title: The test of everyday attention reveals significant sustained volitional attention and working memory deficits in friedreich ataxia.
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.
authors.affiliation: Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Monash University, Clayton, Victoria, Australia.
authors.forename: Felicity
authors.initials: F
authors.lastname: Klopper
authors.forename: Martin B
authors.initials: MB
authors.lastname: Delatycki
authors.forename: Louise A
authors.initials: LA
authors.lastname: Corben
authors.forename: John L
authors.initials: JL
authors.lastname: Bradshaw
authors.forename: Gary
authors.initials: G
authors.lastname: Rance
authors.forename: Nellie
authors.initials: N
authors.lastname: Georgiou-Karistianis
mesh_terms: D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D001288:Attention / Q000502:physiology*; D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis / Q000209:etiology*; D016022:Case-Control Studies; D004185:Disability Evaluation; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications*; D006801:Humans; D008297:Male; D008569:Memory Disorders / Q000175:diagnosis / Q000209:etiology*; D008570:Memory, Short-Term / Q000502:physiology*; D009483:Neuropsychological Tests; D018709:Statistics, Nonparametric; D055815:Young Adult