Among nineteen patients whose CK levels were more than tenfold higher and 50-fold lower than the ULN, twelve had dysferlinopathy, four had LGMD-R1-calpain3-related and two had dystroglycanopathies (one had LGMD-R9-FKRP-related and one had LGMD-R20-ISPD-related) (P > 0.05). Here, CRPPA is linked to neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.