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Gangliogliomas in adults.

BACKGROUND: Gangliogliomas are rare tumors occurring in both children and adults that are characterized by the presence of neoplastic cells resembling both neurons and glia. METHODS: The authors reviewed 18 adults patients with intracranial gangliogliomas treated at the study institutions between October 1987 and November 1995. RESULTS: The median age at diagnosis was 33.7 years, with a range of 21 to 55 years. Median follow-up was 37.7 months, with a range of 4 months to 13 years. Clinical manifestations among the patients included seizures (13), headache (2), ataxia (1), and hemiparesis and paresthesias (1 patient each). Tumors were located in the temporal lobe (6 patients), temporal lobe and thalamus (1 patient), frontal lobe (5 patients), cerebellum (4 patients), and insula or thalamus (1 patient each). Thirteen patients underwent gross total resection, 4 underwent incomplete resection, and 1 underwent only a stereotactic biopsy. Treatment modalities included surgery only, surgery plus radiation, and surgery plus radiation and chemotherapy. Median survival was 90.3 months, with a range of 14 months to 13 years. Three patients were dead at follow-up with a mean survival of 32.3 months. These patients showed anaplastic features in their pathology at initial surgery or surgery for recurrence. CONCLUSIONS: In adults, gangliogliomas have a relatively favorable prognosis; however, the presence of anaplastic features predicts a worse outcome.

Adult↗

Comparison of neuropsychological outcomes after selective amygdalohippocampectomy versus anterior temporal lobectomy.

Transsylvian selective amygdalohippocampectomy (TSA) is an operative technique designed to spare unaffected brain tissue during surgical treatment for mesial temporal lobe epilepsy. In contrast to standard anterior temporal lobectomy (ATL), the advantages of TSA with respect to postoperative cognitive outcome are equivocal. We compared cognitive function before and after surgery in 49 patients with unilateral mesial temporal lobe seizures who underwent either ATL (n=17) or TSA (n=32). All patients received neuropsychological testing before and 1 year after surgery. The intelligence quotient (IQ) increased postoperatively in both surgical groups. Memory evaluation in the ATL group revealed a postoperative decline in nonverbal memory after right-sided resection and a postoperative decline in verbal memory after left-sided resection. In the TSA group, there was a slight postoperative decline only in verbal memory after left-sided resection, but other memory function was well preserved. In particular, there was significant postoperative improvement in verbal memory after right-sided resection. Overall, memory function was better preserved in the TSA group than in the ATL group.

Adult↗

Intracranial EEG monitoring in Landau-Kleffner syndrome associated with left temporal lobe astrocytoma.

A 3 1/2-year-old boy developed partial complex seizures with right-sided motor activity, occasionally secondarily generalized at age 18 months. Initial EEG showed left-sided focal epileptiform discharges. Seizures became refractory to antiepileptic drugs (AEDs). At age 3 years, there was severe language deterioration consistent with Landau-Kleffner syndrome (LKS). At that time, an EEG showed almost continual generalized spikes and polyspikes, worse during sleep. Video-EEG showed generalized tonic seizures associated with generalized EEG ictal activity. Magnetic resonance imaging (MRI) showed a cystic and solid lesion of the left hippocampal and parahippocampal gyri without surrounding edema. Subdural strip electrodes under the left temporal and overlying the left frontal lobes demonstrated interictal spikes simultaneously in all recording contacts. Ictal EEG activity occurred in the temporal electrodes 0.2-1 s before appearing in the frontal electrodes. After left temporal lobectomy with subtotal resection of a low-grade astrocytoma, he had an immediate marked decrease in seizures. In 1 month he was seizure-free, and in 6 months had no further seizures and markedly improved language. These findings provide further evidence that left temporal structural epileptogenic lesions may contribute significantly to the pathophysiology of LKS.

Aphasia↗

Case of the month. August 1996--frontal lobe tumor in 11 year old girl.

An 11 year old girl presented with an 8 month history of left temporal headaches with new onset of nausea and vomiting with increased severity of headaches. An MRI scan showed a frontal lobe mass. The tumor was resected and histologic studies demonstrated a central neurocytoma. The clinical, radiologic and pathologic aspects (including immunohistochemistry and electron microscopy) of central neurocytomas are reviewed and the atypical features of this case described.

Brain Neoplasms↗

Neuropsychological characteristics of the syndrome of mesial temporal lobe epilepsy.

OBJECTIVE: To identify the neuropsychological features of the syndrome of mesial temporal lobe epilepsy (MTLE), a surgically remediable epileptic syndrome defined by the presence of hippocampal sclerosis, using a broad and comprehensive neuropsychological test battery. SETTING: Epilepsy surgery center. PATIENTS: After scalp adn invasive electroencephalographic monitoring, a consecutive series of 107 adults were found to have intractable complex partial seizures of unilateral left (n = 62) or right (n = 45) temporal lobe origin. Patients were included if they were not retarded and had left hemisphere dominance for speech but no magnetic resonance imaging abnormalities other than hipocampal sclerosis. Histopathological analyses of resected hippocamppi showed that 66 patients had hippocampal sclerosis (MTLE+), and 41 did not have evidence of significant hippocampal sclerosis (MTLE-). INTERVENTIONS: None. MAIN DEPENDENT MEASURES: A comprehensive battery of neuropsychological tests that included measures of intelligence, academic achievement, language, visuoperceptual or visuospatial function, memory and learning, attention, and problem-solving abilities. RESULTS: The syndrome of MTLE was associated with considerable generalized cognitive impairment (in intelligence, academic achievement, language, and visuospatial functions), but not related to adequacy of performances in other selected cognitive domains (attention or concentration, executive functions). Material-specific memory effects were obtained-primarily for verbal memory in association with left-sided MTLE. CONCLUSIONS: Distinct neuropsychological features of spared, compromised, and laterality-specific cognitive impairments characterize the syndrome of MTLE. This information needs to be incorporated into formal syndrome criteria.

Adult↗

[Image-guided surgery for epilepsy].

Availability of a neuronavigation system for epilepsy surgery was reported, and its practical use was discussed. Four of nine patients with intractable epilepsy underwent surgical procedures using a neuronavigation system, Viewing wand, from November 1995 to August 1996, in our hospital. The ages of patients were between 9 to 46 years old. Three of them had temporal lobe epilepsy and one had generalized tonic seizures. One of the temporal lobe epilepsy cases had focal cortical dysplasia in the left posterior temporal lobe, and the other one showed that left hippocampal atrophy on MR images. The remaining two patient had no abnormality on MR images. All patients underwent video-EEG monitoring and habitual seizures were recorded at least three times. Ictal and/or interictal SPECT and neuropsychological testing were also performed. Electrocorticograms were recorded intraoperatively in all patients. Surgical procedures using the neuronavigation system were anterior temporal lobectomy, corpus callosotomy and lesionectomy of focal cortical dysplasia. A patient with temporal lobe epilepsy underwent implantation of depth electrodes under the neuronavigation. In temporal lobectomy, image-guided surgery helped to make a decision concerning the safely-resectable size of the lateral temporal cortex and hippocampus. The hippocampus was resected with minimum surgical damage and it made possible a complete histopathological examination. In corpus callosotomy, although it was not easy to confirm the length of the callosal section, the neuronavigation system enabled this to be done quickly. The real-time navigation showed the accurate operating position on three-dimensional images. The location of focal cortical dysplasia was often difficult to identify macroscopically. However, the location of the lesion can be projected to the skin surface under the neuronavigation system. The width of skin incision and craniotomy was able to be made smaller, and the surgery was able to be performed less invasively. The Viewing Wand system was accurate, reliable and easy to operate in these procedures. The navigating error was 2-5 mm. Using CT image data of 5 mm thickness the error was greater, although use of MR image data of 2 mm thickness resulted in relatively small error up to 2-3 mm. The first major factor of the error was the fiducial registration of the patient's head. While the registration was made more strictly with multiple fiducial points, the error was smaller. The second factor was movement of patient's head and/or the navigation arm. The arm and the head should be fixed tightly to the operating table, and it is better if they are fixed together with a supporting arm. The third factor was intraoperative brain shift caused by flow out of the cerebrospinal fluid or removal of mass lesions. This type of error is common in all navigation systems. However, it may be avoided making some real-time feedback system. With the Viewing Wand system, repetition of the intraoperative registration using intracranial anatomical structures reduces this type of error. On the other hand, there were some difficulties on stereotaxic procedures, such as implantation of depth electrodes, using the Viewing Wand. The error was larger than that recorded in other frame-based stereotaxic apparatus. This problem may be improved by a supporting system to fix the probe position. As a neuronavigation system can be widely applied to neurosurgical procedures, we consider that epilepsy and skull-base surgery are the best targets for it because of the minimum possible brain shift. We hope that accurate and less-invasive surgery using a neuronavigation system will contribute to a better outcome for epilepsy patients.

Adolescent↗

Prenatal removal of frontal association cortex in the fetal rhesus monkey: anatomical and functional consequences in postnatal life.

Anatomical and behavioral consequences were examined following bilateral or unilateral resections of the presumptive dorsolateral prefrontal cortex in 4 rhesus monkey fetuses at E102 (102nd embryonic day), E104, E106 and E119, with subsequent replacement in utero and delivery at later stages of development. Unoperated monkeys of appropriate pre- and postnatal ages served as controls for certain features of morphogenesis and tectogenesis. Monkeys given comparable unilateral or bilateral lesions at selected postnatal ages were used as controls for the behavioral effects of prenatal resection as well as for neuropathological examination. The behavioral evaluation provided evidence for extraordinary preservation of function after frontal cortical resection in the prenatal period. During the entire course of its postnatal development, a monkey that sustained bilateral resection as a fetus at E106 performed surprisingly well and clearly with the competence of unoperated monkeys of identical or older ages on tasks selective for dorsolateral prefrontal cortical function; it did not exhibit deficits characteristic of animals that have comparable or even smaller resections as juveniles or adults. Following either unilateral or bilateral resection prior to E106, ectopic sulci and gyri appeared in intact regions of the frontal lobe of each hemisphere, as well as in locations at a considerable distance from the lesions, such as the temporal and occipital lobes. The cytoarchitectonic characteristics of the cortex in these anomalous gyri were typical of adjacent cortex in the regions in which they appeared. Examination of the parvocellular subdivision of the mediodorsal thalamic nucleus yielded the novel finding that this principal source of essential projections to the dorsolateral prefrontal cortex contained a virtually normal number of neurons in postnatal monkeys, following unilateral or bilateral lesions performed at or before E106, and exhibited only a partial loss of neurons after surgery at E119. The same cells regularly degenerate following comparable resections in the postnatal period. The survival of these neurons in prenatally operated monkeys is unprecedented, and can be explained by several hypotheses, including the possibility that their axons become rerouted to their structures in the absence of their normal targets. The present results thus demonstrate that ablation of a circumscript area of the frontal cortex in a non-human primate a full two months before birth may leave the functions later to be subserved by that area unaffected. Such early surgical intervention results in significant alteration in the gross morphological characteristics of the cortex, and apparently induces a functionally compensatory reorganization of thalamocortical connections. Similar principles may account for sparing of function following early damage to the human brain.

Animals↗

Management of CNS mucormycosis in the pediatric patient.

Rhinocerebral mucormycosis (RM) is a rare, rapidly progressive disorder caused by fungi from the Mucoraceae family. With extensive central nervous system involvement, this disease is uniformly fatal within weeks. Mucormycosis normally presents in poorly controlled diabetics, intravenous drug abusers and immunocompromised patients. Many have advocated radical surgical resection (i.e. exenteration of the cavernous sinus with carotid sacrifice and en bloc resection) with administration of amphotericin B. We present a case of mucormycosis involving the paranasal sinuses and cranial base in a pediatric patient who experienced long-term survival with a more limited resection. We also present a review of the relevant literature. A 14-year-old diabetic male presented with RM with involvement of the bilateral frontal lobes, right basal ganglia and temporal lobe. Additionally, there was involvement of the sphenoid sinus and right cavernous sinus with extension into the posterior fossa along the course of the trigeminal nerve and encasement with narrowing of the right carotid artery. The patient underwent sinus endoscopy with debridement of necrotic fungal tissue and bone. This was followed by craniotomy with evacuation of bifrontal, right temporal and basal ganglia abscesses in such a way that all abscess cavities communicated. An Ommaya reservoir was placed into the largest cavity. The patient continued to receive intrathecal and intravenous antibiotics as well as hyperbaric oxygen therapy. The patient was clinically and radiographically free of disease 1 year after diagnosis. While invasive RM is generally a fatal disease, this rare disorder can be treated successfully without radical resection, particularly if multimodality treatment options are implemented.

Adolescent↗

Localization of temporal lobe foci by ictal EEG patterns.

Identifying patients whose complex partial seizures originate in temporal neocortex rather than in hippocampus is important because such patients have less favorable outcomes with standard anteromesial temporal resections. We reviewed scalp-recorded ictal EEGs of 93 epilepsy surgery candidates who either underwent intracranial EEG monitoring (n = 58) or who were referred directly for temporal lobectomy (n = 35). We definded seven patterns of early seizure discharges, grouped patients according to their seizure pattern, and correlated these with the site of seizure onset determined by intracranial EEG. Categorization by seizure pattern was also compared with brain magnetic resonance imaging (MRI) findings intracarotid amobarbital (Wada) testing. An initial, regular 5- to 9- Hz inferotemporal rhythm (type 1A) was most specific for hippocampal-onset seizures. Less commonly, a similar vertex/parasagittal positive rhythm (type 1B) or a combination of types 1B and 1A rhythms (type 1C) was recorded. Seizures originating in temporal neocortex were most often associated with irregular, polymorphic, 2- to 5-Hz lateralized activity (type 2A). This pattern was commonly followed by a type 1A theta rhythm (type 2B) or was preceded by repetitive, sometimes periodic, sharp waves (type 2C). Seizures without a clear lateralized EEG discharge (type 3) were most commonly of temporal neocortical origin. These associations between type of seizure pattern and probable site of cerebral origin were statistically significant. MRI and Wada testing did not have as much specificity as ictal patterns in differentiating among seizure origins. We conclude that the initial pattern of ictal discharge on scalp EEG can assist in distinguishing seizures of temporal neocortical onset from those of hippocampal onset. This information can be used to identify patients for invasive monitoring.

Amobarbital↗

Neuropsychological evaluation before and after surgical treatment of temporal lobe epilepsy.

We present the results of pre- and postoperative neuropsychological evaluations of 58 patients with temporal lobe epilepsy who underwent a chronic intracranial EEG monitoring and a subsequent standard anterior temporal lobectomy. Wada's test provided valuable information on the speech dominant side and on the focus localization. Some warning signs as well as verbal automatisms indicated the effect for focus localization and lateralization. The results of interictal neuropsychological tests suggested that each subgroup of TLE performed differently. A postoperative neuropsychological performance has improved in many tests that may be explained by the diminished epileptic bombardment resulting from the resection.

Adolescent↗

Anterior temporal lobectomy, hippocampal sclerosis, and memory: recent neuropsychological findings.

Anterior temporal lobectomy (ATL) is an effective and increasingly utilized treatment for nonlesional, intractable mesial temporal lobe epilepsy. However, this surgery results in domain-specific neuropsychological morbidity for a subset of patients. Within the past decade, multidisciplinary studies have revealed that left ATL patients without significant sclerosis in the resected hippocampus are most at risk for a substantial postacute decline in the ability to encode new verbal information. These patients are also at risk for a significant decrement in confrontation naming and other retrieval-based language abilities. The memory deficit is not attributable to this disruption of language. A relationship between hippocampal sclerosis (HS) status and memory performance has not been identified consistently in right ATL patients, but investigation of new visuospatial measures continues. The influence of variables other than HS on neuropsychological outcome is also discussed.

Anomia↗

Invasive monitoring of limbic epilepsy using stereotactic depth and subdural strip electrodes: surgical technique.

BACKGROUND: In spite of advances in noninvasive localization of seizure foci, some cases of intractable limbic epilepsy still require invasive recordings in order to identify the site of seizure onset. This necessitates a safe and reliable method for placing depth and subdural electrodes in the mesial temporal and orbitofrontal regions. The University of Florida has devised a system that utilizes CRW-based stereotactic placement of bitemporal depth electrodes in conjunction with placement of subdural strips over the inferolateral temporal lobe and orbitofrontal cortex. This report describes the surgical technique and initial clinical experience using this method. METHODS: Depth electrodes are placed along the long axis of the hippocampi via an occipital approach. A CRW-based stereotactic system was developed that incorporates both computed tomography (CT) and magnetic resonance imaging (MRI) for selection of target and entry sites and displaying the electrode trajectory. Subdural strip electrodes are placed over the inferolateral temporal and orbitofrontal regions through burr holes. RESULTS: This method has been used in 18 patients (depth electrodes only in three patients and depth electrodes with subdural strips in 15 patients). This information lead to surgical resections in 15 patients. No resection was recommended in three patients (two with bitemporal onset and one with no seizures after 6 weeks). Complications were limited to an unplanned removal of one electrode and an asymptomatic lateral temporal lobe contusion in one patient. CONCLUSIONS: This method provides a safe and effective way to sample bilateral mesial temporal and orbitofrontal regions in cases of intractable limbic epilepsy.

Electrodes, Implanted↗

Operative strategies for patients with cortical dysplastic lesions and intractable epilepsy.

Cortical dysplastic lesions (CDLs) are usually identified by magnetic resonance imaging (MRI). Clinical, electrographic and histologic findings suggest that focal CDLs (FCDLs) are highly epileptogenic, often involve the rolandic cortex, and can present variable degrees of histopathologic abnormalities. An ictal or "ictal-like" bursting pattern of electrographic activity was recorded over dysplastic cortex in 65% of our patients. Resective surgery can eliminate or significantly reduce seizure frequency in many medically intractable patients, depending on lesion location, degree, and extent of histopathologic abnormalities. Best results are achieved when complete or major excision of both the MRI-visible lesion and the cortical areas displaying ictal electrographic activity can be performed. This is more likely when the degree of histopathologic abnormality is mild to moderate or when the lesion is in a temporal lobe. More severe histopathologic abnormalities and central insular or multilobar lesions usually lead to less favorable results: either major excision of the visualized lesion is impractical or the lesion is microscopically more extensive than shown by MRI. Multilobar resection or hemispherectomy for patients with infantile spasms associated with CDLs and for patients with hemimegalencephaly are often associated with dramatic improvement in seizure control. Callosotomy can be performed in selected patients with diffuse CDLs who have intractable drop attacks.

Adolescent↗

Surgical outcome of epilepsy caused by cortical dysplasia.

PURPOSE: Cortical dysplasia (CD) is the second most common pathologic entity in surgically treated epilepsy. To delineate its surgical outcome and prognostic factors, we performed a retrospective analysis of patients operated on at a single institute. METHODS: Between September 1994 and December 2000, 128 cases with CD were operated on at our institute. The male/female ratio was 85:43, and the mean age was 27 years (SD, 7.6 years). The mean postoperative follow-up period was 26.9 months (SD, 12.0 months). Seizure outcome and severity of CD were classified according to Engel's and Mischel's classifications, respectively. Severity of CD was mild in 69, moderate in 21, and severe in 38. Lobar distribution was temporal in 54, frontal in 48, and others in 26. Cortical resection, lobectomy, or hemispherectomy was done in all patients. Univariate analysis was done followed by multivariate analysis by using computerized statistical software. RESULTS: Postoperative seizure outcome was Engel class I in 58 (45.3%), II in 17 (13.3%), III in 16 (12.5%), and IV in 37 (28.9%). Transient postoperative complications occurred in 14 (10.9%) patients without mortality or permanent neurologic impairment. Multivariate analysis revealed severity of CD and frontal lobe resection as independent prognostic factors for worse outcome (p = 0.001 and 0.003, respectively). CONCLUSIONS: Epilepsy surgery for CD achieved 45.3% seizure-free rate with 10.9% transient postoperative complications in our institute. Worse postoperative seizure outcome was observed in cases with mild CD and frontal lobe resection.

Adolescent↗

Surgery for central, parietal and occipital epilepsy.

Patients with epileptogenic lesions that are more or less restricted to the central, parietal or occipital regions represent 14% of our total epilepsy surgery series and consisted of 322 patients at the end of 1980. Tumoral or major vascular lesions were present in 117 patients (37%), but this report is limited to the remaining 203 patients (63%) with non-tumoral epileptogenic lesions. The presumed etiology was birth trauma or anoxia in 25%, postnatal brain trauma in 25%, and unknown in 24%, miscellaneous lesions were present in 16%, post-inflammatory gliosis or multiple factors were present in the remaining 10%. Follow-up data of 2-51 years' duration (median 18 years) are available for 186 of these 203 patients. Sixty-three patients (34%) remain seizure-free. Forty-two patients (23%) have a marked reduction of seizures. The remaining 81 patients (44%) have a lesser reduction. The variation in clinical seizure patterns is considerably greater than in temporal lobe epilepsy. Focal sensorimotor phenomena are common at the onset, whether the seizures begin in the central region or further posterior. The success following cortical resection is equally gratifying and the failures just as distressing as in surgery for epilepsy, involving other brain regions.

Adult↗

Quality of life after successful epilepsy surgery: evaluation by occupational achievement and income acquisition.

OBJECTIVES: A comprehensive epilepsy surgery program has been developed at Chulalongkorn University Hospital, Thailand to reach an international standard level, rendering patients good surgical outcomes comparable to developed countries. After successful operation, seizure free patients have become independent, self earning or employed. However, quality of life (QOL) in terms of occupational achievement and income acquisition after epilepsy surgery has never been studied. These indicators reflect the ultimate QOL in the aspects of actual independency, intact brain functions, mental health and psycho-social interactions. The authors therefore conduct the study on improvement of QOL after successful epilepsy surgery using these parameters. MATERIAL AND METHOD: One hundred and eleven intractable epilepsy who have become seizure free to worthwhile improved (Engel class I to III) after standard presurgical evaluation and epilepsy surgery from January 2002 to December 2004 were evaluated. The patients were followed up for 3 years. The occupational status and incomes were categorized according to the ranking of the patients' functioning levels. The pre and post surgery work abilities, employment and incomes were interviewed and compared. Mc Nemar test and paired t-test were used for statistical analyses. RESULTS: The average age of the 111 adults (54 males and 57 females) was 33.7 +/- 9.2 years. Eighty two percent of the patients had temporal lobe epilepsy (TLE) with mesial temporal sclerosis (MTS) and underwent standard anterior temporal lobectomy. The rest had tumors, cortical dysplasia or scar and received lesionectomy or cortical resection assisted by intra-operative or intra-cranial EEG. The overall seizure free rate is 83.8%. The occupational status of the subjects was shown to improve significantly after surgery from unemployed to higher categories of professional achievement (p < 0.001). The number of unemployed and no income individuals decreased from 66 to 25 cases (62.1% reduction rate) after surgery (p < 0. 001). Reciprocally, the number of persons who achieved professional jobs with regular incomes or salaries increase from 30 to 53 cases (43.4% increasing rate) (p < 0.001). The patients who have not acquired any income increment showed improvement in working ability after epilepsy surgery. The average annual incomes per capita shows the increasing rate of 45.08%, from 55,657.85 Baht (approximately U.S. dollars 1390) to 80,748.15 Baht (approximately U.S. dollars 2018), with strong statistical significance (p < 0.001). The improvement is best seen in seizure free than in non-seizure free subjects. CONCLUSION: The present study, to the authors 'knowledge, is the first to use work abilities, professional achievement and income acquisition to assess the ultimate QOL after epilepsy surgery. Most subjects have been shown to significantly improve their postoperative lives in terms of occupational accomplishment and income increment, especially in seizure free individuals. The need for expansion of epilepsy surgery is emphasized.

Achievement↗