Alternative Names & Classifications:

Acid Maltase Deficiency (CLINICAL), Glycogenosis Type II (CLINICAL), Glycogen Storage Disease Due To Acid Maltase Deficiency (ICD-11), Glycogen Storage Disease Type II (MESH)

Description

Specifically, a deficiency of the enzyme acid alpha-glucosidase leads to glycogen accumulation in the lysosomes, particularly in muscle tissue. This causes progressive muscle weakness and can severely affect the heart and respiratory system.