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Table 2 Performance comparison in clinical variables between ARSACS patients at different disease stages

From: From motor performance to participation: a quantitative descriptive study in adults with autosomal recessive spastic ataxia of Charlevoix-Saguenay

  Total No walking difficulty Walking aid Wheelchair p-value**
n = 28 n = 7 n = 11 n = 10
Upper limb functions (dexterity, coordination and strength)
 PPT (no. of pegs) 5.2 (2.4)a 7.9 (0.90) 5.4 (1.6) 2.8 (1.7) < 0.001
  min-max 0.5–9.5 6.5–9.5 3.0–8.5 0.5–6.5
 NHPT (seconds) 58.2 (37.7) 33.8 (3.5) 45.0 (8.8) 89.6 (49.0) < 0.001
  min-max 28.6–211.2 28.6–37.5 35.1–61.0 35.9–211.2
 SFNT (no. of targets) 11.5 (3.7) 14.4 (4.2) 12.4 (1.7) 8.4 (3.0) 0.002
  min-max 5.5–23.0 10.0–23.0 10.5–15.0 5.5–15.5
 Grip strength (kg) 28.9 (9.8) 30.0 (11.9) 27.8 (10.1) 29.2 (9.0) 0.736
  min-max 11.3–52.3 13.3–44.0 16.7–52.3 11.3–38.3
 Pinch strength (kg) 6.8 (1.9) 7.6 (1.6) 6.1 (1.5) 7.0 (2.3) 0.294
  min-max 3.7–10.5 4.5–9.3 4.1–8.8 3.7–10.5
Lower limb functions (coordination)
 LEMOCOT (no. of targets) 17.0 (10.1) 27.7 (3.6) 19.3 (5.8) 6.9 (7.3) < 0.001
  min-max 0–34.5 22.0–32.5 13.5–34.5 0–21.5
Mobility and balance
 6MWTb (meters) 235.3 (116.3) 355.0 (78.8) 177.4 (53.9) 35.0c 0.002
  min-max 35.0–459.5 256.0–459.5 99.0–270.0 35.0
 10mWTb (speed, m/s) 0.87 (0.44) 1.33 (0.21) 0.71 (0.26) 0.15 (0.02)c 0.001
  min-max 0.14–1.6 1.1–1.6 0.33–1.2 0.14–0.17
 BBS 22.1 (19.1) 47.3 (5.8) 24.0 (10.6) 2.5 (4.2) < 0.001
  min-max 0–56 42–56 11–42 0–13
Disease severity
 SPRS 24.3 (11.2) 11.1 (4.3) 21.8 (4.1) 36.1 (6.9) < 0.001
  min-max 3.0–50.0 3.0–16.0 16.0–28.0 26.0–50.0
 SARA 20.6 (8.9) 10.3 (3.0) 18.0 (2.3) 30.7 (4.5) < 0.001
  min-max 6.0–36.0 6.0–13.5 13.0–22.0 23.0–36.0
Participation and health-related quality of life
 LIFE-H 7.8 (1.2) 9.3 (0.57) 7.7 (0.50) 6.9 (1.4) 0.001
  min-max 4.0–9.7 8.3–9.7 6.9–8.6 4.0–8.9
 Barthel Index 84.3 (22.1) 99.3 (1.9) 94.1 (7.0) 63.0 (24.9) < 0.001
  min-max 25–100 95–100 80–100 25–95
 SF-12v2 (MCS) 54.9 (12.8) 51.8 (13.3) 59.9 (9.4) 51.5 (15.0) 0.141
  min-max 13.1–72.5 22.8–61.5 39.8–72.5 13.1–66.2
 SF-12v2 (PCS) 41.4 (5.8) 41.4 (6.6) 39.4 (5.5) 43.6 (5.3) 0.333
  min-max 27.0–51.5 32.3–49.9 27.0–46.7 38.0–51.5
  1. **Comparison between < 40 years and ≥ 40 years using a Krsukal-Wallis Test; results in bold are significant (p-value < 0.05)
  2. aResults are presented as Mean (standard deviation)
  3. b19 participants were able to performed the 6MWT and 20 participants the 10mWT in the total sample
  4. cOne participant out of 10 have performed the 6MWT and two participants performed the 10mWT in the Wheelchair disease stage