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Journal of Child Neurology
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Neurocutaneous Melanosis: Report of Three Cases and Up-to-date Review

Efterpi Pavlidou, MD

Paediatric-Neurology Department, Ippokratio Hospital, Aristotle University of Thessaloniki, Greece

Christian Hagel, MD, PhD

Institute of Neuropathology, University Medical Center, Hamburg, Germany

Antigoni Papavasilliou, MD

Department of Neurology, Pendeli Children's Hospital, Athens, Greece

Sotiris Giouroukos, MD, PhD

Agia Sophia Hospital, University of Athens, Greece

Christos Panteliadis, MD, PhD

Paediatric-Neurology Department, Ippokratio Hospital, Aristotle University of Thessaloniki, Greece, cpantel{at}hol.gr

Neurocutaneous melanosis is a rare noninherited embryonic neuroectodermal dysplasia, which is observed sporadically and never affects the entire integument. The hallmark of neurocutaneous melanosis in the neonatal period is the presence of a large bilateral hairy dark nevus with satellite nevi over the trunk and neck. The diagnosis should be considered in neonates with large pigmented nevi and in those with more than 3 hairy dark nevi regardless of their size. Neonates with neurocutaneous melanosis are at risk of developing neurological problems. The most common neurological complications are hydrocephalus, seizures, cranial nerve dysfunction, and signs of spinal cord and root involvement. The authors report 3 cases of histologically confirmed neurocutaneous melanosis and describe the course of neurological symptoms and clinical findings including cognitive tests and neuroimaging. The case reports are complemented by an up-to date review on this clinical entity.

Key Words: neurocutaneous melanosis • nevi • literature review

Journal of Child Neurology, Vol. 23, No. 12, 1382-1391 (2008)
DOI: 10.1177/0883073808319069


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