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Clinical and electrographic manifestations of lesional neocortical temporal lobe epilepsy.

To determine whether lesional neocortical temporal lobe epilepsy (NTLE) can be differentiated from mesial temporal lobe epilepsy (MTLE) during the noninvasive presurgical evaluation, we compared the historical features, seizure symptomatology, and surface EEG of 8 patients seizure free after neocortical temporal resection with preservation of mesial structures and 20 patients after anterior temporal lobectomy for MTLE. Seizure symptomatology of 107 seizures (28 NTLE, 79 MTLE) was analyzed. One hundred one ictal EEGs (19 NTLE, 82 MTLE) were reviewed for activity at seizure onset; presence, distribution, and frequency of lateralized rhythmic activity (LRA); and distribution of postictal slowing. Seizure symptomatology and EEG data were compared between groups, and sensitivity, specificity, and positive and negative predictive values were determined for variables that differed significantly. Multiple logistic regression was used to determine whether patients could be correctly classified as having MTLE or NTLE. MTLE patients were younger at onset of habitual seizures and more likely to have a prior history of febrile seizures, CNS infection, perinatal complications, or head injury. NTLE seizures lacked features commonly exhibited in MTLE, including automatisms, contralateral dystonia, searching head movements, body shifting, hyperventilation, and postictal cough or sigh. NTLE ictal EEG recordings demonstrated lower mean frequency of LRA that frequently had a hemispheric distribution, whereas LRA in MTLE seizures was maximal over the ipsilateral temporal region. We conclude that it may be possible to differentiate lesional NTLE from MTLE on the basis of historical features, seizure symptomatology, and ictal surface EEG recordings. This may assist in the identification of patients with medically refractory nonlesional NTLE who frequently require intracranial monitoring and more extensive or tailored resections.

Adult↗

Severe Amnesia in Epilepsy: Causes, Anatomopsychological Considerations, and Treatment.

Severe amnesia in epileptic patients is a catastrophic condition that may be due to different etiologies. Because of the striking findings and thorough neuropsychological studies of Patient H.M., the literature has focused on postsurgical occurrence of such memory impairment, with much less emphasis on other causes. Here we summarize, for comparison, the history of H.M. We report five patients with pronounced memory loss who had extensive neuropsychological and electroencephalographic testing. MRI was also performed in four of the patients, MRI volumetric measurements of amygdala and hippocampal formation in three, and measurements of entorhinal cortex in two. The amnesia occurred after head trauma in one patient, following encephalitis in one, after partial status epilepticus in two, and after unilateral surgical resection in a woman with bilateral lesions. On the basis of these studies it was impossible to distinguish the role of recurrent temporal lobe epileptic seizures as distinct from underlying lesions in the genesis and course of the memory loss. We review here the anatomical substrate, neuropsychological, and other investigations and the etiological factors leading to the amnesia in these patients, together with current concepts regarding possible causes of such severe memory dysfunction. In patients with this degree of severity of memory deficit, temporal resection in an attempt to control seizures did not lead to a measurable increase in memory problems. It also, however, did not bring about worthwhile improvement in seizure control.

Journal Article↗

Surgical management of pediatric tumor-associated epilepsy.

Brain tumors are a common cause of seizures in children. Early surgical treatment can improve seizure outcome, but controversy exists regarding the most appropriate type of surgical intervention. Some studies suggest tumor resection alone is sufficient, while others recommend mapping and resection of the surrounding epileptogenic foci to optimize seizure outcome. To address this issue, we reviewed the charts of 34 pediatric patients aged 18 months to 20 years with medically intractable epilepsy and primary brain tumors. The average age at operation was 12.6 years, and patients had seizures for an average of 6.4 years. The majority of tumors were located in the temporal lobe. Seventeen patients, because of tumor location near an eloquent area, underwent extraoperative mapping using subdural electrode grids prior to definitive tumor resection. Fourteen of these patients had a gross total tumor resection, yet only two had a distinct zone of ictal onset identified and resected. The remaining 17 patients had tumors either in the nondominant hemisphere or far removed from speech-sensitive areas, and therefore did not undergo extraoperative subdural electroencephalograph mapping. Fourteen of these patients also had a gross total tumor resection, while none had intraoperative electrocorticography to guide the resection of additional nontumoral tissue. Overall, of the 28 patients treated with a gross total tumor resection, 24 (86%) are seizure free, while the other four are significantly improved. Of the six patients who had a subtotal tumor removal, five have persistent seizures. The mean follow-up was 3.6 years. We conclude that in children and adolescents, completeness of tumor resection is the most important factor in determining seizure outcome. The routine mapping and resection of epileptogenic foci might not be necessary in the majority of patients. As a corollary, the use of subdural electrode grids in pediatric patients with tumor-associated epilepsy should be limited to cases requiring extraoperative cortical stimulation for localization of nearby eloquent cortex.

Adolescent↗

Multiple intracranial seeding of craniopharyngioma after repeated surgery--case report.

A 17-year-old woman presented with a rare case of intracranial seeding of craniopharyngioma after repeated surgery. She initially presented with secondary amenorrhea and visual impairment. Magnetic resonance imaging revealed a suprasellar mass. Subtotal removal of the tumor was performed. The diagnosis was adamantinomatous craniopharyngioma. Seven months later, the patient underwent a second operation for recurrence of the craniopharyngioma. Subsequently, ventriculoperitoneal (VP) shunting and gamma knife surgery were performed. Twenty-seven months after the first operation, multiple cystic lesions were found in the right frontal and temporal lobes. Positive tumor cytology was observed in the cerebrospinal fluid obtained from the VP shunt chamber. These tumors were subtotally resected. However, the patient died from consecutive tumor recurrence 4 years after the initial diagnosis.

Adolescent↗

The myth of silent cortex and the morbidity of epileptogenic tissue: implications for temporal lobectomy.

This article reviews two commonly held myths regarding temporal lobe epilepsy-it is a static disorder with minimal morbidity and mortality, and epileptogenic tissue impairs only the functions of the seizure focus-and one myth concerning temporal lobe functions-they contain areas of nonfunctional, "silent" cortex. Chronic temporal lobe epilepsy can cause progressive structural, cognitive, and behavioral changes. Aside from the seizure focus, primary epileptogenic cortex may have a deleterious influence on distant brain areas. Removing this "nociferous" cortex and reducing the antiepileptic drug burden can improve cognitive or behavioral and metabolic function in areas remote from the resection. Anterior temporal lobectomy often removes functional tissue that may or may not be epileptogenic. Because normal brain does not contain functionless, "silent" areas, the procedure can have negative as well as positive cognitive or behavioral consequences. To improve the outcomes of focal cortical resections for seizure control, we need to better define functional and nociferous cortex and more clearly understand their boundaries and interactions.

Cerebral Cortex↗

[Localizing and prognosis value of 99Tcm-ECD spect in patients with refractory temporal lobe epilepsies].

Interictal and ictal 99Tcm ECD-SPECT were retrospectively studied in 46 patients with refractory temporal lobe epilepsy. Forty two of these patients underwent an anterior temporal lobectomy with amygdalo-hippocampectomy or a cortical resection. SPECT findings as indicator of localization for the epileptogenic zone (EZ) and surgical prognosis were validated by comparison with other investigations including video-EEG monitoring with surface electrodes in all the patients, intracranial electrodes in 18 patients and the results of post-surgery outcome. Reliability of SPECT for localizing the epileptogenic zone (EZ) was found for mesial temporal epilepsy (28 patients) and the presumed bilateral temporal epilepsies (7 patients). In these latter cases, ECD-SPECT findings may serve to replace invasive methods with use of intracranial electrodes. SPECTs were unreliable when EZ was undetermined whether mesial or lateral by other non invasive investigations. Unilateral mesial temporal hyperperfusion associated with hypoperfusion of contralateral mesial structures evidenced by ictal SPECTs appear to be preoperative criteria for a successful outcome after surgery.

Adolescent↗

Surgical treatment for refractory temporal lobe epilepsy in the elderly: seizure outcome and neuropsychological sequels compared with a younger cohort.

PURPOSE: Surgical treatment of refractory temporal lobe epilepsy (TLE) is promising for selected patients, but only little experience has been acquired in operating on older patients, especially with limited resections. We intend to delineate clinical and surgical factors influencing outcome in patients older than 50 years at operation and to compare the results with those of a younger patient cohort. METHODS: Fifty-two patients older than 50 years were operated on for intractable mesial or combined mesiolateral TLE between 1991 and 2002. The mean age at operation was 55 years, and the mean duration of epilepsy was 33 years. Forty selective amygdalohippocampectomies (33 for hippocampal sclerosis, seven for removal of a mesiotemporal lesion), five lateral temporal lesionectomies plus amygdalohippocampectomy, and seven anterior temporal lobectomies were performed. Eleven (21%) patients had undergone invasive presurgical video-EEG monitoring. The mean follow-up period was 33 months. We compared the results with those of a younger cohort operated on in the same time period. RESULTS: Thirty-seven older patients attained complete seizure control (71% class I), and 10 patients had only rare postoperative seizures (19% class II). Four patients improved >75% (8% class III), and one patient did not improve (2% class IV). The same rate of seizure control was attained by 11 patients older than 60 years at surgery. These results were not significantly different from those in a younger patient group. A trend toward better seizure control was noted in 16 patients with an epilepsy duration of <30 years (all class I or II), and in 20 patients with a seizure frequency of fewer than five seizures per month (all class I or II). No mortality resulted from a total of 65 diagnostic and therapeutic procedures. A 3.8% permanent neurologic morbidity (dysphasia and hemiparesis) was noted. Hemianopia occurred in three (5.9%) patients. Neuropsychological testing revealed low preoperative performances and some gradual further deterioration after surgery. CONCLUSIONS: Results of surgery for TLE with mainly limited resections are promising in patients older than 50 years and older 60 years, despite the long seizure history. As expected, the risk of complications is somewhat higher compared with that in a younger control group. The impact of low neuropsychological performance is a concern.

Adult↗

Pleomorphic xanthoastrocytoma: a developmental glioneuronal tumor with prominent glioproliferative changes.

Pleomorphic xanthoastrocytoma (PXA) is a rare primary brain tumor, which usually involves the superficial cortex in children and young adults. Although PXA is considered an astrocytic tumor, recent studies suggest the presence of neuronal differentiation and an origin from multipotential precursor cells. To clarify the clinicopathological characteristics, which have not been well established due to the rarity of this tumor, eight cases of histologically proven PXA were retrospectively analyzed, paying special attention to immunohistochemical findings and associated cortical dysplasia. Three males and five females of median age 17 years (range, 11-24 years) were enrolled in the study. All patients presented with epilepsy. Tumor sites included the temporal (n = 5), frontal (n = 2), and temporoparietal (n = 1) lobes. 18F-FDG PET (positron emission tomography) showed hypometabolism of PXA. Immunohistochemical study was performed in six cases using various antibodies for glial and neuronal markers. Gross total resection of the PXA was achieved in all cases. The follow-up period ranged from 7 to 14 years except for two recent cases. All patients are doing well with neither radiological nor clinical evidence of recurrence. The surgical treatment rendered all patients seizure free. The three temporal lobe PXAs were associated with cortical dysplasia. In one tumor, clusters of dysmorphic ganglion cells were found. All examined PXAs demonstrated immunoreactivity for both neuronal and glial markers in the various tumor cells. The immunohistochemical studies undertaken as a part of this study support the proposed astrocytic and neuronal differentiation of PXA, and indicate that PXA is probably a developmental neuroglial tumor with prominent glioproliferative changes associated with focal cortical dysplasia. Cortical dysplasia may be a cause of persistent epilepsy even after complete surgical excision of PXA.

Adolescent↗

Long-term outcome after surgical treatment of temporal lobe epilepsy in children.

OBJECT: Surgery is an accepted treatment for carefully selected patients with focal epilepsy. In the present study, the authors assessed clinical and surgery-related data obtained in a large series of children suffering from intractable temporal lobe epilepsy (TLE). METHODS: Etiological, pathological, and clinical features of possible prognostic significance were studied in 109 children who underwent surgery for TLE at the Montreal Neurological Institute and Hospital and the Montreal Children's Hospital between 1985 and 2000. The mean age of patients at seizure onset was 5.5 years and the duration of epilepsy ranged from 0.1 to 17.6 years. Preoperative magnetic resonance imaging revealed mesial sclerosis in 51 patients, a mass lesion in 45, and no visible abnormalities in 12. In six patients invasive monitoring was required. Cortical amygdalohippocampectomy was performed in 72% of patients, whereas 20% underwent transcortical selective amygdalohippocampectomy. In 23 patients a second surgical intervention was necessary. Low-grade tumors were found in 35% and mesial sclerosis was confirmed on pathological evaluation in 45%. Outcome was excellent (seizure free or > 90% reduction) in 94 patients (86%). The patients were followed prospectively for a median of 10.9 years (range 5-20.2 years). There were no permanent neurological complications and no deaths. CONCLUSIONS: Successful postsurgical outcomes, especially in patients treated for mesial temporal lobe sclerosis and lesion-related epilepsies, can be obtained in pediatric patients suffering minimal complications. Unfavorable outcomes are most likely to occur when epileptiform discharges are bitemporal or multifocal in distribution and in cases involving incomplete resection of mesiotemporal structures.

Adolescent↗

Basic targets and the different epilepsies.

The follow up of 49 stereotactically operated epileptics is graphically demonstrated and the 79 operations in basic targets within the amygdala and the area of the anterior pole of the thalamus are described. The patients had valuable gaining in 80%; The continued developement of stereotactic methods in the future is urgent, so long as 30% of the epileptics respond poorly to drug therapy and only 5-10% are suitable to the traditional resection methods.

Adolescent↗

Can intraoperative electrocorticography patterns predict surgical outcome in patients with temporal lobe epilepsy secondary to unilateral mesial temporal sclerosis?

INTRODUCTION: Intraoperative electrocorticography (ECoG) can be performed in cases of temporal lobe epilepsy due to hippocampal sclerosis (TLE-HS). However, its significance and correlation with surgical outcome are still controversial. OBJECTIVES: To analyze the electrophysiological characteristics of temporal lobe structures during ECoG of patients with TLE-HS, with emphasis on the comparison between pre- and post-resection recordings and surgical outcome. PATIENTS AND METHODS: Seventeen patients with refractory TLE-HS submitted to corticoamigdalohipocampectomy were included in the study. Clinical variables included age at the onset, duration of epilepsy and seizure outcome. The post-operative follow-up ranged from 24 to 36 months. According to outcome subjects were divided in two subgroups: (A) individuals free of seizures (Engel 1A), and (B) individuals not-free of seizures (Engel 1B-IV). Four patterns of ECoG findings were identified: isolated discharges; high frequency spikes (HFS); continuous discharges; combination of isolated discharges and HFS. According to predominant topography ECoG was classified as mediobasal, lateral (or neocortical), mediobasal and lateral. RESULTS: The progressive removal of the temporal pole and the hippocampus was associated with significant decrease of neocortical spikes. No correlation between clinical variables and seizure outcome was observed. Patients who only had isolated spikes on intraoperative ECoG presented a statistical trend for excellent surgical control. Patients who presented temporal pole blurring on MRI also had better post-surgical seizure outcome. CONCLUSIONS: This study showed that out of diverse clinical and laboratory variables, only isolated discharges on intraoperative ECoG and temporal pole blurring on MRI predicted excellent post-surgical seizure outcome. However, other studies with larger number of patients are still necessary to confirm these findings.

Adult↗

Cortical seeding of a craniopharyngioma after craniotomy: Case report.

BACKGROUND: Cortical seeding of a craniopharyngioma has been rarely reported. We present a case that ectopically recurred along the tract of a previous surgical route. METHODS: A 27-year-old woman presented earlier with a suprasellar craniopharyngioma. A left frontotemporal craniotomy was done with subtotal resection of the tumor because it was strongly adhered to the optic chiasm. Histopathology confirmed the diagnosis of craniopharyngioma. Six months after, the patient presented with decreased visual acuity and diplopia. She was reoperated through the previous craniotomy with a total resection. One year after the second surgery, the patient presented with seizures that were difficult to control. Magnetic resonance imaging revealed a contrast-enhancing tumor with cystic and solid components on the left temporal lobe cortex. The primary tumor bed was intact. The patient was reoperated, and the temporal lobe tumor was totally removed. Histologic studies showed an adamantinomatous craniopharyngioma. The patient was free of neurologic abnormalities, and no new lesion was found in the magnetic resonance imaging performed 1 year after the last surgery. CONCLUSIONS: Although craniopharyngiomas exhibit a benign histopathologic pattern, a total resection combined with careful inspection and irrigation of the surgical field is the optimal treatment for preventing local and ectopic recurrences. It is strongly recommended that the concerned patients have a long-term clinical and neuroimaging follow-up.

Adult↗

Bilateral crossed cerebello-cerebral diaschisis and mutism after surgery for cerebellar medulloblastoma.

A 7-year-old boy developed mutism after surgery for cerebellar medulloblastoma. Postoperative magnetic resonance imaging (MRI) showed atrophy of the cerebellar vermis and both cerebellar hemispheres, predominantly on the right side. Single photon emission computed tomography (SPECT) with technetium-99m-ethyl cysteinate dimer (Tc-99m ECD) revealed decreased cerebral blood flow (CBF) in the bilateral thalami, bilateral medial frontal lobes, and left temporal lobe in addition to the cerebellar vermis and both cerebellar hemispheres when mutism was manifest, indicating the existence of bilateral crossed cerebello-cerebral diaschisis (BCCCD). Circulatory disturbance in both cerebellar hemispheres secondary to tumor resection probably caused BCCCD in both cerebral hemispheres, predominantly in the left, via the dentatothalamocortical pathway (DTCP). With recovery of his mutism, CBF increased in the right thalamus, bilateral medial frontal lobes and left temporal lobe. Thus BCCCD was improved, with only a slight decrease in CBF still persisting in the left thalamus. The mechanism of mutism may have involved damage to the cerebellar vermis (the site of incision at operation), the left dentate nucleus (heavily infiltrated by the tumor) and the right dentate nucleus of the cerebellum (affected by circulatory disturbance secondary to acute postoperative edema). The SPECT findings suggested that mutism was associated with BCCCD-induced cerebral circulatory and metabolic hypofunction in the supplementary motor area mediated via the DTCP.

Cerebellar Neoplasms↗

Tumoral form of cerebral schistosomiasis mansoni. A report of four cases and a review of the literature.

We describe four cases of the tumoral form of cerebral schistosomiasis mansoni. The patients had symptoms of increased intracranial pressure and focal neurological signs that varied according to the site of the lesion. Computerized tomography showed a hyperdense, enhancing lesion located in the cerebellum (2 patients), frontal lobe and thalamus (1 patient), and temporal subdural region (1 patient), with associated mass effects. The lesion was resected in three patients and a stereotactic biopsy was performed in one. Histopathologic specimens of all four patients revealed multiple schistosomal granulomas in various evolutive phases. Two of these patients differ from previously described cases; one because of the subdural location of the lesion, mimicking a meningioma, and the other one because two lesions were present.

Adult↗

Stereotactic radiosurgery for patients with ACTH-producing pituitary adenomas after prior adrenalectomy.

PURPOSE: To review the results of stereotactic radiosurgery for patients with adrenocorticotropic hormone (ACTH)-producing pituitary adenomas after bilateral adrenalectomy. METHODS AND MATERIALS: Eleven patients with ACTH-producing pituitary adenomas after bilateral adrenalectomy underwent radiosurgery between 1990 and 1999. Nine patients had documented tumor growth, hyperpigmentation, and elevated ACTH levels (median 920 ng/mL) at the time of radiosurgery. Five of these patients had tumor enlargement despite prior fractionated radiotherapy (median dose 50 Gy). Two patients were treated prophylactically within 1 month of their adrenalectomies to prevent future tumor growth. The median follow-up was 37 months (range 22-74). RESULTS: Tumor growth control was achieved in 9 patients (82%); 2 patients had had continued tumor growth after radiosurgery. The ACTH levels decreased a median of 66% (range -99% to +27%); 4 patients had normal ACTH levels. Three patients had radiation-related complications, including diplopia (n = 2), ipsilateral blindness (n = 1), testosterone/growth hormone deficiency (n = 1), and asymptomatic temporal lobe radiation necrosis (n = 1): all had received prior radiotherapy. One patient who had undergone three prior resections and radiotherapy died 59 months after radiosurgery despite two additional attempts at tumor resection. CONCLUSION: Although our experience is limited, it appears that radiosurgery provides tumor control for most patients with ACTH-producing pituitary adenomas who have undergone bilateral adrenalectomy.

Adenoma↗

[Surgical treatment of giant cerebral arteriovenous malformations].

Despite advances in the surgical management of cerebral arteriovenous malformations (AVMs), giant (AVMs (greater than 5 cm] are still considered unsuitable for direct surgical resection by most neurosurgeons. Some of the lesions are being treated with embolization, or embolization followed by surgical excision. Embolization alone is not curative and carries potential risks of neurological deficit as well involves multiple procedures. Fourteen patients with giant AVMs underwent surgical resection without prior embolization. Four of the AVMs were located primarily in the frontal lobe, two in the temporal lobe, one each in the parietal and occipital lobes, while six AVMs were localized to two lobes (temporal-occipital or parietal-occipital). Four patients had associated aneurysms with the arteriovenous malformation. Eight patients presented primarily with seizures. One of these had multiple subarachnoid hemorrhages while another had symptoms suggestive of transient vertebrobasilar ischemia. Two patients had one or more subarachnoid hemorrhages. The primary complaint in the remaining four patients was headache with other associated symptoms. The patients with AVMs involving the optic radiation have had varying degrees of visual field deficit not interfering with their function. There were no deaths and only three patients had deterioration of neurological function. One of these three had an intra cerebral hemorrhage secondary to an associated aneurysm rupture. We feel that the majority of these giant AVMs are amenable to direct surgical excision. It is difficult to asses, from the literature, the benefit of embolization prior to surgical excision in cases of giant AVMs. At least in one report dealing with combined treatment of seven giant AVMs, some authors stressed that preoperative embolization did not significantly alter the blood flow and, hence, potential of bleeding at the time of operation. Blood loss has not been a significant problem in our experience. When there is an associated aneurysm, it should be treated prior to or at the time of excision of the malformation.

Adolescent↗

A grading system for hippocampal sclerosis based on the degree of hippocampal mossy fiber sprouting.

In patients suffering from temporal lobe epilepsy (TLE) a highly variable degree of hippocampal sclerosis (HS) can be observed. For standard neuropathological evaluation after hippocampal resection, neuronal cell loss in the hippocampal subareas is assessed (Wyler score 0-4) [Wyler et al. (1992) J Epilepsy 5: 220-225]. Other marked morphological changes in the sclerotic hippocampus are gliosis and loss of mossy fibers in the hilus and mossy fiber sprouting in the supragranular layer. In this study we quantified changes in mossy fiber density using Timm's stain in resected hippocampal tissue from patients with various degrees of sclerosis. We found that tissue specimens from patients without sclerosis (W0) show almost no mossy fiber sprouting. Patients with moderate sclerosis show sprouting without fiber loss in the hilus, whereas specimens from patients with severe sclerosis show sprouting as well as fiber loss in the hilus. Thus, analysis of mossy fiber abundance in hilus and supragranular layer by the rapid and simple Timm's stain is a sensitive measure for hippocampal sclerosis. It provides a reliable rapid tool for neuropathological evaluation, even if the tissue only contains dentate gyrus due to the sectioning procedure.

Axons↗