The cutaneous angioma of Sturge-Weber syndrome.

نویسندگان

  • Sarah A Morrow
  • Craig Campbell
چکیده

A 16-month-old male presented with focal motor seizures with secondary generalization. On physical exam, a bilateral facial cutaneous angioma, sharply demarcated by the distribution of the ophthalmic and maxillary branches of the trigeminal nerve, was noted (Figure 1). Magnetic resonance imaging (MRI) demonstrated calcification and atrophy of the right frontal lobe as well as an associated pial angioma, consistent with the clinical suspicion of Surge-Weber syndrome (SWS) (Figure 2). However, no abnormality was seen in the right hemisphere, or in the parietal or occipital lobes. Further evaluation also revealed unilateral glaucoma on the right, but with bilateral diffuse choroidal hemangiomas. Surge-Weber syndrome is a non-hereditary neurocutaneous disorder characterized by leptomeningeal angiomatosis with an associated facial cutaneous angioma, also called a port-wine nevus. The abnormality is thought to be due to a malformation of the embryonic vascular plexus within the cephalic mesenchyme at five to eight weeks gestation, interfering with the development of vascular drainage.1 Due to poor drainage of the eye, face, leptomeninges and brain, angiomatosis develops as an alternate pathway for venous drainage,1 leading to multiple neurological and ophthalmologic complications, including seizures, headaches, stroke like episodes and glaucoma.

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Background: Sturge-Weber syndrome is one of the rare neurocutaneous disorders with frequency of approximately 1 per 50,000. Sturge-Weber syndrome consists of a constellation of symptoms and signs including a facial nevus (port wine stain), seizure and hemiparesis. In many cases it may associate with mental retardation. Case presentation: A 7- year old girl with mental retardation and a large fa...

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Convusions and homonymous hemianopsia as initial manifestations of Sturge-Weber syndrome in a 64-year-old male.

Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is the most frequent neurocutaneous syndrome, characterised by vascular malformations which manifest sporadically. The full presentation includes brain vascular malformations (pial or leptomeningeal angioma), cutaneous abnormalities (facial angioma) and ocular abnormalities (choroidal angioma). We present the case of a patient wi...

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Outcome of Sturge-Weber syndrome in 52 adults.

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder characterized by cutaneous facial angioma, leptomeningeal angioma associated with seizures and other neurologic complications including mental retardation, and glaucoma. Only limited information about long-term outcome, including the societal integration of adult patients, is available in the literature. Data on 52 adults with SWS, ages 1...

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عنوان ژورنال:
  • The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques

دوره 35 4  شماره 

صفحات  -

تاریخ انتشار 2008