ERP abnormalities elicited by word repetition in fragile X-associated tremor/ataxia syndrome (FXTAS) and amnestic MCI

Jin Chen Yang, Lillian Chi, Sara Teichholtz, Andrea Schneider, Rawi Nanakul, Ralph Nowacki, Andreea Seritan, Bruce R Reed, Charles DeCarli, Vicente J. Iragui, Marta Kutas, Paul J Hagerman, Randi J Hagerman, John M Olichney

Research output: Contribution to journalArticlepeer-review

13 Scopus citations


Fragile 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. Methods: 32-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. Results: Both 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. Conclusion: FXTAS 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.

Original languageEnglish (US)
Pages (from-to)34-42
Number of pages9
Issue number1
StatePublished - 2014


  • Dementia
  • EEG
  • Fragile X permutation
  • Language
  • Memory

ASJC Scopus subject areas

  • Behavioral Neuroscience
  • Cognitive Neuroscience
  • Experimental and Cognitive Psychology
  • Arts and Humanities (miscellaneous)


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