Fronto-temporal Epilepsy Research Articles

Bilateral thalamic responsive neurostimulation for multifocal, bilateral frontotemporal epilepsy: illustrative case.

BACKGROUNDPatients with refractory, bilateral, multifocal epilepsy have few treatment options that typically include a combination of antiseizure medications (ASMs) and vagus nerve stimulation (VNS). A man in his 40s presented with epilepsy refractory to a combination of five ASMs plus VNS; he was still experiencing 7–10 seizures per week. His seizure network involved multiple foci in both frontal and temporal lobes. Bilateral depth electrodes were implanted into the centromedian/parafascicular (CM/PF) complex of the thalamus and connected to the responsive neurostimulation (RNS) system for closed-loop stimulation and neurophysiological monitoring.OBSERVATIONSThe patient reported clear improvement in his seizures since the procedure, with a markedly reduced number of seizures and decreased seizure intensity. He also reported stretches of seizure freedom not typical of his preoperative baseline, and his remaining seizures were milder, more often with preserved awareness. Generalized seizures with loss of consciousness have decreased to about one per month. RNS data confirmed a right-sided predominance of the bilateral seizure onsets.LESSONSIn this patient with multifocal, bilateral frontotemporal epilepsy, RNS of the CM/PF thalamic complex combined with VNS was found to be beneficial. The RNS device was able to detect seizures propagating through the thalamus, and stimulation produced a decrease in seizure burden and intensity.

Network reorganization during verbal fluency task in fronto-temporal epilepsy: A functional near-infrared spectroscopy study

This is the first study to use functional near-infrared spectroscopy (fNIRS) to investigate how the lateralization of the epileptogenic zone affects the reconfiguration of task-related network patterns. Eleven left fronto-temporal epilepsy (L-FTE) and 11 right fronto-temporal epilepsy (R-FTE), as well as 22 age- and gender-matched controls, were enrolled. Signals from 52-channel fNIRS were recorded while the subject was undertaking verbal fluency tasks (VFTs), which included categorical (CFT) and letter (LFT) fluency tasks. Three analytic methods were used to study the network topology: network-based analysis, hub identification, and proportional threshold to select the top 20% strongest connections for both graph theory parameters and clinical correlation. Performance of CFT is accomplished primarily using the ventral pathway, and bilateral ventral pathways are augmented in fronto-temporal epilepsy patients by strengthening the inter-hemispheric connections, especially for R-FTE. LFT mainly employed the dorsal pathway, and further prioritized the left dorsal pathway in strengthening intra-hemispheric connections in fronto-temporal epilepsy, especially L-FTE. The top 20% of the strongest connections only present differences in CFT network compared with the controls. R-FTE increased inter-hemispheric network density, while L-FTE decreased inter-hemispheric average characteristic path length. Accumulative seizure burden only affects L-FTE network. Better LFT performance and longer educational years seem to promote left fronto-temporal networks, and decreased the demand from RR intra-hemispheric connectivity in L-FTE. LFT scores in R-FTE are maintained by preserved RR intra-hemispheric networks. However, CFT scores and educational years seem to have no effect on the CFT network topology in both FTE.

Case Report: Aperiodic Fluctuations of Neural Activity in the Ictal MEG of a Child With Drug-Resistant Fronto-Temporal Epilepsy.

Successful surgical treatment of patients with focal drug-resistant epilepsy remains challenging, especially in cases for which it is difficult to define the area of cortex from which seizures originate, the seizure onset zone (SOZ). Various diagnostic methods are needed to select surgical candidates and determine the extent of resection. Interictal magnetoencephalography (MEG) with source imaging has proven to be useful for presurgical evaluation, but the use of ictal MEG data remains limited. The purpose of the present study was to determine whether pre-ictal variations of spectral properties of neural activity from ictal MEG recordings are predictive of SOZ location.We performed a 4 h overnight MEG recording in an 8-year-old child with drug-resistant focal epilepsy of suspected right fronto-temporal origin and captured one ~45-s seizure. The patient underwent a right temporal resection from the anterior temporal neocortex and amygdala to the mid-posterior temporal neocortex, sparing the hippocampus proper. She remains seizure-free 21 months postoperatively. The histopathological assessment confirmed frank focal cortical dysplasia (FCD) type IIa in the MEG-defined SOZ, which was based on source imaging of averaged ictal spikes at seizure onset. We investigated temporal changes (inter-ictal, pre-ictal, and ictal periods) together with spatial differences (SOZ vs. control regions) in spectral parameters of background brain activity, namely the aperiodic broadband offset and slope, and assessed how they confounded the interpretation of apparent variations of signal power in typical electrophysiological bands. Our data show that the SOZ was associated with a higher aperiodic offset and exponent during the seizure compared to control regions. Both parameters increased in all regions from 2 min before the seizure onwards. Regions anatomically closer to the SOZ also expressed higher values compared to contralateral regions, potentially indicating ictal spread. We also show that narrow-band power changes were caused by these fluctuations in the aperiodic component of ongoing brain activity. Our results indicate that the broadband aperiodic component of ongoing brain activity cannot be reduced to background noise of no physiological interest, and rather may be indicative of the neuropathophysiology of the SOZ. We believe these findings will inspire future studies of ictal MEG cases and confirm their significance.

P 125 Ring chromosome 20 Epilepsy syndrome in children Variable EEG- and electro-clinical pattern

Background Children with ring chromosome 20 epilepsy syndrome (rchr20es) typically show severe, drug-resistant focal epilepsies characterized by frontal semiology, high susceptibility for non-convulsive status epilepticus (NCSE) and epilepsy-associated cognitive and behavioral deteriorations. EEG has been reported to show frequent or continuous subclinical seizure patterns together with characteristic rhythmic theta waves (Caneveni et al. 1998). Here, we report on clinical and EEG data in four patients. Patients and methods Retrospective analysis of clinical and video-EEG-long-term-monitoring data of four patients (all female) with rchr20es. Age at onset of epilepsy ranged from 5 to 8 years, the number of antiepileptic drugs already used ranged from 6 to 10, and all four patients had suffered NCSE previously. Semiology: all four patients showed auras (epigastric: 2, fear: 2, visual: 1, somatosensory: 1) and seizures (hypermotor: 3, tonic: 3, dialeptic: 2, psychomotor: 2). Clinical epilepsy syndromes were frontal lobe epilepsies (n = 3) and frontal/ temporal lobe epilepsy (n = 1). EEG showed regional abnormalities in frontal (n = 4), temporal (n = 3) and parieto-occiptal (n = 1) areas, with subclinical seizure patterns and/ or EEG-status elements (with serial spikes, polyspikes, sharp waves, or spike wave complexes; n = 3), rhythmic theta (n = 2), 1–2 Hz slow sharp wave complexes (n = 1), spike wave complexes/low amplitude fast activity +high amplitude delta burst (n = 1), alpha/theta seizure activity (n = 1). MRI was essentially normal in all patients, with minor abnormalities in frontal (2) or temporal areas (2). Conclusion Consistent with the literature, our patients showed severe drug-resistant epilepsies with an electro-clinical complex of a fronto-temporal epilepsy with frequent, long-lasting or continuous subclinical seizure patterns and/or NCSE. Rhythmic theta as a “characteristic” EEG pattern was, however, only observed in 2/4 of our patients. Therefore, a rchr20es should be suspected in such patients also in the absence of this EEG feature. This information will help in an early identification of rchr20es, which is especially important since, without this specific diagnosis, many affected patients are considered candidates for pre-surgical diagnostics or even epilepsy surgery.

Intracranial stimulation for children with epilepsy.

To evaluate the efficacy of intracranial stimulation to treat refractory epilepsy in children. This is a retrospective analysis of a pilot study on all 8 children who had intracranial electrical stimulation for the investigation and treatment of refractory epilepsy at King's College Hospital between 2014 and 2015. Five children (one with temporal lobe epilepsy and four with frontal lobe epilepsy) had subacute cortical stimulation (SCS) for a period of 20-161h during intracranial video-telemetry. Efficacy of stimulation was evaluated by counting interictal discharges and seizures. Two children had thalamic deep brain stimulation (DBS) of the centromedian nucleus (one with idiopathic generalized epilepsy, one with presumed symptomatic generalized epilepsy), and one child on the anterior nucleus (right fronto-temporal epilepsy). The incidence of interictal discharges was evaluated visually and quantified automatically. Among the three children with DBS, two had >60% improvement in seizure frequency and severity and one had no improvement. Among the five children with SCS, four showed improvement in seizure frequency (>50%) and one chid did not show improvement. Procedures were well tolerated by children. Cortical and thalamic stimulation appear to be effective and well tolerated in children with refractory epilepsy. SCS can be used to identify the focus and predict the effects of resective surgery or chronic cortical stimulation. Further larger studies are necessary.

Crying with sorrow evoked by electrocortical stimulation

Dacrystic seizures are rare and have been reported in patients with hypothalamic hamartoma as well as fronto-temporal epilepsy, involving the non-dominant hemisphere. We describe the first reported case of cortical stimulation of the left posterior orbito-frontal gyrus, generating consistent and reproducible crying with affective content in a 41-year-old woman with medically intractable left temporal lobe epilepsy, who underwent extraoperative intracranial video-EEG monitoring for resective non-lesional epilepsy surgery.

P02-282 Gilles de la Tourette syndrome and epilepsy: A case report

Introduction:The association between tics, epilepsy and obsessive-compulsive symptoms, is still little explored in literature.Case report:The authors describe case of a male patient, 25 years old, with some obsessive personality traits and with the appearance of simple multiple tics, at 7-8 years old. The tics were vocal and motor, particularly facial and worsen with anxiety. Of the personal history is highlighted the existence of repetitive tonsilitis and in the family history the presence of tics in 3 paternal first cousins. This patient started only consultation of Neurology in 2003 and was medicated with haloperidol 1 mg / day. Already in 2007 it was triggered absence crisis, so that after additional study (EEG and MRI) and detection of focal paroxysmal activity in the left fronto-temporal region, the patient was medicated with levetiracetam 1000 mg / day. Currently holds the following therapy: levetiracetam 1500 mg / day, clonidine 0225 mg / day and buspirone 30 mg / day, with clinical improvement occurred.Conclusions:This patient on one hand, presents DSM-IV-TR (2001) diagnostic criteria for Gilles de la Tourette syndrome and on the other hand, criteria for the diagnosis of fronto-temporal epilepsy. It is also relevant the patient"s family history, that is consistent with the high risk of Tourette Syndrome in the first degree relatives.

Troubles bipolaires de l'humeur et épilepsie : une discussion à partir de deux cas cliniques

The comorbidity between bipolar disorders and epilepsy knows a growing interest not only because of the probable neurochemical underpinnings of those two disorders but also because of the better research of the bipolar spectrum symptoms among the epileptic patients. We report two interesting cases. The first one is a 17 year old teenager suffering from a bipolar disorder I and of a symptomatic (cerebral palsy) right fronto-temporal epilepsy. The manic episode subsided thanks to carbamazepine treatment. The second case is a 19-year-old man suffering from a rapid cycling bipolar disorder II and from a symptomatic (cerebral palsy) generalized epilepsy. The depressive episode subsided thanks to parotexine treatment. The mood and epileptic stabilization has been obtained thanks to valproate of sodium. These two case reports are discussed from a diagnostic and therapeutic point of view and they are followed by a review of the physiopathological hypotheses. Considering the high prevalence of the bipolar spectrum, treating the bipolar symptoms is a major issue for the epileptic patients quality of life.

Ictal urinary urge: further evidence for lateralization to the nondominant hemisphere.

To determine the lateralizing value of ictal urinary urgency. A retrospective database search was performed for patients with ictal urinary urgency admitted to the Epilepsy Monitoring Unit at the Cleveland Clinic between 1994 and 2001. Six patients were identified; intracarotid amytal test demonstrated left hemispheric speech dominance in five cases. The sixth patient continued to speak during right temporal seizures. EEG and imaging data supported right temporal or frontotemporal epilepsy in all six cases. Two patients were seizure free after focal right hemispheric resection. Ictal urinary urge appears to be a lateralizing sign for nondominant temporal lobe epilepsy.

Improved sensitivity of 18FDG-positron emission tomography scans in frontal and "frontal plus" epilepsy.

We evaluated three techniques of analyzing 18FDG-positron emission tomography-(PET) scans in 23 cases of presumed frontal lobe epilepsy (FLE): routine visual or "qualitative linear," "qualitative normalized," and quantitative normalized approaches. Patients were then classified as having pure frontal, probable frontal, frontoparietal and frontotemporal epilepsy based on prolonged surface EEG monitoring with video, magnetic resonance imaging (MRI), chronic intracranial recording (CIR), and results of surgical excision. Overall sensitivity and accuracy of the scans was 52 and 48%for qualitative linear analysis, which was equivalent to that of MRI, and 69 and 43% for qualitative normalized analysis. Quantitative normalized analysis had 96% sensitivity and 74 - 78% accuracy and also detected 9 of 11 (81%) abnormalities in nonlesional cases, improving routine sensitivity from 1 of 11 (9%). We conclude that qualitative linear (routine) analysis is inadequate for diagnosis of FLE lobe or "frontal-plus" epilepsies and does not add to the MRI scan. Because qualitative normalized images improve on routine analysis only slightly, quantitative techniques should be applied for preoperaive evaluations.

Intracerebral low frequency electrical stimulation: a new tool for the definition of the "epileptogenic area"?

Low Frequency (1 Hz) Electrical Stimulation (LFES) has been systematically utilized, during stereo-EEG investigations, in 24 consecutive young adult patients considered for surgical treatment of severe drug-resistant partial epilepsy. Ninety seizures (1-14/patient) identical to the spontaneous ones previously recorded were thus obtained in 19 patients (79%). LFES is less effective for induction of seizures than high frequency (50 Hz) stimulation (5.9% vs 22.9%), and it also provokes less "false positive" responses (1% vs 17%). The main "sensitive" structures to LFES are the hippocampus, the amygdala, and the hippocampal gyrus. However, seizures were also induced by stimulating the temporal lobe white matter, the temporal pole, and the temporal neocortex, as well as the orbito-frontal cortex (in the only patient with fronto-temporal epilepsy). The more frequently observed electrical pattern is a gradual increase of spikes and spikes and waves frequency, with or without occurrence of low voltage fast activity. The high percentage of early "subjective" manifestations similar to the spontaneous ones, the lack of major electrical artifact, and the good visualization of the spatial evolution of the induced-discharge, strongly suggest that LFES is of great help for defining the "epileptogenic area".

Aging and the Extrapyramidal System

To evaluate the efficacy of intracranial stimulation to treat refractory epilepsy in children.This is a retrospective analysis of a pilot study on all 8 children who had intracranial electrical stimulation for the investigation and treatment of refractory epilepsy at King's College Hospital between 2014 and 2015. Five children (one with temporal lobe epilepsy and four with frontal lobe epilepsy) had subacute cortical stimulation (SCS) for a period of 20–161 h during intracranial video-telemetry. Efficacy of stimulation was evaluated by counting interictal discharges and seizures. Two children had thalamic deep brain stimulation (DBS) of the centromedian nucleus (one with idiopathic generalized epilepsy, one with presumed symptomatic generalized epilepsy), and one child on the anterior nucleus (right fronto-temporal epilepsy). The incidence of interictal discharges was evaluated visually and quantified automatically.Among the three children with DBS, two had >60% improvement in seizure frequency and severity and one had no improvement. Among the five children with SCS, four showed improvement in seizure frequency (>50%) and one chid did not show improvement. Procedures were well tolerated by children.Cortical and thalamic stimulation appear to be effective and well tolerated in children with refractory epilepsy. SCS can be used to identify the focus and predict the effects of resective surgery or chronic cortical stimulation. Further larger studies are necessary.

OSPOLOT: a preliminary communication.

This clinical trial appears to have shown that (a) OSPOLOT is an efficient anti-convulsant, (b) it does have a specific effect on the emotional personality and behaviour disorders associated with epilepsy and it appears to be the first anti-epileptic drug to have these properties. It would appear to be especially suitable for cases of fronto-temporal epilepsy, although it does not affect all those cases. Clinically, it would appear to be specific for what is called psychomotor epilepsy, or what used to be designated as epilepsy with hysteria. Previously, these cases were not only not improved by anti-convulsant drugs, but very often as the fits were brought under control the behaviour and emotional disorder became worse. One frequently had the experience of having a patient refuse medication because he himself was aware of this. Another point that seems to come out of this trial is that many elements which hitherto have been called hysterical may, in fact, be epileptic phenomena. For example, exhibitionism, attacks of suicidal depression, paranoid or psychopathic attitudes, and anxiety, which at present are sometimes classed as hysterical manifestations in an epileptic personality, might be proved to be direct epileptic manifestations.