title: Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing.
abstract: Selective verb and noun deficits have been observed in a number of neurological conditions and their occurrence has been interpreted as evidence for different neural networks underlying the processing of specific word categories. We describe the first case of a familial occurrence of a selective deficit of verb processing. Father (Individual I) and son (Individual II) developed a movement disorder resembling progressive supranuclear palsy (PSP) and associated with dementia. A second child of Individual II remained symptom-free on consecutive examinations. The dissociation between the processing of nouns and verbs in Individuals I and II was confirmed with different methods, including a longitudinal assessment of naming, comprehension, picture and word association, as well as a lexical decision task. The difference remained stable on follow-up testing despite overall deterioration. It was associated with left-sided frontal hypometabolism on FDG-PET imaging (Individual II) and with ubiquitin-positive inclusions on post-mortem examination (Individual I). The association of a selective verb deficit with a familial movement disorder raises the question whether related genetic factors might influence both movements and their abstract conceptual representations in the form of action verbs. By demonstrating a link between pathology, genetics, imaging and abstract cognitive impairments this study advances our understanding of degenerative brain disease with implications for both neuroscience and clinical practice.
authors.affiliation: MRC Cognition and Brain Sciences Unit, Addenbrooke's Hospital, UK.
authors.forename: Thomas H
authors.initials: TH
authors.lastname: Bak
authors.forename: Despina
authors.initials: D
authors.lastname: Yancopoulou
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Nestor
authors.forename: John H
authors.initials: JH
authors.lastname: Xuereb
authors.forename: Maria G
authors.initials: MG
authors.lastname: Spillantini
authors.forename: Friedemann
authors.initials: F
authors.lastname: Pulvermüller
authors.forename: John R
authors.initials: JR
authors.lastname: Hodges
mesh_terms: D000328:Adult; D000368:Aged; D003704:Dementia / Q000235:genetics* / Q000473:pathology / Q000523:psychology; D005260:Female; D005625:Frontal Lobe / Q000737:chemistry / Q000473:pathology; D006579:Heterozygote; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D002479:Inclusion Bodies / Q000737:chemistry; D008297:Male; D009039:Motion Perception; D016501:Neurites / Q000737:chemistry; D009474:Neurons / Q000737:chemistry; D009483:Neuropsychological Tests; D010375:Pedigree; D049268:Positron-Emission Tomography; D013494:Supranuclear Palsy, Progressive / Q000235:genetics* / Q000473:pathology / Q000523:psychology*; D025801:Ubiquitin / Q000032:analysis; D014705:Verbal Behavior; D016875:tau Proteins / Q000235:genetics
chemical_list: D025801:Ubiquitin; D016875:tau Proteins