The test of everyday attention reveals significant sustained volitional attention and working memory deficits in friedreich ataxia

J Int Neuropsychol Soc. 2011 Jan;17(1):196-200. doi: 10.1017/S1355617710001347. Epub 2010 Nov 17.

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.

MeSH terms

  • Activities of Daily Living*
  • Adolescent
  • Adult
  • Attention / physiology*
  • Attention Deficit Disorder with Hyperactivity / diagnosis
  • Attention Deficit Disorder with Hyperactivity / etiology*
  • Case-Control Studies
  • Disability Evaluation
  • Female
  • Friedreich Ataxia / complications*
  • Humans
  • Male
  • Memory Disorders / diagnosis
  • Memory Disorders / etiology*
  • Memory, Short-Term / physiology*
  • Neuropsychological Tests
  • Statistics, Nonparametric
  • Young Adult