Glycogen-Storage Disease

Abstract

A case of glycogen-storage disease (Coritype II), with accumulation of glycogen in the myocardium, skeletal muscle, smooth muscle, liver, kidney, central nervous system, and autonomic ganglia is presented. Glycogen storage in the autonomic ganglia of the intestinal tract (Plexus-myentericus-Auerbach), which gave rise clinically to Hirschsprung's disease, could explain the feeding problems and constipation often observed in these cases. The classification into four different types of Cori and Andersen appears to be the most satisfactory one at the present time. These types are different disease entities with the same expression of abnormality, the deposition of glycogen; but the pathogenesis and the clinical and pathologic findings are strikingly different. Glycogen storage of the heart is probably more common than the review of the literature indicates. Many of these cases are missed, mainly types III and IV, because the possibility is not considered and the patient is inadequately studied in life and during necropsy.