated with bvAD by comparing it to behavioral variant frontotemporal dementia (bvFTD), amnestic AD (aAD), and subjects with normal cognition.
Subjects included 16 bvAD, 67 bvFTD, 18 aAD patients, and 26 healthy controls. Neuropsychological assessment and MRI data were compared between these groups.
Compared to bvFTD, bvAD showed more significant visuospatial impairments (Rey Figure copy and recall), more irritability (Neuropsychological Inventory), and equivalent verbal memory (Philadelphia Verbal Learning Test). Compared to aAD, bvAD indicated more executive dysfunction (F-letter fluency) and better visuospatial performance. Neuroimaging analysis found that bvAD showed cortical thinning relative to bvFTD posteriorly in left temporal-occipital regions; bvFTD had cortical thinning relative to bvAD in left inferior frontal cortex. bvAD had cortical thinning relative to aAD in prefrontal and anterior temporal regions. All patient groups had lower volumes than controls in both anterior and posterior hippocampus. However, bvAD patients had higher average volume than aAD patients in posterior hippocampus and higher volume than bvFTD patients in anterior hippocampus after adjustment for age and intracranial volume.
Findings demonstrated that underlying pathology mediates disease presentation in bvAD and bvFTD.
阿尔茨海默病(AD)的一个研究较少的变体,即行为/执行功能障碍型 AD(bvAD),与进行性人格、行为和/或执行功能障碍以及额颞叶萎缩有关。
通过将其与行为变异额颞叶痴呆(bvFTD)、遗忘型 AD(aAD)和认知正常的受试者进行比较,本研究描述了与 bvAD 相关的神经心理学和神经解剖学特征。
受试者包括 16 名 bvAD 患者、67 名 bvFTD 患者、18 名 aAD 患者和 26 名健康对照者。对这些组进行神经心理学评估和 MRI 数据比较。
与 bvFTD 相比,bvAD 表现出更显著的视空间损伤(Rey 图形复制和回忆)、更易怒(神经心理学评估量表)和等效的语言记忆(费城语言学习测试)。与 aAD 相比,bvAD 表现出更多的执行功能障碍(F 字母流畅性)和更好的视空间表现。神经影像学分析发现,bvAD 与 bvFTD 相比,左侧颞枕叶区域后部皮质变薄;与 bvAD 相比,bvFTD 左侧额下回皮质变薄。与 aAD 相比,bvAD 在额前和颞前区域皮质变薄。所有患者组在前、后海马体积均低于对照组。然而,在调整年龄和颅内体积后,bvAD 患者在后海马的平均体积高于 aAD 患者,在前海马的体积高于 bvFTD 患者。
研究结果表明,潜在的病理学介导了 bvAD 和 bvFTD 的疾病表现。