NORMAL SUPEROXIDE RADICAL PRODUCTION IN THE NEURONAL CEROID-LIPOFUSCINOSES

The neuronal ceroid-lipofuscinoses (NCL) are a group of inherited prog ressive encephalopathies. Striking histomorphological feature of the N CL is the accumulation of storage material within the lysosomes in neu ral and extraneural cells. The basic underlying defect causing the I d isease is not known. Presupposing a disturbance in lipid peroxidation, some authors recommend antioxidant treatment to slow down the progres sion of the disease. In this study: the superoxide radical production of polymorphonuclear leukocytes as one potential source of reactive ox ygen species nas measured in this disorder for the first time. No sign ificant difference in this production between affected individuals and healthy controls could be detected. Our findings cast doubt on the th erapeutic benefit of antioxidant treatment.