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

被引：57

作者：

Brega, Angela G. ^{[1
]}

Goodrich, Glenn ^{[1
]}

Bennett, Rachael E. ^{[1
]}

Hessl, David ^{[2
,3
]}

Engle, Karen ^{[1
]}

Leehey, Maureen A. ^{[4
]}

Bounds, Lanee S. ^{[1
]}

Paulich, Marsha J. ^{[1
]}

Hagerman, Randi J. ^{[2
,5
]}

Hagerman, Paul J. ^{[6
]}

Cogswell, Jennifer B. ^{[2
]}

Tassone, Flora ^{[6
]}

Reynolds, Ann ^{[7
]}

Kooken, Robert ^{[1
]}

Kenny, Michael ^{[8
]}

Grigsby, Jim ^{[1
]}

机构：

[1] Univ Colorado, Dept Med, Aurora, CO 80045 USA

[2] Univ Calif Davis, Med Ctr, MIND Inst, Sacramento, CA 95817 USA

[3] Univ Calif Davis, Med Ctr, Dept Psychiat & Behav Sci, Sacramento, CA 95817 USA

[4] Univ Colorado, Dept Neurol, Denver, CO 80202 USA

[5] Univ Calif Davis, Med Ctr, Dept Pediat, Sacramento, CA 95817 USA

[6] Univ Calif Davis, Sch Med, Dept Biochem & Mol Med, Davis, CA 95616 USA

[7] Childrens Hosp, Denver, CO 80218 USA

[8] Univ Colorado, CU Aging Ctr, Colorado Springs, CO 80907 USA

来源：

JOURNAL OF CLINICAL AND EXPERIMENTAL NEUROPSYCHOLOGY | 2008年 / 30卷 / 08期

关键词：

Fragile X; Premutation; Cognition disorders; Trinucleotide repeats; Executive cognitive function;

D O I：

10.1080/13803390701819044

中图分类号：

B849 [应用心理学];

学科分类号：

040203 ;

摘要：

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.

引用

页码：853 / 869

页数：17

共 102 条

[1] Volumetric brain changes in females with fragile X-associated tremor/ataxia syndrome (FXTAS) [J].