Microcephaly with peculiar demyelination mimicking Pelizaeus-Merzbacher disease.

The neuropathological studies of a 4-year old idiot with microcephaly revealed a striking demyelinating disease compatible with Pelizaeus-Merzbacher disease. The case seemed to be best classified as the patchy demyelination type, but the most unusual finding was a prominent vasculature in some demyelinated areas. Intrauterine hypoxia was proposed as a causative factor of this type of demyelinative lesions.