Abnormal semantic processing in females with fragile X-associated tremor/ataxia syndrome.

Abnormal semantic processing in females with fragile X-associated tremor/ataxia syndrome.
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DOI:
10.1111/gbb.12114
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发表时间:
2014-02
期刊:
Genes, brain, and behavior
影响因子:
--
通讯作者:
Olichney JM
Olichney JM
中科院分区:
其他
文献类型:
--
作者:
Yang JC;Simon C;Schneider A;Seritan AL;Hamilton L;Hagerman PJ;Hagerman RJ;Olichney JM

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FXTAS是一种神经退行性疾病,影响晚年脆性X(FMR 1)基因前突变携带者。研究表明,FXTAS的认知障碍包括执行功能障碍、工作记忆和视觉空间缺陷。然而,对FXTAS女性的认知知之甚少。因此,我们研究了语义处理和言语记忆的女性FXTAS患者与事件相关电位(ERP)和神经心理测试。61名女性(34名FXTAS法师= 62.7,27名对照组法师= 60.4)被试在类别判断任务中进行了32通道ERP研究。将N400和P600振幅数据提交至ANCOVA。神经心理学测试显示,女性FXTAS患者的语言学习和执行功能表现较低。ERP分析显示,FXTAS组的N400一致性效应(不一致-一致)显著降低。女性FXTAS组N400一致性效应的降低主要是由于对一致性新词的N400波幅增加。没有发现N400重复效应或P600重复效应的显著异常,表明FXTAS女性分别保留了内隐记忆和言语记忆。N400一致性效应的降低表明女性FXTAS患者的语义期望异常和/或语义网络紊乱。增强的N400振幅一致的新词可能反映了FXTAS女性认知灵活性的下降,使得获得不太典型的类别范例词更加困难。
FXTAS, a neurodegenerative disorder, affects Fragile X (FMR1) gene premutation carriers in late-life. Studies have shown cognitive impairments in FXTAS including executive dysfunction, working memory, and visuospatial deficits. However, less is known about cognition in females with FXTAS. Thus, we examined semantic processing and verbal memory in female FXTAS patients with event-related potentials (ERPs) and neuropsychological testing. 61 females (34 FXTAS Mage = 62.7, 27 controls Mage = 60.4) were studied with 32-channel ERPs during a category judgment task in which semantically congruous (50%) and incongruous items were repeated ~10–140s later. N400 and P600 amplitude data were submitted to ANCOVA. Neuropsychological testing demonstrated lower performance in verbal learning and executive function in females with FXTAS. ERP analyses revealed a significant reduction of the N400 congruity effect (incongruous–congruous) in the FXTAS group. The N400 congruity effect reduction in females with FXTAS was mainly due to increased N400 amplitude to congruous new words. No significant abnormalities of the N400 repetition effect or the P600 repetition effect were found, indicating preserved implicit memory and verbal memory, respectively, in females with FXTAS. The decreased N400 congruity effect suggests abnormal semantic expectancy and/or semantic network disorganization in female FXTAS patients. The enhanced N400 amplitude to congruous new words may reflect decreased cognitive flexibility among FXTAS women, making access to less typical category exemplar words more difficult.
DOI: 10.1007/978-3-642-21649-7_14
发表时间: 2012-01-01
期刊: MODELING FRAGILE X SYNDROME
影响因子: --
作者:
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