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Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes pp 287–309Cite as

Sturge-Weber Syndrome

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Abstract

Sturge-Weber syndrome (SWS) (OMIM # 185300), also known as encephalofacial or encephalotrigeminal angiomatosis or meningofacial angiomatosis, is a (usually) sporadic congenital neurocutaneous disorder affecting the cephalic venous microvasculature. The hallmark anomaly is a capillary malformation affecting: (a) the brain and meninges with or without involvement of (b) the choroid and/or episclera or conjunctive and (c) the skin (the latter typically in the cranial nerve V1-V3 territory including the mouth, pharynx and nasal mucosa or elsewhere in the body) (Baselga 2004, Gorlin et al. 2001, Thomas-Sohl et al. 2004, Di Rocco and Tamburrini 2006). Many incomplete forms, lacking one or more features of this triad exist (Baselga 2004). Other clinical features associated with SWS are seizures, glaucoma, headache, transient stroke-like neurological deficits, and behavioural problems. Hemiparesis, hemiatrophy, and hemianopia may occur contralaterally to the cortical abnormality (Thomas-Sohl et al. 2004).

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Authors and Affiliations

  1. Paediatric Neurology Service, University Hospital La Paz, University of Madrid, Paseo de la Castellana, 261, 28046, Madrid, Spain (IPC)

    Ignacio Pascual-Castroviejo MD

  2. Department of Radiology, Vascular and Interventional Radiology, St. John West Shore Hospital, University Hospitals Case Medical Center, 29000 Center Ridge Road, 44145, Westlake, OHIO, USA (OK)

    Orhan Konez MD

  3. Section of Paediatric Neurosurgery, Department of Paediatrics, Catholic University of Sacred Heart, Largo Gemelli, 8, 00168, Rome, Italy (CDR)

    Concezio Di Rocco MD

  4. Institute of Neurological Science, National Research Council, Viale R. Margherita, 6, 95125, Catania, Italy (MR)

    Martino Ruggieri MD, PhD

  5. Institute of Neurological Science, National Research Council, Catania

    Martino Ruggieri MD, PhD

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  1. Institute of Neurological Science, National Research Council, Catania, Italy

    Martino Ruggieri

  2. University Hospital La Paz, Madrid, Spain

    Ignacio Pascual-Castroviejo

  3. Institute of Neurosurgery, Catholic University of the Sacred Heart, Rome, Italy

    Concezio Di Rocco

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Pascual-Castroviejo, I., Konez, O., Rocco, C.D., Ruggieri, M. (2008). Sturge-Weber Syndrome. In: Ruggieri, M., Pascual-Castroviejo, I., Di Rocco, C. (eds) Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-69500-5_10

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