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