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

Mov Disord. 2006 Oct;21(10):1741-4. doi: 10.1002/mds.21001.

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 FXTAS completed a questionnaire to determine whether any medications had been effective for neurological symptoms. Of 11 subjects with definite 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 FXTAS can derive improvement from medication treatment for some of their symptoms.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Aged
  • Anxiety Disorders / drug therapy
  • Benzodiazepines / therapeutic use
  • Carbidopa / therapeutic use
  • Central Nervous System Agents / therapeutic use*
  • Cerebellar Ataxia / drug therapy*
  • Cholinesterase Inhibitors / therapeutic use
  • Dopamine Agonists / therapeutic use
  • Female
  • Fragile X Syndrome / drug therapy*
  • Humans
  • Levodopa / therapeutic use
  • Male
  • Memory Disorders / drug therapy
  • Middle Aged
  • Neurologic Examination / drug effects
  • Parkinsonian Disorders / drug therapy*
  • Serotonin Uptake Inhibitors / therapeutic use
  • Treatment Outcome
  • Tremor / drug therapy*

Substances

  • Adrenergic beta-Antagonists
  • Central Nervous System Agents
  • Cholinesterase Inhibitors
  • Dopamine Agonists
  • Serotonin Uptake Inhibitors
  • Benzodiazepines
  • Levodopa
  • Carbidopa