Aside from the myopathic features (fiber size variation, internal myonuclei and fiber splitting), inflammatory components appear frequently in biopsy analysis of specific LGMD subtypes like dysferlinopathies (133), anoctaminopathies (94) and dystroglycanopathies and rarely in others, including LGMD2A (134), LGMD2C (135) and LGMD2D (manuscript in press). This evidence concerns the gene CAPN3 and neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.