Objective. - The aim of this study was to determine the success rate of sEEG in locating the epileptogenic zone (EZ) in patients with pharmaco-resistant epilepsy. Secondary objectives were to analyze sEEG-related morbidity and outcomes for post-sEEG thermocoagulation and cortical resection. Methods. - Data were collected on 49 sEEGs from 46 consecutive patients between 2010 and 2018. Following sEEG, either resective or palliative surgery with vagus nerve stimulation was performed. In 8 patients, EZ thermocoagulation was performed before EEG leads were withdrawn. Outcomes were collected based on the Engel and ILAE outcome scales. Results. - sEEG was contributive in 45 of 49 recordings, with a success rate of 92% in locating the EZ. Minor complications, such as transient neurologic deficit and electrode implantation failures, occurred in 6%. One major complication occurred, with death due to atypical late hematoma. Thermocoagulation was performed in 8 patients and stopped or significantly reduced seizure frequency in 7 (88%). Outcome of surgical resection (n=33) was good, with 20 (61%) seizure-free patients and 32 (97%) with definite improvement. Conclusions. - Our findings suggest that sEEG is an effective technique for EZ location in patients with drug-resistant epilepsy. sEEG was contributive in up to 92% of patients, allowing thermocoagulation and/or surgical resection that resulted in seizure-freedom in two-thirds and seizure-reduction in one-third of cases. This study highlights the need for strict selection of implantation candidates, with strong initial hypothesis as to EZ location. (C) 2020 Elsevier Masson SAS. All rights reserved.

Purpose: Surgical planning of depth electrode implantation in stereo-electro-encephalography (SEEG) routinely uses magnetic resonance imaging (MRI) alone. Accurate visualization of arteries and veins in the vicinity of the electrode is essential to plan a safe trajectory to presumably reduce the risk of intracranial bleeding. The goal of this study was to compare multidetector row computerized tomographic angiography (MDCTA) with MRI for the visualization of vessels along each planned trajectory in patients who undergo SEEG.Materials and methods: Ten consecutive patients who were scheduled to undergo SEEG procedure were included. T1-weighted gadolinium-chelate enhanced MR sequence, stereotactic MDCT and MDCTA were performed after fixation of Leksell's frame. For each of the 106 planned stereotactic trajectories, the number of vessels in a 4.0 mm diameter circle around the trajectory from the dura mater to the target that were visible on MDCTA were compared to that of visible vessels in the same areas on MRI.Results: Ten vessels (10/106; 9.4%) were seen on MRI and 66 (66/106; 62.3%) on MDCTA (P < 0.0001). All vessels visible on MRI were visible on MDCTA. The difference in number of visible vessels between the two techniques remained significant for the different lobes (i.e., frontal lobe, temporal lobe and parieto-occipital lobe).Conclusion: MDCTA enabled visualization of more vessels than MRI based SEEG. MDCTA may help neurosurgeons better define the trajectory of the electrode and reduce the risk of intracranial bleeding. (C) 2016 Published by Elsevier Masson SAS on behalf of the Editions francaises de radiologie.

Circa 500 accidenti vascolari cerebrali hanno luogo ogni anno in Francia nel bambino: infarti arteriosi, trombosi venose cerebrali ed emorragie cerebrali. La clinica è, il più delle volte, evocatrice. Talvolta i segni possono essere poco specifici, e sono l’anamnesi, l’esame clinico e il contesto clinico sottostante che fanno sospettare la diagnosi. Le eziologie sono spesso differenti da quelle dell’adulto, richiedendo un bilancio eziologico specifico, per esempio per le arteriopatie cerebrali transitorie, la causa più frequente degli infarti arteriosi cerebrali del bambino. La gestione in fase acuta degli infarti arteriosi si basa sul mantenimento dell’omeostasi e dell’emodinamica e sul riconoscimento di cause che richiedono un trattamento specifico urgente, eventualmente trombolitico. Le trombosi venose cerebrali sono un’indicazione all’anticoagulazione. Può essere necessario il trattamento di una causa associata, in particolare infettiva. Sono spesso riscontrati una patologia sottostante o un fattore favorente. Le emorragie cerebrali devono essere gestite in collaborazione con un’equipe di neurochirurgia. Le malformazioni arterovenose e i disturbi dell’emostasi sono da ricercare, in quanto a rischio di recidiva dell’emorragia. La diagnostica per immagini ha un posto importante nella strategia diagnostica degli accidenti vascolari cerebrali del bambino. La strutturazione di filiere di assistenza per questi bambini è un obiettivo per il miglioramento della loro gestione.

Le purpura thrombopénique immunologique (PTI) est une maladie hémorragique liée à la production d’auto-anticorps dirigés contre certaines glycoprotéines membranaires plaquettaires. La survenue de thromboses n’est pas un événement classique au cours du PTI. Cependant, de récentes études ont montré une augmentation du risque thrombotique dans cette situation. Nous rapportons le cas d’une enfant de sept ans ayant présenté une thrombose veineuse cérébrale étendue révélatrice d’un PTI aigu. Au cours du PTI, les thromboses pourraient être favorisées par la libération de microparticules plaquettaires pro-thrombotiques et par l’activation plaquettaire secondaire à l’interaction entre les auto-anticorps et les glycoprotéines plaquettaires. L’augmentation du risque thrombo-embolique devrait être prise en compte chez tous les sujets atteints de PTI, y compris chez les enfants.

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. (C) 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Spinal cord stimulation (SCS) has been demonstrated to be an effective treatment for postoperative persistent leg pain after spine surgery, but treatment of the back pain component remains much more difficult, as it comprises mixed neuropathic and mechanical pain mechanisms. Moreover, these patients could present damaged tissues at the site of SCS lead implantation as a result of previous spine surgery. It can therefore be logically assumed that minimizing the surgical invasiveness of SCS implantation would be beneficial for these patients. Several studies have demonstrated the value of Minimal Access Spine Technologies (MAST) in spine surgery, but only a few case reports have been published concerning the use of MAST techniques for SCS. Therefore, we were prompted to conduct a second ESTIMET ancillary study to prospectively analyse the potential impact and benefits of MAST technique during SCS lead implantation versus an open surgical approach.This is a multicentre, comparative, ancillary study conducted in 61 patients among the 115 enrolled patients ESTIMET study. One arm comprises patients undergoing multicolumn lead implantation via a Conventional Open Approach (COA) and the other arm comprises patients implanted by a MAST approach. Patients will be followed for 12 months after lead implantation. The following data will be collected: elevation of muscle enzymes (serum CPK), scar size, blood loss, infection rate, operating time and global, leg, back and scar NPRS.The first patient of this ancillary study was enrolled on 21 May 2012 and recruitment has now been achieved. Primary endpoint findings are expected to be available in 2015.Minimally invasive techniques have now been used for spine surgery for the past 12 years, and could also be useful in the context of SCS lead implantation, especially in patients with chronic back pain prior to implantation.La stimulation médullaire épidurale (SME) a démontré son efficacité dans le traitement des radiculalgies chroniques postopératoires. Cependant, le traitement de la composante lombaire reste beaucoup plus difficile car premièrement celle-ci présente des mécanismes douloureux mixtes, neuropathiques et mécaniques, et deuxièmement ces patients peuvent présenter des lésions tissulaires au niveau du site pressenti pour l’implantation de l’électrode, résultant de chirurgies rachidiennes antérieures. On peut donc logiquement supposer que le fait de minimiser le caractère invasif de l’implantation de la SME pourrait être bénéfique pour ces patients. Plusieurs études ont démontré l’intérêt des techniques mini-invasives (MAST) dans les chirurgies du rachis, mais seuls quelques cas ont été publiés concernant l’utilisation des techniques MAST pour la SME. Nous avons décidé de mener une seconde étude ancillaire ESTIMET afin d’analyser l’impact et les avantages potentiels de la technique MAST lors de l’implantation d’électrode de SME par rapport à une approche chirurgicale à ciel ouvert.Il s’agit d’une étude ancillaire multicentrique, comparative réalisée chez 61 patients parmi les 115 patients inclus dans l’étude ESTIMET. Un bras comprend des patients implantés d’une électrode multi-colonnes par une approche conventionnelle à ciel ouvert et l’autre bras comprend des patients implantés par une approche MAST. Les patients seront suivis pendant 12 mois après l’implantation de l’électrode. Les données suivantes seront collectées : élévation des enzymes musculaires (CPK sérique), taille de la cicatrice, perte sanguine, taux d’infection, temps opératoire et EVN radiculaire, lombaire et cicatricielle.Le premier patient de cette étude ancillaire a été inclus le 21 mai 2012. Le recrutement est maintenant finalisé. Les résultats finaux devraient être disponibles d’ici début 2015.Les techniques de chirurgie mini-invasive sont maintenant utilisées depuis plus de 12 ans en chirurgie rachidienne. Leur intérêt semble réel pour l’implantation d’électrodes de stimulation médullaire multi-colonnes, en limitant probablement le traumatisme opératoire chez ces patients, dont le dos est fragilisé. Ceci nécessite d’être confirmé par des études randomisées.

Les accidents vasculaires cérébraux (AVC) sont une réalité en pédiatrie et, quel que soit l’âge du patient, leur prise en charge doit mettre en œuvre les moyens adaptés. L’hospitalisation en unité de soins continus ou de réanimation est recommandée. Les traitements symptomatiques sont tous applicables à l’enfant et recommandés, qu’il s’agisse du maintien de l’homéostasie ou de l’échange transfusionnel des drépanocytaires. Les traitements spécifiques tels que la thrombolyse ou la thrombectomie mécanique ne sont pas recommandés chez l’enfant. Les adolescents peuvent cependant en bénéficier au cas par cas après discussion multidisciplinaire et peuvent être pris en charge en unité neurovasculaire d’adultes. Les indications chirurgicales sont adaptées de celles de l’adulte. La prise en charge des thromboses veineuses cérébrales est identique à celle de l’adulte. Le dialogue multidisciplinaire entre neuropédiatre et médecin(s) d’adulte est préconisé afin de permettre une prise en charge optimale.

Stroke in children is not rare. Although there are no randomized trials on childhood stroke, except in sickle cell disease patients, several international guidelines have described quality criteria for stroke management in children. Age-adapted management is required, involving collaboration with a pediatric neurologist and hospitalization in a pediatric intensive care or continuous care unit. All symptomatic treatments used in adults can be recommended in children, including homeostasis assessment and maintenance or blood exchange in sickle cell disease patients. Specific treatments such as thrombolysis or mechanical thrombectomy are not recommended in children, except in the framework of clinical trials, but can be beneficial in adolescents. Multidisciplinary decision-making should be the rule in such situations. Adolescents may be managed in adult stroke units. Indications for surgery in children are adapted from adult guidelines. Appropriate management of cerebral venous thrombosis in children is similar to that in adults. The best management possible can be achieved through a multidisciplinary dialogue between the pediatric neurologist and the adult intensivist or neurologist. (C) 2012 Elsevier Masson SAS. All rights reserved.

We report the case of an eight-year-old patient referred by his primary care physician for chronic headache. Bilateral papilledema was observed along with right sixth cranial nerve palsy, leading to the diagnosis of intracranial hypertension. Head CT showed no mass lesion. Lyme serology was positive by both Elisa and Western blot. Anti-Borrelia antibodies were positive in the cerebrospinal fluid, with intrathecal synthesis confirming neuroborreliosis. Clinical response to ceftriaxone and acetazolamide was favorable. Intracranial hypertension is rarely caused by Lyme disease. Ophthalmologists should be aware of this clinical presentation, since the presenting clinical signs may be purely ophthalmologic. In addition, early diagnosis may avoid optic nerve atrophy or disease spread. (C) 2012 Elsevier Masson SAS. All rights reserved.

Anticoagulation is recommended in the acute phase of cerebral venous thrombosis in adults, then for 3-12 months. In children, 2 consensus reports published in 2008 also recommend use of anticoagulants, whereas conclusions diverge for newborns. These consensus reports are based on observational studies, authors' experience, and comparisons with adult pathology. In view of the original studies published since then, the French Society of Pediatric Neurology (Société française de neurologie pédiatrique [SFNP]) wished to update the level of evidence and the knowledge in this domain. The results from the analysis of the literature show that anticoagulation is widely used in pediatrics. It is well-tolerated in children (class I, level of evidence B) and probably in the newborn (class IIa, level of evidence B). In the acute phase of cerebral venous thrombosis, anticoagulation is probably effective in reducing the risk of death in children (class IIa, level of evidence B). It is not possible to draw a conclusion on newborns (class IIb). Over the longer term, anticoagulation is effective in reducing the risk of recurrence (class I, level of evidence B). Since this risk is highly dependent on a number of individual factors (the main ones being the child's age, the cause of the thrombosis, and the kinetics of the sinus recanalization), the duration of anticoagulation should be analyzed individually (class I, level of evidence B). All in all, the convergence of the results, the physiopathologic arguments, and the concordance with the data on adult patients has led to the following recommendations: in the absence of a contra-indication, it is reasonable to propose anticoagulation in the acute phase of cerebral venous thrombosis in children. Prolonging this treatment for 3-6 months is indicated depending on the number of individual factors. In the absence of a contra-indication, anticoagulation may be considered individually in the acute phase of cerebral venous thrombosis in newborns for 6-12 weeks.

Nous rapportons le cas d'une encéphalite auto immune rare chez une jeune fille de 11 ans. Un tableau pseudo démentiel s'est constitué sur 3 mois: coexistaient une confusion, des hallucinations avec alternance d'états catatoniques et d'accès d'agitation. S'y associaient des mouvements anormaux et une dysrégulation thermique. Aucun déficit moteur ou crises convulsives n'étaient notés. L'IRM cérébrale et la ponction lombaire étaient normales en dehors d'un index gamma à 0,75. L'EEG était lent. Au terme d'explorations exhaustives et suite à l'inefficacité des boli de corticoïdes, des Ac anti NMDA (N-Methyl-D-aspartate) on été documentés dans le LCR. C'est une encéphalite limbique avec une présentation neuro-psychiatrique. Dans 80 % des cas, il s'agit d'un syndrome paranéoplasique chez une femme atteinte d'un tératome mature de l'ovaire d'évolution favorable après ablation de la tumeur. De rares cas sont décrits, l'antigène n'ayant été identifié qu'en 2007. Ici, en l'absence de tumeur documentée, un traitement immuno-modulateur a associé des cures d'immunoglobulines polyvalentes, des plasmaphérèses et du rituximab. S'est produit alors une amélioration clinique mais avec persistance de troubles cognitifs invalidants.

Présentation de 4 cas de syndrome de Sneddon apparu avant 1 an. Revue des données cliniques, biologiques et IRM avec ARM complétées par artériographie. Le mode de révélation était univoque par des convulsions suivies d'hémiplégie partiellement régressive. L'IRM au décours immédiat montrait une ischémie étendue récente d'un territoire sylvien superficiel. L'étude artérielle retrouvait des sténoses bilatérales sévères sur la division antérieure des carotides internes pouvant faire discuter un Moya-Moya. Un accident controlatéral survenait de manière rapprochée (moyenne 5 mois). En plus des signes neurologiques les enfants présentaient des signes cutanés (livedo, 4), une HTA sévère (3) avec glomérulopafhie (2) et cardiomyopathie (2), une atteinte digestive (2) et hépatique (1). La biologie retrouvait des anomalies auto-immunes (AC anti muscle lisse, AC anti phospholipides, facteurs anti-nucléaires). L'évolution a été rapidement sévère avec 2 décès et 2 évolutions neurologiques graves malgré les différents traitements immunosuppresseurs, ne permettant pas la réalisation d'un geste neurochirurgical de revascularisation. Un AVC sévère survenant avant 1 an, avec des signes extraneurologiques (livedo, HTA…), doit faire évoquer un syndrome de Sneddon et rechercher une pathologie auto-immune.