The cutaneous angioma of Sturge-Weber syndrome.
نویسندگان
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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Sturge-Weber syndrome is characterized by the association of a trigeminal port-wine facial angioma with a pial venular angioma, usually ipsilateral to the facial nevus. However, a bilateral pial angioma may coexist with a unilateral facial nevus (1 ), and pial angioma may be present without any facial nevus (2] . The pial angioma is responsible for partial seizures, hemiplegia, and mental retar...
متن کاملConvusions 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...
متن کاملA case of acute angle-closure glaucoma secondary to posterior scleritis in patient with Sturge-Weber syndrome.
BACKGROUND Sturge-Weber syndrome has been known to be frequently associated with facial cutaneous angioma and ipsilateral glaucoma. However, as far as we know, no cases accompanied by acute angle-closure glaucoma have been reported in patients with Sturge-Weber syndrome. CASE A 14-year-old boy with unilateral acute angle-closure glaucoma secondary to posterior scleritis associated with Sturge...
متن کاملOutcome 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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ورودعنوان ژورنال:
- The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
دوره 35 4 شماره
صفحات -
تاریخ انتشار 2008