Spielmeyer-Vogt disease: clinical and neurophysiological aspects

Recent neurophysiological studies have identified a characteristic profile in the case of certain progressive infantile encephalopathies which, together with clinical data, may assist with the diagnosis. The authors report a case of juvenile ceroid lipofuscinosis (Spielmeyer-Vogt disease) and stress the importance of carrying out neurophysiological investigations for diagnostic purposes and for controlling the progression of the disease. The effect of antioxidant therapy (sodium selenite and vitamin E) on these investigations and on the natural evolution of the disease is also discussed.