Jens De Cock1, Julie Snauwaert2, Walter Van Rompaey3, Marie-Anne Morren2, Philippe Demaerel4. 1. Radiology, University Hospitals Leuven, and Department of Imaging and Pathology, KU Leuven, Belgium. 2. Dermatology, University Hospitals Leuven, and Department of Oncology, KU Leuven, Belgium. 3. Radiology, GZA Ziekenhuizen Sint-Augustinus, Wilrijk, Belgium. 4. Radiology, University Hospitals Leuven, and Department of Imaging and Pathology, KU Leuven, Belgium. Electronic address: philippe.demaerel@uzleuven.be.
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.
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.
Authors: Francis Shih; Stephen Yip; Patrick J McDonald; Albert E Chudley; Marc R Del Bigio Journal: Acta Neuropathol Commun Date: 2014-10-21 Impact factor: 7.801