Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between July 1990 and October 1994 26 patients were operated on for 26 intracranial and 2 intraorbital cavernous hemangiomas. We found seizures in 62% of our patients, focal neurological deficits and unspecific complains (like headache or dizziness) in 19% each. The average follow-up period was 12 months, 24 patients could be included in this study. 12/14 patients of the seizure group improved, 10/14 reported a complete relieve of their epilepsy. All 5 patients with focal deficits improved, among them 2 with no residual deficit. Finally 3/5 patients with unspecific complains improved, 2/5 remained unchanged. The functional morbidity was 4% (one slight aphasic syndrome), we did not have any mortality. Therefore we conclude that a neurosurgical treatment is indicated in any case of cavernous hemangioma with focal deficits or intractable epilepsy. The operation should also be considered in patients with supressed seizures by anti-convulsants, if the malformation is not located in an eloquent area. Due to the risk of spontaneous bleeding (comparable to incidental aneurysms), the indication for a neurosurgical treatment mainly depends on the location of the cavernoma in cases of its accidental discovery.
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PMID:[Cavernoma. Indications for surgical removal and outcome]. 868 8

We described a 34-year-old woman with a right frontal cavernous angioma who presented with complex partial status epilepticus (CPSE). In the first CPSE, complex partial seizure with complete unresponsiveness and tonic motor manifestation on her left arm occurred repeatedly at short intervals. Her consciousness was mildly disturbed between the seizures. An hour after the intra-venous administration of diazepam, her consciousness returned to normal. In the second CPSE, no motor manifestation was found, but complete unresponsiveness of short duration accompanied with stare and moderately impaired consciousness continued to occur. We emphasize the importance of not missing this rare non-convulsive epileptic status, especially when it does not present motor manifestations.
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PMID:[Complex partial status epilepticus in a patient with a frontal cavernous angiomas]. 885 56

Familial occurrence of intracerebral cavernous angioma has been rarely reported. We report two histologically verified cases of cavernous angioma among brothers and review relevant cases in the literature. Case 1 is that of a 3-year-old boy who suffered front acute onset of headache, vomiting, and tonic-clonic type seizure. CT revealed a well-demarcated tumor with partial hemorrhage in the left frontal lobe which was strongly enhanced with contrast Medium. Complete excision was carried out and the patient had a satisfactory clinical course and was able to be followed up for 13 years after the Surgery. Case 2 is that of a 17-year-old boy who was the elder brother of case 1 and presented with gradually increasing episodes of a psychomotor seizure which started at the age of 16. CT and MRI revealed a well-demarcated tumor in the left subcortical temporal lobe and an asymptomatic small calcified lesion in the left subcortical parietal lobe. The temporal tumor was totally excised and histologically diagnosed as cavernous angioma. The seizures gradually decreased and eventually disappeared one year after the surgery. This report reviews 13 previously reported cases, and surgical indication for asymptomatic cases.
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PMID:[Familial occurrence of intracerebral cavernous angioma: report of cases in brothers]. 892 18

The prognosis of Sturge-Weber syndrome (SWS) is partly related to early occurrence of seizures but the diagnosis of this phakomatosis may be difficult during the 1st year of life. We have performed a retrospective study of seven patients with confirmed SWS (age 7 days to 3 months). None of the patients was asymptomatic at the time of the study. They all underwent MRI (T1 and T2 sequences) and single photon emission computed tomography (SPECT) at the same time. Regional cerebral blood flow was measured using xenon-133. In all cases, myelination appeared to be accelerated in the areas underlying the leptomeningeal angioma on both MRI sequences. In five cases, SPECT showed hyperperfusion in the damaged hemisphere. In one case, the SPECT was symmetrical and in another it showed hypoperfusion in the damaged hemisphere which was already atrophied. These data suggest that the accelerated myelination is not related to ischemia but to transient hyperperfusion. This MRI pattern can be helpful for the early diagnosis of SWS, which is of utmost importance for preventive antiepileptic treatment.
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PMID:Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations. 892 71

Sturge-Weber syndrome is a disorder characterized by ipsilateral cavernous hemangioma of the face, uvea, and brain in patients who may present with an enlarged eye, exudative retinal detachment, glaucoma, and seizures. This report presents the clinicopathologic findings of an otherwise healthy infant with ipsilateral arteriovenous and capillary hemangiomas of the face and uveal tract, microphthalmos, iris heterochromia, hypotony, and absence of central nervous system involvement. The association of an arteriovenous-capillary angioma of the ocular adnexa and ipsilateral uveal tract is a syndrome that is distinct from Sturge-Weber syndrome.
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PMID:Ipsilateral facial and uveal arteriovenous and capillary angioma, microphthalmos, heterochromia of the iris, and hypotony: an oculocutaneous syndrome simulating Sturge-Weber syndrome. 898 98

In a consecutive series of 11 patients with a history of medically refractory temporal lobe epilepsy, we studied the appearance of ictal patterns detected by epidural screw electrodes (ESE) placed in an effort to obviate depth electrode recording. All had prior scalp-sphenoidal monitoring with significant obscuration of ictal EEG by movement and muscle artifact. Six to 10 ESE were placed at bilateral temporal locations of the 10% system. Ictal findings in 4-15 partial seizures from 8 patients with mesiobasal (MBTLE) and 3 patients with neocortical (NCTLE) epilepsy were correlated with imaging and histology. Five of 8 patients with MBTLE had discrete high frequency discharges at onset, evolving within 10 s to focal theta at ESE, before appearing at scalp electrodes. All had Class IA outcome with mesial temporal sclerosis (MTS). Three of 8 patients with MBTLE and MTS had less discrete onsets, with Class 1A, 1B and 3A outcomes. Two patients with NCTLE had lesions; a grade II oligoastrocytoma with rhythmic delta at ESE and Class 4 outcome, and an anterior temporal cavernous angioma with variable onsets, some of which were high frequency, with a Class 1A outcome. The third NCTLE patient demonstrated focal theta as an ictal pattern, had no lesion and a Class 1A outcome. Ictal onsets may be well localized in selected patients by ESE. A majority of patients with MTS display well defined high frequency ictal discharges.
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PMID:Ictal patterns in temporal lobe epilepsy recorded by epidural screw electrodes. 912 71

Modern treatment of childhood acute lymphoblastic leukaemia (ALL) has dramatically improved the prognosis for children with this disease. Therapeutic approaches consist of multimodal chemotherapy and radiotherapy with significant long-term side-effects. We report on 4 children out of a group of 120 newly diagnosed patients with ALL, who survived the disease for more than 2 years and developed a cerebral haemorrhage after chemotherapy and fractionated cranial irradiation. Following a period of 2-12 years the four children presented with acute neurological signs and symptoms. i.e. seizures, ataxia and hemiparesis. CT and MRI revealed intracerebral mass lesions, interpreted as haemorrhage. After neurosurgery the patients neurological state improved. Histological examination confirmed the suspected diagnosis of bleeding cavernous haemangioma or capillary telangiectases. There are two possibilities to explain these rare alterations: they may be pre-existent to the disease and therapy or they may be caused by irradiation. CONCLUSION Acute neurological symptoms in patients treated for ALL may be caused by spontaneous cerebral haemorrhaging of cavernous haemangiomas or capillary telangiectases induced by chemotherapy and/or radiotherapy.
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PMID:Cerebral haemorrhage in long-term survivors of childhood acute lymphoblastic leukaemia. 917 78

Cavernous angioma is a vascular malformation that affect 0.5 to 0.7% of the population making up 8 to 15% of cerebrovascular malformations. It is the second vascular malformation in frequency of the central nervous system, supplanted only by classic arteriovenous malformation. It may occur in two forms: a sporadic form characterized by isolated lesions: and a familial form characterized by multiple lesions with an autosomal dominant mode of inheritance with high penetrance and varied expressivity in the proportion M1:F1. Symptoms related to cavernous angioma are seizures, headache or progressive neurologic deficit. The authors present a Chinese family with familial cavernous angioma. Manifestations of the disease occurred in three generations affecting only females. Clinical, neuroimage, pathological, natural course and genetical aspects of the disease are discussed.
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PMID:[Familial cavernous angioma. Report in 3 generations]. 920 48

We report the case of a patient with progressive seizures caused by a cavernous angioma in association with a venous angioma in the right parietal lobe. The radiological findings, the pathogenesis of this association and the importance of surgical treatment of a cavernous angioma with conservative treatment of a venous angioma are discussed.
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PMID:The association of cavernous and venous angioma. Case report and review of the literature. 933 36

We present two patients with unilateral occipital gyriform calcification and seizures. Gyriform or serpentine calcification as revealed by computed tomography (CT) scan is rare and is a characteristic finding of Sturge-Weber syndrome (SWS) and celiac disease (CD). These patients had neither the facial nevus flammeus or neurological deficits characteristic of SWS, nor the gastrointestinal symptoms characteristic of CD. CD is often accompanied by cerebral occipital calcification indistinguishable from that of SWS. We demonstrate the presence of cerebral leptomeningeal angiomatosis (LA) by Gadolinium-DTPA-enhanced magnetic resonance imaging (MRI) but could not detect LA by either CT scanning or angiography. It has been reported that contrast-enhanced MRI is useful to detect LA in SWS. However, we found no reports of enhanced MRI in patients with SWS without facial angioma. If future studies can demonstrate the absence of cortical enhancement by contrast-enhanced MRI in CD with cerebral calcifications, enhanced MRI would become an important tool for differentiating CD from SWS.
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PMID:Enhanced magnetic resonance imaging of leptomeningeal angiomatosis. 939 Jul 6


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