Cases reported "Epilepsy"

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1/432. Surgical management of pediatric tumor-associated epilepsy.

    brain tumors are a common cause of seizures in children. Early surgical treatment can improve seizure outcome, but controversy exists regarding the most appropriate type of surgical intervention. Some studies suggest tumor resection alone is sufficient, while others recommend mapping and resection of the surrounding epileptogenic foci to optimize seizure outcome. To address this issue, we reviewed the charts of 34 pediatric patients aged 18 months to 20 years with medically intractable epilepsy and primary brain tumors. The average age at operation was 12.6 years, and patients had seizures for an average of 6.4 years. The majority of tumors were located in the temporal lobe. Seventeen patients, because of tumor location near an eloquent area, underwent extraoperative mapping using subdural electrode grids prior to definitive tumor resection. Fourteen of these patients had a gross total tumor resection, yet only two had a distinct zone of ictal onset identified and resected. The remaining 17 patients had tumors either in the nondominant hemisphere or far removed from speech-sensitive areas, and therefore did not undergo extraoperative subdural electroencephalograph mapping. Fourteen of these patients also had a gross total tumor resection, while none had intraoperative electrocorticography to guide the resection of additional nontumoral tissue. overall, of the 28 patients treated with a gross total tumor resection, 24 (86%) are seizure free, while the other four are significantly improved. Of the six patients who had a subtotal tumor removal, five have persistent seizures. The mean follow-up was 3.6 years. We conclude that in children and adolescents, completeness of tumor resection is the most important factor in determining seizure outcome. The routine mapping and resection of epileptogenic foci might not be necessary in the majority of patients. As a corollary, the use of subdural electrode grids in pediatric patients with tumor-associated epilepsy should be limited to cases requiring extraoperative cortical stimulation for localization of nearby eloquent cortex.
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keywords = brain
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2/432. Schinzel-Giedion syndrome: evidence for a neurodegenerative process.

    We report on a case of Schinzel-Giedion syndrome in which serial magnetic resonance (MR) brain-imaging studies demonstrated a progressive neurodegenerative process. These findings in addition to "coarse" facial appearance and skeletal abnormality suggest that a progressive metabolic defect underlies this syndrome. However, results of detailed investigations for metabolic disorder were all normal.
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keywords = metabolic disorder, brain
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3/432. Characterization of nodular neuronal heterotopia in children.

    Neuronal heterotopia are seen in various pathologies and are associated with intractable epilepsy. We examined brain tissue from four children with subcortical or periventricular nodular heterotopia of different aetiologies: one with severe epilepsy following focal brain trauma at 17 weeks gestation, one with hemimegalencephaly and intractable epilepsy, one with focal cortical dysplasia and intractable epilepsy, and one dysmorphic term infant with associated hydrocephalus and polymicrogyria. The connectivity of nodules was investigated using histological and carbocyanine dye (DiI) tracing techniques. DiI crystal placement adjacent to heterotopic nodules revealed numerous DiI-labelled fibres within a 2-3 mm radius of the crystals. Although we observed labelled fibres closely surrounding nodules, the majority did not penetrate them. Placement of DiI crystals within nodules also identified a limited number of projections out of the nodules and in one case there was evidence for connectivity between adjacent nodules. The cellular and neurochemical composition of nodules was also examined using immunohistochemistry for calretinin and neuropeptide y (NPY), which are normally expressed in GABAergic cortical interneurons. Within heterotopic nodules from all cases, numerous calretinin-positive neurons were identified, along with a few cell bodies and many processes positive for NPY. Calretinin-positive neurons within nodules were less morphologically complex than those in the cortex, which may reflect incomplete differentiation into an inhibitory neuronal phenotype. There were also abnormal clusters of calretinin-positive cells in the overlying cortical plate, indicating that the migratory defect which produces heterotopic nodules also affects development of the cortex itself. Thus, heterotopic nodules consisting of multiple neuronal cell types are associated with malformation in the overlying cortical plate, and have limited connectivity with other brain regions. This abnormal development of connectivity may affect neuronal maturation and consequently the balance of excitation and inhibition in neuronal circuits, leading to their epileptogenic potential.
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keywords = brain
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4/432. Benign idiopathic partial epilepsy and brain lesion.

    A 14-year-old girl had severe head trauma from a dog bite at the age of 9 days. This resulted in extensive brain damage, tetraplegia, mental retardation, and epilepsy. The seizures were of rolandic type, and the EEG showed multifocal sharp waves. The course was benign. The initial diagnosis of a pure symptomatic epilepsy was revised after demonstrating typical benign focal sharp waves in the EEG of the healthy sister. Thus a phenocopy of a benign partial epilepsy by the brain lesion could be excluded with sufficient certainty. This observation allows the conclusion that the genetic disposition underlying the sharp-wave trait characteristic of benign partial epilepsies can be involved also in the pathogenesis of seemingly pure symptomatic epilepsies. EEG studies on siblings of such patients are needed to exclude possible phenocopies.
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keywords = brain
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5/432. Psychoneuroendocrine aspects of temporolimbic epilepsy. Part III: case reports.

    Many reproductive steroids have neuroactive effects that can modulate neuronal excitability and influence emotions. Emotional disorders may result when 1) abnormal endocrine states interact with normal brain, 2) normal endocrine states interact with abnormal brain, and 3) abnormal endocrine states interact with abnormal brain. An understanding of these pathogenetic relationships and the potential therapeutic role of reproductive hormones should lead to a more effective and comprehensive management of women and men with anxiety and mood disorders.
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keywords = brain
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6/432. Epileptogenic mineralization: pathological variants with good prognosis.

    Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic "brain stone." Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy.
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keywords = brain
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7/432. Unexpected death in persons with symptomatic epilepsy due to glial brain tumors: a report of two cases and review of the literature.

    Two cases of unexpected death in persons with epileptic seizures due to a brain tumor are presented which encompassed an astrocytoma WHO grade II and an anaplastic astrocytoma WHO grade III. A 35-year-old man was found somnolent and disoriented at home. A computed tomography (CT) scan revealed a tumor of the right frontal lobe suggestive for an oligodendroglioma. During an angiographic examination the patient experienced an epileptic seizure. Some weeks later, the man was found dead in front of his house with a fresh bite mark of the tongue. Neuropathological examination revealed an astrocytoma WHO grade II of the right frontal lobe. A 47-year-old man plunged into a swimming-pool and was found submerged some minutes later. After resuscitation he survived comatose for 8 days but finally died due to severe hypoxic brain damage. He had been operated on a brain tumor of the temporal lobe 1 year before the accident. Neuropathological examination revealed residual tumor tissue at the operation site corresponding to an anaplastic astrocytoma WHO grade III. Although rare, death in persons with epileptic seizures due to brain tumors is an important mechanism of death encountered by the forensic pathologist.
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8/432. Behavioral psychophysiological intervention in a mentally retarded epileptic patient with brain lesion.

    Behavioral psychophysiological treatment entailing Slow Cortical Potential (SCP) biofeedback training and behavioral self-control training was conducted with a 27-year-old male epileptic patient (seizures for 23 years) with Wechsler IQ 64 who underwent callosotomy. The patient had 12/week secondary generalized tonic-clonic seizures. The treatment, consisting of 43 SCP training sessions and 22 behavioral control sessions, yielded a highly significant reduction of seizure frequency to about 7.5/week; such a decrease had never been observed after administration of new anticonvulsant drugs, nor after the callosotomy. During SCP feedback training, the patient was able to produce highly-significant cortical differentiation of SCPs of about 4 microV. In addition, he developed several new behaviors indicating growing ability of self-perception and self-regulation. These findings suggest that a combination of SCP biofeedback with behavioral treatment of epilepsy can be used even in mentally retarded patients with organic brain disorders.
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ranking = 5
keywords = brain
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9/432. Spatiotemporal EEG analysis and distributed source estimation in presurgical epilepsy evaluation.

    In the attempts to localize electric sources in the brain on the basis of multichannel EEG and/or MEG measurements, distributed source estimation procedures have become of increasing interest. Several commercial software packages offer such localization programs and results using these methods are seen more and more frequently in the literature. It is crucial that the users understand the similarities and differences of these methods and that they become aware of the advantages and limitations that are inherent to each approach. This review provides this information from a theoretical as well as from a practical point of view. The theoretical part gives the algorithmic basis of the electromagnetic inverse problem and shows how the different a priori assumptions are formally integrated in these equations. The authors restrict this formalism to the linear inverse solutions i.e., those solutions in which the inversion procedure can be represented as a matrix applied to the data. It will be shown that their properties can be best characterized by their resolution kernels and that methods with optimal resolution matrices can be designed. The authors also discuss the important problem of regularization strategies that are used to minimize the influence of noise. Finally, a new kind of inverse solution, termed ELECTRA (for ELECTRical Analysis), is presented that is based on constraining the source model on the basis of the currents that can actually be measured by the scalp recorded EEG. The practical part of the review illustrates the localization procedures with different clinical data sets. Three aspects become important when working with real data: 1) Clinical data is usually far from ideal (limited number of electrodes, noise, etc.). The behavior of inverse procedures in such unfortunate situations has to be evaluated. 2) The selection of the time points or time periods of interest is crucial, especially in the analysis of spontaneous EEG. 3) Additional information coming from other modalities is usually available and can be incorporated. The authors are illustrating these important points in the case of interictal and ictal epileptiform activity. Spike averaging, frequency domain source localization, and temporal segmentation based on electric field topographies will be discussed. Finally, the technique of EEG-triggered functional magnetic resonance imaging (fMRI) will be illustrated, where EEG is recorded in the magnet and is used to synchronize fMRI acquisition with interictal events. The analysis of both functional data, i.e. the EEG in terms of three-dimensional source localization and the EEG-triggered fMRI, combines the advantages of the two techniques: the temporal resolution of the EEG and the spatial resolution of the fMRI.
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ranking = 1
keywords = brain
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10/432. Neuronal cytoskeletal changes are an early consequence of repetitive head injury.

    While neuropathological studies have established the pathology of dementia pugilistica to be similar to that of Alzheimer's disease, there is little information about the early histological changes caused by the repetitive trauma that eventually produces dementia pugilistica. We have examined the brains of four young men and a frontal lobectomy specimen from a fifth, age range 23-28 years, all of whom suffered mild chronic head injury. There were two boxers, a footballer, a mentally subnormal man with a long history of head banging, and an epileptic patient who repeatedly hit his head during seizures. The four autopsy cases were widely sampled; the lobectomy specimen was serially sliced after fixation. Routine stains were performed; inmmunostaining included beta-amyloid precursor protein, amyloid beta-protein (Abeta), tau and apolipoprotein E (apoE). Pathological findings in all five cases were of neocortical neurofibrillary tangles (NFTs) and neuropil threads, with groups of tangles consistently situated around blood vessels in the worst affected regions. No Abeta immunoreactivity was detected. The amount of neuronal apoE expression varied widely between the cases with no clear relation to the NFTs. The apoE genotype was determined in only two cases (both epsilon3/epsilon3). It appears that repetitive head injury in young adults is initially associated with neocortical NFT formation in the absence of Abeta deposition. The distribution of the tau pathology suggests that the pathogenesis of cytoskeletal abnormalities may involve damage to blood vessels or perivascular elements.
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