Table 1.
Total n=32 |
EOA n=11 |
DCD n=10 |
Central hypotonia n=11 |
p | |
---|---|---|---|---|---|
Age, y:moa | |||||
Mean (SD) | 9:10 (3:3) | 11:4 (3:6) | 8:8 (2:4) | 9:4 (3:6) | 0.170 |
Range | 4:0–17:0 | 6:0–17:0 | 6:0–13:0 | 4:0–14:0 | |
Disease onset, y:mo | |||||
Median (IQR) | 1:6 (0:6–3:9) | 2:0 (0:6–4:0) | 1:6 (0:6–3:3) | 1:6 (0:6–3:0) | 0.914 |
Range | 0:0–8:0 | 0:0–8:0 | 0:0–5:0 | 0:0–5:0 | |
Disease duration, y:moa | |||||
Mean (SD) | 7:11 (3:1) | 8:11 (3:11) | 7:4 (2:5) | 7:7 (2:11) | 0.449 |
Range | 2:6–15:0 | 3:0–15:0 | 4:0–11:0 | 2:6–12:6 | |
Phenotypic severityb | |||||
Median (IQR) | 1 (1–2) | 2 (1–2) | 1 (1–2) | 1 (0–1) | 0.001c |
Range | 0–3 | 1–3 | 1–2 | 0–1 | |
SARATOTAL | |||||
Median (IQR) | 3.0 (0.0–8.0) | 9.5 (8.0–14.0) | 2.8 (0.4–3.6) | 0.0 (0.0–2.3)d | <0.001c |
Range | 0.0–15.5d | 4.5–15.5 | 0.0–8.0d | 0.0–3.0d | |
SARAGAIT | |||||
Median (IQR) | 2.0 (0.0–3.9) | 5.5 (3.5–7) | 1.3 (0.0–2.5)d | 0.0 (0.0–0.5)d | <0.001c |
Range | 0.0–8.0d | 2.0–8.0 | 0.0–2.5d | 0.0–2.0d | |
SARAFINGER‐NOSE/FINGER‐CHASE | |||||
Median (IQR) | 0.75 (0.0–2.0) | 2.0 (1.0–2.5) | 0.3 (0.0–1.0) | 0.0 (0.0–0.5) | <0.001c |
Range | 0.0–3.5 | 0.5–3.5 | 0.0–2.0 | 0.0–1.25 | |
SARAKINETIC | |||||
Median (IQR) | 1.1 (0.0–3.4) | 4.5 (2.5–6.0) | 0.8 (0.0–2.1)d | 0.0 (0.0–1.0)d | <0.001c |
Range | 0.0–6.5d | 0.5–6.5 | 0.0–4.5d | 0.0–2.0d | |
SARASPEECH | |||||
Median (IQR) | 0.0 (0.0–2.0) | 2.0 (2.0–3.0) | 0.0 (0.0–0.0) | 0.0 (0.0–0.0) | <0.001c |
Range | 0.0–3.5 | 0.0–3.5 | 0.0–1.0 | 0.0–0.0 | |
PBS | |||||
Median (IQR) | 54.1 (50.5–55.9) | 48.9 (43.3–53.5) | 54.3 (53.0–55.4) | 56.0 (55.9–56.0)e | 0.001c |
Range | 23.9–56.0e | 23.9–56.0e | 44.0–55.9 | 51.3–56.0e |
Normal distribution.
Phenotypic severity of primary feature (0=normal, 1=mild, 2=moderate, 3=severe).
Significantly different. Although most p‐values reached the level of significance, early‐onset ataxia (EOA) and developmental coordination disorder revealed a quantitative and phenotypic overlap. From this perspective, one cannot clinically use absolute Scale for Assessment and Rating of Ataxia (SARA) scores for group assignment.
Negative age‐corrected SARA scores are expressed as 0 (i.e. optimal value).
Age‐corrected Paediatric Balance Scale (PBS) scores >56 are expressed as 56 (i.e. optimum values). IQR, interquartile range.