Cerebellar vermis abnormalities and cognitive functions in individuals with Williams syndrome

Res Dev Disabil. 2013 Jul;34(7):2118-26. doi: 10.1016/j.ridd.2013.03.026. Epub 2013 Apr 30.

Abstract

In Williams syndrome (WS) cerebellar measures were only indirectly related to behavioral outcomes. T1-weighted magnetic resonance images and neuropsychological data were acquired to investigate whether cerebellar vermis differences were present in 12 WS individuals compared with 13 chronological age-matched controls and whether WS cerebellar vermis measures were related to cognitive scores. In WS participants, we observed a significant increase in the volume of the posterior superior cerebellar vermis (lobules VI-VII) and an atypical ratio between width and height of the cerebellar vermis. Furthermore, we found an inverse correlation between cerebellar posterior vermis volume and scores on implicit learning, phonological fluency and the verbal short-term memory tasks. The present study supported a role for the posterior cerebellar vermis in higher cognitive processes and indicated that the cerebellar vermis abnormalities (enlargement) in WS individuals have an effect in worsening the cognitive performance in specific domains.

MeSH terms

  • Adolescent
  • Adult
  • Cerebellum / pathology*
  • Cognition / physiology*
  • Female
  • Humans
  • Male
  • Neuropsychological Tests
  • Williams Syndrome / pathology*
  • Williams Syndrome / psychology