Fractional crystallization and melting within binary systems with solid solution

Abstract

<h3>Objective</h3> Previous research suggests attention and white matter (WM) abnormalities in individuals with mucopolysaccharidosis type I (MPS I); this cross-sectional comparison is one of the first to examine the relationship of WM structural abnormalities as measured by corpus callosum (CC) volumes with attention scores to evaluate this relationship in a larger sample of patients with MPS I. <h3>Methods</h3> Volumetric MRI data and performance on a computerized measure of sustained attention were compared for 18 participants with the severe form of MPS I (MPS IH), 18 participants with the attenuated form of MPS I (MPS I_ATT), and 60 typically developing age-matched controls. <h3>Results</h3> The MPS I groups showed below-average mean attention scores (<i>p</i> &lt; 0.001) and smaller CC volumes (<i>p</i> &lt; 0.001) than controls. No significant associations were found between attention performance and CC volume for controls. Attention was associated with posterior CC volumes in the participants with MPS IH (<i>p</i> = 0.053) and total (<i>p</i> = 0.007) and anterior (<i>p</i> &lt; 0.001) CC volumes in participants with MPS I_ATT. <h3>Conclusions</h3> We found that attention and CC volumes were reduced in participants with MPS I compared to typically developing controls. Smaller CC volumes in participants with MPS I were associated with decreased attention; such an association was not seen in controls. While hematopoietic cell transplantation used to treat MPS IH may compound these effects, attention difficulties were also seen in the MPS I_ATT group, suggesting that disease effects contribute substantially to the clinical attentional difficulties seen in this population.