G.P.127

N Doorenweerd,Eve M Dumas,Eidrees Ghariq,Sophie Schmid, C S M Straathof,Pietro Spitali, H B Ginjaar, B H Wokke, D G M Schrans,J C Van Den Bergen,E W Van Zwet,Andrew G Webb,M A Van Buchem,M J P Van Osch, J J G Verschuuren,Jos G M Hendriksen,E H Niks, H E Kan

NEUROMUSCULAR DISORDERS(2014)

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摘要
Duchenne muscular dystrophy (DMD) is a neuromuscular disorder known to be associated with specific learning and behavioral disabilities. Recently, we found cerebral anatomical alterations in boys with DMD, especially in those predicted to lack the Dp140 isoform of the dystrophin protein (DMD_Dp140-). To investigate whether these changes are accompanied by changes in cerebral blood supply, we now report on an arterial spin labeling (ASL) MRI study of the same boys, in whom brain perfusion was measured non-invasively. T1-weighted and pseudo continuous ASL scans were obtained on a 3T MR scanner from 27 boys with DMD and 20 age-matched healthy control boys (ages 8–18 years). The mean whole brain grey matter (GM) perfusion was quantified per individual, and voxelwise group analysis was performed to locate regions with different cerebral blood flow (CBF) using cluster-based multiple comparison correction. Group comparisons were made between DMD and controls using a t -test, and between DMD_Dp140+ and DMD_Dp140− and controls using ANOVA. DMD boys had significantly lower mean whole brain GM CBF (41.7 ± 7.8 versus 49.9 ± 8.7 mL/100 g/min in controls ( p
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