Please use this identifier to cite or link to this item: http://ahro.austin.org.au/austinjspui/handle/1/22295
Title: Longitudinal Increases in Cerebral Brain Activation During Working Memory Performance in Friedreich Ataxia: 24-Month Data from IMAGE-FRDA.
Authors: Shishegar, Rosita;Harding, Ian H;Corben, Louise A;Delatycki, Martin B;Storey, Elsdon;Egan, Gary F;Georgiou-Karistianis, Nellie
Affiliation: Department of Medicine, Monash University, Melbourne, Australia
Department of Paediatrics, The University of Melbourne, Melbourne, Australia
Clinical Genetics, Austin Health, Heidelberg, Victoria, Australia
School of Psychological Sciences and the Turner Institute for Brain and Mental Health, Faculty of Medicine, Nursing and Health Sciences, Monash University, Melbourne, Australia
Monash Biomedical Imaging, Monash University, Melbourne, Australia
The Australian e-Health Research Centre, CSIRO, Melbourne, Australia
Bruce Lefroy Centre for Genetic Health Research, Murdoch Children's Research Institute, Melbourne, Australia
Issue Date: 2-Jan-2020
EDate: 2020-01-02
Citation: Cerebellum (London, England) 2020; online first: 2 January
Abstract: Friedreich ataxia (FRDA) has been associated with functional abnormalities in cerebral and cerebellar networks, particularly in the ventral attention network. However, how functional alterations change with disease progression remains largely unknown. Longitudinal changes in brain activation, associated with working memory performance (N-back task), and grey matter volume were assessed over 24 months in 21 individuals with FRDA and 28 healthy controls using functional and structural magnetic resonance imaging, respectively. Participants also completed a neurocognitive battery assessing working memory (digit span), executive function (Stroop, Haylings), and set-shifting (Trail Making Test). Individuals with FRDA displayed significantly increased brain activation over 24 months in ventral attention brain regions, including bilateral insula and inferior frontal gyrus (pars triangularis and pars opercularis), compared with controls, but there was no difference in working memory (N-back) performance between groups. Moreover, there were no significant differences in grey matter volume changes between groups. Significant correlations between brain activations and both clinical severity and age at disease onset were observed in FRDA individuals only at 24 months. There was significant longitudinal decline in Trail Making Test (TMT) difference score (B-A) in individuals with FRDA, compared with controls. These findings provide the first evidence of increased longitudinal activation over time in the cerebral cortex in FRDA, compared with controls, despite comparable working memory performance. This finding represents a possible compensatory response in the ventral attention network to help sustain working memory performance in individuals with FRDA.
URI: http://ahro.austin.org.au/austinjspui/handle/1/22295
DOI: 10.1007/s12311-019-01094-6
PubMed URL: 31898277
Type: Journal Article
Subjects: Compensation
Friedreich ataxia
Functional activation
Longitudinal
Working memory
fMRI
Appears in Collections:Journal articles

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