1/17. Sturge Weber syndrome with intraoral manifestations. A case report.A 15 Year old female patient with Sturge Weber Syndrome is presented. This neurocutaneous syndrome consists of angiomatosis of the skin and mucosa as well as the leptomeninges. This case report describes the classic presentation of the syndrome, emphasizing the oral manifestations. The radiographic and CT scan show the typical "tram line" intracranial calcifications. This case report presents a typical case of Sturge Weber Syndrome. It gives the radiological and CT scan findings and the important role played by them in the diagnosis of this syndrome. Emphasis is given to the differentiation of diphenylhydantoin induced gingival hyperplasia from the angiomatous enlargement of the gingiva before any treatment is planned.- - - - - - - - - - ranking = 1keywords = neurocutaneous syndrome, neurocutaneous (Clic here for more details about this article) |
2/17. Double phakomatosis.In the phakomatoses--von Recklinghausen's disease, the sturge-weber syndrome, tuberous sclerosis, and von hippel-lindau disease--pathologic changes in the eye are often evident. Most cases are described as a single entity. The following is a case report of concomitant sturge-weber syndrome and von Recklinghausen's disease in which both conditions involved the eye.- - - - - - - - - - ranking = 4.3581806560256keywords = phakomatoses (Clic here for more details about this article) |
3/17. sturge-weber syndrome: a rare cause of gastrointestinal hemorrhage.A case of sturge-weber syndrome (SWS) with gastrointestinal hemorrhage is presented. SWS is a neurocutaneous disorder characterized by cutaneous facial angioma leptomeningeal angioma with seizures and other neurologic complications. Associated anomalies beyond the encephalofacial territory are very rare. The patient presented repeated bleeds from extensive gastric varices of the fundus secondary to a splenic venous malformation. This is the first report on this association to our knowledge.- - - - - - - - - - ranking = 0.20426708783292keywords = neurocutaneous (Clic here for more details about this article) |
4/17. Hypomelanosis of Ito with sturge-weber syndrome-like leptomeningeal angiomatosis.The term hypomelanosis of Ito (HI) is applied to individuals with skin hypopigmentation following the lines of Blaschko (type 1a of patterns indicative of somatic mosaicism as defined by Happle). Even though originally described as a purely cutaneous disease, subsequent reports of HI have included a 30-94% association with multiple extracutaneous manifestations. The frequency of extracutaneous associations has led many authors to consider HI to be neurocutaneous disorder. We report a male infant with cutaneous hypomelanosis along the lines of Blaschko distributed on the left half of the body who developed status epilepticus. neuroimaging studies disclosed an angiomatous enlargement of the right choroid plexus and a gyral pattern of cortical and subcortical calcification in the right occipital region. Thus a diagnosis could be made of HI and associated sturge-weber syndrome-like leptomeningeal angiomatosis. This previously unreported association lends further support to the consideration of hypomelanosis of Ito as a marker of somatic mosaicism with frequently associated neurologic abnormalities. A relationship between HI and sturge-weber syndrome, two neuroectodermal disorders with a genetic mosaicism basis, might be possible due to nonallelic twin-spotting which in the embryologic period would define an abnormal development of neural, vascular, and cutaneous structures.- - - - - - - - - - ranking = 0.20426708783292keywords = neurocutaneous (Clic here for more details about this article) |
5/17. Klippel-Trenaunay syndrome and sturge-weber syndrome: variations on a theme?We describe a patient with a complex neurocutaneous syndrome of congenital vascular malformations, abnormalities of brain and bones, and soft tissue hypertrophy of one leg. According to eponymous classification schemes, the patient can be assigned to two different clinical entities. Using the lethal gene theory it is possible to unify these different syndromes and to explain the overlap and diversity of these congenital vascular syndromes. We argue that it is better to describe such vascular malformation syndromes in anatomical/histological or functional terms and map the extent of the disease, rather than name it according to the eponymous classification.- - - - - - - - - - ranking = 1keywords = neurocutaneous syndrome, neurocutaneous (Clic here for more details about this article) |
6/17. Leptomeningeal angiomatosis with infantile spasms.We describe a 7-month-old female with leptomeningeal angiomatosis who developed infantile spasms. She did not manifest facial nevus or ocular choroidal angioma. Leptomeningeal angiomatosis is characterized by venous angiomas of leptomeninges and usually accompanied by facial nevus, a condition known as sturge-weber syndrome. In sturge-weber syndrome, leptomeningeal angiomas can cause infantile spasms but much less frequently than in other neurocutaneous syndromes, such as tuberous sclerosis. This patient is the first reported case of leptomeningeal angiomatosis without facial nevus who developed infantile spasms. Leptomeningeal angiomas should be taken into consideration as a cause of infantile spasms, even in the absence of facial nevus. We suggest that this case is clinically within the spectrum of sturge-weber syndrome, and that the embryologic origin of this case is similar to that of sturge-weber syndrome.- - - - - - - - - - ranking = 1keywords = neurocutaneous syndrome, neurocutaneous (Clic here for more details about this article) |
7/17. Incomplete monosymptomatic leptomeningeal angiomatosis.Phakomatoses or neurocutaneous syndromes are an important cause of seizures in the pediatric age group. The sturge-weber syndrome may affect the eye, skin and brain at different times. The skin lesions need not always manifest. We report a case of isolated affection of the central nervous system in a case of sturge-weber syndrome in the absence of ocular or cutaneous manifestations. Our case qualifies to be called incomplete monosymptomatic leptomeningeal angiomatosis.- - - - - - - - - - ranking = 1keywords = neurocutaneous syndrome, neurocutaneous (Clic here for more details about this article) |
8/17. Sturge Weber syndrome.A variant of hemangioma and an uncommon congenital condition, the Sturge Weber syndrome (SWS), also called encephalo trigeminal angiomatosis, is a neurocutaneous disorder with angiomas involving the skin of the face (cutaneous angioma) and pia arachnoid (leptomeningeal angioma). It occurs typically in the ophthalmic and maxillary distributions of the trigeminal nerve (1,2). Here we present a case of a 14 year old girl.- - - - - - - - - - ranking = 0.20426708783292keywords = neurocutaneous (Clic here for more details about this article) |
9/17. sturge-weber syndrome: deep venous occlusion and the radiologic spectrum.sturge-weber syndrome is a neurocutaneous syndrome with a facial port-wine nevus and neurologic features, typically including seizures and hemiparesis. glaucoma may also occur. MRI features include leptomeningeal angiomatosis, cortical and pial calcifications, and angiomatous change of the choroid plexus. We reviewed a subset of patients with sturge-weber syndrome with the rare finding of deep venous occlusion, and present such a case, unusual by comparison to previously reported cases of sturge-weber syndrome with deep venous occlusion. Six previously reported cases were reviewed. All cases presented with seizures; five of six had evidence of leptomeningeal angiomatosis; half had cerebral hemiatrophy. This report presents a unique case lacking clinical seizures, but with a port-wine stain and congenital glaucoma. This patient lacked the radiologic findings of leptomeningeal angiomatosis and hemicerebral atrophy, but demonstrated deep venous occlusion with frontal venous collaterals. There is a wide spectrum of findings in sturge-weber syndrome. The lack of seizures and angiomatosis in this case are likely "true-true" and related. The case illustrates the unusual finding of deep venous occlusion in sturge-weber syndrome occurring without leptomeningeal angiomatosis. Additionally, it demonstrates that although the initial evaluation is normal, patients may later manifest clinical characteristics of sturge-weber syndrome.- - - - - - - - - - ranking = 1keywords = neurocutaneous syndrome, neurocutaneous (Clic here for more details about this article) |
10/17. choroid plexus hemangioma with port-wine nevus of the face: relationship to sturge-weber syndrome--case report.The authors describe a case of choroid plexus hemangioma in a 49-year-old male. Computed tomographic scan showed an isodense mass at the trigone of the right lateral ventricle with homogeneous enhancement. He also displayed a port-wine nevus on the ipsilateral side of the face. At operation, the tumor was found not to adhere to the lateral ventricular wall but to be connected to the choroid plexus, and was colored similarly to the facial nevus. Histological examination showed a capillary hemangioma with many crowded capillaries. This case was not included in the category of sturge-weber syndrome but is thought to be closely related, considering the syndrome from the viewpoint of generalized neurocutaneous hemangiomatosis.- - - - - - - - - - ranking = 0.20426708783292keywords = neurocutaneous (Clic here for more details about this article) |
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