Abstract
We compared intraneuronal polyglucosan body (PGB) in brains from two patients diagnosed as having Lafora disease and from 18 aged dogs (age range: 10 to 22 years, various breeds). PGBs appeared as various-sized spheroids intensely stained with periodic acid-Schiff (PAS) in both humans and aged dogs. Immunohistochemistry with a monoclonal antibody raised against human polyglucosan showed positive staining of the PGBs. Ultrastructurally, PGBs in both humans and aged dogs were composed of fibril-like structures 4 to 20 nm wide. Electron-dense material formed the central cores of the fibrils, but was also scattered in their peripheral areas. The fibril-like structures were intensely stained upon application of the Thiéry procedure. Immunoelectron microscopy showed that the fibril-like structures were specifically labeled with gold particles. The histological, immunohistochemical and ultrastructural features of the PGBs in humans and aged dogs were quite similar.
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