title: Semantic deficits in amyotrophic lateral sclerosis.
abstract: 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.
authors.affiliation: Neuroscience Research Australia , Sydney.
authors.forename: Felicity V C
authors.initials: FV
authors.lastname: Leslie
authors.forename: Sharpley
authors.initials: S
authors.lastname: Hsieh
authors.forename: Jashelle
authors.initials: J
authors.lastname: Caga
authors.forename: Sharon A
authors.initials: SA
authors.lastname: Savage
authors.forename: Eneida
authors.initials: E
authors.lastname: Mioshi
authors.forename: Michael
authors.initials: M
authors.lastname: Hornberger
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Kiernan
authors.forename: John R
authors.initials: JR
authors.lastname: Hodges
authors.forename: James R
authors.initials: JR
authors.lastname: Burrell
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000453:epidemiology; D001284:Atrophy; D001921:Brain / Q000473:pathology; D004185:Disability Evaluation; D005260:Female; D057180:Frontotemporal Dementia / Q000175:diagnosis; D006801:Humans; D007806:Language Disorders / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D009483:Neuropsychological Tests; D012189:Retrospective Studies; D012660:Semantics
keywords: Dementia; cognition; imaging