Table 1.
Clinical characteristics of DM1 and DM2 patients and controls at baseline (T1) and follow-up (T2).
Table 2.
Neuropsychological performance controlled for motor function and age effects in DM1 and DM2 patients and healthy controls, test results at baseline (T1) and follow-up (T2).
Fig 1.
Differences in changes of neuropsychological test performances between groups over time.
(A, B, C) Comparison of DM1 patients and controls over time. (A) Reaction time: indicating a decline in DM1 patients compared to controls (p = 0.047, difference of means = 14.49, 95% confidence interval (CI): -21.53 to 50.51). (B) Choice reaction time test: indicating a decline in selective attention in DM1 patients over time compared to controls (p = 0.039, difference of means = -16.03, 95% CI:-54.90 to 22.84). (C) Block design: indicating a decline in DM1 patients over time compared to controls (p = 0.048, difference of means = -7.07, 95% CI: -15.23 to 1.09). (D) Comparison of DM2 patients and controls over time, Block design: indicating a decline in DM2 patients over time compared to controls (p = 0.003, difference of means = -0.30, 95% CI: -5.31 to 4.71). The bimanual Pegboard task and age at follow-up were used as covariates to correct for motor impairment and age effects. Block design = higher numbers indicating better results Choice reaction time test, Reaction time = lower numbers indicating better results Estimated marginal means describe mean values of the respective neuropsychological test (reaction time, choice reaction time in selective attention test, score in block design test) that are adjusted for other variables (age, motor performance).
Table 3.
Sleep and fatigue scales in DM1 and DM2 patients compared with healthy controls at baseline and follow-up.
Table 4.
White matter hyperintensities rated according to the ARWMC scale in DM1 and DM2 patients and control subjects at baseline (T1) and follow-up (T2).
Fig 2.
Voxel-based morphometry, group comparisons at baseline and follow-up.
Displayed results of VBM analyses are based on a threshold of pfalse discovery rate < 0.05 at voxel-level with an extended cluster threshold of 10 voxels. The coordinates refer to the MNI reference space. VBM analyses were performed in 13 DM1, 15 DM2 patients, and 13 controls. (A) Gray matter decrease in DM1 patients compared with controls at baseline (light red) and at follow-up (light and dark red). Dark red areas had not been affected at baseline. (B) Gray matter decrease in DM2 patients compared with controls at baseline and at follow-up (no clusters detected). (C) White matter decrease in DM1 patients compared with controls at baseline (light blue) and at follow-up (light and dark blue). (D) White matter decrease in DM2 patients compared with controls at baseline (light blue) and at follow-up (light and dark blue). Dark blue areas had not been affected at baseline.
Fig 3.
Diffusion tensor imaging group comparison at baseline and follow-up. Displayed results of tract-based spatial statistics analyses of different diffusivity indices (FA, AD, RD, MD) are based on a corrected threshold of pfamily wise error < 0.05 and overlaid on the MNI152 Template (resolution 1x1x1 mm) included in FSL. The coordinates refer to the MNI reference space. DTI analyses were performed in 15 DM1 patients and 14 controls. (A) FA reduction in DM1 patients compared with controls at baseline and (B) follow-up. (C) Increase in AD in DM1 patients compared with controls at baseline and (D) follow-up. (E) Increase in RD in DM1 patients compared with controls at baseline and (F) follow-up. (G) Increase of MD in DM1 patients compared with controls at baseline and (H) follow-up.
Fig 4.
Diffusion tensor imaging group comparison at baseline and follow-up. Displayed results of tract-based spatial statistics analyses of different diffusivity indices (FA, AD, RD, MD) are based on a corrected threshold of pfamily wise error < 0.05 and overlaid on the MNI152 Template (resolution 1x1x1 mm) included in FSL. The coordinates refer to the MNI reference space. DTI analyses were performed in 16 DM2 patients and 14 controls. (A) FA reduction in DM2 patients compared with controls at baseline and (B) follow-up. (C) No increase in AD in DM2 patients compared with controls at baseline (no significant voxels detected) and (D) follow-up (no significant voxels detected). (E) No increase in RD in DM2 patients compared with controls at baseline (no significant voxels detected), but at (F) follow-up. (G) No increase of MD in DM2 patients compared with controls at baseline (no significant voxels detected) and (H) follow-up (no significant voxels detected).