Muscular phenotypes associated with LMNA mutations (LMNA-RM) include three main groups based on the distribution of muscle weakness or age at onset: limb-girdle muscular dystrophy type 1B (LGMD1B), Emery-Dreifuss muscular dystrophy type 2 (EDMD2) and a form of congenital muscular dystrophy (LMNA-CMD) [3]. This evidence concerns the gene LMNA and Autosomal dominant limb-girdle muscular dystrophy type 1B.