[EEG changes in the course of progressive cerebral disorders in children (author's transl)]

This survey presents EEG-data on the following disorders: Globoid-cell leucodystrophy (Krabbe), Metachromatic Leukodystrophy, GM2 Gangliosidosis I (Tay-Sachs), Neuronal Ceroidlipofuscinosis. During Krabbe's disease the background activity of the EEG slows down, multifocal and generalized hypersynchroneous activity (HSA) appears, in the final stage hypsarrythmia is found occasionally. In contrast HSA during Sulfatide lipidosis appears only in the final stages. The EEG changes in Tay Sachs disease are minimal in the beginning; later only high amplitude slowing and especially focal abnormalities with spikes and sharp-waves will appear and in advanced stages there is generalized depression of amplitudes. The Elektroretinogramm (ERG) remains intact but VEP disappear. In contrast, in Neuronal Ceroidlipofuscinosis irregular spikes and sharp-waves can be found shortly after onset of the disease. Later on there is a generalized depression of amplitudes; photic driving and differences between sleeping and waking diminish from the 3rd year of life on; thereafter, the EEG becomes isoelectric. The ERG is extinguished from the very beginning in infantile, lateinfantile and juvenile type of Ceroid-Lipofuscinosis.