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Textbook of Pediatric Neurosurgery pp 1–13Cite as

Sturge-Weber Syndrome

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Abstract

Sturge-Weber syndrome (SWS), also called encephalotrigeminal angiomatosis, is a rare, sporadic, neurocutaneous syndrome affecting the cephalic microvasculature. Its prevalence is actually estimated at 1 per 50,000 live births, although the identification of milder forms has recently led to an increase of this frequency estimate. Men and women are equally affected and there is no racial bias. Familial cases are rare; this aspect, together with the lack of clinical similarity in monozygotic twins, has suggested a somatic mutation as a possible mean of disease transmission. Differently from other neurocutaneous syndromes, such as von Hippel-Lindau, von Recklinghausen, and the Bourneville-Pringle disease, Sturge-Weber syndrome is not associated with intracranial neoplasms. However, in this syndrome an extensive capillary-venous malformation affecting one and occasionally both cerebral hemispheres may be associated with intractable epilepsy and progressive mental retardation, which may eventually require extensive neurosurgical procedures (lobectomy or hemispherectomy).

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Authors and Affiliations

  1. Pediatric Neurosurgery, Catholic University Medical School, Rome, Italy

    G. Tamburrini & M. Mohsen Amen

  2. Pediatric Neurosurgery, International Neuroscience Institute, Hannover, Germany

    C. Di Rocco

Authors
  1. G. Tamburrini
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  2. M. Mohsen Amen
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  3. C. Di Rocco
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Corresponding author

Correspondence to C. Di Rocco .

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. International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli, Catholic University Medical School, Rome, Italy

    Gianpiero Tamburrini

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Tamburrini, G., Mohsen Amen, M., Di Rocco, C. (2018). Sturge-Weber Syndrome. 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_44-2

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  • DOI: https://doi.org/10.1007/978-3-319-31512-6_44-2

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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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Chapter history

  1. Latest

    Sturge-Weber Syndrome
    Published:
    19 March 2018

    DOI: https://doi.org/10.1007/978-3-319-31512-6_44-2

  2. Original

    Sturge-Weber Syndrome
    Published:
    27 January 2018

    DOI: https://doi.org/10.1007/978-3-319-31512-6_44-1

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