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Abstract #2299

Myelin-Water-Imaging in Hypomyelinating Leukodystrophies

Steffi F. Dreha-Kulaczewski1,2, Hagen H. Kitzler3, Hannes Wahl3, Peter Dechent4, Jutta Gärtner5, Sean Deoni6,7, and Robert Steinfeld5

1Department of Pediatric and Adolescent Medicine, Department of Pediatric and Adolescent Medicine, University Medical Center Goettingen, Goettingen, Germany, 2Department of Neuroradiology, University Medical Center Goettingen, Goettingen, Germany, 3Department of Neuroadiology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany, 4Department of Cognitive Neurology, University Medical Center Goettingen, Goettingen, Germany, 5Department of Pediatrics and Adolescent Medicine, University Medical Center Goettingen, Goettingen, Germany, 6Advanced Baby Imaging Lab, Brown University School of Engineering, Providence, Providence, RI, United States, 7Department of Radiology, University of Colorado Denver, Denver, CO, United States

Emerging clinical research in hypomyelinating leukodystrophies (HLD) necessitates definition of surrogate markers as endpoints in possible clinical trials. Standardized assessment of myelination using myelin sensitive MRI is therefore highly desirable. Proposed protocols comprise DTI, MT-imaging and myelin water imaging employing the mcDESPOT sequence. We report first experiences applying mcDEPSOT in patients with cerebral folate deficiency. Myelin water fraction (MWF) allowed assessment of subtle gradual and regional changes with sufficient spatial resolution. The extent of the myelin deficit in diffuse hypomyelination can more reliably be evaluated using the MWF parameter. McDESPOT seems a feasible, system independent method to study pediatric HLD.

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