Fig. 2.

Distinctive patterns of fatty infiltration in DGC-related muscular dystrophies. a Representative cases of DGC-related muscular dystrophies showing distinctive patterns of muscle involvement. c–g and q–s Representative cases of sarcoglycanopathies and dystroglycanopathy showing the concentric fatty infiltration pattern. o, p, t, and u Representative cases of dystrophinopathies showing the trefoil with single fruit sign. g and n A representative case of sarcoglycanopathies showing both patterns. j–m Representative cases of sarcoglycanopathies and dystroglycanopathy showing severe fatty infiltration and relative sparing of the medial part of adductor longus. c and j, patient 1; d and k, patient 16; e and l, patient 22; f and m, patient 29; g and n, patient 17; h and o, patient 36; i and p, patient 50; q, patient 21; r, patient 4; s, patient 28; t, patient 38; u, patient 52. b Scheme of the DGC in skeletal muscle adapted from [1]. AL, adductor longus; Ab, adductor brevis; AM, adductor magnus; VL, vastus lateralis; RF, rectus femoris; VI, vastus intermedius; VM, vastus medialis; Sa, Sartorius; Gr, gracilis; BFSH, biceps femoris, short head; BFLH, biceps femoris, long head; ST, semitendinosus; SM, semimembranosus. DGC, dystrophin-glycoprotein complex; SG, sarcoglycan; DG, dystroglycan; LGMD, limb-girdle muscular dystrophy; DMD, Duchenne muscular dystrophy; BMD, Becker muscular dystrophy