DSC MR Perfusion Microvascular Abnormalities Can Predict Lesion Progression in Cerebral X-Linked Adrenoleukodystrophy (P6.122)

Introduction: X-linked Adrenoleukodystrophy (ALD) is a neurodegenerative disorder that results from mutations in the ABCD1 gene. About 60[percnt] of male carriers develop a cerebral rapid progressive inflammatory demyelination and death within few years. Effective treatments are available but carry high toxicity. Onset and disease course of cerebral ALD are unpredictable. We set out to assess changes in microvascular perfusion of white matter using dynamic susceptibility contrast magnetic resonance imaging (DSC-MRI). u2028Methods: Twenty-four patients with cerebral adrenoleukodystrophy were evaluated between 2006 and 2015. Microvascular flow patterns, determined as capillary transit time distribution (CTH), were estimated from raw DSC-MRI perfusion data. Regions of interest (ROIs) were drawn in co-registered maps (A: non-enhancing T2-hyperintense core, B: T1-post contrast enhancing rim, and C: T2- hyperintense perilesional WM, D: adjacent normal appearing white matter and distant normal appearing white matter: dNAWM). Available longitudinal data was analyzed regarding areas converting to T2-hyperintensity on follow-up. u2028Results: We found distinct abnormalities in microvascular flow pattern in perilesional white matter (Zone D) while normal appearing as determined on structural imaging (n=34; ANOVA between group differences p=0.0002, post hoc analysis with Dunnett’s correction: dNAWM vs. A, p Disclosure: Dr. Lauer has nothing to disclose. Dr. Cai has nothing to disclose. Dr. Da has nothing to disclose. Dr. Aziz-Bose has nothing to disclose. Dr. Ou has nothing to disclose. Dr. Kalpathy-Cramer has nothing to disclose. Dr. Bo Hansen has nothing to disclose. Dr. Mouridsen has nothing to disclose. Dr. Rosen has nothing to disclose. Dr. Eichler has received research support from Bluebird Bio and Vertex Pharmaceuticals. Dr. Musolino has nothing to disclose.