14800-DvRappard

113 Quantitative MR spectroscopic imaging in metachromatic leukodystrophy 7 Table 2 . Demographics and quantitative MR results of control subjects and MLD patients at baseline controls good outcome moderate outcome poor outcome n - male/female age / y median range 16 10.6 4.2 - 8m/8f 29.6 5 17.8 6.5 - 2m/3f 28.4 3 32.5 26.9 - 3f 35.3 8 7.9 7.1 - 1m/7f 22.5 Cr / mM a 3.77 ± 0.38 4.80 ± 0.52 b 4.61 ± 0.58 3.12 ± 0.82 c NAA / mM a 6.99 ± 0.65 6.53 ± 0.98 5.56 ± 0.20 b 1.99 ± 0.73 b,c,d Cho / mM a 1.49 ± 0.21 2.11 ± 0.23 b 1.84 ± 0.24 1.67 ± 0.41 Ins / mM a 2.95 ± 0.46 6.85 ± 1.41 b 7.25 ± 1.25 7.05 ± 1.88 b Glu / mM a 4.68 ± 0.50 4.80 ± 0.80 4.46 ± 0.75 1.68 ± 0.55 b,c,d Glx / mM a 6.88 ± 0.59 8.43 ± 0.38 b 6.50 ± 0.89 4.82 ± 1.28 b,c Lac / mM a 0.33 ± 0.08 0.59 ± 0.14 0.80 ± 0.40 1.57 ± 0.66 b,c MRI score a 9.4 ± 5.4 14.7 ± 3.2 20.1 ± 3.0 c lesion volume / mL a 32.5 ± 20.7 43.9 ± 11.8 111.7 ± 25.1 c,d Values are given as mean with standard deviation, unless indicated otherwise Actual metabolite concentrations in overall WM (NAWM + lesions) are shown, but statistical comparisons were performed on age-corrected values (see methods). a: group-wise GLM, p<0.001; b,c,d: post-hoc Dunnett’s T3, p<0.05, b) compared to controls, c) compared to good outcome, d) compared to moderate outcome. Figure 2: Mean metabolite concentrations in overall WM (NAWM+lesions) of control subjects (black, n=16) and MLD patients at baseline, categorized per clinical outcome: good outcome (green, n=5), moderate outcome (orange, n=3), poor outcome (red, n=8). Error bars indicate standard deviations. Based on age-corrected values, all metabolites showed significant overall group differences (p<0.001). See Table 2 for differences between groups with post-hoc Dunnett’s T3 analysis.

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