OBJECTIVE:There is currently scarce data on the electroclinical characteristics of epilepsy associated with synapsin 1 (SYN1) pathogenic variations. We examined clinical and electro-encephalographic (EEG) features in patients with epilepsy and SYN1 variants, with the aim of identifying a distinctive electroclinical pattern. METHODS:In this retrospective multicenter study, we collected and reviewed demographic, genetic, and epilepsy data of 19 male patients with SYN1 variants. Specifically, we analyzed interictal EEG data for all patients, and electro-clinical data from 10 epileptic seizures in 5 patients, using prolonged video-EEG monitoring recordings. Inter-ictal EEG functional connectivity parameters and frequency spectrum of the 10 patients over 12 years of age, were computed and compared with those of 56 age- and sex-matched controls. RESULTS:The main electroclinical features of epilepsy in patients with SYN1 were (1) EEG background and organization mainly normal; (2) interictal abnormalities are often rare or not visible on EEG; (3) more than 60% of patients had reflex seizures (cutaneous contact with water and defecation being the main triggers) isolated or associated with spontaneous seizures; (4) electro-clinical semiology of seizures was mainly temporal or temporo-insulo/perisylvian with a notable autonomic component; and (5) ictal EEG showed a characteristic rhythmic theta/delta activity predominating in temporo-perisylvian regions at the beginning of most seizures. Comparing patients with SYN1 to healthy subjects, we observed a shift to lower frequency bands in power spectrum of interictal EEG and an increased connectivity in both temporal regions. INTERPRETATION:A distinct epilepsy syndrome emerges in patients with SYN1, with a rather characteristic clinical and EEG pattern suggesting predominant temporo-insular involvement. ANN NEUROL 2024.

Objectives In patients with mesial temporal lobe epilepsy (mTLE) and normal MRI, anterior temporal lobectomy sparing the hippocampus might be considered because of the risk of post-operative memory deficit. However, it is unclear whether some patients with normal MRI and non-invasive EEG and semiological pattern highly suggestive of mesial temporal seizures demonstrate a seizure onset network sparing the hippocampus, potentially warranting surgery. Methods A retrospective study of 17 patients with mTLE epilepsy and normal MRI who underwent SEEG. Only patients whose non-invasive presurgical data suggested an unilateral mesial temporal epileptogenic zone (EZ), as defined by combination of ictal semiology and ictal EEG during scalp video-EEG, were included. SEEG data were analyzed using both visual and quantitative approaches. Two EZ organization were defined: (i) EZ involved the hippocampus at the onset of the ictal discharge (HIP group): (ii) patients in whom a delay>1sec was observed between the seizure onset and the involvement of the hippocampus (nHIP group). Non-invasive clinical and functional imaging data, as well as post-operative outcomes, were compared across groups. Results Eleven patients were included in HIP group and 6 in the nHIP group. In the nHIP group, the maximal epileptogenicity was in the amygdala in five patients and in the entorhinal cortex in one. The hippocampus normalized interictal spiking activity was not different between groups. None of the patients characteristics collected during the non-invasive presurgical workup was associated with the SEEG-based organization of the EZ. Twelve patients underwent a surgical resection, including temporal cortectomy sparing hippocampus in six. Seizure and neuropsychological post-operative outcomes were similar. Conclusion In patients with MRI-normal mTLE, SEEG should be included in the surgical decision-making process because seizure organization cannot be predicted from non-invasive investigations. When hippocampus is not included in the EZ, temporal resection sparing the hippocampus can be considered.

Abstract IntroductionEpilepsy is a major feature of multiple types of lesional epilepsy (LE) and non-lesional (NLE). However, there is an important heterogeneity in the use of such nomenclature. A favorable outcome is correlated to an identifiable cause and the extent of resection. Here, we evaluate the use of intraoperative MRI (iMRI) combined with neuronavigation, in improving the complete and/or adequate resection rates in patients with intractable epilepsy of various etiologies. We further discuss whether this results in better clinical outcomes. Material and methodsA medical librarian performed a comprehensive review of literature, searching the Medline®, Embase®, Index Medicus® and Cochrane® databases. Two reviewers independently applied following inclusion criteria: reports of at least two cases of any age undergoing microsurgical resection for epilepsy surgery, the use of iMRI as mandatory for intraoperative assessment, evaluation of LE versus NLE, describing if second resection after iMRI was necessary, detailing the extent of resection, qualitative reports of seizure outcome (Engel class). ResultsWe report 15 studies, encompassing 867 patients, with various types of LE and NLE. Most common surgical indications for the use of iMRI in LE were dysembrioplastic neuroepithelial tumor (DNET) and gangliogliomas, cavernomas, hypothalamic hamartomas, or primary brain tumors. For NLE these were sclerosis, dysplasia, ischemia and gliosis, which are further separately detailed in a supplementary material. With the use of iMRI, the rate of complete or adequate resection significantly increased from 74.8% (95% confidence interval (CI) 67.3-82.3%) to 97.6% (95% confidence interval (CI) 95.8-99.3%). Hundred and twenty-six cases out of 820 underwent additional resection after iMRI, for an overall rate of 24.4% (range 16.9-31.8). This was translated in an overall clinical benefit with 77.1% (95% CI 71-83.2%) Engel class I at last follow-up (p<0.001).DiscussionIntraoperative MRI might improve the extent of resection in LE and NLE. This might translate into a favorable clinical outcome (Engel class I as high as 77%). This is fairly comparable to what one expects in temporal lobe epilepsy surgery. We suggest that iMRI is beneficial when proposed in selected cases of intractable epilepsy. However, the reader should take into account that such data are not provided by randomized controlled trials and that such conclusions should be carefully interpreted.

Background and purpose. - Hospitals admitting acute strokes should offer access to mechanical thrombectomy (MT), but local organisations are still based on facilities available before MT was proven effective. MT rates and outcomes at population levels are needed to adapt organisations. We evaluated rates of MT and outcomes in inhabitants from the North-of-France (NoF) area. Method. - We prospectively evaluated rates of MT and outcomes of patients at 3 months, good outcomes being defined as a modified Rankin scale (mRS) 0 to 2 or like the pre-stroke mRS. Results. - During the study period (2016-2017), 666 patients underwent MT (454, 68.1% associated with intravenous thrombolysis [IVT]). Besides, 1595 other patients received IVT alone. The rate of MT was 81 (95% confidence interval [CI] 72-90) per million inhabitants-year, ranging from 36 to 108 between districts. The rate of IVT was 249 (95% CI 234-264) per million inhabitants-year, ranging from 155 to 268. After 3 months, 279 (41.9%) patients who underwent MT had good outcomes, and 167 (25.1%) had died. Patients living outside the district of Lille where the only MT centre is, were less likely to have good outcomes at 3 months, after adjustment on age, sex, baseline severity, and delay. Conclusion. - The rate of MT is one of the highest reported up to now, even in low-rate districts, but outcomes were significantly worse in patients living outside the district of Lille, and this is not only explained by the delay. (C) 2019 Elsevier Masson SAS. All rights reserved.

Chez certains patients épileptiques fragiles, la prise orale des traitements peut s’avérer difficile et leur administration intraveineuse apparaître trop invasive. Dans ce cas, l’administration sous-cutanée (SC) peut être envisagée. Nous proposons une protocolisation de l’utilisation SC du lacosamide et un retour d’expérience sur le cas de 4 patients hospitalisés aux soins palliatifs du CHU de Lille. Une protocolisation d’administration du lacosamide SC a été rédigée. L’osmolalité de solution de lacosamide aux différentes concentrations usuelles (selon le RCP du produit) a été mesurée via un osmomètre Radiometer : pur à 100 mg/mL, 4 mg/mL (200 mg/50 mL) et 6 mg/mL (300 mg/50 mL) dans du NaCl 0,9 %. Le pH a été mesuré via un pH-mètre sur ces différentes solutions. Une consultation de la littérature (MeSH Term) de l’utilisation du lacosamide SC a été effectuée. À toutes les concentrations testées, pH et osmolalité étaient compatibles avec une administration SC. Les quatre patients présentaient des troubles de vigilance rendant l’administration orale impossible et une utilisation par voie IV trop contraignante. Pour 3/4, le lacosamide a été relayé de per os à SC. La tolérance locale fut bonne (4/4) à 4 mg/mL même lors d’une administration pendant 7 jours. Après amélioration clinique, un patient a repris le lacosamide per os. La littérature ne rapporte l’utilisation du lacosamide SC qu’au travers d’un case report dans lequel l’administration rapide non diluée a été rapportée et bien tolérée. Une administration plus lente est préconisée dans le protocole pour améliorer la tolérance (1 mL/min). Nous n’avons pas évalué l’efficacité du traitement. L’administration SC de lacosamide a amélioré le confort des 4 patients. Les bolus n’ont jamais été essayés mais semblent réalisables (lacosamide non dilué). Des dosages sanguins permettraient de juger l’efficacité.

The limbic system has well-known functions in the regulation of human emotions and behaviour in general and sexual behaviour in particular. However, it is not clear which components of the limbic system are involved in orgasmic feelings. Although orgasmic aura can be elicited by direct electrical stimulation of the right mesial temporal lobe, the location of spontaneous and isolated orgasmic auras have not yet been reported in the literature. Here, we report on the first case of spontaneous orgasmic aura associated with a discharge in the right amygdala, following an investigation with depth electrodes in a woman with temporal lobe epilepsy. Her ictal orgasmic feeling reportedly felt the same as her physiological orgasms. This case sheds light on the amygdala's key role in human sexual function.

Objective: To compare the diagnostic accuracy of standard (st) and long-term video (lt) EEG in elderly patients with suspected non-convulsive seizures. Methods: Over a 12-month period, we prospectively included all elderly (over-65) hospitalized patients having undergone lt-EEG for suspected non-convulsive seizures (n = 43). st-EEG was defined as the first 20 min of each lt-EEG. We recorded the patients' clinical and imaging characteristics and final diagnosis and assessed the respective diagnostic values of st-EEG and lt- EEG.Results: Epileptiform discharges were detected on standard EEG in only 7% of patients and in 28% of patients on Lt-EEG (p = 0.004). Non-convulsive seizures were recorded in 1 case vs. 4, respectively. Nine of 40 negative standard EEG showed later epileptiform activities. The median time to occurrence of the first epileptiform activities was 46.5 min (interquartile range: 36.5-239.75 min). Epileptiform activity occurred during sleep only in 33% patients with a negative st-EEG. Dementia was associated with a positive lt-EEG (p:0.047).Conclusion: Lt-EEG was clearly superior to standard EEG for detecting epileptiform activity in elderly when suspecting non convulsive seizures. Significance: St-EEG has a low diagnostic yield in elderly patients with suspected non-convulsive seizures and so lt-EEG is preferable in this situation. (C) 2016 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Le lacosamide est indiqué dans la prise en charge des crises partielles avec ou sans généralisation secondaire, mais l’usage de la forme IV dans les situations d’urgence reste mal codifié. Identifier les indications ainsi que les modalités d’administration (bolus et/ou titration, posologie, durée) du lacosamide IV prescrites au CHRU de Lille chez les patients adultes, tous services confondus. Il s’agit d’une étude prospective, observationnelle, non interventionnelle et monocentrique, menée durant 6 mois. Tous les services du CHRU de Lille ont été inclus. Les caractéristiques des patients (âge, sexe, poids), les indications, les posologies (bolus et/ou titration), la durée de traitement ainsi que les modalités de relai entre les voies IV et per os ont été relevés. Trente-huit patients ont été inclus (sex-ratio 0,73). L’âge médian est de 68 ans [39 ; 93] et le poids médian de 72 kg [47 ; 105]. Parmi les patients, 55,3 % étaient des épileptiques connus. L’administration IV du lacosamide concernait 13 états de mal épileptique partiel (34,2 %), 16 crises répétées (42,1 %) et 9 cas de voie orale impossible (23,7 %). Seuls 13,2 % des patients ont reçu un bolus de 200 mg de lacosamide et 57,9 % ont bénéficié d’un relai per os. Un tiers des prescriptions de lacosamide IV concernaient des patients en état de mal épileptique partiel, pour lequel il n’existe pas de recommandation formalisée sur l’utilisation du lacosamide par voie IV. Notre étude a mis en exergue une hétérogénéité des pratiques entre les services de soins, notamment sur les doses utilisées en bolus et les modalités de relai per os. Le lacosamide administré par voie IV pourrait avoir sa place dans la stratégie de prise en charge de l’état de mal épileptique partiel, mais une harmonisation des pratiques s’avère indispensable.

L’EEG continue chez l’enfant et le nouveau-né connaît, comme chez l’adulte, ces dernières années un développement croissant tout particulièrement chez le patient gravement malade, très fragile [3]. Un consensus a été établi entre 17 centres de neurologie, pédiatrie ou d’épilepsie aux États-Unis en 2015 sur les indications de cette technique chez ces patients adultes comme enfants [1]. En effet, ces patients présentent, entre autres, un risque accru de crises qui sont essentiellement infracliniques en particulier après arrêt cardio-respiratoire, dans les premières 24 heures ; le début en dehors des heures ouvrables représentant 1 cas sur 2. Une evidence based review publiée en 2006 en Amérique du Nord, dénote l’intérêt du monitoring EEG vidéo pour la prise en charge de l’adulte car montre une mortalité inférieure pour une durée d’enregistrement et un coût identiques aux EEG standards. Les recommandations Françaises de 2014 soulignent l’importance des enregistrements continus chez l’enfant dans l’état de mal convulsif pour détecter des crises infracliniques surtout dans l’état de mal occasionnel. L’étude prospective de Lan et al. [2] relève l’importance de la vidéo-EEG continue par rapport à l’EEG continue sans vidéo en particulier dans les 72 premières heures après le début du coma pour la détection de ces crises infracliniques et ainsi contrer l’atteinte cérébrale secondaire à ces crises infracliniques. On élimine aussi tout artefact environnemental, de même le monitoring EEG vidéo présente une grande utilité pour l’évaluation pronostique par la réactivité aux stimuli. On peut ainsi évaluer selon ces études la profondeur du coma et guider vers un mauvais pronostic sur l’aspect et la réactivité de l’EEG, et une mauvaise architecture du sommeil. La plasticité cérébrale chez l’enfant incite à une intervention précoce pour améliorer le pronostic. Quelle est la faisabilité du monitoring EEG vidéo en France en 2016 ? À travers des exemples, différentes techniques disponibles de traitement du signal recueilli seront évoquées : aEEG, DSA, évaluation du « seizure burden »… ainsi que les limites de ces données en comparaison avec la lecture directe de l’EEG conventionnel. L’organisation médicale et paramédicale des unités de neurophysiologie clinique pour la pratique du monitoring EEG vidéo dans les heures ouvrables ou non sera discutée. La lecture de l’EEG, conventionnel ou non, sur place ou par prise de main à distance pose un problème organisationnel. Cette lecture doit-elle aboutir ou non à un compte rendu standardisé de l’EEG conventionnel toutes les 12 heures pour des adaptations thérapeutiques et des évaluations pronostiques rapides ? Comment valoriser et faire reconnaître cette activité ? Par une différenciation des actes technique et de lecture et par une évaluation du temps passé pour chaque acte ? Une enquête initiée par la SNCLF sur ces derniers points paraît souhaitable.

La prise en charge des patients épileptiques connus est une problématique quotidienne dans les services d’urgences. Nous proposons ici une utilisation raisonnée et pertinente des examens complémentaires (électroencéphalogramme et imagerie cérébrale) en fonction de différentes situations cliniques.

Background and purpose: Seizure is a frequent reason of admission in emergency department (ED) but little is known about the proportion and the characteristics of known epileptic patients (KEPs) who used emergency services.Methods: Over a 12-month period, we prospectively recruited adults admitted for seizure to a tertiary hospital ED. For KEPs, clinical epilepsy features and characteristics of the admission were collected.Results: Of the 60,578 ED admissions, 990 were related to seizure; 580 of these admissions concerned 448 different KEPs (257 males; median age: 44); 339 were residents in the health district. Epilepsy was structural/metabolic in 268 (59.8%) patients, genetic in 44 (9.8%) and unknown/undetermined in 136 (30.3%); 218 (48.7%) patients were under a single antiepileptic drug and 135 (30.1%) were followed by an epileptologist. Of the 580 KEP admissions, 440(75.8%) concerned patients who had called the emergency medical assistance number, 252 (43.4%) with a discharge diagnosis of usual seizure and 43 (7.4%) of a status epilepticus. Half the KEPs were discharged without hospitalization. We estimated that 9.0% of KEPs residing in the district had used the ED during the period.Conclusion: Proportion of KEPs using ED is high. Most of the admissions concerned usual seizures suggesting that staff training and educational programmes for patients and for their relatives need to be improved. The organization of the prehospital and of the emergency medical services should also be adjusted to this specific need. Further research should be conducted to optimize the seizure care pathway for KEPs. (C) 2015 Elsevier B.V. All rights reserved.

OBJECTIVE: Although patients presenting seizures are commonly admitted in the emergency department (ED), the specific use of resources for known epileptic patients (KEP) is rarely studied. DESIGN/METHODS: All consecutive KEP admitted for seizures at our academic ED were included prospectively from November 2011 to November 2012.We collected pre and intra hospital data including clinical features, informations about management and use of resources. Hospitalized patients or outpatients for whom a brain imaging or/and an electroencephalogram were performed in ED were considered as users of resources. RESULTS: Among the 60 578 admissions in ED, 580 files (1%) concerned 451 KEP (344 males; median age 43 years, interquartile range (IQR)15-91years). Four hundred thirty nine (76%) patients utilized emergency medical system (EMS) call centre and 65 (11%) received therapeutics before the admission. One hundred fifty nine (27%) patients were assessed by brain imaging and 91(16%) by EEG. The median ED length of stay in ED was 5h32 (IQR 4:05-8:12 ). At the end of ED management, 50% of patients were hospitalized with a median length of stay of 5 days (interquartile range 2-9 days). Two hundred fifty-five (43%) patients were admitted for usual seizure without additional investigations excepting a blood test. Patients no users of resources were younger, more frequently men, followed by a specialist of epilepsy and admitted for usual seizure (p<0.005). The use of EMS did not differ between patients associated with a consumption of resources and the others. CONCLUSIONS: The admission of KEP was associated to a high amount of resources. Our results suggest that some ED visits could be avoid in a large number of patients. Further studies should be performed in order to optimize the seizure care pathway for KEP. Study Supported by:

OBJECTIVE: To evaluate the use of the electroencephalogram (EEG) and its relevancy in known epileptic patients admitted in emergency department (ED) for seizure(s). DESIGN/METHODS: All consecutive patients admitted for seizures at our academic ED were included prospectively, categorized as known epileptic patient and unknown epileptic patient from November 2011 to November 2012. Biographic and clinical features, characteristics of epilepsy, information about EEG and its relevance were collected. The EEG was considered useful when it provides valuable information that could improve the patient care management. RESULTS: 580 admissions in ED were analyzed for 451 patients (mean age 46 ± 20 years). Patients had structural epilepsy in 59% and were treated in 42% at least with 2 anti-epileptic drugs. Patients were admitted for usual seizures in 43.5%. An EEG was performed in 44.6%. EEG was significantly more ordered in aged patients (p<0.05) and when the epileptic syndrome was not clearly identified (p<0.05). Patients admitted for a usual seizure were less likely to benefit of EEG (p<0.05), however EEG was still performed in 17%. Only 7% of these EEG were considered as useful. Considering all the EEG performed, 24% were considered as useful. The relevance of the EEG was greater in non convulsivant status epilepticus suspicion (p<0.05), when a seizure occurs in ED (p<0.05) and in case of EEG performed within the 6 hours of the ED admission (p<0.05). CONCLUSIONS: EEG can be useful in ED for patients with known epilepsy in clearly defined situations. If EEG appears relevant in the NCSE management, it seems less useful in case of usual seizures recurrence. These results suggest that recommendations on EEG use in known epileptic patients should be defined in order to optimize the neurophysiological resources and the patient care management in ED. Study Supported by: Disclosure: Dr. Tyvaert has nothing to disclose. Dr. Girot has nothing to disclose. Dr. Lenne has nothing to disclose. Dr. Williate has nothing to disclose. Dr. Szurhaj has nothing to disclose. Dr. Leblanc has nothing to disclose. Dr. Chochoi has nothing to disclose. Dr. Hubert has nothing to disclose. Dr. Derambure has nothing to disclose.

OBJECTIVE: To estimate the frequency of emergency department (ED) visits and the modalities of admission for known epileptic patients (KEP) as well as their profiles. DESIGN/METHODS: Between November 2011 and November 2012, we prospectively recruited adults with a seizure diagnostic in ED and intensive care unit (ICU) in a tertiary hospital of a large-sized French city. Patients with established epilepsy were identified. We collected clinical features, characteristics of epilepsy, and modalities of admission. In one year, the number of patients affected by an active epilepsy was estimated at 3765 in our district (584400 inhabitants), based on age-adjusted prevalence value provided by the literature (5.4/1000 inhabitants; Picot et al, 2008). RESULTS: Among the 60 578 admissions in ED, 989 (95% confidence interval [CI]: 1.6% [1.5%-1.7%]) were linked to seizure motive and 580 files (0.96% [0.88%-1.03%]) concerned 451 KEP (344 males; median age 43 years SD 20.3Y). Among these 451 patients, 339 depended on our district. The frequency of ED visit for KEP from our district was 9.0% per year [8.1%-9.9%]. Among the 580 files, 437 (75.3% [71.8%-78.9%]) involved patients with symptomatic partial epilepsy and 247 (42.6% [38.6%-46.6%]) involved patients treated at least with 2 anti-epileptic drugs. One hundred seventy-five patients were followed by a specialist of epilepsy (30.2% [26.4%-33.9%]). Concerning the pre-hospital management, 439 (75.7% [72.2%-79.2%]) patients utilized emergency medical system call centre and 413 (71.2% [67.5%-74.9%]) came from their place of residence, 50 (8.6% [6.3%-10.9%]) patients were admitted directly in ICU. Seventy-seven (13.3% [10.5%-16.0%]) admissions were associated with traumatism or head injury, 299 (51.6% [47.5%-55.6%]) patients were admitted for usual seizures and 44 (7.6% [5.4%-9.7%]) had a final diagnosis of status epilepticus. CONCLUSIONS: The frequency of ED visit for KEP is high and concerned in at least one out of two cases patients with usual seizure. Further studies should be performed in order to optimize the seizure care pathway for KEP. Study Supported by: Disclosure: Dr. Girot has nothing to disclose. Dr. Derambure has nothing to disclose. Dr. Chochoi has nothing to disclose. Dr. Leblanc has nothing to disclose. Dr. Goldstein has nothing to disclose. Dr. Szurhaj has nothing to disclose. Dr. Williate has nothing to disclose. Dr. Lenne has nothing to disclose. Dr. Wiel has nothing to disclose. Dr. Hubert has nothing to disclose. Dr. Richard has nothing to disclose. Dr. Tyvaert has nothing to disclose.