NEUROCUTANEOUS MELANOSIS - CASE-REPORT AND A BRIEF REVIEW

Neurocutaneous melanosis is a rare congenital syndrome characterised b y large or numerous congenital pigmented naevi and excessive prolifera tion of melanin-containing cells in the leptomeninges. The process is diffuse or multifocal, and has a tendency to infiltrate the neural tis sue and the cerebrospinal cord; remote metastases may occur. There is usually histological evidence of malignancy (cellular pleomorphism and mitotic activity). Involvement of the basal cisterns is apt to cause internal hydrocephalus, and the prognosis is grave even when there is no histological evidence of malignancy. We present the case history an d necropsy findings of a baby boy with neurocutaneous melanosis, follo wed by a brief review.