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. 2019 Sep 17;62(1):75–82. doi: 10.1111/dmcn.14355

Table 1.

Patient characteristics

 

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
a

Normal distribution.

b

Phenotypic severity of primary feature (0=normal, 1=mild, 2=moderate, 3=severe).

c

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.

d

Negative age‐corrected SARA scores are expressed as 0 (i.e. optimal value).

e

Age‐corrected Paediatric Balance Scale (PBS) scores >56 are expressed as 56 (i.e. optimum values). IQR, interquartile range.