par Grégoire, Anne ;Dourov, Nicolas
Référence Acta Neuropathologica, 6, 1, page (70-79)
Publication Publié, 1966-03
Article révisé par les pairs
Résumé : This case of glycogenosis seams different from all the previously described forms of this disease. There are neither hepatic nor muscular evident glycogen accumulation. However, in brain cortex, β glycogen particles are stored in the glial processes, both astrocytic and oligodendrocytic, while axons, dendrites, and synaptic junctions are filled with glycogen in α or giant α particles. The morphology of these latter ones is "en rosettes" as that of the normal α particles but their diameter is 200 to 350 mμ instead of 100 to 150 mμ. Clinically this disease appeared as a progressive cerebral degeneration without any sign of disturbance in the hydrocarbon metabolism. No pathogenic explanation seems actually satisfactory. © 1966 Springer-Verlag.