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The Test of Everyday Attention Reveals Significant Sustained Volitional Attention and Working Memory Deficits in Friedreich Ataxia

Published online by Cambridge University Press:  17 November 2010

Felicity Klopper ,
Martin B Delatycki ,
Louise A Corben ,
John L Bradshaw ,
Gary Rance  and
Nellie Georgiou-Karistianis
Felicity Klopper
Affiliation:
Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Monash University, Clayton, Victoria, Australia
Martin B Delatycki
Affiliation:
Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, Victoria, Australia Department of Clinical Genetics, Austin Health, Heidelberg, Victoria, Australia Department of Medicine, University of Melbourne, Austin Health, Heidelberg, Victoria, Australia
Louise A Corben
Affiliation:
Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Monash University, Clayton, Victoria, Australia Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, Victoria, Australia
John L Bradshaw
Affiliation:
Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Monash University, Clayton, Victoria, Australia
Gary Rance
Affiliation:
Department of Otolaryngology, University of Melbourne, Parkville, Victoria, Australia
Nellie Georgiou-Karistianis*
Affiliation:
Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Monash University, Clayton, Victoria, Australia
*
Correspondence and reprint requests to: Nellie Georgiou-Karistianis, Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton Victoria, 3800, Australia. E-mail: nellie.georgiou-karistianis@med.monash.edu.au
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Abstract

Sustained volitional attention and working memory capacity was examined for the first time in people with Friedreich ataxia (FRDA). We administered subtests of the Test of Everyday Attention to 16 individuals with molecularly confirmed FRDA and gender-, age-, and IQ-matched controls. Clinically significant impairment in working memory and sustained volitional attention was evident. Working memory deficits correlated significantly with GAA repeat number on the shorter allele of the FXN gene, and separately, with disease severity, as measured by the Friedreich Ataxia Rating Scale score. Sustained volitional attention was not correlated with disease parameters, suggesting that this impairment may not be related to the disease process in a simple way. The deficits observed may be the result of disruption to corticocerebellar pathways, or directly related to the cortical and/or cerebellar pathology evident in people with FRDA. (JINS, 2011, 17, 000–000)

Keywords

Friedreich ataxiaAttentionWorking memoryMemoryShort-termCognitionVolition
Type
Brief Communication
Information
Journal of the International Neuropsychological Society , Volume 17 , Issue 1 , January 2011 , pp. 196 - 200
Copyright
Copyright © The International Neuropsychological Society 2010

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