Decreased functional brain activation in Friedreich ataxia using the Simon effect task

The present study applied the Simon effect task to examine the pattern of functional brain reorganization in individuals with Friedreich ataxia (FRDA), using functional magnetic resonance imaging (fMRI). Thirteen individuals with FRDA and 14 age and sex matched controls participated, and were required to respond to either congruent or incongruent arrow stimuli, presented either to the left or right of a screen, via laterally-located button press responses. Although the Simon effect (incongruent minus congruent stimuli) showed common regions of activation in both groups, including the superior and middle prefrontal cortices, insulae, superior and inferior parietal lobules (LPs, LPi), occipital cortex and cerebellum, there was reduced functional activation across a range of brain regions (cortical, subcortical and cerebellar) in individuals with FRDA. The greater Simon effect behaviourally in individuals with FRDA, compared with controls, together with concomitant reductions in functional brain activation and reduced functional connectivity between cortical and sub-cortical regions, implies a likely disruption of cortico-cerebellar loops and ineffective engagement of cognitive/attention regions required for response suppression.

► We report the first attentional fMRI study in individuals with FRDA.
► The Simon effects task elicits reduced functional brain activity in FRDA.
► Reduced functional connectivity in individuals with FRDA.
► Results suggest ineffective engagement of attention brain regions in FRDA.
► Results suggest disruption of cortico-ponto-cerebello-thalamo-cortical loops in FRDA.