Sturge–Weber syndrome or Sturge–Weber–Krabbe disease, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and. Sturge-Weber syndrome, or encephalotrigeminal angiomatosis, is a phakomatosis characterised by facial port wine stains and pial angiomas. It is part of a wide. Combined Sturge-Weber-Dimitri and Klippel-Trénaunay-Weber .. Liaras, H.: Un cas de syndrome de Klippel-Trénaunay avec angiomatose osseuse localisée.

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Central Nervous SystemPaediatrics. Retrieved 8 May Professionals Summary information Greekpdf Polskipdf Anesthesia guidelines Englishpdf. Its effect is independent of race, gender or age, and it has few to no side effects. Our website uses cookies to enhance your experience. Clinical description The facial capillary malformation classically referred to as angioma is a port-wine stain PWS that is generally present at birth and located on the forehead or upper eyelid on one or both sides of the face.

Sometimes, the PWS may also cover the maxillary and mandibular areas of the face and in some cases may extend to the trunk and limbs. Case 9 Case 9. Due to the high prevalence of epilepsy in children with SWS, the majority of patients will have an EEG obtained, even at a young age.

Read it at Google Books – Find it at Amazon. Diagnosis is confirmed by imaging techniques radiography, Deber scan or MRI with contrast showing, as the disease progresses, ipsilateral cerebral hemiatrophy, cortical calcifications delineating the cerebral gyri, and leptomeningeal angiomatosis.

Increased pressure within the eye can cause the eyeball to enlarge and bulge out of its socket buphthalmos. A study on Sturge-Weber syndrome: Case 4 Case 4. Less common aspects include an increased risk of growth hormone deficiency.

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Treatment for Angjomatose syndrome is symptomatic. The Sturge Weber Awareness Day is a collaboration with international Sturge Weber support groups to raise public and professional awareness of Sturge Weber syndrome around the world. Amer J Dis Child. A case of partial epilepsy, apparently due to a lesion of one of the vaso-motor centers of the brain.

The charity exists to support those affected by Sturge Weber syndrome, promote research into the condition and raise awareness of the condition amongst both public and professionals.

J Magn Reson Imaging.

Sign in to access your subscriptions Sign in to your personal account. Cerebral vascular malformations are also present. Case 6 Case 6. However, there has been a paucity of dermatologic writing on the Sturge-Weber syndrome.

Journal of Neurology and Psychopathology. See other articles in PMC that cite the published article. Categorical data were analyzed by Fisher exact test, medians were compared by a Mann-Whitney test, and means with a two-tailed t test assuming unequal variance.

Sturge–Weber syndrome – Wikipedia

In addition, these were routine 30 minute EEGs and may have also fluctuated even within the same day in these patients. D ICD – This type is the most common.

angiomatosse No difference was seen between scores 0 mean 2. The differential is a combination of that for multiple intracranial calcificationscerebral hemiatrophy and leptomeningeal enhancementand therefore includes:. Edit article Share article View revision history. As some children were webrr for facial port-wine stain and were later found not to have SWS, and some were unknown in regards to imaging these children were also excluded; therefore 44 had confirmed SWS by MRI and in total 81 EEGs were then available for analysis.

This score was obtained at clinic visit and is a composite of seizure frequency, hemiparesis, cognition, and vision subscores.

Two children were excluded due to lack of consent for research analysis. Brain surgery involving removing the portion of the brain that is affected by the disorder can be successful in controlling the seizures so that the patient has only a few seizures that are much less intense than pre-surgery.


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Sturge–Weber syndrome

This page was last edited on 12 Augustat Laser treatment may be used to lighten or remove the birthmark. Sturge-Weber syndrome was first described by Sturge infollowed sturgr Weber and Volland in who described the intracranial calcification. Top of the page – Article Zturge. Alternatively modern anticonvulsants or treatment with low-dose aspirin may currently produce some modification in the previously noted EEG findings.

When one side of the brain is affected and anticonvulsants prove ineffective, the standard treatment is neurosurgery to remove or disconnect the affected part of the brain hemispherectomy. Ophthalmological examination is also essential to identify and treat ocular involvement 4.

This EEG score, at least based on this study, should not at this time overall be used to guide medication management, predict prognosis, or suggest deterioration without clinical correlation. The symptoms can include glaucoma, cerebral blood flow abnormalities and headaches. Of these children, we obtained at Kennedy Krieger Institute a total of EEGs in 88 children for clinical reasons anigomatose as part of a research protocol; patient or parent consent was obtained to angiomqtose the EEGs and approval obtained from the Johns Hopkins Institutional Review Board.

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