Table 2.

Glycogen accumulation within specific tissues

Glycogen accumulation	Patient 1	Patient 2	Patient 3
Skeletal muscle
Quadriceps femoris	Severe	Severe	Moderate
Psoas	Severe
Sternothyroid	Severe
Proximal esophagus	Severe		Moderate
Deltoid	Moderate	Severe	Mild
Intercostal	Moderate
Diaphragm	Moderate	Moderate	Minimal
Extraocular	Moderate	Moderate	Moderate
Smooth muscle
Eye
Iris sphincter	Severe	Severe	Severe
Ciliary body	Moderate	Mild	Mild
Genitourinary
Urinary bladder	Severe	Severe	Moderate
Blood vessels
Medium-sized arteries in sclera and orbit	Severe	Severe
Respiratory
Bronchi	Mild	Mild	Moderate
Gastrointestinal
Esophagus	Mild		Moderate
Stomach	Mild	Moderate	Moderate
Small intestine	Mild	Mild	None
Colon	Mild	Mod	Moderate
Heart
Cardiac myocytes
Outer myocardium of anterior, lateral, and posterior wall of left ventricle	Severe	Moderate	Moderate
Inner myocardium, subendocardium of anterior, lateral
and posterior walls of LV	Moderate	Moderate	Severe
Mid-myocardium of anterior, lateral, posterior walls of LV
Conduction system	Mild	Mild	Moderate
Sinoatrial node
Atrioventricular node
Bundle of His	Severe
Cardiac weight (mean for age)	Severe		Moderate to severe
	Severe		Moderate
	83 g (55 g)	120 g (49 g)	200 g (43 g)
Peripheral nervous system
Sural nerve	Moderate	Moderate	Moderate
Ganglion cells (neurons)
Small intestine	Moderate	Severe	Severe
Colon	Moderate	Severe	Severe
Adrenal glands	None	Minimal	Moderate
Eyes
Lens epithelial cells	Severe	Moderate	Severe
Retinal ganglion cells	Moderate	Moderate	Moderate
Corneal endothelial cells	Moderate	Moderate	Mild
Iris pigmented epithelium	Mild	Moderate	Moderate
Photoreceptors	None	Moderate	Moderate
Optic nerve glial cells	Moderate	Moderate	Severe