Cognitive deficits in Machado‐Josephs disease

Abstract

Cognitive function was examined in 6 patients with genetically confirmed Machado-Joseph disease (MJD) and 15 age- and ethnically matched controls using a series of subtests from the Cambridge Neuropsychological Test Automated Battery (CANTAB), a touch screen-based testing system previously validated in a number of movement and neurodegenerative disorders. The MJD patients had deficits in visual attentional function that were characterized by a slowing of the processing of visual information when task demands were high and an inability to shift attention to previously irrelevant stimulus dimensions to discriminate between complex stimuli. Tests of learning and visual memory were normal. These results demonstrate that specific cognitive deficits occur in patients with MJD, independent of motor dysfunction, and these deficits may reflect disruption to frontosubcortical pathways.

Neurological and Cognitive Recovery Following Abstinence from Petrol Sniffing

“…Raw data that were positively skewed were normalized using logarithmic base 10 (log) transformation and negatively skewed data were normalized using arcsine transformations (eg, Maruff et al, 1996Maruff et al, , 1998. Individual data for age, blood lead level, saccade, and cognitive indices were compared with a 2 (time: baseline, follow-up) Â 3 (group: controls, nonencephalopathic sniffers, encephalopathic sniffers) analyses of variance (ANO-VA).…”

“…The cognitive test battery was drawn from the touchscreen based Cambridge Automated Neuropsychological Test Battery (CAN-TAB) that included tests of basic movement reaction time (simple reaction time task), visual recognition memory (pattern recognition task), visual attention (visual search task), and visuo-spatial learning/memory (pattern-location paired associative learning task). Methods for the use of these tests in indigenous groups have been described in detail previously (Maruff et al, 1996(Maruff et al, , 1998Cairney et al, 2003).…”

Saccade and Cognitive Function in Chronic Kava Users

“…Accuracy measures on the CANTAB tasks that were scored as percentage correct, formed negatively skewed distributions and arcsine transformations were used to normalize these distributions. Similar transformations have been used in previous studies using CANTAB measures (Owen et al, , 1995Maruff et al, 1996Maruff et al, , 1998. Where variables did not meet the assumptions for ANOVA after transformation or where data was categorical, scores were submitted to Kruskal-Wallis nonparametric ANOVA to compare groups.…”

“…The neuropsychometric test battery was drawn from the touchscreen-based CANTAB. All of these tests have been described in detail previously (Owen et al, , 1995Maruff et al, 1996Maruff et al, , 1998. Briefly, for the motor function task, individuals were required to use their dominant hand to touch the middle of a cross that was presented with random timing and at random locations on the computer screen.…”

“…From this CANTAB battery, we include tests of basic motor function (motor screening), visual recognition memory (pattern recognition), visual attention (visual search) and visuo-spatial learning/memory (pattern-location paired associate learning). Owing to their simplicity, these saccade and cognitive tests have been consistently validated in healthy and brain-impaired adults and children, and have consequently proven sensitive to a wide spectrum of performance levels, subtle disruptions of attentional and memory systems, and to the effects of both sedating and stimulant drugs (Tedeschi et al, 1983a, b;Hotson et al, 1986;Sahakian et al, 1988;Currie et al, 1992Currie et al, , 1993Owen et al, 1993Owen et al, , 1995Maruff et al, 1996Maruff et al, , 1998Elliot et al, 1997;Robbins et al, 1997;Cairney et al, 2001). Since these tests have been used in neuroimaging studies and during singlecell recordings in primates, performance measures can be interpreted within robust brain-behavior models.…”