Name = PAGENAME
DiseasesDB = 12572
ICD10 = ICD10|Q|85|8|q|80
ICD9 = ICD9|759.6
OMIM = 185300
eMedicineSubj = neuro
eMedicineTopic = 356
MeshID = D013341
Sturge-Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare
congenitalneurological and skindisorder. It is one of the phakomatoses, and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is caused by an arteriovenous malformationthat occurs in the cerebrumof the brain on the same side as the physical signs described above. Normally, only one side of the head is affected.
It is an embryonal developmental anomaly resulting from errors in
mesodermal and ectodermal development. Unlike other neurocutaneous disorders (phakomatoses), Sturge-Weber does not have a hereditary tendency but occurs sporadically.
Sturge-Weber syndrome is indicated at birth by
seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid of one side of the face. The birthmark can vary in color from light pink to deep purple, and is caused by an overabundance of capillaries around the ophthalmic branch of the trigeminal nerve, just beneath the surface of the face. There is also malformation of blood vessels in the pia materoverlying the brain, on the same side of the head as the birthmark. This causes calcificationof tissue and loss of nerve cells in the cerebral cortex. Neurological symptoms include seizures that begin in infancy and may worsen with age. Convulsions usually happen on the side of the body opposite the birthmark, and vary in severity. There may be muscle weakness on the same side. Some children will have developmental delays and mental retardation; most will have glaucoma(increased pressure within the eye), which can be present at birth or develop later. Increased pressure within the eye can cause the eyeball to enlarge and bulge out of its socket ( buphthalmos). Sturge-Weber syndrome rarely affects other body organs.
Treatment for Sturge-Weber syndrome is symptomatic. Laser treatment may be used to lighten or remove the birthmark.
Anticonvulsantmedications may be used to control seizures. Doctors recommend yearly monitoring for glaucoma, and surgery may be performed on more serious cases. Physical therapyshould be considered for infants and children with muscle weakness. Educational therapy is often prescribed for those with mental retardation or developmental delays, but there is no complete treatment for the delays.Brain surgery involving removing the portion of the brain that is affected by the disorder can be successful in controlling the seizures experienced so that the patient has only a few seizures that are much less intense than pre-surgery.
Although it is possible for the birthmark and atrophy in the cerebral cortex to be present without symptoms, most infants will develop convulsive seizures during their first year of life. There is a greater likelihood of intellectual impairment when seizures start before the age of 2 and are resistant to treatment.
It is named for
William Allen Sturgeand Frederick Parkes Weber. [WhoNamedIt|synd|1764] [W. A. Sturge. A case of partial epilepsy, apparently due to a lesion of one of the vasomotor centres of the brain. Transactions of the Clinical Society of London, 1879, 12: 162. ] [F. P. Weber. Right-sided hemi-hypertrophy resulting from right-sided congenital spastic hemiplegia, with a morbid condition of the left side of the brain, revealed by radiograms. Journal of Neurology and Psychopathology, London, 1922, 3: 134-139. ]
* [http://www.seattlechildrens.org/our_services/clinical_services/vascular_anomalies/sturge_weber.asp Information about Sturge-Weber Syndrome] from Children's Hospital, Seattle.
* [http://swscommunity.org/ Sturge-Weber Syndrome Community]
* [http://www.sturge-weber.org/ Sturge-Weber foundation]
* [http://www.pgcfa.org/med-news/med-news-19.htm Sturge-Weber Syndrome Research Project, PGCFA Medical News]
* [http://www.nature.com/bdj/journal/v195/n1/fig_tab/4810275f2.html Face] at nature.com
* [http://adam.about.com/encyclopedia/2536.htm Legs] at
Wikimedia Foundation. 2010.
Look at other dictionaries:
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