Table 5. Association between cognitive profile and prevalence of intellectual disability, ADHD, and ASD with mutation type in each dystrophin disruption groups.
Dp427 (N = 10) | Dp140 (N = 37) | Dp71 (N = 4) | ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
Deletion (N = 9) | Duplication (N = 1) | Point Mutation (N = 0) | p value | Deletion (N = 26) | Duplication (N = 7) | Point Mutation (N = 4) | p value | Deletion (N = 1) | Duplication (N = 3) | Point Mutation (N = 0) | p value | |
WISC | ||||||||||||
Total IQ Score | 81.1 ± 25.6 | 98.00 | N/A | 0.553 | 82.3 ± 17.7 | 86.3 ± 19.0 | 93.7 ± 13.6 | 0.547 | 37.0 | 49.0 ± 29.5 | N/A | 0.758 |
Verbal | 83.4 ± 29.5 | 104.00 | N/A | 0.531 | 84.3 ± 17.1 | 91.8 ± 8.5 | 97.3 ± 22.0 | 0.425 | 35.0 | 54.7 ± 30.4 | N/A | 0.632 |
Performance | 81.4 ± 19.5 | 92.00 | N/A | 0.624 | 75.7 ± 17.0 | 81.0 ± 5.2 | 90.0 ± 3.6 | 0.314 | 35.0 | 48.7 ± 29.2 | N/A | 0.724 |
VABS | ||||||||||||
ABC | 36.6 ± 9.8 | 76.00 | N/A | 0.022* | 45.5 ± 15.5 | 56.0 ± 2.8 | 53.5 ± 24.4 | 0.584 | 33.0 | 43.0 ± 26.9 | N/A | 0.778 |
Communication | 43.4 ± 10.0 | 84.00 | N/A | 0.021* | 52.5 ± 18.5 | 64.5 ± 14.9 | 57.5 ± 25.7 | 0.702 | 37.0 | 43.0 ± 24.0 | N/A | 0.849 |
Daily Living | 58.2 ± 23.0 | 70.00 | N/A | 0.664 | 42.3 ± 23.2 | 48.5 ± 14.9 | 57.0 ± 38.3 | 0.623 | 20.0 | 42.3 ± 33.7 | N/A | 0.624 |
Socialization | 61.8 ± 13.4 | 86.00 | N/A | 0.176 | 59.5 ± 21.3 | 61.0 ± 1.4 | 58.0 ±26.2 | 0.986 | 51.0 | 53.7 ± 34.0 | N/A | 0.952 |
Prevalence of intellectual disability, ADHD, and ASD | ||||||||||||
Intellectual disability | 3 (33.3%) | 0 | 0 | 1.000 | 5 (19.2%) | 1 (14.3%) | 1 (25%) | 0.892 | 1 (100%) | 3 (100%) | 0 | - |
ADHD | 1 (11.1%) | 0 | 0 | 1.000 | 2 (7.7%) | 1 (14.3%) | 0 | 0.705 | 0 | 0 | 0 | - |
ASD | 0 | 0 | 0 | - | 0 | 0 | 0 | - | 0 | 0 | 0 | - |
Note: significance set as p<0.05; ABC, Adaptive Behavior Composite; WISC, Wechsler Intelligence Scale for Children; VABS, Vineland Adaptive Behavior Scales; ADHD, attention deficit disorder; ASD, autism spectrum disorder.