دانلود مقاله ISI انگلیسی شماره 74022
عنوان فارسی مقاله

تغییرات شناختی وابسته به سن در ناقل سندرم ایکس شکننده

کد مقاله سال انتشار مقاله انگلیسی ترجمه فارسی تعداد کلمات
74022 2008 9 صفحه PDF سفارش دهید محاسبه نشده
خرید مقاله
پس از پرداخت، فوراً می توانید مقاله را دانلود فرمایید.
عنوان انگلیسی
Age-dependent cognitive changes in carriers of the fragile X syndrome
منبع

Publisher : Elsevier - Science Direct (الزویر - ساینس دایرکت)

Journal : Cortex, Volume 44, Issue 6, June 2008, Pages 628–636

کلمات کلیدی
سندرم ایکس شکننده؛ آتاکسی شکننده ؛ شناخت؛ توجه
پیش نمایش مقاله
پیش نمایش مقاله تغییرات شناختی وابسته به سن در ناقل سندرم ایکس شکننده

چکیده انگلیسی

Fragile X syndrome is a neurodevelopmental disorder that is caused by the silencing of a single gene on the X chromosome, the fragile X mental retardation 1 (FMR1) gene. Affected individuals display a unique neurocognitive phenotype that includes significant impairment in inhibitory control, selective attention, working memory, and visual–spatial cognition. In contrast, little is known about the trajectory and specificity of any cognitive impairment associated with the fragile X premutation (i.e., “carrier status”) or its relationship with the recently identified neurodegenerative disorder, fragile X-associated tremor/ataxia syndrome (FXTAS). In the present study, we evaluated a broad sample of 40 premutation males (PM) aged 18–69 years matched on age and IQ to 67 unaffected comparison males (NC). Performance was compared across a range of cognitive domains known to be impaired in fragile X syndrome (i.e., “full mutation”). Tremor was also assessed using a self-report neurological questionnaire. PM displayed statistically significant deficits in their ability to inhibit prepotent responses, differentiating them from NC from age 30 onwards. With increasing age, the two groups follow different trajectories, with PM developing progressively more severe problems in inhibitory control. This deficit also has a strong co-occurrence in males displaying FXTAS-related symptomatology (p < .001). Selective attention was also impaired in PM but did not show any disproportionate aging effect. No other cognitive deficits were observed. We conclude that an inhibitory deficit and its impact across the lifespan are specifically associated with the fragile X premutation status, and may be a precursor for development of a more severe form of cognitive impairment or dementia, which has been reported in patients with the diagnosis of FXTAS.

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