Advanced search
Publication Cover
Journal of Clinical and Experimental Neuropsychology Volume 30, 2008 - Issue 8
Submit an article Journal homepage
260
Views
65
CrossRef citations to date
0
Altmetric
Original Articles

The primary cognitive deficit among males with fragile X-associated tremor/ataxia syndrome (FXTAS) is a dysexecutive syndrome

Angela G. Brega Department of Medicine, University of Colorado, Denver, Aurora, CO, USA
,
Glenn Goodrich Department of Medicine, University of Colorado, Denver, Aurora, CO, USA
,
Rachael E. Bennett Department of Medicine, University of Colorado, Denver, Aurora, CO, USA
,
David Hessl M.I.N.D. Institute, University of California, Davis, Medical Center, Sacramento, CA, USA; Department of Psychiatry and Behavioral Sciences, University of California, Davis, Medical Center, Sacramento, CA, USA
,
Karen Engle Department of Medicine, University of Colorado, Denver, Aurora, CO, USA
,
Maureen A. Leehey Department of Neurology, University of Colorado, Denver, CO, USA
,
Lanee S. Bounds Department of Medicine, University of Colorado, Denver, Aurora, CO, USA
,
Marsha J. Paulich Department of Medicine, University of Colorado, Denver, Aurora, CO, USA
,
Randi J. Hagerman M.I.N.D. Institute, University of California, Davis, Medical Center, Sacramento, CA, USA; Department of Pediatrics, University of California, Davis, Medical Center, Sacramento, CA, USA
,
Paul J. Hagerman Department of Biochemistry and Molecular Medicine, University of California, Davis, School of Medicine, Davis, CA, USA
,
Jennifer B. Cogswell M.I.N.D. Institute, University of California, Davis, Medical Center, Sacramento, CA, USA
,
Flora Tassone Department of Biochemistry and Molecular Medicine, University of California, Davis, School of Medicine, Davis, CA, USA
,
Ann Reynolds The Children's Hospital, Denver, CO, USA
,
Robert Kooken Department of Medicine, University of Colorado, Denver, Aurora, CO, USA
,
Michael Kenny C.U. Aging Center, University of Colorado at Colorado Springs, Colorado Springs, CO, USA
&
Jim Grigsby Department of Medicine, University of Colorado, Denver, Aurora, CO, USACorrespondence[email protected]
 show all
Pages 853-869 | Received 01 Oct 2007, Accepted 19 Nov 2007, Published online: 20 Oct 2008

Citations (65)

Keep up to date with the latest research on this topic with citation updates for this article.

Subscribe to citation updates

Read on this site (6)

Erin E. Robertson, Deborah A. Hall, Andrew R. McAsey & Joan A. O’Keefe. (2016) Fragile X-associated tremor/ataxia syndrome: phenotypic comparisons with other movement disorders. The Clinical Neuropsychologist 30:6, pages 849-900.
Read now
Jim Grigsby, Angela G. Brega, Rachael E. Bennett, James A. Bourgeois, Andreea L. Seritan, Glenn K. Goodrich & Randi J. Hagerman. (2016) Clinically significant psychiatric symptoms among male carriers of the fragile X premutation, with and without FXTAS, and the mediating influence of executive functioning. The Clinical Neuropsychologist 30:6, pages 944-959.
Read now
Jim Grigsby. (2016) The fragile X mental retardation 1 gene (FMR1): historical perspective, phenotypes, mechanism, pathology, and epidemiology. The Clinical Neuropsychologist 30:6, pages 815-833.
Read now
Christopher M. Filley. (2016) Fragile X tremor ataxia syndrome and white matter dementia. The Clinical Neuropsychologist 30:6, pages 901-912.
Read now
Rachael Cherie Birch, Darren Robert Hocking & Julian Norman Trollor. (2016) Prevalence and predictors of subjective memory complaints in adult male carriers of the FMR1 premutation. The Clinical Neuropsychologist 30:6, pages 834-848.
Read now
Louise W. Gane, Ana-Maria Iosif, Linda Flynn-Wilson, Marielle Venturino, Randi J. Hagerman & Andreea L. Seritan. (2010) Assessment of patient and caregiver needs in fragile X-associated tremor/ataxia syndrome by utilizing Q-sort methodology. Aging & Mental Health 14:8, pages 1000-1007.
Read now

Articles from other publishers (59)

Heather Fielding-Gebhardt, Shannon E. Kelly, Kathryn E. Unruh, Lauren M. Schmitt, Stormi L. Pulver, Pravin Khemani & Matthew W. Mosconi. (2023) Sensorimotor and inhibitory control in aging FMR1 premutation carriers. Frontiers in Human Neuroscience 17.
Crossref
Osnat Segal, Tamar Kowal, Yonit Banet-Levi & Lidia V. Gabis. (2023) Executive Function and Working Memory Deficits in Females with Fragile X Premutation. Life 13:3, pages 813.
Crossref
Nell Maltman, Leann Smith DaWalt, Jinkuk Hong, Mei Wang Baker, Elizabeth M. Berry-Kravis, Murray H. Brilliant & Marsha Mailick. (2023) FMR1 CGG Repeats and Stress Influence Self-Reported Cognitive Functioning in Mothers . American Journal on Intellectual and Developmental Disabilities 128:1, pages 1-20.
Crossref
Jinkuk Hong, Robert S. Dembo, Leann Smith DaWalt, Murray Brilliant, Elizabeth M. Berry-Kravis & Marsha Mailick. (2022) The effect of college degree attainment on neurodegenerative symptoms in genetically at-risk women. SSM - Population Health 19, pages 101262.
Crossref
Sizhe Zhang, Lu Shen & Bin Jiao. (2022) Cognitive Dysfunction in Repeat Expansion Diseases: A Review. Frontiers in Aging Neuroscience 14.
Crossref
Jun Yi Wang, Jim Grigsby, Diego Placido, Hongjiang Wei, Flora Tassone, Kyoungmi Kim, David Hessl, Susan M. Rivera & Randi J. Hagerman. (2022) Clinical and Molecular Correlates of Abnormal Changes in the Cerebellum and Globus Pallidus in Fragile X Premutation. Frontiers in Neurology 13.
Crossref
Nell Maltman, Janna Guilfoyle, Kritika Nayar, Gary E. Martin, Molly Winston, Joseph C. Y. Lau, Lauren Bush, Shivani Patel, Michelle Lee, John Sideris, Deborah A. Hall, Lili Zhou, Kevin Sharp, Elizabeth Berry-Kravis & Molly Losh. (2021) The Phenotypic Profile Associated With the FMR1 Premutation in Women: An Investigation of Clinical-Behavioral, Social-Cognitive, and Executive Abilities. Frontiers in Psychiatry 12.
Crossref
Andrea Schneider, Scott Summers, Flora Tassone, Andreea Seritan, David Hessl, Paul Hagerman & Randi Hagerman. (2020) Women with Fragile X–associated Tremor/Ataxia Syndrome. Movement Disorders Clinical Practice 7:8, pages 910-919.
Crossref
Maria Jimena Salcedo-Arellano, Ana Maria Cabal-Herrera, Nattaporn Tassanakijpanich, Yingratana A. McLennan & Randi J. Hagerman. (2020) Ataxia as the Major Manifestation of Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS): Case Series. Biomedicines 8:5, pages 136.
Crossref
Fariha Islam & Will Lee. (2020) A Case of Generalized Chorea Presenting as an Early Feature of Fragile‐X Associated Tremor/Ataxia Syndrome . Movement Disorders Clinical Practice 7:4, pages 464-466.
Crossref
Elena Palomino, María Sotillo & José María López-Frutos. (2020) Functioning of Cognitive Memory Inhibition Processes in People with Down Syndrome: An Empirical Study. The Spanish Journal of Psychology 23.
Crossref
M.J. Leigh & R.J. Hagerman. 2020. Neurodevelopmental Disorders. Neurodevelopmental Disorders 351 375 .
M.J. Leigh & R.J. Hagerman. 2020. Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease. Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease 311 332 .
Wilmar Saldarriaga, María J. Salcedo‐Arellano, Tatiana Rodriguez‐Guerrero, Marcela Ríos, Andrés Fandiño‐Losada, Julian Ramirez‐Cheyne, Pamela J. Lein, Flora Tassone & Randi J. Hagerman. (2018) Increased severity of fragile X spectrum disorders in the agricultural community of Ricaurte, Colombia. International Journal of Developmental Neuroscience 72:1, pages 1-5.
Crossref
Ryan Shickman, Jessica Famula, Flora Tassone, Maureen Leehey, Emilio Ferrer, Susan M. Rivera & David Hessl. (2018) Age- and CGG repeat-related slowing of manual movement in fragile X carriers: A prodrome of fragile X-associated tremor ataxia syndrome?. Movement Disorders 33:4, pages 628-636.
Crossref
Chava Creque & Stephanie A. Kolakowsky-Hayner. 2018. Encyclopedia of Clinical Neuropsychology. Encyclopedia of Clinical Neuropsychology 1249 1249 .
Annie L Shelton, Kim Cornish & Joanne Fielding. (2017) Long term verbal memory recall deficits in fragile X premutation females. Neurobiology of Learning and Memory 144, pages 131-135.
Crossref
Chava Creque & Stephanie A. Kolakowsky-Hayner. 2017. Encyclopedia of Clinical Neuropsychology. Encyclopedia of Clinical Neuropsychology 1 2 .
Annie L. Shelton, Kim M. Cornish, Claudine M. Kraan, Reymundo Lozano, Minh Bui & Joanne Fielding. (2016) Executive Dysfunction in Female FMR1 Premutation Carriers. The Cerebellum 15:5, pages 565-569.
Crossref
Cecilia Giulivi, Eleonora Napoli, Flora Tassone, Julian Halmai & Randi Hagerman. (2016) Plasma Biomarkers for Monitoring Brain Pathophysiology in FMR1 Premutation Carriers. Frontiers in Molecular Neuroscience 9.
Crossref
Randi J. Hagerman & Paul Hagerman. (2016) Fragile X-associated tremor/ataxia syndrome — features, mechanisms and management. Nature Reviews Neurology 12:7, pages 403-412.
Crossref
Jin-Chen Yang, Annette Rodriguez, Ashley Royston, Yu-Qiong Niu, Merve Avar, Ryan Brill, Christa Simon, Jim Grigsby, Randi J. Hagerman & John M. Olichney. (2016) Memantine Improves Attentional Processes in Fragile X-Associated Tremor/Ataxia Syndrome: Electrophysiological Evidence from a Randomized Controlled Trial. Scientific Reports 6:1.
Crossref
Praveen Dayalu, Roger L. Albin & Henry Paulson. 2016. Non‐Alzheimer's and Atypical Dementia. Non‐Alzheimer's and Atypical Dementia 90 102 .
Emily G. Allen, Maureen A. Leehey, Flora Tassone & Stephanie Sherman. 2016. FXTAS, FXPOI, and Other Premutation Disorders. FXTAS, FXPOI, and Other Premutation Disorders 129 160 .
Emily S. Halket, Jun Yi Wang, David Hessl & Susan M. Rivera. 2016. FXTAS, FXPOI, and Other Premutation Disorders. FXTAS, FXPOI, and Other Premutation Disorders 71 85 .
Jim Grigsby, Andreea L. Seritan, James A. Bourgeois & Anson Kairys. 2016. FXTAS, FXPOI, and Other Premutation Disorders. FXTAS, FXPOI, and Other Premutation Disorders 39 69 .
Randi J. Hagerman, Anne Wheeler, Sarah Fitzpatrick & Jessica Hunter. 2016. FXTAS, FXPOI, and Other Premutation Disorders. FXTAS, FXPOI, and Other Premutation Disorders 241 262 .
Christopher M. FilleyMark S. BrownKaren OnderkoMegan RayRachael E. BennettElizabeth Berry-KravisJim Grigsby. (2015) White matter disease and cognitive impairment in FMR1 premutation carriers . Neurology 84:21, pages 2146-2152.
Crossref
Annie L. Shelton, Kim M. Cornish, David E. Godler, Meaghan Clough, Claudine Kraan, Minh Bui & Joanne Fielding. (2015) Delineation of the working memory profile in female FMR1 premutation carriers: The effect of cognitive load on ocular motor responses. Behavioural Brain Research 282, pages 194-200.
Crossref
Ling M. Wong, Flora Tassone, Susan M. Rivera & Tony J. Simon. (2015) Temporal dynamics of attentional selection in adult male carriers of the fragile X premutation allele and adult controls. Frontiers in Human Neuroscience 9.
Crossref
Ling M Wong, Naomi J Goodrich-Hunsaker, Yingratana A McLennan, Flora Tassone, Susan M Rivera & Tony J Simon. (2014) A cross-sectional analysis of orienting of visuospatial attention in child and adult carriers of the fragile X premutation. Journal of Neurodevelopmental Disorders 6:1.
Crossref
Anne C Wheeler, Donald B Bailey Jr, Elizabeth Berry-Kravis, Jan Greenberg, Molly Losh, Marsha Mailick, Montserrat Milà, John M Olichney, Laia Rodriguez-Revenga, Stephanie Sherman, Leann Smith, Scott Summers, Jin-Chen Yang & Randi Hagerman. (2014) Associated features in females with an FMR1 premutation. Journal of Neurodevelopmental Disorders 6:1.
Crossref
Jim Grigsby, Kim Cornish, Darren Hocking, Claudine Kraan, John M Olichney, Susan M Rivera, Andrea Schneider, Stephanie Sherman, Jun Yi Wang & Jin-Chen Yang. (2014) The cognitive neuropsychological phenotype of carriers of the FMR1 premutation. Journal of Neurodevelopmental Disorders 6:1.
Crossref
R. C. Birch, K. M. Cornish, D. R. Hocking & J. N. Trollor. (2014) Understanding the Neuropsychiatric Phenotype of Fragile X-Associated Tremor Ataxia Syndrome: a Systematic Review. Neuropsychology Review 24:4, pages 491-513.
Crossref
Jin-Chen Yang, Yu-Qiong Niu, Christa Simon, Andreea L Seritan, Lawrence Chen, Andrea Schneider, Shayan T Moghaddam, Paul J Hagerman, Randi J Hagerman & John M Olichney. (2014) Memantine Effects on Verbal Memory in Fragile X-associated Tremor/Ataxia Syndrome (FXTAS): a Double-Blind Brain Potential Study. Neuropsychopharmacology 39:12, pages 2760-2768.
Crossref
Annie L. Shelton, Kim Cornish, Claudine Kraan, Nellie Georgiou-Karistianis, Sylvia A. Metcalfe, John L. Bradshaw, Darren R. Hocking, Alison D. Archibald, Jonathan Cohen, Julian N. Trollor & Joanne Fielding. (2014) Exploring inhibitory deficits in female premutation carriers of fragile X syndrome: Through eye movements. Brain and Cognition 85, pages 201-208.
Crossref
J.-C. Yang, C. Simon, A. Schneider, A. L. Seritan, L. Hamilton, P. J. Hagerman, R. J. Hagerman & J. M. Olichney. (2014) Abnormal semantic processing in females with fragile X-associated tremor/ataxia syndrome. Genes, Brain and Behavior 13:2, pages 152-162.
Crossref
J.-C. Yang, S.-H. Chan, S. Khan, A. Schneider, R. Nanakul, S. Teichholtz, Y.-Q. Niu, A. Seritan, F. Tassone, J. Grigsby, P. J. Hagerman, R. J. Hagerman & J. M. Olichney. (2012) Neural Substrates of Executive Dysfunction in Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS): a Brain Potential Study. Cerebral Cortex 23:11, pages 2657-2666.
Crossref
Jun Yi Wang, Randi J. Hagerman & Susan M. Rivera. (2013) A multimodal imaging analysis of subcortical gray matter in fragile X premutation carriers. Movement Disorders 28:9, pages 1278-1284.
Crossref
Andrea Schneider, Andrew Ligsay & Randi J. Hagerman. (2013) Fragile X syndrome: An aging perspective. Developmental Disabilities Research Reviews 18:1, pages 68-74.
Crossref
Audra M. Sterling, Marsha Mailick, Jan Greenberg, Steven F. Warren & Nancy Brady. (2013) Language dysfluencies in females with the FMR1 premutation. Brain and Cognition 82:1, pages 84-89.
Crossref
Claudine M. Kraan, Darren R. Hocking, John L. Bradshaw, Joanne Fielding, Jonathan Cohen, Nellie Georgiou-Karistianis & Kim M. Cornish. (2013) Neurobehavioural evidence for the involvement of the FMR1 gene in female carriers of fragile X syndrome. Neuroscience & Biobehavioral Reviews 37:3, pages 522-547.
Crossref
M.J. Leigh & R.J. Hagerman. 2013. Neural Circuit Development and Function in the Brain. Neural Circuit Development and Function in the Brain 631 650 .
Ling M Wong, Naomi J Goodrich-Hunsaker, Yingratana McLennan, Flora Tassone, Danielle Harvey, Susan M Rivera & Tony J Simon. (2012) Young adult male carriers of the fragile X premutation exhibit genetically modulated impairments in visuospatial tasks controlled for psychomotor speed. Journal of Neurodevelopmental Disorders 4:1.
Crossref
F. Tassone, C. M. Greco, M. R. Hunsaker, A. L. Seritan, R. F. Berman, L. W. Gane, S. Jacquemont, K. Basuta, L.‐W. Jin, P. J. Hagerman & R. J. Hagerman. (2012) Neuropathological, clinical and molecular pathology in female fragile X premutation carriers with and without FXTAS. Genes, Brain and Behavior 11:5, pages 577-585.
Crossref
Darren R. Hocking, Cary S. Kogan & Kim M. Cornish. (2012) Selective spatial processing deficits in an at-risk subgroup of the fragile X premutation. Brain and Cognition 79:1, pages 39-44.
Crossref
Maureen A. Leehey & Paul J. Hagerman. 2012. Ataxic Disorders. Ataxic Disorders 373 386 .
Flora Tassone & Randi Hagerman. 2012. Modeling Fragile X Syndrome. Modeling Fragile X Syndrome 337 357 .
Michael R. Hunsaker, Gloria Arque, Robert F. Berman, Rob Willemsen & Renate K. Hukema. 2012. Modeling Fragile X Syndrome. Modeling Fragile X Syndrome 255 269 .
Kim M. CornishDarren R. HockingSimon A. MossCary S. Kogan. (2011) Selective executive markers of at-risk profiles associated with the fragile X premutation. Neurology 77:7, pages 618-622.
Crossref
Jorge L. Juncos, Joash T. Lazarus, Emily Graves-Allen, Lisa Shubeck, Michelle Rusin, Gloria Novak, Deborah Hamilton, Julia Rohr & Stephanie L. Sherman. (2011) New clinical findings in the fragile X-associated tremor ataxia syndrome (FXTAS). neurogenetics 12:2, pages 123-135.
Crossref
Beth Kuczynski & Stephanie A. Kolakowsky-Hayner. 2011. Encyclopedia of Clinical Neuropsychology. Encyclopedia of Clinical Neuropsychology 912 912 .
Cary S. Kogan & Kim M. Cornish. (2010) Mapping self-reports of working memory deficits to executive dysfunction in Fragile X Mental Retardation 1 (FMR1) gene premutation carriers asymptomatic for FXTAS. Brain and Cognition 73:3, pages 236-243.
Crossref
E. Amirian, Judith Baxter, Jim Grigsby, Douglas Curran-Everett, John E. Hokanson & Lucinda L. Bryant. (2010) Executive function (capacity for behavioral self-regulation) and decline predicted mortality in a longitudinal study in Southern Colorado. Journal of Clinical Epidemiology 63:3, pages 307-314.
Crossref
Jim Grigsby, Angela G. Brega, Andreea L. Seritan & James A. Bourgeois. 2010. The Fragile X-Associated Tremor Ataxia Syndrome (FXTAS). The Fragile X-Associated Tremor Ataxia Syndrome (FXTAS) 31 53 .
Maureen A. Leehey, Elizabeth Berry-Kravis, Christopher G. Goetz & Randi J. Hagerman. 2010. The Fragile X-Associated Tremor Ataxia Syndrome (FXTAS). The Fragile X-Associated Tremor Ataxia Syndrome (FXTAS) 1 16 .
C. Finke, R. Horváth, E. Holinski-Feder & C.J. Ploner. (2009) Fragiles X-assoziiertes Tremor-/Ataxie-SyndromFragile X-associated tremor/ataxia syndrome. Der Nervenarzt 80:12, pages 1473-1479.
Crossref
Andreea L. Seritan, Andrea Schneider, John M. Olichney, Maureen A. Leehey, R. Scott Akins & Randi J. Hagerman. (2009) Conversion disorder in women with the FMR1 premutation . American Journal of Medical Genetics Part A 149A:11, pages 2501-2506.
Crossref
Carl Feinstein & Lovina Chahal. (2009) Psychiatric Phenotypes Associated with Neurogenetic Disorders. Psychiatric Clinics of North America 32:1, pages 15-37.
Crossref

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Order Reprints Request Corporate Permissions

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

Request Academic Permissions

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.

Related research

People also read lists articles that other readers of this article have read.

Recommended articles lists articles that we recommend and is powered by our AI driven recommendation engine.

Cited by lists all citing articles based on Crossref citations.
Articles with the Crossref icon will open in a new tab.