Acid proteinase and peroxidase activity in spielmeyer-Vogt's syndrome (Batten's syndrome-Stengel's syndrome)

Abstract

The acid proteinase and the peroxidase activity of peripheral leucocytes of respectively 12 and 13 patients suffering from Spielmeyer-Vogt's disease, alias Batten's disease, alias Stengel's disease was studied. Cathepsin D activity was found to be normal in the disease, but the cathepsin B activity was decreased by 24 percent. However, the decrease was insignificant. The peroxidase assay was made using Guajacol as well as p-phenylenediamine as substrate. When the assay was performed with p-phenylenediamine, the enzymic activities were significantly decreased (p less than or equal to 1%) compared to the activity of normal control leucocytes. Assaying peroxidase activity by means of Guajacol revealed a non-significant difference between the two groups. The data obtained indicate the syndrome under study to be due to hampered peroxidase activity. The defect found explains the occurrence of lipofuchsin in the nervous system previously demonstrated.