Treatment and Management of FXTAS

  • Elizabeth Berry-Kravis
  • Deborah A. Hall
  • Maureen A. Leehey
  • Randi J. Hagerman


Fragile X-associated tremor/ataxia syndrome (FXTAS) affects older adult carriers of the FMR1 premutation and can be associated with a broad array of clinical symptoms and presentations including tremor, ataxia, parkinsonism, executive function disturbance and dementia, psychiatric symptoms of anxiety, depression and disinhibition, peripheral neuropathy, autonomic dysfunction, hormonal dysfunction, and pain syndromes. Although controlled trials have not been carried out in individuals with FXTAS, there is information available regarding symptomatic treatments. Treatment is generally supportive, directed at component symptoms that are most problematic, and makes use of evidence regarding effectiveness of medications and other interventions for treatment of disorders that have phenotypic overlap with FXTAS. This chapter summarizes available treatments and supports that can be helpful for persons with FXTAS.


Deep Brain Stimulation Botulinum Toxin Chronic Fatigue Syndrome Essential Tremor Normal Pressure Hydrocephalus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



This work was partially supported by the following grants: NIA AG032115, NIDCR DE19583, NICHD HD036071, NINDS NS052487, and HD 022074 and 90DD0956 from the Health and Human Services Administration of Developmental Disabilities.


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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Elizabeth Berry-Kravis
    • 1
  • Deborah A. Hall
    • 2
  • Maureen A. Leehey
    • 3
  • Randi J. Hagerman
    • 4
  1. 1.Departments of Pediatrics, Neurological Sciences, and BiochemistryRush University Medical CenterChicagoUSA
  2. 2.Department of Neurological SciencesRush University Medical CenterChicagoUSA
  3. 3.Department of NeurologyUniversity of Colorado Health Sciences CenterDenverUSA
  4. 4.Department of Pediatrics, M.I.N.D. InstituteUniversity of California at Davis Medical CenterSacramentoUSA

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