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      Neurocutaneous melanocytosis.

      Handbook of clinical neurology

      therapy, history, diagnosis, complications, Neurocutaneous Syndromes, Melanosis, Humans, History, 19th Century, Dandy-Walker Syndrome, pathology, Brain

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          Abstract

          Neurocutaneous melanosis or neurocutaneous melanocytosis is a rare sporadic congenital disorder characterized by the presence of giant and/or multiple satellite congenital melanocytic nevi in the skin and benign melanocytic pigmentation of the leptomeninges. These two defining features were recognized more than a century ago. A third characteristic feature is proliferative nodules arising from giant nevi. The etiology is unknown, but neurocutaneous melanosis is considered a developmental disorder of melanocyte precursors from neural crest. The distinctive unique distribution of the congenital giant nevi that gives a "garment" appearance is also an expression of the neural crest. The neurological manifestations often appear in infancy.The special association of neurocutaneous melanosis with Dandy-Walker malformation complex may be explained by a common pathogenesis. Mortality in infancy and childhood is high. Copyright © 2013 Elsevier B.V. All rights reserved.

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          Journal
          23622187
          10.1016/B978-0-444-52891-9.00042-7

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