Louise Corben - Experimental Neuropsychology Research Unit

Friedreich ataxia (FRDA), a multisystem autosomal-recessive disease, is the most common of the genetically inherited ataxias, affecting approximately 1 in 29,000 individuals.The limited literature available on the cognitive components of FRDA implicates a slowing of motor and reaction time suggestive of reduced information processing speed in the setting of intact executive function. However, the exact nature of these impairments remains poorly understood.

This study aims to establish the cognitive behavioural profile particularly in the context of information processing, of people with FRDA. It also aims to correlate such a profile with the clinical and genetic indicators of FRDA. This project will utilise well established behavioural tasks designed to address which brain regions are involved in the pathophysiology of FRDA. In conjunction with a parallel project, it will also utilise behavioural measures from tasks completed while undergoing functional magnetic resonance imaging (fMRI). This will enable correlation of the structural and functional changes in brain regions that are hypothesized to be implicated in the pathophysiology of FRDA. It is envisaged that this project will facilitate a comprehensive understanding of the cognitive impairment associated with FRDA and the factors that may impact on such impairment.