[Neurocutaneous melanoblastosis (author's transl)]

Primary melanomas, diffuse melanoses or melanoblastoses of the CNS are rare findings. The combination of such changes with giant hairy naevi on dysontogenetic base in known as neurocutaneous melanosis (mélanose neurocutanée Touraine). A case of a 27-year-old man with giant pigment naevi is described, who died 7 months after first neurologic symptoms caused by diffuse progredient cerebrospinal leptomeningeal melanoblastosis. Difficulties of clinical diagnosis and morphological classification of leptomeningeal changes are discussed.