Skip to main content

Table 5 Musculoskeletal abnormalities detected by pGALS in MPS type II

From: Assessment of musculoskeletal abnormalities in children with mucopolysaccharidoses using pGALS

Manoeuvre tested

pGALS instruction

MPS II (n = 4)

Shoulder flexion

Reach arms up

12/12 (100%)

Shoulder abduction, external rotation

Hands behind neck

12/12 (100%)

Elbow extension

Reach up

12/12 (100%)

Wrist flexion

Hands together back to back

12/12 (100%)

Wrist extension

Hands palm to palm

11/11 (100%)

MCP/DIP/PIP extension

Hands out in front

12/12 (100%)

Cervical spine lateral flexion

Touch ear to shoulder

12/12 (100%)

MCP, DIP, PIP extension

Hands and wrists together

11/12 (92%)

TMJ excursion

Open mouth wide and try to put 3 fingers inside

10/12 (83%)

MCP/DIP/PIP flexion

Make a fist

10/12 (83%)

Elbow extension

Hands out in front

9/11 (82%)

Forearm supination

Turn hand over

9/11 (82%)

Ankle dorsiflexion

Walk on heels

7/9 (78%)

Neck extension

Look up to the ceiling

9/12 (75%)

Gait

Observe patient walking

7/10 (70%)

Forward flexion of spine

Bend forwards. Observe curvature of spine from all sides.

8/12 (67%)

Opposition of thumb and 3 rd -5 th fingers

Touch tips of fingers with thumb

8/12 (67%)

Spinal deformity

 

5/9 (56%)

Opposition of thumb and index finger

Touch tip of finger with thumb

5/12 (42%)

Elbow flexion

Put hands behind neck

5/12 (42%)

Knee extension

Bring ankle up to bottom

4/12 (33%)

Knee flexion

7/12 (58%)

Ankle plantar flexion

Walk on tip-toes

2/9 (22%)

Elbow flexion

Put hands and wrists together

2/11 (18%)

  1. Figure Key: ANA = antinuclear antibody; ESR = erythrocyte sedimentation rate; IDUA = iduronidase, alpha-L; JIA = juvenile idiopathic arthritis; NSAIDs = non-steroidal anti-inflammatory drugs; pGALS = paediatric Gait, Arms, Legs and Spine; RA = rheumatoid arthritis; uGAG = urinary glycosaminoglycans.