1/46. Sturge--Weber syndrome: report of an unusual cutaneous distribution.This is an 11 month old girl who has been referred to our institute for a seizure disorder. Her physical examination showed angiomas on the left side of her face, and more extensive and prominent ones on her palms and soles. Her right face and arm were smaller than the left, associated with hypotonia and moderate weakness in the right forearm. Computerized tomography of head showed intracranial calcification in the left fronto-parietal region, and brain MRI, with gadolinium, revealed an extensive leptomeningeal angioma over the entire left hemisphere. This case was diagnosed as sturge-weber syndrome with unusual cutaneous manifestations.- - - - - - - - - - ranking = 1keywords = seizure (Clic here for more details about this article) |
2/46. Intracranial calcification mimicking the sturge-weber syndrome: a consequence of cerebral folic acid deficiency?Cerebral cortical calcification identical to that of the sturge-weber syndrome was observed in two children. In one child the calcification appeared after intrathecal administration of methotrexate and skull irradiation because of leukemia involving the central nervous system. In the other child, who had coeliac disease and epilepsy, the calcification appeared after treatment with anticonvulsants. This treatment was also contributing to the development of profound megaloblastic anemia. The unspecificity of the Sturge-Weber calcification is stressed and the hypothesis is put forward that the calcification may be secondary to folic acid deficiency interfering with the matabolism in the central nervous system.- - - - - - - - - - ranking = 0.76403525723056keywords = epilepsy (Clic here for more details about this article) |
3/46. 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 = 1keywords = seizure (Clic here for more details about this article) |
4/46. sturge-weber syndrome involved frontoparietal region without facial nevus.Classic sturge-weber syndrome is characterized by a facial nevus and hamartomatous lesion in the brain. Hamartomatous lesions are usually located in the ipsilateral occipital region of the facial nevus. The other lobes may be involved. A few cases of sturge-weber syndrome without facial nevus have been reported. A 9-month-old male was admitted with the complaint of afebrile seizures two times. We observed the third seizure, which was complex partial, in the clinic. There was no facial hemangioma. On computed tomography and magnetic resonance imaging of the brain we observed findings concordant with the angioma in the right frontoparietal region, although the occipital region was intact. This patient appears to be the first with sturge-weber syndrome without facial nevus, and with involvement of the frontoparietal region but not the occipital region. We do not know the embryologic basis of this association.- - - - - - - - - - ranking = 2keywords = seizure (Clic here for more details about this article) |
5/46. 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 = 5.2921057716917keywords = seizure, epilepsy (Clic here for more details about this article) |
6/46. Transient focal leptomeningeal enhancement in sturge-weber syndrome.The authors describe a 36-year-old man with sturge-weber syndrome who presented with focal seizures and subsequently developed a temporary post-ictal hemianopia. magnetic resonance imaging of the brain demonstrated focal leptomeningeal enhancement, which subsequently resolved.- - - - - - - - - - ranking = 1keywords = seizure (Clic here for more details about this article) |
7/46. The value of the brain scan and cerebral arteriogram in the sturge-weber syndrome.In a series of eight patients with the sturge-weber syndrome, the brain scan was shown to be the most accurate, non-invasive, diagnostic test, being abnormal in all eight patients. In four cases examined by cerebral arteriography, this study was also conspicuously abnormal. Some of the neurological disturbances occurring in these patients are more likely to be due to transient ischaemic attacks and cerebral infarctions than to epilepsy.- - - - - - - - - - ranking = 0.76403525723056keywords = epilepsy (Clic here for more details about this article) |
8/46. Epileptic monocular nystagmus.The authors describe a case of epileptic monocular nystagmus in a cognitively intact adult with normal vision. Focal seizures originated in the occipital lobe contralateral to the involved eye, and an associated structural lesion was thought to represent a forme fruste of sturge-weber syndrome. It is hypothesized that the seizure discharge either activated a cortical saccade region and caused simultaneous supranuclear inhibition of ipsilateral eye movement or triggered monocular eye movement commands.- - - - - - - - - - ranking = 2keywords = seizure (Clic here for more details about this article) |
9/46. Ictal magnetic source imaging as a localizing tool in partial epilepsy.OBJECTIVE: To determine the feasibility and usefulness of ictal magnetoencephalography (MEG) recordings in the presurgical evaluation of patients with epilepsy. methods: Twenty patients with frequent or predictable seizures were studied with the intent to capture seizures using a large array single-probe 37-channel or dual-probe 74-channel biomagnetometer. RESULTS: Successful ictal MEG recordings were made in 6 of 20 patients with neocortical epilepsy. In one other patient, a seizure was captured but movement artifact made MEG recordings impossible. As determined by invasive EEG recording and postsurgical outcome, ictal MEG provided localizing information that was superior to interictal MEG in three of the six patients. Localization of ictal onset by MEG was at least equivalent to invasive EEG in five of the six patients, and was superior in two patients as determined by postsurgical outcome. CONCLUSION: Larger studies are necessary to confirm that ictal MEG recordings in patients with frequent or easily provoked neocortical seizures can contribute localizing information equivalent or superior to invasive EEG recording.- - - - - - - - - - ranking = 8.5842115433834keywords = seizure, epilepsy (Clic here for more details about this article) |
10/46. 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 = 2keywords = seizure (Clic here for more details about this article) |
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