6E11, a highly selective inhibitor of Receptor-Interacting Protein Kinase 1, protects cells against cold hypoxia-reoxygenation injury.

Necroptosis is a programmed cell death pathway that has been shown to be of central pathophysiological relevance in multiple disorders (hepatitis, brain and cardiac ischemia, pancreatitis, viral infection and inflammatory diseases). Necroptosis is driven by two serine threonine kinases, RIPK1 (Receptor Interacting Protein Kinase 1) and RIPK3, and a pseudo-kinase MLKL (Mixed Lineage Kinase domain-Like) associated in a multi-protein complex called necrosome. In order to find new inhibitors for use in human therapy, a chemical library containing highly diverse chemical structures was screened using a cell-based assay. The compound 6E11, a natural product derivative, was characterized as a positive hit. Interestingly, this flavanone compound: inhibits necroptosis induced by death receptors ligands TNF-α (Tumor Necrosis Factor) or TRAIL (TNF-Related Apoptosis-Inducing Ligand); is an extremely selective inhibitor, among kinases, of human RIPK1 enzymatic activity with a nM Kd; has a non-ATP competitive mode of action and a novel putative binding site; is weakly cytotoxic towards human primary blood leukocytes or retinal pigment epithelial cells at effective concentrations; protects human aortic endothelial cells (HAEC) from cold hypoxia/reoxygenation injury more effectively than necrostatin-1 (Nec-1) and Nec-1s. Altogether, these data demonstrate that 6E11 is a novel potent small molecular inhibitor of RIPK1-driven necroptosis.

Innovative interactive flexible docking method for multi-scale reconstruction elucidates dystrophin molecular assembly.

At present, our molecular knowledge of dystrophin, the protein encoded by the DMD gene and mutated in myopathy patients, remains limited. To get around the absence of its atomic structure, we have developed an innovative interactive docking method based on the BioSpring software in combination with Small-angle X-ray Scattering (SAXS) data. BioSpring allows interactive handling of biological macromolecules thanks to an augmented Elastic Network Model (aENM) that combines the spring network with non-bonded terms between atoms or pseudo-atoms. This approach can be used for building molecular assemblies even on a desktop or a laptop computer thanks to code optimizations including parallel computing and GPU programming. By combining atomistic and coarse-grained models, the approach significantly simplifies the set-up of multi-scale scenarios. BioSpring is remarkably efficient for the preparation of numeric simulations or for the design of biomolecular models integrating qualitative experimental data restraints. The combination of this program and SAXS allowed us to propose the first high-resolution models of the filamentous central domain of dystrophin, covering repeats 11 to 17. Low-resolution interactive docking experiments driven by a potential grid enabled us to propose how dystrophin may associate with F-actin and nNOS. This information provides an insight into medically relevant discoveries to come.

Outcome of surgery in children with focal cortical dysplasia younger than 5 years explored by stereo-electroencephalography.

PURPOSE: Focal cortical dysplasia (FCD) is the most frequent etiology for drug-resistant epilepsy in young children. Complete removal of the lesion is mandatory to cure the epilepsy. Stereo-EEG (SEEG) is an excellent method to delimitate the zone to be resected in older children and adults. We studied its feasibility in younger children. METHODS: We retrospectively studied 19 children under 5 years of age who underwent SEEG between January 2009 and December 2012 and were subsequently operated on. FCD was diagnosed in all. We reviewed magnetic resonance imaging (MRI), electrophysiological and clinical data, as well as postoperative seizure outcome. We also included fluoro-deoxyglucose positron emission tomography (FDG-PET) studies, which had been systematically performed before invasive recording in 16 of the 19 children. RESULTS: The mean patient's age at the time of SEEG was 38.6 months, and the mean age at seizure onset was 8 months. Three patients had normal MRI. No SEEG-associated complications occurred. We were able to delineate the epileptogenic zone in all children, and electrode stimulation localized the motor area when necessary (12 patients). Hypometabolic areas on FDG-PET included the epileptogenic zone in 13 of the 16 children, with a lobar concordance in 9 (56 %) and the same anatomical extent in 6 (38 %). Twelve children subsequently underwent focal or sublobar resection, six had multilobar resection, and one had hemispherotomy. The etiology was FCD type 2 in 15 and FCD type 1 or type 3 in three children. Eighty-four percent of our population have remained seizure-free at a mean follow-up of 29 months (12-48 months). CONCLUSION: Although children with FCD can successfully undergo resective surgery without invasive EEG, poor seizure semiology at this age inclines to perform SEEG when the dysplastic lesion is ill-defined and/or the electroclinical correlation is unclear. In cases with normal imaging as well as with suspected huge malformations, as was the case in 52 % of our patients, we consider it to be indispensable.

Selective suppression of excessive GluN2C expression rescues early epilepsy in a tuberous sclerosis murine model.

Tuberous sclerosis complex (TSC), caused by dominant mutations in either TSC1 or TSC2 tumour suppressor genes is characterized by the presence of brain malformations, the cortical tubers that are thought to contribute to the generation of pharmacoresistant epilepsy. Here we report that tuberless heterozygote Tsc1(+/-) mice show functional upregulation of cortical GluN2C-containing N-methyl-D-aspartate receptors (NMDARs) in an mTOR-dependent manner and exhibit recurrent, unprovoked seizures during early postnatal life (

Insular and insulo-opercular epilepsy in childhood: an SEEG study.

PURPOSE: In recent years, there have been series analysing the electro-clinical correlations of insular epilepsy in adult populations. In contrast, the ictal semiology in children with insular epilepsy is poorly described. Considering that early and successful surgery may greatly improve the cognitive outcome and quality of life, it is worthwhile to deepen our knowledge of insular epilepsy in children. METHODS: We retrospectively evaluated ten children with drug-resistant focal insular epilepsy who had been consecutively explored with stereoelectroencephalography (SEEG), followed by individually tailored resective surgery that included part of the insula in all cases. A detailed anatomo-electro-clinical analysis of non-invasive EEG and SEEG data was performed. At least one of the electrodes explored the insular cortex. SEEG analysis confirmed that the insular cortex was included in the ictal onset zone. RESULTS: Epilepsy onset was mostly during the first year of life, characterized by subtle seizures as well as spasms and myoclonic seizures. Later on, neurovegetative signs and asymmetric tonic and hypermotor seizures (HMS) dominated the ictal semiology. The epileptogenic zone was frequently wider than insular with frontal and central predominance. In eight patients, the tailored resection included a lesion. In seven patients, an Engel class 1 outcome as well as neuropsychological and behavioural improvement was obtained. CONCLUSIONS: SEEG is feasible and useful in children with drug-resistant insular epilepsy which is often characterized by autonomic symptoms as the initial symptoms and should be suspected in cases with HMS, asymmetric tonic seizures and even asymmetric spasms. Early propagation is mostly frontal and central. Analysis of a larger population is required to refine these findings.

Spatiotemporal source analysis in scalp EEG vs. intracerebral EEG and SPECT: a case study in a 2-year-old child.

OBJECTIVE: This case study aims to demonstrate that spatiotemporal spike discrimination and source analysis are effective to monitor the development of sources of epileptic activity in time and space. Therefore, they can provide clinically useful information allowing a better understanding of the pathophysiology of individual seizures with time- and space-resolved characteristics of successive epileptic states, including interictal, preictal, postictal, and ictal states. METHODS: High spatial resolution scalp EEGs (HR-EEG) were acquired from a 2-year-old girl with refractory central epilepsy and single-focus seizures as confirmed by intracerebral EEG recordings and ictal single-photon emission computed tomography (SPECT). Evaluation of HR-EEG consists of the following three global steps: (1) creation of the initial head model, (2) automatic spike and seizure detection, and finally (3) source localization. During the source localization phase, epileptic states are determined to allow state-based spike detection and localization of underlying sources for each spike. In a final cluster analysis, localization results are integrated to determine the possible sources of epileptic activity. The results were compared with the cerebral locations identified by intracerebral EEG recordings and SPECT. RESULTS: The results obtained with this approach were concordant with those of MRI, SPECT and distribution of intracerebral potentials. Dipole cluster centres found for spikes in interictal, preictal, ictal and postictal states were situated an average of 6.3mm from the intracerebral contacts with the highest voltage. Both amplitude and shape of spikes change between states. Dispersion of the dipoles was higher in the preictal state than in the postictal state. Two clusters of spikes were identified. The centres of these clusters changed position periodically during the various epileptic states. CONCLUSION: High-resolution surface EEG evaluated by an advanced algorithmic approach can be used to investigate the spatiotemporal characteristics of sources located in the epileptic focus. The results were validated by standard methods, ensuring good spatial resolution by MRI and SPECT and optimal temporal resolution by intracerebral EEG. Surface EEG can be used to identify different spike clusters and sources of the successive epileptic states. The method that was used in this study will provide physicians with a better understanding of the pathophysiological characteristics of epileptic activities. In particular, this method may be useful for more effective positioning of implantable intracerebral electrodes.

Heterotopia associated with hippocampal sclerosis: an under-recognized cause of early onset epilepsy in children operated on for temporal lobe epilepsy.

PURPOSE: The aim of this study is to report on 52 children operated on for pharmacoresistant temporal lobe epilepsy, with special emphasis on histopathology and correlation with clinical features. METHODS: Charts were retrospectively analyzed. All children underwent comprehensive clinical, electrophysiological and radiological investigations before surgery. Surgical procedures were tailored according to scalp, foramen ovale and eventually depth electrode recordings. Histopathology was compared with clinical variables (χ (2) and Fisher's exact tests). Outcome was evaluated using the Engel scale. RESULTS: Developmental tumor was found in 14 cases, malformation of cortical development (MCD) in 26, isolated hippocampal sclerosis (HS) in 5 and gliosis in 7. Dual pathology (DP) affected 18 patients and the main extrahippocampal lesion consisted of microscopic sub-cortical heterotopias (HS-HT) for 15 patients who shared a particular clinical pattern: a history of febrile seizures (FS) and/or brain injury, early onset of epilepsy without latent period from FS to the first temporal seizure, and a particularly good outcome following surgery. CONCLUSION: In our pediatric temporal lobe surgery series, the prevalence for MCD and for DP was higher than in adult series. Age at seizure onset depends on pathology, and is earlier when involving the neocortex rather than only the hippocampus. We identify the association HS-HT (the most frequent DP in this series), with particular clinical features.

[Disability and epilepsy]. / Handicap et épilepsie.

The purpose of this article is to familiarize radiologists with the different aspects of severe drug resistant epilepsy. These result in three levels of disability: the disease itself (seizures and their impact, underlying cause), social impact (restrictions, safety and precautions, education, activities of daily life) and issues related to the medical treatment (long term medication intake, side effects, complications). First, clinical and EEG diagnosis will be reviewed to move on to MRI with attention to technical and protocol considerations followed by the imaging features of specific entities causing severe drug resistant epilepsy: migration and gyration disorders, Rasmussen's encephalitis, Sturge Weber disease and post-ischemic sequelae. Finally, current data and the imaging features of hemispherotomy, a radical treatment for epilepsy, will be presented.

[CT and MR brain imaging following hemispherotomy]. / Imagerie scanner et IRM de l'encéphale après hémisphérotomie.

PURPOSE: The aim of the study was to define the usual and pathological modifications arising in the brain following hemispherotomy for intractable epilepsy in children. METHODS: Preoperative MRI and postoperative imaging scans (CT in the first week, MRI at 3 months and 1 year after surgery) were reviewed in a series of 52 patients, average age 8 years and 7 months, with intractable epilepsy due to dysplasia, Rasmussen's encephalitis, ischemic lesions and/or Sturge-Weber disease. The posterior fossa, brain parenchyma, ventricles and subdural space were also analyzed. RESULTS: Hemispheric scarring was a typical finding on CT and MRI as a consequence of the surgical procedure. Also frequently seen were small subdural effusions, bleeding along the surgical scar on early CT, and chronic subdural effusions with no mass effect on mid-term and late MRI scans. Other features - such as large subdural effusions that required external shunts and hydrocephalus - were rare, but severe, and considered to be postoperative complications. In contrast to the complications associated with other surgical techniques such as hemispherectomy, infection, extensive edema or hemosiderosis were never found in our series. CONCLUSION: Hemispherotomy is a surgical technique performed to treat intractable epilepsy. Our findings will help to identify the typical morphology of postsurgical scars, and to differentiate the usual features and complications seen in the postoperative period on CT and MRI brain scans.

[Surgical disconnection of hypothalamic hamartomas]. / Déconnexion chirurgicale des hamartomes hypothalamiques.

BACKGROUND AND PURPOSE: Surgical resection of hypothalamic hamartomas (HHs) associated with drug-resistant gelastic epilepsy carries a considerable risk of neurological and endocrine morbidity. Alternative surgical routes and techniques have therefore been proposed, especially for broadly attached lesions and for those with a third ventricular location. METHODS: We present an updated series of 43 patients (aged nine months to 34 years), operated on from 1998 through 2005 at our institution. The hamartoma was disconnected using a microsurgical pterional approach of those lesions extending from the hypothalamic floor downward into the interpeduncular cistern. When the HH presented as a paramedian mass, partly or exclusively bulging into the third ventricle, with a rather vertical plane of attachment, we chose a frameless stereotactic endoscopic technique to disconnect the lesion. In several of our patients, both methods were applied subsequently. RESULTS: Surgery-related morbidity was lower with the ventricular endoscopic technique. Twenty-one patients (50%) are seizure-free and two patients (5%) almost seizure-free, while in 17 patients (40%), there was a significant seizure reduction. Two patients (5%) had no postoperative improvement. According to the different topographic features of the HHs, for which we have recently proposed a classification into four subtypes, the intraventricularly located hamartoma had the best prognosis following endoscopic disconnection. Ten of the 12 patients (83%) with this HH location became seizure-free. CONCLUSIONS: Resection of epilepsy-related HHs can be replaced by disconnective procedures. Our results confirm their feasibility and acceptable morbidity, with particularly good seizure outcome in patients with intraventricularly located HHs.

[Electrophysiological investigations in childhood epilepsy surgery]. / Explorations électroencéphalographiques préchirurgicales de l'enfant.

Epilepsy surgery in children is a functional surgery: its goal is to perform the resection of the epileptic brain tissue while sparing the eloquent cortex. Prolonged scalp video-EEGs allow recording of all types of seizures and play a crucial role in localizing the epileptogenic zone. Furthermore, EEG data correlation with clinical and radiological findings provides a guide for the surgical strategy: either resection without further investigations or an invasive recording procedure. In prehemispherotomy evaluation, EEG recordings confirm that limited resections are not indicated and demonstrate that the opposite hemisphere is not involved. If invasive recordings are needed, they consist in foramen ovale electrode insertion, which provides valuable information in mesial temporal lobe epilepsy, stereoelectroencephalography for children older than two years, and subdural grids associated with depth electrodes in infants or when the eloquent areas need to be carefully investigated. Such investigations allow tailoring surgery to each child.

[Long-term outcome after hemispheric disconnection]. / Evaluation à long terme des déconnexions hémisphériques.

Hemispheric disconnection has been largely proposed for patients with severe epilepsy associated with a congenital or acquired hemispheric cerebral pathology. The classical procedure of anatomical hemispherectomy was progressively abandoned by neurosurgeons in order to avoid postoperative complications since then hemispherotomy techniques have been developed. Globally, with hemispheric disconnection, the rate of patients becoming seizure-free has been between 50 and 80%. The factors affecting seizure control have not been completely elucidated, but several authors suggested that differences in etiology as well as the hemispheric disconnection technique used may partially explain this variability. The percentage of seizure-free patients is higher with hemispherotomy techniques and in the group of patients with Rasmussen encephalitis, Sturge-Weber syndrome, and vascular insults. Depending on overall long-term progression, there is an improvement compared to preoperative status even if children exhibit heterogenous abilities. The lowest scores are observed for motor skills but communication and socialization are relatively well-preserved and strongly related to the duration of epilepsy: the longer the duration, the lower the scores were. Neuropsychological outcome following hemispheric disconnection makes it possible to study the development of hemispheric specialization during infancy and to provide information on cognitive recovery. Cerebral reorganization has been proved to exist in motor and language recovery. Ipsilateral corticospinal pathways seem to be involved in the movement of hemiplegic limbs. Everyday language can be supported by both hemispheres, but there is an early hemispheric specialization of the left hemisphere according to metaphonologic abilities.

[Epilepsy surgery during infancy and early childhood in France]. / Chirurgie de l'épilepsie de l'enfant et du nourrisson en France.

BACKGROUND AND PURPOSE: We present the epilepsy surgery activity in infants and children at the Fondation Rothschild Hospital, the main center dedicated to this activity in France. METHOD: A prospective study was conducted from 2003 to 2007 based on three populations: (1) children selected as candidates for surgery, (2) children undergoing presurgical evaluation and (3) children undergoing surgical procedures for epilepsy. RESULTS: Children selected as candidates for surgery: 304 children were referred and discussed by our multidisciplinary staff. They came from Paris and its suburbs (40%), the provinces (43%) or from other countries (14%). Sixty-one percent of them were included in our surgery program and 24% were excluded. Sixty-one percent of them were under 10 years of age. Children undergoing presurgical evaluation: 296 children were recorded: 140 EEG (47%), 46 with foramen ovale electrodes (16%) and 110 with invasive recording techniques (37%). Seventy percent of these children were under 10 years of age. Children undergoing surgical procedures: 316 children underwent surgery; 68% of them were under 10 years of age. The surgical procedures were focal resection (136 children), vertical parasagittal hemispherotomy (77 children), resection and or disconnection for hypothalamic hamartoma (69 children) and 34 had palliative surgery (callosotomy or vagal nerve stimulation). CONCLUSION: Eighty to 100 children undergo surgery each year in our department for drug-resistant partial epilepsy; 70% of them are less than 10 years of age. This activity is part of a network of pediatric neurologists who are deeply involved in treatment of severe epilepsy in children.

[Parasagittal vertical hemispherotomy: surgical procedure]. / Hémisphérotomie verticale parasagittale: technique opératoire.

After the occurrence of long-term complications of hemispherectomy, various methods have been used to reduce them. The aim was to reduce the large dead space left inside the skull. We have developed an original hemispheric disconnection technique that can achieve the same results as anatomic hemispherectomy in epilepsy, with excellent short- and long term-reliability. The detailed technique is described.

Long term response to steroid therapy in Rasmussen encephalitis.

Rasmussen encephalitis (RE) is a severe and progressive focal epilepsy of unknown origin that leads to deterioration of motor and cognitive function. In a previous study, we described positive effect of high doses of steroids during the first year after the onset of RE. The objective of this study was to evaluate this therapy at long term. We reviewed 11 patients (7 girls and 4 boys) with RE of the right hemisphere (7) and the left (4) at a follow-up of 9+/-2 years. Age at onset of RE ranged from 2 to 14 years. Six patients had no benefit from steroid therapy and underwent hemispherotomy. Five had significant reduction of seizure frequency with disappearance of epilepsia partialis continua, and improved motor function. Of these, two died of unexpected sudden death 5 and 7 years after seizure control. Two others with initial response experienced progressive recurrence of seizures 1 to 4 years after the end of steroid therapy and required hemispherotomy. Finally, only one patient exhibited total cessation of seizures with steroids for 3 years, but seizures progressively recurred although the frequency was moderate. Our data confirm that although steroid treatment can be useful when given early in the course of RE, long term relapse can occur among the good responders requiring delayed hemispheric disconnection.

[Recent advances in pathogenic concepts and therapeutic strategies in Rasmussen's encephalitis]. / Avancées actuelles sur les concepts pathogéniques et thérapeutiques de l'encéphalite de Rasmussen.

Rasmussen's encephalitis (RE) is a rare inflammatory brain disease mainly affecting children and characterised by intractable epilepsy involving a single hemisphere that undergoes progressive atrophy. RE is characterized by refractory focal seizures, often associated with epilepsia partialis continua, progressive unilateral motor defect, slow EEG activity over the entire contralateral hemisphere, with focal white matter hyperintensity and insular cortical atrophy on neuroimaging. Surgical exclusion of the affected hemisphere is the only treatment that interrupts progression of the disease. Pathogenic concepts have considered viruses, autoimmune antibodies and autoimmune cytotoxic T lymphocytes that might contribute to the initiating or perpetuating events in the central nervous system. Based on these concepts, different therapeutic strategies have been pursued, such as antiviral agents, plasmapheresis, immuno-adsorption, immunosuppression or immunomodulation with intravenous immunoglobulins. However, due to the lack of large studies, to date there is no established therapeutic strategy for this devastating condition. In this review, we give an overview of the current state of immunopathogenic concepts for Rasmussen's encephalitis and discuss the different therapeutic options for future perspectives.

[Surgery for intractable focal epilepsy in children]. / Chirurgie de l'épilepsie chez l'enfant.

Surgery of drug resistant epilepsy in children is a functional surgery which aims at the suppression of the epileptogenic focus. The surgical decision is adopted or rejected as a result of the clinical examination and complementary investigations following a multidisciplinary discussion in which the benefit-risk ratio is being carefully weighed. The objective of the presurgical investigations, dominated by scalp-recorded video-electroencephalography and magnetic resonance imaging, is the localization of the epileptogenic focus. The invasive surgical investigations with intracranial electrode placement are indicated when the limits of a planned resection have to be defined precisely or when the focus is located nearby a functionally eloquent region (sensori-motor and language region). Their identification is possible by means of electrical stimulation. Two methods of exploration are available: The combination of subdural and intracerebral electrode placement through craniotomy and the stereotactic placement of intracerebral depth electrodes (Stereo-EEG). The choice of either of these two methods depends on the child's age and on the topography of the epileptogenic focus. Surgery can be either palliative, with the purpose of reducing the intensity and/or the frequency of a certain seizure type (callosotomy, multiple subpial transsections) or curative, aiming at a suppression of the epileptogenic focus through a resective or a disconnective surgical procedure. We have operated on a population of 148 children and infants between 3 months and 10 years of age during the years 1993 to 2001: In the infant group, dysplastic lesions represented the predominant etiology. In the age group of 3 to 10 years, the underlying pathology was more distributed (dysplastic lesions, epileptogenic tumors, Rasmussen's encephalitis, hypothalamic hamartomas). Extratemporal focal resections required, in the majority of cases, an invasive preoperative exploration and hemispherotomy was frequent in this group (44 p. 100). Sixty-seven percent of the children were seizure-free, with an acceptable morbidity rate, primarily due to shunt placement following hemispherotomy in children with dysplastic lesions. Our preliminary results concerning quality of life and socialization are encouraging and favor surgical treatment at an early stage.

Behavioural epileptic seizures: a clinical and intracranial EEG study in 8 children with frontal lobe epilepsy.

We report on eight children who underwent prolonged invasive video-EEG recording (IC-EEG) for intractable frontal lobe epilepsy and whose seizures consisted of behaviour changes. Seizures were recorded on a BMSI computer with 128 channels connected to the Gotman software of a stellate system; their identification was made both clinically and by automatic detection of paroxysmal electrical events. Behavioural epileptic seizures (BES) consisted of various clinical signs comprising mood change, sudden agitation, unexpected quietness, and subtle change of awareness or awakening. In 2 patients, seizures consisted in repetitive movements that we referred to as epileptic stereotypes. BES came from the prefrontal areas of the brain. Most of them were overlooked or misdiagnosed as behavioural manifestations, especially in children with mental deficiency and autistic features. Given the improvement of behaviour and mental functions following surgery, we assume that BES may contribute to generate mental and behavioural dysfunction.

Ictal SPECT in children with epilepsy: comparison with intracranial EEG and relation to postsurgical outcome.

In order to validate the ability of ictal single photon emission computed tomography (SPECT) to localize the epileptogenic zone (EZ) in children, we compared in 20 patients aged from 10 months to 17 years (mean 6.5 years) the topography of the area of increased ictal perfusion (IPA), determined on the basis of ictal minus interictal scan values, with that of the EZ determined by intracranial EEG recordings and assessed its relationship with the postsurgical outcome. Eighteen patients had symptomatic epilepsy and 10 had extratemporal epilepsy. All patients except one had an ictal injection (mean time lag from clinical seizure onset was 18 s). Ictal and interictal SPECT images were successively co-registered, normalized, subtracted, smoothed and superimposed on MRI. All patients with ictal injection exhibited one or several IPAs. The topography of the 'highest' IPA, i.e. the maximal cerebral blood flow (CBF) change between ictal and interictal SPECT, significantly colocalized with the site of onset of the discharge, and that of the lower IPAs with that of the area of propagation (P < 0.0001). At a threshold of 30% of the maximal CBF change, the IPAs detected the onset of the discharge with a sensitivity of 0.80 and a specificity of 0.70. The highest IPA localized the EZ in 12 out of 15 patients. In the three others it missed the EZ and showed the area of propagation because of rapid seizure propagation or of infraclinical seizure onset. Among the patients with favourable surgery outcome, the highest IPA colocalized with the resected area in 70% of cases. Ictal SPECT could therefore plays an important role as a non-invasive presurgical method of investigation by optimizing the placement of intracranial electrodes, thus improving the postsurgery outcome of paediatric partial epilepsy.