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[Supratentorial primitive neuroectodermal tumor: a single center experience and comparison with the literature].

Supratentorial primitive neuroectodermal tumors (stPNETs) are malignant tumors. We saw within three years six children with stPNETs. In four of the six children radical resection could be achieved. All had craniospinal irradiation and chemotherapy according to the HIT-91 protocol. The two children with incomplete resection died due to tumor progression after 7 and 10 months. Two of the 4 children with complete tumor resection had local relapses 8 months after diagnosis and died after 14 and 18 months. One child had a diffuse meningeal relapse 12 months after diagnosis. Despite (high-dose) systemic chemotherapy and intraventricular mafosfamide, he died 21 months after diagnosis due to tumor although remission could be achieved. Only one child is still in remission 86 months after diagnosis.

Brain Neoplasms↗

Cognition and behavior after temporal lobectomy in pediatric patients with intractable epilepsy.

The increased use of surgical intervention for intractable epilepsy during childhood has resulted in a critical need for information concerning possible cognitive and behavioral changes in pediatric patients after surgery. In this pilot study, comprehensive neuropsychologic evaluations were completed on nine children who had a temporal lobectomy for intractable epilepsy before 16 years of age. Performances before and after surgery were compared using cognitive and behavioral measures. Repeated measures analysis of variance did not indicate differences in performance on the basis of laterality of surgery, although the number of left (n = 5) vs right (n = 4) temporal resections was small. Paired comparison t tests, which included all patients, did not suggest marked changes in cognitive functioning after surgery, although decreases in delayed verbal memory were evident. Positive effects on quality of life during the first year after surgical intervention were suggested by reduced internalizing symptoms and increased social interaction. Replication of this study is recommended with a larger number of patients and multicenter collaboration.

Adolescent↗

Neuropsychological aspects of epilepsy surgery.

Neuropsychological evaluation of a patient's cognitive capabilities before and after epilepsy surgery is essential in elective epilepsy surgery. On the one hand, neuropsychology provides accessory information regarding the localization and lateralization of epilepsy-associated cognitive impairment; on the other hand, it is a useful tool for quality and outcome control of epilepsy surgery which helps to make surgery more effective and safe. Evaluation of the adequacy of the brain tissues to be resected and of the patient's mental reserve capacities allows for a prediction of the postoperative cognitive development. Successful surgery can stop mental decline due to chronic epilepsy and it can reverse this negative trend by release of functions and capacities that were secondarily affected before surgery. However, surgery bears the risk of additional impairments which, in interaction with normal or even pathological processes of mental aging, may accelerate cognitive decline at an older age. From a neuropsychological point of view, early recognition of pharmacoresistance is important along with early and complete seizure control with maximal sparing of functional tissues.

Anterior Temporal Lobectomy↗

Failed surgery for epilepsy. A study of persistence and recurrence of seizures following temporal resection.

From a series of 282 consecutive temporal resections for medically intractable epilepsy associated with mesial temporal sclerosis (MTS), dysembryoplastic neuroepithelial tumour (DNT) or non-specific pathology (NSP), 51 patients had persistent or recurrent seizures occurring at least monthly. Of these patients, 44 underwent detailed assessment of their postoperative seizures, which included clinical evaluation, interictal and ictal EEG and high-resolution MRI. Of the 20 patients with MTS in the original pathology, 14 (70%) had postoperative seizures arising in the hemisphere of the resection, the majority (12 patients) in the temporal region. Although MRI demonstrated residual hippocampus in five of these 12 patients, only one patient was considered to have seizures arising there, whilst the remainder had electroclinical evidence of seizure onset in the neocortex. In contrast, five of the MTS relapses (25%) had seizure onset exclusively in the contralateral temporal region. Among the 14 patients with non-specific pathology, relapse was also predominantly from the ipsilateral hemisphere (64%), but more relapsed from extratemporal sites compared with the MTS cases, including two with NSP who had occipital structural abnormalities. Although 70% of the 10 patients with DNT had postoperative partial seizures arising in the ipsilateral hemisphere, many (60%) had evidence of a more diffuse disorder with additional generalized seizures, cognitive and behavioural disturbance and multifocal and generalized EEG abnormalities. Nine patients (20%) had immediate postoperative seizure-free periods of at least 1 year, and seven of these had MTS in the operative specimen. Of these seven patients, four had ipsilateral temporal seizures and three had contralateral temporal seizures. Overall, few missed lesions were discovered on postoperative MRI and reoperations were performed or considered possible in a minority of cases. Despite well-defined preoperative electroclinical syndromes of temporal lobe epilepsy, many patients relapsed unexpectedly, either immediately or remotely from the time of surgery. Maturing epileptogenicity in a surgical scar was not, however, considered to be a significant primary mechanism in patients who relapsed after a seizure-free interval.

Adolescent↗

Parameters for direct cortical electrical stimulation in the human: histopathologic confirmation.

Safe parameters for electrical cortical stimulation in humans are difficult to estimate from the animal experimental literature. We therefore examined the light microscopic histology at a total of 11 sites of direct subdural electrical stimulation, taken as part of anterior temporal lobectomies in 3 patients. Stimulations had been done through 3.175 mm diameter electrodes, with 0.3 msec square wave pulses of alternating polarity at 50 pulses/sec. In 2 patients, one site each had been used as a common reference for stimulation, receiving over 251 stimulation trials, most of 2-5 sec duration, at currents of 12.5-15.0 mA, 1 day prior to resection. The maximum charge per phase was 4.0-4.4 microC; the maximum charge density was 52-57 microC per geometric cm2 per pulse at the electrode surfaces. Comparison of hematoxylin and eosin, periodic acid-Schiff, and cresyl violet-stained material from the electrode sites with that from other regions did not show any histologic abnormalities attributable to the electrical stimulation. The relatively brief and intermittent periods utilized for human stimulation testing do not appear to cause structural damage at the light microscopic level at charge densities that exceed the threshold for damage established in animal studies with more continuous, chronic stimulation schedules.

Electric Stimulation↗

[A case of primary meningeal melanocytoma of the left middle fossa].

A 29-year-old woman presented with a primary meningeal melanocytoma of the left middle fossa manifesting as headache and nausea. Computed tomography (CT) with contrast medium demonstrated a clearly demarcated, homogeneously enhanced high density area surrounded by a cyst in the left temporal lobe. Magnetic resonance (MR) imaging showed the mass as a slightly high signal intensity area on the T1-weighted image and as a low signal intensity area on the T2-weighted image. Cerebral angiography revealed shift of the middle cerebral artery but no tumor stain. The solid part of the tumor had rapidly increased in size with reduction of the cyst 3 months later. Left frontotemporal craniotomy disclosed a clearly demarcated jet-black tumor attached to the dura. The black-colored lesion in the dura and the bone extended to the skull base, so malignant melanoma was a possibility. The solid part of the tumor was gross totally removed, and the dura and the skull bone were preserved. Histological examination of the tumor specimen revealed meningeal melanocytoma. Melanophages were present in the specimen of the black-colored lesion in the dura and the bone, but no neoplastic infiltration was present. The postoperative course was uneventful, and the patient's headache and nausea disappeared. CT and MR imaging taken 1 month later confirmed total removal of the tumor. No recurrence has been observed for 2.5 years after surgery. Preoperative differentiation of meningeal melanocytoma from malignant melanoma is difficult, but the primary goal of therapy is gross total resection of the solid part of the tumor irrespective of tumor type.

Adult↗

Hearing preservation following a transtemporal resection of an acoustic schwannoma: a case report.

The transtemporal or translabyrinthine approach is generally regarded as the most reliable method of ensuring total tumor removal and preservation of facial nerve function in the resection of a cerebellopontine angle tumor. This is particularly true in removing a tumor that has a significant extension into the patient's internal auditory canal. An anticipated deficit associated with the approach is a total hearing loss in the patient's hearing on the operated side. We have encountered a patient, however, who was serially documented as having serviceable hearing after a transtemporal removal of an acoustic schwannoma. Plausible reasons for the functional preservation of some of this patient's hearing are included in the discussion.

Cerebellopontine Angle↗

Nucleus dorsalis superficialis (lateralis dorsalis) of the thalamus and the limbic system in man.

Although the earlier supposition was that the n. dorsalils superficialis (n. lateralis dorsalis) of the thalamus projected to the parietal region, more recent evidence has linked it to the posterior cingulate gyrus and possibly adjacent regions near the splenium of the corpus callosum. An afferent supply from lower levels was in more doubt, although some report had been made of cell and fibre degeneration in the n. dorsalis superficialis after extensive temporal resections and section of the fornix in lower primates. The five human hemispheres of the present study all had lesions of long duration below the level of the splenium of the corpus callosum in the posteromedial temporal region. All showed marked degeneration in the fornix and n. dorsalis superficialis. In favourably stained cases, gliotic fascicles could be followed from the descending column of the fornix to the n. dorsalis superficialis via the region lateral to the stria medullaris thalami. The cell loss in the nucleus thus appeared to be an instance of anterograde transynptic degeneration. These cases provided an interesting instance in which human infarctions provided natural lesions that would have been hard to duplicate in experimental animals.

Basal Ganglia↗

Selective coexpression of NMDAR2A/B and NMDAR1 subunit proteins in dysplastic neurons of human epileptic cortex.

NR1 and NR2 are the two gene families for the NMDA receptor. In vitro studies show that while NR2 alone is nonfunctional, NR1 alone produces weak currents to glutamate or NMDA. We previously showed by immunocytochemistry (ICC) that in normal appearing, nonepileptic human cortical neurons, only NR1 and not NR2 proteins were expressed, in contrast to the presence of both NR1 and NR2 in normal rat cortical neurons. We also showed, in dysplastic epileptic cortex, that both NR1 and NR2 were highly expressed using ICC on adjacent 30-microm sections. However, the relative coexpressions of NR1 and NR2 proteins in single neurons in single sections of human epileptic cortex were unknown. In this study, we used double-labeled immunofluorescence and confocal microscopy to examine the distribution and coexpression of subunit proteins for NR1 and NR2A/B in both nondysplastic (control comparison) and dysplastic regions of human brain resected for the treatment of intractable epilepsy (11 patients). In nondysplastic regions, cortical neurons did not have immunoreactivity (ir) for NR2A/B, whereas NR1-ir was abundant. By contrast, dysplastic neurons in the regions with epileptic cortical dysplasia showed intense NR2A/B-ir in the somata and their dendritic processes. These same NR2A/B-ir dysplastic neurons were colabeled by NR1. These results demonstrate directly that dysplastic neurons express both NR2A/B and NR1 proteins, whereas nondysplastic cortical neurons express only NR1 proteins. Selective coexpression of NR2A/B and NR1 in dysplastic neurons suggests that NR2A/B may form heteromeric NR1-NR2 coassemblies and hyperexcitability in dysplastic neurons that could contribute to focal seizure onset.

Animals↗

Predictors of outcome of epilepsy surgery: multivariate analysis with validation.

PURPOSE: To identify predictors of outcome of epilepsy surgery, using the Duke experience, applying multivariate analysis and validation techniques. To compare the results of different modeling algorithms. Few previous studies have reported multivariate analysis, or validated their results. METHODS: Records of 116 patients with focal resections for intractable epilepsy from January 1, 1980 through June 30, 1989 were analyzed. Primary outcome variable was patient's condition in second postoperative year: seizure free (except auras), or not. Three predictors of biologic interest were specified a priori for confirmatory analysis. Additional predictors were considered within exploratory analysis. Logistic regression techniques were applied to assess relations with pre- and postoperative predictors. Internal validity was assessed by repeated random selection of training and validation samples, used in conjunction with bootstrap techniques. RESULTS: By using multivariate analysis, percentage of epileptic EEG activity arising from the site of resection and either imaging localization or lack of use of invasive monitoring were the only statistically significant preoperative predictors for good outcome at 2 years. Presence of seizures within 2 months of surgery was a significant postoperative predictor for a poor outcome. Adding more variables did not result in significantly improved models. Use of validation techniques reduced the degree of optimism in the predictive value of the models. CONCLUSIONS: Pooling of data from multiple institutions is needed to attain the large sample sizes needed for multivariate analysis with validation.

Adolescent↗

[Children with drug-resistant partial epilepsy: criteria for the identification of surgical candidates].

The majority of candidates for epilepsy surgery, in both children and adults, belong to the syndromic category of partial epilepsies. However, particularly in children, the clinical expression of epilepsy may sometimes be misleading, as paroxysmal events may present as generalized seizures although having a focal onset. The spectrum of surgical possibilities for early-onset epilepsy has tended to widen rapidly and to include not only other focal epilepsies but also more difficult types. This is because developmental lesions that are the major cause of infantile epilepsy are often poorly localized, involve extensive brain areas and require extensive operations (e.g. hemispherotomy). Surgery is also used for progressive conditions such as Rasmussen's encephalitis or Sturge-Weber syndrome. Some forms of generalized symptomatic epilepsy may also benefit from palliative surgical procedures (callosotomy in cases with repetitive drop attacks often as a manifestation of a Lennox-Gastaut syndrome). The timing of surgery partly depends on the probable effectiveness of the operation available for each particular patient. Candidates for epilepsy surgery must be identified early in the process of the disease, thus increasing the chances for a satisfactory cognitive and behavioral outcome. The decision should be taken by experienced multidisciplinary groups, that will also ensure post-operative follow-up, both in terms of medical management and psycho-social integration. Prospective studies are still needed, to evaluate the long-term cognitive evolution of children operated on early for their epilepsy and cured. Increasing evidence indicates that drug therapy is likely to fail to achieve control of the seizures when two-three of the major drugs, properly chosen by competent clinicians, have not obtained satisfactory results, i.e. full control of seizures and absence of side effects. Testing of all possible drugs is not advisable, because this process would be excessively long when surgery is a reasonable possibility. The requirements for resective surgery in children with partial drug-resistant epilepsy vary with the type of resection considered. Three basic requirements apply to almost all cases: (a) the epileptogenic area must be localized to a territory whose removal is contemplated; (b) that no other independent epileptogenic area exists in those areas that are not included in the planned resection, and (c) that any possible deficit resulting from resection must be acceptable. The current experience confirms that the complete resection of the epileptogenic area is the major condition for a satisfactory surgical result. The possibilities for resective surgery depend upon the localization of the ictal onset zone, its relationship with adjacent functional brain areas and the availability of convergent data that point to a single localization. Epilepsy surgery mandates a multidisciplinary approach that requires special skills and sophisticated instruments and materials that cannot be improvised. A global evaluation of risks and expected benefits is always required. All decisions require a close collaboration between the epilepsy team, the patient and the family. With the exception of high quality MRI and video-EEG recording of seizures, the need to perform any other complementary presurgical investigation should be evaluated on an individual basis.

Anticonvulsants↗

Subdural and depth electrodes in the presurgical evaluation of epilepsy.

From 1987 to 1992, invasive EEG studies using subdural strips, grids or depth electrodes were performed in a total of 160 patients with medically intractable epilepsy, in whom scalp EEG was insufficient to localize the epileptogenic focus. Dependent on the individual requirements, these different electrode types were used alone or in combination. Multiple strip electrodes with 4 to 16 contacts were implanted in 157 cases through burrholes, grids with up to 64 contacts in 15 cases via boneflaps, and intrahippocampal depth electrodes in 36 cases using stereotactic procedures. In every case, localization of the electrodes with respect to brain structures was controlled by CT scan and MRI. Visual and computerized analysis of extra-operative recordings allowed the localization of a resectable epileptogenic focus in 143 patients (89%), who subsequently were referred for surgery, whereas surgery had to be denied to 17 patients (11%). We did not encounter any permanent morbidity or mortality in our series. In our experience, EEG-monitoring with chronically implanted electrodes is a feasible technique which contributes essentially to the exact localization of the epileptogenic focus, since it allows nearly artefact-free recording of the ictal and interictal activity. Moreover, grid electrodes can be used for extra-operative functional topographic mapping of eloquent brain areas.

Adolescent↗

Psychiatric outcome after temporal lobectomy: a predictive study.

PURPOSE: The occurrence of psychiatric symptoms after temporal lobectomy is well documented. The aim of the present study was to identify preoperative factors that predict postoperative psychiatric outcome. METHODS: We studied the case notes of 121 patients (from an initial sample of 167) who underwent temporal lobectomy at the National Hospital of Neurology and Neurosurgery, Queen Square, London, between 1988 and 1997. Data concerning gender, laterality of lesion, pathology, seizure outcome, psychiatric history, psychiatric outcome, resection volume, telemetry, and MRI scans were systematically collected. Factors that predict the occurrence of postoperative psychiatric symptomatology were investigated using correlational, chi(2), and logistic regression techniques. RESULTS: Poor postoperative psychiatric outcome in general was positively associated with preoperative bilateral independent spike discharges at telemetry. The size of surgical resection was positively correlated with the occurrence of postoperative emotional lability. The laterality of the epileptogenic lesion was not associated with a poor psychiatric outcome. Developmental lesions were associated with a good psychiatric outcome at a marginally significant level. Patients with a preoperative psychiatric history and de novo psychiatric symptomatology had a poorer surgical outcome in terms of seizure frequency, also at a marginally significant level. A significant correlation was found between a past psychiatric history and seizure outcome. CONCLUSIONS: We identified a high frequency of psychiatric symptoms both before and after temporal lobectomy, demonstrating that it is not a benign procedure from the point of view of psychopathology. Our results show that there are certain predictive factors that may help identify patients most at risk for postoperative psychiatric disorders.

Adult↗

Experience with orbital electrodes in the patients operated on for epilepsy--results of temporofrontal resections.

We introduced the orbital electrodes (Fb-frontobasal) in clinical practice in 1983. Since then we have operated on 112 patients for medically intractable "temporal" epileptic fits. In this series there were 45 patients (40%) with independent focal interictal EEG epileptic abnormalities over frontobasal cortex (with or without independent spiking over interomedial temporal region). In these patients we performed a small temporal lobectomy (with amygdaloectomy and resection of pes hippocampi) (TL) and resection of frontoorbital cortex (FOCR). The surgical results are as follows: There are 75 patients with follow-up one to 5 years. TL and FOCR were performed in 27 patients (36%). In this group seizure-free are 25 (93%) and 2 patients (7%) are much better. In the series of 48 patients examined by sphenoidal electrodes and operated for "temporal" fits (TL) by the same author (R.I.) (follow-up 5-13 years), seizure-free are 38 (79%), much better are 8 (17%) and unchanged are 2 (4%) patients. The difference in proportion of seizure-free patients in these two series is statistically significant (t = 2.53;p less than 0.05). Our results suggest that in some patients suffering with "temporal" seizures a well defined EEG picture suggests a new clinical entity the "temporo-frontal" epilepsy, the surgical treatment of which gives excellent results.

Amygdala↗

Gradual recovery from dyslexia and related serial magnetoencephalographic changes in the lexicosemantic centers after resection of a mesial temporal astrocytoma. Case report.

Letter-perception centers are not held in as high regard as motor- and language-related cortices during planning of neurosurgical procedures, and there have been no reports suggesting cortical reorganization of reading ability. The authors describe a patient with a left mesial temporal glioma in whom two letter-perception centers (the anterior portion of the left superior temporal gyrus and the left fusiform gyrus) were successfully localized before surgery by performing magnetoencephalography (MEG) during reading tasks. Control MEG examinations of 15 healthy volunteers were also performed to assist in a careful interpretation of patient results. Although a radical resection of the mesial temporal glioma, which involved the left fusiform gyrus, caused severe dyslexia, the patient's impaired reading skills improved gradually during a 1-year postoperative period. In the meantime, the spared left superior temporal gyrus displayed an overshot recovery of MEG responses. During the postoperative period there was no obvious recovery in MEG signals and no compensatory activity in the contralateral fusiform gyrus. This case demonstrates that lexicosemantic centers involved in the reading process can be noninvasively localized using MEG and that the results obtained are highly reliable for surgical planning. The results of the repeated MEG reflected sequentially the patient's recovery from dyslexia. This is the first report in which MEG studies have been shown to predict preoperatively the risk of dyslexia and demonstrate its serial physiological recovery.

Adult↗

Seizure outcome of lesionectomy in glioneuronal tumors associated with epilepsy in children.

OBJECT: Glioneuronal tumors (ganglioglioma, dysembryoplastic neuroepithelial tumors [DNTs]) are commonly associated with partial seizures. The optimal surgical treatment of such tumors, however, has not been fully established; it is still unclear whether lesionectomy itself can be used to control seizures or if epileptogenic areas adjacent to the tumor should also be removed. To address this uncertainty, the authors analyzed seizure outcome in a series of children with epileptogenic glioneuronal tumors that had been treated only by lesionectomy. METHODS: The authors retrospectively reviewed 15 children surgically treated for glioneuronal tumors associated with epilepsy. Patients ranged in age from 3 to 18 years (mean 12.6 years); there were 12 boys and three girls. The interval between onset of seizures and surgery ranged from 0.5 to 16 years (mean 6.1 years). Ten patients (66.6%) suffered complex partial seizures and five (33.3%) simple partial seizures. Seizure frequency varied from several per day to one per month. Nine tumors (60%) were temporal and six extratemporal; in all patients resection was limited to the tumor. The follow-up duration ranged from 1 to 11 years (mean 5.6 years). Gross-total removal was achieved in 13 patients and subtotal in two. The histological diagnosis was ganglioglioma in 11 cases and DNT in four. At last follow up 13 patients (86.6%) were Engel Class I, one was Engel Class II, and one was Engel Class III. CONCLUSIONS: The results of this study indicate that lesionectomy may provide good long-term seizure control in the majority of children with epileptogenic glioneuronal tumors.

Adolescent↗

Statistical mapping of scalp-recorded ictal EEG records using wavelet analysis.

PURPOSE: The wavelet transform (WT) is well suited for the analysis of signals whose characteristics vary rapidly over time. We devised a computerized method for objective scoring of scalp-recorded seizures that takes advantage of the WT. METHODS: Using wavelet coefficients as a metric, we devised a statistical scoring method aimed at detecting significant and sustained rhythmic buildup. The approach was used to create spatiotemporal significance maps for each seizure. Each seizure was also independently analyzed by computer and an expert reader not involved in the clinical workup or computer analysis of these patients. Hierarchical decision rules for determining seizure lateralization and localization were established from a training set of seizures and subsequently tested on those from an independent test set of seizures. The test dataset included a total of 57 scalp-recorded seizures from 18 patients, each with a > or =12-month seizure-free surgical outcome. RESULTS: Validation was determined by the site of surgical resection. Of the 57 seizure records in the test dataset, the computerized approach resulted in 48 correctly lateralized seizures as compared to 34 for the expert reader. Further, the computer correctly localized 41 seizures to the expert's 31. CONCLUSIONS: The method presented appears to provide an objective basis for the intrachannel scoring of ictal EEGs with minimal interference from artifacts and intermittent discharges. Although the approach has so far shown a substantial improvement over expert scoring in estimating the lateralization and locus of seizure onset, further testing is required to fully evaluate fully its diagnostic accuracy.

Brain Mapping↗

Outcome of epilepsy surgery in focal cortical dysplasia.

OBJECTIVE: To describe the outcome of surgery in patients with drug resistant epilepsy and a histopathological diagnosis of focal cortical dysplasia. METHODS AND SUBJECTS: Analysis of histories and presurgical and follow up data was carried out in 53 patients with a histological diagnosis of focal cortical dysplasia. Their mean age was 24.0 years (range 5 to 46), and they included 14 children and adolescents. Mean age at seizure onset was 12.4 years (0.4 to 36) and mean seizure duration was 11.6 years (1 to 45). RESULTS: The presurgical detection rate of focal cortical dysplasia with magnetic resonance imaging (MRI) was 96%. There were 24 temporal and 29 extratemporal resections; additional multiple subpial transections were done in 12 cases to prevent spread of seizure discharges. There was a 6% rate of complications with permanent neurological deficit, but no deaths. All resected specimens were classified by neuropathological criteria as focal cortical dysplasia. Balloon cells were seen in most cases of extratemporal focal cortical dysplasia. After a mean follow up of 50 months, 38 patients (72%) were seizure-free, two (4%) had less than two seizures a year, nine (17%) had a reduction of seizure frequency of more than 75%, and four (8%) had no improvement. Seizure outcome was similar after temporal and extratemporal surgery. The patients in need of multilobar surgery had the poorest outcome. CONCLUSIONS: Circumscribed lesionectomy of focal dysplastic lesions provides seizure relief in patients with chronic drug resistant temporal and extratemporal epilepsy. There was a trend for the best seizure outcome to be in patients with early presurgical evaluation and early surgery, and in whom lesions were identified on the preoperative MRI studies.

Adolescent↗