Neurophysiological investigations in two cases of Alexander's disease with infantile onset

Repeated neurophysiological investigations over a six year period are reported in two children with histologically proven Alexander's leucodystrophy. The EEG alterations were already detectable at an early stage of the disease with subsequent progressive deterioration in both cases. There was no definite abnormality in the ERG-VEP studies which were only carried out in the younger patient. The EEG features in the course of the illness are different from those seen in most of the identified genetically determined neurometabolic diseases as well as in the group of so-called leucodystrophies of similar age groups. However, the EEG-ERG-VEP features are not sufficiently characteristic to be diagnostic.