Methods and compositions for the treatment of cytoplasmic glycogen storage disorders

A method of reducing the level of cytoplasmic glycogen in a subject having a cytoplasmic glycogen storage disease, the method comprising:

• administering weekly to the subject a first therapeutically effective amount of about 40 mg/kg to about 100 mg/kg acid alpha-glucosidase until a desired response is obtained, wherein the desired response is selected from the group consisting of improved hypoglycemia, reduced growth retardation, reduced hepatomegaly, improved hepatic function, improved liver function, reduced liver inflammation, reduced hepatomegaly, improved cardiac status, reduced cardiomyopathy, reduced myopathy, reduced glycogen content, and a combination thereof;
• and
• administering at a regular interval to the subject a second therapeutically effective dose of about 20 mg/kg to about 80 mg/kg acid alpha-glucosidase,
• wherein the cytoplasmic glycogen storage disease is glycogen storage disease type I (GSD I), glycogen storage disease Ill (GSD III), glycogen storage disease IV (GSD IV), glycogen storage disease V (GSD V), glycogen storage disease VI (GSD VI), glycogen storage disease VII (GSD VII), glycogen storage disease IX (GSD IX), glycogen storage disease XI (GSD XI), glycogen storage disease XII (GSD XII), glycogen storage disease XIII (GSD XIII), glycogen storage disease XIV (GSD XIV), Danon disease, Lafora disease, or a condition associated with a protein kinase gamma subunit 2-deficiency (PRKAG2).