Summary
Lafora bodies were studied in a parietal cortex biopsy of a patient suffering from progressive myoclonic epilepsy, both histochemically (with the optical microscope) and in the electron microscope. The bodies appear at the electron microscope as fibrillar and granular masses, not surrounded by a membrane. Most of them closely resemble corpora amylacea, but are located in neurons or their processes. The PAS positive “dust-like” particles, previously described in this disease with the light microscope, present, at the electron microscope, the same fundamental structure. Lafora bodies do not contain glycogen but mainly mucopolysaccharides. The hypothesis of their lysosomal nature is discarded.
Résumé
Les corps de Lafora ont été étudiés histochimiquement au microscope optique, et au microscope électronique dans une biopsie de cortex pariétal prélevée chez un patient souffrant d'épilepsie myoclonique. Ils apparaissent au microscope électronique comme des masses fibrillaires et granulaires non délimitées par une membrane. La plupart d'entre eux ressemblent aux corps amylacés, mais ils sont localisés dans les neurones ou leurs prolongements. Les très petites inclusions PAS positives présentent au microscope électronique la même structure fondamentale que les corps de Lafora. Ces derniers ne contiennent pas de glycogène, mais essentiellement des mucopolysaccharides. L'hypothèse de leur nature lysosomiale est écartée.
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Van Hoof, F., Hageman-Bal, M. Progressive familial myoclonic epilepsy with lafora bodies. Acta Neuropathol 7, 315–326 (1967). https://doi.org/10.1007/BF00688087
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DOI: https://doi.org/10.1007/BF00688087