Lafora Disease - Lafora Bodies

Lafora Bodies

Lafora disease is distinguished by the presence of inclusions called "Lafora bodies" within the cytoplasm, the viscous fluidic matrix inside of cells. Lafora bodies are composed of abnormal glycogen called polyglucosans. These starch-like polyglucosans are insoluble and hence precipitate inside cells.

Polyglucosan bodies appear with age; in Lafora disease, their numbers have increased enormously. Lafora bodies have been observed in virtually all organs of patients with the disease. In the brain, their presence appears to be restricted to neurons; they do not seem to present in astrocytes. Their morphology varies from tissue to tissue, but they generally contain a central core and have a peripheral fluffy appearance.

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