Massive occurrence of polyglucosan bodies (PB) was found in the surgically removed temporal lobe of a 34 year-old woman presenting with complex partial seizures. The term of PB was proposed in order to group Lafora bodies (LB), corpora amylacea (CA) and Bielschowsky bodies (BB) on the basis of their biochemical similitude. A rigorous histochemical differentiation between these anomalies appears to be impossible at present. LB, BB and CA are mainly made up of glucose polymers i.e. polyglocusans. The pathway(s) leading from glycogen accumulation to PB formation is still unknown. PB are a hallmark of two diseases: Lafora disease and adult polyglucosan body disease. PB have occasionally been reported in rare cases of a variety of other neurological diseases. In all cases they were located within the neurons. This site characterizes Lafora bodies. BB are intraneuronal inclusions but restricted to neurons of the external pallidum. CA occur predominantly in the astroglia during the course of ageing. The significance of these structures depends on their regional distribution. The resemblance does not imply a common etiology for all conditions in which such bodies occur; it is probably due to the sharing of the final path in their causative pathway. Our case does not correspond to any of the classical diseases in which PB have been found. In our opinion, the patient exhibited a localized form of glycogen storage disease.