Mutations in β, γ, δ and α sarcoglycans have beenassociated with limb-girdle muscular dystrophy,myoclonic dystonia and dilated cardiomyopathy, but nophenotype relation has yet been reported for SGCZ (7)in a proportion of cases, by mutations of the maternallyimprinted epsilon-sarcoglycan gene (SGCE. The gene discussed is SGCE; the disease is myoclonus-dystonia syndrome.