Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Neurocutaneous melanosis


Other Names for this Disease
  • Melanosis, neurocutaneous
  • Neurocutaneous melanosis syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview


Neurocutaneous melanosis (NCM) is a rare, non-inherited condition of the central nervous system. It is characterized by melanocytic nevi in both the skin and the brain. Two-thirds of people with NCM have giant congenital melanocytic nevi, and the remaining one-third have numerous lesions but no giant lesions. Most patients present with neurological features early in life, which can be secondary to intracranial hemorrhages (bleeding in the brain), impairment of cerebrospinal fluid circulation (fluid around the brain and spinal cord), and/or malignant transformation of the melanocytes.[1]

The prognosis of patients with symptomatic neurocutaneous melanosis is extremely poor, even in the absence of malignancy. Chemotherapy has been ineffective in the few patients in whom it has been tried.[1]

Last updated: 4/18/2011

References

  1. Di Rocco F, Sabatino G et al. Neurocutaneous melanosis. Childs Nerv Syst. 2004; http://www.ncbi.nlm.nih.gov/pubmed/14576958. Accessed 4/18/2011.
Your Questions Answered
by the Genetic and Rare Diseases Information Center

Please contact us with your questions about Neurocutaneous melanosis. We will answer your question and update these pages with new resources and information.
On this page

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Neurocutaneous melanosis. Click on the link to view a sample search on this topic.