Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems

Randi J. Hagerman, Deborah A. Hall, Sarah Coffey, Maureen Leehey, James Bourgeois, John Gould, Lin Zhang, Andreea Seritan, Elizabeth Berry-Kravis, John Olichney, Joshua W. Miller, Amy L. Fong, Randall Carpenter, Cathy Bodine, Louise W. Gane, Edgar Rainin, Hillary Hagerman, Paul J. Hagerman

Research output: Contribution to journalReview article

102 Scopus citations

Abstract

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a progressive neurological disorder that affects older adult carriers, predominantly males, of premutation alleles (55 to 200 CGG repeats) of the fragile X (FMR1) gene. Principal features of FXTAS are intention tremor, ataxia, parkinsonism, cognitive decline, and peripheral neuropathy; ancillary features include, autonomic dysfunction, and psychiatric symptoms of anxiety, depression, and disinhibition. Although controlled trials have not been carried out in individuals with FXTAS, there is a significant amount of anecdotal information regarding various treatment modalities. Moreover, there exists a great deal of evidence regarding the efficacy of various medications for treatment of other disorders (eg, Alzheimer disease) that have substantial phenotypic overlap with FXTAS. The current review summarizes what is currently known regarding the symptomatic treatment, or potential for treatment, of FXTAS.

Original languageEnglish (US)
Pages (from-to)251-262
Number of pages12
JournalClinical Interventions in Aging
Volume3
Issue number2
StatePublished - Jul 29 2008
Externally publishedYes

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All Science Journal Classification (ASJC) codes

  • Geriatrics and Gerontology

Keywords

  • Ataxia
  • Dementia
  • Fragile X syndrome
  • Neurodegeneraton
  • Parkinsonism
  • Tremor

Cite this

Hagerman, R. J., Hall, D. A., Coffey, S., Leehey, M., Bourgeois, J., Gould, J., Zhang, L., Seritan, A., Berry-Kravis, E., Olichney, J., Miller, J. W., Fong, A. L., Carpenter, R., Bodine, C., Gane, L. W., Rainin, E., Hagerman, H., & Hagerman, P. J. (2008). Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems. Clinical Interventions in Aging, 3(2), 251-262.