Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing

Thomas H Bak, Despina Yancopoulou, Peter J Nestor, John H Xuereb, Maria G Spillantini, Friedemann Pulvermüller, John R Hodges

Research output: Contribution to journalArticlepeer-review

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.
Original languageEnglish
Pages (from-to)321-32
Number of pages12
JournalBrain
Volume129
Issue numberPt 2
DOIs
Publication statusPublished - 2006

Keywords

  • Adult
  • Aged
  • Dementia
  • Female
  • Frontal Lobe
  • Heterozygote
  • Humans
  • Immunohistochemistry
  • Inclusion Bodies
  • Male
  • Motion Perception
  • Neurites
  • Neurons
  • Neuropsychological Tests
  • Pedigree
  • Positron-Emission Tomography
  • Supranuclear Palsy, Progressive
  • Ubiquitin
  • Verbal Behavior
  • tau Proteins

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