@article{oai:hirosakiuhw.repo.nii.ac.jp:00000044, author = {Yoshimura, Noriaki}, issue = {1}, month = {Mar}, note = {Article, Lafora disease (LD) is an autosomal recessively inheritable metabolic disorder of carbohydrate, characterized clinically by myoclonus, epileptic convulsions and mental deterioration, and pathologically by widespread presence of polyglucosan bodies (Lafora bodies; LB) in the CNS neurons and the cells of other organs. Although the histochemistry and ultrastructure of LB have been well reported, localization of LB within neuronal networks has not been well clarified yet. The purpose of the present study is to elucidate this issue. A male patient with LD who died at 30 years of age was autopsied. The autopsy case was reported elsewhere. This time, specimens taken from the frontal and temporal cortices were observed by electron microscopy. There were a number of synapses on the cell membrane which covered large LB within the perikaryon and dendritic shaft. LB were encountered at times in myelinated axons. Very small LB (ø <2 μm) were occasionally found in the presynaptic terminals of asymmetric synapses, but hardly found in the postsynaptic endings. It is known that disrupting the delicate balance of inhibitory and excitatory synaptic transmission can trigger the disordered, synchronous firing of neurons that underlies a seizure. The results of the present study strongly suggest that disruption of the delicate balance of such synaptic transmissions, which can trigger the disordered synchronous discharges of neurons, may well take place in the cerebral cortices in LD., 弘前医療福祉大学紀要, 4(1), 2013, p.13-24}, pages = {13--24}, title = {Brain pathology of Lafora disease : localization of polyglucosan aggregations (Lafora bodies) within neuronal networks of the cerebral cortex}, volume = {4}, year = {2013} }