Table 3 Musculoskeletal abnormalities detected by pGALS in MPS type I (Hurler)
From: Assessment of musculoskeletal abnormalities in children with mucopolysaccharidoses using pGALS
Manoeuvre tested | pGALS instruction | MPS I (Hurler) (n = 5) |
|---|---|---|
Spinal deformity | 15/15 (100%) | |
Shoulder flexion | Reach arms up | 13/15 (87%) |
TMJ excursion | Open mouth wide and try to put 3 fingers inside | 13/15 (87%) |
Forward flexion of spine | Bend forwards. Observe curvature of spine from all sides | 11/15 (73%) |
Shoulder abduction, external rotation | Hands behind neck | 11/15 (73%) |
Wrist flexion | Hands together back to back | 10/15 (67%) |
Elbow extension | Reach up | 9/15 (60%) |
Gait | Observe patient walking | 9/15 (60%) |
MCP, DIP, PIP extension | Hands and wrists together | 8/15 (53%) |
Neck extension | Look up to the ceiling | 7/15 (47%) |
Wrist extension | Hands palm to palm | 6/15 (40%) |
MCP/DIP/PIP extension | Hands out in front | 6/15 (40%) |
Ankle dorsiflexion | Walk on heels | 6/15 (40%) |
Elbow extension | Hands out in front | 4/11 (36%) |
Cervical spine lateral flexion | Touch ear to shoulder | 5/15 (33%) |
Forearm supination | Turn hand over | 4/12 (33%) |
Opposition of thumb and 3 rd -5 th fingers | Touch tips of fingers with thumb | 4/15 (27%) |
Knee extension | Bring ankle up to bottom | 2/14 (14%) |
Knee flexion | 1/15 (7%) | |
Elbow flexion | Put hands behind neck | 1/15 (7%) |
Elbow flexion | Put hands and wrists together | 1/15 (7%) |
Ankle plantar flexion | Walk on tip-toes | 0/15 (0%) |
MCP/DIP/PIP flexion | Make a fist | 0/15 (0%) |
Opposition of thumb and index finger | Touch tip of finger with thumb | 0/15 (0%) |