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Clear cell pleomorphic xanthoastrocytoma: case report.

Pleomorphic xanthoastrocytoma (PXA) is a well-described astrocytic neoplasm with distinctive clinical and pathological features. Although most patients with PXAs are cured by surgical excision, other patients experience malignant progression and tumor recurrence. We describe a 47-year-old woman with a left temporal lobe PXA that had classic histopathological characteristics as well as extensive clear cell and focal papillary changes, and some anaplastic findings. The patient has now suffered two recurrences after complete resection. The case illustrates a rare, previously undescribed histological variant of PXA, with a prominent clear cell and focal papillary morphology. The study of histologically similar cases is needed to determine whether this variant is always associated with a greater likelihood of recurrence.

Astrocytoma↗

Compulsive polydipsia following meningioma resection: an epileptic phenomenon? Case report.

The authors report the case of an individual who developed compulsive polydipsia following resection of a left sphenoidal ridge meningioma. The episodic, stereotyped nature of his symptoms, response to treatment, and electroencephalographic and magnetic resonance imaging findings are all highly consistent with temporal lobe-onset epilepsy. The pathophysiology of this underrecognized phenomenon is discussed.

Adult↗

["Adenoid" glioblastoma].

A surgical case of glioblastoma which showed a pronounced "adenoid" (or "epithelioid") appearance was reported. The patient was an 81-year-old woman, who presented with unsteady gait. Neuroradiological examination revealed three discrete mass lesions located in the 1-frontal, 1-parieto-occipital, and r-occipito-temporal lobes. Despite the subtotal removal of two of the three lesions and postoperative chemotherapy and radiotherapy, the patient died 21 months after the onset of illness. Histopathological examination of the resected tumors revealed typical features of glioblastoma in the peripheral region of the tumor. In the central region, the tumor cells were arranged in a papillary fashion or formed solid, sheet-like cell nests and were surrounded by fibrous connective tissue septa. Although the histopathological appearance of the tumor closely resembled metastasis of adenocarcinoma, the immunohistochemical and ultrastructural studies of the tumor failed to detect evidence of a definite differentiation towards epithelial cells.

Aged↗

Emotional responses to unpleasant music correlates with damage to the parahippocampal cortex.

Music is typically a pleasurable experience. But under certain circumstances, music can also be unpleasant, for example, when a young child randomly hits piano keys. Such unpleasant musical experiences have been shown to activate a network of brain structures involved in emotion, mostly located in the medial temporal lobe: the parahippocampal gyrus, amygdala, hippocampus and temporal pole. However, the differential roles of these regions remain largely unknown. In this study, pleasant and unpleasant music was presented to 17 patients with variable excisions of the medial temporal lobe, as well as to 19 matched controls. The pleasant music corresponded to happy and sad selections taken from the classical instrumental repertoire; the unpleasant music was the dissonant arrangement of the same selections. Only patients with substantial resections of the left or right parahippocampal cortex (PHC) gave highly abnormal judgements to dissonant music; they rated dissonant music as slightly pleasant while controls found it unpleasant. This indifference to dissonance was correlated with the remaining volume in the PHC, but was unrelated to the volume of the surrounding structures. The impairment was specific: the same patients judged consonant music to be pleasant, and were able to judge music as happy or sad. Furthermore, this lack of responsiveness to unpleasantness was not due to a perceptual disorder, because all patients were able to detect intentional errors in the musical excerpts. Moreover, the impairment differed from that induced by amygdala damage alone. These findings are consistent with a two-dimensional model of defensive responses to aversive stimuli, in which the PHC and the amygdala subserve different roles.

Adult↗

Surgical treatment for neocortical temporal lobe epilepsy: clinical and surgical aspects and seizure outcome.

OBJECT: The goal of this paper was to describe the clinical and surgical aspects of a group of patients suffering from drug-resistant neocortical temporal lobe epilepsy (TLE), as well as seizure outcomes and factors affecting seizure outcomes in these patients. METHODS: This study was based on data prospectively collected and retrospectively evaluated. Sixty-two patients with neocortical TLE constituted the study population. Only patients who underwent corticectomies, lesionectomies, lateral anterior lobe resections, and/or multiple subpial transections were included. The pathological areas resected in these patients could be separated into three groups composed of 35 neoplastic lesions, 23 nonneoplastic lesions, and three nonlesional areas. The mean duration of follow-up review in these patients was 21.9+/-14 months. Outcomes were categorized according to Engel classes. Class I was found in 79% of the patients and Class II in 11%. Invasive presurgical evaluation was performed in 43% of the patients. There were only temporary complications (3.3% surgical and 1.6% neurological) and no deaths. In summary, lesions confirmed on histological examination were rarely found in patients with neocortical TLE. Low-grade tumors were the most commonly found lesions in these patients and the most common tumor was ganglioglioma. Outcome was best for those patients with neoplastic lesions and was independent of the duration of their seizures. Outcome was little influenced by the type of resection performed and was found to be as good as that achieved in patients with mesial TLE. CONCLUSIONS: These results demonstrate that the concept of lateral or neocortical TLE as a distinct entity is useful. Surgery for neocortical TLE can be considered a viable treatment option that is associated with a low morbidity rate and good outcomes.

Adolescent↗

How large must an epileptic focus be to cause an electrographic status epilepticus--a case report.

Based on experimental data from animal studies different theories regarding the size of an epileptic focus have been postulated which range from single pacemaker cells to extended neuronal networks. We report a case which gives further information about the size of a human epileptic focus which can trigger manifest epileptic seizures. We report a 22-year-old man with medically refractory temporal lobe epilepsy. This patient suffered from brief complex partial seizures and frequent epigastric auras. To differentiate a mesiotemporal from a temporolateral seizure origin the patient was implanted with a 10 contact depth electrode from a posterior approach into the right hippocampus, and additional temporobasal/temporolateral subdural strip electrodes. Depth recordings revealed an electrographic status with continuous rhythmic sharp wave activity (1 Hz), the field of which was confined to a diameter of less than 1 cm in the anterior hippocampus, whereas temporobasal subdural strip electrodes did not display this activity. Periodically, spread of this activity occurred to the amygdala, to the posterior part of the hippocampus, and less often to the temporobasal cortex. Most seizure patterns remained subclinical, few of them became symptomatic as partial seizures. This case demonstrates that a hippocampal epileptic focus causing electrographic focal status epilepticus may be limited to a volume of less than 1 cm in diameter. This observation is discussed with regard to implantation strategies and to possible superselective resective or modulatory approaches in the treatment of such limited epileptogenic areas.

Adult↗

Focal cortical dysplasia of Taylor, balloon cell subtype: MR differentiation from low-grade tumors.

PURPOSE: To test the hypothesis that focal cortical dysplasia of Taylor (FCDT) can be distinguished from low-grade tumors by means of clinical and MR findings. METHODS: We examined 10 clinical and 19 MR imaging variables in patients who underwent surgery for intractable epilepsy over an 8-year period. The 54 patients with low-grade glial neoplasms were compared with the eight patients who had balloon cell FCDT. RESULTS: Statistically significant differences were seen with respect to eight of the MR variables and none of the clinical variables. MR findings suggesting dysplasia rather than tumor included the presence of gray matter thickening associated with a homogeneous hyperintense signal in the subcortical white matter that tapers as it extends to the lateral ventricle. A frontal lobe location favors dysplasia, while a temporal lobe (especially medial temporal lobe) location is more suggestive of a neoplasm. CONCLUSION: Several MR features help distinguish balloon cell FCDT from neoplasms, especially cortical thickening and a tapered signal to the ventricle. This distinction is important for surgical planning, as the decision to operate and the extent of surgical resection often depend on the presence or absence of neoplastic tissue.

Adolescent↗

Medial temporal lobe epilepsy: videotape analysis of objective clinical seizure characteristics.

PURPOSE: The syndrome of temporal lobe epilepsy has been described in great detail. Here we focus specifically on the clinical manifestations of seizures originating in the hippocampus and surrounding mesial temporal structures. METHODS: Seizure origin was confirmed in 67 cases by depth EEG recording and surgical cure after mesial temporal resection. RESULTS: Among nonlateralized manifestations, we commonly found oral automatisms, pupillary dilatation, impaired consciousness, and generalized rigidity. Appendicular automatisms were often ipsilateral to the seizure focus, whereas dystonia and postictal hemiparesis were usually contralateral. Head deviation, when it occurred early in the seizure, was an ipsilateral finding, but was contralateral to the seizure focus when it occurred late. Clear ictal speech and quick recovery were found when seizures originated in the non-language-dominant hemisphere, but postictal aphasia and prolonged recovery time were characteristic of seizure origin in the language-dominant hemisphere. CONCLUSIONS: These signs help to define the mesial temporal lobe epilepsy (MTLE) syndrome and often provide information as to the side of seizure origin.

Automatism↗

The effects of lateralized temporal lobe dysfunction on formal and semantic word fluency.

Word fluency performance was studied in 32 patients with unilateral temporal lobe epilepsy (TLE) and 25 healthy dextral controls. Two word fluency tasks were administered conforming to either formal-based criteria or semantic-based criteria. Performance for TLE patients was assessed both pre-operatively and approximately 1 week following anterior temporal lobectomy. Both formal and semantic word fluency decreased regardless of resection laterality. Left TLE patients performed significantly worse at both pre- and post-operative assessments compared to the right TLE patients, while right TLE patients performed significantly poorer than controls on all verbal fluency criteria at pre- and post-operative assessments. In addition, both TLE and control groups produced significantly fewer formal and semantic words during the second 30-sec portion of each task. These findings are discussed in terms of temporal lobe contributions to word fluency production and lexical semantic processing.

Adult↗

Electrocorticography and outcome in frontal lobe epilepsy.

The prognostic significance of epileptiform activity (EA) recorded at electrocorticography (ECOG) was examined in a group of 60 consecutive non-tumoral patients with intractable frontal lobe epilepsy (FLE). Pre-excision EA was documented as absent, focal (one gyrus), regional (two gyri), lobar (3 gyri) or multilobar (frontal + temporal gyri). Post-excision EA was documented as absent, restricted to the resection border, or recorded distant to the resection border, and was quantitated by spike frequency. Pre-excision EA from < or = 2 gyri and absence of post-resection EA correlated with Class I or II (Engel classification) outcome while pre-excision EA from > or = 3 gyri and persistent post-resection EA, especially distant to the resection border, correlated with Class III or IV outcome (P < 0.001). A significant correlation between poorer outcomes and increased abundance of distant post-resection EA was observed (P < 0.001). EA restricted to the resection border was not significantly correlated with outcome. Presence of a circumscribed lesion correlated with Class I outcome (P < 0.01) and absence of pathological abnormality correlated with Class IV outcome (P < 0.05). Neither side nor extent of surgical excision correlated with outcome. EA recorded at ECOG is of prognostic significance in FLE. A lobar or multilobar distribution of pre-excision EA and persistent post-excision EA distant to the resection border, especially when abundant, are highly unfavorable prognostic indicators. In contrast, a restricted distribution of pre-excision EA and absence of post-resection EA both herald a favorable outcome.

Adolescent↗

Neuronal recordings in human lateral temporal lobe during verbal paired associate learning.

The temporal lobe has a role in memory and learning. The objective of this study was to assess the activity of neurons in the temporal cortex of humans during learning. Single neuron activity was recorded with extracellular microelectrodes in adults undergoing awake craniotomies for resections for medically intractable epilepsy. Participants were administered control tasks of overt reading, silent reading, nonword visual controls and a recent verbal memory task with distracters followed by a verbal paired associates (PA) paradigm. Forty-nine neurons were identified and characterized by their relationship to language and memory. Neurons related to overt speech (Type A neurons) showed increased activity during learning in patients who were good learners compared to patients who were poor learners. Neurons not related to language or to memory (Type D neurons) showed increased activity with exposure to unlearned word pairs in poor learners compared with those who were good learners. Activity in all groups diminished with practice. Levels of activity in specific types of neurons in human temporal lobe differ in patients who learn word pairs rapidly or poorly.

Craniotomy↗

Outcome after surgery in patients with refractory temporal lobe epilepsy and normal MRI.

Our purpose is to determine predictors of outcome in patients with refractory temporal lobe epilepsy and normal high resolution magnetic resonance imaging (MRI) who undergo surgical therapy. We identified 23 patients who underwent temporal lobectomy and had normal pre-operative MRI, including surface coil phased array temporal lobe imaging. All were followed at least 2 years after surgery. We graded outcome as seizure-free, > 75% reduction in seizures, or < 75% reduction in seizures. We examined pre-operative interictal and ictal electroencephalographic (EEG) findings, age of onset, gender, duration of epilepsy, risk factors, family history, physical findings, age at operation, side of operation, and pathology of resected tissue in order to determine if any of these factors were associated with outcome. Overall, 48% (11/23) of patients were seizure-free, 39% (9/23) had > 75% reduction in seizures, while 13% (3/23) had < 75% reduction in seizures. Only the EEG findings were useful in predicting outcome. When ictal onsets arose from basal-temporal regions, 61% (11/18) of patients were seizure-free, while none (0/5) were seizure-free when seizures arose from mid-posterior temporal regions (P = 0.04). Interictally, if all epileptiform patterns were localized exclusively to one basal-temporal region, a finding that invariably correlated with ictal onsets, 78% (7/9) of patients were seizure-free, while only 29% (4/14) were seizure-free if discharges were bilateral or multifocal (P = 0.04). We conclude that surgery may be a reasonable treatment for some patients with intractable temporal lobe seizures and normal MRI. The best outcomes occur when seizure onsets and interictal epileptiform patterns are exclusive to one basal-temporal region. Unfavorable outcomes are most likely to occur when ictal origins are from mid-posterior temporal regions and when interictal discharges are bitemporal or multifocal in distribution.

Adolescent↗

Comparative study of interictal PET and ictal SPECT in complex partial seizures.

OBJECTIVE: To compare the sensitivity of ictal 99mTc-HMPAO single photon emission computed tomography (SPECT) with interictal 18F-fluoro-deoxyglucose positron emission tomography (PET) in localization of the epileptogenic focus in patients with medically intractable complex partial seizures (MI-CPS). MATERIAL AND METHODS: Retrospective analysis was performed on patients with MI-CPS who underwent anterior temporal lobectomy from January 1993 onwards when PET became available to us for clinical studies at the Indiana University Medical Center. There were 38 female and 29 male patients (total = 67) with MI-CPS, 10 to 55.5 years of age (mean 31) and duration of their epilepsy from 1-46 years (mean 21). Interictal PET was evaluated for evidence of focal hypometabolism and ictal SPECT for focal perfusion abnormality (hyperperfusion or hypoperfusion) by visual analysis. RESULTS: Both ictal SPECT and interictal FDG-PET studies were obtained in 36 patients with MI-CPS. PET showed definite hypometabolism in 30 and questionable hypometabolism in an additional two patients. Ictal SPECT correctly localized the seizure focus in 27 patients by demonstrating ictal hyperperfusion whereas in one the hyperperfusion was falsely localized. In an additional seven patients the ictal SPECT provided probable localization by demonstrating ictal hypoperfusion in the appropriate temporal lobe. The sensitivity of ictal SPECT and interictal PET was 34/36 and 32/36, respectively, the difference was not statistically significant (chi 2y = 0.18, DF = 1, P = 0.67). In six of the 36 patients the two tests were complementary to each other in providing localizing information. CONCLUSION: Ictal SPECT and interictal PET are equally sensitive and reliable techniques in localizing the epileptogenic focus in patients with MI-CPS. They play a critical role in providing localization in MRI negative patients allowing surgical resection to be undertaken in many without additional invasive electrographic monitoring.

Adolescent↗

The 'foramen ovale electrode': a new recording method for the preoperative evaluation of patients suffering from mesio-basal temporal lobe epilepsy.

A technique to record from the mesio-basal aspect of the temporal lobe by a subdural electrode inserted through the foramen ovale (FO) is described. For this purpose a special electrode was developed that, in addition, allowed epicortical DC recording. After having tested its value experimentally in dogs with penicillin-induced hippocampal seizure foci, this 'FO electrode' was subsequently applied to 10 patients. Nine of them suffered from drug-resistant complex partial seizures suspected to be of mesio-basal limbic origin. Seven patients had in addition to the FO electrode depth electrodes stereotactically implanted within the hippocampus and nucleus amygdalae, thus allowing an evaluation of this new FO recording technique by direct comparison with stereo-EEG. Our results indicate that interictal epileptiform potentials as well as ictal discharges are reliably picked up by the FO electrode whenever nucleus amygdalae, hippocampus and parahippocampal gyrus show synchronous epileptic activity. As a rule, these events are not reflected in conventional scalp EEG records. Epileptiform activity in subcompartments of the mesio-basal limbic structures is not reliably reflected by the FO electrode. Negative epicortical DC shifts recorded by the FO electrode are a sensitive indicator of epileptic activity within the underlying deeper structures. In conclusion, in certain patients with drug-resistant epilepsy of suspected mesio-basal limbic origin the use of FO recording in the presurgical evaluation might provide sufficient information to give an indication for resective surgery, either by anterior temporal lobectomy or by selective amygdalo-hippocampectomy.

Amygdala↗

Variability of presentation in medial temporal lobe epilepsy: a study of 30 operated cases.

INTRODUCTION: To evaluate the homogeneity of patients operated on for MTLE. MATERIAL AND METHODS: 30 consecutive patients prospectively investigated with clinical, EEG, neuropsychology, MRI, FDG-PET criteria and eventually intracranial EEG, who underwent antero-medial temporal resection (follow-up: 24 months). RESULTS: Clear and well-lateralised MTLE was non-invasively ascertained in 22 patients (73%). Eight patients (27%) had a less pure presentation due to divergent scalp EEG features (bitemporal, widespread or extratemporal), unusual auras or absence of MRI-based hippocampal sclerosis or FDG-PET hypometabolism. They were explored by invasive monitoring which confirmed medial temporal origin of seizures. Outcome was excellent in 95% of the pure cases (Engel's class I) and less favorable in the more difficult ones (65% of class I). CONCLUSIONS: MTLE criteria, including the strong contribution of brain imaging, permit to select accurately a large percentage of patients. However a consistant number of patients present a less pure presentation suggesting more complex epileptogenic networks.

Adolescent↗

Right atrial metastatic melanoma in a patient with transient ischemic attacks.

A 65-year-old-man was admitted for evaluation of a transient ischemic attack. A 4.5 x 5.3-cm right atrial mass and a patent foramen ovale were identified by echocardiography. A 0.5-cm lesion was identified in the left temporal lobe of the brain by magnetic resonance imaging. Positron emission tomography was used to differentiate a tumor from an infarct in the brain. The cardiac and the brain lesions were successfully resected. Histopathologic study of the atrial and cerebral tissue demonstrated that these were metastases from a previously excised scalp desmoplastic malignant melanoma. The patient remains well at 14 months' follow-up.

Aged↗

GABA(B) receptor autoradiography in hippocampal sclerosis associated with human temporal lobe epilepsy.

1. Metabotropic gamma-aminobutyric acid receptors (GABA(B)) exist both pre- and postsynaptically throughout the brain, mediating the suppression of neurotransmitter release and late inhibitory postsynaptic potentials. Investigation of GABA(B) receptors in rodent models of temporal lobe epilepsy (TLE) suggests that expression or function of these receptors may be altered in the disorder. 2. The aim of the present study was to investigate the expression of GABA(B) receptors in samples of hippocampus surgically resected from patients with hippocampal sclerosis (HS) related intractable TLE, and compare this expression with samples of neurologically normal post-mortem (PM) control hippocampal tissue. Appropriate measures of neuronal loss associated with HS were investigated for comparison with receptor binding data. 3. Receptor autoradiography with [(3)H]-GABA in the presence of isoguvacine, and quantitative densitometric analysis were used to investigate GABA(B) receptor expression (B(max)) and affinity (K(D)) in 11 HS samples and eight controls. A three-dimensional cell counting technique was used to assess neuronal density in both groups. 4. GABA(B) receptor density was significantly reduced in CA1, CA2, CA3, hilus and dentate gyrus, and increased in the subiculum, of HS cases as compared with PM controls. Neuronal loss was significant in all regions measured. When adjusted for neuronal loss, CA1 GABA(B) receptor expression appeared significantly upregulated (P:<0.05). 5. In HS/TLE, GABA(B) receptor expression per remaining neurone appears increased in CA1. This finding, and increased [(3)H]-GABA affinity at CA3 and hilar GABA(B) receptors, suggests altered GABA(B) receptor function may occur in human HS/TLE, possibly as a result of synaptic reorganization.

Adult↗

Clinical characteristics and surgical outcome of patients with temporal lobe tumors and epilepsy.

This is a retrospective study of 21 surgically treated patients with temporal lobe tumors and epilepsy. Evaluation included clinical data, EEG findings, structural scans, pathological diagnosis and post-surgical follow-up. There were 9 cases of ganglioglioma, 5 pilocytic astrocytoma, 3 ganglioneuroma, 2 dysembryoplastic neuroepithelial tumor, 1 pleomorphic xantoastrocytoma, and 1 meningioangiomatosis. Mean follow-up time was 22 months and outcome was evaluated according to Engel's classification; 76.2% were classified in class I and 23.8% in II and III. All patients classes II and III had been submitted to mesial and neocortical resections. There were no differences related to clinical characteristics, pathological diagnosis or duration of follow-up in patients seizure-free or not. All patients had abnormal MRI and ten of these had normal CT; the MRI characteristics were compared to pathological diagnosis and specific histological characteristics of the tumors were not discernible by MRI. We concluded that MRI was essential for the diagnosis and precise location of TL tumors. Ganglioglioma was the most frequent tumor and lesionectomy associated to mesial resection doesn't guarantee a better prognosis.

Adolescent↗