Cases reported "Epilepsy"

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1/67. 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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2/67. Retrolabyrinthine presigmoid transpetrosal approach for selective subtemporal amygdalohippocampectomy.

    The retrolabyrinthine presigmoid transpetrosal approach is a modification of the subtemporal approach which is suitable for complete amygdalectomy. By drilling away the retrolabyrinthine presigmoid petrosal bone, at least 1 cm more space below and 1 cm more space medially is obtained than in the subtemporal approach, and temporal retraction pressure is diminished when approaching from below. Operative results according to the Engel's classification of seizure control, and pre- and postoperative Wechsler adult intelligence Scale (WAIS), revised WAIS, and Wechsler intelligence Scale for Children scores were measured in 16 patients treated by normal or modified subtemporal amygdalohippocampectomy. Postoperative follow-up ranged from 8 to 79 months. There has been no morbidity or mortality among these 16 patients, and postoperative seizure frequency has been diminished to less than 10% of the preoperative level in 15 of the 16. In eight patients, seizures have been eliminated totally. Subtemporal amygdalohippocampectomy achieved significantly increased performance and full scale intelligence quotient within 2 months after surgery, compared to preoperative levels. Subtemporal amygdalohippocampectomy is an alternative to the transsylvian approach, but is less invasive.
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3/67. Surgical treatment of intractable epilepsy originating from the primary sensory area of the hand--case report.

    A 14-year-old right-handed girl presented with intractable epilepsy originating from the primary sensory area of the hand, manifesting as sensory partial seizures in the left hand with secondary generalization. Neurological examination showed no abnormal findings. magnetic resonance imaging, magnetoencephalography, and cortical stimulation using chronic subdural electrodes demonstrated a lesion located in the primary sensory cortex of the hand, in which the ictal onset zone was identified by 24-hour intracranial electroencephalographic recording. Surgical resection of the lesion and multiple subpial transections of the adjacent cortices were performed. The histological diagnosis was dysembryoplastic neuroepithelial tumor (DNT). She was completely free of seizures without permanent sensory deficits postoperatively. DNT located in the primary sensory hand area may be resectable without causing postoperative sensory deficits, if accurate functional mapping and surgical resection are performed.
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4/67. Transient epileptic foci associated with intracranial hemorrhage in patients with subdural and epidural electrode placement.

    We report two cases of transient epileptic foci in humans associated with placement of intracranial electrodes. The abnormalities consisted of restricted areas of active, almost continuous, rhythmic spiking, intermittently evolving into electrographic seizure activity, which resolved spontaneously within 3-4 days. The first occurred after placement of a subdural electrode grid and the second following insertion of epidural peg electrodes. neuroimaging demonstrated a small subdural hematoma overlying the grid and a focal intraparenchymal hemorrhage underlying the affected epidural electrodes. The insertion of intracranial electrodes may be complicated by the induction of transient epileptic foci unrelated to a patient's typical epileptic generator.
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5/67. Triple-technique (MR imaging, single-photon emission CT, and CT) coregistration for image-guided surgical evaluation of patients with intractable epilepsy.

    Ictal and interictal single-photon emission CT (SPECT) play an increasingly important role in the surgical evaluation of patients with epilepsy. We present a method of coregistration of MR, SPECT, and CT images to correlate structural data (MR imaging), blood flow changes (SPECT), and location of subdural electrodes (CT) for patients undergoing image-guided surgical treatment of epilepsy. MR-SPECT root mean square (rms) mismatch distances were 2.1 to 2.5 mm, and MR-CT rms mismatch distances were 1.0 to 4.5 mm. Coregistration assisted in image-guided placement of subdural electrodes and in surgical resection of the suspected epileptogenic focus.
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6/67. Activation of the remaining hemisphere following stimulation of the blind hemifield in hemispherectomized subjects.

    We used functional magnetic resonance imaging (fMRI) to investigate the neural substrates mediating residual vision in the "blind" hemifield of hemispherectomized patients. The visual stimuli were semicircular gratings moving in opposite directions on a dynamic random-dot background. They were specifically constructed to eliminate intra- and extraocular light scatter and optimize the activation of extrastriate cortical areas and their subcortical relays. Multislice T2*-weighted gradient echo (GE) echoplanar imaging (EPI) images (TR/TE = 4 s/45 ms, flip angle 90 degrees ) were acquired during activation and baseline visual stimulation. An activation minus baseline subtraction was performed, and the acquired t statistic map transformed into the stereotaxic coordinate space of Talairach and Tournoux. In seven normal control subjects, right hemifield stimulation produced significant activation foci in contralateral V1/V2, V3/V3A, VP, and V5 (MT). Significant activation was also produced in homologous regions of the right occipital lobe with left hemifield stimulation. Stimulation of the intact hemifield in hemispherectomized patients resulted in activation of similar areas exclusively within the contralateral hemisphere. Stimulation of the anopic hemifield produced statistically significant activation in the ipsilateral occipital lobe (putative area V5 or MT) and areas V3/V3A in the only subject with blindsight. We conclude that the remaining hemisphere may contribute to residual visual functions in the blind hemifield of hemispherectomized patients, possibly through the collicular-pulvinar route since the activated areas are known to receive their afferents from these subcortical nuclei.
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keywords = space
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7/67. Dysembryoplastic neuroepithelial tumor (DNT): an ultrastructural study of six cases.

    We report six cases od DNT with a detailed ultrastructural characteristics. The patient age ranged from 7 to 16 years (mean 12), the location was temporal in three cases and frontal, temporooccipital and parietooccipital in each of one remaining cases. The predominant clinical feature in each case was history of episodes of intractable seizures. Histopathologically, the neoplasms were multinodular, each nodule was well-circumscribed and was composed of glioneuronal elements embedded in the variable amount of myxoid matrix. The oligodendroglial-like cells (OLC) predominated in the nodules with some accompanying mature neurons. The nodules were frequently surrounded by small calcifications which could be found also within the tumors. OLCs were immunoreactive for S-100 protein and neurons had the expression of synaptophysin and neurofilament proteins. Ultrastructurally, each tumor consisted of three major elements: neoplastic cells (OLC), elongated processes forming neuropil-like structure and expanded "mucoid" extracellular space: the latter gave an impression of cellular elements floating within it. Neoplastic cells had round, oval or elongated nuclei, no discernible nucleoli and a relatively narrow rim of the cytoplasm. Some nuclei were irregular and invaginated and pseudoinclusions were observed; a part of cytoplasm sequestered within pseudoinclusions often appeared degenerated with large blabs and electron-lucent vesicles, some of these contained in turn semicircular profiles of unknown significance. The second element consisted of innumerable cellular processes. Some of these were elongated and formed stacks connected by symmetrical symmetric or asymmetric adhesive plaque junctions. The others had shorter "neck" containing microtubules, these extended into bullous extensions. Dense-cored vesicles were occasionally observed, in both cytoplasm of neoplastic cells and within processes. In one cell, cross-sectioned annulate lamellae were found. In cytoplasm of a few cells, unusual inclusions reminiscent ribosome-lamellae complexes were observed. These were cylindrical resembling "laboratory tubes" with a cone-like endings. At higher power, walls of the "tubes" resolved into layered structures composed of several laminae; between laminae, ribosome-like structures were visible.
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keywords = space
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8/67. The role of intracranial electrode reevaluation in epilepsy patients after failed initial invasive monitoring.

    PURPOSE: Intracranial electrode recording often provides localization of the site of seizure onset to allow epilepsy surgery. In patients whose invasive evaluation fails to localize seizure origin, the utility of further invasive monitoring is unknown. This study was undertaken to explore the hypothesis that a second intracranial investigation is selected patients warrants consideration and can lead to successful epilepsy surgery. methods: A series of 110 consecutive patients with partial epilepsy who had undergone intracranial electrode evaluation (by subdural strip, subdural grid, and/or depth electrodes) between February 1992 and October 1998 was retrospectively analyzed. Of these, failed localization of seizure origin was thought to be due to sampling error in 13 patients. Nine of these 13 patients underwent a second intracranial investigation. RESULTS: Reevaluation with intracranial electrodes resulted in satisfactory seizure-onset localization in seven of nine patients, and these seven had epilepsy surgery. Three frontal, two temporal, and one occipital resection as well as one multiple subpial transection were performed. Six patients have become seizure free, and one was not significantly improved. The mean follow-up is 2.8 years. There was no permanent morbidity. CONCLUSIONS: In selected patients in whom invasive monitoring fails to identify the site of seizure origin, reinvestigation with intracranial electrodes can achieve localization of the region of seizure onset and allow successful surgical treatment.
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keywords = subdural
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9/67. Representation of visuotactile space in the split brain.

    Recent neurophysiological research in the monkey has revealed bimodal neuronal cells with both tactile receptive fields on the hand and visual receptive fields that follow the hands as they move, suggesting the existence of a bimodal map of visuotactile space. Using a cross-modal congruency task, we examined the representation of visuotactile space in normal people and in a split-brain patient (J. W.) as the right arm assumed different postures. The results showed that the congruency effects from distracting lights followed the hand around in space in normal people, but failed to do so in the split-brain patient when the hand crossed the midline. This suggests that cross-cortical connections are required to remap visual space to the current hand position when the hand crosses the midline.
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keywords = space
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10/67. A new syndrome with craniofacial and skeletal dysmorphisms and developmental delay.

    We report a 16-year-old boy with multiple craniofacial and skeletal dysmorphic features including brachycephaly, acrocephaly, hypertelorism, wide palpebral fissures, broad nose, anteverted nares, broad columella, long and smooth philtrum, thin upper lip, macrostomia, carp-like mouth, micrognathia, low-set and posteriorly angulated ears with small and abnormal pinnae, a low posterior hairline, a short neck, hypoplastic and widely-spaced nipples, multiple severe pterygia, an umbilical hernia, metatarsus varus, low implantation of the halluces, and delayed motor and language development. An MRI of the head showed bilateral frontal pachygyria but no sign of heterotopia. The unique features of our patient suggest that he represents a new syndrome.
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