McArdle's disease is a metabolic myopathy of glycogen utilization caus
ed by an absence or deficiency of myophosphorylase. The muscle biopsy
features include increased deposition of subsarcolemmal glycogen and a
bsent phosphorylase histochemical staining of myofibers. We report the
clinical and unique pathologic findings in three cases of McArdle's d
isease with prominent type 1 fiber atrophy.