Memory & Aging Center, Department of Neurology, University of California in San Francisco, 1651 4th St, San Francisco, CA, 94158, United States; Jona Goldrich center for Alzheimer's and Memory disorders, Department of Neurology, Cedars Sinai Medical Center, 127 S. San Vicente Blvd, Suite A 6600, Los Angeles, CA, 90048, United States.
Memory & Aging Center, Department of Neurology, University of California in San Francisco, 1651 4th St, San Francisco, CA, 94158, United States.
Neuropsychologia. 2024 Dec 15;205:109020. doi: 10.1016/j.neuropsychologia.2024.109020. Epub 2024 Oct 22.
Semantic dementia (SD) patients including semantic variant primary progressive aphasia (svPPA) and semantic behavioral variant frontotemporal dementia (sbvFTD) patients show semantic difficulties identifying faces and known people related to right anterior temporal lobe (ATL) atrophy. However, it remains unclear whether they also have perceptual deficits in face recognition.
We selected 74 SD patients (54 with svPPA and predominant left ATL atrophy and 20 with sbvFTD and predominant right ATL atrophy) and 36 cognitively healthy controls (HC) from UCSF Memory and Aging Center. They underwent a perceptual face processing test (Benton facial recognition test-short version; BFRT-S), and semantic face processing tests (UCSF Famous people battery - Recognition, Naming, Semantic associations - pictures and words subtests), as well as structural magnetic resonance imaging (MRI). Neural correlates with the task's performance were conducted with a Voxel-based morphometry approach using CAT12.
svPPA and sbvFTD patients were impaired on all semantic face processing tests, with sbvFTD patients performing significantly lower on the famous faces' recognition task in comparison to svPPA, and svPPA performing significantly lower on the naming task in comparison to sbvFTD. These tasks predominantly correlated with grey matter (GM) volumes in the right and left ATL, respectively. Compared to HC, both svPPA and sbvFTD patients showed preserved performance on the perceptual face processing test (BFRT-S), and performance on the BFRT-S negatively correlated with GM volume in the right posterior superior temporal sulcus (pSTS).
Our results suggest that early in the disease, with the atrophy mostly restricted to the anterior temporal regions, SD patients do not present with perceptual deficits. However, more severe SD cases with atrophy in right posterior temporal regions might show lower performance on face perception tests, in addition to the semantic face processing deficits. Early sparing of face perceptual deficits in SD patients, regardless of hemispheric lateralization, furthers our understanding of clinical phenomenology and therapeutical approaches of this complex disease.
语义痴呆症(SD)患者,包括语义变异原发性进行性失语症(svPPA)和语义行为变异额颞叶痴呆症(sbvFTD)患者,在识别面部和与右侧颞叶前部(ATL)萎缩相关的熟人时表现出语义困难。然而,目前尚不清楚他们是否也存在面部识别的知觉缺陷。
我们从 UCSF 记忆与衰老中心选择了 74 名 SD 患者(54 名 svPPA 患者,左 ATL 萎缩为主,20 名 sbvFTD 患者,右 ATL 萎缩为主)和 36 名认知健康对照者(HC)。他们接受了知觉面孔处理测试(Benton 面部识别测试-短版;BFRT-S)、语义面孔处理测试(UCSF 名人电池-识别、命名、语义关联-图片和单词子测试),以及结构磁共振成像(MRI)。使用 CAT12 进行基于体素的形态测量学方法,对任务表现的神经相关性进行了研究。
svPPA 和 sbvFTD 患者在所有语义面孔处理测试中都存在障碍,sbvFTD 患者在著名面孔识别任务中的表现明显低于 svPPA,而 svPPA 在命名任务中的表现明显低于 sbvFTD。这些任务主要与右侧和左侧 ATL 的灰质(GM)体积相关。与 HC 相比,svPPA 和 sbvFTD 患者在知觉面孔处理测试(BFRT-S)中的表现均得到保留,并且 BFRT-S 的表现与右侧后颞上沟(pSTS)GM 体积呈负相关。
我们的结果表明,在疾病早期,当萎缩主要局限于前颞叶区域时,SD 患者不会出现知觉缺陷。然而,对于右侧后部颞区萎缩更为严重的 SD 病例,除了语义面孔处理缺陷外,他们可能在面孔知觉测试中的表现较低。SD 患者早期保留面孔知觉缺陷,无论偏侧化如何,都进一步加深了我们对这种复杂疾病的临床表现和治疗方法的理解。