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From the Departments of Pediatrics (K.K., I.N., V.K., K.A.D., J.B.E., E.G.S.), Psychiatry (J.R.W., B.A.M., K.O.L.), and Genetics and Metabolism (C.B.W.), University of Minnesota Medical Center; Division of Biostatistics (K.D.R.), University of Minnesota School of Public Health, Minneapolis; Department of Psychology (E.G.M., J.R.), Hospital for Sick Children-Toronto, Ontario, Canada; Department of Human Genetics (N.A., S.C.), Emory University, Atlanta, GA; and Division of Gastroenterology and Nutrition (P.H.), UCSF Benioff Children's Hospital Oakland, San Francisco, CA. Dr. Kovac is now at the School of Medicine, Washington University in St. Louis, MO. Dr. Raiman is now at the Department of Inherited Metabolic Diseases, Birmingham Children's Hospital, UK. K.A. Delaney is now at Biomarin Pharmaceuticals, San Rafael, CA.
Published: May 2019
AI Article Synopsis
Article Abstract
Objective: 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.
Methods: 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), and 60 typically developing age-matched controls.
Results: The MPS I groups showed below-average mean attention scores ( < 0.001) and smaller CC volumes ( < 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 ( = 0.053) and total ( = 0.007) and anterior ( < 0.001) CC volumes in participants with MPS I.
Conclusions: 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 group, suggesting that disease effects contribute substantially to the clinical attentional difficulties seen in this population.
