Background
Methods
Participants
The repeated nine-hole peg test (r9HPT)
Statistical analyses
Results
Patients
SMA | SMA type 2 | SMA type 3a | SMA type 3b/4 | Healthy controls | Disease controls | ||
---|---|---|---|---|---|---|---|
(N = 52) | (N = 23) | (N = 11) | (N = 18) | (N = 17) | (N = 29) | ||
Gender, male | 17 (33%) | 5 (22%) | 3 (27%) | 9 (50%) | 3 (18%) | 23 (79%) | |
Age, y, median (range)a | 28 (7–72) | 18 (6–70) | 29 (9–52) | 41 (14–72) | 33 (6–73) | 13 (8–76) | |
Age symptom onset, m, median (range) | 18 (4–456) | 10 (4–30) | 18 (8–24) | 138 (42–456) | NA | 21 (10–672) | |
Disease duration, y, median (range)a | 22 (1–69) | 17 (5–69) | 29 (7–51) | 24 (1–66) | NA | 8 (1–20) | |
Ambulant, N (%) | 16 (31%) | 0 | 3 (27%) | 13 (72%) | 17 (100%) | 23 (79%) | |
SMN2 copy number |
3
| 30 | 21 | 7 | 2 | – | – |
4
| 20 | 1 | 3 | 16 | – | – | |
U
| 2 | 1 | 1 | – | – | – | |
No. of r9HPT completed |
1×
| 9 | 5 | 2 | 2 | 1 | 3 |
2×
| 43 | 18 | 9 | 16 | 16 | 26 | |
Time between 1st and 2nd r9HPT, d, median (range) | 97 (0–696) | 158 (0–696) | 69 (0–249) | 69 (0–407) | 0 (0–253) | 254 (0–469) | |
Mean speed, s/round, (range)a | 45.6 (20–156) | 22.1 (17–41) | 18.8 (18–35) | 16.8 (14–26) | 29.2 (18–113) |
Repeated nine-hole peg test
Gender | Age (y) | Disease | Disease duration (y) | ambulation | Slopea |
---|---|---|---|---|---|
Male | 16.92 | Duchenne | 15.42 | non-ambulant | 34.5 |
Female | 30.25 | SMA type 2 | 29.50 | non-ambulant | 21.8 |
Male | 23.75 | SMA type 2 | 22.83 | non-ambulant | 21.6 |
Male | 7.68 | SMA type 2 | 7.39 | non-ambulant | 9.9 |
Female | 7.69 | SMA type 2 | 7.19 | non-ambulant | 6.0 |
Male | 13.49 | SMA type 2 | 12.66 | non-ambulant | 5.0 |
Female | 70.28 | SMA type 2 | 69.37 | non-ambulant | 3.2 |
Female | 21.40 | SMA type 2 | 21.06 | non-ambulant | 2.2 |
Male | 9.54 | SMA type 2 | 8.74 | non-ambulant | 1.6 |
Male | 48.59 | SMA type 3b/4 | 44.09 | non-ambulant | 1.5 |
Female | 25.94 | SMA type 2 | 25.52 | non-ambulant | 1.4 |
Female | 38.77 | SMA type 3a | 37.61 | non-ambulant | 1.4 |
Female | 24.41 | SMA type 2 | 22.91 | non-ambulant | 1.2 |
Female | 5.69 | SMA type 2 | 4.61 | non-ambulant | 1.1 |
Male | 75.70 | PMA | 19.70 | ambulant | 0.8 |
Male | 9.56 | Becker myotonia | 8.14 | ambulant | 0.7 |
Female | 37.98 | SMA type 2 | 37.44 | non-ambulant | 0.6 |
Female | 9.95 | SMA type 3a | 8.29 | non-ambulant | 0.6 |
Female | 5.69 | SMA type 2 | 4.69 | non-ambulant | 0.5 |
Female | 29.51 | Healthy control | na | ambulant | 0.5 |
Male | 38.00 | SMA type 3b/4 | 13.50 | ambulant | 0.4 |
Male | 13.33 | Duchenne | u | non-ambulant | 0.4 |
Female | 13.64 | SMA type 2 | 12.93 | non-ambulant | 0.4 |
Male | 8.92 | SMA type 3a | 7.33 | ambulant | 0.2 |
Male | 21.98 | SMA type 3b/4 | u | ambulant | 0.2 |
Female | 12.08 | SMA type 2 | 11.33 | non-ambulant | 0.2 |
Female | 21.83 | SMA type 3a | 20.42 | non-ambulant | 0.2 |
Female | 7.73 | HMSN | 6.40 | ambulant | 0.2 |
Male | 13.34 | Duchenne | 11.84 | non-ambulant | 0.1 |
Male | 34.86 | SMA type 3b/4 | 31.36 | ambulant | 0.1 |
Male | 8.41 | Duchenne | u | ambulant | 0.1 |
Male | 14.62 | Duchenne | u | ambulant | 0.1 |
Female | 15.25 | HMSN | 13.92 | ambulant | 0.1 |