Fig. 2.

Progressive muscle involvement in GNE myopathy. Representative lower extremity muscle MRI images of patients with GNE myopathy, with advancing disease progression from left to right. Coronal T1-weighted muscle MRI images (upper panels). Axial T1-weighted images of the mid-femoral thigh (middle panels). Axial T1-weighted images of the lower leg (lower panels). Progressive muscle atrophy of the lower extremities is noted initially in the anterior tibialis muscle (B lower panel), followed by involvement of muscles in the calves (C lower panel) and posterior thigh muscles (B, C middle panel), and finally involvement of the quadriceps in advanced stages of the disease (D middle panel)