POLYGLUCOSAN BODIES - AN UNUSUAL DISCOVERY IN A CASE OF TEMPORAL EPILEPSY - REVIEW OF THE LITERATURE

Massive occurence of polyglucosan bodies (PB) was found in the surgica lly removed temporal lobe of a 34 year-old woman presenting with compl ex partial seizures. The term of PB was proposed in order to group Laf ora bodies (LB), corpora amylacea (CA) and Bielschowsky bodies (BB) on the basis of their biochemical similitude. A rigorous histochemical d ifferentiation between these anomalies appears to be impossible at pre sent. LB, BB and CA are mainly made up of glucose polymers i.e. polygl ocusans. The pathway(s) leading from glycogen accumulation to PB forma tion is still unknown. PB are a hallmark of two diseases: Lafora disea se and adult polyglucosan body disease. PB have occasionally been repo rted in rare cases of a variety of other neurological diseases. In all cases they were located within the neurons. This site characterizes L afora bodies. BB are intraneuronal inclusions but restricted to neuron s of the external pallidum. CA occur predominantly in the astroglia du ring the course of ageing. The significance of these structures depend s on their regional distribution. The resemblance does not imply a com mon etiology for all conditions in which such bodies occur; it is prob ably 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.