Changes in nervous tissue in bovine generalized glycogenosis type II.

Generalized Glycogenosis type II has been diagnosed in seven calves from a herd of cattle maintained at the Murdoch University Veterinary School Farm. The syndromes seen are equivalent to the infantile and childhood forms of the disease in humans. In the brain and spinal cord of the calves acid alpha-glucosidase activity was depressed and glycogen deposition was markedly increased. Swelling, vacuolation and glycogen deposition was present in neurones of the central and autonomic nervous systems and retina, in glia and in Schwann cells and fibroblasts within peripheral nerves. The distribution was similar to that seen in human cases. The glycogen was both membrane bound and free within the cytoplasm. Changes similar to those seen in axonal dystrophy were present and some nerve fibres showed Wallerian degeneration. Evidence of nerve cell destruction and nerve cell loss were not seen and the predominant clinical signs were related to muscle weakness.