Symptomatic neurocutaneous melanosis with unusual benign course: A case report and review

Abstract

Neurocutaneous melanosis (NCM) is a very uncommon, nonfamilial "neurocutaneous syndrome." It is characterized by the presence of "multiple or large congenital nevi" with central nervous system (CNS) melanocytic deposits. [1] Rokitansky published the first report of NCM in 1861. In his report, he described a mentally disabled girl who was born with a "large congenital melanocytic nevus" and eventually died due to hydrocephalus complications. At autopsy, he found that she had brain melanocytic accumulations. [2] It was named NCM in 1948 by Van Bogaert. The accurate incident of NCM is unidentified. It is very rare, with only about 100-200 cases reported. Both sexes are affected equally, and there is no racial or ethnic predominance identified.