Leptomeningeal melanoblastosis is an uncommon but devastating malignan
cy. A case of primary leptomeningeal amelanotic melanoblastosis in a 6
4-year old male is reported. Clinical findings included headache, atax
ia, psychopathological disorders and progressive paraparesis. CT-scans
of the brain showed an internal hydrocephalus. The disease was diagno
sed on the basis of cerebrospinal fluid cytology by light microscopy.
It was finally confirmed by autopsy which showed a primary melanoblast
oma of the meninges with only few pigmented cells and radiculomedullar
y infiltration.