Neurocutaneous Melanosis with Hydrocephalus and Dandy-Walker Variant

Asian J Neurosurg. 2021 Dec 18;16(4):876-880. doi: 10.4103/ajns.AJNS_542_20. eCollection 2021 Oct-Dec.

Abstract

Neurocutaneous melanosis (NCM) is one of the rare, congenital, noninheritable phakomatoses characterized by the presence of large and/or multiple congenital melanocytic cutaneous nevi associated with intracranial leptomeningeal melanocytosis. NCM usually presents before 2 years of age. So far 302 cases have been reported in literature. We report a case of NCM presenting with obstructive hydrocephalus and Dandy-Walker Variant in a young adult.

Keywords: Dandy-Walker variant; hydrocephalus; neurocutaneous melanosis; tuberculous meningitis.

Publication types

  • Case Reports