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Abstract #2877

Exploring Myelin Imaging Biomarkers in Pelizaeus-Merzbacher Disease

Caroline Koehler1, Paul Kuntke1, Prativa Sahoo2, Hannes Wahl1, Sean C Deoni3, Hagen H. Kitzler1, and Steffi Dreha-Kulaczewski2
1Institut for Diagnostic and Interventional Neuroradiology, University Hospital Carl Gustav Carus, Dresden, Germany, 2Pediatrics and Adolescent Medicine, University Medicine Göttingen, Goettingen, Germany, 3Advanced Baby Imaging Lab, Memorial Hospital of Rhode Island, Pawtucket, RI, United States


Advanced MRI techniques bear great potential to explore myelination in hypomyelinating leukodystrophies such as Pelizaeus-Merzbacher disease (PMD) in-vivo. Quantitative imaging biomarkers which reflect myelin, its spatial distribution, and dynamics during brain maturation are not readily available i.e. to monitor natural disease courses. In a pilot study including three PMD patients, multi-component relaxation which enables the evaluation of relative myelination by estimating the myelin water fraction (MWF) was obtained from 56 region of interest (ROI). Determination of ROIs was facilitated by coregistration of the patients data to a pediatric atlas and compared to age matched healthy controls.

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