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PubMed1999

Postoperative seizure control and antiepileptic drug use in pediatric epilepsy surgery patients: the UCLA experience, 1986-1997.

PURPOSE: Young children with refractory symptomatic epilepsy are at risk for developing neurologic and cognitive disabilities. Stopping the seizures may prevent these disabilities, but it is unclear whether resective surgery is associated with adequate long-term seizure control. METHODS: This study determined pre- and postsurgery seizure frequency and antiepileptic drug (AED) use (6 months to 10 years) in children with symptomatic seizures from unilateral cortical dysplasia (CD; n = 64) and non-CD etiologies (i.e., ischemia, infection; n = 71), and compared them with older temporal lobe epilepsy (TLE; n = 31) patients with complex partial seizures. RESULTS: Compared with presurgery, postsurgery seizure frequencies were decreased for CD, non-CD, and TLE patients (p < 0.002), and there were no differences between the three groups from 6 to 24 months after surgery (p > 0.12). At 5 years after surgery, seizure frequencies were greater in CD compared with TLE cases (p = 0.009). Compared with presurgery, the number of AEDs declined after surgery in all three groups (p < 0.002), and positively correlated with seizure frequencies (p = 0.0001). CONCLUSIONS: This study indicates that seizure relief and AED use after resective surgery for symptomatic CD and non-CD etiologies was comparable with complex partial TLE cases up to 2 years after surgery. Furthermore, at 5 years after surgery, CD patients had outcomes better than those before surgery, but worse than TLE cases. In young children, these findings support the concept that early removal of symptomatic pathologic substrates is associated with seizure control and reduced AED use, similar to that noted in older TLE cases up to 2 years after surgery. Seizure control may reduce the risk of developing the seizure-related encephalopathy associated with severe symptomatic early-onset childhood epilepsy.

Age Factors↗
PubMed2005

Social cognition and epilepsy surgery.

Human social behavior depends on a set of perceptive, mnemonic, and interpretive abilities that together may be termed social cognition. Lesion and functional imaging studies of social cognitive functions implicate the temporal lobes (in particular, the nondominant temporal lobe) and mesial temporal structures as critical at the front end of social cognitive processes. The frontal lobes, in turn, function to interpret and to modulate these processes via top-down control. Damage to frontal regions is associated with specific derangements in social behavior. Chronic focal-onset epilepsy and its surgical treatment commonly affect these neuroanatomic regions and might therefore impact social function. Postoperative social function helps determine quality of life for both patients and families. There is some evidence that resective seizure surgery affects social cognition, but there are significant weaknesses in our current knowledge that can be overcome with comprehensive longitudinal research.

Cognition↗
PubMed2006

Papillary glioneuronal tumor.

The descriptive term papillary glioneuronal tumor (PGNT) has been repeatedly applied to a morphologic subset of low-grade mixed glial-neuronal neoplasia of juvenile and young adult patients. We report on a 13-year-old boy with PGNT of the left temporal lobe, who presented with headaches and a single generalized seizure. On magnetic resonance imaging, tumor was seen as a large, moderately enhancing paraventricular mass with cyst-mural nodule configuration and slight midline shift. Perifocal edema was virtually absent. Gross total resection could be performed, followed by an uneventful recovery. Histologically, the tumor exhibited similar, if not identical, features as reported previously. These comprised a patterned biphasic mixture of sheets of synaptophysin-expressing small round cells and pseudorosettes of GFAP-positive rudimentary astrocytes along vascular cores. Focally, the latter imprinted a pseudopapillary aspect on this otherwise solid lesion. Both cellular components expressed non-polysialylated neural cell adhesion molecule (NCAM)-L species, and several overlapping areas of synaptophysin and GFAP immunoreactivity were present. The mean MIB-1 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation, or necrosis were consistently lacking. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular neurocytic neoplasia, whose paradigmatic presentation may allow for consideration as an entity.

Adolescent↗
PubMed1998

Successful surgical removal of a large arteriovenous malformation in a patient with hemophilia: case report.

OBJECTIVE AND IMPORTANCE: This is the first reported case of the successful surgical removal of a large arteriovenous malformation (AVM) in a patient with hemophilia A. CLINICAL PRESENTATION: A 19-year-old male patient was admitted to our department with intracranial hemorrhage. He had previously been diagnosed with hemophilia A and a cerebral AVM. Carotid angiography revealed a large AVM in the right temporal and parietal lobes. The neurological and neuroradiological findings, especially those of single photon emission computed tomography, identified an area of devitalization around the lesion, which was thought to reduce the risk of new deficits resulting from surgical manipulation. INTERVENTION: We resected the AVM in conjunction with supplemental infusions of Factor VIII before, during, and after the operation. A slight cerebral hemorrhage on the 7th postoperative day was observed despite control with Factor VIII, but the patient was discharged without any new deficits. CONCLUSION: We evaluated and managed all problems of a patient with multiple complications and achieved a medical cure.

Adult↗
PubMedDate not supplied

Restored activation of primary motor area from motor reorganization and improved motor function after brain tumor resection.

BACKGROUND AND PURPOSE: Reorganization of brain function may result in preservation of motor function in patients with brain tumors. The goal of the present study was to investigate whether function of the primary motor area (M1) was restored and whether motor function improved after brain tumor resection. METHODS: Five patients with metastatic brain tumors located within or near M1 underwent awake surgery with intraoperative cortical mapping and continuous task monitoring. Preoperative and postoperative functional MR imaging (fMRI) was performed during hand clenching, and diffusion tensor imaging (DTI) was performed in 1 case to further characterize the area activated in fMRI. RESULTS: Preoperative fMRI performed during hand clenching demonstrated reorganization of motor function. In patients with severe paresis (cases 3, 4, and 5), clenching of the affected hand induced a large blood oxygen level-dependent response in the right hemisphere, mainly in the anterior temporal lobe, despite the location site of the tumor. Postoperative fMRI during hand clenching demonstrated activation of the contralateral M1. Furthermore, in case 5, DTI detected tracts, possibly the inferior longitudinal fasciculus, arising from anterior temporal activated area as well as tracts connecting the premotor and M1 activated area. This patient demonstrated mirror movement of the hand during the course of motor function recovery. CONCLUSIONS: Tumor resection resulted in restoration of M1 function and improved motor function in patients with preoperative reorganization of M1 function. Furthermore, the preoperative reorganization of motor function in cases with severe paresis may be related to changes in the right hemisphere, including the temporal lobe.

Aged↗
PubMed1996

[A case of intractable temporal lobe epilepsy: discrepancy between epileptogenic area and structural lesion demonstrated by subdural recording].

We reported a 25-year-old man with intractable right temporal lobe epilepsy in whom subdural recording successfully delineated the epileptogenic area in the right anterior mesial to basal temporal region in spite of the presence of a postoperative lesion in the right posterior temporal region. The patient underwent right posterior temporal resection due to ependymoma at the age of 8 years. Since age 19, he had suffered from medically intractable psychomotor seizures. Ictal scalp-recorded EEG suggested an epileptogenic area at the right temporal area, but it could not specifically demarcate the site because of bone deficits from the previous surgery. Ictal SPECT showed a hyperactive area in the right anterior temporal area, which was inconsistent with the MRI finding. In case of partial epilepsy associated with structural lesion like in the present case, if results of various non-invasive studies are discrepant as to the epileptogenic area, invasive studies using, for example, subdural EEG monitoring is necessary to determine the responsible area.

Adult↗
PubMed1995

Lymphocytic hypophysitis: a rare cause of hyperprolactinaemia.

Lymphocytic hypophysitis is one of the rare causes of hypopituitarism and intrasellar mass observed almost exclusively in women. A temporal relationship to pregnancy has been identified in many but not all of the 34 reported cases. Intra-operative differentiation from non-secretory macroadenoma is important, as resection of the anterior lobe is unnecessary in these individuals and pituitary function may spontaneously return to normal. The authors present the case of a woman with symptomatic hyperprolactinaemia 10 years after her last pregnancy.

Adult↗
PubMedDate not supplied

[In vivo and in vitro neurophysiological features of epilepsy].

INTRODUCTION: Some electrophysiological aspects of the interictal activity in temporal lobe epilepsy (TLE) are under debate. However, surgery is often guided by both, pre and intra operative recordings of interictal activity. Some of these studies include intraoperative electrocorticography (ECoG) that guide tailored resection. OBJECTIVE: To analyze several aspects of interictal activity recorded by ECoG and to explore its electrophysiological basis in vitro. PATIENTS AND METHODS: ECoGs from thirteen patients suffering drug resistant TLE were analyzed. Both linear and non linear methods were introduced to explore the ECoG signals. In two cases, neocortical tissue was also electrophysiologically studied in vitro. RESULTS: Interictal ECoG activity showed a mesial origin, a cortical origin or a mixed mesial/temporal origin. Both in cortical and mesial/cortical origin, cortical interictal ECoG signals are characterized by spatiotemporal clusters of activity. In vitro study of cortical tissue from these clusters showed alterations in the synaptic control of excitability. CONCLUSION: Our results suggest an electrophysiological basis of interictal activity. Combination of linear and non linear methods is a very useful tool to explore on line the interictal ECoG activity during surgery.

6-Cyano-7-nitroquinoxaline-2,3-dione↗
PubMed1995

Evaluation for surgical treatment of partial epilepsy: an overview.

When a focal epilepsy proves refractory to medical therapy, surgical treatment is available and increasingly used. Most interventions consist of focal cortical resections, and by far the most common operation is a temporal lobectomy. The presurgical evaluation is complex and multidisciplinary. It includes clinical evaluation, EEG-video monitoring, neuropsychological testing, and structural as well as functional imaging. When surface EEG fails to identify the epileptogenic zone with sufficient confidence, several invasive methods are available, each with its advantages and limitations. In addition to neurophysiologic data, when there is convergence of structural imaging (MRI) and functional testing (Wada test, neuropsychological evaluation, nuclear imaging), a single focus can be identified, and a focal resection is likely to be successful. Surgery is a well-accepted and effective therapeutic modality for patients with refractory epilepsy. When surgical candidates are selected appropriately, results are excellent, especially for temporal lobe epilepsy.

Electroencephalography↗
PubMed2006

Caspase-3 cleavage and nuclear localization of caspase-activated DNase in human temporal lobe epilepsy.

Programmed cell death (apoptosis) signaling pathways have been implicated in seizure-induced neuronal death and the pathogenesis of human temporal lobe epilepsy (TLE). End-stage DNA fragmentation during cell death may be mediated by nucleases including caspase-activated DNase (CAD), apoptosis-inducing factor (AIF) and endonuclease G. In the present study, we investigated the subcellular localization of these nucleases in resected hippocampus from TLE patients and autopsy controls. Subcellular fractionation determined levels of CAD were significantly higher in the nuclear fraction of TLE samples compared with controls, and semiquantitative immunohistochemistry revealed cleaved caspase-3 positive cells in TLE sections but not controls. While mitochondrial levels of AIF and endonuclease G were higher in TLE samples than controls, nuclear localization of AIF was limited and restricted to cells that were negative for cleaved caspase-3. Nuclear accumulation of endonuclease G was not found in TLE samples. These data support ongoing caspase-dependent apoptosis signaling in human TLE and suggest that interventions targeting such pathways may have potential as adjunctive neuroprotective therapy in epilepsy.

Apoptosis↗
PubMed2006

Large-scale expression study of human mesial temporal lobe epilepsy: evidence for dysregulation of the neurotransmission and complement systems in the entorhinal cortex.

Human mesial temporal lobe epilepsies (MTLE) are the most frequent form of partial epilepsies and display frequent pharmacoresistance. The molecular alterations underlying human MTLE remain poorly understood. A two-step transcriptional analysis consisting in cDNA microarray experiments followed by quantitative RT-PCR validations was performed. Because the entorhinal cortex (EC) plays an important role in the pathophysiology of the MTLE and usually discloses no detectable or little cell loss, resected EC and each corresponding lateral temporal neocortex (LTC) of MTLE patients were used as the source of disease-associated and control RNAs, respectively. Six genes encoding (i) a serotonin receptor (HTR2A) and a neuropeptide Y receptor type 1 (NPY1R), (ii) a protein (FHL2) associating with the KCNE1 (minK) potassium channel subunit and with presenilin-2 and (iii) three immune system-related proteins (C3, HLA-DR-gamma and CD99), were found consistently downregulated or upregulated in the EC of MTLE patients as compared with non-epileptic autopsy controls. Quantitative western blot analyses confirmed decreased expression of NPY1R in all eight MTLE patients tested. Immunohistochemistry experiments revealed the existence of a perivascular infiltration of C3 positive leucocytes and/or detected membrane attack complexes on a subset of neurons, within the EC of nine out of eleven MTLE patients. To summarize, a large-scale microarray expression study on the EC of MTLE patients led to the identification of six candidate genes for human MTLE pathophysiology. Altered expression of NPY1R and C3 was also demonstrated at the protein level. Overall, our data indicate that local dysregulation of the neurotransmission and complement systems in the EC is a frequent event in human MTLE.

Adult↗
PubMed2004

Non-enhancing de novo glioblastoma: report of two cases.

Malignant gliomas arise from two distinct pathways, as de novo lesions or from secondary transformation from low-grade lesions. Herein, we describe the cases of two patients to illustrate the proposition that de novo malignant gliomas can originate as non-enhancing tumors and rapidly progress to a pattern of ring enhancement characteristic of a glioblastoma. Both patients presented with new-onset seizures (simple partial and generalized). Their neurological examinations were unremarkable. Initial MRI evaluations revealed a right precentral gyrus and right medial temporal lobe lesions in each case, respectively. These lesions demonstrated increased T2 signal changes without contrast enhancement. The biopsy of the right frontal lesion in the first patient was consistent with an anaplastic astrocytoma; the second patient was followed expectantly. Repeat MRI for both patients within 17 weeks disclosed ring-enhancing lesions, consistent with an unusually rapid evolution to glioblastoma multiforme (GBM). Subsequent resection of the right medial temporal lesion in the second patient revealed a GBM. Neither tumor displayed abnormal overexpression of P53 by immunohistochemistry. Early MRI of de novo glioblastomas may demonstrate a non-enhancing tumor suggestive of a low-grade lesion. These tumors can rapidly evolve into ring-enhancing lesions more consistent with the traditional imaging findings.

Adult↗
PubMed2001

Pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection.

To describe the clinical features, histologic characteristics, and management of patients with pleomorphic xanthoastrocytoma (PXA), we reviewed data on 13 children who had histologically confirmed PXA and were referred to the neuro-oncology service between 1985 and 1999. Neuro-imaging with CT and/or MRI documented the anatomic location, tumor extent, and degree of resection. There were 3 males and 10 females; median age was 12.9 years (range, 8.2-17.2 years). The most frequent presentations included seizures (n = 8) and headache (n = 5). Tumor sites included temporal (n = 5), parietal (n = 3), frontal (n = 1), frontoparietal (n = 1), parietooccipital (n = 1), and temporoparietal (n = 1) lobes and the spinal cord (n = 1). CT/MRI revealed a cystic component in 6 patients, with cyst wall enhancement in 3 patients. The solid component was uniformly enhancing in 11 patients. Vasogenic edema was present in 9 patients, and calcification was noted in 4 patients. Histopathologic findings included meningeal invasion in 12 patients, calcifications in 4, and necrosis in 2. Mitotic figures (1-12 per high-power field) were seen in 8 patients. Gross total resection was achieved in 8 patients, near total resection in 1, and subtotal resection in 4. Ten patients were alive with a median follow-up of 41 months at this writing. Two patients died of progressive disease, and 1 died of an unrelated cause. In conclusion, pleomorphic xanthoastrocytoma is a rare neoplasm in childhood, commonly presenting with seizures. Gross total resection without adjuvant therapy provides prolonged disease control, as seen in 6 of 7 patients (85%) in our series.

Adolescent↗
PubMed1986

Metabolic, morphologic and electrophysiologic profiles of human temporal lobe foci: an attempt at correlation.

Our current understanding of focal seizures strongly suggests a model of damage and associated synaptic reorganization that leads to periodic 'spontaneous' hyperexcitation and/or sustained firing that generates seizures. Our working model of a human epileptic focus assumes that there will often be inexcitability near the damage (hippocampus proper); however, the anomalous circuitry will occasionally lead to hyperexcitability whenever these anomalous (epileptic) circuits are activated synchronously in sufficient numbers to propagate discharges to normal tissue (e.g., the presubiculum) which would be normally excitable. The key role of rebound excitation following prolonged inhibition in the hippocampus is strongly supported by both physiologic and GAD immunologic results. Our ability to directly test the level of excitability of hippocampal and presubicular neurons in vivo, followed by microanatomical studies of the same resected tissue, will allow us to test our model and revise it as our results become more complete. Also, our findings will be important for relating synaptic mechanisms of seizure genesis to those demonstrated in various experimental models of focal hippocampal seizures.

Epilepsy, Temporal Lobe↗
PubMed1996

[A successfully operated case of tuberous sclerosis presenting with intractable epilepsy].

A 20-year-old male patient with tuberous sclerosis was admitted to our hospital complaining of intractable epilepsy. He had been suffering from frequent seizures despite anticonvulsant treatment since the age of 1 year. CT scan and magnetic resonance images showed multiple calcified lesions at the paraventricle and temporal lobe on both sides. Electroencephalogram showed left temporal lobe dominant spike waves. After deep electrodes were inserted into the left amygdala and hippocampus and subdural electrodes were placed on the left temporal cortex, clinical seizures were monitored by video and EEG. The primary focus was found in the amygdala. Left temporal lobectomy was carried out through the cranio-orbital zygomatic approach. Postoperatively, the patient has been seizure-free for one year. Despite multiple intracerebral lesions in tuberous sclerosis, resection of the primary epileptogenic focus is needed to solve the seizure problem if the focus is localized.

Adult↗
PubMed1999

Prediction of presence of hippocampal sclerosis from intracarotid amobarbital procedure memory asymmetry scores and epilepsy on set age.

Identification of the pathological status of the hippocampus prior to surgery is important since the absence of hippocampal sclerosis (HS) carries risks to memory function following anterior temporal lobectomy (ATL). We studied 62 patients undergoing ATL (31 L, 31 R) for intractable epilepsy of temporal lobe origin in whom no pathology was identified apart from HS. An intracarotid amobarbital procedure (IAP) was performed as part of the preoperative evaluation. All patients were left hemisphere dominant for language. IAP memory testing was according to the protocol of Loring. We examined IAP memory asymmetry scores at four levels of difference (<2, > or =2, > or =4, > or =6) as a function of the presence (HS+) or absence (HS-) of HS. A logistic regression analysis was performed with HS+ as the dependent variable, and age at onset of epilepsy, age at time of surgery, gender, side of surgery and significant IAP memory asymmetry as independent variables. At each level of memory asymmetry, onset age and memory asymmetry were the only predictors of HS+. Younger age at onset was associated with HS+. Curves were constructed showing probability of HS+ for age at onset for each level of asymmetry. These can be used to predict the likelihood of presence of HS based on age at onset of epilepsy and the IAP memory asymmetry score. It is concluded that IAP memory asymmetry scores reflect the functional and pathological status of the hippocampus, and greater asymmetry increases the probability of finding HS in the resected hippocampus.

Adult↗
PubMed2001

Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: a case report and review of the literature on de novo growth of cavernomas.

BACKGROUND: Cerebral cavernomas are rare vascular anomalies and their expansile growth has been considered to be mainly due to recurrent hemorrhages. They are not generally reported to show aggressive behavior. CASE DESCRIPTION: A 27-year-old male presented with headache, visual disturbances, and a 17-year history of seizures. He was known to have a temporal lobe lesion on CT scan, consistent with a diagnosis of cavernous malformation but with no relevant family history. Serial clinical and radiological follow-up revealed a progressive increase in the size of the lesion with formation of a growing cyst of 7 cm in diameter, which produced mass effect, resulting in the clinical picture of cerebral herniation. The patient underwent emergency surgical resection with symptomatic relief. There was no evidence of significant hemorrhage at surgery. CONCLUSION: Cavernous malformations can show expansile growth without any evidence of a presaging hemorrhagic event and, in the manner of a neoplastic lesion, present with raised intracranial pressure and cerebral herniation.

Adolescent↗