Evaluation of the possible role of coenzyme Q(10) and vitamin E in juvenile neuronal ceroid-lipofuscinosis (JNCL)

The juvenile type of neuronal ceroid lipofuscinosis (JNCL) is a recessively inherited, progressive neurodegenerative disease. In this study the levels of the antioxidant factors coenzyme Q(10) (CoQ(10)) and vitamin E (alpha-tocopherol) were measured in plasma samples of 29 JNCL patients and compared to 48 healthy controls. A significant reduction of the coenzyme Q(10) lever (0.59 +/- 0.25 mu g/ml) was observed in JNCL patients when compared to control subjects (0.80 +/- 0.26 mu g/ml). The level of vitamin E was also reduced markedly in JNCL patients when compared to controls (10.4 +/- 4.1 and 12.1 +/- 4.5 mu g/ml, respectively). The low levels of CoQ(10) and vitamin E in JNCL patients may indicate an impaired antioxidant protection in this disease. (C) 1997 Elsevier Science Ltd.