Working memory deficits in Individuals with Friedreich Ataxia: The IMAGE-FRDA study
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1
Monash University, School of Psychology and Psychiatry, Australia
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2
Monash University, Monash Biomedical Imaging, Australia
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3
Monash University (Alfred Hospital Campus), Department of Medicine (Neurosciences), Australia
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4
Murdoch Children’s Research Institute, Bruce Lefroy Centre for Genetic Health Research, Australia
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5
University of Melbourne (Austin Hospital campus), Department of Clinical Genetics, Department of Medicine, Australia
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6
Monash University, School of Psychological Sciences, Australia
Background
Friedreich ataxia (FRDA) is the commonest of all the inherited ataxias, causing progressive degeneration of nerve tissue in the spinal cord, peripheral sensory nerves and cerebellum. Although motor dysfunction is the hallmark of this disease, some degree of cognitive impairment is only now becoming accepted as part of the gross symptomatology. In this investigation, we sought to characterise working memory deficits in individuals with FRDA.
Methods
Both individuals with FRDA (n=18), and age and gender matched controls (n=22), completed an N-BACK working memory task consisting of 0-BACK and 2-BACK conditions. Under the 0-BACK reference condition, participants were required to respond to a single pre-specified target letter. Under the 2-BACK working memory condition, participants were required to respond to any letter that repeated itself two trials previously. Reaction time and accuracy scores were collected for both the 0-BACK and 2-BACK conditions.
Results
Individuals with FRDA performed less accurately over both N-BACK conditions, and performed significantly less accurately than control participants with increasing memory load (i.e., 2-BACK). Further, individuals with FRDA displayed increased reaction times for both N-BACK conditions compared with control participants.
Conclusions
These findings provide evidence for cognitive impairment in the gross symptomatology of FRDA. In particular, the working memory deficits suggest evidence of functional brain reorganisation in individuals with FRDA, perhaps as part of a disruption of cortico-cerebellar pathways involving cognitive/working memory brain networks.
Keywords:
Cognition,
Friedreich Ataxia,
executive functions,
working memory,
n-back task
Conference:
XII International Conference on Cognitive Neuroscience (ICON-XII), Brisbane, Queensland, Australia, 27 Jul - 31 Jul, 2014.
Presentation Type:
Poster
Topic:
Cognition and Executive Processes
Citation:
Georgiou-Karistianis
N,
Stagnitti
M,
Egan
G,
Storey
E,
Delatycki
M and
Corben
L
(2015). Working memory deficits in Individuals with Friedreich Ataxia: The IMAGE-FRDA study.
Conference Abstract:
XII International Conference on Cognitive Neuroscience (ICON-XII).
doi: 10.3389/conf.fnhum.2015.217.00191
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Received:
19 Feb 2015;
Published Online:
24 Apr 2015.
*
Correspondence:
Prof. Nellie Georgiou-Karistianis, Monash University, School of Psychology and Psychiatry, Melbourne, Australia, nellie.georgiou-karistianis@monash.edu