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Preoperative predictors of anterior temporal language areas.

OBJECT: Although it is known that 5 to 10% of patients have language areas anterior to the rolandic cortex, many surgeons still perform standard anterior temporal lobectomies for epilepsy of mesial onset and report minimal long-term dysphasia. The authors examined the importance of language mapping before anterior temporal lobectomy. METHODS: The authors mapped naming, reading, and speech arrest in a series of 67 patients via stimulation of long-term implanted subdural grids before resective epilepsy surgery and correlated the presence of language areas in the anterior temporal lobe with preoperative demographic and neuropsychometric data. Naming (p < 0.03) and reading (p < 0.05) errors were more common than speech arrest in patients undergoing surgery in the anterior temporal lobe. In the approximate region of a standard anterior temporal lobectomy, including 2.5 cm of the superior temporal gyrus and 4.5 cm of both the middle and inferior temporal gyrus, the authors identified language areas in 14.5% of patients tested. Between 1.5 and 3.5 cm from the temporal tip, patients who had seizure onset before 6 years of age had more naming (p < 0.02) and reading (p < 0.01) areas than those in whom seizure onset occurred after age 6 years. Patients with a verbal intelligence quotient (IQ) lower than 90 had more naming (p < 0.05) and reading (p < 0.02) areas than those with an IQ higher than 90. Finally, patients who were either left handed or right hemisphere memory dominant had more naming (p < 0.05) and reading (p < 0.02) areas than right-handed patients with bilateral or left hemisphere memory lateralization. Postoperative neuropsychometric testing showed a trend toward a greater decline in naming ability in patients who were least likely to have anterior language areas, that is, those with higher verbal IQ and later seizure onset. CONCLUSIONS: Preoperative identification of markers of left hemisphere damage, such as early seizure onset, poor verbal IQ, left handedness, and right hemisphere memory dominance should alert neurosurgeons to the possibility of encountering essential language areas in the anterior temporal lobe (1.5-3.5 cm from the temporal tip). Naming and reading tasks are required to identify these areas. Whether removal of these areas necessarily induces long-term impairment in verbal abilities is unknown; however, in patients with a low verbal IQ and early seizure onset, these areas appear to be less critical for language processing.

Adolescent↗

Spontaneous hemorrhage associated with a pilomyxoid astrocytoma. Case report.

Pilomyxoid astrocytomas have been identified as a more aggressive variant of pilocytic astrocytomas that occur in infants and in young children. These tumors are characterized by a perivascular arrangement of pilocytic cells that has a monomorphous architecture with uniform, elongated bipolar cells loosely ranged within a prominent myxoid background. The authors present the case of a 24-year-old man with a pilomyxoid astrocytoma of the temporal lobe, who presented with a hemorrhage. The patient underwent gross-total tumor resection, and no evidence of residual or recurrent tumor was found on magnetic resonance images at the 6-month follow-up examination. The occurrence of a pilomyxoid astrocytoma in an adult suggests that this tumor is not limited to children. The tumor described in this report is unique because of its presentation with a hemorrhage, which has not been previously described in cases of pilomyxoid astrocytomas and is rarely found in those of pilocytic astrocytomas. Although this tumor predominantly exhibited the pattern of a pilomyxoid astrocytoma, there was a small focus of pilocytic astrocytoma, indicating that there is a spectrum of histological components found in these tumors and that certain elements may be associated with a more aggressive phenotype. In this paper the authors review the literature on pilomyxoid astrocytomas and discuss the unique aspects of this particular tumor presentation.

Adult↗

[Importance and limitations of the validation of functional MRI of motor function and language using preoperative cortical stimulation].

UNLABELLED: This chapter describes and discusses the value of the localization of functional areas obtained from functional MRI in brain tumor cases. Correlation method is cortical brain mapping by intraoperative stimulation. The experience reported here is focused on the study of motricity and language. METHODS: Twenty two patients with tumors of the rolandic region (n = 16) or in the temporal lobe (n = 6) underwent functional MR mapping and subsequently cortical mapping before tumor resection. The tasks chosen were a flexion and extension of the fingers or a naming task. We used 3D reconstructed images of the surface of the brain to assess intra and post operatively the functional MRI and stimulation data. RESULTS: For the motor correlation, in each case, the results of direct cortical mapping matched those obtained with functional MRI, both positively and negatively, although the extent of the functional activations was larger than the area required to elicit the corresponding movement during intraoperative brain mapping. For the language correlation and for the task chosen, only the results of the precentral areas matched those of functional MRI. CONCLUSIONS: Functional MRI can be used preoperatively to assess motor functional area in patients with rolandic tumors. More studies are needed to validate intraoperatively the language areas and the real extent of functional MRI activations. Finally, the observed discrepancy between functional MRI and cortical stimulation is likely due to the rather profound differences between both techniques, in terms of neurophysiology, practical applications and statistical analysis.

Adult↗

[Surgery for epilepsy: evaluation and indication].

Surgery for epilepsy is effective for the controlling of seizures in patients with pharmacoresistant forms. To screen candidates for surgery, diagnosis must be certain sure and the form must be drug resistant. Seizures must interfere with quality of life and the patient must be medically and psychologically stable. Selecting patients for surgery demands meticulous evaluation, including prolonged video-EEG to record seizures, magnetic resonance and, often, additional function imaging tests and neuropsychological examination. The most frequently performed surgery and the type with the best results is temporal lobectomy in patients with complex partial seizures arising in the mesial temporal lobe. Other techniques, such as hemispherectomy, callostomy or reduced cortical or extratemporal lobar resection, can yield improved or total control over seizures in certain patients.

Brain↗

Epilepsy surgery in developing countries.

Epilepsy surgery (ES) is a well-accepted treatment for medically intractable epilepsy patients in developed countries, but it is highly technology dependent. Such technology is not usually available in developing countries. For presurgical evaluation, magnetic resonance imaging (MRI) and electroencephalogram recording while videotaping the patient have been important. High technology equipment will, in conjunction with MRI, identify approximately 70% of ES candidates. Introducing ES into developing countries will require determining the candidates that are appropriate for the existing medical infrastructure. This article reviews ES and its possible introduction into conditions existing in developing countries. The authors address (a) the types of patients to be considered for resective ES (some patients require a fairly standard series of noninvasive studies: others will require extensive invasive studies), (b) ways to determine which patients might be appropriate for the existing situation (unilateral mesial temporal lobe epilepsy detected with MRI, epilepsy with a circumscribed MRI lesion, hemispheric lesions, circumscribed MRI detected neuronal migration, and development disorders), (c) surgical procedures (local resection, functional hemispherectomy, multiple subpial transections, corpus callosotomy, and implantation of a vagal nerve stimulator), (d) special considerations for introducing ES into developing countries (medical infrastructure, technology, seizure monitoring systems, selective intracarotid/carotid Amytal testing, and surgical equipment), and (e) the limitations, realistic expectations, personnel requirements, and educational function for selected professionals. Delivery of the technology and expertise to perform ES in developing regions of the world is a realizable project, but it would be limited by available technology and existing medical infrastructure. It should be possible in most areas to train local personnel and thereby leave a lasting legacy.

Cerebral Cortex↗

Anterior temporal language areas in patients with early onset of temporal lobe epilepsy.

Eighteen consecutive patients undergoing dominant temporal lobectomy underwent preoperative cortical stimulation for language localization. Patients with naming deficits on anterior (4.5 cm from the temporal pole) temporal lobe stimulation had earlier seizure onset vs those without such deficits (5.8 yr vs 12.9 yr; p < 0.04). There was a similar trend for reading errors (6.3 yr vs 12.4 yr; p < 0.052). Resections always spared at least 1 cm anterior to any language area. There was no significant difference in postoperative neuropsychological tests between patients with and without anterior language representation. Early onset of dominant temporal lobe seizure foci leads to a more widespread or atypical distribution of language areas. Individual variability should be considered in epilepsy surgery to reduce postoperative language deficits.

Adolescent↗

Temporal lobe focal cortical dysplasia: MRI imaging using FLAIR shows lesions consistent with neoplasia.

Focal cortical dysplasia (FCD), a form of neuronal migration disorder, is a malformative lesion of the neocortex that occurs during development of the brain. It can cause partial and generalized epilepsy. Seizures occur at an early age and are often resistant to medication. Surgical resection has been found to be beneficial in these patients. Dual pathology, in the form of mesial temporal sclerosis, has been associated with FCD. At the Children's Hospital of Eastern Ontario, four patients with temporal lobe FCD have recently, been identified. This paper discusses how these children presented and how they were managed, with particular emphasis on their MRI findings and differential diagnoses. In three of the four patients neuroimaging studies showed lesions consistent with a neoplastic process because of the large volume and mass effect. Radiologically, FCD may mimic the MRI appearance of tumors, such as dysembryoplastic neuroepithelial tumors, primitive neuroectodermal tumors, gangliogliomas, oligodendrogliomas, and astrocytomas. These lesions are best visualized on fluid-attenuated inversion recovery (FLAIR) imaging, a technique that has recently become applicable in the clinical setting, as we help demonstrate in this series. With better MRI capability, milder forms of FCD and microdysplasia may be distinguished.

Brain Diseases↗

Localizing value of epileptic visual auras.

It is difficult to differentiate between seizures of occipital or temporal lobe origin in patients with focal epileptic seizures associated with visual aura. These are often suspected to originate from the visual cortex, which causes reluctance to propose resection as treatment for the affected patient. To determine the value of localizing different types of visual auras, we report on 20 patients experiencing visual aura from a series of 878 surgically treated patients suffering from intractable focal seizures. In all of these patients, a morphological abnormality was identified on MRI (n = 18) or cranial CT (n = 2). These abnormalities were shown to represent the morphological correlate of the epileptogenic zone in each case, as demonstrated by intracranial ictal EEG recordings and/or seizure freedom after focal resective surgery. Elementary hallucinations, illusions and visual loss were reported not only by all patients with occipital lobe epilepsy, but also by patients with occipitotemporal and anteromedial temporal seizure onset. Complex hallucinations never occurred in occipital lobe seizures but were present in the two other groups. The same correlation was found for concentric changes of visual field (tunnel vision), a newly described ictal phenomenon. We conclude that elementary hallucinations, illusions and visual loss, although typical for occipital lobe epilepsy, can also occur in anteromedial temporal or occipitotemporal seizures and are therefore not a discordant feature in presurgical evaluation of patients with suspected temporal lobe epilepsy. Complex hallucinations and tunnel vision, however, should be considered concordant only with the assumption of an anteromedial temporal or occipitotemporal seizure onset.

Adolescent↗

Preoperative EEG predicts memory and selective cognitive functions after temporal lobe surgery.

Preoperative and postoperative cognitive and memory functions, psychiatric outcome, and EEGs were evaluated in 32 epileptic patients who underwent temporal lobe surgery. The presence and location of preoperative slow wave focus in routine EEG predicted memory functions of the non-resected side after surgery. Neuropsychological tests of the function of the frontal lobes also showed improvement. Moreover, psychiatric ratings showed that seizure free patients had significantly less affective symptoms postoperatively than those who were still exhibiting seizures. After temporal lobectomies, successful outcome in postoperative memory functions can be achieved in patients with unilateral slow wave activity in preoperative EEGs. This study suggests a new role for routine EEG in preoperative evaluation of patients with temporal lobe epilepsy.

Adolescent↗

Depth electrode studies in temporal lobe epilepsy: relation to quantitative magnetic resonance imaging and operative outcome.

We performed a retrospective study of 30 patients with presumed intractable temporal lobe epilepsy (TLE) who underwent chronic intracranial EEG monitoring (CIEM). Multicontact depth electrodes were stereotactically implanted through the medial occipital lobe into amygdala and hippocampus. All patients had previously undergone extracranial ictal EEG monitoring that proved inadequate to localize the epileptogenic zone. No morbidity was associated with CIEM in the 30 patients. Twenty-five patients were shown to have exclusively or predominantly unilateral temporal lobe seizures, and 5 patients had bitemporal seizures without unilateral predominance; 24 patients subsequently underwent an anterotemporal lobe cortical resection. Twenty-one patients have been followed a minimum of 1 year postoperatively. Nine patients (43%) had a class I outcome (seizure-free, auras only, or provoked seizures), 3 patients (14%) had a class II outcome (> or = 95% seizure reduction), 4 patients (19%) had a class III outcome (> or = 50% seizure reduction); and 5 patients (24%) had a class IV outcome (< 50% seizure reduction or no change). A prolonged interhemispheric propagation time (p < 0.01) and magnetic resonance imaging (MRI)-identified hippocampal atrophy (p < 0.01) correlated with a favorable surgical outcome. Results of this study may prove useful in counseling patients who undergo CIEM before temporal lobe surgery.

Adolescent↗

Predictors of outcome and pathological considerations in the surgical treatment of intractable epilepsy associated with temporal lobe lesions.

OBJECTIVES: To evaluate the influence of clinical, investigative, and pathological factors on seizure remission after temporal lobectomy for medically intractable epilepsy associated with focal lesions other than hippocampal sclerosis. METHODS: From a series of 234 consecutive "en bloc" temporal resections for medically intractable epilepsy performed between 1976 and 1995, neuropathological examination disclosed a focal lesion in 80. The preoperative clinical, neuropsychological, interictal EEG, and neuroimaging characteristics of these patients were assembled in a computerised database. The original neuropathological material was re-examined for lesion classification and completeness of removal. The presence of additional cortical dysplasia and mesial temporal sclerosis was also noted. Survival analysis was performed using Kaplan-Meier curves and actuarial statistics. Logistic regression analysis was used to establish the independent significance of the clinical variables. RESULTS: The probability of achieving a 1 year seizure remission was 71% by 5 years of follow up. Factors predicting a poor outcome on multivariate analysis included the need for special schooling and a long duration of epilepsy. Generalised tonic-clonic seizures, interictal EEG discharges confined to the resected lobe, demonstration of the lesion preoperatively on CT, and complete histological resection of the lesion were not predictive of outcome. Neuropsychological tests correctly predicted outcome in left sided cases but apparently congruent findings in right sided resections were associated with a poor outcome. Pathological reclassification established the dysembryoplastic neuroepithelial tumour as the commonest neoplasm (87%) in this series, with a significantly better seizure outcome than for developmental lesions, such as focal cortical dysplasia. CONCLUSIONS: The findings highlight the importance of dysembryoplastic neuroepithelial tumour in the pathogenesis of medically refractory lesional temporal lobe epilepsy and the prognostic significance of preoperative duration of epilepsy emphasises the need for early recognition and surgical treatment. Cognitive and behavioural dysfunction, however, is associated with a lower seizure remission rate, independent of duration of epilepsy.

Adolescent↗

Total resection of an intracerebral hemangioendothelioma in an infant. Case report and review of the literature.

Hemangioendotheliomas (HEs) are vasoformative tumors rarely seen in the CNS. Histopathological features determining aggressive phenotypes have not been well defined. Potential cytogenetic alterations in these tumors have not been previously reported. We present a 4-month-old male infant with a temporal lobe HE. Fluorescence in situ hybridization and spectral karyotype analysis revealed translocation of chromosome 11q23. This case constitutes the first report of a gross total resection of an intracerebral HE in the pediatric age group, and of potential cytogenetic alterations involved in its pathogenesis. Based on our report and review of the literature, we note a discrepancy between histopathological criteria of aggressive tumor biology and clinical behavior. Further study of chromosomal abnormalities may be helpful in defining factors associated with a higher risk of malignant behavior. We conclude that gross total resection is currently the best available treatment option for these tumors, regardless of histology.

Biomarkers, Tumor↗

Arteriovenous malformations of the medial temporal lobe. Surgical approach and neuroradiological characterization.

Arteriovenous malformations (AVM's) of the medial temporal lobe frequently involve the basal ganglia and the thalamus and, for this reason, are commonly judged to be inoperable. However, when the medial involvement is limited to the posterolateral part of the thalamus and to the inferior portion of the basal ganglia lateral to the internal capsule, the lesions may be excised safely. Three patients who underwent successful excision of AVM's of this region are presented. A transcortical surgical approach through the inferior portion of the temporal lobe was used to minimize retraction of the temporal lobe and damage to the optic radiation, and to avoid postoperative dysphasia. The following neuroradiological criteria indicate that the bulk of the lesion is in the temporal lobe that only noncritical portions of the basal ganglia and thalamus are involved, and that therefore, surgical resection is relatively safe: 1) primary supply by the anterior choroidal artery and by laterally oriented branches of the posterior cerebral artery; 2) primary venous drainage into the basal vein of Rosenthal and medial Sylvian veins; 3) projection below the plane of the middle cerebral artery in the lateral carotid arteriogram; 4) projection lateral to the sweep of the posterior cerebral artery in the anteroposterior or Towne's view of te vertebral angiogram; and 5) demonstration of an intratemporal clot or intraventricular blood by computerized tomography.

Adult↗

Epileptic nystagmus in infancy.

Epileptic nystagmus (EN) is a rare form of nystagmus that occurs only during epileptic seizures. We report an infantile case in which EN was first noted at 10 days of age. Electronystagmography showed a right-beating nystagmus with predominantly linear slow phases that traversed the midline. Neuro-imaging revealed dysplasia of the left middle temporal gyrus extending posteriorly into the parieto-occipital cortex. The right hemisphere and subcortical structures appeared normal. Perfusion studies demonstrated interictal hypoperfusion with ictal hyperperfusion in the left temporal lobe. Electrocorticography demonstrated spiking over the left temporal-parieto-occipital region. Following extensive surgical resection of this area and weaning of anti-convulsants, the child has remained seizure-free without nystagmus. This case demonstrates the cortical origin of EN, and shows that infant cortex has functioning efferent connections to brainstem oculomotor centres from 10 days of age.

Electroencephalography↗

Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: clinicopathologic features and review of the literature.

We present a unique case of biopsy-proven necrotizing sarcoidosis involving the central nervous system (CNS) in a 52-year-old woman. The patient presented with a 3-month history of left-sided headache and sharp, shooting pains on the left side of her face. She also has a previous history of sarcoidosis, histopathologically confirmed on parotid gland biopsy 24 years before. Imaging studies of the present lesion revealed a 1.8 x 1.4-cm mass in the left temporal lobe with signal intensity suggestive of meningioma or low-grade glial neoplasm. Surgical resection was initiated, and intraoperative consultation with frozen sections revealed granulomata. The lesion was biopsied, and surgical intervention was terminated. Permanent sections failed to reveal bacteria, mycobacteria, fungi, or foreign bodies. A diagnosis of necrotizing neurosarcoidosis was rendered. The patient was administered steroid therapy and clinically responded favorably. At the most recent follow-up almost 2 years later, there was no evidence of recurrence or progression. Necrotizing sarcoidosis has been reported most commonly in the lungs and rarely in other organ systems. We report the first histologically proven case involving the CNS as well as a rare example of sarcoidosis and necrotizing sarcoid granulomatosis in the same patient. Sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of a granulomatous mass lesion involving the CNS, particularly in the context of a history of systemic disease.

Brain Diseases↗

Cerebral mass due to neurocutaneous melanosis: eight years later.

Neurocutaneous melanosis (NCM) is associated most commonly with giant congenital melanocytic nevi (CMN), in particular those on the scalp or in a posterior axial location that are accompanied by satellite congenital nevi. It also can occur in patients with multiple medium-sized CMN. In general, the prognosis of those with symptomatic NCM is poor, even in the absence of malignancy, while the prognosis of those with asymptomatic NCM detected via screening varies and is more difficult to predict. Herein we report an asymptomatic patient with a giant CMN and multiple satellite nevi who had a screening magnetic resonance imaging (MRI) study at age 5 months that showed a rounded area of increased signal in the right temporal lobe on T1-weighted images, suggestive of parenchymal melanosis. This melanotic mass was resected at age 10 months, and histologic examination of the surgical specimen showed prominent perivascular collections of benign, pigment-containing melanocytes within cerebral tissue. The patient remains healthy 8 years later. His excellent long-term outcome and other reports of NCM with localized central nervous system (CNS) involvement apparent on MRI may have implications for management, including early imaging of patients with high-risk CMN and potential surgical intervention for NCM.

Brain Neoplasms↗

Juvenile pilocytic astrocytoma of the cerebrum in adults. A distinctive neoplasm with favorable prognosis.

Seven adults (15 years of age or older) with juvenile pilocytic astrocytoma of the cerebrum were treated at Barnes Hospital in St. Louis, Missouri, from 1950 to 1981. This group comprised 7% of the total adult population with well differentiated astrocytoma and astrocytoma with anaplastic foci treated over a 30-year period. All seven patients were less than 30 years old at the time of presentation. The tumors were characterized by distinctive histology, a predilection for the temporal lobe, the presence of a major cyst, and relative gross circumscription making total surgical resection feasible. In contrast to patients with diffusely infiltrating cerebral astrocytoma, those with juvenile pilocytic astrocytoma have had an extremely favorable course, regardless of treatment. Four were treated with surgery alone after total gross tumor excision, and three received postoperative irradiation after subtotal excision. Six were alive 27, 24, 17, 9, 5, and 2 years after initial surgery. One patient died 7 years after surgery and radiation as a result of cerebral radiation necrosis. Juvenile pilocytic astrocytoma of the cerebrum is a recognizable, distinctive entity with natural history and pathological characteristics similar to the relatively benign cerebellar astrocytoma of childhood.

Adolescent↗

Transparahippocampal selective amygdalohippocampectomy in children and adolescents: efficacy of the procedure and cognitive morbidity in patients.

OBJECT: Unilateral resection of the hippocampus and amygdala can be used to treat medically intractable mesial temporal lobe seizures. To date seizure outcome and the extent of cognitive morbidity have been unknown in children following the transparahippocampal variation of selective amygdalohippocampectomy (TSA), which prompted the present prospective study. METHODS: Preoperative examinations and outcomes in 22 consecutive children and adolescents who underwent TSA were studied. Cognitive and psychological morbidity were assessed using standard neuropsychological instruments. The authors evaluated relationships between seizure control and cognitive morbidity and 13 and nine clinical variables, respectively. Seizure control was achieved in 11 (65%) of 17 patients (>2 years follow up). Among 13 clinical variables, the only preoperative finding that had a significant bearing on seizure control was the presence of unilateral hypometabolism, which could be observed on [18F]fluorodeoxyglucose-positron emission tomography scans (p<0.001). Patients with seizure control showed significant improvements in verbal and full scale intelligence quotients (both p = 0.05). Patients with longer preoperative durations of seizures exhibited more cognitive impairment that persisted postoperatively. Cognitive outcome analysis based on nine clinical factors revealed no significant difference in cognitive parameters postoperatively, except that significant improvement occurred in rote verbal memory scores among patients who underwent right-sided TSA (p = 0.01). Individually, 81% of the children achieved significant improvement in at least one of seven cognitive parameters, and 52% had stable or improved scores in all parameters. CONCLUSIONS: The results indicate that TSA is a safe effective approach for the treatment of medically intractable mesial temporal lobe seizures in children with minimum effect on cognitive morbidity. Given that the literature suggests that children suffer progressive cognitive morbidity from persistent seizures, the results of this study support early surgical intervention for this group of children.

Adolescent↗