Table 4 Musculoskeletal abnormalities detected by pGALS in MPS attenuated type I
From: Assessment of musculoskeletal abnormalities in children with mucopolysaccharidoses using pGALS
Manoeuvre tested | pGALS instruction | MPS Ia (n = 8) |
|---|---|---|
Shoulder abduction, external rotation | Hands behind neck | 24/24 (100%) |
Shoulder flexion | Reach arms up | 23/24 (96%) |
Elbow extension | Reach up | 17/24 (71%) |
Wrist flexion | Hands together back to back | 17/24 (71%) |
Wrist extension | Hands palm to palm | 16/24 (67%) |
MCP, DIP, PIP extension | Hands and wrists together | 16/24 (67%) |
Gait | Observe patient walking | 11/17 (65%) |
TMJ excursion | Open mouth wide and try to put 3 fingers inside | 15/24 (62%) |
Spinal deformity | 7/19 (56%) | |
Forward flexion of spine | Bend forwards. Observe curvature of spine from all sides | 10/19 (53%) |
Neck extension | Look up to the ceiling | 12/23 (52%) |
Cervical spine lateral flexion | Touch ear to shoulder | 12/23 (52%) |
MCP/DIP/PIP extension | Hands out in front | 11/22 (50%) |
Elbow extension | Hands out in front | 8/16 (50%) |
Ankle dorsiflexion | Walk on heels | 9/18 (50%) |
MCP/DIP/PIP flexion | Make a fist | 10/24 (42%) |
Opposition of thumb and 3 rd -5 th fingers | Touch tips of fingers with thumb | 10/24 (42%) |
Elbow flexion | Put hands behind neck | 5/12 (42%) |
Forearm supination | Turn hand over | 6/18 (33%) |
Knee extension | Bring ankle up to bottom | 5/15 (33%) |
Knee flexion | 3/15 (20%) | |
Opposition of thumb and index finger | Touch tip of finger with thumb | 5/24 (21%) |
Elbow flexion | Put hands and wrists together | 4/24 (17%) |
Ankle plantar flexion | Walk on tip-toes | 2/18 (11%) |