Experience of antioxidant treatment in neuronal ceroid-lipofuscinosis of Spielmeyer-Sjögren type.

A therapeutic trial with antioxidants in neuronal ceroid lipofuscinosis (NCL) of Spielmeyer-Sjögren type is presented. The series consisted of 46 patients, 23 of whom received antioxidants while 23 served as controls. At the start of the trial the patients were classified into two groups on the basis of their IQs. Eleven children, aged 6 1/2 to 12 years, were of low normal or subnormal intelligence (treatment group I). After an observation period of 5 to 6 years a significant change in IQ was seen in 6 children; an improvement was noticed in 2 boys and a deterioration in 4 patients. The IQs were higher, the neurological signs less marked and epilepsy less frequent among the patients receiving antioxidants than among the controls. The therapy did not benefit vision and it was unsuccessful in advanced cases. The results are discussed and compared with those reported by other authors.