Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia.
Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia.
复制标题
右颞叶退化和社会情感语义：语义行为变异额颞叶痴呆。
DOI：
10.1093/brain/awac217
复制
发表时间：
2022
期刊：
影响因子：
--
通讯作者：
Grinberg,
中科院分区：
文献类型：
--
作者：
Younes,Kyan;Borghesani,Valentina;Montembeault,Maxime;Spina,Salvatore;Mandelli,MariaLuisa;Welch,ArianeE;Weis,Elizabeth;Callahan,Patrick;Elahi,FannyM;Hua,AliceY;Perry,DavidC;Karydas,Anna;Geschwind,Daniel;Huang,Eric;Grinberg,
局灶性前颞叶变性通常优先累及左半球或右半球。左侧为主的前颞叶萎缩患者表现出严重的失范和言语语义缺陷，符合语义变异型原发性进行性失语和语义性痴呆的标准，而早期右前颞叶萎缩的患者由于对其症状了解较少，因此更难诊断。局灶性右前颞叶萎缩与显著的情绪和行为改变有关，患者经常符合或继续符合行为变异型额颞叶痴呆的标准。早期症状的不确定性和缺乏全面的临床解剖学框架继续阻碍着右前颞叶疾病患者的正确诊断和护理。在这里，我们研究了大量的、特征良好的、纵向的右前颞叶占优势的退行性变患者，并提出了新的标准和病因学。我们从我们的数据库中确定了临床诊断为行为变异额颞叶痴呆或语义变异型原发进行性失语症的个体(n=478)。根据神经影像标准，我们将患者分为三组：以右侧前颞叶为主的相对保留额叶的萎缩(n=46)，以额叶为主的相对保留右侧前颞叶的萎缩(n=79)，以左侧为主的前颞叶为主的萎缩相对保留额叶(n=75)。我们比较了这些组的临床、神经心理、遗传和病理特征，在右前颞叶占优势的组中，最早的症状是移情能力丧失(27%)、个人特异性语义障碍(23%)和复杂的强迫和僵化的思维过程(18%)。在测试中，这组人在情绪心理理论、对名人的识别(从名字和面孔)和面部情感命名(尽管保留了面孔感知)方面表现出比以额叶和左侧为主的前颞叶占优势的组更大的障碍。仅前3年的临床症状就高度敏感(81%)和特异度(84%)区分右前颞叶占优势组和额叶占优势组。额颞叶退行性变-反式反应DNA结合蛋白(84%)是右侧前颞叶占优势的组最常见的病理改变。右侧前颞叶占优势的退行性变的特征是早期丧失同理心和个人特有的知识，这是由于对社会情绪相关概念的语义记忆进行性下降所导致的缺陷。根据我们的研究结果，我们概述了新的诊断标准，并提出了语义行为变异额颞叶痴呆的名称，突出了潜在的认知机制和主要的症状学。这些诊断标准将有助于早期、局灶性右前颞叶退行性变患者的早期识别和治疗，以及额颞叶退行性变-DNA结合蛋白病理的存活。
Focal anterior temporal lobe degeneration often preferentially affects the left or right hemisphere. While patients with left-predominant anterior temporal lobe atrophy show severe anomia and verbal semantic deficits and meet criteria for semantic variant primary progressive aphasia and semantic dementia, patients with early right anterior temporal lobe atrophy are more difficult to diagnose as their symptoms are less well understood. Focal right anterior temporal lobe atrophy is associated with prominent emotional and behavioural changes, and patients often meet, or go on to meet, criteria for behavioural variant frontotemporal dementia. Uncertainty around early symptoms and absence of an overarching clinico-anatomical framework continue to hinder proper diagnosis and care of patients with right anterior temporal lobe disease. Here, we examine a large, well-characterized, longitudinal cohort of patients with right anterior temporal lobe-predominant degeneration and propose new criteria and nosology.We identified individuals from our database with a clinical diagnosis of behavioural variant frontotemporal dementia or semantic variant primary progressive aphasia and a structural MRI (n= 478). On the basis of neuroimaging criteria, we defined three patient groups: right anterior temporal lobe-predominant atrophy with relative sparing of the frontal lobes (n= 46), frontal-predominant atrophy with relative sparing of the right anterior temporal lobe (n= 79) and left-predominant anterior temporal lobe-predominant atrophy with relative sparing of the frontal lobes (n= 75). We compared the clinical, neuropsychological, genetic and pathological profiles of these groups.In the right anterior temporal lobe-predominant group, the earliest symptoms were loss of empathy (27%), person-specific semantic impairment (23%) and complex compulsions and rigid thought process (18%). On testing, this group exhibited greater impairments in Emotional Theory of Mind, recognition of famous people (from names and faces) and facial affect naming (despite preserved face perception) than the frontal- and left-predominant anterior temporal lobe-predominant groups. The clinical symptoms in the first 3 years of the disease alone were highly sensitive (81%) and specific (84%) differentiating right anterior temporal lobe-predominant from frontal-predominant groups. Frontotemporal lobar degeneration-transactive response DNA binding protein (84%) was the most common pathology of the right anterior temporal lobe-predominant group.Right anterior temporal lobe-predominant degeneration is characterized by early loss of empathy and person-specific knowledge, deficits that are caused by progressive decline in semantic memory for concepts of socioemotional relevance. Guided by our results, we outline new diagnostic criteria and propose the name, ‘semantic behavioural variant frontotemporal dementia’, which highlights the underlying cognitive mechanism and the predominant symptomatology. These diagnostic criteria will facilitate early identification and care of patients with early, focal right anterior temporal lobe degeneration as well asin vivoprediction of frontotemporal lobar degeneration-transactive response DNA binding protein pathology.