Article Figures & Data
Figures
- FIG 1.
DTI TBSS analysis comparing healthy controls with patients with DMD shows areas with significantly (P < .01, family-wise error corrected) decreased FA and increased MD and RD in patients with DMD. Green represents the WM skeleton, while red and maroon are clusters of significance.
- FIG 2.
Healthy control versus DMD (distal) Dp140– TBSS results. RD (A), MD (B), and AD (C) are significantly increased in patients compared with healthy controls in both the cerebellar WM and cerebral WM with relative sparing of the frontal WM (P < .01 family-wise error–corrected). D, FA did not show any significant differences; however, the trend was noted because many areas showed FA differences at the less conservative statistical threshold of P < .05 (family-wise error–corrected). Green represents the WM skeleton, while red and maroon are clusters of significance.
- FIG 3.
(A) Healthy controls versus DMD (proximal) Dp140+: TBSS for FA shows significantly lower FA in the patient group in the areas highlighted in red (P < .01). No significant differences in any of the diffusivity metrics are noted. (B) DMD distal (Dp140–) versus DMD proximal (Dp140+): TBSS shows higher AD in patients with a distal mutation involving the cerebellar WM and right occipitoparietal-temporal WM. The rest of the diffusion metrics did not show any significant difference.
Tables
Patients with DMD (Range) Controls (Range) Age at recruitment (mean) (yr) 8.0 ± 1.2 (6–10) 8.2 ± 1.4 (6–10) Age at onset (mean) (yr) 4.1 ± 1.8 (1–9) Age at presentation (mean) (range) (yr) 7.5 ± 1.4 (4–10) Duration (mean) (mo) 40.8 ± 25.2 (6–120) MDFRS domains (mean) Mobility (maximum 36) 24.35 ± 3.272 (15–30) Basic ADL (maximum 24) 18.77 ± 2.547 (12–25) Arm function (maximum 28) 22.07 ± 1.885 (19–25) Impairment (maximum 44) 34.18 ± 3.568 (23–40) Total score (maximum 132) 99.37 ± 10.55 (69–117) Note:—ADL indicates activities of daily living; MDFRS, Muscular Dystrophy Functional Rating scale.
- Table 2:
Comparison of the intelligent quotient (IQ) values of WISC-III scale between the two patient subgroups (DMD proximal and distal), and healthy controls
SN Test Group (I) Group (J) Difference (I-J) Significance 95% CI, Lower-to-Upper Bounds 1. Verbal IQ Control Proximal 17.258a .000 8.79–25.73 Distal 29.699a .000 22.27–37.12 Proximal Distal 12.441a .002 3.76–21.12 2. Performance IQ Control Proximal 15.350a .000 6.88–23.82 Distal 24.442a .000 17.01–31.87 Proximal Distal 9.092a .037 0.41–17.77 3. Full-scale IQ Control Proximal 18.238a .000 9.50–26.98 Distal 30.141a .000 22.48–37.81 Proximal Distal 11.903a .005 2.95–20.86 4. Verbal Composition Control Proximal 17.608a .000 9.32–25.90 Distal 27.612a .000 20.34–34.88 Proximal Distal 10.005a .015 1.51–18.50 5. Perceptual Organization Index Control Proximal 15.352a .000 7.47–23.23 Distal 23.272a .000 16.37–30.18 Proximal Distal 7.920 .056 –0.15–15.99 6. Freedom from Distractibility Index Control Proximal 8.183 .092 –0.90–17.26 Distal 23.293a .000 15.33–31.26 Proximal Distal 15.110a .000 5.80–24.41 Note:—SN indicates serial number.
↵a level of significance.
