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Primary metabolic myopathy as a type of congenital myopathies was first described by McArdle in 1951. Glycogen storage disease is a disease caused by genetic mutations involved in glycogen synthesis, glycogenolysis or glycolysis. Several types of glycogen storage disease are known to cause metabolic myopathies. We report a case of adult onset metabolic myopathy with glycogen storage. (Korean J Clin Neurophysiol 2014;16:81-85)doi:10.14253/kjcn.2014.16.2.81 fatcat:meqoygdh65bnrccuzr2dcxf224