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Hypothalamic hamartomas: with special reference to gelastic epilepsy and surgery.

This study presents six patients with hypothalamic hamartomas diagnosed on the basis of magnetic resonance imaging. Histological confirmation was performed in three patients who underwent surgery. Immunohistological assays were used to determine the neurosecretory pattern. Four patients presented with epilepsy, including gelastic seizures. Other symptoms included behavioral abnormalities in four patients and precocious puberty and visual impairment in two patients. One patient presented associated developmental defects. Good results without morbidity were achieved with surgical resectioning in two patients with large hamartomas associated with behavioral abnormalities and gelastic epilepsy that was unresponsive to conventional medical treatment and in one patient with visual impairment. We propose a classification of the hypothalamic hamartomas based on topographical and clinical data obtained from 36 selected cases in the literature and six of our own cases. This classification should help to classify the various treatment methods and the surgical risks into four subgroups (Types la, lb, lla, and llb). We conclude that the surgical approach is a realistic alternative in certain cases, including large and broad-based Type llb hamartomas associated with gelastic epilepsy and behavioral disorders.

Adult↗

Surgery for epilepsy: a review.

This year marks the centennial of the first surgical resection for epilepsy performed by Horsley. While epilepsy is generally considered a disease best treated with anticonvulsant medications, surgical therapy is of significant benefit to some patients in whom medical therapy has proved ineffective. Anterior temporal lobectomy has been the single most successful operation commonly performed today. In this paper we review current techniques of preoperative evaluation, the role of scalp and intracranial electrophysiologic monitoring, as well as the contribution of PET and MRI scanning to improving the selection of patients for surgical therapy. The role of focal cortical excisions, hemispherectomy, and corpus callosotomy in the surgical armamentarium is also outlined. A plea is made for establishment of additional regionalized centers for epilepsy surgery in which close collaboration among neurologists, neuropsychologists, neurosurgeons and neurophysiologists can enhance patient care and advance our knowledge of the partial epilepsies and human cerebral function.

Corpus Callosum↗

The effects of human hippocampal resection on the serial position curve.

The purpose of this study was to examine the contribution of the human hippocampal formation to the classic serial position curve. Seventy-seven patients who underwent anterior temporal lobectomy (ATL) (47 left, 30 right) were administered a list learning task before and after surgery, and changes in the serial position curve were examined. Forty nonsurgical patients with complex partial seizures were tested at comparable intervals and served as controls. Changes in the serial position curve were seen only after left ATL, and almost exclusively among patients without hippocampal sclerosis. Patients without left hippocampal sclerosis, and who therefore underwent resection of hippocampus that was to a considerable degree structurally (and presumably functionally) intact, showed significant declines in recall from the primacy and middle portions of the list compared to all other groups. There was no change in the recency portion of the list. Patients with left hippocampal sclerosis showed only a modest decline in recall from the middle region compared only to the control group, and the right ATL groups did not show any significant changes in serial position recall. These findings demonstrate the contribution of the left hippocampus to those discrete portions of the serial position curve which rely on secondary memory, and have implications for assessing the effects of ATL on memory function.

Adult↗

Integration of EEG, MRI, and SPECT in localizing the seizure focus for epilepsy surgery.

Several modalities are now available for detecting the structural and the functional abnormalities of a seizure focus. This article discusses the principles and techniques that can be used to integrate the data derived from different test modalities in delineating the seizure focus in epilepsy surgery candidates. An approach in integrating EEG, MRI, and SPECT abnormalities is described to demonstrate how the spatial relationships among them can be precisely determined by coregistering images of the abnormalities on the MRI. The recently developed technique of subtraction ictal SPECT coregistered to MRI (SISCOM) can reveal a discrete hyperperfusion focus with its relationship to the cerebral anatomy. The SISCOM focus can also serve as a target for intracranial electrode implantation and for subsequent surgical resection. This can be achieved by using a computer-based system of relating the image space to the surgical field. The limitations of each test in localizing the surgical seizure focus must be recognized when the value of each test is interpreted relative to those of other tests. In many patients, not all tests will show localizing abnormalities, and the foci determined by different tests may be incongruent. When the location of the focus is not compatible with the possible origin of the patient's habitual seizures, further evaluation with other tests, including intracranial EEG recordings, is necessary. The decision in determining which tests and how many to employ for localizing the surgical focus must be individualized for each patient.

Adult↗

Growing blood clot mimicking an aneurysm on postoperative computerized tomography and magnetic resonance imaging studies. Case report and review of the literature.

The authors describe the case of a 15-year-old boy who underwent resection of a large left temporal tumor. During a normal postoperative course, computerized tomography (CT) scanning demonstrated a spherically hyperdense structure near the internal carotid artery, enlarging on a control CT scan. A suspected false aneurysm was confirmed on magnetic resonance imaging; angiographic studies were negative. The authors believed they were dealing with a thrombosed false aneurysm and they performed operative revision. Intraoperatively the "aneurysm" could be dissected off the internal carotid artery and no lesion of the arterial wall was obvious. Histological findings showed a fresh blood clot. This case demonstrates that a blood clot may mimic an aneurysm on CT and magnetic resonance studies, which has not been described earlier. The origin of the blood clot remains unclear.

Adolescent↗

Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients.

OBJECT: Cerebral hemispherectomy for intractable seizures has evolved over the past 50 years, and current operations focus less on brain resection and more on disconnection. In addition, cases involving cortical dysplasia and Rasmussen encephalitis are being identified and surgically treated in younger individuals. Few studies have been conducted to compare whether there are perioperative differences based on hemispherectomy technique and/or pathological substrate in pediatric patients with epilepsy. METHODS: In this study the authors compared, stratified by disease, anatomical (37 cases) and Rasmussen functional hemispherectomy (32 cases) with a new modified lateral hemispherotomy (46 cases). Pathological processes included cortical dysplasia (55 cases), Rasmussen encephalitis (21 cases), infarction/ischemia (27 cases), and other/miscellaneous (12 cases). The authors found differences in perioperative clinical factors based on operative technique and/or pathological substrate. In terms of technique, the lateral hemispherotomy was associated with the least intraoperative blood loss, shortest intensive care unit stay, and lowest complication rate. The anatomical hemispherectomy was associated with the longest hospital stay, delayed oral food intake, highest postsurgery fevers, and the highest incidence of shunt requirement. The functional hemispherectomy was associated with the highest reoperation rate for recurrent seizures (25%). In terms of pathology, patients with cortical dysplasia were the youngest at surgery, suffered the greatest amount of blood loss, and required the longest operative/anesthesia times compared with the other pathologically defined groups. Postoperative seizure control (range 0.5-2 years) was not statistically different according to technique or disease process and was similar to that in cases of pediatric temporal lobe epilepsy. CONCLUSIONS: The authors found differences in perioperative risks and hospital course but not postsurgery seizure control, which vary by hemispherectomy technique and/or disease process. The modified lateral hemispherotomy approach offers various advantages related to operative blood loss and reoperation compared with anatomical and functional hemispherectomies that are especially relevant in younger patients with cortical dysplasia and Rasmussen encephalitis with small and/or malformed ventricles.

Cerebral Cortex↗

Seizure outcome after epilepsy surgery in children and adolescents.

Few epilepsy surgery outcome data are available from series of pediatric patients. We studied seizure outcome in 136 pediatric patients who had surgery for intractable epilepsy at The Cleveland Clinic between January 1990 and June 1996, with a postoperative follow-up of 1 to 7.5 years (mean, 3.6 years). Sixty-two children (3 months to 12 years old at time of surgery) were compared with 74 adolescents (13-20 years old). Extratemporal or multilobar resections and hemispherectomies were similarly frequent among children (50%) and adolescents (44%), but these procedures strongly predominated in infancy (90% of patients 0-2 years of age). The remaining patients had temporal resection. Cortical dysplasia and low-grade tumor were the most common causes and hippocampal sclerosis was rare. Seizure-free outcome was achieved for 69% of adolescents, 68% of children, and 60% of the infant subgroup, overall; for 23 (74%) of 31 children and 33 (80%) of 41 adolescents after temporal resection; for 11 (58%) of 19 children and 15 (52%) of 29 adolescents after extratemporal or multilobar resection; and for 8 (67%) of 12 children and 3 (75%) of 4 adolescents after functional hemispherectomy. Seizure-free outcome was more frequent after temporal resection (56 of 72, 78%) than after extratemporal or multilobar resection (26 of 48, 54%; 41 of 48 with a focal lesion on magnetic resonance imaging), and among patients with tumor (36 of 44, 82%) versus cortical dysplasia (16 of 31, 52%). The frequency of seizure-free outcome after epilepsy surgery was similar for infants, children, and adolescents, and comparable with results from adult series. Most patients in each age, surgery type, and causal group were free from seizures after surgery. These results suggest that children should be considered for surgical evaluation at whatever age they manifest with severe, intractable, disabling localization-related epilepsy.

Adolescent↗

Is it worth pursuing surgery for epilepsy in patients with normal neuroimaging?

OBJECTIVE: To determine whether it is worth pursuing surgery for the treatment of epilepsy in patients with normal neuroimaging. METHODS: Two patient populations were studied: (1) 136 consecutive patients who were surgically treated; (2) 105 consecutive patients assessed with chronically implanted intracranial electrodes within the same period. Sixty patients belonged to both groups, and included all 21 patients who had normal neuroimaging. RESULTS: There were no differences in the proportion of patients with favourable outcome between those with normal and those with abnormal neuroimaging, irrespective of whether intracranial recordings were required. Among the 19 operated patients with normal neuroimaging, 74% had a favourable outcome (Engel's seizure outcome grades I and II), and among the 93 patients with abnormal neuroimaging, 73% had favourable outcome (p = 0.96). In patients with temporal resections, 92% of the 13 patients with normal neuroimaging had a favourable outcome, whereas among the 70 patients with abnormal neuroimaging, 80% had a favourable outcome (p = 0.44). In patients with extratemporal resections, two of the six patients with normal neuroimaging had a favourable outcome, while 12 of the 23 patients with abnormal neuroimaging had a favourable outcome (p = 0.65). Among the 105 patients studied with intracranial electrodes, five suffered transitory deficits as a result of implantation, and two suffered permanent deficits (one hemiplegia caused by haematoma and one mild dysphasia resulting from haemorrhage). CONCLUSIONS: It is worth pursuing surgery in patients with normal neuroimaging because it results in good seizure control and the incidence of permanent deficits associated with intracranial studies is low.

Adolescent↗

Histopathological and immunohistochemical changes in neurosurgically resected epileptic foci.

Neurosurgical resection of an epileptic focus was performed in eleven patients suffering from drug resistant focal epilepsy. The clinical result was favourable in nine cases and corresponds to the earlier results. The routinely processed biopsy specimens obtained from the brain resections were stained with haematoxylin-eosin and with specific antisera to GFAP, S-100, NSE, laminin, and fibronectin using the peroxidase-antiperoxidase technique. The main pathological finding was gliosis in eight cases, neuronal degeneration in two cases, and a vascular malformation in one case. The anti-GFAP as a specific marker of astrocytes made the astroglial proliferation clearly visible, demonstrating an astroglial scar in four cases and a moderately to strongly increased amount of astroglial cells in another four cases. Anti-S-100 and anti-fibronectin are not as specific markers. They stained both neurones and glial cells with comparable results to that of anti-GFAP but with a lower specificity and sensitivity. Anti-NSE showed decreased amounts of neurones in most of the heavily gliotic lesions and also stained glial cells in some cases. Anti-laminin stained the pial and vascular basement membranes and revealed an increased vasculature in two cases. From these results, it appears that GFAP immunostaining is a highly demonstrative means for the visualization of astrogliosis in epileptic lesions and may be of help in identifying slight focal changes. An exact demonstration of neuronal loss or other neuronal changes still waits for a more specific marker than NSE. A favourable clinical outcome after neurosurgery seems to be associated with the patients showing a clearly gliotic brain lesion in one temporal lobe.

Adolescent↗

The prognostic value of residual spikes in the postexcision electrocorticogram after temporal lobectomy.

We correlated the postresection electrocorticograms (ECoGs) of 80 patients who underwent temporal lobectomy under general anesthesia for treatment of intractable complex partial seizures with surgical results in three groups: seizure/aura free (32 patients), auras only (16 patients), and one or more postoperative seizures (32 patients) at mean follow-up times of 34, 31, and 38 months, respectively. Spontaneous "residual spikes," ie, present after all resections, were present in 47% of patients who had no postoperative seizures or auras. However, they occurred in 72% of patients with any postoperative seizures (p less than 0.05). The location (convexity, mesial, or edge of resections) or the distribution (unifocal versus multifocal) of the residual spikes was not of prognostic value. Quantitative studies in 5-minute epochs of the postexcision ECoGs did not reveal a significant difference in the morphology of the residual spikes, ie, the amplitude or firing pattern (single versus polyspike), in the three groups. The group with postoperative seizures showed a higher number of spikes per epoch (greater than or equal to 50), but it was not significant. Although the study shows that patients with residual spikes may have good prognosis, they are at significantly higher risk for postoperative seizures as compared with those without residual spikes. The possibility that intensity of firing of residual spikes may be an additional predictor of outcome warrants further study.

Cerebral Cortex↗

Quinolinic acid concentrations in brain and cerebrospinal fluid of patients with intractable complex partial seizures.

Quinolinic acid (QUIN) is a neurotoxin and convulsant when injected directly into the brains of experimental animals and as such has been implicated in the etiology of human seizure disorders. In the present study, we quantified QUIN in cerebrospinal fluid (CSF) and in spiking (focus) and nonspiking (nonfocus) regions of surgically resected human temporal neocortex. L-tryptophan (L-TRP), the putative precursor of QUIN, was also measured in brain, along with CSF concentrations of L-TRP, 5-hydroxyindoleacetic acid (5-HIAA), and homovanillic acid (HVA). In brain tissue, no differences were found in the concentrations of QUIN and L-TRP between focus and nonfocus regions in 15 pairs of samples. No differences were found in CSF, L-TRP, 5-HIAA, or HVA concentrations between 11 neurologically normal controls and 15 interictal (no seizures for greater than 24 h) and 20 postictal (within 50 min of seizure) samples from epileptic patients. However, CSF QUIN concentrations were significantly lower (32%) in the epileptic patients as compared with controls, which may indicate a generalized disturbance in brain QUIN metabolism or perhaps a response to antiepileptic drugs.

Adolescent↗

Melatonin reduces low-Mg2+ epileptiform activity in human temporal slices.

Seizure susceptibility waxes and wanes in an apparently circadian manner in many epileptic patients. Fluctuations of melatonin concentration with highest levels during the night and lowest levels in the early morning could be involved in this phenomenon. Therefore, the action of melatonin on epileptic activity was tested. The experiments were carried out on human temporal neocortical slices cut from tissue resected for surgical treatment of epilepsy. Autoradiographic studies were performed on parallel slices with 100-120 pmol 2-[125I]iodomelatonin/l in the absence or presence of unlabelled melatonin. High-affinity binding sites of melatonin could be demonstrated in layers II-V of the temporal cortex. The binding was saturable, specific and occurred with low capacity. In electrophysiological studies, epileptiform field potentials were elicited by omission of Mg2+ from the superfusate and recorded from layers II-V. The frequency of occurrence of epileptiform field potentials was reduced to 0.5 of the initial value with application of melatonin (10 and 100 nmol/l) in each case. This effect was reversible upon washing. The findings favour the hypothesis that melatonin depresses epileptiform neuronal activity through specific neocortical receptors.

Autoradiography↗

Gamma Knife radiosurgery relative to microsurgery: epilepsy.

There is a strong rationale for investigation of the role of Gamma Knife radiosurgery in the treatment of medically intractable epilepsy. To explore this potential application, the well established and highly successful current outcomes associated with microsurgical treatment were reviewed, and include for temporal resections seizure-free results in 65-70% of patients, with permanent morbidity of less than 5% and mortality less than 1%. Advantages of open surgery include the opportunity to conduct electrocorticography and functional mapping, excellent visualization enabling an assured and discrete line of resection, freedom in general from volume constraints, and immediate efficacy. The preliminary evidence in support of radiosurgical treatment includes several series of patients with epilepsy associated with arteriovenous malformation, tumor, or cavernous anginoma in which results approaching those of surgery have been achieved. In a series of 15 patients with mesial temporal sclerosis, all but one patient with follow-up of at least one year are seizure-free; morbidity has been limited to one asymptomatic field defect. A series of ten patients with epilepsy associated with hypothalamic hamartoma has achieved seizure improvement in six of eight patients with at least one year follow-up, and no major morbidity has been seen. Radiosurgical treatment for intractable epilepsy appears to be effective in a majority of patients and can be performed safely and efficiently. Early experimental evidence suggests that seizure control might in some instances be achievable without ablation of the pathological target.

Epilepsy↗

[Results of surgical treatment of epilepsy with bilateral temporal lesions].

Nineteen patients with recurrence of paroxysmal syndrome after temporal focus resection and 37--after stereotaxic destruction of amygdala nuclei from both sides operated in the periods of 1967-1984 and 1989-1999, respectively, were studied. Duration of follow up study was from 1 to 35 years. The results of the study were compared to a level of patient's "real work-status" at the moment of examination. Dynamic postoperative EEG investigation revealed a number of focal activity features. Specifically, there were lost of leading focus signs, moving of intensive epileptogenesis focus to the opposite side or primary revealing of contralateral focus in different postoperative periods. Such patients are in need of anticonvulsive medicine because of possibility of a relapse within 2 or 6 years after surgery. According to the results obtained, open surgical intervention in the leading epileptic focus (anterotemporal lobectomy) in patients with bilateral temporal foci seems to be inexpedient. One of the treatment methods is stereotaxic bilateral amygdalotomy.

Adolescent↗

Surgery for intractable epilepsy: issues and outcome.

For certain forms of childhood epilepsy that remain uncontrolled despite adequate treatment with standard antiepileptic medication, surgical therapy should be considered as a potential avenue of treatment. The prognosis for seizure control after early surgery is generally favorable and is at least comparable with that of adults. Remissions are often long-standing or permanent. The results are particularly beneficial if a discrete region of abnormal epileptogenic tissue can be excised. The three major procedures--focal resection, corpus callosotomy, and hemispherectomy--have all been performed successfully in children, and their complication rates are low. With the exception of the obvious benefit conferred by alleviating seizures at a younger age, earlier surgery also improves later psychosocial status and adaptive function. By contrast, persistent seizure activity decreases the likelihood of achieving a functionally independent and economically productive adult life. The psychosocial benefits therefore constitute the most important argument in favor of early surgical intervention. Although relatively little is known about the neuropsychologic consequences of early surgery, there are few deleterious long-term cognitive or behavioral deficits if surgery is uncomplicated. However, relatively little is known about neural reorganization after early surgical excision or hemispheric disconnection, and this remains the greatest challenge for the future.

Adolescent↗

Bradycardia and sinus arrest following saline irrigation of the brain during epilepsy surgery.

Adverse cardiac events during the intraoperative period are life-threatening. The authors report three episodes of severe bradycardia and sinus arrest that occurred in a patient undergoing anterior temporal lobectomy and amygdalo-hippocampectomy for the treatment of epilepsy. The first episode occurred during resection of the amygdala; the other two episodes were observed during subsequent irrigation of the exposed brain structures, most likely the brain stem structures, because of a rent that the surgeon had deliberately made into the basilar cistern for better anatomic appreciation of the structures to be excised. The patient responded well to treatment with no adverse outcomes. The probable mechanisms leading to this event are discussed; the authors excluded insular cortex stimulation, the effects of the anesthetic drugs used, and venous air embolism as a cause of bradycardia and sinus arrest. The amygdala resection was the most likely cause of the first episode of bradycardia; the second episode of bradycardia and sinus arrest occurred because of inadvertent stimulation of brain structures by the high temperature (42 degree C) of the saline used for irrigation. To counter its effects, saline irrigation at room temperature (20 degree C) was started, and this caused the third episode of bradycardia, most likely because of "temperature shock" of the exposed brain. Prompt communication with the surgical team and vigilance are crucial for the appropriate management of such an incident, which may pose a threat to life. Avoiding irrigation of the exposed brain with high-temperature saline may prevent such an incident.

Adolescent↗

[Management of malignant gliomas diagnosed during pregnancy].

INTRODUCTION: Glioma is seldom diagnosed during pregnancy. In this situation management presents difficult problems for both neuro-oncologists and obstetricians. We report four cases and discuss the management of this unusual situation. CASE REPORT: The first patient was admitted to hospital at 29 weeks' gestation because of a generalized seizure and a right hemiparesis. MRI showed a left fronto-insular lesion. A stereotactic biopsy was obtained and revealed an anaplastic oligodendroglioma. With corticosteroids the patient remained stable until cesarean delivery at 36 weeks. In post-partum additional treatment with chemotherapy was started. The second patient was hospitalized at 26 weeks' gestation because of cranial hypertension, right hemiparesis and aphasia. MRI showed an important left fronto-parietal lesion. Partial resection was performed at 28 weeks. Histology revealed a glioblastoma multiforme. With corticosteroids the patient remained stable until cesarean delivery at 33 weeks. In post-partum additional treatment with radiotherapy and chemotherapy was started. The third patient was admitted to the hospital at 12 weeks' gestation because of cranial hypertension. MRI showed a left frontal lesion. A subtotal resection was done at 13 weeks. Histology revealed a glioblastoma multiforme. Two weeks after surgery the patient's neurological condition worsened and in agreement with the patient a therapeutic abortion was decided. Afterwards additional treatment with radiotherapy and chemotherapy was started. The last patient received combined treatment with radiotherapy and chemotherapy for local recurrence of a mesencephalic high-grade glioma. A posteriori it was discovered that the patient was at 4 months' gestation during this treatment. Cesarean delivery was done at 36 weeks. The child was normal at birth and is still in good health 5 years later. CONCLUSION: The management of gliomas diagnosed during pregnancy should not be different from the standard management of gliomas in young non-pregnant adults. Pregnant women because of their young age can have a long survival. Their pregnancy should not prevent them from receiving the best treatment for their glioma. Treatment will depend upon clinico-radiological presentation, histology, gestational age and the patient's desires. Generally speaking, surgical resection of high-grade gliomas should not be delayed during pregnancy. Progress in anesthesia and neurosurgery have greatly reduced the risks for the foetus. After delivery, if the delay between surgery and delivery is too long it is possible to begin cerebral radiotherapy during pregnancy. After the first trimester of gestation this treatment can be given without any important risks for the child.

Abortion, Therapeutic↗

Function-specific high-probability "nodes" identified in posterior language cortex.

PURPOSE: Posterior, "Wenicke's," language areas have a high degree of between-subject variability, as shown by electrical-stimulation mapping. We investigated the possibility of an organized structure in the distribution of posterior language areas. METHODS: Extraoperative subdural grid stimulation was performed on 67 left hemisphere-dominant patients before resective epilepsy surgery during counting, naming, and reading. Intersubject-averaged language maps were generated in which stimulation disrupted only one language function and not the others, or combinations of language functions. RESULTS: Language sites, although highly variable between subjects, were not organized randomly and appeared to be arranged into several focal, non-contiguous, higher probability "nodes" devoted to different aspects of language processing. Speech-arrest sites were concentrated in classic Wernicke's area. Areas where stimulation induced only reading errors were found in the posterior middle temporal gyrus and the inferior parietal lobule. These regions may correspond with an orthographic input lexicon. Areas eliciting only naming errors were found in the posterior inferior temporal gyrus extending into the mid-middle temporal gyrus and may represent a visual-representation input lexicon. Sites where stimulation elicited errors in both naming and reading were more variable in location than sites devoted to only one function, extended farther anteriorly along the temporal neocortex, and may correspond with a semantic lexicon. CONCLUSIONS: The existence of high-probability nodes in posterior language cortex supports a modality-specific modular architecture and the possibility of a conserved, universal structure.

Adolescent↗