| The primary cognitive deficit among males with fragile X-associated tremor/ataxia syndrome (FXTAS) is a dysexecutive syndrome. | |
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MedLine Citation:
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PMID: 18608667 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder associated with a premutation trinucleotide repeat expansion in the fragile X mental retardation 1 gene. Symptoms include gait ataxia, action tremor, and cognitive impairment. The objectives of the study were to clarify the nature of the dysexecutive syndrome observed in FXTAS and to assess the contribution of executive impairment to deficits in nonexecutive cognitive functions. Compared to controls, men with FXTAS demonstrated significant executive impairment, which was found to mediate group differences in most other cognitive abilities. Asymptomatic premutation carriers performed similarly to controls on all but two measures of executive functioning. These findings suggest that the impairment of nonexecutive cognitive skills in FXTAS is in large part secondary to executive dysfunction. |
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Authors:
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Angela G Brega; Glenn Goodrich; Rachael E Bennett; David Hessl; Karen Engle; Maureen A Leehey; Lanee S Bounds; Marsha J Paulich; Randi J Hagerman; Paul J Hagerman; Jennifer B Cogswell; Flora Tassone; Ann Reynolds; Robert Kooken; Michael Kenny; Jim Grigsby |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural Date: 2008-02-15 |
Journal Detail:
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Title: Journal of clinical and experimental neuropsychology Volume: 30 ISSN: 1744-411X ISO Abbreviation: J Clin Exp Neuropsychol Publication Date: 2008 Nov |
Date Detail:
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Created Date: 2010-03-12 Completed Date: 2010-06-07 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8502170 Medline TA: J Clin Exp Neuropsychol Country: England |
Other Details:
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Languages: eng Pagination: 853-69 Citation Subset: IM |
Affiliation:
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Department of Medicine, University of Colorado, Denver, Aurora, CO, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Aged Ataxia / complications* Cognition Disorders / etiology* Executive Function / physiology* Fragile X Syndrome / complications* Humans Male Memory / physiology Memory, Short-Term / physiology Mental Processes / physiology Neuropsychological Tests Semantics Verbal Learning / physiology |
| Grant Support | |
ID/Acronym/Agency:
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NS044299/NS/NINDS NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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