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Neurocutaneous Syndromes (Phakomatoses)

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Computed Tomography of Congenital Brain Malformations

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Abstract

This group of inherited disorders is characterized primarily by skin and central nervous system (CNS) lesions. They are also known as phakomatoses, in reference to the associated skin lesions, a relationship that is explained by the common origin of ectoderm and neuro-ectoderm. Visceral lesions (mesodermal dysplasia) also may be present. These disorders are autosomally dominant and show a great propensity to develop neoplasia.

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Authors and Affiliations

  1. Section of Neuroradiology, Yale University School of Medicine, New Haven, CT, USA

    Mohammad Sarwar M.D. (Associate Professor of Diagnostic Radiology)

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  1. Mohammad Sarwar M.D.
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© 1985 Warren H. Green, Inc.

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Sarwar, M. (1985). Neurocutaneous Syndromes (Phakomatoses). In: Computed Tomography of Congenital Brain Malformations. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-2571-0_8

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  • DOI: https://doi.org/10.1007/978-1-4613-2571-0_8

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4612-9614-0

  • Online ISBN: 978-1-4613-2571-0

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