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RATIONALE AND OBJECTIVES Metachromatic leukodystrophy is a lysosomal storage disorder leading to progressive demyelination of brain white matter. This is sensitively detected using magnetic resonance imaging. The volume of demyelination, the "demyelination load," could serve as a useful parameter for assessing both the natural course of the disease and(More)
OBJECTIVE Metachromatic leukodystrophy (MLD) is a rare metabolic disorder leading to demyelination and rapid neurologic deterioration. As therapeutic options evolve, it seems essential to understand and quantify progression of the natural disease. The aim of this study was to assess cerebral volumetric changes in children with MLD in comparison to normal(More)
Lesion-behaviour mapping analyses require the demarcation of the brain lesion on each (usually transverse) slice of the individual stroke patient's brain image. To date, this is generally thought to be most precise when done manually, which is, however, both time-consuming and potentially observer-dependent. Fully automated lesion demarcation methods have(More)
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