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Table 1.

Demography and clinical status of the study population.

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Table 1 Expand

Table 2.

Clinical details of the Myotonic Dystrophy (DM) patients.

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Table 2 Expand

Table 3.

Numbers of abnormal neuropsychological test results in DM1 and DM2.

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Table 3 Expand

Table 4.

Volumetry results within the DM 1 and DM 2 groups and in the healthy controls.

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Table 4 Expand

Fig 1.

Local atrophy of GM (yellow) and WM (blue) in 12 DM1 patients compared to 28 healthy controls.

Voxelwise multiple regression analysis with group and age as covariates. Age was used as a covariate of no interest. Areas with adjusted p at cluster level < 0.05 after FWE correction are shown; X,Y,Z: MNI-coordinates: negative X-values reflect left side and positive X-values right sided location.

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Fig 1 Expand

Fig 2.

Local atrophy of GM (yellow) and WM (blue) in 15 DM2 patients compared to 28 healthy controls.

Voxelwise multiple regression analysis with group and age as covariates. Age was used as a covariate of no interest. Areas with adjusted p at cluster level < 0.05 after FWE correction are shown; X,Y,Z: MNI-coordinates: negative X-values reflect left side and positive X-values right sided location.

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Fig 2 Expand

Fig 3.

Depression and brainstem volume.

a) Negative correlations between white matter and the depression grade in 12 DM1 (green) and 15 DM2 (red) patients: voxelwise multiple regression with the depression score (BDI-II) as covariate; areas with uncorrected p<0.001 at peak level are shown; X,Y,Z: MNI-coordinates: negative X-values reflect left side and positive X-values right sided location. b) Brainstem volume of DM1 and DM2 patients in dependency of the depression grade: no depression: score < = 11; mild depression: score 12–19; significant depression: score > 20; significance of group differences by Mann-Whitney-U tests.

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Fig 3 Expand

Fig 4.

ESS and brain grey and white matter in DM2.

a) Significant negative correlations between GM and WM and Epworth sleepiness score in 15 DM2 patients detected by voxelwise multiple regression analysis. Age was used as a covariate of no interest. Clusters with FWE corrected cluster p-values < 0.07 are depicted; MNI coordinates: negative X-values reflect left side and positive X-values right sided location. b) Scatterplot of global brain GM and WM in dependency on ESS; the lines and annotations reflect the results of the linear regression analysis.

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Fig 4 Expand

Fig 5.

Flexibility of thinking and brain GM.

Areas of significant negative correlations between GM volume and flexibility of thinking (RWT semantic verbal fluency with change of categories) in 12 DM1 patients (green) and 15 DM2 patients (red). Voxelwise multiple regression analysis with the test outcome (pathologic / normal) and age as covariates. Age was used as a covariate of no interest. Areas with adjusted p at cluster level < 0.05 after FWE correction are shown; X,Y,Z: MNI-coordinates: negative X-values reflect left side and positive X-values right sided location.

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Fig 5 Expand