Early onset of neurological symptoms in fragile X premutation carriers exposed to neurotoxins

Ripon Paul, Isaac N Pessah, Louise Gane, Michele Ono, Paul J Hagerman, James A Brunberg, Flora Tassone, James A. Bourgeois, Patrick E. Adams, Danh V. Nguyen, Randi J Hagerman

Research output: Contribution to journalArticlepeer-review

33 Scopus citations


We present four cases of fragile X premutation carriers with early neurological symptoms, including symptoms consistent with multiple sclerosis (MS) and fragile X-associated tremor/ataxia syndrome (FXTAS). Each patient had significant exposure to one or more environmental neurotoxicants that have documented neurotoxicity (i.e. hexachlorocyclopentadiene or C56, Agent Orange, and 2,4- or 2,6-toluene diisocyanate and dichlormate). We hypothesize that premutation carriers are a vulnerable group to neurotoxins because elevated mRNA in the premutation can lead to early cell death and brain disease, leading to neuropsychiatric and neurological symptoms consistent with FXTAS.

Original languageEnglish (US)
Pages (from-to)399-402
Number of pages4
Issue number4
StatePublished - Aug 2010


  • Agent Orange
  • FMR1
  • Fragile X-associated tremor/ataxia syndrome
  • Hexachlorocyclopentadiene
  • Industrial chemical exposure
  • Premutation

ASJC Scopus subject areas

  • Neuroscience(all)
  • Toxicology


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