Neurocutaneous melanosis with hydrocephalus, intraspinal arachnoid collections and syringomyelia: case report and literature review

Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome, characterise d by large or numerous congenital pigmented nevi and excessive proliferatio n of melanin-containing cells in the leptomeninges. We report the MR findin gs in the brain and spine of a child with NCM who underwent neurosurgical t reatment and was followed up for 8 years. The findings in this child (small hyperintense collections of melanocytes in both temporal lobes, mild menin geal enhancement along the spine and the development of an extensive subara chnoid CSF accumulation with cord compression and syringomyelia) are believ ed to be exceptionally rare.