ERP abnormalities elicited by word repetition in fragile X-associated tremor/ataxia syndrome (FXTAS) and amnestic MCI.
ERP abnormalities elicited by word repetition in fragile X-associated tremor/ataxia syndrome (FXTAS) and amnestic MCI.
复制标题
脆性 X 相关震颤/共济失调综合征 (FXTAS) 和遗忘性 MCI 中单词重复引起的 ERP 异常。
DOI：
10.1016/j.neuropsychologia.2014.08.001
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发表时间：
2014
期刊：
影响因子：
2.6
通讯作者：
Olichney,JohnM
中科院分区：
文献类型：
--
作者：
Yang,Jin-Chen;Chi,Lillian;Teichholtz,Sara;Schneider,Andrea;Nanakul,Rawi;Nowacki,Ralph;Seritan,Andreea;Reed,Bruce;DeCarli,Charles;Iragui,VicenteJ;Kutas,Marta;Hagerman,PaulJ;Hagerman,RandiJ;Olichney,JohnM
BackgroundFragile X-associated tremor/ataxia syndrome (FXTAS), a neurodegenerative disorder caused byFMR1gene premutations, typically associated with frontal-subcortical type cognitive impairments. High prevalence (~50%) of superimposed Alzheimer׳s pathology has been reported inFMR1premutation carriers, and standardized neuropsychological tests have not yielded any robust discriminators between FXTAS and Alzheimer׳s disease (AD) dementia. The similarities/differences in memory processes between FXTAS and early AD remain underexplored.Methods32-channel event-related potentials (ERPs) were obtained from a semantic judgment task in which semantically congruous (50%) and incongruous pairs repeat pseudorandomly. The N400 and late positive component (LPC) of 25 FXTAS patients (Mage=71.2, MMSE=26.6) were compared to a matched group of 25 patients with MCI or early AD (1 mild AD dementia, 24 amnestic MCI, of whom 18 later converted to AD;Mage=73.4, MMSE=26.4), and 25 healthy elderly.ResultsBoth patient groups showed similar reductions in the N400 repetition effect and N400 congruity effect amplitudes, compared to controls, reflecting abnormal semantic priming and repetition priming. The MCI/AD group, however, had significantly smaller LPC word repetition effects and poorer learning and memory on the CVLT than FXTAS. The LPC and N400 repetition effects both correlated with verbal memory across all subjects, but only N400 correlated with memory in FXTAS.ConclusionFXTAS patients show relative sparing of the LPC repetition effect, and less disruption of explicit memory than prodromal/early AD. N400 abnormalities in FXTAS appear to account for much of their mild impairments in verbal learning and memory.
