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Frameless isocentric stereotactic laser beam guide for image-directed microsurgery.

We have developed a frameless stereotactic multi-articulated arm suitable for image-directed microsurgery and surgical navigation. The system comprises a frameless isocentric mechanism and a three-dimensional (3 D) digitizer for intra-operative monitoring. Five joints of our multi-articulated arm are equipped with electromagnetic clutches, and the arms are provided with counterbalancing weights to ensure safe, smooth movement. Stereotactic microsurgical procedures were carried out with a laser beam guide mounted at the tip of the isocentric arm. We have used this system in 22 cases to resect tumours or epileptic foci. There were 12 cases of glioma, 5 cases of deep seated benign tumours, 3 cases of multiple metastatic tumours, and 2 cases of temporal lobe epilepsy. Postoperative enhanced CT images indicated total or subtotal resection in all cases. We also have used this system to determine the approach for clipping of a posterior cerebral artery aneurysm in 2 cases. The error of mechanical accuracy of the system was less than 1 mm. We believe that this system will prove useful in image-directed microsurgical procedures.

Brain Mapping↗

Relationships between MR-imaged total amount of tissue removed, resection scores of specific mediobasal limbic subcompartments and clinical outcome following selective amygdalohippocampectomy.

Of 204 patients who underwent a selective amygdalohippocampectomy at our hospital, we studied 30 in whom special pre- and postoperative MR imaging was carried out and who were followed up for at least 1 year postoperatively. We measured the total size of the resection and the extent to which the following specific mediobasal temporal lobe structures had been removed: amygdala, hippocampus, pes hippocampi, dentate gyrus, parahippocampal gyrus, uncus and subiculum. Postoperative seizure control was correlated with the overall size of the resection as well as with the resection scores of the above mentioned limbic subcompartments. The mean size of the removed tissue was 7.2 cm3 (range: 2.1-17.7). The mean resection scores of the limbic subcompartments (in percentages) are: amygdala 92%, hippocampus 46%, pes hippocampi 92%, dentate gyrus 45%, parahippocampal gyrus 32%, uncus 92%, subiculum 40%. Although a small resection did not exclude a good outcome, the general tendency was that a better outcome was obtained from a larger resection. With regard to the resection scores of the limbic subcompartments, a positive correlation emerged between good postoperative outcome and the radicality of the removal of the parahippocampal gyrus (and the subiculum, which has been evaluated separately). These findings support our previously formulated amplifier hypothesis for the parahippocampal gyrus.

Adult↗

Frequent association of cortical dysplasia in dysembryoplastic neuroepithelial tumor treated by epilepsy surgery.

BACKGROUND: We report on our experience with epilepsy surgery in the treatment of localization-related epilepsy caused by dysembryoplastic neuroepithelial tumor (DNT) aimed at achieving the best seizure control. METHODS: A retrospective analysis was performed on the pathological reports as well as on clinical data of 24 case patients with medically intractable epilepsy with DNT treated surgically between 1995 and 2000 at the Samsung Medical Center. Resective surgery was performed using subdural electrodes or intraoperative electrocorticography in all patients. RESULTS: The mean follow-up period was 57.2 months. Two patients had rare seizures transiently after surgery but remained free from seizures after 6 months. Others remained completely free from seizures. There was a strong tendency of temporal lobe involvement (19 cases; 79.2%). Size of tumors located at medial temporal regions was significantly smaller than those at lateral temporal or frontal lobes (P < .05). A rather radical resection (tumor plus surrounding tissue showing active epileptogenicity) was performed in all but one case where only focal lesionectomy was done. In 20 of the 24 cases (83.3%), association of cortical dysplasia (CD) was found on pathological examination. CONCLUSIONS: We conclude that DNT is frequently associated with CD, with a wide area of epileptogenic activity that might be related to the presence of CD around the DNT. Comprehensive preoperative investigations for accurate localization of epileptogenic activity, meticulous brain mapping, and a rather radical resection of pathological areas might be essential for the achievement of excellent seizure control in DNT-associated epilepsy.

Adolescent↗

Temporal evolution and prognostic significance of postoperative spikes after selective amygdala-hippocampectomy.

The purpose of this study was to quantify the temporal evolution and to determine the prognostic significance of spikes on serial postoperative EEGs after selective amygdala-hippocampectomy. The authors performed postoperative EEGs 4 months, 1 year, and 2 years after surgery in 31 patients with unilateral mesial temporal lobe epilepsy. Spike frequency was determined ipsilateral to the resection (group I, no spikes; group II, 1 to 10 spikes; group III, 11 to 20 spikes; group IV, more than 21 spikes during the 30-minute recording). The temporal evolution of postoperative spike frequency was assessed, and these parameters were correlated with surgical outcome. Twenty-two patients showed spikes on postoperative EEG. Spike frequency decreased over time in 14 patients, while no changes or minimal changes occurred in seven patients, and spike frequency increased in one patient. Nine patients had no spikes. There was no correlation between occurrence, frequency, and temporal evolution of spikes with postoperative seizure control. The authors' results demonstrate a progressive decrease of spike frequency during the postoperative period after selective amygdala-hippocampectomy, although occurrence, frequency, and temporal evolution of postoperative spikes had no implications on surgical outcome.

Adult↗

Human epileptic astrocytes exhibit increased gap junction coupling.

Fluorescence Recovery After Photobleach (FRAP) was used to quantify astrocyte gap junction coupling from tissues surgically resected from medically intractable epilepsy patients. Mesial temporal lobe cases provided hippocampus, surrounding hyperexcitable parahippocampus and normal cortex for culture. Cortical tumor cases yielded astrocytoma proper, cortex margins with normal EEG activity, and hyperexcitable cortex. Cells isolated from cortex surrounding astrocytomas and the parahippocampus surrounding the hippocampus showed an increase in glutamate-induced Ca2+ oscillations and intercellular Ca2+ waves. Gap-junction coupling was more pronounced in cells isolated from hyperexcitable tissue than from normal tissues as judged by their faster and more complete fluorescence recovery from laser bleach [FRAP]. This data suggests that intractable epilepsy may be associated with alterations in glial gap junction coupling.

Astrocytes↗

Malignant progression in pleomorphic xanthoastrocytoma: personal experience and review of the literature.

Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor, recently classified as a neuroglial tumor. It generally occurs in children and young adults and shows benign behaviour (WHO II), although an anaplastic variant and malignant potential have been described. Pleomorphic xanthoastrocytomas with malignant transformation have been reported in three out of eight patients operated on for this type of tumor in our department in the last 15 years. The three patients were two adult women and a child, the primary tumors were located in the cortex of the right temporal lobe, and treatment consisted of complete surgical resection. Histological examination revealed simple PXA in two patients and a PXA with anaplastic foci in the other. Mean recurrence time was 5.7 years, with the original xanthoastrocytoma evolving to glioblastoma in two cases and anaplastic astrocytoma in the third. All three patients underwent a second operation, followed by adjuvant therapies. Two died from tumor progression and one from brain edema after intracerebral haemorrhage. A review of the available PXA literature dating back to 1979 revealed 16 cases of primary anaplastic astrocytoma and 21 cases of PXA with malignant transformation. Our experience adds three more cases of malignant transformations, outlining once again the potential malignancy of pleomorphic xanthoastrocytomas and the fact that prognosis in these cases is the same as for primary anaplastic astrocytoma and glioblastoma. Analysis of glioneuronal markers, Ki67 and p53 in all pleomorphic xanthoastrocytomas did not prove to be a discriminating factor to identify a subgroup of xanthoastrocytomas prone to malignancy. Accordingly, these tumors demand close long-term clinical and radiological follow-up.

Adult↗

Amygdala damage impairs emotion recognition from music.

The role of the amygdala in recognition of danger is well established for visual stimuli such as faces. A similar role in another class of emotionally potent stimuli -- music -- has been recently suggested by the study of epileptic patients with unilateral resection of the anteromedian part of the temporal lobe [Gosselin, N., Peretz, I., Noulhiane, M., Hasboun, D., Beckett, C., & Baulac, M., et al. (2005). Impaired recognition of scary music following unilateral temporal lobe excision. Brain, 128(Pt 3), 628-640]. The goal of the present study was to assess the specific role of the amygdala in the recognition of fear from music. To this aim, we investigated a rare subject, S.M., who has complete bilateral damage relatively restricted to the amygdala and not encompassing other sectors of the temporal lobe. In Experiment 1, S.M. and four matched controls were asked to rate the intensity of fear, peacefulness, happiness, and sadness from computer-generated instrumental music purposely created to express those emotions. Subjects also rated the arousal and valence of each musical stimulus. An error detection task assessed basic auditory perceptual function. S.M. performed normally in this perceptual task, but was selectively impaired in the recognition of scary and sad music. In contrast, her recognition of happy music was normal. Furthermore, S.M. judged the scary music to be less arousing and the peaceful music less relaxing than did the controls. Overall, the pattern of impairment in S.M. is similar to that previously reported in patients with unilateral anteromedial temporal lobe damage. S.M.'s impaired emotional judgments occur in the face of otherwise intact processing of musical features that are emotionally determinant. The use of tempo and mode cues in distinguishing happy from sad music was also spared in S.M. Thus, the amygdala appears to be necessary for emotional processing of music rather than the perceptual processing itself.

Adult↗

Drug treatment of epilepsy: when does it fail and how to optimize its use?

Although modern community-based studies have shown that a majority of people with newly diagnosed epilepsy will enter long-term remission, seizures remain refractory to treatment in a substantial proportion of this population--perhaps as much as 40%. A consensus is being reached that, for operational purposes, pharmacoresistance can be suspected when two appropriately chosen, well-tolerated, first-line antiepileptic drugs (AEDs) or one monotherapy and one combination regimen have failed due to lack of efficacy. Poor prognostic factors include lack of response to the first AED, specific syndromes, symptomatic etiology, family history of epilepsy, psychiatric comorbidity, and high frequency of seizures. These observations suggest that prognosis can often be determined early in the course of the disorder. We propose a management paradigm that aims to maximize the chance of successful AED therapy, including the early use of "rational polytherapy" for patients not responding to monotherapy, and to identify efficiently patients suitable for "curative" resective surgery, in particular those with mesial temporal lobe epilepsy. An orderly approach to each epilepsy syndrome will optimize the chance of perfect seizure control and help more patients achieve a fulfilling life.

Adolescent↗

Functional specificity in the right human auditory cortex for perceiving pitch direction.

Previous lesion and functional imaging studies in humans suggest a greater involvement of right rather than left auditory cortical areas in certain aspects of pitch processing. In the present study, adaptive psychophysical procedures were used to determine auditory perceptual thresholds in 14 neurologically normal subjects, and in 31 patients who had undergone surgical resection from either the right or left temporal lobe for the relief of intractable epilepsy. In a subset of the patients, the lesion encroached significantly upon the gyrus of Heschl or its underlying white matter as determined from MRI analysis. Subjects were asked to perform two different perceptual tasks on the same set of stimuli. In a pitch discrimination task, the subject had to decide whether two elements of a pure tone pair were the same or different. In a task requiring the judgement of direction of pitch change, subjects decided whether pitch rose or fell from the first tone to the second. Thresholds were determined by measuring the minimum pitch difference required for correct task performance. Mean thresholds in the pitch discrimination task did not differ between patient groups and control subjects. In contrast, patients with temporal lobe excisions that encroached upon the gyrus of Heschl in the right hemisphere (but not in the left) showed significantly elevated thresholds when judging the direction of pitch change. These findings support a specialization of function linked to right auditory cortical areas for the processing of pitch direction, and specifically suggest a dissociation between simple sensory discrimination and higher order perception.

Adult↗

[Dysembryoplastic neuroepithelial tumor. Case report].

Dysembryoplastic neuroepithelial tumor (DNT) is a relatively new neuroepithelial neoplasm that was first described by Daumas-Duport et al. in 1988. It was incorporated in the revised World Health Organization classification of brain tumors in 1993. The authors present the first case of DNT reported in the Latin-American literature. A 16-year-old woman had been found at age of five years to have complex partial seizures accompanied by secondary generalization. She was treated with various anti-epileptic drugs but always with incomplete control of seizures activity. Neurological examination was normal. Magnetic resonance imaging demonstrated a lesion in the left temporal lobe. She underwent a craniotomy with complete resection of the tumor. Histological study showed atypical neuroglial proliferation characteristic of DNT. Monoclonal antibody immunostaining for glial fibrillary acidic protein and S-100 protein confirmed the glial component, and neuronal specific enolase and synaptophysin emphasized the neuronal component.

Adolescent↗

When is imaging enough?

Despite tremendous recent advances in both structural and functional neuroimaging, these tests alone are not enough to recommend surgery for medically refractory epilepsy. A detailed history, careful description of the ictal events, and the neurological examination will always be necessary. Interictal EEG and neuropsychological testing also add important information. In many situations, however, video-EEG monitoring of habitual seizures now plays a confirmatory, rather than a primary, role in the presurgical evaluation, and can occasionally contribute confounding data that delay or prevent surgical intervention. In the present climate of cost-containment, it is reasonable to ask if this expensive diagnostic tool is always needed when it is clear by other means that seizures are not psychogenic, particularly in certain pediatric surgically remediably syndromes such as those catastrophic epilepsys of infants and young children amenable to hemispherectomy, and drop attack that can be treated with corpus callosotomy, as well as mesial temporal lobe epilepsy and seizures due to discrete resectable lesions that often become medically intractable in adolescence. Retrospective studies should be carried out to determine when long-term, in-patient, video-EEG monitoring in these conditions add information that appropriately changes the therapeutic approach.

Adolescent↗

Primary intracerebral malignant melanoma with leptomeningeal spread, extradural extension and lung metastasis.

A rare primary intracerebral solitary malignant melanoma with extracranial metastasis is reported. The patient was a 66-year-old man who was revealed to have a tumor in the right temporal lobe by MRI. The tumor was totally resected and the histopathological diagnosis was malignant melanoma. Despite postoperative treatment with chemo-radiotherapy, tumor recurred with multiple subarachnoid dissemination. Complete autopsy showed marked leptomeningeal dissemination of tumor in the cerebro-spinal compartment. In the outside of the central nervous system, a microscopic metastatic nodule was found in the right lung. Metastatic tumor in the spinal cord showed the extradural extension which might have led to hematogenous metastasis to the lung.

Aged↗

Corpora amylacea: a marker for mesial temporal sclerosis.

Mesial temporal sclerosis (MTS) is the most frequently encountered abnormality in temporal lobectomies performed for medically intractable seizure disorders. The pathologic diagnosis of MTS relies on the identification of neuronal loss affecting various regions of the hippocampus. However, neuronal loss is often difficult to assess, particularly in lobectomies that are not performed en bloc. Because of this difficulty the presence of hippocampal pathology is often indeterminate. In this report we describe our experience with 73 temporal lobectomies performed for seizure disorders. In 58%, increased numbers of corpora amylacea (CoA) were found in association with MTS. The relationship between CoA and the pathogenetic mechanisms underlying MTS remains speculative. However, the association between MTS and corpora amylacea is important to recognize since the identification of abundant numbers of CoA provides a marker for MTS that can be useful in cases in which neuronal loss and gliosis are difficult to assess. For this purpose, it is strongly recommended that tissues resected from the hippocampus and amygdala for temporal lobe epilepsy be stained with LFB-PAS to highlight CoA.

Adolescent↗

Association of pleomorphic xanthoastrocytoma with cortical dysplasia and neuronal tumors. A report of three cases.

BACKGROUND: Several recent publications indicate that pleomorphic xanthoastrocytoma (PXA) may occasionally express ganglionic cell differentiation, linking this type of tumor to other benign, mixed glioneuronal neoplasms. Furthermore, ganglionic tumors and less frequently some benign glial tumors in the central nervous system, could be associated with a variety of mild developmental abnormalities of the cerebral cortex that are classified under the broad term cortical dysplasia. The association of cortical dysplasia with PXA and a neuronal pattern of differentiation has not been reported previously. METHODS: The authors present clinical, radiologic, immunohistochemical, and ultrastructural findings in three patients with cortical dysplasia. The cortical dysplasia was continuous with PXA, displaying an additional component of neuronal cell differentiation. RESULTS: The ages of the patients were 23, 47, and 52 years. Resection of the tumors was predated by temporal lobe seizures by 6 years in 1 patient and by more than 30 years in the others. In all 3 cases, radiologic studies conducted 3-6 years before surgery showed either no abnormality or lesions that were initially nonprogressing. CONCLUSIONS: The longstanding clinical history of seizures and the existence of radiologically documented dormant lesions several years before tumor removal both suggest that PXA in these patients very likely developed in benign hamartomatous cortical lesions or in preexisting cortical dysplasia. The authors' findings, along with the consistent occurrence of PXA in the gray matter in the majority of the reported cases, indicate that these tumors may originate either from a subclass of astrocytes histogenetically and topographically associated with neurons or from multipotential neuroectodermal precursor cells common to neurons and astrocytes.

Adult↗

Disseminating histologically benign multiple papilloma of the choroid plexus: case report.

A rare case of extensively disseminating multiple benign choroid plexus papilloma is shown. The patient first reported with high-grade hydrocephalus and two tumours in the 3rd and 4th ventricle was treated by atrioventricular shunt insertion, subtotal resection of the 4th ventricle tumour, and adjuvant 60Co irradiation of the posterior fossa. The dissemination that followed was revealed by computerised tomography and magnetic resonance imaging, and involved both the supra- and infratentorial ventricular systems, spinal canal, and brain parenchyma. Three years after the resection of the 4th ventricle tumour, the patient underwent excision of a temporal lobe lesion for relief of neurological symptoms, but showed no improvement and died 5 years after the primary diagnosis of CNS tumour. An autopsy was not performed. Analysis of the primarily resected mass showed distinct papillary pattern with no anaplasia, mitoses, multinucleation orgiant cell formation, and cytokeratin positivity at the absence of vimentin and glial fibrillary acidic protein. Analysis ofthe temporal lobe tumour again showed definite papillary formation with no signs of malignisation and virtually no mitotic figures, and the presence of cytokeratin, but not vimentin or glial fibrillary acidic protein. On both occasions, the diagnosis was choroid plexus papilloma (WHO grade I).

Brain↗

Late-onset drop attacks in temporal lobe epilepsy: a reevaluation of the concept of temporal lobe syncope.

We report the clinical, radiologic, and EEG features of six patients with temporal lobe drop attacks (TLDA), all of whom underwent temporal resection. Postoperative follow-up of at least 1 year was available in all. TLDA were never the first manifestation but followed the onset of epilepsy after a long delay ranging from 7 to 43 years (mean, 24.4 years). Seizures were of unilateral temporal origin. In one patient, stereo EEG recording of TLDA showed rapid spread of the ictal discharge away from the temporal lobe in less than 1 second. Postoperatively, three patients were seizure free; one has had no TLDA but experiences sporadic auras; another, despite a reduction of more than 50%, continues to have complex partial seizures and TLDA; and the sixth has had sporadic secondarily generalized seizures upon reduction of antiepileptic medication. In conclusion, drop attacks may occur in temporal lobe epilepsy, usually long after the onset of epilepsy. They lead to increased disability and suggest a rapid spread of the ictal discharge and possible involvement of the pontine reticular formation rather than the presence of bitemporal foci or an extratemporal origin.

Adult↗

Reduction of herniated temporal lobe in patients with severe head injury and uncal herniation.

Thirty-two patients with severe head injury (Glasgow coma scale 5-7/15) and uncal herniation were treated surgically from January 1988 to June 1990. Reduction of the herniated temporal lobe in addition to classical surgical procedures (craniotomy with evacuation of hematoma and resection of the contused brain) was performed in 10 patients (group A). The remaining 22 patients (group B) were treated similarly but without reduction of the herniated temporal lobe. In group A, there was one operative mortality. Eight patients made a rapid and complete recovery of pupil size and light reflex within two days after the operation, and one recovered within two months after the operation. The recovery of motor strength was also rapid in these nine patients. After three to 20 months (average, 11 months) of follow-up, the outcome was good in two patients, and the other seven were moderately disabled. In group B, 12 patients (55%) died after the operation. When compared with group A, the surviving 10 patients made a slower and less satisfactory recovery of oculomotor nerve function and motor status, and had a worse outcome after four to 25 months (average, 14 months) of follow-up (moderately disabled, six patients; severely disabled, two patients; vegetative, two patients). From my experience, it appears that the procedure of reduction is simple and seems to result in a more rapid and complete recovery of oculomotor nerve function and motor status, and it may even contribute to a better overall outcome.

Adult↗

Temporal lobectomy with amygdalectomy and minimal hippocampal resection: review of 100 cases.

The evidence for the role of the amygdala in temporal lobe seizures is supported by this follow-up (2-20 years) of 100 patients who were treated surgically by excision of the antero-lateral temporal cortex, most or all of the amygdala and minimal resection of the hippocampus. The findings showed 53 patients seizure-free or with rare or occasional seizures, 10 patients with marked seizure reduction and 37 with moderate or less reduction of seizures. The results are the same as in another 100 patients where, in addition, half or more of the hippocampus was resected.

Amygdala↗