Symptomatic treatment in the fragile X-associated tremor/ataxia syndrome

Deborah A. Hall, Elizabeth Berry-Kravis, Randi J Hagerman, Paul J Hagerman, Cathlin D. Rice, Maureen A. Leehey

Research output: Contribution to journalArticle

47 Scopus citations

Abstract

There is no established treatment for the neurological features of the recently discovered fragile X-associated tremor/ataxia syndrome (FXTAS). Fifty-six patients with FX-TAS completed a questionnaire to determine whether any medications had been effective for neurological symptoms. Of 11 subjects with defirdte FXTAS, 8 (70%) were on medications for their neurological symptoms, whereas most subjects with possible or probable FXTAS, 31 (70%) of 45 subjects, were not on medications. Although no therapy was uniformly effective for intention tremor, ataxia, Parkinsonism, memory loss, or anxiety, some subjects with intention tremor or Parkinsonism reported improvement with medications frequently used in other movement disorders. Overall., all 22 subjects on medications reported improvement in one or more symptoms. Lack of insight, recall bias, and cognitive impairment may have resulted in an underestimation of the beneficial effect of medical therapy. This study suggests that patients with FX-TAS can derive improvement from medication treatment for some of their symptoms.

Original languageEnglish (US)
Pages (from-to)1741-1744
Number of pages4
JournalMovement Disorders
Volume21
Issue number10
DOIs
StatePublished - Oct 2006

Keywords

  • Ataxia
  • Fragile X
  • FXTAS
  • Medications
  • Tremor

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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