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Cerebrocutaneous Melanosis

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Textbook of Pediatric Neurosurgery

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Abstract

Neurocutaneous melanosis (NCM) is a rare phakomatosis with focal or diffuse proliferation of melanin-producing cells in both the skin and the leptomeninges. Two-thirds of the affected subjects present giant congenital melanocytic nevi and the remaining third numerous melanocytic nevi in the absence of single giant skin lesions. Though normal neurological development can be observed in the first months after birth, most of the patients present neurological manifestations early in life secondary to intracranial hemorrhages, accumulations of melanocytic cells in various districts of the intracranial spaces, impairment of cerebrospinal fluid circulation leading to acute hydrocephalus, and subdural fluid collections. However, malignant transformation of the melanocytes is thought to account for the early demise which characterizes the syndrome. Even in the absence of malignancy, the overall prognosis of NCM is quite poor due to the persisting unavailability of an effective treatment.

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Author information

Authors and Affiliations

  1. Neurosurgical Department, Helios Klinik Hildesheim, Senator-Braunallee 33, 31135, Hildesheim, Germany

    Wolf Lüdemann

  2. International Neuroscience Institute, Rudolf Pichlmayrstr. 4, 30625, Hannover, Germany

    Concezio Di Rocco

Authors
  1. Wolf Lüdemann
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  2. Concezio Di Rocco
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Corresponding author

Correspondence to Wolf Lüdemann .

Editor information

Editors and Affiliations

  1. International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Oakland Medical Center, Oakland, California, USA

    Dachling Pang

  3. University of Toronto Hospital for Sick Children, Toronto, Ontario, Canada

    James T. Rutka

Section Editor information

  1. Director of Pediatric Neurosurgery, International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Pediatric Neurosurgery, Institute of Neurosurgery, Catholic University Medical School, Largo “A. Gemelli”,8, 00168, Rome, Italy

    Gianpiero Tamburrini

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© 2017 Springer International Publishing AG

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Lüdemann, W., Di Rocco, C. (2017). Cerebrocutaneous Melanosis. In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-31512-6_48-1

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  • DOI: https://doi.org/10.1007/978-3-319-31512-6_48-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-31512-6

  • Online ISBN: 978-3-319-31512-6

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

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