CELLULAR-DISTRIBUTION OF LESIONS IN BATTEN-DISEASE

One of the characteristic manifestations of chronic neuronal lipofusci nosis (Batten disease) is a marked predisposition for epileptic seizur es. The management of these seizures is very difficult. The present st udy was initiated to determine what mechanisms could account for the s eizure disorder, Tissue was examined from a patient with a history of Batten disease that was histologically verified. Reduced silver and Go lgi impregnations were done on the parietal cortex of the patient. The re was no evidence of the marked dendritic abnormalities seen in class ic epileptic foci. Instead there was marked swelling and dilatation of the axon hillock and initial segment, This finding suggested that inh ibition of these pyramidal neurons was markedly attenuated due to disr uption of initial segment inhibitory synapses, Studies are continuing to determine if the GABA decreases seen in Batten disease may in part be due to trophic sequences brought about by loss of these critical in hibitory synapses. (C) 1995 Wiley-Liss, Inc.