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[Temporal mesial sclerosis syndrome in epilepsy].

Mesial temporal sclerosis is a frequent cause of epilepsy. Patients with mesial temporal sclerosis usually have an early brain insult, a febrile convulsion in most cases, and a seizure free interval of variable duration. This is followed by complex partial seizures with stereotypic semiology. Histological analysis of the hippocampal formation demonstrates a well defined pattern of cell loss and axonal proliferation. This pattern indicates selective cell vulnerability to the excitotoxic process that causes mesial temporal sclerosis, and synaptic and axonal reorganization, which are involved in the pathogenesis of this disorder. Epilepsy associated with mesial temporal sclerosis is not a static process, neurons in this region of the temporal lobe are physiologically and biochemically active, participating in the pathophysiology of the disease by facilitating the recurrence of seizures. There are no pathognomonic findings in mesial temporal sclerosis, but its confident diagnosis can be achieved by convergence of different lines of evidence, including clinical, morphological, and functional findings. Complex partial seizures are often resistant to antiepileptic medication, while surgical resection of the epileptic focus provides seizure freedom in a large number of patients.

Amygdala↗

Depth electroencephalography in selection of refractory epilepsy for surgery. Our experience with the suboccipital approach.

Despite new diagnostic tools, the precise localisation of an epileptic focal discharge remains an important step in the surgical treatment of epilepsy. Conventional EEG not always gives enough information to decide about surgery and more invasive methods have to be used. Epidural, subdural, and intra-parenchymatous electrodes have been used to come closer to the epileptic foci. Superficial hemispheric foci are well recorded by conventional epidural or subdural, strip or grid electrodes. Deeper foci, located in the medial temporal lobe or limbic areas are much more difficult to access from surface electrodes and other methods have to be used. Stereotactic placed multielectrodes and foramen ovale electrodes are most commonly implanted. Since 1986 we have used multi-contact cylindrical soft subdural electrodes. At the beginning we made the electrodes in our Department. Later on they were commercially available. In our Clinic the electrodes are usually introduced via a suboccipital approach and directed to the medial aspects of the temporal lobes until the temporal poles on both sides. Usually a median electrode located in the interhemispheric fissure, and covering gyrus cinguli is also placed. Out of 60 procedures done for different kinds of epilepsy, including cases with tumour or other surgical epileptogenic lesions, a total of 14 patients with genuine temporal lobe epilepsy have been studied and operated by this method. The advantages of the method as compared to stereotactic intraparenchymatous implanted electrodes are: less risk of bleeding and the fact that the brain tissue in those sensitive areas remains intact. Compared to foramen ovale electrodes our technique covers larger area of the temporal lobes and allows to insert a medial limbic electrode, but demands 2 burr holes. A further advantage of our technique is that the electrodes remain in place until surgery, allowing for their use as anatomical landmarks for tailoring the extension of the resection. This approach has been found to be simple, safe and reliable. A further improvement may be the simultaneous use of PEG epidural electrodes to obtain an overall view of the electrical activity of the brain including surface, deep temporal and midline cortical areas.

Brain↗

Benign focal epileptiform discharges of childhood and hippocampal sclerosis.

PURPOSE: Benign focal epileptiform discharges of childhood (BFEDCs) are common EEG findings between ages 4 and 14 years. This epoch of maturational development overlaps with the age at presentation of temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) in children. METHODS: From our series of 17 preadolescent children who eventually underwent anteromesial temporal resection for medically refractory TLE due to HS, we identified two children, plus one thereafter, who were initially dismissed as candidates for epilepsy surgery because of abundant extratemporal sharp waves, which were bilateral in two cases. The sharp waves had the distinctive morphology, distribution, and sleep activation suggestive of BFEDCs, but the medical intractability and seizure symptoms were unusual for benign focal epilepsy of childhood. RESULTS: In each case, surgical candidacy was clarified when magnetic resonance imaging (MRI) showed unilateral HS and video-EEG demonstrated seizure onset in the ipsilateral anteromesial temporal region. The postoperative freedom from seizures in each case (follow-up, 2 to 4 years) confirmed that HS was the primary epileptogenic process, and that the BFEDCs were incidental or an atypical secondary manifestation. CONCLUSIONS: These cases illustrate the need for more extensive study of children with BFEDCs when medical intractability and seizure symptoms speak against a simple diagnosis of benign focal epilepsy of childhood. In addition, we observed that the BFEDCs in two of our children had an unusual bilateral occipitofrontal distribution, and we speculate that the coexistence of the BFEDCs in children with HS may not be an incidental finding.

Child↗

Unidirectional whole body turning: a new lateralising sign in complex partial seizures.

BACKGROUND: The lateralising significance of unidirectional whole body turning in patients with complex partial seizures (CPS) arising from the temporal lobe was evaluated. METHODS: A total of 330 patients undergoing long term video-EEG study were included. "Unidirectional whole body turning" was defined as rotation of the trunk, head, and limbs by >90 degrees and lasting >10 s. EEG correlates, MRI, and SPECT findings were compared and outcome after surgery was noted for patients with follow up data for >1 year. RESULTS: Unidirectional whole body turning was observed in 13 patients with a mean age of 18+/-8 years. Concordance of the side of whole body turning with the EEG focus and MRI findings was observed in 11 of the 13 patients (84.7%) and in 26 of 28 seizures (92.8%). The six patients who underwent temporal lobectomy or resection of lesion, opposite to the direction of body turning, had good seizure outcome. CONCLUSION: Unidirectional whole body turning is a new lateralising sign in temporal lobe CPS with good predictive value for epileptogenic focus contralateral to the direction of turning.

Adolescent↗

Simple partial seizures (isolated auras) in medial temporal lobe epilepsy.

We analysed whether the medial temporal lobe epilepsy (MTLE) with isolated auras makes any difference in the clinical picture in comparison with MTLE in which only complex partial seizures (CPS) occur. We included 100 patients (aged 16-59 years) with unilateral medial temporal lobe lesions who consecutively underwent presurgical evaluation due to intractable MTLE and who became completely aura- and seizure-free after the anterior temporal resection. Preoperatively, isolated auras were present in 70 patients. These patients were categorised into the IA group. The remaining 30 patients in whom the auras preceded seizures were categorised into the NIA group. We found no difference between the two groups for the age at onset, epilepsy duration or aura types. Conversely, a right-sided epileptogenic region (61%) occurred more frequently in the IA group than in the NIA group (27%, P = 0.001). Conclusively, isolated auras show affinity to the right hemisphere. One explanation may be that seizures stop more quickly in the right hemisphere. Another hypothesis is that consciousness can be disturbed much easier by the ictal activity in left temporal seizures: auras evolve more frequently to CPS due to the disturbance of consciousness.

Adolescent↗

Posterior quadrantic dysplasia or hemi-hemimegalencephaly: a characteristic brain malformation.

INTRODUCTION: Posterior quadrantic dysplasia (PQD), a developmental malformation involving the temporal, parietal, and occipital lobes of one cerebral hemisphere, leads to intractable epilepsy. OBJECTIVE: To characterize the clinical features of 19 patients with PQD and analyze the postsurgical outcome of those who underwent resection of dysplastic tissue. METHODS: The extent and nature of the malformation were primarily assessed with high-resolution brain imaging. Fourteen patients underwent complete or partial temporoparieto-occipital resection or temporal resection associated with parieto-occipital disconnection. Postoperative follow-up period ranged from 8 months to 7 years. The authors used the Engel classification for postoperative outcome. RESULTS: All patients were sporadic. Clinical features included infantile spasms, partial seizures, mental retardation, mild hemiparesis, and visual field defects. Neuroimaging localized the malformation within the posterior cerebral quadrant contralateral to the neurologic deficit and demonstrated hemi-hemimegalencephaly in 14 of 19 patients and multilobar cortical dysplasia in 5 of 19 patients. The authors observed class I outcome in six patients. Two patients had class II and four patients had class III outcome. Class IV outcome was seen in two patients. After surgery, two patients developed mild hemiparesis, and two developed a visual field defect. CONCLUSIONS: Widespread cortical dysplasia is more frequent in the posterior quadrant. In our series, posterior quadrantic dysplasia represents either hemi-hemimegalencephaly or multilobar cortical dysplasia. Individuals with posterior quadrantic dysplasia share a spectrum of clinical features. The intractable epilepsy in these patients may be alleviated by a large quadrantic temporoparieto-occipital resection.

Adolescent↗

Intracerebral ganglioglioma: clinical and radiological study of eleven surgically treated cases with follow-up.

BACKGROUND: Gangliogliomas are rare benign tumors of the CNS consisting of differentiated neural elements and low-grade glial cells. METHODS: We reviewed our experience of 11 patients with histologically proven ganglioglioma who were surgically treated since 1986 at Cukurova University Medical Center. These patients presented at 18 to 45 years of age. Five were women and six were men. The most common initial symptom was seizures (in nine of 11 patients), which had sometimes persisted over long periods of time. At the time of diagnosis, four patients had focal neurological deficits and three had signs or symptoms of increased intracranial pressure. The cystic and well-circumscribed characteristics of these lesions were detected on computed tomography (CT). Despite their appearance on CT, all but one of the lesions were found to be mostly solid at operation. Magnetic resonance imaging (MRI) in six patients revealed abnormally high signal intensity on T2-imaging. The temporal lobe was the main tumor location (seven patients). All cases were diagnosed according to the Russel and Rubinstein histological criteria for ganglioglioma. RESULTS: Ten patients had radical total resection and one had subtotal resection. No patient underwent postoperative radiation or chemotherapy. Except for one, all are still alive and free of progressive disease 1 to 11 years (mean 6.2) after operation. Six are seizure-free and three have improved seizure control under anticonvulsant therapy. CONCLUSIONS: We conclude that ganglioglioma is a distinct histological phenomenon with mildly predictable clinical symptoms (seizures), mildly characteristic radiological features, and long-term survival after surgical resection without the need of adjuvant treatment such as radiotherapy.

Adolescent↗

Impaired recognition of scary music following unilateral temporal lobe excision.

Music constitutes an ideal means to create a sense of suspense in films. However, there has been minimal investigation into the underlying cerebral organization for perceiving danger created by music. In comparison, the amygdala's role in recognition of fear in non-musical contexts has been well established. The present study sought to fill this gap in exploring how patients with amygdala resection recognize emotional expression in music. To this aim, we tested 16 patients with left (LTR; n = 8) or right (RTR; n = 8) medial temporal resection (including amygdala) for the relief of medically intractable seizures and 16 matched controls in an emotion recognition task involving instrumental music. The musical selections were purposely created to induce fear, peacefulness, happiness and sadness. Participants were asked to rate to what extent each musical passage expressed these four emotions on 10-point scales. In order to check for the presence of a perceptual problem, the same musical selections were presented to the participants in an error detection task. None of the patients was found to perform below controls in the perceptual task. In contrast, both LTR and RTR patients were found to be impaired in the recognition of scary music. Recognition of happy and sad music was normal. These findings suggest that the anteromedial temporal lobe (including the amygdala) plays a role in the recognition of danger in a musical context.

Acoustic Stimulation↗

The neuropathology of temporal lobe epilepsy.

Complex partial epilepsy arising in the temporal lobe has been associated with several types of pathologic lesions including Ammon's horn sclerosis, malformations, neoplasms, and inflammatory scars from infarcts or infection. These lesions are usually situated at various sites in the medial temporal lobe, so that one of the enigmas of attempting to understand the pathogenesis of TLE pertains to the clinical manifestation of a single epileptic disorder which is associated with dissimilar lesions at dissimilar sites. Recent demonstrations of an alteration in temporal lobe anatomy, i.e. malformations of the normal circuitry of the temporal lobe and foci of microdysgenesis, have given rise to the hypothesis that insults which occur during a critical period of brain development could alter the connections within the hippocampus and predispose it to increased excitability and seizurogenesis. Such a hypothesis forces us to reconsider TLE in reference to risk factors which may act as "teratogens" and produce these malformations. These malformations may range from a subtle alteration in the neurotransmitters of the dentate gyrus to large areas of cortical dysplasia or the hamartomatous neoplasms seen in TLE. A reevaluation of the neuroanatomical disruptions created by the various lesions may allow us to define a minimal optimal surgical resection for each lesion; or, the definitions of neurotransmitter deficits may lead to alternative pharmacologic therapies. As neuropathologists we have the exciting opportunity to participate in the definition of the neuropathology of temporal lobe epilepsy.

Animals↗

Kainic acid induced hippocampal seizures in rats: comparisons of acute and chronic seizures using intrahippocampal versus systemic injections.

Hyppocampal epilepsy is a recently defined syndrome occurring in 65% of all temporal lobe epilepsies as defined by: 1) electrographic (EEG) onset in the hippocampus (HC) prior to EEG seizures elsewhere, 2) post-resection hippocampal sclerosis and mossy fiber synaptic reorganizations and 3) relief of typical complex partial seizures after surgical resection of the hyppocampus. We used intrahippocampal kainic acid injections V2 in rats at different developmental ages (postnatal 7 through adult) to develop long term spontaneous HC EEG spikes, EEG seizures, and behavioral seizures. Split-screen video/EEG monitoring demonstrated that this intrahippocampal kainic acid model produced progressive development of: 1) ipsilateral interictal spikes, 2) later polyspike complexes, 3) bilaterally-asynchronous EEG spiking, 4) unilateral HC EEG seizure onsets with occasional secondarily generalized spread to apposite HC and motor cortex to elicit complex partial seizures, and 5) in all seizing rats there was mossy fiber synaptic reorganization, even when injected at age 7 days. These results indicate that the intrahippocampal kainic acid injection model is similar to human hippocampal epilepsy.

Action Potentials↗

Coexistence of temporal lobe and idiopathic generalized epilepsies.

OBJECTIVE: To assess the interrelation of idiopathic generalized epilepsy (IGE) and temporal lobe epilepsy (TLE) when they coexist in the same patient. METHODS: The authors reviewed the electroclinical features of 350 consecutive patients who had temporal resection between 1975 and 1997 at the Maudsley and King's College Hospitals, London. RESULTS: Two patients had the unusual combination of TLE and IGE (0.57%). In the first, the clinical onset of juvenile myoclonic epilepsy followed the surgical resolution of his partial seizures but had been heralded for at least 5 years by subclinical spontaneous and photically induced generalized spike-wave discharges. In the second, TLE and juvenile absence epilepsy had a long parallel course before surgery. After surgery he had no further partial seizures. CONCLUSION: These cases suggest that when an idiopathic absence or myoclonic syndrome manifests in a patient with symptomatic TLE, the phenotype may not be a merged syndrome. Rather, the two conditions can retain their inherent electroclinical profile, responsiveness to treatment, and prognosis.

Adult↗

Neurosurgical management of epilepsy: a personal perspective in 1983.

The therapeutic goal in the neurosurgical treatment of medically intractable epilepsy is complete seizure control, for both biologic and psychosocial reasons. Cortical resections are more likely to accomplish this than other surgical alternatives for epilepsy. Although abnormalities on new imaging techniques (CT, positron emission scanning) aid in identifying the epileptic focus, interictal epileptiform EEG changes remain the main indicator of focal origin of the seizures. Where this is equivocal, direct brain recording of spontaneous seizures with subdural electrodes is of value in identifying the side and lobe of seizure onset. The cortical resection is then tailored by the extent of the interictal electrocorticographic abnormalities and functional identification of essential areas such as those for language, using an electrical stimulation mapping technique, under local anesthesia. With this approach, half of the patients with temporal lobe foci are seizure-free since the time of operation, over two-thirds become so with time, and over three-quarters have at least very major reductions in seizure frequency.

Brain Mapping↗

Reoperation for recurrent temporal lobe epilepsy.

The indications for the risks and outcome of reoperation for medically refractory temporal lobe epilepsy have not been well documented. A retrospective review is presented of 40 patients who underwent reoperation on the temporal lobe for recurrent seizures. The mean patient age at the first operation was 22 +/- 7 years (+/- standard deviation). Electrocorticography during the first operation showed interictal epileptic abnormalities from surface electrodes in 97% of the cases and from depth electrodes in the mesiotemporal structures in 38%. The seizures recurred with the same pattern within 6 months after the first operation in 60% of patients and within 2 years in 90%. Postoperative neuroimaging studies showed residual mesiotemporal structures in all cases. The mean time between the two operations was 5.5 +/- 5 years and the mean patient age at the second operation was 28 +/- 8 years. The second operation involved focal resection of the mesiotemporal structures in 30 cases. The mean postoperative follow-up period was 4.8 +/- 2.7 years (range 2 to 11 years). After the second operation, 63% of the patients were seizure-free or had rare seizures (one or two per year). There were no permanent neurological complications. Patients who did not benefit from reoperation had electroencephalographic abnormalities in multiple brain areas. Reoperation for temporal lobe epilepsy effectively controls seizures in the majority of patients, and the procedure is safe if rigorous technical rules are observed. More complete resection of mesiotemporal structures during the first operation, even in the absence of intraoperative electrographic abnormalities, could prevent the need for reoperation in defined cases.

Adolescent↗

Olfactory epileptic auras.

BACKGROUND: Olfactory epileptic auras are rare, constituting about 0.9% of all auras, and are typically described as unpleasant. They have usually been associated with tumors, but in some recent studies they have not. METHODS: We identified 13 patients (7 male, 6 female) with olfactory epileptic auras from 1423 patients with partial epilepsy evaluated for intractable seizures between 1991 and 1996. All had routine EEGs and MRI. Twelve underwent prolonged video-EEG monitoring. RESULTS: Olfactory sensations were of various types but unpleasant in only seven. Auras evolved to complex partial seizures (automotor, hypermotor, or dialeptic seizures) in 12 patients, with further evolution to generalized tonic-clonic seizures in three and aphasic seizures in one patient. The EEG focus was localized to the mesial temporal region in all. Ten patients had a mesial temporal tumor; in one patient, it extended to the superior temporal gyrus, and in another, the frontal lobe. The tumor involved only the amygdala in two patients and both amygdala and hippocampus in six; none had hippocampal involvement alone. Surgery was performed in nine patients. All except one with partial tumor resection had a seizure-free outcome. This patient also became seizure-free after repeat surgery to remove residual tumor tissue in the amygdala. CONCLUSIONS: Olfactory auras are not necessarily unpleasant. The amygdala is the most likely symptomatogenic zone of olfactory auras. Tumors are the commonest etiology; mesial temporal sclerosis is a relatively rare cause.

Adolescent↗

Multiple arteriovenous malformations with hemorrhage.

BACKGROUND: A rare case of a left temporal arteriovenous malformation (AVM) with bleeding 10 days after removal of another hemorrhagic AVM in the frontal lobe is reported. METHOD: A 47-year-old man had an attack of headache and vomiting and was admitted to our hospital. On admission, a CT scan showed subcortical hemorrhage in the left frontal lobe. Left carotid angiography revealed four AVMs and a vertebro-basilar angiogram demonstrated a vascular malformation. FINDINGS: First, a hemorrhagic AVM was removed with a hematoma. Following the operation, left carotid angiograms continued to show, AVMs, particularly a temporal AVM with increased blood flow, and after 10 days this hemorrhaged. This was surgically resected and the remaining small AVMs were treated by stereotactic radiosurgery. INTERPRETATION: The patient had a high risk of hemorrhage. Adding hemodynamic stress to this situation, hemorrhage would have been expected to occur at an early time after the initial intervention for hemorrhagic AVM. Considering the risk of hemorrhage, other AVMs should undergo surgery as soon as possible after resection of hemorrhagic AVM.

Cerebral Angiography↗

Unusual seizure types.

There is no consensus as to what constitutes a diagnostic complex partial seizure type for any given focus. Nevertheless, some forms are seen infrequently, such as in the patient just described who had parietal seizures, whereas others, such as the bland onset followed by oral-alimentary automatisms of temporal lobe epilepsy, are more common. It helps to be aware of the possible origins of these unusual forms when differentiating electrical seizures from pseudoseizures, planning for use of specific pharmacologic treatments, or planning a surgical resection. Although one cannot recommend a surgical resection based on semiology alone, one can place intracranial electrodes more strategically when the hypothesis regarding the seizure focus includes the clinical characteristics of the patient's seizures.

Epilepsy, Frontal Lobe↗

Extent of medial temporal resection on outcome from anterior temporal lobectomy: a randomized prospective study.

WE REPORT A prospective, randomized, blinded clinical trial comparing seizure and neuropsychological outcomes from anterior temporal lobectomies between two groups of patients. One group (n = 34) underwent hippocampal resection posteriorly to the anterior edge of the cerebral peduncle (partial hippocampectomy). In the other group (n = 36), the hippocampus was removed further to the level of the superior colliculus (total hippocampectomy). The amount of lateral cortical resection was the same between groups. Patients were and neuropsychological morbidity. At 1 year postoperatively, the total hippocampectomy group had a statistically superior seizure outcome compared with the partial hippocampectomy group (69 versus 38% seizure-free), and examination of time to first seizure (survival analysis) revealed significantly superior outcomes associated with total hippocampectomy. There was no increased neuropsychological morbidity associated with the more extensive hippocampal resection.

Adolescent↗