Difference between revisions of "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:<ref name=pmid20137589/><ref name=pmid7747906>{{cite journal |author=Reich DS, Wiatrak BJ |title=Upper airway obstruction in Sturge-Weber and Klippel-Trenaunay-Weber syndromes |journal=Ann. Otol. Rhinol. Laryngol. |volume=104 |issue=5 |pages=364–8 |year=1995 |month=May |pmid=7747906 |doi= |url=}}</ref><ref>URL: [http://www.ncbi.nlm.nih.gov/omim/185300 http://www.ncbi.nlm.nih.gov/omim/185300]. Accessed on: 6 October 2010.</ref> | '''Sturge-Weber syndrome''', also known as '''encephalotrigeminal angiomatosis''', is a congenital thingy that is characterized by the triad:<ref name=pmid20137589/><ref name=pmid7747906>{{cite journal |author=Reich DS, Wiatrak BJ |title=Upper airway obstruction in Sturge-Weber and Klippel-Trenaunay-Weber syndromes |journal=Ann. Otol. Rhinol. Laryngol. |volume=104 |issue=5 |pages=364–8 |year=1995 |month=May |pmid=7747906 |doi= |url=}}</ref><ref>URL: [http://www.ncbi.nlm.nih.gov/omim/185300 http://www.ncbi.nlm.nih.gov/omim/185300]. Accessed on: 6 October 2010.</ref> | ||
#Trigeminal distribution unilateral capillary malformations (flame nevus). | #Trigeminal distribution unilateral capillary malformations (''flame nevus'' or ''port-wine stain''). | ||
#Leptomeningeal vascular malformations ( | #Leptomeningeal vascular malformations (angiomatosis). | ||
#Vascular malformations of the choroid plexus | **Results in intracranial calcifications. | ||
**Associated with neurologic signs. | |||
#Vascular malformations of the choroid plexus associated with glaucoma. | |||
Associations: | Associations: | ||
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==Prevalence== | ==Prevalence== | ||
*1 in 50 000 live births.<ref name=pmid20137589>{{cite journal |author=Zhou J, Li NY, Zhou XJ, Wang JD, Ma HH, Zhang RS |title=Sturge-Weber syndrome: a case report and review of literatures |journal=Chin. Med. J. |volume=123 |issue=1 |pages=117–21 |year=2010 |month=January |pmid=20137589 |doi= |url=http://www.cmj.org/Periodical/paperlist.asp?id=LW20101560192150671.&linkintype=pubmed}}</ref> | *1 in 50 000 live births.<ref name=pmid20137589>{{cite journal |author=Zhou J, Li NY, Zhou XJ, Wang JD, Ma HH, Zhang RS |title=Sturge-Weber syndrome: a case report and review of literatures |journal=Chin. Med. J. |volume=123 |issue=1 |pages=117–21 |year=2010 |month=January |pmid=20137589 |doi= |url=http://www.cmj.org/Periodical/paperlist.asp?id=LW20101560192150671.&linkintype=pubmed}}</ref> | ||
==See also== | ==See also== | ||
Revision as of 21:01, 6 October 2010
Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis, is a congenital thingy that is characterized by the triad:[1][2][3]
- Trigeminal distribution unilateral capillary malformations (flame nevus or port-wine stain).
- Leptomeningeal vascular malformations (angiomatosis).
- Results in intracranial calcifications.
- Associated with neurologic signs.
- 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
- ↑ 1.0 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.
- ↑ 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.
- ↑ URL: http://www.ncbi.nlm.nih.gov/omim/185300. Accessed on: 6 October 2010.