TABLE 1.
Mean age in years (standard deviation), gender, adaptive behavior skills, Autism Spectrum characteristics, and aggression in children with Angelman syndrome divided by genetic subtype: “deletion” (Class I and II) and “non deletion” (imprinting center defect, UBE3A mutation and uniparental disomy).
| Deletion | Non deletion | F/X2 | df | p | ||
| N | 14 | 7 | ||||
| Age (years) | Mean (SD) | 12.07 (3.73) | 8.14 (3.98) | 4.96 | 1 | 0.04* |
| Range | 4–15 | 5–15 | ||||
| Gender | % Male | 50.0 | 28.6 | 0.88 | 1 | 0.64 |
| Self help scorea | Mean (SD) | 4.14 (0.53) | 6.29 (1.11) | 36.54 | 1 | <0.01** |
| Mobilitya | % Mobile | 79.6 (11) | 100.0 (7) | 1.75 | 1 | 0.52 |
| Visiona | % Normal | 93.9 (13) | 100 (7) | 0.53 | 1 | 1.00 |
| Hearinga | % Normal | 100.0 (14) | 100 (7) | – | – | – |
| Speecha | % Verbal | 0.0 (0) | 0.0 (0) | – | – | – |
| SCQb | Communication | 11.02 (2.79) | 4.41 (4.47) | −3.05 | 1 | 0.002** |
| Social interaction | 8.06 (2.68) | 3.57 (1.40) | −3.08 | 1 | 0.002** | |
| Repetitive behavior | 4.14 (1.79) | 4.00 (1.53) | −0.38 | 1 | 0.74 | |
| Total score | 19.85 (4.29) | 11.43 (4.50) | −3.07 | 1 | 0.001** | |
| Aggression | % Presence (n) | 71.4 (10) | 100 (7) | 2.47 | 1 | 0.26 |
*p < 0.05, **p < 0.01. aInformation obtained from the Wessex Scale (Kushlick et al., 1973). bInformation obtained from the Social Communication Questionnaire (SCQ; Rutter et al., 2003). cInformation obtained from the Challenging Behavior Questionnaire (Hyman et al., 2002).