Mutations in the FKRP gene exhibit a wide spectrum of clinical severity, ranging from severe congenital muscular dystrophies to limb-girdle muscular dystrophy type 2I (LGMD2I).9, 10, 12 LGMD2I can present as mild or severe depending on the age of onset. The gene discussed is FKRP; the disease is autosomal recessive limb-girdle muscular dystrophy type 2I.