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

Randi J Hagerman1,2, Deborah A Hall3, Sarah Coffey1,2, Maureen Leehey3, James Bourgeois4, John Gould5, Lin Zhang6, Andreea Seritan4, Elizabeth Berry-Kravis7–9, John Olichney6, Joshua W Miller10, Amy L Fong11, Randall Carpenter12, Cathy Bodine13, Louise W Gane1,2, Edgar Rainin1, Hillary Hag...

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Autores principales:	Randi J Hagerman, Deborah A Hall, Sarah Coffey, Maureen Leehey, James Bourgeois, et al
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2008
Materias:	Geriatrics RC952-954.6
Acceso en línea:	https://doaj.org/article/88a08fb93ee1401f94007fdb6717d763
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spelling	oai:doaj.org-article:88a08fb93ee1401f94007fdb6717d7632021-12-02T03:35:54ZTreatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems1178-1998https://doaj.org/article/88a08fb93ee1401f94007fdb6717d7632008-06-01T00:00:00Zhttps://www.dovepress.com/treatment-of-fragile-x-associated-tremor-ataxia-syndrome-fxtas-and-rel-peer-reviewed-article-CIAhttps://doaj.org/toc/1178-1998Randi J Hagerman1,2, Deborah A Hall3, Sarah Coffey1,2, Maureen Leehey3, James Bourgeois4, John Gould5, Lin Zhang6, Andreea Seritan4, Elizabeth Berry-Kravis7–9, John Olichney6, Joshua W Miller10, Amy L Fong11, Randall Carpenter12, Cathy Bodine13, Louise W Gane1,2, Edgar Rainin1, Hillary Hagerman1, Paul J Hagerman141M.I.N.D. Institute, 2Department of Pediatrics, 4Department of Psychiatry & Behavioral Sciences, 5Department of Urology, 6Department of Neurology, 10Department of Pathology and Laboratory Medicine, 14Department of Biochemistry and Molecular Medicine, University of California, Davis, School of Medicine, Sacramento, CA, USA; 3Department of Neurology, University of Colorado, Denver, CO, USA; 7Department of Pediatrics, Neurology, and Biochemistry, 8Department of Neurological Sciences, 9Department of Biochemistry, Rush University Medical Center, Chicago, IL, USA; 11Physical Edge, Inc., Davis, CA, USA; 12Seaside Therapeutics, Cambridge, MA, USA; 13Department of Physical Medicine and Rehabilitation, University of Colorado Health Sciences Center, Denver, CO, USAAbstract: 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.Keywords: fragile X syndrome, dementia, ataxia, neurodegeneration, parkinsonism, tremorRandi J HagermanDeborah A HallSarah CoffeyMaureen LeeheyJames Bourgeoiset alDove Medical PressarticleGeriatricsRC952-954.6ENClinical Interventions in Aging, Vol Volume 3, Pp 251-262 (2008)
institution	DOAJ
collection	DOAJ
language	EN
topic	Geriatrics RC952-954.6
spellingShingle	Geriatrics RC952-954.6 Randi J Hagerman Deborah A Hall Sarah Coffey Maureen Leehey James Bourgeois et al Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems
description	Randi J Hagerman1,2, Deborah A Hall3, Sarah Coffey1,2, Maureen Leehey3, James Bourgeois4, John Gould5, Lin Zhang6, Andreea Seritan4, Elizabeth Berry-Kravis7–9, John Olichney6, Joshua W Miller10, Amy L Fong11, Randall Carpenter12, Cathy Bodine13, Louise W Gane1,2, Edgar Rainin1, Hillary Hagerman1, Paul J Hagerman141M.I.N.D. Institute, 2Department of Pediatrics, 4Department of Psychiatry & Behavioral Sciences, 5Department of Urology, 6Department of Neurology, 10Department of Pathology and Laboratory Medicine, 14Department of Biochemistry and Molecular Medicine, University of California, Davis, School of Medicine, Sacramento, CA, USA; 3Department of Neurology, University of Colorado, Denver, CO, USA; 7Department of Pediatrics, Neurology, and Biochemistry, 8Department of Neurological Sciences, 9Department of Biochemistry, Rush University Medical Center, Chicago, IL, USA; 11Physical Edge, Inc., Davis, CA, USA; 12Seaside Therapeutics, Cambridge, MA, USA; 13Department of Physical Medicine and Rehabilitation, University of Colorado Health Sciences Center, Denver, CO, USAAbstract: 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.Keywords: fragile X syndrome, dementia, ataxia, neurodegeneration, parkinsonism, tremor
format	article
author	Randi J Hagerman Deborah A Hall Sarah Coffey Maureen Leehey James Bourgeois et al
author_facet	Randi J Hagerman Deborah A Hall Sarah Coffey Maureen Leehey James Bourgeois et al
author_sort	Randi J Hagerman
title	Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems
title_short	Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems
title_full	Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems
title_fullStr	Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems
title_full_unstemmed	Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems
title_sort	treatment of fragile x-associated tremor ataxia syndrome (fxtas) and related neurological problems
publisher	Dove Medical Press
publishDate	2008
url	https://doaj.org/article/88a08fb93ee1401f94007fdb6717d763
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Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems

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