Table 1.

Clinical Summary of the Patients

Clinical Presentation	III-3	IV-6
Proximal atrophy	+	+
DTR patella/ankle	0/0	trace/0
Deltoid (L/R)	1–2/1–2	4+/4+
Biceps (L/R)	4−/4−	4/4
Triceps (L/R)	4−/4−	4+/4+
FDI (L/R)	4/4	5/5
EIP (L/R)	4/4	Not Tested
Hip flexors (L/R)	0–1/0–1	4−/4−
Hip extensors (L/R)	0–1/0–1	2/2
Knee flexors (L/R)	0/0	3+/3
Knee extensors (L/R)	1/1	4/4
Ankle dorsiflexion (L/R)	5/5	Limited by tight heel cords
Ankle plantarflexion (L/R)	4−/4−	4/4
Scapula winging	+	+
FVC (% predicted)	42	85
MIP (cm H2O)	−60	−87
Creatine Kinase level (U/L)	181	3000