[Anatomo-clinical and neurochemical aspects of Pelizaeus-Merzbacher disease]

The classification of the diffuse cerebral sclerosis has been possible because of the new advances in biochemical and histochemical studies of myelin and of neuropathological findings. The enzymatic disturbances in metachromatic leucodystrophy and in Krabbe's disease were determined. That allowed the definition of new diagnostic methods, safe and no traumatic. About the sudanophilic leucodystrophy, it is, even now, more doubtful: in this paper, we compared the data of own two observations of Pelizaeus-Merzbacher's disease with the 52 cases from the literature.