Sturge-Weber syndrome

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Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis, is a congenital thingy that is characterized by the triad:[1][2][3]

  1. Trigeminal distribution unilateral capillary malformations (flame nevus or port-wine stain).
  2. Leptomeningeal vascular malformations (angiomatosis).
    • Results in intracranial calcifications.
  3. Vascular malformations of the choroid plexus associated with glaucoma.

Associations:

  • Intractable seizures.
  • Mental retardation.
  • Recurrent stroke-like episodes.

Prevalence

  • 1 in 50 000 live births.[1]

See also

References

  1. 1.0 1.1 Zhou J, Li NY, Zhou XJ, Wang JD, Ma HH, Zhang RS (January 2010). "Sturge-Weber syndrome: a case report and review of literatures". Chin. Med. J. 123 (1): 117–21. PMID 20137589. http://www.cmj.org/Periodical/paperlist.asp?id=LW20101560192150671.&linkintype=pubmed.
  2. Reich DS, Wiatrak BJ (May 1995). "Upper airway obstruction in Sturge-Weber and Klippel-Trenaunay-Weber syndromes". Ann. Otol. Rhinol. Laryngol. 104 (5): 364–8. PMID 7747906.
  3. URL: http://www.ncbi.nlm.nih.gov/omim/185300. Accessed on: 6 October 2010.