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

Overtreatment by radiotherapy and/or chemotherapy for central nervous system tumors in infancy and childhood may be deleterious, so the recognition of surgically curable clinicopathological entities is mandatory. The dysembryoplastic neuroepithelial tumor is a complex multinodular lesion consisting of glial nodules, associated with a specific glioneuronal element and/or with focal cortical dysplasia, and occurring in young patients presenting with intractable, mostly complex partial, seizures without neurological deterioration. We report on 14 patients; 9 were from a series of 600 pediatric patients with intracranial central nervous system tumors studied at a single institution from 1988 to 1993, and 5 were referred from other pediatric hospitals. Six tumors were frontal, six were temporal, one was parietal, and one was occipitoparietal. Computed tomographic scans disclosed hypodense lesions with cystic appearances in 4 patients and slight focal postcontrast enhancements in only 2 patients, whereas magnetic resonance imaging, available for 7 of 14 patients, showed hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images. Deformities of the overlying cranium were also observed in five patients. The age range at the time of surgery (excluding a 20-year-old male patient who underwent surgery at the main pediatric hospital) was 2.6 to 13 years, with a mean of 6.68 years. The male to female patient ratio was 10:4, and the duration of symptoms was 0.2 to 6 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Dysembryoplastic neuroepithelial tumor: morphological, immunocytochemical, and deoxyribonucleic acid analyses in a pediatric series. 775 46

We present the histologic study of two patients who underwent cerebral cortex resection for partial seizures linked with cortical dysplasia. The distinction of areas of seizure origin from areas of seizure propagation was made according to stereoelectroencephalographic criteria. Samples of epileptogenic tissue were studied by using cytoarchitectonic and immunohistochemical stainings. We mapped the catecholaminergic afferents by employing antisera directed against tyrosine hydroxylase and dopamine-beta-hydroxylase enzymes. The epileptic activity was correlated with the underlying patterns of cytoarchitectonic and immunohistochemical changes. The neuropathological features were focal and consisted of large neurons dispersed through all but the first cortical layer (associated in one case to giant glial cells), of variable disturbance of lamination, of neuronal ectopia in the white matter and of moderate proliferation of small glial cells. Areas of seizure onset coincided with that of dysplastic zones. Both laminar distribution and density of catecholaminergic fibers were altered in the dysplastic cortices (area of seizure onset) and there was an increase in the density of tyrosine hydroxylase-immunoreactive fibers in the surrounding areas of seizure propagation. Our results indicate that these developmental epileptogenic lesions were associated with abnormal neuronal circuitry. They provide evidence at the structural level of the increase in tyrosine hydroxylase activity previously reported in spiking areas of human epileptogenic cerebral cortex and they suggest that catecholamines may contribute toward limiting seizure activity propagation.
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PMID:Altered patterns of catecholaminergic fibers in focal cortical dysplasia in two patients with partial seizures. 784 71

The authors studied 11 patients with developmental malformations and seizures originating from the frontal and central regions. Patients with centrally located seizures had primary involvement of the face or mouth; clonic activity involving the limb was also seen. Seizures among those with frontal lesions were primarily unilateral or bilateral tonic motor. Secondary generalized tonic-clonic seizures preceded by focal manifestations occurred infrequently in those with central localization, but were not rare in the frontal group. MRI revealed abnormalities in 10 patients, nine of whom underwent surgical resection with good results. Focal cortical dysplasia may be the underlying epileptogenic abnormality in young patients with clinical features suggestive of central or frontal cortical involvement.
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PMID:Frontal and central lobe focal dysplasia: clinical, EEG and imaging features. 785 72

Multiple subpial transection (MST) is a novel technique in surgery for epilepsy, employed in patients where some or all of the epileptogenic zone cannot be resected because it lies in a vital cortical area. Twenty one patients subjected to MST were reviewed. Eighteen patients had medically intractable epilepsy and three patients had Landau-Kleffner syndrome. Their ages ranged from 6 to 47 (mean 15-9) and duration of epilepsy ranged from 0.33 to 42 (mean 8.6) years. Preoperative MRI showed focal abnormalities in eight cases. Detailed electrophysiological examination was carried out on all patients. Brain resection was performed in addition to MST in 12 patients. A further six patients underwent brain biopsy. Three patients with Landau-Kleffner syndrome were subjected neither to resection nor to biopsy. Histopathological examination showed Rasmussen's syndrome in six patients, cortical dysplasia in six, cerebral tumour in one, and non-specific changes in five. Multiple subpial transection was carried out mainly in precentral and postcentral regions. Eighteen patients have been followed up for one to five years, and three for 10 months. The three patients with Landau-Kleffner syndrome were mute before operation and have shown substantial recovery of speech. Of the other 18, 11 showed a worthwhile decrease in seizure frequency. None of the patients developed chronic neurological deficits attributable to MST. It is concluded that MST leads to worthwhile seizure control without major neurological deficit in patients who would otherwise be inoperable.
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PMID:Multiple subpial transection: a review of 21 cases. 789 19

The case of a 15-year-old right-handed girl with developmental delay, mild retardation, astereognosis, and tactile discrimination impairment in the left arm, is reported. During sleep, right partial simple motor seizures with sialorrhea and saliva pooling were present, sometimes followed by secondary generalization. Absences and drop attacks were also observed. Electroencephalography revealed left centrotemporal spike waves spreading to the homologous contralateral region, generalized 3-Hz spike-and-wave complexes enhanced by hyperventilation, and generalized polyspike-and-wave discharges during drowsiness. Magnetic resonance imaging showed right perisylvian (opercular) malformation. Benign epilepsy of childhood with centrotemporal spikes (BECCTS) is a focal idiopathic epilepsy presumed to be of genetic origin. Although brain damage is not expected, structural lesions including opercular macrogyria have been reported. This coexistence has been considered mainly causal and only exceptionally causal. The Foix-Chavany-Marie syndrome or operculum syndrome is the result of bilateral opercular damage. In our child the right structural and the left functional lesions appeared mutually activated, yielding a transient opercular syndrome. The concomitance of BECCTS and developmental opercular dysplasia is suggestive of a common genetic substratum.
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PMID:Benign epilepsy of childhood with centrotemporal spikes and unilateral developmental opercular dysplasia. 792 39

With the development of modern imaging techniques, disturbances of neuronal migration appear to be a major cause of epilepsy, mental retardation and chronic neurological disability in childhood. Sixty-nine cases are presented, including 46 of diffuse migration abnormalities and 23 of localized dysplasia. Patients with diffuse migration disorders presented with mental retardation, gross motor impairment and severe seizure disorders whereas in those with focal anomalies, epilepsy was the chief complaint. Magnetic resonance imaging, although usually diagnostic of migration disorders often does not allow definition of the pathologic type. Some EEG patterns, such as high amplitude fast rhythms or the theta-delta pattern are highly suggestive. Most cases of abnormal migration are sporadic and probably acquired. Some are due to chromosomal anomalies, especially of chromosome 17p where a gene for lissencephaly has been mapped. Familial cases occur with both recessive and possibly dominant inheritance. Epilepsy due to migration abnormalities is often intractable. Resection of dysplastic cortex may be effective for localized disease and callosotomy has been proposed for diffuse anomalies.
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PMID:The place of neuronal migration abnormalities in child neurology. 800 Sep 73

A case of early epileptic encephalopathy (EIEE) with suppression-bursts or Ohtahara's syndrome, associated with focal cortical dysplasia is reported. Infantile spasms and brief tonic unilateral seizures began on the fifth day of life. Interictal EEG demonstrated an asymmetrical "suppression-burst" pattern with no wake or sleep cycling. Seizures were refractory to all antiepileptic drug (AED) and steroid therapy. Magnetic resonance imaging (MRI) showed right frontotemporal cortical thickening. After three weeks of an ineffective medical treatment a preoperative evaluation with single photon emission computed tomography (SPECT) and electrocorticography (ECoG) was performed to characterize epileptogenic focus. Surgical resection of the precentral area was performed at age 1 month. Neuropathologic examinations confirmed diagnosis of focal cortical dysplasia by identifying cytoarchitectural disarray and ectopic neurons located deep in subcortical white matter. During follow-up, 1-year postoperative the child had a single febrile seizure. Neurologic examination showed minor developmental delay and slight left-sided weakness.
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PMID:Surgical treatment of an early epileptic encephalopathy with suppression-bursts and focal cortical dysplasia. 800 6

The cytoskeletal abnormalities of cortical neurons in human cerebral cortical dysplasia were compared by immunohistochemical methods to the neurofibrillary tangles of Alzheimer's disease (AD). Surgical specimens from cortical resections performed for the treatment of intractable childhood seizures as well as autopsied samples from AD patients were analyzed with different antibodies directed against high- or medium-molecular mass neurofilament epitopes, phosphorylated or non-phosphorylated forms of neurofilaments, ubiquitin, the microtubule-associated protein tau, and paired helical filaments (PHF), a defining feature of AD tangles. A strong abnormal increase in immunoreactivity to the high and medium molecular mass neurofilament epitopes was seen in hypertrophic neurons of cortical dysplasia. These neurofilamentous accumulations of cortical dysplasia as well as AD tangles also displayed immunoreactivity with antibodies against phosphorylated and non-phosphorylated neuro-filament epitopes, tau and ubiquitin. Only the AD tangles, however, were immunoreactive to the antiserum to PHF. These results replicate and extend our previous findings that the neurofibrillary accumulations in cerebral cortical dysplasia share some common antigens with the neurofibrillary tangles of AD but do not demonstrate immunoreactivity to PHF antiserum. The results also suggest that the cytoskeletal abnormalities observed in neurons of cortical dysplasia may result in part from alterations in the level of expression, in phosphorylation state or in transport of cytoskeletal components.
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PMID:Neuronal cytoskeletal abnormalities in human cerebral cortical dysplasia. 805 2

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

Five patients with treatable partial epilepsy and unilateral opercular neuronal migration disorder (NMD) are reported. Outcome was considered favorable when seizure control was prompt and complete with appropriate antiepileptic drug (AED) therapy, and when there was no relapse after AED discontinuation. Minor cortical sensorimotor defects were noted in 4 patients. All had normal mental status. No epileptic discharges were observed in the EEG of 4 patients, but rolandic spikes were observed in 1. The electroclinical and evolutive features suggested partial idiopathic epilepsy in 2 patients. Cases with focal neuronal migration disorders and favorable epilepsy outcome are probably more common than has been reported previously. The extent and location of the underlying microscopic lesions associated with the radiologically detectable cortical dysplasia may influence clinical outcome.
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PMID:Treatable partial epilepsy and unilateral opercular neuronal migration disorder. 833 May 68


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