Younger sitters (n = 107) | Older sitters (n = 73) | Lost sitters (n = 37) | Lost walkers (n = 39) | |
---|---|---|---|---|
Age at symptom onset (months) | 10.5 ± 5.9 | 11.3 ± 5.1 | 10.0 ± 4.4 | 23.8 ± 16.1 |
Age at start of treatment (months) | 30.8 ± 13.2 | 120.9 ± 39.1 | 98.5 ± 61.6 | 134.7 ± 54.6 |
SMN2 copy number | ||||
1 SMN2 | 0 (0%) | 1 (1.4%) | 0 (0%) | 0 (0%) |
2 SMN2 | 11 (10.3%) | 5 (6.8%) | 2 (5.4%) | 0 (0%) |
3 SMN2 | 78 (72.9%) | 37 (50.7%) | 23 (62.2%) | 26 (66.7%) |
≥ 4 SMN2 | 12 (11.2) | 11 (15.1%) | 3 (8.1%) | 6 (15.4%) |
Unknown | 6 (5.6%) | 19 (26.0%) | 9 (24.3%) | 7 (17.9%) |
HFMSE score (n) | 20.7 ± 11.4 (52) | 15.7 ± 12.4 (47) | 13.7 ± 14.4 (15) | 25.9 ± 9.1 (33) |
RULM score (n) | 16.2 ± 7.1 (26) | 19.0 ± 7.5 (51) | 12.8 ± 7.1 (18) | 26.0 ± 6.1 (29) |
Sitting without support | 100% | 100% | 0% | 89.7% |
Non-invasive ventilator support | 12 (11.2%) | 16 (21.9%) | 9 (24.3%) | 2 (5.1%) |
Tube feeding | 4 (3.7%) | 5 (6.8%) | 5 (13.5%) | 0 (0%) |
Scoliosis | 30 (28.0%) | 62 (85.0%) | 26 (70.3%) | 29 (74.4%) |
Contractures | 1 (0.9%) | 13 (17.8%) | 9 (24.3%) | 7 (17.9%) |