Table 2 Musculoskeletal abnormalities detected by pGALS (number of abnormal observations to total observations)
From: Assessment of musculoskeletal abnormalities in children with mucopolysaccharidoses using pGALS
Manoeuvre tested | pGALS instruction | All MPS (n = 18 patients) 1203 total observations | MPS I (n = 13) | MPS II (n = 4) | Mannosidosis (n = 1) |
|---|---|---|---|---|---|
Shoulder abduction, external rotation | Hands behind neck | 49/54 (91%) | 35/39 (90%) | 12/12 (100%) | 2/3 (67%) |
Shoulder flexion | Reach arms up | 48/54 (89%) | 36/39 (93%) | 12/12 (100%) | 0/3 (0%) |
Wrist flexion | Hands together back to back | 41/54 (76%) | 27/39 (69%) | 12/12 (100%) | 2/3 (67%) |
Elbow extension | Reach up | 39/53 (74%) | 26/39 (90%) | 12/12 (100%) | 1/2 (50%) |
TMJ excursion | Open mouth wide and try to put 3 fingers inside | 39/54 (72%) | 28/39 (72%) | 10/12 (83%) | 1/3 (33%) |
MCP, DIP, PIP extension | Hands and wrists together | 37/54 (69%) | 24/39 (62%) | 11/12 (92%) | 2/3 (67%) |
Forward flexion of spine | Bend forwards. Observe curvature of spine from all sides | 31/50 (62%) | 21/35 (60%) | 8/12 (67%) | 2/3 (67%) |
Spinal deformity | Observe patient standing and then bending forwards | 28/45 (62%) | 22/34 (65%) | 5/9 (56%) | 1/2 (50%) |
Gait | Observe patient walking | 28/45 (62%) | 20/32 (63%) | 7/10 (70%) | 1/3 (33%) |
Wrist extension | Hands palm to palm | 33/53 (60%) | 22/39 (56%) | 11/11 (100%) | 0/3 (0%) |
MCP/DIP/PIP extension | Hands out in front | 29/52 (56%) | 17/37 (46%) | 12/12 (100%) | 0/3 (0%) |
Cervical spine lateral flexion | Touch ear to shoulder | 29/53 (55%) | 17/38 (45%) | 12/12 (100%) | 0/3 (0%) |
Elbow extension | Hands out in front | 22/40 (55%) | 12/27 (44%) | 9/11 (82%) | 1/2 (50%) |
Ankle dorsiflexion | Walk on heels | 24/45 (53%) | 15/33 (45%) | 7/9 (78%) | 2/3 (67%) |
Neck extension | Look up to the ceiling | 28/53 (53%) | 19/38 (50%) | 9/12 (75%) | 0/3 (0%) |
Forearm supination | Turn hand over | 20/42 (48%) | 10/30 (33%) | 9/11 (82%) | 1/1 (100%) |
Opposition of thumb and 3 rd -5 th fingers | Touch tips of fingers with thumb | 23/54 (43%) | 14/39 (36%) | 8/12 (67%) | 1/3 (33%) |
MCP/DIP/PIP flexion | Make a fist | 20/54 (37%) | 10/39 (26%) | 10/12 (83%) | 0/3 (0%) |
Elbow flexion | Put hands behind neck | 12/42 (29%) | 6/27 (33%) | 5/12 (42%) | 1/3 (33%) |
Knee extension | Bring ankle up to bottom | 12/44 (27%) | 7/29 (24%) | 4/12 (33%) | 1/3 (33%) |
Knee flexion | 11/45 (24%) | 4/30 (13%) | 7/12 (58%) | 0/3 (0%) | |
Opposition of thumb and index finger | Touch tip of finger with thumb | 10/54 (19%) | 5/39 (13%) | 5/12 (42%) | 0/3 (0%) |
Elbow flexion | Put hands and wrists together | 7/53 (13%) | 5/39 (13%) | 2/11 (18%) | 0/3 (0%) |
Ankle plantar flexion | Walk on tip-toes | 4/45 (9%) | 2/33 (6%) | 2/9 (22%) | 0/3 (0%) |