Yadollahikhales Golnaz, Mandelli Maria Luisa, Ezzes Zoe, Pillai Janhavi, Ratnasiri Buddhika, Baquirin David Paul, Miller Zachary, de Leon Jessica, Tee Boon Lead, Seeley William, Rosen Howard, Miller Bruce, Kramer Joel, Sturm Virginia, Gorno-Tempini Maria Luisa, Montembeault Maxime
medRxiv. 2024 Jul 12:2024.07.10.24310157. doi: 10.1101/2024.07.10.24310157.
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 gray 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.
语义性痴呆(SD)患者,包括语义变异型原发性进行性失语(svPPA)患者和语义行为变异型额颞叶痴呆(sbvFTD)患者,在识别面孔和与右前颞叶(ATL)萎缩相关的熟人方面存在语义困难。然而,他们在人脸识别中是否也存在感知缺陷仍不清楚。
我们从加州大学旧金山分校记忆与衰老中心选取了74名SD患者(54名svPPA患者且主要为左侧ATL萎缩,20名sbvFTD患者且主要为右侧ATL萎缩)和36名认知健康对照者(HC)。他们接受了感知面孔加工测试(本顿面部识别测试 - 简短版;BFRT - S)、语义面孔加工测试(加州大学旧金山分校名人测试组 - 识别、命名、语义联想 - 图片和单词子测试)以及结构磁共振成像(MRI)。使用CAT12通过基于体素的形态测量方法对与任务表现相关的神经关联进行了研究。
svPPA和sbvFTD患者在所有语义面孔加工测试中均表现受损,与svPPA患者相比,sbvFTD患者在名人面孔识别任务中的表现显著更低,与sbvFTD患者相比,svPPA患者在命名任务中的表现显著更低。这些任务分别主要与右侧和左侧ATL的灰质(GM)体积相关。与HC相比,svPPA和sbvFTD患者在感知面孔加工测试(BFRT - S)中表现均正常,且BFRT - S的表现与右侧后颞上沟(pSTS)的GM体积呈负相关。
我们的结果表明,在疾病早期,萎缩主要局限于前颞叶区域时,SD患者不存在感知缺陷。然而,除了语义面孔加工缺陷外,右侧后颞叶区域萎缩的更严重SD病例在面孔感知测试中可能表现更差。SD患者早期保留面孔感知缺陷,无论半球侧化情况如何,都有助于我们进一步理解这种复杂疾病的临床现象学和治疗方法。