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Measuring Inhibition and Cognitive Flexibility in Friedreich Ataxia.

Louise A Corben1,2,3, Felicity Klopper4, Monique Stagnitti4

  • 1Experimental Neuropsychology Research Unit, School of Psychological Sciences, Monash University, Clayton, Victoria, Australia. louise.corben@vcgs.org.au.

Cerebellum (London, England)
|February 24, 2017
PubMed
Summary

Friedreich ataxia (FRDA) impairs cognitive flexibility and inhibitory control, specifically the ability to predict and suppress responses. These deficits were observed on the Hayling Sentence Completion Test but not other executive function tests.

Keywords:
Ataxia, FriedreichCerebellumCognitive flexibilityInhibition (psychology)Verbal response suppression

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Area of Science:

  • Neuroscience
  • Genetics
  • Cognitive Psychology

Background:

  • Friedreich ataxia (FRDA) is a rare autosomal recessive neurodegenerative disorder.
  • FRDA typically affects motor function but can have subtle cognitive impacts.

Purpose of the Study:

  • To investigate inhibitory processes and cognitive flexibility in individuals with FRDA.
  • To assess the ability to suppress predictable verbal responses in FRDA patients.

Main Methods:

  • Administered the Hayling Sentence Completion Test (HSCT), Trail Making Test, and Stroop Test.
  • Compared performance of 43 individuals with FRDA against 42 age- and gender-matched controls.

Main Results:

  • No significant group differences were found on the Stroop or Trail Making Test.
  • Individuals with FRDA showed significant impairment in cognitive flexibility and response inhibition on the HSCT.
  • These deficits did not correlate with clinical FRDA characteristics.

Conclusions:

  • FRDA specifically impacts inhibition and predictive capacity, suggesting cerebellar dysfunction and disrupted cortico-thalamo-cerebellar connectivity.
  • Executive functions beyond inhibition and prediction remained intact, indicating a specific cognitive deficit profile in FRDA.