Cases reported "Epilepsy"

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1/304. Does the tuberous sclerosis complex include intracranial aneurysms? A case report with a review of the literature.

    BACKGROUND: tuberous sclerosis is a protean, genetically determined disease that may involve any organ or tissue and lead to a great number of symptoms and clinical features. OBJECTIVE: diagnosis can be very difficult in cases with incomplete manifestations (formes fruste) lacking the classic signs of the disease. MATERIALS AND methods: We report a case fulfilling the diagnostic criteria for tuberous sclerosis (shagreen patches, hypomelanotic macules, renal cysts and angiomyolipomas, and "migration tracts" in the cerebral white matter) in association with a giant intracranial aneurysm, but lacking mental retardation, epilepsy and facial angiofibroma. RESULTS: Fourteen other cases of tuberous sclerosis and intracranial aneurysms, all but one without any clear sign of polycystic kidney disease, were found in the literature. CONCLUSION: We suggest that vascular dysplasias in general and aneurysms (mainly intracranial) in particular can be added to the other non-primary diagnostic features for the clinical diagnosis of tuberous sclerosis.
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keywords = cerebral
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2/304. Asynchronous pentobarbital-induced burst suppression with corpus callosum hemorrhage.

    OBJECTIVE: We describe the electroencephalographic (EEG) findings in a 9-year-old girl, who presented with generalized tonic-clonic status epilepticus requiring pentobarbital anesthesia, and correlate these findings with clinicoradiologic evidence of a ruptured AVM with hemorrhage into the body of the corpus callosum. methods: EEG analysis accompanied by clinical assessment, CT and MRI scans, and cerebral angiography were performed. RESULTS: With pentobarbital coma, the EEG showed burst suppression with prominent interhemispheric asynchrony. Suppression epochs >2 s in duration and with amplitude <20 microV in all channels were identified. In 12 min of the EEG analyzed, 6 unilateral and 20 bilateral epochs occurred. Of the 20 bilateral suppression epochs, interhemispheric asynchrony of >1 s was noted at onset for 5 epochs and at offset for one. Chi-square analysis revealed an equal tendency for unilateral suppressions to occur over either hemisphere, and for suppression in one hemisphere to begin before the other. CONCLUSIONS: We conclude that the corpus callosum plays a critical role in interhemispheric synchronization of cortical neuronal electrical activity and propose that: (1) normally, the corpus callosum modulates interhemispheric synchronization of cortical inhibition; and (2) with corpus callosal disruption, cortical areas are 'released' from such synchronization.
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ranking = 1
keywords = cerebral
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3/304. Illusory contour perception and amodal boundary completion: evidence of a dissociation following callosotomy.

    A fundamental problem in form perception is how the visual system can link together spatially separated contour fragments to form the percept of a unitary shape. Illusory contours and amodal completion are two phenomena that demonstrate this linking process. In the present study we investigate these phenomena in the divided hemispheres of two callosotomy ("split-brain") patients. The data suggest that dissociable neural mechanisms are responsible for the generation of illusory contours and amodal completion. Although both cerebral hemispheres appear to be equally capable of perceiving illusory contours, amodal completion is more readily utilized by the right hemisphere. These results suggest that illusory contours may be attributable to low-level visual processes common to both hemispheres, whereas amodal completion reflects a higher-level, lateralized process.
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keywords = cerebral
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4/304. Unruptured cerebral aneurysms causing seizure disorder (report of two cases).

    seizures as a presenting feature of unruptured cerebral aneurysm are unusual. We report two cases of unruptured cerebral aneurysm presented with seizures. In both cases the seizures controlled following surgical ablation of the aneurysm.
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ranking = 6
keywords = cerebral
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5/304. Noninvasive continuous monitoring of cerebral oxygenation periictally using near-infrared spectroscopy: a preliminary report.

    PURPOSE: To report on the use of near-infrared spectroscopy (NIRS) to examine the changes in cerebral oxygenation in the periictal period in patients with seizures. methods: Cerebral hemoglobin oxygen availability was monitored continuously and noninvasively with NIRS in three patients (one in the pediatric intensive care unit (ICU) and two in epilepsy-monitoring units) in conjunction with continuous EEG monitoring. Ictal events were recorded and compared with the pre-, intra-, and postictal periods for cerebral oxygen availability, as defined by oxygenated hemoglobin (HbO2), deoxygenated hemoglobin (Hb), and the redox state of cytochrome oxidase (cytox). RESULTS: Several important preliminary observations were made by using this technology. First, a preictal increase in cerebral oxygenation began between 1 and 2 h and >10 h before the ictal event. Second, despite adequate perfusion, based on an observed increased HbO2, reduction in cytox indicates a perfusion-metabolism mismatch during seizure activity. Third, continued seizure activity and even isolated ictal events were associated with decreased cerebral oxygen availability. Fourth, differences in cerebral oxygen availability were noted between different types of seizures (e.g., electrographic seizures were accompanied by rapid reductions in HbO2 and cerebral blood volume without reduction of cytox, whereas electroclinical seizures were characterized by marked increases in HbO2 with or without reduction of cytox). CONCLUSIONS: In this preliminary report on the use of NIRS for patients with seizures, we believe that NIRS allows continuous and noninvasive monitoring of changes in cerebral oxygenation periictally, thereby permitting investigations into the pathophysiology of seizures and the exploration of the potential of cerebral oximetry as a tool for seizure localization.
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ranking = 12
keywords = cerebral
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6/304. Immunohistochemical analysis in a case of idiopathic Lennox-Gastaut syndrome.

    We herein report a neuropathological and immunohistochemical analysis of a brain from a 25-year-old male with idiopathic type of Lennox-Gastaut syndrome (LGS). The clinical pictures, such as seizure type and progressive mental deterioration with an initial normal psychomotor and mental development in a man were typical of LGS. A routine neuropathological examination showed no pronounced changes, such as neuronal loss, morphologically abnormal neurons, inflammation, vascular changes, Lafora bodies and tumor cells, except that mild gliosis was seen only in CA4 of the hippocampus. Numerous corpora amylacea were observed throughout the cerebral cortices subjacent to the pia mater. An immunohistochemical analysis showed no marked findings for such proteins as glutamate transporters, glutamate decarboxylase, glutamine synthetase, neuronal cytoskeleton proteins and heat-shock proteins. However, intense ubiquitin-immunostained neurons were only found in CA4 of the hippocampus, whereas numerous astrocytes showed a strong immunoreaction for glial fibrillary acidic protein, but showed an exclusively reduced immunoreactivity for metallothionein-I/II, zinc-chelating protein. Our findings thus suggest that the pathology in the hippocampus is either causally or consequentially associated with the seizures occurring in LGS.
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ranking = 1
keywords = cerebral
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7/304. Somatosensory representation in patients who have undergone hemispherectomy: a functional magnetic resonance imaging study.

    OBJECT: Removal or disconnection of an entire cerebral hemisphere is occasionally used to treat refractory seizures. patients who have undergone a hemispherectomy provide useful models to study the reorganization of cortical somatosensory representation. This plasticity may be a consequence of the pathological lesion, the hemispherectomy itself, or both. methods: Three patients who had undergone hemispherectomy were studied with functional magnetic resonance (fMR) imaging. Responses to sensory stimulation in normal hands and hands opposite the lesioned hemisphere were studied. Multislice T2*-weighted gradient-echo echoplanar images were obtained using a 1.5-tesla MR imager. The activation condition consisted of somatosensory stimulation of the index finger. A T1-weighted anatomical MR image was acquired. The fMR and anatomical MR images were coregistered, and statistically significant activation foci (p < 0.01) were identified. Stimulation of the normal hand produced activation in the primary somatosensory cortex (SI) in all patients. Stimulation of the impaired hand resulted in activation of the ipsilateral parietal operculum (second somatosensory area [SII]) and posterior parietal lobe (Brodmann's Area 7) in all cases, but no activation was elicited in the SI in any patient. In addition, other areas within the ipsilateral frontal and parietal lobes were activated in some individuals. CONCLUSIONS: Residual somatosensory function in the hand opposite the lesioned hemisphere is mediated by the SII and other cortical regions in the intact hemisphere, without involvement of the SI.
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ranking = 1
keywords = cerebral
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8/304. Bilateral focal polymicrogyria in ehlers-danlos syndrome.

    BACKGROUND: ehlers-danlos syndrome (EDS) is a heterogeneous group of generalized connective tissue disorders that has been described in association with epilepsy and cerebral cortical dysplasia, mostly gray matter heterotopias, in 3 reports. However, to our knowledge, association of EDS with another type of cortical cerebral dysplasia, bilateral focal polymicrogyria, has never previously been described. SETTING: Two research-oriented hospitals. patients: We describe 2 patients with EDS and bilateral polymicrogyria. The first, a 29-year-old black man, presented with EDS of unspecified type, seizures, and bilateral frontocentral and frontoposterior polymicrogyria with hypoplasia of the inferior part of the cerebellar vermis. The second, a 20-year-old woman, had type III EDS, seizures and congenital bilateral perisylvian syndrome with polymicrogyria. CONCLUSIONS: The association of bilateral focal polymicrogyria and EDS in these 2 patients suggests that extracellular matrix proteins implicated in the pathogenesis of EDS, such as collagen and tenascin, may play an important role in cerebral cortical formation and organization. In a clinical setting, the association of EDS with these cortical structural lesions has implications for diagnosis and management.
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ranking = 3
keywords = cerebral
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9/304. Valproate-induced hyperammonemic encephalopathy in the presence of topiramate.

    Two adult patients with focal epilepsy who tolerated valproate (VPA) well in different combinations of anticonvulsants developed hyperammonemic encephalopathy when treated with VPA in combination with topiramate (TPM). Topiramate may contribute to the increased ammonia level by its inhibition of carbonic anhydrase and cerebral glutamine synthetase, thereby facilitating VPA/TPM hyperammonemic encephalopathy. Recovery occurred after withdrawal of VPA or TPM.
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ranking = 1
keywords = cerebral
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10/304. The electroencephalogram through a software microscope: non-invasive localization and visualization of epileptic seizure activity from inside the brain.

    OBJECTIVE: We developed a novel non-invasive analysis to localize the source and visualize the time course of electrical activity generated inside the brain but unclear from the scalp. This analysis applies to signals with unique waveform characteristics, such as seizures. methods: The method extracts activity from an EEG data matrix as a spatiotemporal component having waveforms uncorrelated to the other concurrent activities. The method also provides the location and orientation of the dipole generating this activity. We applied this method to ten scalp seizures in three patients with temporal lobe epilepsy and single-focus seizures confirmed by intracerebral recordings. A realistic head model based on MRI was used for computation of field distributions. RESULTS: When seizure activity was still not visually identifiable on the scalp, the method demonstrated in all scalp seizures a source in the temporal neocortex corresponding clearly to the region of seizure activity in intracerebral recordings. Frequency characteristics of the estimated activities also resembled those of the intracerebral seizures. CONCLUSIONS: This method enables estimation of focal brain activity when its effect on scalp EEG is unclear to visual examination. It works in situations where currently available source analyses methods, which require noiseless definite activity, are not applicable.
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ranking = 3
keywords = cerebral
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