Attention and corpus callosum volumes in individuals with mucopolysaccharidosis type i

Kelly E. King, Kyle D. Rudser, Igor Nestrasil, Victor Kovac, Kathleen A. Delaney, Jeffrey R. Wozniak, Bryon A. Mueller, Kelvin O. Lim, Julie B. Eisengart, Eva G. Mamak, Julian Raiman, Nadia Ali, Stephanie Cagle, Paul Harmatz, Chester B. Whitley, Elsa G. Shapiro

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


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 ATT), and 60 typically developing age-matched controls.

RESULTS: The MPS I groups showed below-average mean attention scores ( p < 0.001) and smaller CC volumes ( p < 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 ( p = 0.053) and total ( p = 0.007) and anterior ( p < 0.001) CC volumes in participants with MPS I ATT.

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 ATT group, suggesting that disease effects contribute substantially to the clinical attentional difficulties seen in this population.

Original languageEnglish (US)
Pages (from-to)E2321-E2328
Issue number20
StatePublished - May 14 2019

Bibliographical note

Publisher Copyright:
© American Academy of Neurology.


  • Adolescent
  • Attention/physiology
  • Case-Control Studies
  • Child
  • Corpus Callosum/diagnostic imaging
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Mucopolysaccharidosis I/diagnostic imaging
  • Organ Size
  • White Matter/diagnostic imaging

PubMed: MeSH publication types

  • Research Support, Non-U.S. Gov't
  • Journal Article
  • Research Support, N.I.H., Extramural


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