All are features of Pompe's disease except

Type II(Pompe) disease results from lysosomal storage of glycogen in skeletal muscles,cardiac muscles, and central nervous system. There is progressive cardiomyopathy.ECG shows left axis deviation,short PR interval & large QRS.Heart failure with dyspnoea and cyanosis may occur.Skeletal muscles show hypotonia and marked weakness.The tongue is large & protruding.It is also characterised by hepatomegaly and hypoglycemia.