Cognitive-motor interference during postural control indicates at-risk cerebellar profiles in females with the FMR1 premutation

Behav Brain Res. 2013 Sep 15:253:329-36. doi: 10.1016/j.bbr.2013.07.033. Epub 2013 Jul 27.

Abstract

Recent investigations report a higher risk of motor symptoms in females with the FMR1 premutation (PM-carriers) than has hitherto been appreciated. Here we examined basic sensorimotor and postural control under different sensory and attentional dual-task demands. Physiological performance and postural sway measures from the Physiological Profile Assessment (Lord et al., 2003 [39]) were conducted in 28 female PM-carriers (mean age: 41.32±8.03) and 31 female controls with normal FMR1 alleles (mean age: 41.61±8.3). Multiple regression analyses were conducted to examine the moderating role of CGG-repeat length on the relation between age and postural sway under dual-task interference. In female PM-carriers, our results showed significantly poorer proprioceptive awareness, slower reaction time, and greater postural displacement when performing a concurrent verbal fluency task. Significantly, these findings showed age- and genetically-modulated changes in dual-task postural displacement in the medio-lateral direction in female PM-carriers. These findings highlight the sensitivity of postural control paradigms in identifying early cerebellar postural changes that may act as surrogate markers of future decline in female PM-carriers.

Keywords: Cerebellar motor networks; Cognitive-neuromotor interaction; Fragile X Mental Retardation gene 1 (FMR1); Fragile X Mental Retardation protein (FMRP); Fragile X Tremor Ataxia Syndrome (FXTAS); Physiological Profile Assessment; Postural control.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aging / physiology
  • Cerebellum / physiopathology*
  • Cognition / physiology*
  • Cohort Studies
  • DNA / genetics
  • Female
  • Fragile X Mental Retardation Protein / genetics*
  • Fragile X Syndrome / genetics*
  • Fragile X Syndrome / physiopathology*
  • Fragile X Syndrome / psychology
  • Humans
  • Middle Aged
  • Movement / physiology*
  • Mutation / genetics*
  • Postural Balance / genetics
  • Postural Balance / physiology
  • Posture / physiology*
  • Proprioception / genetics
  • Proprioception / physiology
  • Psychomotor Performance / physiology
  • Trinucleotide Repeat Expansion
  • Trinucleotide Repeats / genetics
  • Verbal Behavior / physiology
  • Young Adult

Substances

  • FMR1 protein, human
  • Fragile X Mental Retardation Protein
  • DNA