Lipoyl dehydrogenase activity of erythrocytes in Spielmeyer-Vogt-Batten's disease

Using the method for the determination of the lipoyl dehydrogenase activity in intact erythrocytes described by Seet and Lee (1975), it was demonstrated that in patients with Spielmeyer-Vogt-Batten's disease, this activity was around the lower limit of normal. In these patients, the enzymatic activity is significantly reduced to such an extent that it may affect the function and metabolism of the erythrocytes.