Prepulse inhibition in patients with fragile X-associated tremor ataxia syndrome

Andrea Schneider, Elizabeth Ballinger, Alyssa Chavez, Flora Tassone, Randi J Hagerman, David R Hessl

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset neurodegenerative disorder that affects carriers of the fragile X premutation, typically after age 50. Common symptoms include intention tremor, ataxia, neuropathy, autonomic dysfunction, cognitive decline, and dementia. The objectives of this study were to determine if patients with FXTAS have altered prepulse inhibition (PPI; a measure of sensorimotor gating), and to study possible correlations between PPI, molecular status, and cognitive performance. A passive acoustic PPI paradigm was applied in 163 subjects; 121 carriers of the fragile X premutation, and 42 healthy controls. There were significant differences in PPI between premutation carriers with FXTAS and controls at PPI 60 ms, and at 120 ms. This effect was more prominent in the male FXTAS patients. There was a tendency to an impaired PPI in female premutation carriers at the 120 ms condition. There was a significant correlation between the PPI deficit and a higher CGG repeat number. The results show an impairment in sensorimotor gating processes in male carriers of the fragile X premutation, which is more prominent in patients with FXTAS.

Original languageEnglish (US)
Pages (from-to)1045-1053
Number of pages9
JournalNeurobiology of Aging
Issue number6
StatePublished - Jun 2012


  • Fragile X premutation
  • PPI
  • Prepulse inhibition
  • Sensorimotor gating

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)
  • Aging
  • Developmental Biology
  • Geriatrics and Gerontology


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