Defective neurotransmission has not been reported in nuclear envelope-associated muscular dystrophies, and so we assessed neurophysiology (EMG) on one patient with LGMD (LMNA mutations) and two with Emery–Dreifuss muscular dystrophy, the archetypal nuclear envelopathy (one patient had LMNA mutations and the other had EMD mutations). This evidence concerns the gene LMNA and limb-girdle muscular dystrophy.