Neurocutaneous Disorders

  • Gerald S. Golden
Part of the Topics in Pediatrics book series (TIPE)


A number of heterogeneous conditions characterized by abnormalities of the skin and central nervous system are traditionally classified as the neurocutaneous disorders, or phakomatoses. These are distinct entities, however, with no genetic overlap. The occurrence of two of these conditions in the same individual or family probably represents the workings of chance.


Hereditary Hemorrhagic Telangiectasia Acoustic Neuroma Internal Auditory Canal Intracranial Calcification Incontinentia Pigmenti 
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    Kanter, W. R., Eldridge, R., Fabricant, R., Allen, J. C., and Koerber, T., 1981, Central neurofibromatosis with bilateral acoustic neuroma: Genetic, clinical and biochemical distinctions from peripheral neurofibromatosis, Neurology 30: 851–859.Google Scholar
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    Enjolras, O., Riche, M. C., and Merland, J. J., 1985, Facial port-wine stains and Sturge—Weber syndrome, Pediatrics 76: 48–51.PubMedGoogle Scholar
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    Avrahami, E., Harel, S., Jurgenson, U., and Cohn, D., 1985, Computed tomographic demonstration of brain changes in incontinentia pigmenti, Am. J. Dis. Child. 139: 372–374.PubMedGoogle Scholar

Additional Reading

  1. Berg, B. O., 1982, Neurocutaneous syndromes, in: The Practice of Pediatric Neurology ( K. S. Swaiman and F. S. Wright, eds.), C.V. Mosby, St. Louis, pp. 914–934.Google Scholar
  2. Rapin, I., and Chalhub, E. G., 1982, Phakomatoses and other neurocutaneous syndromes, in: Pediatrics, 17th ed. ( A. M. Rudolph and J. I. E. Hoffman, eds.), Appleton-Century-Crofts, Norwalk, CT, pp. 1762–1766.Google Scholar

Copyright information

© Plenum Publishing Corporation 1987

Authors and Affiliations

  • Gerald S. Golden
    • 1
  1. 1.Department of Neurology, The Health Science CenterUniversity of Tennessee, MemphisMemphisUSA

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