ArticleNeuropsychologia2014
ERP abnormalities elicited by word repetition in fragile X-associated tremor/ataxia syndrome (FXTAS) and amnestic MCI.
Article in Neuropsychologia, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 28 citations in OpenAlex.
- Open-Label Allopregnanolone Treatment of Men with Fragile X-Associated Tremor/Ataxia Syndrome.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2017Trial
- Cognitive dysfunction in women with theJournal of Alzheimer's disease : JAD · 2026Article
- Semantic and Syntactic Language Differences Associated with theJournal of neurolinguistics · 2026Article
- Cognitive-behavioral phenotypes inFrontiers in molecular neuroscience · 2026Article
- Language use predicts symptoms of fragile X-associated tremor/ataxia syndrome in men and women with the FMR1 premutation.Scientific reports · 2024Article
- Review
- Vocabulary Repetition Following Multisensory Instruction Is Ineffective on L2 Sentence Comprehension: Evidence From the N400.Frontiers in psychology · 2022Article
- The Role of Glutamate in Language and Language Disorders - Evidence from ERP and Pharmacologic Studies.Neuroscience and biobehavioral reviews · 2020Review
- Dysfunction of Inferior Parietal Lobule During Sensory Gating in Patients With Amnestic Mild Cognitive Impairment.Frontiers in aging neuroscience · 2020Article
- FN400 and LPC Responses to Different Degrees of Sensory Involvement: A Study of Sentence Comprehension.Advances in cognitive psychology · 2020Article
- Auditory repetition suppression alterations in relation to cognitive functioning in fragile X syndrome: a combined EEG and machine learning approach.Journal of neurodevelopmental disorders · 2018Article
- Cognitive Deficits and Associated ERP N400 Abnormalities in FXTAS With Parkinsonism.Frontiers in genetics · 2018Article
- Fragile X-Associated Tremor/Ataxia Syndrome: Unmet Needs and a Path for the Future.Frontiers in genetics · 2018Article
- Curvilinear Association Between Language Disfluency andFrontiers in genetics · 2018Article
- Can a Neurosteroid Ameliorate Fragile X-Associated Tremor/Ataxia Syndrome?Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2017Article
- Risk Factors for Cognitive Impairment in Fragile X-Associated Tremor/Ataxia Syndrome.Journal of geriatric psychiatry and neurology · 2016Article
- Fragile X-associated tremor/ataxia syndrome: phenotypic comparisons with other movement disorders.The Clinical neuropsychologist · 2016Review
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Authors and funding
14 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundFragile X-associated tremor/ataxia syndrome (FXTAS), a neurodegenerative disorder caused by FMR1 gene premutations, typically associated with frontal-subcortical type cognitive impairments. High prevalence (~50%) of superimposed Alzheimer׳s pathology has been reported in FMR1 premutation 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 (M(age)=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; M(age)=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.
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