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 Miller, Amy L. Fong, Randall Carpenter, Cathy Bodine, Louise W. Gane, Edgar Rainin, Hillary Hagerman, Paul J. Hagerman

Research output: Contribution to journalReview article

97 Citations (Scopus)

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

Fingerprint

Parkinsonian Disorders
Peripheral Nervous System Diseases
Tremor
Ataxia
Nervous System Diseases
Psychiatry
Alzheimer Disease
Anxiety
Alleles
Fragile X Tremor Ataxia Syndrome
Depression
Genes
Cognitive Dysfunction

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., ... 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.
Hagerman, Randi J. ; Hall, Deborah A. ; Coffey, Sarah ; Leehey, Maureen ; Bourgeois, James ; Gould, John ; Zhang, Lin ; Seritan, Andreea ; Berry-Kravis, Elizabeth ; Olichney, John ; Miller, Joshua ; Fong, Amy L. ; Carpenter, Randall ; Bodine, Cathy ; Gane, Louise W. ; Rainin, Edgar ; Hagerman, Hillary ; Hagerman, Paul J. / Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems. In: Clinical Interventions in Aging. 2008 ; Vol. 3, No. 2. pp. 251-262.
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Hagerman, RJ, Hall, DA, Coffey, S, Leehey, M, Bourgeois, J, Gould, J, Zhang, L, Seritan, A, Berry-Kravis, E, Olichney, J, Miller, J, Fong, AL, Carpenter, R, Bodine, C, Gane, LW, Rainin, E, Hagerman, H & Hagerman, PJ 2008, 'Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems', Clinical Interventions in Aging, vol. 3, no. 2, pp. 251-262.

Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems. / Hagerman, Randi J.; Hall, Deborah A.; Coffey, Sarah; Leehey, Maureen; Bourgeois, James; Gould, John; Zhang, Lin; Seritan, Andreea; Berry-Kravis, Elizabeth; Olichney, John; Miller, Joshua; Fong, Amy L.; Carpenter, Randall; Bodine, Cathy; Gane, Louise W.; Rainin, Edgar; Hagerman, Hillary; Hagerman, Paul J.

In: Clinical Interventions in Aging, Vol. 3, No. 2, 29.07.2008, p. 251-262.

Research output: Contribution to journalReview article

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AU - Hagerman, Randi J.

AU - Hall, Deborah A.

AU - Coffey, Sarah

AU - Leehey, Maureen

AU - Bourgeois, James

AU - Gould, John

AU - Zhang, Lin

AU - Seritan, Andreea

AU - Berry-Kravis, Elizabeth

AU - Olichney, John

AU - Miller, Joshua

AU - Fong, Amy L.

AU - Carpenter, Randall

AU - Bodine, Cathy

AU - Gane, Louise W.

AU - Rainin, Edgar

AU - Hagerman, Hillary

AU - Hagerman, Paul J.

PY - 2008/7/29

Y1 - 2008/7/29

N2 - 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.

AB - 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.

KW - Ataxia

KW - Dementia

KW - Fragile X syndrome

KW - Neurodegeneraton

KW - Parkinsonism

KW - Tremor

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Hagerman RJ, Hall DA, Coffey S, Leehey M, Bourgeois J, Gould J et al. Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems. Clinical Interventions in Aging. 2008 Jul 29;3(2):251-262.