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Abstract #0914

Metabolic and microstructural MPSII brain alteration revealed by multiparametric MR imaging and spectroscopy – a combined 3T and 7T study

Alena Svatkova1, Lenka Minarikova2, Petr Bednarik2, Verena Rosenmayr3, Gilbert Hangel2, Bernhard Strasser4, Lukas Hingerl2, Thomas Stulnig3, and Stephan Gruber2
1Department of Medicine III, Medical University of Vienna, Vienna, Austria, 2High Field MR Centre, Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria, 3Clinical Division for Endocrinology and Metabolism, Department of Medicine III, Medical University of Vienna, Vienna, Austria, 4Athinoula A. Martinos Center for Biomedical Imaging, Boston, MA, United States

While glycosaminoglycan deposition in Mucopolysaccharidosis type II, a rare X-linked lysosomal storage disorder, unquestionably alters the brain, metabolic and microstructural MR markers have not been yet established. Thus, we utilized 3T diffusion MRI and fine-tuned semi-LASER MR spectroscopy as well as in-house developed 7T 3D-FID-MRS imaging to examine differences between seven MPSII and eight age-matched healthy males. Analyses revealed profound deficit in the supratentorial white matter consistent with de/dysmyelination on both diffusion and spectroscopy as well as decrease of neuronal population or hypometabolism measured as glutamate deficit in the posterior cingulate cortex, which is a critical hub of neurocognitive networks.

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