Abstract | AIM: METHODS: SUBJECTS: 7 MPS IH participants at least 5 years post-HCT were compared with 7 attenuated participants who were treated with ERT. MEASURES: IQ, attention, spatial ability, and memory were assessed. Medical history and an unsedated MRI scan using diffusion tensor imaging (DTI) were acquired. RESULTS: Despite clinically equivalent IQ and memory, children with MPS IH had poorer attention span than those with attenuated MPS I as well as decreased fractional anisotropy (FA) of the corpus callosum. A relationship between attention scores and FA was found in the MPS IH group but not the attenuated group. FA was also related to the frequency of medical events. INTERPRETATION: In children with MPS IH, both the treatment and the disease affect attention functions associated with poor white matter integrity.
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Authors | Elsa Shapiro, O Evren Guler, Kyle Rudser, Kathleen Delaney, Kendra Bjoraker, Chester Whitley, Jakub Tolar, Paul Orchard, James Provenzale, Kathleen M Thomas |
Journal | Molecular genetics and metabolism
(Mol Genet Metab)
Vol. 107
Issue 1-2
Pg. 116-21
(Sep 2012)
ISSN: 1096-7206 [Electronic] United States |
PMID | 22867884
(Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
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Copyright | Copyright © 2012 Elsevier Inc. All rights reserved. |
Topics |
- Adolescent
- Adult
- Brain
(pathology, physiopathology)
- Child
- Diffusion Tensor Imaging
- Enzyme Replacement Therapy
- Female
- Hematopoietic Stem Cell Transplantation
- Humans
- Male
- Mucopolysaccharidosis I
(diagnosis, psychology, therapy)
- Treatment Outcome
- Young Adult
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