Brain Advance Access originally published online on December 5, 2005
Brain 2006 129(2):321-332; doi:10.1093/brain/awh701
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Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing
1 MRC Cognition and Brain Sciences Unit, 2 Neurology Unit, Addenbrooke's Hospital, 3 Centre for Brain Repair and 4 Department of Pathology, University of Cambridge, Cambridge, UK
Correspondence to: Prof. John R. Hodges, MRC-CBU, 15 Chaucer Road, Cambridge CB2 2EF, UK E-mail: john.hodges{at}mrc-cbu.cam.ac.uk
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.
Key Words: selective verb impairment; parkinsonism; progressive supranuclear palsy (PSP); dementia; ubiquitin-positive inclusions
Abbreviations: CMRglc = cerebral metabolic rate of glucose; DPT = Doors and People Test; DRS = Dementia Rating Scale; FAS =; FDG = 2-[18F]fluoro-2-deoxy-D-glucose; KDT = Kissing and Dancing Test; MMSE = Mini-Mental State Examination; PPT = Pyramids and Palm Trees test; PSP = progressive supranuclear palsy; RBMT = Rivermead Behavioral memory Test; ROI = region of interest; TEA = Test of Everyday Attention; TROG = Test of the Reception of Grammar; WAIS = Wechsler Adult Intelligence Scale; VOSP = Visual Object and Space Perception Battery
Received April 8, 2005. Revised September 15, 2005. Accepted October 27, 2005.
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