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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gangliogliomas, dysembryoplastic neuroepithelial tumors (DNT) and glioneuronal malformations are frequently encountered in patients with pharmacoresistant focal epilepsies. In order to characterize the neurochemical profile of these neoplastic and malformative glioneuronal lesions, we have examined the presence of the alpha 1 subunit of the GABAA receptor, the N-methyl-D-aspartate receptor subunit 1 (NR1), glutamate decarboxylase, tyrosine hydroxylase, somatostatin, parvalbumin, and calretinin in 60 gangliogliomas, 11 DNT, 10 tuberous sclerosis-like lesions and 17 non-tuberous sclerosis-like glioneuronal malformations. All DNT and tuberous sclerosis-like lesions, 59 gangliogliomas (98%), and 13 non-tuberous sclerosis-like hamartias (76%) were positive for at least one of the markers. Despite a great variation between and within the different entities, the neurochemical profile was generally reminiscent of normal neocortex: glutamate decarboxylase, GABAA receptor and NR1 which are common in neocortical neurons were present in the great majority of the lesions and often showed high labeling indices. There were three tuberous sclerosis-like lesions (30%) that contained both NR1 and glutamate decarboxylase immunoreactive giant cells in addition to well-differentiated ganglion cells. This supports the idea that at least some of these giant cells are of neuronal origin. The oligodendroglia-like cells of DNT and glioneuronal hamartias did not show immunoreactivity for any of the markers. The very high incidence of ganglioglial lesions in patients with chronic focal epilepsies and the presence of neurotransmitter-producing enzymes, neurotransmitter receptors, neuropeptides, and calcium-binding proteins in many of these lesions suggests that they may play an active role in the pathogenesis of epileptic seizures.
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PMID:Neurochemical profile of glioneuronal lesions from patients with pharmacoresistant focal epilepsies. 766 58

In utero migrational abnormalities account for most cases of cortical dysplasia. The histopathologic appearance of cortical dysplasia is often varied, making recognition and classification difficult. We studied 52 patients with cortical dysplasia who underwent partial lobectomy for medically intractable seizures in order to devise a simple histopathologic classification schema. The incidence of observed dysplasia in lobectomy specimens over an 11-year period (n = 360) was 14%. Patients ranged in age from 3 months to 47 years at the time of surgery (mean, 19 years; 29 male and 23 female patients). The temporal lobe was involved in 34 patients, frontal lobe in 18, parietal lobe in four, and occipital lobe in three. In three patients multiple lobes showed dysplasia. Dysplasia was right-sided in 29 patients and left-sided in 23 patients. Dysplasia was focal in 23 patients, multifocal in four patients, and diffuse in 25 patients. Three main histologic patterns of cortical dysplasia were observed: (1) a cortical laminar architectural disorganization and/or malalignment of neurons (26 patients), (2) clusters of atypical neurons and glia within the cortex (28 patients), and (3) a hypercellular molecular layer with increased numbers of neurons and glia (31 patients). In 23 patients more than one pattern of dysplasia was identified. Coexistent tumors were present in 13 patients, including ganglioglioma (eight patients), dysembryoplastic neuroepithelial tumor (three patients), and low-grade astrocytoma (two patients). Tuberous sclerosis was present in four patients. We conclude that most types of cortical dysplasia can be divided into three main histologic patterns, facilitating the recognition of dysplasia. In addition to the known association with tuberous sclerosis, tumors may coexist with cortical dysplasia.
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PMID:Cortical dysplasia: a histopathologic study of 52 cases of partial lobectomy in patients with epilepsy. 775 Sep 33

Clinical, radiological, and histopathological features of 51 surgically treated gangliogliomas were evaluated retrospectively. The most common presenting symptoms were epileptic seizures (47 patients (92%)). Focal neurological deficits occurred in 8% of the patients. The duration of symptoms at the time of operation ranged from three months to 45 years, mean 11 years. The temporal lobe was affected in 43 patients (84%), the frontal lobe in five patients (10%), and the occipital lobe in one patient (2%). Two of the tumours (4%) were localised infratentorially. On MRI, solid tumour parts usually showed a pronounced signal increase on proton density images and a less pronounced signal increase on T2 weighted images, whereas solid components were mainly isointense on T1 weighted images. Contrast enhancement was noted in 16 of 36 patients (44%). Cystic tumour parts were found in 23 of 40 patients (57%), all characterised by signal increase on T2 weighted images and decreased T1 signals. Signal deviation of cystic tumour parts on proton density images was variable. Computed tomography was performed in 17 patients and showed hypodense lesions in 10 (59%), and calcifications in seven (41%) cases. Surgery included complete tumour removal in 44 patients (86%) and partial resection in seven (14%). In six patients (12%) there were transient postoperative complications. One patient (2%) died postoperatively due to pulmonary embolism. Histopathological examination of the surgical specimens showed low grade gangliogliomas in 49 cases (96%) and anaplastic gangliogliomas in two (4%). Control MRI of 31 patients with a mean follow up period of 16 months was uneventful in all but one case of an anaplastic ganglioglioma. In all patients in whom the ganglioglioma was associated with medically intractable seizures the operation resulted in complete relief of seizures or a noticeable improvement of the epilepsy.
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PMID:Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. 779 80

We reported four cases of dysembryoplastic neuroepithelial tumor (DNT) with intractable complex partial seizures, and analyzed their clinical and pathological features. The age of patients ranged from four to nineteen years old. Three were boys and one was a girl. The age of seizure onset ranged from eight months to five years. They did not have any neurological deficits. In all cases, CT scan revealed a low density mass in the temporal lobe. Two cases demonstrated calcifications, and another two cysts. The mass demonstrated low signal intensity in T-1 weighted MR images with mild contrast enhancement by Gd-DTPA. Long-term video-EEG monitoring revealed the epileptogenic focus in the temporal lobe on the side of the mass lesion. Anterior temporal lobectomy with total removal of the tumor was carried out in all cases. Hippocampectomy was performed in three cases in which intraoperative electrocorticogram demonstrated spike discharges in the hippocampus. In the surgical specimens, the tumor consisted of increased astrocytes, oligodendrocytes, and some neurons. A cortical nodule appeared in one case, and cortical dysplasia appeared in two cases. It was difficult to distinguish the tumor of case 3 from ganglioglioma because of mild neuronal atypia. The tumor of case 4 demonstrated increased capillary vessels and was also difficult to differentiate from vascular anomalies. The pathological diagnosis of DNT was still difficult. Consequently, we speculated that DNT included some transitional types between congenital anomalies and ganglioglioma.
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PMID:[Clinicopathological features of dysembryoplastic neuroepithelial tumor (DNT): four cases report]. 807 32

This retrospective study focuses on 14 patients with ganglioglioma of the cerebral hemispheres who received surgical treatment in the Neurosciences Department/Neurosurgery of "La Sapienza" University of Rome between 1953 and 1990. The data are analyzed together with those on 98 published cases of ganglioglioma confined to the cerebral hemispheres. These tumours have no sex preference but a decided preference for the young (mean age 18 years). They are characterized by a history of epilepsy often of long standing and sometimes refractory to drugs. The most frequent site is the temporal lobe (61%). At operation the tumour proved to be solid in 52% of cases and the satellite cyst at the tumour wall in 48%. Removal was total in 65% of cases. Even after subtotal removal, the prognosis is good in terms both of survival and of seizure control. Radiotherapy seems to have no role in the treatment of these tumours.
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PMID:Gangliogliomas of the cerebral hemispheres. Report of 14 cases with long-term follow-up and review of the literature. 812 57

The histopathological, clinical, and radiological findings in 25 patients (median age 20.5 years; range 1.7-64.2 years) with gangliogliomas were assessed to correlate degree of astrocytic anaplasia and proliferative potential with recurrence or survival. Most patients (64%) presented with seizures (median Karnofsky Performance Score 90%; range 70-100%). Computerized tomography and magnetic resonance imaging showed nonspecific abnormalities. Neoplastic ganglion cells were defined as heterotopic, irregularly grouped, or having more than one nucleus of bizarre shape or size. The astrocytic component was moderately anaplastic in 15 cases and highly anaplastic (HAA) in 10. Eight patients had gross total resection, 11 had subtotal resection, and six underwent biopsy. Ten patients (five gross total resection, three subtotal resection, two biopsy) had no further treatment, 15 underwent external irradiation, and five had adjuvant chemotherapy. Twenty-four patients are alive 15-394 weeks (median 203.5 weeks) postoperatively; one with ganglioglioma-HAA died at 65 weeks. No tumor recurred after gross total resection. Duration of preoperative symptoms < 1 year, greater anaplasia, and age > 30 years at diagnosis may have increased the risk of recurrence after subtotal resection or biopsy by four, three, and two times, respectively (not significant). Bromodeoxyuridine labeling index (BUdR LI) was < 1% in eight non-recurring tumors and 1.3% in another recurring twice (second recurrence LI = 1.6%). Most patients with ganglioglioma have a good prognosis. After gross total resection, only observation is required. After subtotal resection or biopsy, recurrence is possible. BUdR labeling may guide further therapy.
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PMID:Gangliogliomas: a clinicopathological study of 25 cases and review of the literature. 814 57

Gangliogliomas are relatively rare tumors of the CNS in which ganglion cells and neuroglia coexist. 13 cases are reported, representing 0.61% of a series of 2,121 patients undergoing biopsy for CNS tumors. The clinical, radiological, pathological, electron microscopical and immunohistochemical features were evaluated with clinical follow-up. The age of the patients ranged from 3 to 51 years, including 8 males and 5 females. Duration of symptoms prior to surgery varied from 15 days to 12 years, the most common symptoms being seizures, headache, vomiting and motor disturbances. Computed tomographic scans found high density space-occupying mass in 6 patients and low density space-occupying mass in 3. Ten of the tumors originated form the cerebral hemispheres and three from the midline. The tumors were all sharply delineated and well defined from surrounding tissues. The tumor is generally firm. 8 patients are survived operation for 2-10 years. Neurons and glial cells in the tumor could be demonstrated by electron microscopy and immunohistochemical examination. Anaplastic transformation in the gliomatous component was found in one case. This series suggest that surgical removal of this neoplasm carries a good prognosis.
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PMID:[Clinicopathology of gangliogliomas]. 816 79

We identified 39 patients with chronic epilepsy (seizures > or = 2 years) proven to have primary brain tumors. These cases represent approximately 12% of the surgery cases for epilepsy in the same period. Mean age of seizure onset was 13.2 years: mean duration before operation was 10.5 years. Thirty-eight of 39 had normal neurologic examination. Twenty-six tumors were temporal, 7 were frontal, 4 were parietal, and 2 were occipital. Nine of 26 (34.6%) of the temporal group had contralateral interictal EEG spikes. Pathology was 15 ganglioglioma, 13 low-grade astroctoma, 4 oligodendroglioma, 2 low-grade mixed glioma, 1 pleomorphic xanthoastrocytoma, 2 dysembryoplastic neuroepithelial tumor, and 1 ependymoma. Postoperative seizure frequency (minimum follow-up 6 months) ranged from 15 to 16 seizure-free auras only in patients with temporal tumors and total gross tumor removal (mean follow-up 28 months) to 0 of 6 seizure-free in patients with extratemporal tumors who underwent subtotal resection or biopsy.
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PMID:Chronic intractable epilepsy as the only symptom of primary brain tumor. 824 54

Tumors and cortical dysplasia are associated with epilepsy, but few studies have examined the coexistence of neoplasia and dysplasia in these patients. We studied 13 patients (age 4-29 years) with recurrent seizures of 1 month to 21-year' duration (median 72 months). Ten patients were aged < 21 years. Imaging studies localized the lesion to the temporal lobe (10 patients), parietal lobe (2 patients), and frontal lobe (1 patient). Tumors included ganglioglioma (8 patients), dysembryoplastic neuroepithelial tumor (DNT) (3 patients), and low-grade astrocytoma (2 patients). Cortical dysplasia, including atypical aggregates of neurons (6 patients), multifocal loss of the cortical laminar architecture (7 patients), and neurons in the molecular layer of the cortex (3 patients) were observed near but separate from the tumor. Coexistence of certain tumors with cortical dysplasia, most frequently observed in the pediatric population, suggests a hamartomatous/dysplastic nature of the neoplasms.
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PMID:Coexistence of neoplasia and cortical dysplasia in patients presenting with seizures. 833 May 69

Gangliogliomas are indolent neoplasms that are often associated with long-standing intractable seizures. The seizure-free outcome following ganglioglioma resection alone (or "lesionectomy") has been generally favorable, ranging in most series from 50% to 65%. Thus, the value of resection of epileptogenic cortex in addition to tumor with regard to seizure outcome has been the subject of controversy. The authors describe a series of 12 patients with frontal or temporal lobe gangliogliomas associated with long-standing intractable seizures. In these patients, intraoperative electrocorticography was used to guide the resection of epileptogenic cortex along with tumor. Functional brain mapping, interictal and ictal monitoring of seizures, as well as thorough neuropsychological assessments were performed prior to resection in all cases. Outcome with regard to seizures, tumor recurrence, and neurological deficits was assessed with a mean follow-up period of 3.1 years. There was universal freedom from seizures postoperatively in 11 patients in whom complete or near-complete resection of epileptogenic cortex was achieved. In one patient in whom complete tumor resection and subtotal removal of epileptogenic cortex was achieved, a 95% reduction in seizure frequency was identified. No tumor recurrence or neurological deficits were observed. In a subset of four patients, neuropsychological and cognitive function were evaluated pre- and postoperatively. In these four, a clear trend toward improvement was noted in most functions. Thus, resection of epileptogenic cortex along with tumor may improve seizure outcome in selected patients with tumor-associated epilepsy without engendering identifiable neurological or cognitive deficits attributable to the incremental resection.
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PMID:Intraoperative electrocorticography during tumor resection: impact on seizure outcome in patients with gangliogliomas. 848 71


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