Neurocutaneous melanosis in a newborn with giant congenital melanocytic nevus

Department of Neuroradiology, Erasmus Hospital ULB, Brussels, Belgium, and Department of Clinical Physiopathology, Radiodiagnostic Section, University of Florence, Florence, Italy.
Journal of Neuroradiology (Impact Factor: 1.13). 11/2007; 34(4):272-5. DOI: 10.1016/j.neurad.2007.06.009
Source: PubMed

ABSTRACT Neurocutaneous melanosis (NCM) is known as a rare phakomatose characterised by large or numerous pigmented congenital nevi associated with leptomeninges melanin-containing deposits. We report a case of a newborn presenting at birth with a giant nevus covering about 40% of the total body surface. MRI showed T1 hyperintensities in the right amygdala and predominantly in the cerebellum corresponding to melanocytic cells.

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    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.
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