A cross-sectional analysis of orienting of visuospatial attention in child and adult carriers of the fragile X premutation

Ling M. Wong, Naomi J. Goodrich-Hunsaker, Yingratana A. McLennan, Flora Tassone, Susan M. Rivera, Tony J Simon

Research output: Contribution to journalArticle

Abstract

Background: Fragile X premutation carriers (fXPCs) have an expansion of 55-200 CGG repeats in the FMR1 gene. Male fXPCs are at risk for developing a neurodegenerative motor disorder (fragile X-associated tremor/ataxia syndrome (FXTAS)) often accompanied by cognitive decline. Several broad domains are implicated as core systems of dysfunction in fXPCs, including perceptual processing of spatial information, orienting of attention to space, and inhibiting attention to irrelevant distractors. We tested whether orienting of spatial attention is impaired in fXPCs. Methods: Participants were fXPCs or healthy controls (HCs) asymptomatic for FXTAS. In experiment 1, they were male and female children and adults (aged 7-45 years). They oriented attention in response to volitional (endogenous) and reflexive (exogenous) cues. In experiment 2, the participants were men (aged 18-48 years). They oriented attention in an endogenous cueing task that manipulated the amount of information in the cue. Results: In women, fXPCs exhibited slower reaction times than HCs in both the endogenous and exogenous conditions. In men, fXPCs exhibited slower reaction times than HCs in the exogenous condition and in the challenging endogenous cueing task with probabilistic cues. In children, fXPCs did not differ from HCs. Conclusions: Because adult fXPCs were slower even when controlling for psychomotor speed, results support the interpretation that a core dysfunction in fXPCs is the allocation of spatial attention, while perceptual processing and attention orienting are intact. These findings indicate the importance of considering age and sex when interpreting and generalizing studies of fXPCs.

Original languageEnglish (US)
Article number45
JournalJournal of Neurodevelopmental Disorders
Volume6
Issue number1
DOIs
StatePublished - Dec 11 2014

Fingerprint

Cross-Sectional Studies
Cues
Reaction Time
Automatic Data Processing
Neurodegenerative Diseases
Genes
Fragile X Tremor Ataxia Syndrome

Keywords

  • Cueing
  • Endogenous
  • Exogenous
  • FMR1 gene
  • Fragile X
  • FXTAS

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Cognitive Neuroscience
  • Pediatrics, Perinatology, and Child Health

Cite this

A cross-sectional analysis of orienting of visuospatial attention in child and adult carriers of the fragile X premutation. / Wong, Ling M.; Goodrich-Hunsaker, Naomi J.; McLennan, Yingratana A.; Tassone, Flora; Rivera, Susan M.; Simon, Tony J.

In: Journal of Neurodevelopmental Disorders, Vol. 6, No. 1, 45, 11.12.2014.

Research output: Contribution to journalArticle

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abstract = "Background: Fragile X premutation carriers (fXPCs) have an expansion of 55-200 CGG repeats in the FMR1 gene. Male fXPCs are at risk for developing a neurodegenerative motor disorder (fragile X-associated tremor/ataxia syndrome (FXTAS)) often accompanied by cognitive decline. Several broad domains are implicated as core systems of dysfunction in fXPCs, including perceptual processing of spatial information, orienting of attention to space, and inhibiting attention to irrelevant distractors. We tested whether orienting of spatial attention is impaired in fXPCs. Methods: Participants were fXPCs or healthy controls (HCs) asymptomatic for FXTAS. In experiment 1, they were male and female children and adults (aged 7-45 years). They oriented attention in response to volitional (endogenous) and reflexive (exogenous) cues. In experiment 2, the participants were men (aged 18-48 years). They oriented attention in an endogenous cueing task that manipulated the amount of information in the cue. Results: In women, fXPCs exhibited slower reaction times than HCs in both the endogenous and exogenous conditions. In men, fXPCs exhibited slower reaction times than HCs in the exogenous condition and in the challenging endogenous cueing task with probabilistic cues. In children, fXPCs did not differ from HCs. Conclusions: Because adult fXPCs were slower even when controlling for psychomotor speed, results support the interpretation that a core dysfunction in fXPCs is the allocation of spatial attention, while perceptual processing and attention orienting are intact. These findings indicate the importance of considering age and sex when interpreting and generalizing studies of fXPCs.",
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