Table 1.
Age, mean (SD), y | 12.1 (3.9) |
Body mass, mean (SD), kg | 44.9 (18.0) |
Height, mean (SD), cm | 149.8 (21.7) |
Sex, female, n (%) | 22 (44%) |
Ambulatory status, ambulatory, n (%) | 34 (68%) |
MRC knee extensors, n | 36 |
MRC 3, n (%) | 11 (31%) |
MRC ≥ 4, n (%) | 25 (69%) |
MRC knee flexors, n | 37 |
MRC 3, n (%) | 5 (14%) |
MRC ≥ 4, n (%) | 32 (86%) |
MRC elbow extensors, n | 40 |
MRC 3, n (%) | 5 (12.5%) |
MRC ≥ 4, n (%) | 35 (87.5%) |
MRC elbow flexors, n | 49 |
MRC 3, n (%) | 14 (29%) |
MRC ≥ 4, n (%) | 35 (71%) |
Diagnosis, n (%) | |
Axonal motor neuropathy | 1 (2%) |
Axonal polyneuropathy | 1 (2%) |
Becker muscular dystrophy | 3 (6%) |
Carey Fineman Ziter syndrome | 2 (4%) |
Central core myopathy | 1 (2%) |
Duchenne muscular dystrophy | 1 (2%) |
Hereditary motor and sensory neuropathy | 7 (14%) |
Myotonic dystrophy | 1 (2%) |
Myasthenia gravis | 2 (4%) |
Myotonica congenita (type Becker) | 2 (4%) |
Nemaline myopathy | 1 (2%) |
Silver syndrome | 1 (2%) |
Spinal muscular atrophy | 27 (54%) |
1c | 1 (2%) |
2a | 7 (14%) |
2b | 1 (2%) |
3a | 17 (34%) |
3b | 1 (2%) |
MRC (Medical Research Council) scale.