Brain Advance Access published online on December 5, 2005
Brain, doi:10.1093/brain/awh701
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1 MRC Cognition and Brain Sciences Unit, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK; Neurology Unit, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK
* To whom correspondence should be addressed. 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.
Received April 8, 2005
Revised September 15, 2005
Accepted October 27, 2005
Article
Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing
Thomas H. Bak 1,
Despina Yancopoulou 2,
Peter J. Nestor 3,
John H. Xuereb 4,
Maria G. Spillantini 2,
Friedemann Pulvermüller 5,
and
John R. Hodges 1 *
2 Neurology Unit, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK; Centre for Brain Repair, University of Cambridge, Cambridge, UK
3 Neurology Unit, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK
4 Department of Pathology, University of Cambridge, Cambridge, UK
5 MRC Cognition and Brain Sciences Unit, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK
John R. Hodges, E-mail: john.hodges{at}mrc-cbu.cam.ac.uk
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