Leslie Felicity V C, Hsieh Sharpley, Caga Jashelle, Savage Sharon A, Mioshi Eneida, Hornberger Michael, Kiernan Matthew C, Hodges John R, Burrell James R
Neuroscience Research Australia , Sydney.
Amyotroph Lateral Scler Frontotemporal Degener. 2015 Mar;16(1-2):46-53. doi: 10.3109/21678421.2014.987301. Epub 2014 Dec 27.
Our objective was to investigate, and establish neuroanatomical correlates of, semantic deficits in amyotrophic lateral sclerosis (ALS) and amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD), compared to semantic dementia (SD) and controls. Semantic deficits were evaluated using a naming and semantic knowledge composite score, comprising verbal and non-verbal neuropsychological measures of single-word processing (confrontational naming, comprehension, and semantic association) from the Sydney Language Battery (SYDBAT) and Addenbrooke's Cognitive Examination - Revised (ACE-R). Voxel based morphometry (VBM) analysis was conducted using the region of interest approach. In total, 84 participants were recruited from a multidisciplinary research clinic in Sydney. Participants included 17 patients with ALS, 19 with ALS-FTD, 22 with SD and 26 age- and education-matched healthy controls. Significant semantic deficits were observed in ALS and ALS-FTD compared to controls. The severity of semantic deficits varied across the clinical phenotypes: ALS patients were less impaired than ALS-FTD patients, who in turn were not as impaired as SD patients. Anterior temporal lobe atrophy significantly correlated with semantic deficits. In conclusion, semantic impairment is a feature of ALS and ALS-FTD, and reflects the severity of temporal lobe pathology.
我们的目标是研究并确定肌萎缩侧索硬化症（ALS）和肌萎缩侧索硬化症-额颞叶痴呆（ALS-FTD）患者语义缺陷的神经解剖学关联，并与语义性痴呆（SD）和对照组进行比较。使用命名和语义知识综合评分来评估语义缺陷，该评分包括来自悉尼语言测试电池（SYDBAT）和修订版的Addenbrooke认知检查（ACE-R）的单字处理（对名称、理解和语义关联）的言语和非言语神经心理学测量。使用感兴趣区域方法进行基于体素的形态计量学（VBM）分析。总共从悉尼的一个多学科研究诊所招募了84名参与者。参与者包括17名ALS患者、19名ALS-FTD患者、22名SD患者和26名年龄和教育程度匹配的健康对照者。与对照组相比，在ALS和ALS-FTD患者中观察到明显的语义缺陷。语义缺陷的严重程度因临床表型而异：ALS患者的受损程度低于ALS-FTD患者，而ALS-FTD患者的受损程度又不如SD患者。颞叶前部萎缩与语义缺陷显著相关。总之，语义损害是ALS和ALS-FTD的一个特征，反映了颞叶病理学的严重程度。
