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Abstract #2522

Multimodal quantitative MRI for the assessment of different disease processes in a childhood hypomyelinating leukodystrophy

Prativa Sahoo1, Caroline Köhler2, Irini Gkalimani1, Paul Kunte2, Peter Dechent3, Gunter Helms4, Sean Deoni5, Hagen H Kitzler2, and Steffi Dreha Kulaczewski1
1Department of Pediatrics and Adolescent Medicine, University Medical Center Göttingen, Göttingen, Germany, 2Department of Diagnostic and Interventional Neuroradiology, University Hospital Carl Gustav Carus Dresden, Dresden, Germany, 3Department of Cognitive Neurology, University Medical Center Göttingen, Göttingen, Germany, 4Lund University, Lund, Sweden, 5Bill and Medlinda Gates Foundation, Seattle, WA, United States

Synopsis

Keywords: Neuro, White Matter, DTI , MTIX-linked Pelizaeus Merzbacher disease is characterized by failure of myelin formation and subsequent axonal damage. Severity of disease courses varies depending on the underlying gene mutation. Eight male patients were recruited for the study. MR-imaging protocol included MTI, MWI for evaluation of myelin related parameters and DTI for assessment of axonal integrity. Decreased values in quantitative MTI and MWI parameter maps and ROI analyses indicated severe myelin deficit in all patients. However, FA reflected various degrees of axonal involvement among patients with deferent genotypes. Multimodal MRI can facilitate assessment of simultaneous disease processes in childhood hypomyelinating leukodystrophies.

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Keywords