Selective surgical mamillo-thalamic tract disconnection in hypothalamic hamartoma results in complete disappearance of gelastic seizures.

Hypothalamic hamartoma is a less common condition characterized by the several types of epileptic seizures including the gelastic type. It is reported that gelastic seizures are resistant to medical treatment with anticonvulsants, while stereotactic thermocoagulation or Gamma Knife radiosurgery are effective for seizure control. Here, we report an individual case where direct surgical resection disconnecting hypothalamic hamartoma from mammillothalamic tract resulted in complete disappearance of gelastic seizures without deterioration of cognitive function. A 6-year-old boy developed gelastic seizures at the age of 2 and suffered from precocious puberty. Anticonvulsants including carbamazepine and zonisamide failed to control seizures. The patient underwent direct division of the mammillothalmic tract by removal of hypothalamic hamartoma partially via anterior interhemispheric approach. It was observed that gelastic seizures disappeared completely after the surgical treatment without any endocrine and cognitive dysfunction for a follow-up period of 14 years. The mammillothalamic tract which connects anterior nucleus of thalamus and mammillary bodies plays a key role in gelastic seizures related to hypothalamic hamartoma. In this case, we disconnected the hamartoma specifically from the mammillary bodies and not from the rest of hypothalamus. Effectively, it enabled permanent control of seizures. This result shows that fibers connecting other hypothalamic structures and the dorsomedial nucleus of thalamus are not involved in gelastic seizure propagation from the hypothalamic hamartoma. When surgical treatment of hypothalamic hamartomas is performed it has high morbidity associated with hypothalamic disorders. Therefore, disconnection between hypothalamic hamartoma and mammillary bodies presents a possibility of reducing hypothalamic damage. Surgical disconnection between hamartoma and mammillothalamic tract carries minimal hypothalamic injury risk and our results suggest that it has the potential of seizure control for intractable gelastic seizures with less complications.

Spike-Associated Networks Predict Postsurgical Outcomes in Children With Refractory Epilepsy.

PURPOSE: Up to half of the children undergoing epilepsy surgery will continue to have seizures (szs) despite a cortical resection or ablation. Functional connectivity has shown promise in better identifying the epileptogenic zone. We hypothesized that cortical areas showing high information outflow during interictal epileptiform discharges are part of the epileptogenic zone. METHODS: We identified 22 children with focal epilepsy who had undergone stereo electroencephalography, surgical resection or ablation, and had ≥1 year of postsurgical follow-up. The mean phase slope index, a directed measure of functional connectivity, was calculated for each electrode contact during interictal epileptiform discharges. The positive predictive value and negative predictive value for a sz-free outcome were calculated based on whether high information outflow brain regions were resected. RESULTS: Resection of high outflow (z-score ≥ 1) and very high outflow (z-score ≥ 2) electrode contacts was associated with higher sz freedom (high outflow: χ 2 statistic = 59.1; P < 0.001; very high outflow: χ 2 statistic = 31.3; P < 0.001). The positive predictive value and negative predictive value for sz freedom based on resection at the electrode level increased at higher z-score thresholds with a peak positive predictive value of 0.86 and a peak negative predictive value of 0.9. CONCLUSIONS: Better identification of the epileptogenic zone has the potential to improve epilepsy surgery outcomes. If the surgical plan can be modified to include these very high outflow areas, more children might achieve sz freedom. Conversely, if deficits from resecting these areas are unacceptable, ineffective surgeries could be avoided and alternative therapies offered.

A Retrospective Study of Parietal Lobe Epilepsy: Functional Anatomy and Surgical Treatment.

Context: Parietal lobe epilepsy (PLE) accounts for approximately 5% of all focal epilepsies worldwide,1 and few PLE patients have undergone epilepsy surgery in the past. With the introduction of functional neuroimaging methods, such as interictal fluorodeoxyglucose-positron emission tomography (FDG-PET), stereotactic electroencephalograms (SEEGs), and high-resolution magnetic resonance imaging (MRI), more patients with intractable neocortical epilepsy have been considered for surgical treatment. Objective: The study intended to characterize the clinical features, aura, and presurgical evaluations of patients with PLE, by investigating their demographic and clinical characteristics, and to evaluate the prognostic value of the four diagnostic modalities-MRI, FDG-PET, scalp EEG, and SEEG-in terms of the localization of epileptogenic area. Design: The research team performed a retrospective analysis of outcomes for PLE patients who underwent resistive brain surgery. Setting: The study took place in the Neurosurgery Department of Epilepsy at the Second Hospital of Hebei Medical University in Shijiazhuang, China. Participants: Participants were 9 PLE patients, 4 males and 5 females, who underwent epilepsy surgery at the hospital between 2017 and 2019. Outcome Measures: The measures included demographic data, seizure data, electroencephalogram (EEG) recordings, magnetic resonance imaging (MRI) of the brain, positron emission tomography (PET), and stereotactic electroencephalogram (SEEG). The pathological findings were reviewed. Results: The five participants who had a PET all had positive results. Eight participants who had parietal lobe lesions had an MRI, and four had a stereotactic electroencephalogram (SEEG) that localized the epileptogenic zone. The interictal scalp EEG recordings for seven participants showed an abnormality, and six participants who had ictal surface EEG recordings showed parietal ictal EEG onset. Conclusions: Surgical excision of epileptogenic foci is the main treatment for drug-resistant PLE. Parietal functional anatomy is the basis for understanding and diagnosing PLE. Aura, semiology, interictal EEG, and PET are an important foundation for evaluation of PLE patients, and the SEEG is the most valuable tool, allowing localization of the epileptogenic zone.

Functional maps of direct electrical stimulation-induced speech arrest and anomia: a multicentre retrospective study.

Direct electrical stimulation, the transient 'lesional' method probing brain function, has been utilized in identifying the language cortex and preserving language function during epilepsy and neuro-oncological surgeries for about a century. However, comparison of functional maps of the language cortex across languages/continents based on cortical stimulation remains unclear. We conducted a retrospective multicentre study including four cohorts of direct electrical stimulation mapping from four centres across three continents, where three indigenous languages (English, French and Mandarin) are spoken. All subjects performed the two most common language tasks: number counting and picture naming during stimulation. All language sites were recorded and normalized to the same brain template. Next, Spearman's correlation analysis was performed to explore the consistency of the distributions of the language cortex across centres, a kernel density estimation to localize the peak coordinates, and a hierarchical cluster analysis was performed to detect the crucial epicenters. A total of 598 subjects with 917 speech arrest sites (complete interruption of ongoing counting) and 423 anomia sites (inability to name or misnaming) were included. Different centres presented highly consistent distribution patterns for speech arrest (Spearman's coefficient r ranged from 0.60 to 0.85, all pair-wise correlations P < 0.05), and similar patterns for anomia (Spearman's coefficient r ranged from 0.37 to 0.80). The combinational speech arrest map was divided into four clusters: cluster 1 mainly located in the ventral precentral gyrus and pars opercularis, which contained the peak of speech arrest in the ventral precentral gyrus; cluster 2 in the ventral and dorsal precentral gyrus; cluster 3 in the supplementary motor area; cluster 4 in the posterior superior temporal gyrus and supramarginal gyrus. The anomia map revealed two clusters: one was in the posterior part of the superior and middle temporal gyri, which peaked at the posterior superior temporal gyrus; and the other within the inferior frontal gyrus, peaked at the pars triangularis. This study constitutes the largest series to date of language maps generated from direct electrical stimulation mapping. The consistency of data provides evidence for common language networks across languages, in the context of both speech and naming circuit. Our results not only clinically offer an atlas for language mapping and protection, but also scientifically provide better insight into the functional organization of language networks.

Stereotactic MRI-guided laser interstitial thermal therapy for extratemporal lobe epilepsy.

OBJECTIVE: Magnetic resonance imaging (MRI)-guided laser interstitial thermal therapy (MRg-LITT) is an alternative to open epilepsy surgery. We assess safety and effectiveness of MRg-LITT for extratemporal lobe epilepsy (ETLE) in patients who are considered less favorable for open resection. METHODS: We retrospectively reviewed sequential cases of patients with focal ETLE who underwent MRg-LITT between 2012 and 2019. Epileptogenic zones were determined from standard clinical and imaging data ± stereoelectroencephalography (SEEG). Standard stereotactic techniques, MRI thermometry, and a commercial laser thermal therapy system were used for ablations. Anatomic MRI was used to calculate ablation volumes. Clinical outcomes were determined longitudinally. RESULTS: Thirty-five patients with mean epilepsy duration of 21.3 ± 12.2 years underwent MRg-LITT for focal ETLE at a mean age 36.4 ± 12.7 years. A mean 2.59 ± 1.45 trajectories per patient were used to obtain ablation volumes of 8.8 ± 7.5 cm3 . Mean follow-up was 27.3 ± 19.5 months. Of 32 patients with >12 months of follow-up, 17 (53%) achieved good outcomes (Engel class I + II) of whom 14 (44%) were Engel class I. Subgroup analysis revealed better outcomes for patients with lesional ETLE than for those who were nonlesional, multifocal, or who had failed prior interventions (P = .02). Of 13 patients showing favorable seizure-onset patterns (localized low voltage fast activity or rhythmic spiking on SEEG) prior to ablation, 9 (69%) achieved good outcomes, whereas only 3 of 11 (27%) who show other slower onset patterns achieved good outcomes. Minor adverse events included six patients with transient sensorimotor neurologic deficits and four patients with asymptomatic hemorrhages along the fiber tract. Major adverse events included one patient with a brain abscess that required stereotactic drainage and one patient with persistent hypothalamic obesity. Three deaths-two seizure-associated and one suicide-were unrelated to surgical procedures. SIGNIFICANCE: MRI-guided laser interstitial thermal therapy (or MRg-LITT) was well-tolerated and yielded good outcomes in a heterogeneous group of ETLE patients. Lesional epilepsy and favorable seizure-onset patterns on SEEG predicted higher likelihoods of success.

Laser interstitial thermotherapy (LiTT) in pediatric epilepsy surgery.

Laser interstitial thermotherapy (LiTT) is a novel stereotactic approach to the surgical treatment of severe drug-resistant focal epilepsies. This review extends our recent general review on this topic (Hoppe et al. Laser interstitial thermotherapy [LiTT] in epilepsy surgery. Seizure 2017; 48:45-52) with a focus on children (age <18 years). A PubMed search retrieved 25 uncontrolled case series reports that included a total of 179 pediatric patients as well as 7 review papers that specifically referred to using LiTT in pediatric epilepsy surgery (due August 31, 2018). Hypothalamic hamartomas (HH) represented the most frequent indication (64.2%) while therapeutic evidence for other more frequent etiologies underlying severe focal childhood epilepsies (e.g. focal cortical dysplasia, mesiotemporal sclerosis) is still scarce (n<20). For the published cases, the rate of severe complications was 3.4% and the overall complication rate was 23.5%. The seizure freedom rate (Engel class 1) was 57.5% (including patients with early follow-up and repeat thermoablations). None of the studies included the systematic evaluation of the cognitive outcome. Overall, the published evidence does not yet allow a scientific or clinical judgement on the utility of LiTT for pediatric epilepsy surgery. LiTT is likely to extend the neurosurgical toolbox with regard to deep brain lesions (e.g. HH). However, in cases that are equally accessible for both approaches therapeutic superiority of LiTT over open resective surgery still remains to be demonstrated. Recommendations for controlled though non-randomized outcome studies are provided.

The rough sound of salience enhances aversion through neural synchronisation.

Being able to produce sounds that capture attention and elicit rapid reactions is the prime goal of communication. One strategy, exploited by alarm signals, consists in emitting fast but perceptible amplitude modulations in the roughness range (30-150 Hz). Here, we investigate the perceptual and neural mechanisms underlying aversion to such temporally salient sounds. By measuring subjective aversion to repetitive acoustic transients, we identify a nonlinear pattern of aversion restricted to the roughness range. Using human intracranial recordings, we show that rough sounds do not merely affect local auditory processes but instead synchronise large-scale, supramodal, salience-related networks in a steady-state, sustained manner. Rough sounds synchronise activity throughout superior temporal regions, subcortical and cortical limbic areas, and the frontal cortex, a network classically involved in aversion processing. This pattern correlates with subjective aversion in all these regions, consistent with the hypothesis that roughness enhances auditory aversion through spreading of neural synchronisation.

Thalamocortical dysrhythmia in intraoperative recordings of focal epilepsy.

Resonant interactions between the thalamus and cortex subserve a critical role for maintenance of consciousness as well as cognitive functions. In states of abnormal thalamic inhibition, thalamocortical dysrhythmia (TCD) has been described. The characteristics of TCD include a slowing of resting oscillations, ectopic high-frequency activity, and increased cross-frequency coupling. Here, we demonstrate the presence of TCD in four patients who underwent resective epilepsy surgery with chronically implanted electrodes under anesthesia, continuously recording activity from brain regions at the periphery of the epileptogenic zone before and after resection. Following resection, we report an acceleration of the large-scale network resting frequency coincident with decreases in cross-frequency phase-amplitude coupling. Interregional functional connectivity in the surrounding cortex was also increased following resection of the epileptogenic focus. These findings provide evidence for the presence of TCD in focal epilepsy and highlight the importance of reciprocal thalamocortical oscillatory interactions in defining novel biomarkers for resective surgeries. NEW & NOTEWORTHY Thalamocortical dysrhythmia (TCD) occurs in the context of thalamic dysfacilitation and is characterized by slowing of resting oscillations, ectopic high-frequency activity, and cross-frequency coupling. We provide evidence for TCD in focal epilepsy by studying electrophysiological changes occurring at the periphery of the resection margin. We report acceleration of resting activity coincident with decreased cross-frequency coupling and increased functional connectivity. The study of TCD in epilepsy has implications as a biomarker and therapeutic target.

Clinical features and evolution of the gelastic seizures-hypothalamic hamartoma syndrome.

Gelastic seizures, usually with onset in early infancy, are the hallmark manifestation of hypothalamic hamartoma. This seizure type is directly generated by hamartoma itself, intrinsically epileptogenic because of its anatomofunctional organization. Other types of seizures, focal or generalized, may appear during the evolution, probably resulting from mechanisms of secondary epileptogenesis. Nevertheless, the clinical expression and the severity of the syndrome, ranging from a focal drug-resistant epilepsy to a catastrophic generalized encephalopathy with severe cognitive and behavioral impairments, depends on the size and the site of attachment of the hamartoma. Early suspicion, timely diagnosis, and appropriate treatment are mandatory to reverse a potential catastrophic evolution of this condition.

Hypothalamic hamartoma: Neuropathology and epileptogenesis.

Hypothalamic hamartomas (HHs) are congenital malformations of the ventral hypothalamus resulting in treatment-resistant epilepsy and are intrinsically epileptogenic for the gelastic seizures that are the hallmark symptom of this disorder. This paper reviews the neuropathologic features of HHs associated with epilepsy, with an emphasis on characterizing neuron phenotypes and an ultimate goal of understanding the cellular model of ictogenesis occurring locally within this tissue. We also present previously unpublished findings on Golgi staining of HH. The microarchitecture of HH is relatively simple, with nodular clusters of neurons that vary in size and abundance with poorly defined boundaries. Approximately 80-90% of HH neurons have an interneuron-like phenotype with small, round soma and short, unbranched processes that lack spines. These neurons express glutamic acid decarboxylase and likely utilize γ-aminobutyric acid (GABA) as their primary neurotransmitter. They have intrinsic membrane properties that lead to spontaneous pacemaker-like firing activity. The remaining HH neurons are large cells with pleomorphic, often pyramidal, soma and dendrites that are more likely to be branched and have spines. These neurons appear to be excitatory, projection-type neurons, and have the functionally immature behavior of depolarizing and firing in response to GABA ligands. We hypothesize that the irregular neuronal clusters are the functional unit for ictogenesis. Further research to define and characterize these local networks is required to fully understand the cellular mechanisms responsible for gelastic seizures.

Clinical features and postoperative seizure outcome in patients with drug-resistant gelastic seizures without hypothalamic hamartoma.

OBJECTIVES: The objective of this study was to describe the clinical characteristics and surgical outcome in patients with gelastic seizures without hypothalamic hamartoma. METHODS: We retrospectively reviewed all the video-EEG reports over a 5-year period (2007-2011) for the occurrence of the terms "laugh" or "giggle" in the text body. All the patients with at least one documented gelastic seizure at the epilepsy monitoring unit were studied. In patients who underwent epilepsy surgery, seizure outcomes were analyzed. RESULTS: Sixteen patients (10 females and 6 males) with a mean age of 46.3years were studied. Seven patients had invasive intracranial EEG recordings. Seizure onset zone was in a temporal lobe in four patients and the frontal lobe in one patient. Two patients did not have gelastic seizures during their intracranial EEG monitoring. Nine patients underwent resective epilepsy surgery for their seizures. Six patients (67%) were seizure-free after surgery. CONCLUSION: In adult patients, gelastic seizures can be seen in patients with focal epilepsy without hypothalamic hamartoma. Nonhypothalamic hamartoma gelastic seizures originating from the temporal lobe can be amenable to surgery.

Hypothalamic hamartoma: is the epileptogenic zone always hypothalamic? Arguments for independent (third stage) secondary epileptogenesis.

Gelastic seizures associated with hypothalamic hamartomas (HHs) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography (EEG) abnormalities, precocious puberty, behavioral disturbances, and progressive cognitive deterioration. Surgery in adults provides seizure freedom in only one third of patients. The poor results of epilepsy surgery could be explained by an extrahypothalamic epileptogenic zone. The existence of an independent, secondary epileptogenic area with persistent seizures after resection of the presumably primary lesion supports the concept of a "hypothalamic plus" epilepsy. "Hypothalamic plus" epilepsy could be related to either an extrahypothalamic structural lesion (visible on magnetic resonance imaging [MRI] or on neuropathology) or if the former is absent, to a functional alteration with enhanced epileptogenic properties due to a process termed secondary epileptogenesis. We report two patients with gelastic seizures with HH (gelastic seizures isolated or associated with dyscognitive seizures of temporal origin). Both patients underwent two-step surgery: first an endoscopic resection of the HH, followed at a later time by temporal lobectomy. Both patients became seizure-free only after the temporal lobectomy. In both cases, neuropathology failed to demonstrate a significant structural lesion in the temporal lobe. To our knowledge, for the first time, these two cases suggest the existence of independent secondary epileptogenesis in humans.

Mapping repetition suppression of the P50 evoked response to the human cerebral cortex.

OBJECTIVE: The cerebral network subserving repetition suppression (RS) of the P50 auditory evoked response as observed using paired-identical-stimulus (S1-S2) paradigms is not well-described. METHODS: We analyzed S1-S2 data from electrodes placed on the cortices of 64 epilepsy patients. We identified regions with maximal amplitude responses to S1 (i.e., stimulus registration), regions with maximal suppression of responses to S2 relative to S1 (i.e., RS), and regions with no or minimal RS 30-80 ms post stimulation. RESULTS: Several temporal, parietal and cingulate area regions were shown to have significant initial registration activity (i.e., strong P50 response to S1). Moreover, prefrontal, cingulate, and parietal lobe regions not previously proposed to be part of the P50 habituation neural circuitry were found to exhibit significant RS. CONCLUSIONS: The data suggest that the neural network underlying the initial phases of the RS process may include regions not previously thought to be involved like the parietal and cingulate cortexes. In addition, a significant role for the frontal lobe in mediating this function is supported. SIGNIFICANCE: A number of regions of interest are identified through invasive recording that will allow further probing of the RS function using less invasive technology.

Surgical outcome of gelastic epilepsy of frontal lobe origin: a case report.

Gelastic seizure is an uncommon type of seizure which is characterized by recurrent bouts of unprovoked and stereotyped laughter. It is commonly observed in patients with hypothalamic hamartoma, while its association with other cerebral lesions is rare. The patient was a 15-year-old right-handed male. His chief complaints were recurrent onsets of laughter and unconsciousness for 10 years. On average, he had several to dozens of onsets per day and had failed most antiepileptic medications before admission. Presurgical evaluation included MRI, video-EEG, MEG and subdural electrode EEG. The results of MEG and subdural electrode EEG suggested that the epileptogenic focus was located on the lateral surface of the right frontal lobe, chiefly on the anterior part of the inferior frontal gyrus. The focus was removed under intraoperative ECoG monitoring which was consistent with the results of MEG and subdural electrode EEG. Histopathology revealed focal cortical dysplasia with balloon cells (type II). The patient kept seizure-free during the short-term follow up of 3 months. In the past literature, the medial frontal and basal temporal lobes, besides the hypothalamus, were thought to play major roles in the case of gelastic seizure. Our results suggest that the lateral surface of the frontal lobe might also be one part of the epileptogenic network in gelastic seizures. Removal of the epileptogenic focus under thorough pre-surgical evaluation might result in good seizure control in patients with gelastic seizures.

Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: an ictal SPECT study.

PURPOSE: To topographically localize the ictogenic zone within hypothalamic hamartomas (HHs) and the symptomatogenic zone for gelastic seizure (GS), we analyzed data from both interictal and ictal single photon emission computed tomography (SPECT). METHODS: Ictal SPECT was performed immediately after GS onset in 21 patients with HH (aged 2-36 years, mean 13.8 years) who underwent stereotactic radiofrequency thermocoagulation (SRT). SPECT data were statistically analyzed by means of subtraction ictal SPECT coregistered to magnetic resonance imaging (MRI) (SISCOM) and statistical parametric mapping (SPM). Topographic localization of ictal hyperperfusion areas was evaluated. RESULTS: SISCOM obtained in 27 studies demonstrated ictal hyperperfusion in the HH interface zone in 16 studies of 13 patients (hot HH group). In these patients, HHs were significantly larger than those without hyperperfusion of HH in 11 studies of 8 patients (21.4 ± 10.3 vs. 12.3 ± 7.3 mm in diameter, p < 0.05, t-test). In all patients and in the hot HH group, SPM group analysis of individual differences between interictal and ictal data revealed significantly (p < 0.001) hyperperfused areas in the ipsilateral hypothalamus, mediodorsal (MD) nucleus of the thalamus and putamen, bilateral pontine tegmentum, and contralateral inferior semilunar lobule of the cerebellum. There was no hyperperfusion in the mammillothalamocingulate pathway. DISCUSSION: The present study confirmed that ictogenesis occurs in the HH interface zone, which should accordingly be the target for SRT. We suggest that a thalamopontocerebellar circuit plays an important role for stereotypical and automatic symptomatogenesis of GS and that the hypothalamus and MD nucleus of the thalamus are potentially involved in epileptic encephalopathy.

Functional hemispherectomy in children with epilepsy and CSWS due to unilateral early brain injury including thalamus: sudden recovery of CSWS.

PURPOSE: To try to prove in patients with refractory symptomatic epilepsy due to early brain injury involving thalamus and complicated by CSWS the effects of the isolation of the injured hemisphere, performed with functional hemisperectomy, on epilepsy, namely on CSWS. METHODS: Full clinical follow-up before and after surgery of two cases with CSWS onset at four years in whom functional hemispherecomy was performed with resection of inter-hemispheric connections. RESULTS: An immediate effectiveness of the surgical treatment was observed on both epileptic evolution (no more seizures) and EEG abnormalities. In particular, CSWS completely disappeared, together with a concurrent progressive improving of the cognitive and behavioural disorders. DISCUSSION: The isolation of the injured hemisphere through the section of inter-hemispheric cortico-cortical connections could prevent the contralateral diffusion of discharges coming from the injured cortex and cortico-thalamic network, favouring a normal function of thalamo-cortico-thalamic circuitries in the healthy hemisphere. That could explain the disappearance of CSWS after surgery in our patients and the consequent improvement of cognitive abilities and behaviour.

Mirthful gelastic seizures with ictal involvement of temporobasal regions.

Ictal laughter is the cardinal clinical sign of gelastic seizures in hypothalamic hamartomas and may also occur in extrahypothalamic epilepsies. Laughing consists of an affective and a motor component. It has been suggested that the affective component may result from an involvement of temporobasal structures, whereas the motor part is related to an involvement of the mesial frontal cortex. So far, evidence is based on a limited number of cases with spontaneously recorded seizures or in whom electrical stimulation of invasive intracranial EEG recordings has been performed. We report a patient who suffered from epigastric psychic auras, complex partial seizures with a gelastic component and secondarily generalized seizures. To evaluate a possible epileptogenic role of the hippocampus and dysplastic region in the right mid-temporal gyrus, intracranial monitoring with subdural electrodes over the temporobasal and temporolateral regions, as well as a deep brain electrode in the hippocampus, were performed. During the intial part of the seizure, consisting of an intense retrosternal ascending feeling with sexual connotation, rhythmic spikes in temporolateral contacts were detected. Concomitant with the development of smiling and laughter, a rhythmic activity over the temporobasal regions evolved. The patient became seizure-free following right temporal lobe resection. This case supports the assumption that ictal involvement of temporobasal structures is crucial for gelastic seizure components in patients with temporal lobe epilepsy. Progression to temporobasal regions was associated with the feeling of happiness whereas motor components of laughter occurred later. These findings are in accordance with the interpretation of surface recordings by Dericioglu and co-workers in a similar previous case. [Published with video sequences].

Generators of tibial nerve somatosensory evoked potential: recorded from the mesial surface of the human brain using subdural electrodes.

The generators of the initial cortical component of somatosensory evoked potentials in response to tibial nerve stimulation (Tib-somatosensory evoked potentials) are still uncertain. The purpose of this study is to localize the generators of it. A 15-year-old boy with intractable parietal lobe epilepsy was studied. Subdural electrodes were chronically implanted for presurgical evaluation of epilepsy surgery, covering the primary motor, primary sensory, and supplementary sensorimotor areas of the right leg. Tib-somatosensory evoked potentials were recorded from these areas. Highly localized prominent positive activities were recorded from electrodes on the primary motor area of the leg at 32.4 to 34.0 milliseconds. No corresponding large negative peak was recorded in any other electrodes. Weak negative activities distributed widely around the postcentral area at 33.2 to 33.6 milliseconds, accompanied by similar but positive activities in the precentral area at 32.8 to 33.2 milliseconds. There was an independent positive field on supplementary sensorimotor areas at 34.0 to 34.8 milliseconds. A small negative peak was also recorded but only from a single electrode within supplementary sensorimotor areas at 34.0 milliseconds. Our data suggest that the initial response of Tib-somatosensory evoked potentials has at least three independent generators: a radial dipole on the primary motor, a tangential dipole on the primary sensory area, and a dipole on the supplementary sensorimotor areas oriented perpendicularly to the mesial hemispheric surface.

Symptomatic laughter in a patient with orbitofrontal seizure: A surgical case with intracranial electroencephalographic study: case report.

OBJECTIVE: A rare case of orbitofrontal lobe epilepsy manifesting gelastic seizure is reported. CLINICAL PRESENTATION: A 49-year-old woman had developed weekly complex partial seizures consisting of nonverbal vocalization and unresponsiveness followed by laughter. Magnetic resonance imaging revealed a round tumorous lesion at the posterior side of the right rectal gyrus and medial orbitofrontal gyrus. Neuroimaging studies and electrophysiological examinations, including intracranial electroencephalographic monitoring, suggested the existence of an epileptogenic zone in the ipsilateral orbitofrontal gyrus, including the lesion. INTERVENTION: After partial right prefrontal lobectomy including lesionectomy, the patient became seizure-free during a follow-up period of 33 months. We speculated that the limbic system, including the orbitofrontal lobe and temporal structures, which have a strong connection with the pontine nuclei, might be involved in this patient's gelastic seizure. CONCLUSION: Except for impaired consciousness, the clinical manifestations did not correspond to the characteristics of orbitofrontal seizure described by the International League Against Epilepsy. Symptomatic laughter in epilepsy that originates from the orbitofrontal lobe is very rare. Intracranial electroencephalographic findings and ictal symptomatology associated with epileptogenesis in this rare case are discussed.

Influence of subtraction ictal SPECT on surgical management in focal epilepsy of indeterminate localization: a prospective study.

The impact of functional imaging tests on the decision-making and planning process for epilepsy surgery has never been prospectively assessed. We prospectively evaluated 50 consecutively eligible patients whose noninvasive evaluations showed nonlocalized findings and determined how their SISCOM (subtraction ictal SPECT [single photon emission computed tomography] co-registered to MRI [magnetic resonance imaging]) data altered consensus decisions for epilepsy surgery. At an epilepsy surgery conference where each patient was discussed, consensus decisions were documented after a standardized presentation of data from the noninvasive evaluation (SISCOM findings initially were excluded). Consensus decisions were again documented after presentation of SISCOM data. Consensus decisions changed for 10 of 32 patients (31%) with localizing SISCOM results, whereas the decision changed in only 1 of 18 patients (6%) with nonlocalizing SISCOM results (P<.05). Changes in consensus decisions were as follows: (1) intracranial electrode implantation (IEI) was obviated and resective surgery was recommended (n=2); (2) resective surgery or further evaluation for patients initially not considered surgical candidates (n=2); (3) IEI in patients for whom it was not recommended initially (n=3); (4) increased IEI coverage (n=3); and (5) antiepileptic drug trial or vagal nerve stimulation was recommended instead of IEI (n=1). For some patients whose noninvasive evaluations did not clearly localize a surgical focus, SISCOM data can have a major impact on decisions to recommend resective epilepsy surgery or IEI.