Glycogenosis type II (Pompe's disease) Ultrastructure of peripheral nerves

An ultrastructural study was made of the Schwann cells and vasa ner-vorum of peripheral nerves in a boy with Pompe9s disease who died of cardiac and respiratory failure at the age of 10 months. The cytoplasm of Schwann cells was found to contain excessive depositions of glycogen, and it was concluded that the accumulation most likely was produced by an inherent enzymatic defect within the Schwann cells. Organ transplantation might be justified in the treatment of patients with Pompe9s disease; conceivably allotransplantation of skin alone might be sufficient to compensate for the enzymatic deficit.