1/45. 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 = angiomatosis (Clic here for more details about this article) |
2/45. Bilateral optic neuropathy associated with diffuse cerebral angiomatosis in sturge-weber syndrome.Visual loss in patients with encephalotrigeminal angiomatosis or sturge-weber syndrome usually results from secondary glaucoma or from damage to the retrogeniculate pathways by the angiomatous lesions. Optic neuropathy has not been reported as a component of this syndrome. This report describes a patient who developed bilateral optic neuropathy with progressive visual loss associated with diffuse cerebral angiomatosis in the setting of sturge-weber syndrome.- - - - - - - - - - ranking = 6keywords = angiomatosis (Clic here for more details about this article) |
3/45. Phakomatosis pigmentovascularis with sturge-weber syndrome: a case report.We describe here a Turkish girl with pigmentovascularis type 2b, consisting of disseminated Mongolian-spot-like maculae and unilateral Sturge-Weber angiomatosis.- - - - - - - - - - ranking = 1keywords = angiomatosis (Clic here for more details about this article) |
4/45. angiodysplasia with osteohypertrophy affecting the oromaxillofacial area: clinical findings.The authors report a case of oro-facial port-wine stain angiomatosis with cutaneous and mucosal lesions localized in the first and the second branch surface distribution areas of the right trigeminal nerve in association with right upper and lower lip, cheek, and hemimaxillary true hypertrophy leading to a severe dento-skeletal malocclusion with openbite and facial asymmetry. The authors through a revision of the bibliography locate nosologically this case among the rare congenital angiodysplastic syndromes affecting the maxillo-facial district as the Sturge-Weber and the Klippel-Trenaunay syndromes. They debate also about etiopathogenetic, clinical, differential diagnosis, and therapeutic aspects concerning the treatment of patients affected by this kind of malformation.- - - - - - - - - - ranking = 1keywords = angiomatosis (Clic here for more details about this article) |
5/45. epilepsy surgery in bilateral sturge-weber syndrome.Two infants with severe drug refractory focal epilepsy caused by sturge-weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere. Successful surgical treatment with functional hemispherectomy was followed by good long-term seizure control in both patients. The dramatic seizure control was accompanied by markedly improved quality of life for the family and children. These cases indicate that the spectrum of children that may benefit from epilepsy surgery should not be viewed too restrictively, and subsets of children with localization related epilepsy caused by extensive lesions may be resective surgical candidates with a good seizure outcome prognosis.- - - - - - - - - - ranking = 1keywords = angiomatosis (Clic here for more details about this article) |
6/45. intracranial hypertension in Sturge-Weber/Klippel-Trenaunay-Weber overlap syndrome due to impairment of cerebral venous outflow.klippel-trenaunay-weber syndrome (KTWS) is a rare mesodermal phakomatosis characterized by (1) cutaneous haemangiomata (usually unilateral and involving an extremity) (2) venous varicosities and (3) osseous and soft tissue hypertrophy, also of the affected limb. sturge-weber syndrome (SWS), also a mesodermal phakamatosis, is characterized by meningofacial angiomatosis with cerebral calcification. Overlap between KTWS and SWS is recognized. We describe the case of a young woman with features of both KTWS and SWS who presented with symptoms of acute intracranial hypertension, including headache, vomiting and marked visual impairment. cerebral angiography revealed paucity of the superficial cortical veins overlying one of the cerebral hemispheres and centripetal venous drainage via small deep venous channels. Based upon this pattern of cerebral venous outflow, we postulate a mechanism to explain the acute episode of intracranial hypertension.- - - - - - - - - - ranking = 1keywords = angiomatosis (Clic here for more details about this article) |
7/45. sturge-weber syndrome.Encephalotrigeminal angiomatosis (sturge-weber syndrome) is described with a typical case history.- - - - - - - - - - ranking = 1keywords = angiomatosis (Clic here for more details about this article) |
8/45. 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 = 5keywords = angiomatosis (Clic here for more details about this article) |
9/45. Encephalofacial angiomatosis sparing the occipital lobe and without facial nevus: on the spectrum of sturge-weber syndrome variants?We report two cases of leptomeningeal angiomatosis in atypical frontoparietotemporal locations without an associated facial port-wine stain. Evidence of a leptomeningeal angioma was found in each when they were evaluated for headaches and seizures. The diagnosis of a leptomeningeal angioma was suggested by calcifications noted on computed tomographic scan of the head and confirmed with contrast-enhanced magnetic resonance images of the brain. We hypothesize that given the lack of occipital involvement with the angioma, and therefore the noncontiguous nature of this lesion with the developing upper facial ectoderm, the failure to develop a facial angioma would be expected. We found that the useof an anticonvulsant along with a migraine prophylactic medication appeared to have the greatest efficacy in these two cases, whereas anticonvulsants alone were less helpful. This diagnosis should be considered in any child presenting with seizures or complicated migraines and intracranial calcifications.- - - - - - - - - - ranking = 5keywords = angiomatosis (Clic here for more details about this article) |
10/45. Left-sided facial nevus with contralateral leptomeningeal angiomatosis in a child with sturge-weber syndrome: case report.sturge-weber syndrome is characterized by a facial vascular nevus associated with an ipsilateral leptomeningeal angioma. Variants of this classical presentation have been described in the literature, some of which have prognostic significance. We report a magnetic resonance imaging (MRI)-confirmed variant of a leptomeningeal angioma contralateral to the facial nevus. We describe one patient with sturge-weber syndrome who presented with a left-sided facial nevus, left eye glaucoma, episodes of left-sided weakness, and right-sided leptomeningeal angiomatosis by gadolinium-enhanced brain MRI. The literature regarding variants of sturge-weber syndrome and their prognosis is reviewed. The prognosis for this variant is likely similar to sturge-weber syndrome with an ipsilateral leptomeningeal angioma.- - - - - - - - - - ranking = 5keywords = angiomatosis (Clic here for more details about this article) |
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