title: Neurocutaneous melanosis: follow-up and literature review.
abstract: Neurocutaneous melanosis is a rare, congenital, non-inherited syndrome characterized by numerous and/or large congenital nevi with intracranial leptomeningeal melanocytosis. This report describes two patients, presenting with a giant congenital nevus involving a major portion of the posterior trunk with satellite congenital nevi scattered all over the body, who developed seizures at 4 and 6 months of age, respectively. Changes in follow-up magnetic resonance (MR) examinations over an 8-year period were seen in case 1, while parenchymal melanocytic accumulation was reported in the region of the amygdala in case 2. These cases emphasize that neurocutaneous melanosis should be suspected in patients with giant congenital nevus with or without neurological symptoms. Also, neuroaxial MR screening should be performed in all cases and, ideally, before myelination of the brain to provide the highest sensitivity for detecting melanin deposits in the leptomeninges.
authors.affiliation: Department of neurology and neurosurgery, universidade Federal de São Paulo, UNIFESP, 740, Rua Botucatu, Vila Clementino, 04023-900 São Paulo, Brazil. monica_scattolin@yahoo.com.br
authors.forename: M A A
authors.initials: MA
authors.lastname: Scattolin
authors.forename: J
authors.initials: J
authors.lastname: Lin
authors.forename: M M
authors.initials: MM
authors.lastname: Peruchi
authors.forename: A J
authors.initials: AJ
authors.lastname: Rocha
authors.forename: M R
authors.initials: MR
authors.lastname: Masruha
authors.forename: L C P
authors.initials: LC
authors.lastname: Vilanova
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008548:Melanosis / Q000188:drug therapy* / Q000473:pathology*; D020752:Neurocutaneous Syndromes / Q000188:drug therapy* / Q000473:pathology*; D016896:Treatment Outcome; D014635:Valproic Acid / Q000627:therapeutic use*
chemical_list: D014635:Valproic Acid