Volumetric MRI data correlate to disease severity in metachromatic leukodystrophy.

ABSTRACT:

Objective

Metachromatic leukodystrophy (MLD) is an inherited lysosomal disorder due to a deficiency in arylsulfatase A with progressive demyelination and neurological decline. This retrospective MRI study investigated the extent of cortical involvement at time of diagnosis, and clinical correlates to both conventional and regional volumetric measures of brain involvement.

Methods

3D-T1-weighted MRI scans were used to determine cortical thickness and surface-based cerebral cortical gray matter (GM) and cerebral white matter (WM) volume (GMV and WMV), WM lesions, thalamus, and cerebellum. MRI-MLD severity scores were obtained from FLAIR images. Associations between clinical and imaging data were examined using correlation coefficients.

Results

Twenty patients with MLD (

SUBMITTER: Tillema JM 

PROVIDER: S-EPMC4574810 | biostudies-literature | 2015 Sep

REPOSITORIES: biostudies-literature