Hostname: page-component-586b7cd67f-vdxz6 Total loading time: 0 Render date: 2024-11-22T15:33:06.190Z Has data issue: false hasContentIssue false
  • English
  • Français

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: [email protected]
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Baillieux, H., Jung De Smet, H., Dobbeleir, A., Paquier, P.F., De Deyn, P.P., Marien, P. (2009). Cognitive and affective disturbances following focal cerebellar damage in adults: A neuropsychological and SPECT study. Cortex, 46(7), 869–879. doi: 0.1016/j.cortex.2009.09.002CrossRefGoogle ScholarPubMed
Cohen, J. (1992). A power primer. Psychological Bulletin, 112(1), 155–159.CrossRefGoogle ScholarPubMed
Delatycki, M.B., Williamson, R., Forrest, S.M. (2000). Friedreich ataxia: An overview. J. Med. Genet., 37(1), 1–8. doi: 10.1136/jmg.37.1.1CrossRefGoogle ScholarPubMed
Junck, L., Gilman, S., Gebarski, S.S., Koeppe, R.A., Kluin, K.J., Markel, D.S. (1994). Structural and functional brain imaging in Friedreich’s ataxia. Arch Neurol, 51(4), 349–355.CrossRefGoogle ScholarPubMed
Lodi, R., Tonon, C., Calabrese, V., Schapira, A.H.V. (2006). Friedreich’s ataxia: from disease mechanisms to therapeutic interventions. Antioxidants & Redox Signalling, 8(3–4), 438–443.CrossRefGoogle ScholarPubMed
Mantovan, M.C., Martinuzzi, A., Squarzanti, F., Bolla, A., Silvestri, I., Liessi, G., Angelini, C. (2006). Exploring mental status in Friedreich’s ataxia: a combined neuropsychological, behavioural and neuroimaging study. European Journal of Neurology, 13, 827–835. doi: 10.1111/j.1468-1331.2006.01363.xCrossRefGoogle ScholarPubMed
Nelson, H.E., O’Connell, A. (1978). Dementia: The estimation of pre-morbid intelligence levels using the new adult reading test. Cortex, 14, 234–244.CrossRefGoogle Scholar
Ortuno, F., Ojeda, N., Arbizu, J., Lopez, P., Marti-Climent, J.M., Penuelas, I., Cervera, S. (2002). Sustained attention in a counting task: Normal performance and functional neuroanatomy. NeuroImage, 17, 411–420. doi: 10.1006/nimg.2002.1168CrossRefGoogle Scholar
Pandolfo, M. (2008). Friedreich ataxia. Archives of Neurology, 65(10), 1296–1303. doi: http://dx.doi.org/10.1001/archneur.65.10.1296CrossRefGoogle ScholarPubMed
Phillips, J.G., Schiffter, T., Nicholls, M.E.R., Bradshaw, J.L., Iansek, R., Saling, L.L. (1999). Does old age Parkinson’s disease cause bradyphrenia? J Gerontol A Biol Sci Med Sci, 54(8), M404–409.CrossRefGoogle ScholarPubMed
Rance, G., Fava, R., Baldock, H., Chong, A., Barker, E., Corben, L., Delatycki, M.B. (2008). Speech perception ability in individuals with Friedreich ataxia. Brain: A Journal of Neurology, 131(8), 2002–2012. doi: http://dx.doi.org/10.1093/brain/awn104CrossRefGoogle ScholarPubMed
Robertson, I.H., Ward, T., Ridgeway, V., Nimmo-Smith, I. (1994). The Test of Everyday Attention. Bury St Edmonds, England: Thames Valley Test Company.Google Scholar
Schmahmann, J.D., Sherman, J.C. (1998). The cerebellar cognitive affective syndrome. Brain, 121, 561–579.CrossRefGoogle ScholarPubMed
Subramony, S.H., May, W., Lynch, D., Gomex, C., Fischbeck, K., Hallett, M., Ashizawa, T. (2005). Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale. Neurology, 64, 1261–1262.CrossRefGoogle ScholarPubMed
Wang, H., Fan, J. (2007). Human attentional networks: A connectionist model. Journal of Cognitive Neuroscience, 19(10), 1678–1689.CrossRefGoogle ScholarPubMed
White, M., Lalonde, R., Botez-Marquard, T. (2000). Neuropsychologic and neuropsychiatric characteristics of patients with Friedreich’s ataxia. Acta Neurol Scand, 102(4), 222–226. doi: 10.1034/j.1600-0404.2000.102004222.xCrossRefGoogle ScholarPubMed
Wollmann, T., Barroso, J., Monton, F.I., Nieto, A. (2002). Neuropsychological test performance of patients with Friedriech’s ataxia. Journal of Clinical and Experimental Neuropsychology, 24(5), 677–686.CrossRefGoogle Scholar