Neurological soft signs feature a double dissociation within the language system in Williams syndrome

Alessandro Tavano, Chiara Gagliardi, Sara Martelli, R. Borgatti

Research output: Contribution to journalArticlepeer-review

Abstract

The neurocognitive profile of Williams-Beuren syndrome (WBS) is characterized by visuospatial deficits, apparently fluent language, motor soft signs, and hypersociability. We investigated the association between neuromotor soft signs and visuospatial, executive-attentive, mnestic and linguistic functions in a group of 26 children and young adults with WBS. We hypothesized that neurological soft signs could be an index of subtle neurofunctional deficits and thus provide a behavioural window into the processes underlying neurocognition in Williams-Beuren syndrome. Dysmetria and dystonic movements were selected as grouping neurological variables, indexing cerebellar and basal ganglia dysfunction, respectively. No detrimental effects on visuospatial/visuoconstructive skills were evident following the presence of either neurological variable. As for language skills, participants with dysmetria showed markedly reduced expressive syntactic and lexico-semantic skills as compared to non-affected individuals, while no difference in chronological age was evident. Participants with dystonic movements showed reduced receptive syntax and increased lexical comprehension skills as compared to non-affected individuals, the age factor being significant. In both instances, the effect size was greater for syntactic measures. We take these novel findings as suggestive of a double dissociation between expressive and receptive skills at sentence level within the WBS linguistic phenotype. The investigation of neuromotor soft signs and neuropsychological functions may provide a key to new non-cortico-centric genotype/phenotype relationships.

Original languageEnglish
Pages (from-to)3298-3304
Number of pages7
JournalNeuropsychologia
Volume48
Issue number11
DOIs
Publication statusPublished - Sep 2010

Keywords

  • Basal ganglia
  • Cerebellum
  • Cognition
  • Neurological signs
  • Visuospatial skills
  • Williams-Beuren syndrome

ASJC Scopus subject areas

  • Behavioral Neuroscience
  • Cognitive Neuroscience
  • Experimental and Cognitive Psychology

Fingerprint Dive into the research topics of 'Neurological soft signs feature a double dissociation within the language system in Williams syndrome'. Together they form a unique fingerprint.

Cite this