1/46. Idiopathic rolandic epilepsy with "interictal" facial myoclonia and oromotor deficit: a longitudinal EEG and PET study.PURPOSE: The prognosis of benign epilepsy with centrotemporal spikes (BECTS) is always favorable as far as the epilepsy is concerned. However, some data suggest that affected children may be at risk for minor cognitive impairment. We report here the longitudinal study of a young girl demonstrating that BECTS also may be associated with severe motor disturbances. methods: BECTS (rare left oromotor seizures, right rolandic spike-waves activated during sleep) started at the age of 3 years 6 months in a girl with normal initial psychomotor development. Her clinical, neuropsychological, and EEG status was assessed every 3-6 months. Regional cerebral glucose metabolism was measured by using the [18F]fluorodeoxyglucose-positron emission tomography (FDG-PET) method. RESULTS: Between the age of 5 and 6 years, the girl had (a) increased seizure frequency; (b) brief perioral and palpebral myoclonic jerks, concomitant with the spike component of interictal spike-waves, and (c) persistent but fluctuating oromotor deficits (drooling, dysarthria, dysphagia). The EEG showed a marked increase in abundance and amplitude of wake and sleep interictal abnormalities, which became bilateral. Awake FDG-PET revealed a bilateral increase of glucose metabolism in opercular regions. A complete and definitive EEG and clinical remission occurred at age 5 years 11 months and has persisted since (present age, 7 years 9 months). CONCLUSIONS: This case confirms that during BECTS, epileptiform dysfunctions within rolandic areas may induce "interictal" positive or negative oromotor symptoms, independent of classic seizures.- - - - - - - - - - ranking = 1keywords = epilepsy (Clic here for more details about this article) |
2/46. Paradoxic reaction to lamotrigine in a child with benign focal epilepsy of childhood with centrotemporal spikes.A girl aged 5 years developed nocturnal seizures (mouth twitching, salivation, anarthria, with right arm jerking and occasional secondary generalisation), with frequent focal sharp waves over the left centrotemporal region in her EEG, suggesting benign childhood epilepsy with centrotemporal spikes (BECTS). seizures became diurnal and frequent, not modified by carbamazepine (CBZ) or valproate (VPA) but responding to VPA and lamotrigine (LTG) with recommended dosage schedules for this combination. Her school performance then deteriorated insidiously, with poor memory and concentration, clumsiness, stuttering, and emotional lability. After 4 months, new episodes, < or =10 per day, occurred. These lasted a few seconds; she stared into space, her jaw dropped, her head dropped to the right, and her eyelids flickered. She usually maintained awareness. Attacks were often provoked by blowing or sneezing. Ictal EEG showed anterior-predominant 3/s sharp-slow wave complexes lasting < or =8 s, with bilateral rolandic discharges interictally. Withdrawal of LTG resulted in rapid improvement in cognitive function and gradual remission of the new attacks. CONCLUSIONS: This appears to be a paradoxic reaction to LTG in the setting of BECTS.- - - - - - - - - - ranking = 0.83359333915534keywords = epilepsy, childhood (Clic here for more details about this article) |
3/46. Systematic approach to dipole localization of interictal EEG spikes in children with extratemporal lobe epilepsies.OBJECTIVES: To assess the reliability of dipole localization based on residual variances (RV), using equivalent current dipole analysis of interictal EEG spikes in children with extratemporal lobe epilepsy. methods: Four pediatric patients with extratemporal lobe epilepsy were studied. Digital EEG was recorded from 19 scalp electrodes. Computer programs for spike detection and clustering analysis were used to select spikes. Dipoles were calculated 5 times for each spike using different initial guesses by the moving dipole model. Standard deviation (SD) of the dipole positions was calculated at each time point in the 5 trials. RESULTS: We analyzed the dipoles at 1097 time points from 4 patients. Among 106 time points with RV < 2%, the SD was < 1 mm in 78 (74%), while in those with SD > 1 mm the dipole positions varied between 2.8 and 52.6 mm. Of dipoles with RV < 1%, 26 of 27 (96%) had an SD < 1 mm; the one dipole with SD > 1 mm varied within 2.5 mm. The dipole localizations with RV < 2% corresponded to the epileptogenic zones identified on intracranial invasive video EEG and intraoperative ECoG. CONCLUSIONS: The systematic approach of equivalent current dipole analysis using spike detection, clustering analysis, and an RV < 2% as a standard is useful for identifying extratemporal epileptic regions.- - - - - - - - - - ranking = 0.33336168377317keywords = epilepsy, lobe (Clic here for more details about this article) |
4/46. Benign childhood epilepsy with centrotemporal spikes: is it always benign?Most children with benign childhood epilepsy with centrotemporal spikes have few seizures, and some have only one. We describe two children with interictal and ictal findings consistent with this epileptic syndrome but with severe intractable seizures and cognitive decline that resulted in consideration for epilepsy surgery. Spontaneous remission occured in one child; the other is still young. Despite the high seizure burden and cognitive decline, surgical consideration should be withheld, as these seizures are likely to remit.- - - - - - - - - - ranking = 1.000260005822keywords = epilepsy, childhood (Clic here for more details about this article) |
5/46. Idiopathic partial epilepsy: electroclinical demonstration of a prolonged seizure with sequential rolandic and occipital involvement. Seizure spread due to regional susceptibility?Benign rolandic epilepsy (BRE) and childhood epilepsy with occipital paroxysms (CEOP) are overlapping in age range of presentation. Some children have been reported to manifest occipital and rolandic seizures, as distinct events. However, the presence during the same seizure of rolandic and occipital symptoms is exceptional. We present a 7-year-old boy with BRE in whom we video-EEG recorded two seizures at 4 years of age. The first episode was a classic rolandic seizure during sleep, while the second was prolonged with initial rolandic and late occipital involvement. It is possible that a rolandic seizure, in a child within the age range of both BRE and early onset CEOP, could spread to selectively involve the occipital lobe, because there is susceptibility of cortical neurons of both areas to develop seizures at this age in idiopathic partial epilepsies.- - - - - - - - - - ranking = 1.0000567262377keywords = epilepsy, childhood, lobe (Clic here for more details about this article) |
6/46. epilepsy and perisylvian lipoma/cortical dysplasia complex.Intracranial lipomas are congenital malformations composed of mature adipocytes. They are usually located in the midline, particularly in the pericallosal region, a hemispheric location accounting for only 3 to 7% of cases. review of the literature found 21 previous cases of hemispheric lipoma. Although hemispheric cerebral lipomas are rare, association with epilepsy appears to be frequent. We have recently studied two patients in whom epilepsy was the first clinical manifestation of hemispheric cerebral lipoma in the sylvian region. The patients presented with simple motor partial seizures as the first manifestation of the lesion. Neurological examination was normal. MRI disclosed in both cases a lesion involving the sylvian fissure with characteristics of the lipid signal. MRI also demonstrated abnormalities involving the cerebral cortex in the vicinity of the lesion (pachygyria-like aspect). Partial excision of the lesion was achieved in one patient but was followed by a worsening of seizures and neurological condition (hemiparesis). According to the literature, the prognosis for epilepsy in patients with hemispheric lipoma appears good. Several other arguments support non-surgical management: the lesion is benign and can be identified with a high degree of certainty by imaging; surgery is technically difficult due to adherence to adjacent vascular and cerebral structures and hypervascularity; location near functional brain tissue increases the risk of postoperative sequelae. In addition, mechanisms of epilepsy probably involve vascular and cortical dysplasic abnormalities. In consideration of the complexity of the lesion, hemispheric lipomas are more appropriately classified with localized cortical malformations rather than as simple extracerebral malformations.- - - - - - - - - - ranking = 0.66666666666667keywords = epilepsy (Clic here for more details about this article) |
7/46. reading epilepsy in a patient with previous idiopathic focal epilepsy with centrotemporal spikes.RATIONALE: reading epilepsy (RE) is a form of reflex epilepsy currently classified as an idiopathic localization-related epilepsy (ILAE, 1989). methods: We describe a 30 year-old right-handed male who suffered since the age of 8 from nocturnal partial motor seizures. Clinical features were typical of BECT. We reviewed the EEG recorded at that time which showed centrotemporal spike and waves. He was seizure-free from the age of 12 to the age of 17 when seizures evoked only by reading appeared. No other stimuli provoked seizures. Neurological and neuroradiological (CT and MR) investigations were normal. Baseline video-polygraphic EEG recordings were normal while reading aloud provoked myoclonic jerks in the facial muscles related to bilateral spike and wave discharges. Therapy with carbamazepine and valproic acid strongly reduced seizure frequency. CONCLUSION: Recent papers have debated the difficulties in classifying RE among the generalized or focal syndromes. literature reports describe an association with RE and juvenile myoclonic epilepsy, supporting the hypothesis of an idiopathic generalized form. We report the first documented case with a clear-cut idiopathic localization-related epilepsy evolving to a primary reading epilepsy.- - - - - - - - - - ranking = 2.3333333333333keywords = epilepsy (Clic here for more details about this article) |
8/46. Can protrusion of the tongue stop seizures in Rolandic epilepsy?We report the case of a child with benign partial epilepsy with centro-temporal spikes (BECT) in whom protrusion of the tongue stopped the interictal abnormalities, and describe the polygraphic EEG recording of a seizure which terminated upon voluntary protrusion of the tongue. We mention the close link between the post-central (somatosensory) cortex and pre-central (motor) cortex, and how the primary sensory area has direct access to the motor cortex. We also examine how a tactile stimulus may provoke the inhibition of an epileptic discharge.- - - - - - - - - - ranking = 0.83333333333333keywords = epilepsy (Clic here for more details about this article) |
9/46. Benign childhood epileptic syndromes with occipital spikes: new classification proposed by the International League Against epilepsy.In a recent proposal, the Commission on classification and terminology of the International League Against epilepsy recognized early-onset childhood epilepsy with occipital spikes (Panayiotopoulos type), differentiating it from the only other type of childhood epilepsy with occipital spikes previously accepted: late-onset childhood epilepsy with occipital spikes (Gastaut type). The importance of this newly recognized syndrome of benign childhood partial seizures is that it is very common-only 2.4 times less frequent than benign rolandic epilepsy, and of equally excellent prognosis. It is characterized by a unique seizure type comprising a combination of autonomic and behavioral disturbances, vomiting, deviation of the eyes, and often with impairment of consciousness that can progress to convulsions. These commonly last for more than 3 minutes and in one quarter of cases for hours. One or more of these symptoms can predominate or be absent. Eyes can remain open without deviation, ictal vomiting might not occur, and autonomic and behavioral disturbances can predominate, particularly in the early stages of the ictus, and be missed in nocturnal seizures. Age at onset is 5 years, with a singular or a median of three seizures, which are predominantly nocturnal. Interictal electroencephalography (EEG) frequently shows occipital paroxysms or occipital spikes but one-fifth of the cases have only extraoccipital spikes on normal EEG. Treatment might not be needed. Panayiotopoulos syndrome, like rolandic epilepsy, needs recognition by the general pediatrician because of the invariably excellent prognosis and also because it can be misdiagnosed as an acute cerebral insult.- - - - - - - - - - ranking = 0.83374934264855keywords = epilepsy, childhood (Clic here for more details about this article) |
10/46. Familial paroxysmal exercise-induced dyskinesia and benign epilepsy: a clinical and neurophysiological study of an uncommon disorder.We report a family with 6 members affected by a long-lasting paroxysmal exertion-induced dyskinesia. fasting and stress were precipitating factors. All the patients of this family had also epileptic seizures mainly of generalised type with a favourable outcome. All patients were submitted to a neurophysiological study which included somatosensory evoked potentials by median nerve stimulation (MN-SEPs), somatosensory evoked potentials by posterior tibial nerve stimulation (PTN-SEPs), brainstem auditory evoked potentials (BAEPs), visual evoked potentials (VEPs), motor evoked potentials (MEPs) by magnetic transcranial cortical stimulation (TCS) and electromyography (EMG). The neurophysiological findings suggest a hyperexcitability at the muscular and brain membrane levels, probably due to an ion channel disorder.- - - - - - - - - - ranking = 0.66666666666667keywords = epilepsy (Clic here for more details about this article) |
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