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

Neurocutaneous melanosis (NCM) is a rare, congenital, noninherited neurocutaneous syndrome characterized by the presence of “multiple or large congenital nevi” with central nervous system melanocytic deposits. Majority of cases are asymptomatic. Symptomatic cases present with hydrocephalus and signs and symptoms of increased intracranial pressure. The latter is associated with poor prognosis. We herein report an asymptomatic patient with giant congenital melanocytic nevi. He had a screening magnetic resonance imaging study at the age of 2 months that showed T1 hyperintensities in the region of amygdala of both temporal lobes. He was asymptomatic until the age of 4 years when he presented with seizure activity. A diagnosis of NCM was made. He was discharged on emergency antiepileptic medication. On regular follow-up, he remained seizure free for 1 year after the first episode of seizure. Unlike other cases reported in literature, this, to the best of our knowledge, is the first report of nonprogressive symptomatic NCM.

Key-words:

Neurocutaneous melanosis - congenital melanocytic nevi - leptomeningeal melanosis - prognosis

Publication History

Article published online:
07 July 2022