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  • Title: A newborn with neurocutaneous melanocytosis and Dandy-Walker malformation.
    Author: De Cock J, Snauwaert J, Van Rompaey W, Morren MA, Demaerel P.
    Journal: Pediatr Neurol; 2014 Mar; 50(3):276-8. PubMed ID: 24321541.
    Abstract:
    BACKGROUND: Neurocutaneous melanocytosis is a rare congenital dysplasia of the neuroectodermal melanocyte precursor cells that leads to proliferation of melanin-producing cells in the skin and leptomeninges. PATIENT: We describe a newborn with a giant congenital melanocytic nevus on his back, buttocks, and thighs. His brain magnetic resonance imaging study revealed bilateral T1 hyperintense lesions in the cerebellum and in the amygdala, hydrocephalus, and a Blake's pouch cyst, consistent with neurocutaneous melanocytosis and Dandy-Walker malformation. CONCLUSION: Neurocutaneous melanocytosis has a wide clinical spectrum that includes hydrocephalus, epilepsy, cranial nerve palsy, increased intracranial pressure, and sensorimotor deficits.
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