Table 3 Behaviour-related domain scores (2–9 age group)
From: An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III
Measure/domain | MPS III | ID | p value |
|---|---|---|---|
| Â | Median ( N , range) | Median ( N , range) | Â |
ECBI | Â | Â | Â |
 Intensity score | 128 (8, 63–180) | 115 (9, 57–154) | 0.336 |
 Problem score | 16 (7, 0–27) | 11 (9, 1–20) | 0.761 |
ABC | Â | Â | Â |
 Irritability | 8 (7, 3–31) | 11.5 (10, 1–22) | 0.494 |
 Lethargy | 10 (7, 0–29) | 9.5 (10, 1–29) | 0.732 |
 Stereotypy | 4 (7, 0–12) | 0.5 (10, 0–14) | 0.577 |
 Hyperactivity | 27 (7, 10–38) | 11 (10, 5–28) | 0.031 |
 Inappropriate speech | 3 (7, 0–9) | 0 (10, 0–5) | 0.08 |
 ABC total score | 58 (7, 13–113) | 34 (10, 10–91) | 0.525 |
SBRS | Â | Â | Â |
 Current understanding | 28 (9, 11–35) | 24.5 (10, 5–40) | 0.902 |
 Past understanding | 30 (7, 24–41) | 12.5 (4, 0–35) | 0.130 |
 Current expression | 8 (9, 1–12) | 6.5 (10, 4–14) | 0.967 |
 Past expression | 6 (6, 2–17) | 3 (5, 0–6) | 0.125 |
 Orality | 29 (9, 11–33) | 12.5 (10, 0–24) | 0.005 |
 Body movements | 22 (9, 5–27) | 6.5 (10, 0–18) | 0.013 |
 Interactions with objects | 14 (9, 2–20) | 7 (10, 0–12) | 0.022 |
 Activity and routines | 22 (9, 11–36) | 15.5 (10, 8–26) | 0.078 |
 Emotional function | 5 (9, 0–16) | 4 (10, 0–12) | 0.536 |
 Safety consciousness | 14 (9, 8–18) | 10.5 (10, 6–18) | 0.267 |
 Fearfulness | 28 (9, 16–38) | 28 (10, 9–40) | 0.806 |
 Social interaction | 16 (9, 8–24) | 20 (10, 12–26) | 0.388 |
 Eye contact | 8 (9, 2–18) | 5 (10, 0–10) | 0.201 |
 Emotional engagement | 7 (9, 0–13) | 11.5 (10, 1–16) | 0.234 |
 Comfort seeking | 9 (9, 6–23) | 5.5 (10, 0–24) | 0.078 |
 Attention | 14 (9, 10–18) | 8.5 (10, 5–18) | 0.040 |
 Self-control/compliance | 11 (9, 0–18) | 9.5 (10, 2–15) | 0.461 |
 Mood, anger and aggression | 11 (9, 5–33) | 10 (10, 4–24) | 0.582 |
 Self-gratification | 0 (9, 0–7) | 0.5 (10, 0–11) | 0.649 |